Publications by authors named "Patrick Shenjere"

24 Publications

  • Page 1 of 1

Female Immunity Protects from Cutaneous Squamous Cell Carcinoma.

Clin Cancer Res 2021 Jun 1;27(11):3215-3223. Epub 2021 Apr 1.

Skin Cancer and Ageing Lab, Cancer Research UK Manchester Institute, The University of Manchester, Manchester, United Kingdom.

Purpose: Cancer susceptibility and mortality are higher in males, and the mutational and transcriptomic landscape of cancer differs by sex. The current assumption is that men are at higher risk of epithelial cancers as they expose more to carcinogens and accumulate more damage than women. We present data showing women present with less aggressive primary cutaneous squamous cell carcinoma (cSCC) and early strong immune activation.

Experimental Design: We explored clinical and molecular sexual disparity in immunocompetent and immunosuppressed patients with primary cSCC ( = 738, = 160), advanced-stage cSCC ( = 63, = 20) and FVB/N mice exposed to equal doses of DMBA, as well as in human keratinocytes by whole-exome, bulk, and single-cell RNA sequencing.

Results: We show cSCC is more aggressive in men, and immunocompetent women develop mild cSCC, later in life. To test whether sex drives disparity, we exposed male and female mice to equal doses of carcinogen, and found males present with more aggressive, metastatic cSCC than females. Critically, females activate cancer immune-related expression pathways and CD4 and CD8 T-cell infiltration independently of mutations, a response that is absent in prednisolone-treated animals. In contrast, males increase the rate of mitosis and proliferation in response to carcinogen. Women's skin and keratinocytes also activate immune-cancer fighting pathways and immune cells at UV radiation-damaged sites. Critically, a compromised immune system leads to high-risk, aggressive cSCC specifically in women.

Conclusions: This work shows the immune response is sex biased in cSCC and highlights female immunity offers greater protection than male immunity.
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http://dx.doi.org/10.1158/1078-0432.CCR-20-4261DOI Listing
June 2021

Renal myopericytoma: A case report and literature review.

Urol Case Rep 2021 Mar 15;35:101537. Epub 2020 Dec 15.

North Manchester General Hospital, Pennine Acute NHS Hospitals Trust, Delaunay's Road, Manchester, M8 5RB, UK.

Renal myopericytoma is an extremely rare entity with just 11 cases reported in the literature. We report the case of a 57 year old Caucasian man who was found to have a renal myopericytoma following nephrectomy for suspected renal cell carcinoma. Renal myopericytoma has a distinct morphological overlap with other pericytic tumours and significant histological variation has been noted between cases reported to date. Further characterising this novel tumour is vital to identify subtypes within this spectrum, understand its behaviour and to identify imaging trends which may lead to pre-operative diagnosis in order to potentially avoid radical treatment.
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http://dx.doi.org/10.1016/j.eucr.2020.101537DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7753123PMC
March 2021

Primary glomus tumour of the pituitary gland: diagnostic challenges of a rare and potentially aggressive neoplasm.

Virchows Arch 2021 May 12;478(5):977-984. Epub 2020 Sep 12.

Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.

Primary non-neuroendocrine tumours of the pituitary gland and sella are rare lesions often challenging to diagnose. We describe two cases of clinically aggressive primary glomus tumour of the pituitary gland. The lesions occurred in a 63-year-old male and a 30-year-old female who presented with headache, blurred vision and hypopituitarism. Neuroimaging demonstrated large sellar and suprasellar tumours invading the surrounding structures. Histologically, the lesions were characterised by angiocentric sheets and nests of atypical cells that expressed vimentin, smooth muscle actin and CD34. Perivascular deposition of collagen IV was also a feature. Case 2 expressed synaptophysin. INI-1 (SMARCB1) expression was preserved. Both lesions were mitotically active and demonstrated a Ki-67 labelling index of 30%. Next-generation sequencing performed in case 1 showed no mutations in the reading frame of 37 commonly mutated oncogenes, including BRAF and KRAS. Four pituitary glomus tumours have previously been reported, none of which showed features of malignant glomus tumour. Similar to our two patients, three previous examples displayed aggressive behaviour.
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http://dx.doi.org/10.1007/s00428-020-02923-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8099815PMC
May 2021

E-Cadherin Expression in Blastic Plasmacytoid Dendritic Cell Neoplasms: An Unrecognized Finding and Potential Diagnostic Pitfall.

