Publications by authors named "Andrea Palicelli"

30 Publications

  • Page 1 of 1

Gastrointestinal Manifestations in Systemic Mastocytosis: The Need of a Multidisciplinary Approach.

Cancers (Basel) 2021 Jul 1;13(13). Epub 2021 Jul 1.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, Italy.

Mastocytosis represents a heterogeneous group of neoplastic mast cell disorders. The basic classification into a skin-limited disease and a systemic form with multi-organ involvement remains valid. Systemic mastocytosis is a disease often hard to diagnose, characterized by different symptoms originating from either the release of mast cell mediators or organ damage due to mast cell infiltration. Gastrointestinal symptoms represent one of the major causes of morbidity, being present in 60-80% of patients. A high index of suspicion by clinicians and pathologists is required to reach the diagnosis. Gastrointestinal mastocytosis can be a challenging diagnosis, as symptoms simulate other more common gastrointestinal diseases. The endoscopic appearance is generally unremarkable or nonspecific and gastrointestinal mast cell infiltration can be focal and subtle, requiring an adequate sampling with multiple biopsies by the endoscopists. Special stains, such as CD117, tryptase, and CD25, should be performed in order not to miss the gastrointestinal mast cell infiltrate. A proper patient's workup requires a multidisciplinary approach including gastroenterologists, endoscopists, hematologists, oncologists, and pathologists. The aim of this review is to analyze the clinicopathological features of gastrointestinal involvement in systemic mastocytosis, focusing on the relevance of a multidisciplinary approach.
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http://dx.doi.org/10.3390/cancers13133316DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8269078PMC
July 2021

Risk Assessment in Solitary Fibrous Tumor of the Uterine Corpus: Report of a Case and Systematic Review of the Literature.

Int J Surg Pathol 2021 Jun 28:10668969211025759. Epub 2021 Jun 28.

Department of Obstetrics and Gynecology, ASST Spedali Civili di Brescia, Brescia, Italy.

Solitary fibrous tumor (SFT) is an uncommon fibroblastic tumor occurring preferentially in the pleura, with a variable clinical course. SFT can arise also in numerous extrathoracic sites and very rarely in the female genital tract, with only scarce reports of uterine SFT. We reported a new uterine SFT arising in a 45-year-old woman, and we performed a systematic review of SFT cases of the uterine corpus interrogating the electronic databases PubMed, Web of Science, and Scopus. We identified only 13 patients diagnosed with SFT of the uterine corpus, including our one. Complete clinical workout at disease presentation showed no evidence of extrauterine spread in all cases, except for 1 patient who presented with metastatic disease. Tumor recurrences/metastases occurred in a minority of the patients and were poorly related to clinicopathological risk factors and patients stratification based on different scoring systems. Since the long-term clinical behavior of uterine SFT is limited and poorly predictable, extended follow-up is recommended also for all cases arising in the uterine corpus.
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http://dx.doi.org/10.1177/10668969211025759DOI Listing
June 2021

Solitary vulvar metastasis from early-stage endometrial cancer: Case report and literature review.

Medicine (Baltimore) 2021 Jun;100(22):e25863

Unit of Surgical Gynecol Oncology.

Rationale: Endometrial cancer (EC) is the most common gynecological malignancy in developed countries. It is usually diagnosed at early-stage and presents a favorable prognosis. Conversely, advanced or recurrent disease shows poor outcome. Most recurrences occur within 2 years postoperatively, typically in pelvic and para-aortic lymph nodes, vagina, peritoneum, and lungs. Vulvar metastasis (VM) is indeed anecdotal probably because of the different regional lymphatic drainage from corpus uteri.

Patient Concerns: A 3 cm, reddish, bleeding lesion of the posterior commissura/right labia was found in a 74-year-old woman treated with radical hysterectomy, surgical staging, and adjuvant radiotherapy 1 year before for a grade 2 endometrioid type, International Federation of Gynecology and Obstetrics Stage IB. Vulvar biopsy confirmed the EC recurrence. Pelvic magnetic resonance imaging and positron emission tomography excluded other metastases so VM was radically resected.

Diagnosis: Postoperative histopathology confirmed the diagnosis of grade 2 EC VM.

Interventions: A radical excision of VM was performed.

Outcomes: Patient died from a severe sepsis 27 months after first surgery.

Lessons: Vulvar metastases can show different appearance, occurring as single or diffuse lesions on healthy or injured skin. The surgical approach seems not to influence the metastatic risk, but tumor seeding and vaginal injuries should be avoided. Whether isolated or associated with recurrence in other locations, vulvar metastases imply poor prognosis despite radical treatment. Therefore, any suspected vulvar lesion arisen during EC follow-up should be biopsied and monitored closely, despite that the vulva represents an unusual metastatic site.
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http://dx.doi.org/10.1097/MD.0000000000025863DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8183741PMC
June 2021

What Is New on Ovarian Carcinoma: Integrated Morphologic and Molecular Analysis Following the New 2020 World Health Organization Classification of Female Genital Tumors.

Diagnostics (Basel) 2021 Apr 14;11(4). Epub 2021 Apr 14.

Molecular Pathology Laboratory, IRCCS Azienda Ospedaliero-Universitaria di Bologna/Azienda USL di Bologna, 40138 Bologna, Italy.

Ovarian carcinomas represent a heterogeneous group of neoplasms consisting of separate entities with distinct risk factors, precursor lesions, pathogenesis, patterns of spread, molecular profiles, clinical course, response to chemotherapy, and outcomes. The histologic subtype and the related molecular features are essential for individualized clinical decision-making. The fifth edition of the World Health Organization classification of tumors of the female genital tract divides ovarian carcinomas into at least five main and distinct types of ovarian carcinomas: high-grade serous carcinoma, low-grade serous carcinoma, endometrioid carcinoma, clear cell carcinoma, and mucinous carcinoma. Molecular pathology has improved the knowledge of genomic landscape of ovarian carcinomas identifying peculiar alterations for every histologic subtype. It is well-known that high-grade and low-grade serous carcinomas are separate entities with entirely different morphologic and molecular characteristics. and mutations are typical of high-grade serous carcinoma, whereas and mutations frequently occur in low-grade serous carcinoma. Endometrioid and clear cell carcinomas are frequently associated with endometriosis. Endometrioid tumors are characterized by β-catenin alterations, microsatellite instability, and and mutations, while mutations occur in both endometrioid and clear cell carcinomas. Mucinous carcinomas are uncommon tumors associated with copy-number loss of and alterations and metastasis from other sites should always be considered in the differential diagnosis.
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http://dx.doi.org/10.3390/diagnostics11040697DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8070731PMC
April 2021

Primary effusion lymphoma occurring in the setting of transplanted patients: a systematic review of a rare, life-threatening post-transplantation occurrence.

