Publications by authors named "Shintaro Sugita"

89 Publications

Expression of programmed cell death ligand 1 (PD-L1) at in situ and invasive extramammary Paget's disease and literature review.

Australas J Dermatol 2021 May 12. Epub 2021 May 12.

Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

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http://dx.doi.org/10.1111/ajd.13607DOI Listing
May 2021

[Probable Immunoglobulin G4 (IgG4)-related Periaortitis Requiring Differentiation from Mediastinal Tumor].

Kyobu Geka 2021 Feb;74(2):108-111

Department of Thoracic Surgery, Sapporo Medical University, Sapporo, Japan.

A 42-year-old man with a history of suspected of Behcet's disease underwent oral steroid treatment. During follow-up, chest X-ray revealed an abnormal shadow of the mediastinum. Chest computed tomography(CT) showed a circumferential tumor around the descending thoracic aorta. Enhanced CT showed a lowly and uniformly enhanced tumor at delay phase. A mediastinal tumor was suspected, which prompted a biopsy of the periaortic tumor by video-assisted thoracic surgery (VATS). Histopathological diagnosis showed numerous immunogloblin G4 (IgG4)-positive plasma cells suggesting the possibility of IgG4-related periaortitis. However, based on the diagnostic criteria, the case was comprehensively diagnosed as probable IgG4-related periaortitis, steroid treatment may have affected blood IgG4-positive cells and tissues.
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February 2021

Successful hemostasis of bleeding gastric inflammatory fibroid polyp by endoscopic treatment in a patient with severe COVID-19.

Clin J Gastroenterol 2021 Aug 11;14(4):1008-1013. Epub 2021 Apr 11.

Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, S-1, W-16, Chuo-ku, Sapporo, 063-8543, Japan.

The coronavirus disease-2019 (COVID-19) has rapidly become a pandemic, resulting in a global suspension of non-emergency medical procedures such as screening endoscopic examinations. There have been several reports of COVID-19 patients presenting with gastrointestinal symptoms such as diarrhea and vomiting. In this report, we present a case of successful hemostasis of bleeding gastric inflammatory fibroid polyp by endoscopic treatment in a patient with severe COVID-19. The case was under mechanical ventilation with extracorporeal membrane oxygenation (ECMO), and the airway was on a closed circuit. This indicates that COVID-19 is associated with not only lung injury but also intestinal damage, and that proper protective protocols are essential in guaranteeing the best outcomes for patients and clinical professionals during this pandemic.
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http://dx.doi.org/10.1007/s12328-021-01402-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8038534PMC
August 2021

Stroke Mimics and Chameleons from the Radiological Viewpoint of Glioma Diagnosis.

Neurol Med Chir (Tokyo) 2021 Feb 29;61(2):134-143. Epub 2020 Dec 29.

Department of Neurosurgery, Sapporo Medical University, Sapporo, Hokkaido, Japan.

Gliomas are sometimes difficult to differentiate from strokes and are often misdiagnosed on magnetic resonance imaging (MRI); thus, the terms "stroke mimics" and "stroke chameleons" have been introduced. In this study, we analyzed stroke mimics and stroke chameleons in glioma and discussed the diagnostic perplexity.We retrospectively reviewed cases that were removed from lesions that were considered to be brain tumors. This study enrolled 214 patients who underwent tumor resection for suspected glioma. Clinical characteristics and radiological findings of the patients were compared between the masquerade findings group, which was further divided into two groups: the stroke chameleons and stroke mimics according to their final diagnosis, and the intelligible findings group.Stroke chameleons and stroke mimics were significantly higher in age and smaller in lesion size than the intelligible findings group. In the multivariate analysis, the predictive factor of the masquerade finding group was higher age and smaller size. Stroke mimics group has a tendency to be higher rate of hyperintensity lesion on diffusion-weighted imaging (DWI) compared with stroke chameleons group. The average period from initial diagnosis to pathological diagnosis was 13.50 days in the stroke chameleons and 61.50 days in the stroke mimics, which proved significantly different.Proper diagnosis of glioma and stroke affects a patient's prognosis, and should be diagnosed as soon as possible. However, stroke mimics and stroke chameleons caused by glioma can occur. Thus, the diagnosis of a stroke should take into consideration the possibility of a glioma in real clinical situations.
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http://dx.doi.org/10.2176/nmc.oa.2020-0309DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7905296PMC
February 2021

Multiple lung lesions in a 13-year-old girl with classical nodular sclerosis Hodgkin Lymphoma.