Int J Surg Pathol 2021 May 1;29(3):289-293. Epub 2020 Jul 1.

Department of Histopathology, 5294The Christie NHS Foundation Trust, Manchester, UK.

E-cadherin is expressed in hematopoietic erythroid precursors, but to our knowledge, its expression in blastic plasmacytoid dendritic cell neoplasm (BPDCN) has not been described. We report a case of BPDCN showing strong expression of E-cadherin, arising in a patient with history of primary myelofibrosis. Four more cases of BPDCN tested all showed strong expression of E-cadherin. Lack of awareness of this pattern of expression may lead to erroneous diagnosis of acute erythroid leukemia. It is increasingly becoming important to correctly identify this group of neoplasms, as approved new anti-CD123-targeted therapies are becoming available.
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http://dx.doi.org/10.1177/1066896920938130DOI Listing
May 2021

EORTC SPECTA-AYA: A unique molecular profiling platform for adolescents and young adults with cancer in Europe.

Int J Cancer 2020 08 14;147(4):1180-1184. Epub 2019 Sep 14.

Division of Cancer Sciences, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom.

For most adolescent and young adult (AYA) cancers, age-specific molecular features are poorly understood. EORTC-SPECTA, an academic translational research infrastructure for biomaterial collection, will explicitly recruit AYA patients and will therefore collect empirical data to bridge the molecular gap between pediatric and adult oncology. The initial pilot study, activated in February 2019 across Europe, will recruit 100 AYA patients (aged 12-29 years) with newly diagnosed or relapsed high-grade gliomas and high-grade bone and soft tissue sarcomas. The primary objective of the pilot is to determine feasibility and recruitment rates. Formalin-fixed tumor tissue and whole blood from study participants will be prospectively collected with clinical data and stored centrally at the Integrated BioBank of Luxembourg. Whole exome sequencing of matched tumor and blood, and tumor RNA sequencing and DNA methylation profiling will be performed at the German Cancer Research Center, Heidelberg, Germany. Virtual central pathology review of scanned diagnostic slides will be undertaken by an international expert panel, and diagnostic material returned to the participating centers. A multidisciplinary molecular tumor board will release a clinically validated report to referring clinicians within 4-6 weeks after biopsy. SPECTA-AYA constitutes a major opportunity to gain knowledge about the tumor biology of this unique age group. It incorporates notable innovative aspects: AYA specificity, pan-European academic collaboration, centralized biobanking, comprehensive molecular profiling and virtual central pathology review, among others. SPECTA-AYA will help untangle the tumor particularities of AYAs with cancer and aims to improve their access to novel drugs and personalized medicine.
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http://dx.doi.org/10.1002/ijc.32651DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7383917PMC
August 2020

Multiple primary malignancies associated with a germline SMARCB1 pathogenic variant.

Fam Cancer 2019 10;18(4):445-449

Manchester Centre for Genomic Medicine, St Mary's Hospital, Manchester University Hospitals Foundation Trust, Manchester, M13 9WL, UK.

A 51-year old presented with a 6-month history of increasing pelvic/lower back pain with nocturnal waking and episodes of anorexia and vomiting. Examination revealed right torticollis and Horner's syndrome, and a large abdominal mass arising from the pelvis. Magnetic resonance and positron emission tomography imaging revealed (A) a 14 cm heterogeneous enhancing mass, abutting the left kidney with standardised uptake value max = 2.9, (B) a large heterogeneous enhancing pelvic mass (C) mesenteric adenopathy standardised uptake value max = 10.3 and (D) 6 cm right lung apex mass standardised uptake value max = 4.3. Computerised tomography-guided biopsy of lesion A was reported as neurofibroma with occasional atypia, lesion B a benign uterine leiomyoma and lesion C follicular lymphoma world health organisation Grade 2. Although she had been given the diagnosis of Neurofibromatosis Type-1 (NF1) 25-years previously following removal of an intradural extramedullary schwannoma she had no cutaneous stigmata of NF1. Genetic analysis of blood lymphocyte DNA identified a pathogenic variant in SMARCB1 confirming a diagnosis of schwannomatosis. Following 6-months chemotherapy for lymphoma, surgery was performed to remove lesion A. Histology revealed a malignant peripheral nerve sheath tumour with areas of low and high-grade change. An incidental, well-differentiated small bowel neuroendocrine carcinoma was also excised. Close surveillance continues with no recurrence after 6 years. This case study describes a novel finding of three separate synchronous primary malignancies in a patient with schwannomatosis and a proven SMARCB1 pathogenic variant.
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http://dx.doi.org/10.1007/s10689-019-00138-4DOI Listing
October 2019

The hypoxia marker CAIX is prognostic in the UK phase III VorteX-Biobank cohort: an important resource for translational research in soft tissue sarcoma.