BMC Cancer 2021 Apr 27;21(1):468. Epub 2021 Apr 27.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100, Terni, Italy.

Background: Primary effusion lymphoma is a rare, aggressive large B-cell lymphoma strictly linked to infection by Human Herpes virus 8/Kaposi sarcoma-associated herpes virus. In its classic form, it is characterized by body cavities neoplastic effusions without detectable tumor masses. It often occurs in immunocompromised patients, such as HIV-positive individuals. Primary effusion lymphoma may affect HIV-negative elderly patients from Human Herpes virus 8 endemic regions. So far, rare cases have been reported in transplanted patients. The purpose of our systematic review is to improve our understanding of this type of aggressive lymphoma in the setting of transplantation, focusing on epidemiology, clinical presentation, pathological features, differential diagnosis, treatment and outcome. The role of assessing the viral serological status in donors and recipients is also discussed.

Methods: We performed a systematic review adhering to the PRISMA guidelines. The literature search was conducted on PubMed/MEDLINE, Web of Science, Scopus, EMBASE and Cochrane Library, using the search terms "primary effusion lymphoma" and "post-transplant".

Results: Our search identified 13 cases of post-transplant primary effusion lymphoma, predominantly in solid organ transplant recipients (6 kidney, 3 heart, 2 liver and 1 intestine), with only one case after allogenic bone marrow transplantation. Long-term immunosuppression is important in post-transplant primary effusion lymphoma commonly developing several years after transplantation. Kaposi Sarcoma occurred in association with lymphoma in 4 cases of solid organ recipients. The lymphoma showed the classical presentation with body cavity effusions in absence of tumor masses in 10 cases; 2 cases presented as solid masses, lacking effusions and one case as effusions associated with multiple organ involvement. Primary effusion lymphoma occurring in the setting of transplantation was more often Epstein Barr-virus negative. The prognosis was poor. In addition to chemotherapy, reduction of immunosuppressive treatment, was generally attempted.

Conclusions: Primary effusion lymphoma is a rare, but often fatal post-transplant complication. Its rarity and the difficulty in achieving the diagnosis may lead to miss this complication. Clinicians should suspect primary effusion lymphoma in transplanted patients, presenting generally with unexplained body cavity effusions, although rare cases with solid masses are described.
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http://dx.doi.org/10.1186/s12885-021-08215-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8077837PMC
April 2021

Non-Muscle Invasive Bladder Cancer with Variant Histology: Biological Features and Clinical Implications.

Oncology 2021 18;99(6):345-358. Epub 2021 Mar 18.

Urology Unit, University of Foggia, Bonomo Teaching Hospital, Foggia, Italy.

Background: The most common bladder cancer (BC) histotype is pure urothelial carcinoma (UC), which may undergo divergent differentiation in some cases. Variant histology (VH) presents along variable morphologies, either single or combined between them or with pure UC. From a clinical standpoint, the vast majority of BC is diagnosed at non-invasive or minimally invasive stages, namely as non-muscle invasive BC (NMIBC). There is a wide range of therapeutic options for patients with NMIBC, according to their clinical and pathological features. However, current risk stratification models do not show optimal effectiveness. Evidence from the literature suggests that VH has peculiar biological features, and may be associated with poorer survival outcomes compared to pure UC.

Summary: In order to describe the biological features and prognostic/predictive role of VH in NMIBC, and to discuss current treatment options, we performed a systematic literature search through multiple databases (PubMed/Medline, Google Scholar) for relevant articles according to the following terms, single and/or in combination: "non-muscle invasive bladder cancer," "variant histology," "micropapillary variant," "glandular differentiation," "squamous differentiation," "nested variant," "plasmacytoid variant," and "sarcomatoid variant." We extracted 99 studies including original articles, reviews, and systematic reviews, and subsequently analyzed data from 16 studies reporting on the outcome of NMIBC with VH. We found that the relative rarity of these forms as well as the heterogeneity in study populations and therapeutic protocols results in conflicting findings overall. Key Messages: The presence of VH should be taken into account when counseling a patient with NMIBC, since it may upgrade the disease to high-risk tumor and thus warrant a more aggressive treatment.
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http://dx.doi.org/10.1159/000514759DOI Listing
June 2021

How Can We Treat Vulvar Carcinoma in Pregnancy? A Systematic Review of the Literature.

Cancers (Basel) 2021 Feb 17;13(4). Epub 2021 Feb 17.

Pathology Unit, Azienda Ospedaliero-Universitaria-Ospedali Riuniti di Foggia, 71122 Foggia, Italy.

According to our systematic literature review (PRISMA guidelines), only 37 vulvar squamous cell carcinomas (VSCCs) were diagnosed during pregnancy (age range: 17-41 years). The tumor size range was 0.3-15 cm. The treatment was performed after (14/37, 38%), before (10/37, 27%), or before-and-after delivery (11/37, 30%). We found that 21/37 (57%) cases were stage I, 2 II (5%), 11 III (30%), and 3 IVB (8%). HPV-related features (condylomas/warts; HPV infection; high-grade squamous intraepithelial lesion) were reported in 11/37 (30%) cases. We also found that 9/37 (24%) patients had inflammatory conditions (lichen sclerosus/planus, psoriasis, chronic dermatitis). The time-to-recurrence/progression (12/37, 32%) ranged from 0 to 36 (mean 9) months. Eight women died of disease (22%) 2.5-48 months after diagnosis, 2 (5%) were alive with disease, and 23 (62%) were disease-free at the end of follow-up. Pregnant patients must be followed-up. Even if they are small, newly arising vulvar lesions should be biopsied, especially in women with risk factors (HPV, dermatosis, etc.). The treatment of VSCCs diagnosed in late third trimester might be delayed until postpartum. Elective cesarean section may prevent vulvar wound dehiscence. In the few reported cases, pregnancy/fetal outcomes seemed to not be affected by invasive treatments during pregnancy. However, clinicians must be careful; larger cohorts should define the best treatment. Definite guidelines are lacking, so a multidisciplinary approach and discussion with patients are mandatory.
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http://dx.doi.org/10.3390/cancers13040836DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7921964PMC
February 2021

Immunohistochemical Biomarkers as a Surrogate of Molecular Analysis in Ovarian Carcinomas: A Review of the Literature.