Int J Hematol 2021 Mar 1;113(3):318-319. Epub 2021 Jan 1.

Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan.

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http://dx.doi.org/10.1007/s12185-020-03048-9DOI Listing
March 2021

Primary capicua transcriptional repressor-rearranged sarcoma of the lung.

Jpn J Clin Oncol 2021 Apr;51(4):654-656

Department of Thoracic Surgery, National Hospital Organization, Hokkaido Cancer Center, Sapporo, Japan.

A 60-year-old man had a malignant left lower lung tumour with no metastases and underwent video-assisted thoracoscopic left lower lobectomy and lymphadenectomy. Pathological examination led to a diagnosis of capicua transcriptional repressor (CIC)-rearranged sarcoma. He has had 3.5 years of recurrence-free survival. CIC-rearranged sarcoma is a Ewing-like sarcoma that shows pathological findings similar to Ewing sarcoma. Most of CIC-rearranged sarcoma is CIC-double homeobox 4 protein (DUX4) fusion. Pulmonary CIC-rearranged sarcoma is extremely rare and has an unfavourable prognosis. However, complete resection can produce prognosis of long-term survival, and thus, surgery is an important option.
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http://dx.doi.org/10.1093/jjco/hyaa240DOI Listing
April 2021

Case series of three patients with hereditary diffuse gastric cancer in a single family: Three case reports and review of literature.

World J Gastroenterol 2020 Nov;26(42):6689-6697

Department of Medical Oncology, Sapporo Medical University School of Medicine, Sapporo 060-8556, Hokkaido, Japan.

Background: Hereditary diffuse gastric cancer (HDGC) is a familial cancer syndrome often associated with germline mutations in the gene. However, the frequency of mutations is low in patients with HDGC in East Asian countries. Herein, we report three cases of HDGC harboring a missense variant, c.1679C>G, from a single Japanese family.

Case Summary: A 26-year-old female (Case 1) and a 51-year-old male (father of Case 1), who had a strong family history of gastric cancer, were diagnosed with advanced diffuse gastric cancer. After genetic counselling, a 25-year-old younger brother of Case 1 underwent surveillance esophagogastroduodenoscopy that detected small signet ring cell carcinoma foci as multiple pale lesions in the gastric mucosa. Genetic analysis revealed a c.1679C>G variant in all three patients.

Conclusion: It is important for individuals suspected of having HDGC to be actively offered genetics evaluation. This report will contribute to an increased awareness of HDGC.
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http://dx.doi.org/10.3748/wjg.v26.i42.6689DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7673959PMC
November 2020

Arteriovenous malformation in the pancreatic head initially mimicking a hypervascular mass treated with duodenum-preserving pancreatic head resection: a case report.

Surg Case Rep 2020 Dec 1;6(1):301. Epub 2020 Dec 1.

Department of Surgery, Surgical Oncology and Science, Sapporo Medical University School of Medicine, 291 Minami-1-jo Nishi 16-chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.

Background: The mainstay treatment for arteriovenous malformation in the pancreatic head (Ph-AVM) is standard pancreatectomy, especially pancreaticoduodenectomy (PD), or interventional endovascular treatment. We report the first case of Ph-AVM treated with duodenum-preserving pancreatic head resection (DPPHR) performed to preserve the periampullary organs.