Br J Cancer 2018 03 12;118(5):698-704. Epub 2017 Dec 12.

Translational Radiobiology Group, Division of Cancer Sciences, University of Manchester, Manchester Academic Health Science Centre, Christie Hospital NHS Foundation Trust, Wilmslow Road, Manchester M20 4BX, UK.

Background: Despite high metastasis rates, adjuvant/neoadjuvant systemic therapy for localised soft tissue sarcoma (STS) is not used routinely. Progress requires tailoring therapy to features of tumour biology, which need exploration in well-documented cohorts. Hypoxia has been linked to metastasis in STS and is targetable. This study evaluated hypoxia prognostic markers in the phase III adjuvant radiotherapy VorteX trial.

Methods: Formalin-fixed paraffin-embedded tumour biopsies, fresh tumour/normal tissue and blood were collected before radiotherapy. Immunohistochemistry for HIF-1α, CAIX and GLUT1 was performed on tissue microarrays and assessed by two scorers (one pathologist). Prognostic analysis of disease-free survival (DFS) used Kaplan-Meier and Cox regression.

Results: Biobank and outcome data were available for 203 out of 216 randomised patients. High CAIX expression was associated with worse DFS (hazard ratio 2.28, 95% confidence interval: 1.44-3.59, P<0.001). Hypoxia-inducible factor-1α and GLUT1 were not prognostic. Carbonic anhydrase IX remained prognostic in multivariable analysis.

Conclusions: The VorteX-Biobank contains tissue with linked outcome data and is an important resource for research. This study confirms hypoxia is linked to poor prognosis in STS and suggests that CAIX may be the best known marker. However, overlap between single marker positivity was poor and future work will develop an STS hypoxia gene signature to account for tumour heterogeneity.
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http://dx.doi.org/10.1038/bjc.2017.430DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5846059PMC
March 2018

Outcomes following front-line chemotherapy in peripheral T-cell lymphoma: 10-year experience at The Royal Marsden and The Christie Hospital.

Leuk Lymphoma 2018 07 9;59(7):1586-1595. Epub 2017 Nov 9.

b The University of Manchester and The Christie NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK.

We evaluated the outcomes for patients with peripheral T-cell lymphoma (PTCL) undergoing front-line chemotherapy at our institutions between 2002 and 2012. One hundred and fifty-six patients were eligible, comprising PTCL not otherwise specified (NOS) (n = 50, 32.0%), angioimmunoblastic T-cell lymphoma (AITL) (n = 44, 28.2%), anaplastic large-cell lymphoma (ALCL) ALK negative (n = 23, 14.7%), ALCL ALK positive (n = 16, 10.3%), and other (n = 23, 14.7%). Most patients received CHOP (66.0%) and 13.0% received an autologous hematopoietic progenitor cell transplant (HPCT). With a median follow-up of 63.4 months, 5-year overall survival (OS) and progression-free survival (PFS) was 38.8% and 19.8% respectively. Independent risk factors for inferior OS were age >60 years, International Prognostic Index (IPI) ≥ 2 and lack of complete response to induction. When responding patients were compared by receipt of an autologous HPCT versus not, HPCT was associated with improved PFS (p = .001) and OS (p = .046) and remained significant for PFS in multivariate analysis suggesting a possible therapeutic benefit.
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http://dx.doi.org/10.1080/10428194.2017.1393671DOI Listing
July 2018

Identification of a Potential Molecular Diagnostic Biomarker in Keloid Disease: Syndecan-1 (CD138) Is Overexpressed in Keloid Scar Tissue.

J Invest Dermatol 2016 11 14;136(11):2319-2323. Epub 2016 Jul 14.