Diagnostics (Basel) 2021 Jan 29;11(2). Epub 2021 Jan 29.

Pathology Unit, AUSL-IRCCS Reggio Emilia, 42123 Reggio Emilia, Italy.

The term "ovarian carcinoma" encompasses at least five different malignant neoplasms: high-grade serous carcinoma, low-grade serous carcinoma, endometrioid carcinoma, mucinous carcinoma, and clear cell carcinoma. These five histotypes demonstrated distinctive histological, molecular, and clinical features. The rise of novel target therapies and of a tailored oncological approach has demanded an integrated multidisciplinary approach in the setting of ovarian carcinoma. The need to implement a molecular-based classification in the worldwide diagnostic and therapeutic setting of ovarian cancer demanded a search for easy-to-use and cost-effective molecular-surrogate biomarkers, relying particularly on immunohistochemical analysis. The present review focuses on the role of immunohistochemistry as a surrogate of molecular analysis in the everyday diagnostic approach to ovarian carcinomas.
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http://dx.doi.org/10.3390/diagnostics11020199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7911119PMC
January 2021

Intrauterine Infection: An Unusual Cause of Chorioamnionitis and Miscarriage in a Patient with Undifferentiated Connective Tissue Disease.

Diagnostics (Basel) 2021 Feb 4;11(2). Epub 2021 Feb 4.

Clinical Microbiology Laboratory, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, 42122 Reggio Emilia, Italy.

is a Gram-negative coccobacillus belonging to the family. In children less than 4 years old, invasive infection can induce septic arthritis and osteomyelitis, and more rarely endocarditis, meningitis, ocular infections, and pneumonia. In adults, it may be a cause of endocarditis. To date, acute chorioamnionitis (AC) leading to preterm rupture of membranes (PPROM) and miscarriage has never been reported. Herein, we describe a case of intrauterine fetal death (IUFD) at 22 weeks' gestation due to infection occurred in a patient affected by undifferentiated connective tissue disease (UCTD) in lupus erythematosus systemic (LES) evolution with severe neutropenia. was isolated in placental subamnionic swab and tissue cultures as well as fetal ear, nose, and pharyngeal swabs. Placental histological examination showed necrotizing AC and funisitis. In the fetus, neutrophils were observed within the alveoli and in the gastrointestinal lumen. Maternal medical treatment for UCTD was modified according to the invasive infection. In the event of IUFD due to AC, microbiological cultures on placenta and fetal tissues should always be carried out in order to isolate the etiologic agent and target the correct medical treatment.
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http://dx.doi.org/10.3390/diagnostics11020243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7914692PMC
February 2021

Primary Pulmonary B-Cell Lymphoma: A Review and Update.

Cancers (Basel) 2021 Jan 22;13(3). Epub 2021 Jan 22.

Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, 05100 Terni, Italy.

Primary pulmonary B-cell lymphomas (PP-BCLs) comprise a group of extranodal non-Hodgkin lymphomas of B-cell origin, which primarily affect the lung without evidence of extrapulmonary disease at the time of diagnosis and up to 3 months afterwards. Primary lymphoid proliferations of the lung are most often of B-cell lineage, and include three major entities with different clinical, morphological, and molecular features: primary pulmonary marginal zone lymphoma of mucosa-associated lymphoid tissue (PP-MZL, or MALT lymphoma), primary pulmonary diffuse large B cell lymphoma (PP-DLBCL), and lymphomatoid granulomatosis (LYG). Less common entities include primary effusion B-cell lymphoma (PEL) and intravascular large B cell lymphoma (IVLBCL). A proper workup requires a multidisciplinary approach, including radiologists, pneumologists, thoracic surgeons, pathologists, hemato-oncologists, and radiation oncologists, in order to achieve a correct diagnosis and risk assessment. Aim of this review is to analyze and outline the clinical and pathological features of the most frequent PP-BCLs, and to critically analyze the major issues in their diagnosis and management.
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http://dx.doi.org/10.3390/cancers13030415DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7865219PMC
January 2021

Systemic Mastocytosis Associated with "Smoldering" Multiple Myeloma.

Diagnostics (Basel) 2021 Jan 7;11(1). Epub 2021 Jan 7.

Hematology Unit, CREO, Azienda Ospedaliera di Perugia, University of Perugia, 06129 Perugia, Italy.

A 79-year-old woman presented with a long history of peripheral eosinophilia. Previous right hemicolectomy for colonic polyposis was reported. Laboratory tests were notable for mild macrocitic anaemia and eosinophilia. β2 microglobulin and serum tryptase levels were elevated. Serum immunofixation revealed IgA/kappa monoclonal protein. Bence-Jones protein was positive. Bone marrow (BM) biopsy revealed the coexistence of two neoplastic components. Cohesive clusters of bland-looking, spindle-shaped mast cells, representing 20% of marrow cellularity, were close to aggregates of mature plasma cells occupying 40% of marrow cellularity. Molecular analysis on marrow aspirate demonstrated KIT D816V mutation, TET2 mutation, monoallelic deletion of TP53/17p13 and trisomy of ATM/11q23. A bone density study revealed mild osteoporosis. Full skeletal X-rays and magnetic resonance imaging (MRI) of spine and hips showed multiple, small rarefaction areas and an old L1-L2 fracture, both ascribed to osteoporosis. The association of systemic mastocytosis (SM) and multiple myeloma (MM) is very uncommon. The coexistence of SM with MM placed our patient in the SM with associated clonal haematological non-mast-cell lineage disease (SM-AHN) subtype. Midostaurin therapy was started.
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http://dx.doi.org/10.3390/diagnostics11010088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7825792PMC
January 2021

Chorioamnionitis: An Underrecognized Cause of Preterm Premature Rupture of Membranes in the Second Trimester.

Microorganisms 2021 Jan 3;9(1). Epub 2021 Jan 3.