Case Presentation: A 59-year-old man presenting with back pain underwent contrast-enhanced computed tomography followed by angiography of the anterior superior pancreaticoduodenal artery. He was diagnosed with Ph-AVM and indicated for DPPHR with preservation of the periampullary organs; Ph-AVM's benign nature seldom requires lymph node dissection. During the operation, the right colon was mobilized and the omental bursa was released to expose the periampullary structures. The pancreas was transected just above the superior mesenteric vein. The inferior pancreaticoduodenal artery and papillary arteries branching from the posterior superior pancreaticoduodenal artery were carefully preserved to maintain the blood flow to the lower bile duct and papilla of Vater. The remnant pancreas was reconstructed with pancreaticogastrostomy using the modified Blumgart method. Pathological examination of the resected specimen revealed an irregular course of the arteries and veins concomitant with marked dilation throughout the pancreatic head. The patient was pathologically diagnosed with Ph-AVM. He developed hematemesis caused by a rupture of the pseudoaneurysm on postoperative day 20 and underwent coil embolization. A bilio-enteric fistula and stenosis of the common bile duct were found and treated by placement of an endoscopic biliary stent. At the 8-month follow-up, the Ph-AVM had not recurred.

Conclusions: Compared to PD, DPPHR confers the clinical benefit of preserving the periampullary organs, although further studies are needed to confirm this. Therefore, the choice of this procedure should be based on the surgical morbidities and long-term outcome of the patient.
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http://dx.doi.org/10.1186/s40792-020-01075-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7708544PMC
December 2020

Response to 'Is the case really a SMARCA4-deficient thoracic sarcoma?'

Thorac Cancer 2021 01 18;12(1):141. Epub 2020 Nov 18.

Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan.

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http://dx.doi.org/10.1111/1759-7714.13751DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7779208PMC
January 2021

Successful Treatment by Surgery and Lenvatinib of a Patient with Adrenal Metastasis of Papillary Thyroid Cancer.

Case Rep Oncol Med 2020 5;2020:2107430. Epub 2020 Nov 5.

Department of Medical Oncology, Sapporo Medical University School of Medicine, Japan.

Papillary thyroid cancer (PTC) is considered an indolent cancer, but some PTC patients do present with distant metastases and treatment strategies for such patients are not well established. Recently, lenvatinib, an inhibitor of multiple tyrosine kinases, has been introduced to treat patients with advanced PTC but carries a risk of serious adverse events such as hemorrhage. Here, we report a PTC patient with a left adrenal metastasis and lenvatinib-induced hemorrhage who underwent successful surgical resection and was subsequently treated with a lower dose of lenvatinib. The patient has now been in a stable state with no adverse events for nearly two years. This case highlights the importance of surgical resection of metastatic PTC and subsequent lenvatinib therapy, even when the tumor is at an advanced stage.
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http://dx.doi.org/10.1155/2020/2107430DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7661144PMC
November 2020

Myoepithelioma of soft tissue and bone, and myoepithelioma-like tumors of the vulvar region: Clinicopathological study of 15 cases by PLAG1 immunohistochemistry.

Pathol Int 2020 Dec 17;70(12):965-974. Epub 2020 Sep 17.

Department of Surgical Pathology, Sapporo Medical University, School of Medicine, Hokkaido, Japan.

We demonstrated the clinicopathological findings of 13 myoepitheliomas of soft tissue and bone (MESTBs) and two myoepithelioma-like tumors of the vulvar region (MELTVRs), focusing on the association between nuclear atypia and clinical course, and the utility of immunohistochemistry (IHC) of pleomorphic adenoma gene 1 (PLAG1) for the pathological diagnosis of these tumors. Of the 13 MESTBs, eight, one and four cases exhibited mild, moderate and severe nuclear atypia, respectively. Two cases with venous invasion showed severe nuclear atypia and both died of advanced disease. Two MELTVR cases showed moderate nuclear atypia and had no evidence of disease after surgery. On IHC, 12 of 13 (92.3%) MESTBs showed PLAG1 immunoreactivity and none of the MELTVRs expressed PLAG1. In addition, MELTVRs showed loss of INI1 expression. In contrast, all MESTBs retained INI1 expression. Fluorescence in situ hybridization detected EWSR1, FUS and PLAG1 rearrangement in 5 (38.5%), 0 (0%) and 2 (15.4%) of the 13 MESTBs, respectively. No EWSR1, FUS and PLAG1 rearrangement were observed in the METLVRs. In conclusion, MESTBs with both severe nuclear atypia and venous invasion would be indicative of malignant potential. PLAG1 might be a useful IHC marker in MESTB diagnosis.
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http://dx.doi.org/10.1111/pin.13017DOI Listing
December 2020

Surgically treated gastric melanoma of unknown primary: A case report from a 10-year survivor.