Plastic and Reconstructive Surgery Research, University of Manchester, Manchester, UK; The Dermatology Centre, Institute of Inflammation and Repair, Faculty of Medical and Human Sciences, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. Electronic address:

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http://dx.doi.org/10.1016/j.jid.2016.06.623DOI Listing
November 2016

Tumoral Melanosis Nine Years After Wide Local Excision of a Thin Melanoma.

Dermatol Surg 2016 Jun;42(6):779-80

Department of Plastic and Reconstructive Surgery, and Department of Histopathology, The Christie NHS Foundation Trust, Manchester, United Kingdom.

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http://dx.doi.org/10.1097/DSS.0000000000000710DOI Listing
June 2016

Circulating biomarkers in hepatocellular carcinoma.

Cancer Chemother Pharmacol 2014 Aug 13;74(2):323-32. Epub 2014 Jun 13.

Clinical and Experimental Pharmacology Group, Cancer Research UK Manchester Institute, University of Manchester, Manchester, UK.

Purpose: Our aims are to determine levels of circulating cellular and protein biomarkers in hepatocellular carcinoma (HCC) patients and to analyse any relationships with clinical parameters.

Methods: Fifty-four consenting patients were recruited. Circulating tumour cells (CTCs) were enumerated (by CellSearch) and characterised via filtration [by isolation by size of epithelial tumour cells (ISET)] with downstream immunohistochemistry (IHC). Glypican-3 (GPC3) expression in tumour biopsies and CTCs (by IHC) was compared, and levels of circulating caspase-cleaved and full-length cytokeratin 18 (CK18, measured using M30 and M65 ELISAs) were examined as a putative prognostic factor and marker of tumour burden.

Results: CTCs were identified in 14 out of 50 (28%) patients by CellSearch and in 19 out of 19 (100%) patients by ISET. The presence of GPC3-positive CTCs by ISET was 100% concordant with the presence of GPC3-positive cells in the original tumour (n = 5). No statistically significant correlations were observed between CTC number and clinical characteristics, although trends were noted between CTC subtypes, Child-Pugh score and tumour node metastasis stage. Serum M30 and M65 levels (as continuous variables) significantly correlated with overall survival (OS) in a univariate analysis (p = 0.003 and p < 0.001, respectively); M65 levels remained statistically significant in a multivariate analysis (p = 0.029).

Conclusions: This is the first study to detect GPC3-positive CTCs in HCC, important for drug development with this target. The significant association of circulating CK18 with OS in HCC further exemplifies the utility of circulating biomarkers in cancer.
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http://dx.doi.org/10.1007/s00280-014-2508-7DOI Listing
August 2014

Melanoma with rhabdomyosarcomatous differentiation: two further cases of a rare pathologic pitfall.

Int J Surg Pathol 2014 Sep 29;22(6):512-9. Epub 2014 Apr 29.

Department of Histopathology, Royal Marsden Hospital, London, UK.

We describe 2 new cases of malignant melanoma with divergent rhabdomyoblastic differentiation occurring in adult patients. The patients were women aged 67 and 51 years with primary cutaneous and uterine cervical melanoma, respectively. Rhabdomyoblastic differentiation in melanoma is very rare in adult patients, and to our knowledge, only 7 such cases have been described in the world literature, of which only 4 have conclusive documentation of the presence of rhabdomyoblastic differentiation. We present the fifth and sixth cases of adult melanomas with conclusive divergent rhabdomyoblastic differentiation, including the first noncutaneous (cervical) case; we also review the literature and highlight the potential for underrecognition of this phenomenon.
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http://dx.doi.org/10.1177/1066896914531817DOI Listing
September 2014

Primary extranodal marginal zone B cell lymphoma of the uterus: a case study and review of the literature.

J Clin Pathol 2014 Apr 18;67(4):375-7. Epub 2013 Dec 18.

Department of Histopathology, Manchester Royal Infirmary, Central Manchester University Hospitals NHS Foundation Trust, , Manchester, UK.

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http://dx.doi.org/10.1136/jclinpath-2013-202059DOI Listing
April 2014

Fibroblastic Reticulum Cell Tumor of Spleen: A Case Report.

Int J Surg Pathol 2014 Aug 12;22(5):447-50. Epub 2013 Nov 12.

Stepping Hill Hospital, Stockport, UK.