Clinical Microbiology Laboratory, IRCCS Arcispedale Santa Maria Nuova, 42122 Reggio Emilia, Italy.

is a Gram-negative, rod-shaped bacterium, responsible for hospital and community acquired pneumonia, urinary tract and wound infections, and bloodstream dissemination. infection in pregnancy, leading to acute chorioamnionitis (AC), preterm premature rupture of membranes (PPROM) and early pregnancy loss in the second trimester, has been rarely reported. Herein, we present a case of AC that caused intrauterine fetal demise (IUFD) at 19 weeks + 5 days. The 36-year-old mother was admitted at 18 weeks + 1 day of gestation for threatened abortion. IUFD occurred 11 days after. Fetal postmortem showed severe AC and funisitis, neutrophils within alveoli and intestinal lumen, associated with rod-like bacteria. Fetal blood and lung cultures grew , β-lactamase-non-producing strain. Antibiogram revealed sensitivity for piperacillin/tazobactam. Three days after IUFD, the mother presented with fever (37.8 °C) which persisted for one week. Maternal blood and urine cultures were negative. According to fetal microbiological results, available 6 days after IUFD, initial treatment with amoxicillin/clavulanic acid was replaced with piperacillin/tazobactam with full patient recovery. Therefore, in the event of PPROM and IUFD, fetal microbiological investigations should always be performed to isolate the proper etiologic agent and start the correct medical treatment.
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http://dx.doi.org/10.3390/microorganisms9010096DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7824054PMC
January 2021

Mitotically Active Cellular Fibroma of the Ovary Recurring After the Longest Interval of Time (16 yr): A Challenging Case With Systematic Literature Review.

Int J Gynecol Pathol 2020 Nov 24. Epub 2020 Nov 24.

Pathology Unit, "Maggiore della Carità" Hospital (R.O., A.R., R.B., G.D.D.) Department of Health Science, School of Medicine, University of Eastern Piedmont "Amedeo Avogadro" (R.O., R.B., G.D.D.), Novara Pathology Unit, Azienda Unità Sanitaria Locale-IRCCS, Reggio Emilia (A.P.), Italy.

Cellular fibromas represent ~10% of ovarian fibromas. Mitotically active cellular fibromas show mild nuclear atypia but ≥4 mitoses/10 high-power fields: the clinical course is usually uneventful but literature review is lacking. A 34-yr-old woman underwent left oophorectomy for a 9-cm ovarian mitotically active cellular fibroma at another hospital. The tumor was cellular (spindle cells in fascicular and storiform patterns) revealing mild atypia and 4 nonatypical mitoses/10 high-power fields without necrotic areas. After 16 yr, the tumor recurred as a 5-cm peritoneal nodule on the anterior sigmoid wall near the sigmoid-rectal junction. Frozen section revealed a spindle cell tumor invading the intestinal tunica muscularis propria: a gastrointestinal stromal tumor was favored as previous history was unavailable at that time. Intestinal resection was performed: no residual tumor was found. The patient was followed-up for 8 yr without further recurrences. The peritoneal nodule showed 2 mitoses/10 high-power fields and pericellular reticulin staining. The tumor was variably positive for vimentin/bcl-2/melan-A/CD56/ER/PR/α-inhibin/CD10/calretinin, focally positive for desmin, negative for pan-cytokeratin/actin/EMA/CD34/HMB45/CD117/CD99/S100/synaptophysin. The Ki67-index was ~9%. To our systematic literature review, 7 additional recurrent cases were reported. We describe a mitotically active cellular fibroma recurring after the longest interval of time. Extensive sampling of difficult cases should exclude malignant areas. Moderate nuclear atypia, tumor rupture, adhesions to pelvic/abdominal organs, infarction with extraovarian involvement, and incomplete excision may lead to relapse but there are conflicting data: prolonged follow-up can be suggested in these cases.
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http://dx.doi.org/10.1097/PGP.0000000000000731DOI Listing
November 2020

Accuracy of preoperative endometrial biopsy and intraoperative frozen section in predicting the final pathological diagnosis of endometrial cancer.

Surg Oncol 2020 Dec 10;35:229-235. Epub 2020 Sep 10.

Unit of Surgical Gynecol Oncology, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy.

Background: Histotype and grade of endometrial cancer (EC) are prognostic factors of nodal involvement and thus of survival. Preoperative biopsy (PB) and intraoperative frozen section (FS) are usually used to guide surgical staging on which the choice of adjuvant therapy will be based successively.

Objective: The aim of this study was to assess the agreement rate between PB and FS with final diagnosis (FD) in a series of surgically resected EC.

Materials: All patients submitted to hysterectomy for EC or atypical endometrial hyperplasia in the Reggio Emilia Province hospitals from 2007 to 2018 were included. Concordance rate differences in histotype, grading, myoinvasion, risk of recurrence between PB, FS and FD were assessed with Fisher's exact test and Mc Nemar contingency test.

Results: A total of 352 patients were identified. For 345 patients it was possible to compare PB and FD results. FS examination was performed in 201/352 (57%) cases, while for 21/352 (6%) patients only an intraoperative macroscopic evaluation was done; in the remaining women, FS-exam was omitted. In 14/201 (7%) cases the tumor wasn't grossly identifiable and the random FS-sampling wasn't able to find the tumor site. High diagnostic concordance of tumor type between PB and FD was observed: no significant differences were registered in type 1 and type 2-endometrial cancer identification (83%, 73%, p = 0.121). Significant differences (p = 0.005) were observed comparing FS and FD results: 95% of type 1-ECs were correctly diagnosed by FS, while only 76% of type 2-ECs received a correct diagnosis on FS. PB showed a concordance with FD among tumor grading close to 55% whilst concordance achieved 71% grouping low grade (G1-G2) EC. No significant differences in FS and FD concordance rate were observed between tumor grades. Concordance for low grade was significantly higher than for high grade ECs (89% vs 50%, respectively, p value = 0.014). The concordance rate in evaluating the myoinvasion status between FS and FD was 80% (n: 199 patients), reaching 99% after combining the first 2 groups (0-49% vs ≥ 50%). Twenty-two cases underwent only intraoperative macroscopic evaluation of the myoinvasion, with an accuracy of 91%: only in 1 case the invasion of the cervical stroma was not detected (Stage II), and 1 case the patient was overstaged as Ib. Discrepancies were observed in FS capacity to correctly predict the final ESMO risk group in stage I patients: FS resulted particularly reliable in predicting a low-risk (concordance with FD: 91%) while the accuracy sharply decreased for intermediate- and high-risk patients (62% and 40%, respectively). To investigate the usefulness of FS in EC management, we compared patients who underwent FS (FS-group) or not (no-FS-group). Especially for low risk patients, the FS significantly increased the adequacy of surgical treatment from 53% (no-FS-group) to 72% (FS-group) (p = 0.016).