Pathol Int 2020 Oct 16;70(10):786-792. Epub 2020 Aug 16.

Department of Surgery, Hokkaido Gastroenterology Hospital, Hokkaido, Japan.

We describe an extremely rare case of simultaneous double melanoma of the stomach with no other obvious primary source. The patient has survived for more than 12 years post-complete gastrectomy. The patient was a woman in her seventies who was referred for anemia by another clinic. Esophagogastroscopy revealed an ulcerated gastric tumor that was diagnosed as a gastrointestinal stromal tumor (GIST) by endoscopic biopsy. She was admitted to our hospital for further examination and treatment. Gastroscopy at our institution revealed two submucosal tumors in the gastric wall. Since no metastatic lesions were detected after systemic exploration, multiple GIST of the stomach was diagnosed, and a total gastrectomy was performed. Malignant melanoma was diagnosed in both lesions by a histopathological study of the resected stomach. The patient's postoperative progress was good, and thorough examination of the skin did not result in the discovery of any systemic neoplastic lesions which could be regarded as the source for the primary tumor. No anticancer treatments were used. The patient has survived, with no recurrence for over ten years postsurgery. Strong evidence is presented in this case for the diagnosis and treatment of gastric malignant melanoma.
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http://dx.doi.org/10.1111/pin.12991DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7689816PMC
October 2020

Osteofibrous dysplasia-like adamantinoma treated via intercalary segmental resection with partial cortex preservation using pedicled vascularized fibula graft: a case report.

World J Surg Oncol 2020 Aug 13;18(1):203. Epub 2020 Aug 13.

Department of Orthopedic Surgery, Sapporo Medical University School of Medicine, West 16, South 1, Chuo- ku, Sapporo, Hokkaido, 060-8543, Japan.

Background: Morphologically, osteofibrous dysplasia-like adamantinoma is thought to be intermediate between osteofibrous dysplasia and adamantinoma. Its treatment is not well established owing to its rarity.

Case Presentation: We report about of a 10-year-old girl with osteofibrous dysplasia-like adamantinoma initially diagnosed as osteofibrous dysplasia and treated via intercalary segmental resection with partial cortex preservation using a pedicled vascularized fibula graft for reconstruction. Bone union was observed 9 weeks after surgery. Twenty-two months after the definitive surgery, no recurrence was observed.

Conclusion: This case illustrates the upgrade from osteofibrous dysplasia to osteofibrous dysplasia-like adamantinoma. The surgical method may aid the treatment of osteofibrous dysplasia-like adamantinoma with incomplete cortex involvement of the tumor.
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http://dx.doi.org/10.1186/s12957-020-01983-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7427073PMC
August 2020

Mucinous carcinoma originating in the peritoneum diagnosed by an ascites cell block: a case report.

J Med Case Rep 2020 Jul 14;14(1):99. Epub 2020 Jul 14.

Department of Obstetrics and Gynecology, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan.

Background: Peritoneal carcinoma is a rare disease that is diagnosed and treated in a manner similar to ovarian cancer. In most cases, the histological type is serous carcinoma, and chemotherapy is effective. However, there are a few case reports of mucinous peritoneal carcinoma. We inferred the histological type before surgery using an ascites cell block sample, which was useful for determining the treatment plan.

Case Presentation: Our patient was a 60-year-old Japanese woman. She presented with a feeling of fullness in the abdomen. A computed tomographic scan showed a large quantity of ascitic fluid and thickening of the greater omentum, as well as thickening of the peritoneum at the pouch of Douglas and diaphragm. Hence, peritoneal carcinoma was suspected. The tumor markers carcinoembryonic antigen, cancer antigen 19-9, and cancer antigen 125 were all increased, and no malignant findings were observed in the uterus or ovaries. Cells suggestive of carcinoma were found in the ascitic fluid, and immunostaining by the cell block method suggested the possibility of mucinous carcinoma. The preoperative chemotherapy strategy was changed to short courses, and tumor reduction surgery was planned. Similar to the suspicion before surgery, the pathology results indicated mucinous carcinoma, and the therapeutic effect of chemotherapy was grade 0.