Fibroblastic reticulum cells (FBRCs) belong to a major subtype of stromal support cells in the lymphoid system and rarely give rise to tumors. We report a case of fibroblastic reticulum cell tumor arising in the spleen. The tumor was clinically and radiologically mistaken for a metastatic deposit in the spleen. Microscopically the tumor was composed of spindle cells arranged in fascicles and storiform pattern. The cells had oval to elongated vesicular nuclei and pale eosinophilic cytoplasm with indistinct cell borders. There were admixed inflammatory cells, including large numbers of plasma cells. The tumor cells were positive for smooth muscle actin, desmin, AE1/AE3, and MNF116. They were negative for S100, CD1a, CD21, CD23, CD34, CD31, and CD35 among other markers. The morphological features and immunoprofile of this rare tumor in comparison to the few cases reported in the literature are discussed along with the positive reaction with cytokeratins and their relationship to the smaller subset of FBRCs, the cytokeratin-positive interstitial reticulum cells in the spleen.
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http://dx.doi.org/10.1177/1066896913509009DOI Listing
August 2014

Prevalence and heterogeneity of circulating tumour cells in metastatic cutaneous melanoma.

Melanoma Res 2014 Feb;24(1):40-6

aClinical and Experimental Pharmacology Group, Paterson Institute for Cancer Research bThe Christie NHS Foundation Trust cSchool of Cancer and Enabling Sciences, Manchester Cancer Research Centre and Manchester Academic Health Sciences Centre, University of Manchester, Manchester dAstraZeneca Pharmaceuticals, Alderley Park, UK.

We previously demonstrated that circulating tumour cells (CTCs) are detectable by the MelCAM and high molecular weight melanoma-associated antigen (HMW-MAA)-dependent CellSearch platform. However, CTCs which do not express these capture and detection markers are not detectable by CellSearch. Consequently, we explored the use of isolation by size of epithelial tumour cells (ISET), a marker independent, filtration-based device to determine the prevalence and heterogeneity of CTCs in metastatic cutaneous melanoma patients. Ninety patients were prospectively recruited and blood samples taken before treatment. Patients' blood was filtered using the ISET platform. CTCs were enumerated using dual immunohistochemistry with positive selection by S100 expression and exclusion of leucocytes and endothelial cells expressing CD45 or CD144, respectively. A panel of markers (Melan-A, MITF, MelCAM, high molecular melanoma-associated antigen, CD271 and MAGEC) was also examined. Fifty-one patients (57%) had CTCs (range 1-44 CTCs/4 ml blood) and 12 patients also had circulating tumour microemboli. Seven patients had S100- CTCs, 11 patients' CTCs were S100+ and 33 patients had S100+ and S100- CTCs. Substantial intrapatient and interpatient heterogeneity was observed for all other melanoma-associated markers. CTCs in metastatic cutaneous melanoma are detectable using the flexible marker-independent ISET platform. CTCs display significant marker expression heterogeneity implying that marker-dependent platforms would not detect all CTCs and multimarker assays are now required to reveal the biological significance of this CTC heterogeneity.
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http://dx.doi.org/10.1097/CMR.0000000000000025DOI Listing
February 2014

Ultrastructurally confirmed myofibrosarcoma: a series of 10 new cases, with a discussion on diagnostic criteria.

Int J Surg Pathol 2013 Feb 27;21(1):29-36. Epub 2012 Jul 27.

The Christie NHS Foundation Trust, Manchester, UK.

Some view ultrastructure as key to myofibrosarcoma diagnosis, whereas others argue that electron microscopy is too little used in contemporary practice to be considered an important diagnostic tool. These views are discussed in the context of 10 ultrastructurally confirmed cases of myofibrosarcoma, some occurring at rare sites such as skin and penis. Patient age ranged from 21 to 83 years, with a 6:4 male to female ratio. Size ranged from 2 to 7.5 cm and all had infiltrative margins. Histologically, all consisted of variably cellular fascicles of spindle cells with mild to moderately pleomorphic nuclei, small punctate nucleoli, and eosinophilic cytoplasm. All cases showed α-smooth muscle actin positivity and 2 showed very focal weak positivity for desmin. Ultrastructurally, the tumor cells contained rough endoplasmic reticulum, mainly peripheral smooth-muscle myofilaments, and fibronectin fibrils or fibronexus junctions at the cell surface. The most confident diagnosis of myofibrosarcoma is provided by ultrastructural examination. However, given the right histological appearance, use of a panel of antibodies that includes α-smooth muscle actin, desmin, and h-caldesmon, serves as an acceptable practical way of diagnosing myofibrosarcoma.
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http://dx.doi.org/10.1177/1066896912454568DOI Listing
February 2013

Combined usual and nodular types of vascular transformation of sinuses in the same lymph node.