Conclusions: FS remains a useful tool to tailor surgery in EC-patients, avoiding secondary surgery to complete staging particularly in patients with AH + AHBA, low and intermediate risk ECs that could benefit from adjuvant therapy.
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http://dx.doi.org/10.1016/j.suronc.2020.09.003DOI Listing
December 2020

Acute Radiation Colitis after Preoperative Short-Course Radiotherapy for Rectal Cancer: A Morphological, Immunohistochemical and Genetic Study.

Cancers (Basel) 2020 Sep 9;12(9). Epub 2020 Sep 9.

Pathology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, 42122 Reggio Emilia, Italy.

Preoperative radiotherapy is a widely accepted treatment procedure in rectal cancer. Radiation-induced changes in the tumor are well described, whereas less attention has been given to the non-neoplastic mucosa. Our aim is to provide a detailed analysis of the morphological features present in non-neoplastic mucosa that pathologists need to be familiar with, in order to avoid misdiagnosis, when evaluating rectal cancer specimens of patients preoperatively treated with radiotherapy, especially with short-course regimen. We compared 2 groups of 95 rectal cancer patients treated preoperatively with either short-course (45 patients) or long-course radiotherapy (50 patients). Depending on the type of protocol, different histopathological features, in terms of inflammation, glandular abnormalities and endocrine differentiation were seen in the non-neoplastic mucosa within the irradiated volume. Of note, features mimicking dysplasia, such as crypt distortion, nuclear and cytoplasmic atypia of glandular epithelium, were identified only in the short-course group. DNA mutation analysis, using a panel of 56 genes frequently mutated in cancer, and p53 immunostaining were performed on both tumor and radiation-damaged mucosa in a subset of short course cases. Somatic mutations were identified only in tumors, supporting the concept that tissues with radiation-induced "dysplastic-like" features are not genetically transformed. Pathologists should be aware of the characteristic morphological changes induced by radiation. The presence of features simulating dysplasia in the group treated with short-course radiotherapy may lead to serious diagnostic mistakes, if erroneously interpreted. Next generation sequencing (NGS) analysis further validated the morphological concept that radiation-induced abnormalities do not represent pre-neoplastic lesions.
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http://dx.doi.org/10.3390/cancers12092571DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7563849PMC
September 2020

Endometrial Carcinomas with Intestinal-Type Metaplasia/Differentiation: Does Mismatch Repair System Defects Matter? Case Report and Systematic Review of the Literature.

J Clin Med 2020 Aug 6;9(8). Epub 2020 Aug 6.

Department of Clinical and Experimental Sciences, University of Brescia, Viale Europa 11, 25123 Brescia, Italy.

Background: Intestinal metaplasia/differentiation in primary endometrial carcinomas is an uncommon phenomenon, with only few cases described.

Material And Methods: We performed a systematic review of endometrial carcinomas with intestinal metaplasia/differentiation interrogating the electronic databases Pubmed, Web of Science, and Scopus, and we reported an additional case arising in a 49-year-old woman.

Results: We identified only eight patients diagnosed with endometrial carcinomas exhibiting intestinal metaplasia/differentiation, and additionally our case. Endometrial carcinomas with intestinal-type features can present in pure or mixed forms in association with usual-type endometrioid carcinomas; in mixed forms, the two neoplastic components may derive from a common neoplastic progenitor, as evidenced by the concomitant loss of MSH2 and MSH6 protein expression in our case. Disease recurrences occur in a significant fraction of the cases, including patients diagnosed in low-stage disease.

Conclusions: Endometrial carcinomas with intestinal metaplasia/differentiation are rare and they may represent a more aggressive tumor variant, thus requiring a proper treatment despite the low-tumor stage. The ProMise classification should be performed also in these unusual tumors, since they can be associated with mismatch repair system defects.
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http://dx.doi.org/10.3390/jcm9082552DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7465332PMC
August 2020

What do we know about the cytological features of pure intraductal carcinomas of the salivary glands?

Authors:
Andrea Palicelli

Cytopathology 2020 05 13;31(3):185-192. Epub 2020 Jan 13.

Pathology Unit, Azienda Unità Sanitaria Locale-IRCCS, Reggio Emilia, Italy.

Objective: Intraductal carcinomas (IDCs) are rare, poorly characterised salivary gland tumours. The cytological features of IDCs are even less known. In this paper, a systematic literature review of pure IDCs (without stromal invasion, not associated with other histotypes) of low-grade (LG-IDCs) and high-grade (HG-IDCs) was performed.

Methods: The bibliographic research included multiple databases (PubMed, Scopus, Web of Science). Mild-moderate nuclear atypia favoured LG-IDCs, severe atypia favoured HG-IDCs.

Results: Preoperative fine-needle aspiration cytology (FNAC) was performed in 13/94 published cases (14%): 10 parotid; two oral; one submandibular. All the cases were histologically LG-IDCs, except two parotid IDCs. FNAC results included: negative for malignancy (three of 13 cases, 23%); tumour of uncertain malignant potential (seven of 13, 54%); malignancy (three of 13, 23%). The ductal component was identified in two cases; mucoepidermoid carcinoma was suggested in two additional cases. The grade was underestimated on FNAC evaluation in one HG-IDC as focal high-grade features were present on subsequent histological examination. The cases diagnosed as malignant tumours or describing intermediate atypia resulted in LG-IDCs on subsequent histology. Occasional mitoses were described only in one HG-IDC; this feature may have not been considered in the remaining published cases.

Conclusions: FNAC and clinico-pathological correlation are important aids for clinicians and pathologists. FNAC could assist surgery even if an accurate diagnosis is sometimes impossible. Discrepancy in grading the nuclear atypia between the FNAC material and the resected specimen can occur, sometimes being unavoidable. Further studies are needed to better characterise this rare tumour.
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http://dx.doi.org/10.1111/cyt.12790DOI Listing
May 2020

A tricky and rare cause of pulmonary eosinophilia: myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA.