Conclusions: Determining whether peritoneal carcinoma is serous carcinoma is important for therapy and prognostic prediction. In this case, we encountered a patient for whom surgery was chosen because of drug therapy resistance inferred through histological type estimation using the cell block method. Inferring the histological type by cell block preparation is useful for diagnosis and treatment selection.
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http://dx.doi.org/10.1186/s13256-020-02435-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7359487PMC
July 2020

Fatal fulminant hepatitis induced by combined ipilimumab and nivolumab therapy despite favorable histologic response and confirmed by autopsy in a patient with clear cell renal cell carcinoma.

Immunol Med 2021 Jun 7;44(2):136-141. Epub 2020 Jul 7.

Department of Pathology, School of Medicine, Sapporo Medical University, Sapporo, Japan.

Effective management of immune-related adverse events in patients receiving immunotherapy for cancer is problematic. In this report, we present the case of a 58-year-old man with advanced clear cell renal cell carcinoma who responded well to a combination of ipilimumab and nivolumab. However, after two courses of treatment, he developed fulminant hepatitis and died. An autopsy confirmed that the primary lesion in the left kidney was more than 99% necrotic with only six small residual tumor lesions. These lesions were infiltrated by large numbers of CD8-positive/TIA-1-positive lymphocytes. However, a metastatic lesion in the right kidney harbored few lymphocytes. Furthermore, the tumor cells in the metastatic lesion and one of the residual lesions showed decreased expression of HLA class I molecules, which are a prerequisite for cytotoxic T-lymphocyte-mediated immunotherapy in tumor cells. In this patient, more than 80% of hepatocytes were destroyed and the parenchyma was infiltrated with CD8-positive/TIA-1-positive lymphocytes. The patient had polyuria, which was attributed to neurohypophysitis caused by the infiltration of CD8-positive/TIA-1-positive lymphocytes. We believe that this is an instructive case for immuno-oncologists.
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http://dx.doi.org/10.1080/25785826.2020.1788229DOI Listing
June 2021

Detection of VHL deletion by fluorescence in situ hybridization in extraneuraxial hemangioblastoma of soft tissue.

Pathol Int 2020 07 17;70(7):473-475. Epub 2020 Apr 17.

Department of Surgical Pathology, School of Medicine, Sapporo Medical University, Hokkaido, Japan.

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http://dx.doi.org/10.1111/pin.12935DOI Listing
July 2020

Pulmonary metastasectomy is associated with prolonged survival among patients with bone and soft tissue sarcoma.

Mol Clin Oncol 2020 May 27;12(5):429-434. Epub 2020 Feb 27.

Department of Orthopaedic Surgery, Sapporo Medical University School of Medicine, Sapporo, Hokkaido 060-8543, Japan.

The aim of the present study was to evaluate the survival impact of surgical resection among patients with pulmonary metastases from bone and soft tissue sarcomas. A total of 34 consecutive patients with ≤5 pulmonary metastases from bone and soft tissue sarcomas were retrospectively reviewed. The patients included 19 men and 15 women, with a median age of 64.0 years and a median follow-up of 14.5 months. The oncological outcome was compared between patients who underwent surgical and non-surgical treatment. A total of 22 patients underwent surgery and 12 patients did not undergo surgery. The surgery group had 3- and 5 year overall survival rates of 62 and 53%, respectively. None of the patients in the non-surgery group survived to 3 years. Compared with the non-surgery group, surgery achieved significantly better 3- and 5 year overall survival rates. Pulmonary metastasectomy was associated with significantly improved survival among patients who were aged <64 years (P=0.0155), as well as those who were aged ≥64 years (P=0.0444), which indicated that age was not associated with a difference in survival between the two groups. Therefore, pulmonary metastasectomy may improve the prognosis of patients with pulmonary metastases from bone and soft tissue sarcomas.
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http://dx.doi.org/10.3892/mco.2020.2009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7087474PMC
May 2020

Genetic analyses of mosaic neurofibromatosis type 1 with giant café-au-lait macule, plexiform neurofibroma and multiple melanocytic nevi.