Int J Surg Pathol 2012 Apr 1;20(2):175-7. Epub 2012 Feb 1.

Department of Pathology, University of Milan, Milan, Italy.

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http://dx.doi.org/10.1177/1066896911434550DOI Listing
April 2012

Intra-abdominal clear-cell sarcoma: a report of 3 cases, including 1 case with unusual morphological features, and review of the literature.

Int J Surg Pathol 2012 Aug 13;20(4):378-85. Epub 2011 Nov 13.

Department of Histopathology, The Christie NHS Foundation Trust, Manchester, UK.

Clear-cell sarcoma (CCS) is a soft-tissue neoplasm that morphologically resembles cutaneous malignant melanoma but has a distinct molecular profile. Gastrointestinal and intra-abdominal CCSs are very rare. Here, the authors present 3 cases of intra-abdominal CCS and review the literature. Of these cases, 2 involved the small bowel, and 1 involved the peritoneum. Cases 1 and 3 had the characteristic CCS morphology, but case 2 was morphologically unusual and therefore difficult to diagnose. It had relatively small cells with less prominence of clear cells; many pseudoglandular structures were also present. It also showed aberrant expression of epithelial membrane antigen (EMA). The other 2 cases also involved some diagnostic uncertainty and were therefore referred to specialized centers. The authors wish to emphasize the importance of molecular studies in making a conclusive diagnosis of intra-abdominal CCS.
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http://dx.doi.org/10.1177/1066896911425485DOI Listing
August 2012

Two out of three required: a case of incomplete Carney triad.

Int J Surg Pathol 2012 Jun 26;20(3):265-8. Epub 2011 Jul 26.

Department of Hepatopancreatobiliary and General Surgery, North Manchester General Hospital, Manchester, UK.

A 48-year-old woman presented with chest symptoms. Multiple lesions seen on chest imaging were found to be pulmonary chondromas following surgical resection. Whole body magnetic resonance scan performed to investigate the possibility of Carney triad demonstrated a gastric lesion. This was resected and found to be a gastrointestinal stromal tumor. No evidence of paragangliomata was found on imaging. A diagnosis of incomplete Carney triad was made. Carney triad is a rare multiple neoplastic association of pulmonary chondroma, gastrointestinal stromal tumor, and paraganglioma. At least 2 tumors are required for diagnosis. Most patients are young women. No genetic cause has been identified. Management involves surgical resection of tumors and follow-up for recurrence and investigation for other elements of the triad.
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http://dx.doi.org/10.1177/1066896911415668DOI Listing
June 2012

Phase II study of cisplatin and imatinib in advanced salivary adenoid cystic carcinoma.

Br J Oral Maxillofac Surg 2011 Oct 10;49(7):510-5. Epub 2010 Nov 10.

Glan Clwyd Hospital, Rhyl, Denbighshire LL18 5UJ, United Kingdom.

Patients with adenoid cystic carcinoma of the salivary glands show over-expression of KIT in a high proportion of cases. Options for systemic treatment are limited in locally advanced and metastatic disease. We explored the efficacy of imatinib and cisplatin combined in this group of patients. A Gehan's two-stage, phase II trial was conducted on 28 patients. Those with progressive, locally advanced, and metastatic disease with an over-expression of KIT were treated with single agent imatinib 800 mg daily for two months, followed by a combination of imatinib 400mg daily and cisplatin 80 mg/m(2) at four-weekly intervals for six cycles. This was followed by maintenance single agent imatinib 400mg daily until the disease progressed. Response was monitored using fluorodeoxyglucose positron emission tomography (FDG-PET) and morphological imaging using computed tomography, magnetic resonance, and chest radiographs (CT/MRI/CXR). Morphological imaging showed partial response in three of 28 patients, and five patients showed a response on FDG-PET. In addition, 19 patients had useful stabilisation of disease. The median time to progression and overall survival was 15 months (range 1-43) and 35 months (range 1-75), respectively. The combination of imatinib and cisplatin was reasonably well tolerated. This combination may provide stabilisation in locally advanced and metastatic adenoid cystic carcinoma of the salivary glands.
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http://dx.doi.org/10.1016/j.bjoms.2010.09.013DOI Listing
October 2011

Solid variant of alveolar rhabdomyosarcoma in the head and neck region: a case report of a diagnostic dilemma in a head and neck fine needle aspiration clinic.