BMC Pulm Med 2019 Nov 19;19(1):216. Epub 2019 Nov 19.

Pathology Unit, Ospedale di Terni, University of Perugia, Perugia, Italy.

Background: Eosinophilic lung diseases represent a heterogeneous group of disorders with prominent infiltrate of eosinophils in lung interstitium and alveolar spaces. Peripheral blood eosinophilia is often present. Infections, drugs, allergens, toxic agents have to be evaluated as possible causes of eosinophilic lung infiltrates. The category of myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1 and PCM1-JAK2 represents an uncommon cause of eosinophilic lung infiltrate.

Case Presentation: We report the case of a 70-year old man complaining of dry cough and dyspnea. Ground glass-opacities were seen on imaging studies and peripheral blood eosinophilia was present. A thorough step-wise patient's evaluation led to identify the clonal nature of eosinophilia and the diagnosis of myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA was made.

Conclusions: Correlation with clinical history, laboratory tests and imaging studies is essential to achieve the correct diagnosis when facing with eosinophilic lung infiltrates. A prolonged eosinophilia can cause life-threatening organ damage. Identification of PDGFRA rearrangement, as in the present case, is particularly critical given the sensitivity and excellent response to imatinib, which has completely changed the natural history of this neoplasm.
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http://dx.doi.org/10.1186/s12890-019-0967-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6862820PMC
November 2019

Intraductal carcinomas of the salivary glands: systematic review and classification of 93 published cases.

Authors:
Andrea Palicelli

APMIS 2020 Mar 3;128(3):191-200. Epub 2020 Jan 3.

Pathology Unit, Azienda Unità Sanitaria Locale-IRCCS, Reggio Emilia, Italy.

Intraductal carcinomas (IDCs) are rare, not well-characterized salivary gland tumors. A systematic literature review of pure IDCs (without stromal invasion) of low-grade (LG-IDCs) or high-grade (HG-IDCs) was performed: IDCs were classified using the apocrine (AR+/S100-) vs intercalated (S100+/AR-) classification. Eighty-two LG-IDCs and 11 HG-IDCs were identified (84% parotid; 11% oral; 3% submandibular; 1% lacrimal; and 1% unknown). Out of 11 HG-IDCs, 2 HG-IDCs (18%) recurred as HG-IDC or invasive carcinoma. IDCs were classified as follows: intercalated (30%); mixed apocrine and intercalated (27%); apocrine (11%); oncocytic (6%); intercalated with focal oncocytic features (1%); and unclassifiable (25%). Double AR/S100 expressors (4%) or discrepancies between morphology and immunophenotype (9%) were found. Apocrine features and necrosis were more frequent in HG-IDCs (55%; 45%). Pleomorphism favored HG-IDCs (especially when combined with >10 mitoses/10 HPFs and/or Ki67 index >10%), being associated with apocrine areas at least in 3 HG-IDCs (27%). IDCs were typically mammaglobin+/ER-/PR-/DOG1-. No immunomarker clearly distinguished HG-IDCs from LG-IDCs. About 57% IDCs (16 LG-IDCs, 1 HG-IDC) showed RET rearrangements, including NCOA4-RET (eight intercalated and two unclassifiable IDCs) and TRIM27-RET fusions (two mixed IDCs). No ETV6, ALK-1, ROS, NTRK3, MAML2, MAML3, or PLAG1 rearrangements were identified. Complete excision and total sampling should exclude invasive areas.
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http://dx.doi.org/10.1111/apm.13009DOI Listing
March 2020

Role of ERCC1 expression in colorectal adenoma-carcinoma sequence and relation to other mismatch repair proteins expression, clinicopathological features and prognosis in mucinous and non-mucinous colorectal carcinoma.

Indian J Pathol Microbiol 2019 Jul-Sep;62(3):405-412

Department of Pathology, Faculty of Medicine, Mansoura University, Mansoura, Egypt.

Background: There are several DNA repair pathways that protect cellular DNA from injury, such as nucleotide excision repair (NER) and mismatch repair (MMR). The protein product of the excision repair cross-complementation group 1 (ERCC1) gene plays a pivotal role in NER. The exact relationship between MMR proteins and ERCC1 is not well known in colorectal carcinoma (CRC).

Aim Of The Study: To investigate expression of ERCC1 and MMR proteins in colorectal mucinous carcinoma (MA) and non-mucinous carcinoma (NMA) using tissue microarray technique.

Material And Methods: We studied tumor tissue specimens from 150 patients with colorectal mucinous (MA) and non-mucinous adenocarcinoma (NMA). Tissue microarrays were constructed using modified mechanical pencil tips technique and immunohistochemistry for ERCC1, MLH1, MSH2, MSH6, and PMS2.

Results: NMA showed a significantly more frequent aberrant cytoplasmic expression than MA while MA showed a more frequent intact nuclear expression than NMA. There were no significant differences between the NMA and MA groups in the expression of MMR proteins. In NMA cases, ERCC1 expression was significantly related to MMR status while was not significantly related in MA cases. ERCC1 expression was not significantly related to overall and disease-free survival in both NMA and MA groups.

Conclusion: this study is the first to investigate the relation between MMR status and ERCC1 expression in colorectal MA and NMA. ERCC1 expression was significantly related to MMR status only in NMA cases. Hence, the current study emphasizes that further research about the relation between various DNA repair pathways is needed.
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http://dx.doi.org/10.4103/IJPM.IJPM_684_18DOI Listing
December 2019

Pregnancy-related decidualization of subcutaneous endometriosis occurring in a post-caesarean section scar: Case study and review of the literature.

Pathol Res Pract 2019 Apr 18;215(4):828-831. Epub 2019 Jan 18.