J Dermatol 2020 Jun 4;47(6):658-662. Epub 2020 Apr 4.

Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

Neurofibromatosis type 1 (NF1) is a genodermatosis caused by heterozygous germ line variations in the NF1 gene. A second-hit NF1 aberration results in the formation of café-au-lait macules, cutaneous neurofibroma and plexiform neurofibroma (PNF). Mosaic NF1 (mNF1), caused by a postzygotic NF1 mutation, is characterized by localized or generalized NF1-related manifestations. Although NF1 and mNF1 are associated with pigmentary skin lesions, clinically recognizable melanocytic nevi that developed over PNF have not been reported. Here, we report the first case of multiple melanocytic nevi that developed on a giant café-au-lait macule and PNF. The PNF had biallelic NF1 deletions, a whole deletion of NF1 and a novel intragenic deletion involving exons 25-30. The deletions were not detected in the blood, which resulted in the diagnosis of mNF1. Furthermore, the nevus cells had not only biallelic NF1 deletions but also NRAS Q61R, a common mutation found in congenital melanocytic nevi. These analyses revealed the coexistence of the two different mosaic diseases, mNF1 and congenital melanocytic nevi. For a diagnosis of cases with atypical NF1-like symptoms, genetic analyses using blood and lesional tissues are useful and aid in genetic counseling.
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http://dx.doi.org/10.1111/1346-8138.15327DOI Listing
June 2020

High Specificity of BCL11B and GLG1 for EWSR1-FLI1 and EWSR1-ERG Positive Ewing Sarcoma.

Cancers (Basel) 2020 Mar 10;12(3). Epub 2020 Mar 10.

Max-Eder Research Group for Pediatric Sarcoma Biology, Institute of Pathology, Faculty of Medicine, LMU Munich, 80337 Munich, Germany.

Ewing sarcoma (EwS) is an aggressive cancer displaying an undifferentiated small-round-cell histomorphology that can be easily confused with a broad spectrum of differential diagnoses. Using comparative transcriptomics and immunohistochemistry (IHC), we previously identified BCL11B and GLG1 as potential specific auxiliary IHC markers for -positive EwS. Herein, we aimed at validating the specificity of both markers in a far larger and independent cohort of EwS (including -positive cases) and differential diagnoses. Furthermore, we evaluated their intra-tumoral expression heterogeneity. Thus, we stained tissue microarrays from 133 molecularly confirmed EwS cases and 320 samples from morphological mimics, as well as a series of patient-derived xenograft (PDX) models for BCL11B, GLG1, and CD99, and systematically assessed the immunoreactivity and optimal cut-offs for each marker. These analyses demonstrated that high BCL11B and/or GLG1 immunoreactivity in CD99-positive cases had a specificity of 97.5% and an accuracy of 87.4% for diagnosing EwS solely by IHC, and that the markers were expressed by -positive EwS. Only little intra-tumoral heterogeneity in immunoreactivity was observed for differential diagnoses. These results indicate that BCL11B and GLG1 may help as specific auxiliary IHC markers in diagnosing EwS in conjunction with CD99, especially if confirmatory molecular diagnostics are not available.
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http://dx.doi.org/10.3390/cancers12030644DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139395PMC
March 2020

Molecular Aberrations Associated with Seizure Control in Diffuse Astrocytic and Oligodendroglial Tumors.

Neurol Med Chir (Tokyo) 2020 Mar 31;60(3):147-155. Epub 2020 Jan 31.

Department of Surgical Pathology, Sapporo Medical University.