Acta Cytol 2010 Sep-Oct;54(5 Suppl):849-52

Department of Histopathology, Royal Blackburn Hospital, Haslingden Road, Blackburn BB2 3HH, Lancashire, UK.

Background: Alveolar rhabdomyosarcoma (ARMS) is one of the major categories of rhabdomyosarcomas; it encompasses malignant tumors of striated muscle and occurs more frequently in the extremities. It is uncommonly reported in young adults and extremely rarely found in middle-aged and elderly patients.

Case: A 54-year-old man presented to a rapid head and neck clinic with a history of rapid enlargement of neck lumps on the right side of his neck over a period of 5 weeks. The diagnosis of an undifferentiated malignant small round cell tumor was made from cytologic examination of the aspirated sample, and biopsy of the lesion was advised. On histologic analysis, diagnosis of solid variant of ARMS was made.

Conclusion: A solid variant of ARMS in an older population has not been published in the literature within the settings of a rapid head and neck clinic. Therefore, the remote possibility of this diagnosis should be considered in the differential diagnosis of a malignant, round cell tumor in fine needle aspiration cytology in an older patient's neck lump.
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December 2010

Primary pulmonary osteosarcoma: a report of 4 cases and a review of the literature.

Int J Surg Pathol 2011 Apr 11;19(2):225-9. Epub 2009 Mar 11.

Department of Histopathology, Christie Hospital, Manchester, United Kingdom.

Primary pulmonary osteosarcoma is very rare. Most cases are secondary deposits from primaries arising in the appendicular skeleton. Four cases of primary osteogenic sarcoma of the lung are described and the literature reviewed for previously reported cases. These pulmonary tumors occur in patients who are in their fourth to seventh decades, that is, an older age group than their primary bone equivalent. There is a slight male predominance. There appears to be a propensity for the left lung, especially the left upper lobe. The clinical presentation is similar to primary (epithelial) lung cancer. Differentiation from pleomorphic carcinomas and other sarcomas is discussed. We know of no predisposing factor(s) in our cases for the development of this tumor.
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http://dx.doi.org/10.1177/1066896909332382DOI Listing
April 2011

Pseudo-Gaucher cells in multiple myeloma.

Int J Surg Pathol 2008 Apr;16(2):176-9

Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom.

A case of multiple myeloma is reported in which the bone marrow contained sheets of histiocytes with light microscopic features mimicking Gaucher cells. The patient had no clinical evidence of inherited Gaucher's disease. These pseudo-Gaucher cells obscured neoplastic plasma cells causing diagnostic difficulty.
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http://dx.doi.org/10.1177/1066896907311120DOI Listing
April 2008

CD30-positive T-cell lymphoproliferative disorder of the oral mucosa--an indolent lesion: report of 4 cases.

Int J Surg Pathol 2008 Jul 2;16(3):286-90. Epub 2008 Apr 2.

University Hospitals of Morecambe Bay NHS Trust, Lancaster, United Kingdom.

Four cases of CD30-positive T-cell lymphoproliferative disorder (CD30+ LPD) of the oral mucosa are described. This article aims to draw attention to this entity and to emphasize its usual benign clinical behavior despite its resemblance to T-cell lymphoma. All the patients were adults. Three of the lesions were on the dorsal surface of the tongue and 1 affected the buccal mucosa. All biopsies showed a dense lymphoid infiltrate composed of CD30+ atypical T cells with a polymorphous infiltrate in the background, which included eosinophils. In 1 case, monoclonal T-cell expansion was detected by molecular techniques. Three cases tested for Epstein-Barr virus were all negative. It is concluded that primary CD30+ T-cell LPD of the oral mucosa can be regarded as the oral counterpart of cutaneous CD30+ LPD such as lymphomatoid papulosis or anaplastic large cell lymphoma. Recognition of the condition is important to avoid overtreatment.
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http://dx.doi.org/10.1177/1066896907313755DOI Listing
July 2008
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