Unit of Pathology, Azienda Unità Sanitaria Locale-IRCCS, Reggio Emilia, Italy. Electronic address:

Endometriosis of surgical scars is a rare complication of caesarean sections (incidence: 0.03-0.4%) and other surgical procedures. As endometriosis could be responsive to hormonal stimulation, decidualization and other secondary changes may occur during pregnancy or progestin therapy, sometimes causing a clinically-evident increase in the size of the endometriotic nodules, which could be mistaken for malignant tumors. To our knowledge, we report the 8th subcutaneous case of a pregnancy-related decidualization occurring in a post-caesarean section scar endometriosis. A 33-year-old woman showed a painless, firm, subcutaneous nodule (size: 1 cm) located near the scar of a caesarean section performed 3 years before. Ultrasound examination revealed a well-delimited, hypoechogenic nodule showing perilesional inflammatory reaction without vascular signals. The nodule was considered a post-surgical granuloma: its size did not increase during 4 years of follow-up. Finally, the nodule was totally excised during a second caesarean section performed at 39 weeks of gestation. Histological examination showed nodules of decidualized stromal cells surrounding rare, small, atrophic endometrial glands. Nuclear atypia and mitoses were absent. On immunohistochemical examination, the epithelial cells were pan-CK(AE1/AE3)+/ER+/PR+/S100-/Calretinin-/Vimentin-, while the stromal cells were pan-CK(AE1/AE3)-/Vimentin+/ER+/PR+/CD10+/S100-/Calretinin-. We reviewed the literature, discussing the main clinic-pathological diagnostic pitfalls and the possible differential diagnoses.
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http://dx.doi.org/10.1016/j.prp.2019.01.024DOI Listing
April 2019

Unique facial cutaneous metastasis as the primary presentation of an unexpected voluminous renal cell carcinoma.

G Ital Dermatol Venereol 2018 Nov 9. Epub 2018 Nov 9.

Dermatology Department, "Maggiore della Carità" Hospital and UPO University, Novara, Italy.

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http://dx.doi.org/10.23736/S0392-0488.18.06226-0DOI Listing
November 2018

Unicystic high-grade intraductal carcinoma of the parotid gland: cytological and histological description with clinic-pathologic review of the literature.

APMIS 2018 Sep;126(9):771-776

Laboratory of Pathology, Department of Translational Medicine, UPO School of Medicine, Novara, Italy.

Intraductal carcinoma of the salivary glands is a rare, not well-characterized tumor. We reviewed the literature and report the first case of a high-grade unicystic intraductal carcinoma of the parotid. Formalin-fixed/paraffin-embedded blocks were sectioned and stained for hematoxylin and eosin and immunostains (CAM5.2, EMA, CK5, p53, p63, SMA, S100 protein, DOG1, mammaglobin, AR, ER, PR, Her-2, and Ki67). A 72-year-old man showed a painless nodule (2 cm) in the right parotid region. A 'tumor of uncertain malignant potential' (low grade) was diagnosed by fine-needle aspiration cytology (FNAC). Preoperative magnetic resonance imaging revealed a well-delimited, oval cyst without evidence of parenchymal invasion (T1-scans: homogeneously isointense with hypointense thin peripheral ring; T2-scans: strongly hyperintense). Histological examination confirmed a unilocular cyst lined by a multistratified epithelium arranged in solid, pseudopapillary, cribriform, and 'incomplete cribriform/microcystic' patterns. Tumor cells were CAM5.2+, EMA+, mammaglobin+, AR+, p63+ (focal), CK5+ (focal), p53 (+, 20%), ER-, PR-, S100 protein-, DOG1-, and Her-2-. A continuous peripheral layer of p63+/CK5+/SMA+ myoepithelial cells proved the 'in situ' nature of the tumor. The evidence of focal severe nuclear atypia, high mitotic index (12 mitoses/10HPFs), and high proliferation index (40%) favored a high-grade intraductal carcinoma. Preoperative FNAC and clinic-pathologic correlation are very helpful. Discrepancy in dysplasia grade between FNAC and resected specimen can occasionally occur (especially in case of focal high-grade features). Total sampling should exclude invasive areas or other cystic malignancies.
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http://dx.doi.org/10.1111/apm.12882DOI Listing
September 2018

Paratesticular seminoma: echographic features and histological diagnosis with review of the literature.

APMIS 2018 Mar 7;126(3):267-272. Epub 2018 Feb 7.

Unit of Pathology, 'Maggiore della Carità' Hospital, Novara, Italy.

Primary extratesticular seminomas exceptionally occur in the epididymis or in the paratesticular region/spermatic cord. Some old papers included poor histological description or insufficient photographic documentation, reducing the number of faithful cases: an up-to-date systematic review is lacking. We report the 4th primary seminoma of the paratesticular region/spermatic cord in a 35-year-old man, including the first echographic description. We provide review of the literature and etiopathogenetic discussion. Ultrasound examination showed a right paratesticular, solid, heterogeneous mass (iso-hypoechoic with hyperechoic striae; peri- and intra-lesional vascular signals) with no testicular involvement: the paratesticular origin was confirmed by pathological examination. Despite careful gross examination and extensive sampling, the 6.5-cm extratesticular tumor revealed only one microscopic focus with minimal invasion (<2 mm) of the atrophic testicular parenchyma. Intratubular germ cell neoplasia or morphologic features of a regressed testicular tumor (fibrosis/scar, necrosis, hyalinization, calcification, inflammation) were not found. Primary seminomas of the paratesticular region/spermatic cord occurred at an older mean age and presented as bigger lesions if compared to the 9 primary epididymal seminomas reported in literature. Clinical-pathological correlation and accurate sampling are mandatory for a correct diagnosis.
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http://dx.doi.org/10.1111/apm.12806DOI Listing
March 2018

A unique case of bilateral ovarian splenosis and review of the literature.

APMIS 2017 Sep 25;125(9):844-848. Epub 2017 May 25.

Department of Health Science, School of Medicine, University of Eastern Piedmont "Amedeo Avogadro", Novara, Italy.

Splenosis is an acquired anomaly related to heterotopic auto-transplantation of splenic tissue following abdominal trauma or splenectomy. We report the first definitive bilateral ovarian case in a 65-year-old woman who underwent splenectomy following a motor vehicle accident 44 years prior to presentation. We review the literature and discuss the main differential diagnoses. Gross examination revealed a 1-cm well-circumscribed dark nodule on the surface of each ovary. Paraffin-embedded, formalin-fixed blocks were sectioned and stained with hematoxylin-eosin and immunostains (CK5/6, Calretinin, WT1, Vimentin). The histological presence of both red and white splenic pulp, delimitation from ovarian tissue and ovarian origin of blood supply, as well as medical history, led us to the correct diagnosis. The outer nodular surface was covered by mesothelium (WT1+, CK5/6+, Calretinin+, Vimentin+), which was in continuity with the ovarian surface epithelium. To our knowledge, only six previous cases of ovarian splenosis are reported. Our patient is the oldest, with a very long interval from splenectomy to presentation. Clinically, splenosis may mimic malignancy, and a correct diagnosis avoids unnecessary overtreatment. The differential diagnosis includes an accessory spleen, spleno-gonadal fusion, and splenic hamartoma: they should be excluded to come to the correct diagnosis.
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http://dx.doi.org/10.1111/apm.12714DOI Listing
September 2017

Orbital meningeal melanocytoma: Histological, immunohistochemical and molecular characterization of a case and review of the literature.