Diffuse astrocytic and oligodendroglial tumors are frequently associated with symptomatic epilepsy, and predictive seizure control is important for the improvement of patient quality of life. To elucidate the factors related to drug resistance of brain tumor-associated epilepsy from a pathological perspective. From January 2012 to October 2017, 36 patients diagnosed with diffuse astrocytic or oligodendroglial tumors were included. Assessment for seizure control was performed according to the Engel classification of seizures. Patient clinical, radiological, and pathological data were stratified based on the following 16 variables: age, sex, location of tumor, existence of the preoperative seizure, extent of resection, administration of temozolomide, radiation therapy, recurrence, Karnofsky performance scale, isocitrate dehydrogenase 1, 1p/19q co-deletion, Olig2, platelet-derived growth factor receptor alpha, p53, ATRX, and Ki67. These factors were compared between the well-controlled group and drug-resistant seizure group. Twenty-seven patients experienced seizures; of these, 14 cases were well-controlled, and 13 cases were drug-resistant. Neither clinical nor radiological characteristics were significantly different between these two groups, though p53 immunodetection levels were significantly higher, and the frequency of 1p/19q co-deletion was significantly lower in the group with drug-resistant seizures than in the well-controlled group. In the multivariate analysis, only one item was selected according to stepwise methods, and a significant difference was observed for p53 (OR, 21.600; 95% CI, 2.135-218.579; P = 0.009). Upregulation of p53 may be a molecular mechanism underlying drug resistant epilepsy associated with diffuse astrocytic and oligodendroglial tumors.
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http://dx.doi.org/10.2176/nmc.oa.2019-0218DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7073702PMC
March 2020

Early Lymph Node Metastasis May Predict Poor Prognosis in Soft Tissue Sarcoma.

Int J Surg Oncol 2019 12;2019:6708474. Epub 2019 Dec 12.

Department of Orthopedic Surgery, Sapporo Medical University School of Medicine, Sapporo, Hokkaido 060-8543, Japan.

Background: Lymph node metastasis (LNM) is a relatively rare event in soft tissue sarcoma. An association between the timing of LNM detection and patient prognosis is presently unknown.

Patients And Methods: We retrospectively analyzed the clinicopathological features of 33 patients with LNM between 2001 and 2015. Analysis of the timing of LNM diagnosis was grouped according to patients presenting LNM in either <8 months (the median time from primary tumor diagnosis to LNM) or ≥8 months after primary tumor diagnosis.

Results: A relationship between the primary tumor size and the timing of the LNM was not significantly found ( = 0.0088, =0.96). Sixteen patients had an LNM detection duration of <8 months, and 17 patients had a duration of ≥8 months. The 5-year survival for patients with an LNM detection duration of <8 months and ≥8 months was 19% and 71%, respectively (=0.0016). There were 19 patients with pulmonary metastases. Among them, there were 13 patients with a duration of primary tumor diagnosis to LNM of <8 months and 6 with a duration of ≥8 months (=0.01).

Conclusion: Early LNM (<8 months) may predict poor prognosis in soft tissue sarcoma.
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http://dx.doi.org/10.1155/2019/6708474DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6930751PMC
February 2020

Exceptionally rapid response to pembrolizumab in a SMARCA4-deficient thoracic sarcoma overexpressing PD-L1: A case report.

Thorac Cancer 2019 12 16;10(12):2312-2315. Epub 2019 Oct 16.

Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan.

SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a new clinical entity characterized by SMARCA4 inactivation and has a dismal prognosis because of rapid growth. Effective treatments for SMARCA4-DTS have not yet been developed. Most recently, anti-programmed cell death 1 receptor (PD-1) blockade has been effective for SMARCA4-deficient lung cancer and malignant rhabdoid tumor-like tumors. Here, we describe a patient with SMARCA4-DTC who experienced a marked response to the administration of pembrolizumab. A 70-year-old female was referred to our department for treatment of SMARCA4-DTC. Positron emission tomography-computed tomography had revealed a left mediastinal tumor, peritoneal dissemination and multiple cutaneous metastases at diagnosis. Immunohistochemical analyses revealed 60% of tumor cells expressed programmed cell death ligand 1 (PD-L1). The patient was given pembrolizumab as first-line treatment. Pembrolizumab suppressed tumor growth dramatically, with only one dose leading to a partial response. Our case suggests the immunohistochemical analysis of PD-L1 expression be undertaken for patients with SMARCA4-DTS and that pembrolizumab treatment may be a promising strategy for PD-L1-positive SMARCA4-DTS.
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http://dx.doi.org/10.1111/1759-7714.13215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6885443PMC
December 2019

Total Resection of Unilateral Adult-onset Xanthogranuloma of the Orbit via a Transcranial Orbital Approach.