Pathol Res Pract 2016 Oct 25;212(10):946-953. Epub 2016 Jul 25.

Department of Health Science, School of Medicine, "Maggiore della Carità" Hospital, University of Eastern Piedmont "Amedeo Avogadro", via Solaroli 17, 28100, Novara, Italy; Department of Pathology, "Maggiore della Carità" Hospital, University of Eastern Piedmont "Amedeo Avogadro", Corso Mazzini 18, 28100, Novara, Italy. Electronic address:

Aims: We provide morphological, immunohistochemical and molecular characterization of the 3rd "intermediate-grade" orbital meningeal melanocytoma, testing for the first time Vysis Melanoma FISH Probe Kit. We reviewed the literature in order to discuss the main differential diagnoses and to provide a better molecular description of these unusual tumors of difficult diagnosis and controversial management.

Methods: Histochemical stains (Haematoxylin and Eosin, Perls, reticulin), immunohistochemistry (HMB45, p16, Melan-A, S100, EMA, Ki67, CD68), polymerase chain reaction amplification and sequence analysis (BRAF, exon 15; NRAS exons 2 and 3; c-KIT, exons 11, 13, 17, 18; GNAQ, exons 4 and 5; GNA11, exons 4 and 5) and fluorescent in situ hybridization (RREB1, 6p25; MYB, 6q23; CCND1, 11q13; CEP 6, 6p11.1-q11.1) were performed on paraffin-embedded, formalin-fixed material.

Results: Histological diagnosis of "intermediate-grade" melanocytoma was supported by zonal necrosis and increased Ki67-index (12%). Immunophenotype: HMB45+(strong, >75%), Melan-A+(strong, >75%), p16+(∼20%), S100 -/+ (<5%), EMA -/+ (<5%), CD68 - (positive histiocytes). No gene mutations nor copy-number alterations were identified. The patient was asymptomatic and disease-free 3 years after total surgical excision.

Conclusions: Adequate sampling and accurate immunohistochemical characterization are important for a correct diagnosis. Molecular analysis could provide important additional information (especially for "intermediate-grade" tumors), but further data are needed.
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http://dx.doi.org/10.1016/j.prp.2016.07.009DOI Listing
October 2016

Tungiasis in Italy: An imported case of Tunga penetrans and review of the literature.

Pathol Res Pract 2016 May 2;212(5):475-83. Epub 2016 Feb 2.

Department of Diagnostic Medicine and Public Health, University of Modena and Reggio Emilia, Via del Pozzo 71, Modena 41124, Italy.

Tungiasis is an animal and human parasitic disease caused by fleas of the genus Tunga (Siphonaptera, Tungidae), endemic in equatorial and subtropical regions and rarely described in European countries, where clinicians and general pathologists could be not aware of this parasitic disease. To our knowledge, only 75 cases of human tungiasis (not all described in detail) were previously reported in Italy. We described a new case in a 34-year-old Italian flight attendant who developed a granuloma-like, ulcerated nodule in the subungual region of his left 5th toe, partially detaching the nail, about 20-30 days after his return from Brazil. We performed a detailed review of the literature of the Italian cases, suggesting the use of histochemical stains (especially Trichrome stain) in order to underline parasitic details. Tourism in endemic regions and globalization may result in new cases in developed countries and previously unaffected regions, therefore pathologists should consider this parasitic disease.
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http://dx.doi.org/10.1016/j.prp.2016.02.003DOI Listing
May 2016

Dirofilaria repens mimicking an ovarian mass: histologic and molecular diagnosis.

APMIS 2014 Oct 4;122(10):1045-6. Epub 2014 Apr 4.

Department of Health Science, School of Medicine, University of Eastern Piedmont "Amedeo Avogadro", Novara, Italy.

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http://dx.doi.org/10.1111/apm.12259DOI Listing
October 2014

Langer mesomelic dysplasia in early fetuses: two cases and a literature review.

Fetal Pediatr Pathol 2014 Apr 24;33(2):71-83. Epub 2013 Jul 24.

Anatomia Patologica, Oncologia, Reggio Emilia, Italy.

In the article, we report the autoptic, histological and radiographic phenotype of two fetuses (22 and 12 weeks) with Langer mesomelic dysplasia, a homozygous deletion of the 3' enhancer of the SHOX gene, and consanguineous parents affected by Léri-Weill dyschondrosteosis, performing a literature review of the primary forms of mesomelic dysplasia. A proper identification of the type of mesomelic dysplasia is important for genetic and reproductive counseling, estimation of child growth and prevention and/or treatment of complications. A competent pathologist could provide important diagnostic information, orienting or confirming the echographic or genetic suspect, sometimes suggesting diagnostic hypothesis concerning parental unidentified congenital syndromes.
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http://dx.doi.org/10.3109/15513815.2013.807322DOI Listing
April 2014

[Primary cardiac Burkitt lymphoma in an African child].

G Ital Cardiol (Rome) 2013 Jun;14(6):481-4

St. Elizabeth Catholic General Hospital, Shisong Cardiac Centre, Camerun.

Burkitt lymphoma is a non-Hodgkin lymphoma that is endemic in the Equatorial Belt of Africa, usually affecting children and adolescents with primary head-neck or abdominal involvement. Primary cardiac lymphomas are rare entities (1.3% of all primary cardiac tumors) of difficult clinical identification. Delayed discovery contributes to significant mortality. We report a case of a primitive Burkitt lymphoma in a 14-year-old Cameroonian immunocompetent child, presenting with signs and symptoms of severe right inflow impairment. Echocardiography revealed a right atrial mass involving the right atrial ventricular junction. Surgical excision and chemotherapy regimens, administered according to established protocols, were effective in inducing complete remission at 6 months.
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http://dx.doi.org/10.1714/1280.14165DOI Listing
June 2013
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