NMC Case Rep J 2019 Oct 14;6(4):121-124. Epub 2019 Sep 14.

Department of Neurosurgery, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan.

Adult orbital xanthogranuloma is rare and usually associated with systemic disease. There are several options available to treat this disorder. Periorbital lesions are treated with steroids, chemotherapy, radiotherapy, or local excision; however, there is still no consensus regarding optimal treatment. Here, we report a rare case of orbital xanthogranuloma that was not associated with systemic disease and was treated by transcranial surgery. The patient was a 52-year-old man who presented with a 2-year history of unilateral eye symptoms. A computed tomography scan revealed a well-defined mass in the right orbit. The mass was completely removed via a transcranial orbital approach. The histopathologic diagnosis was xanthogranuloma. No recurrence was observed during 15 months of postoperative follow-up. Complete surgical resection might be an effective treatment option for locally growing sporadic adult xanthogranulomatous disease of the orbit, and allows systemic steroids, chemotherapy, and irradiation to be avoided.
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http://dx.doi.org/10.2176/nmccrj.cr.2018-0304DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6776754PMC
October 2019

Agminated fibroblastic connective tissue nevus in a 1-year-old boy.

Pediatr Dermatol 2019 Nov 30;36(6):997-998. Epub 2019 Aug 30.

Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Japan.

Fibroblastic connective tissue nevus (FCTN) is a benign cutaneous mesenchymal lesion characterized by proliferation of CD34-positive fibroblastic/myofibroblastic spindle-shaped cells. We report a case of agminated FCTN on the right lower abdomen of a 1-year-old boy.
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http://dx.doi.org/10.1111/pde.13950DOI Listing
November 2019

Pneumothorax as an Adverse Event in Patients with Lung Metastasis of Soft Tissue Sarcoma under Eribulin Treatment.

Intern Med 2019 Oct 27;58(20):3009-3012. Epub 2019 Jun 27.

Department of Medical Oncology, Sapporo Medical University School of Medicine, Japan.

Pneumothorax has been reported as a pazopanib-associated adverse event in patients with lung metastases of soft tissue sarcoma (STS). However, pneumothorax triggered by eribulin treatment has never been reported. We herein report two cases of spontaneous pneumothorax in patients with STS treated with eribulin. Both patients experienced pneumothorax accompanied by sudden dyspnea on day 9 or 10 of eribulin treatment. These two cases suggest that spontaneous pneumothorax may occur as an adverse event of eribulin treatment in such patients. We should therefore be alert for the potential development of pneumothorax during eribulin treatment of patients with STS and lung metastases.
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http://dx.doi.org/10.2169/internalmedicine.2790-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6859383PMC
October 2019

Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature.

Diagn Pathol 2019 May 18;14(1):43. Epub 2019 May 18.

Department of Surgical Pathology, Hokkaido University Hospital, N14W4, Kita-ku, Sapporo, Japan.

Background: Desmoplastic small round cell tumor (DSRCT) is a rare soft tissue tumor that generally involves the retroperitoneum, pelvis, omentum and mesentery in younger patients. However, extra-abdominal DSRCT is very rare.

Case Presentation: A 49-year-old Japanese man noticed a mass in the right parotid gland. Ultrasound examination revealed a solid tumor about 2 cm in diameter. Computed tomography (CT) of the whole body revealed no other tumors or lymph node swelling. Superficial parotidectomy was performed. Histologically, the tumor was composed of various-sized tumor cell nests in an abundant fibromyxoid and collagenous background. The tumor cells were small to medium-sized. Immunohistochemistry showed that the tumor cells were immunoreactive for epithelial markers and desmin. They also showed strong nuclear staining with a Wilms tumor 1 (WT1) antibody detecting the C-terminal region (C-WT1), but not the N-terminal region (N-WT1). We also performed 3'/5' expression imbalance assay based on reverse transcription polymerase chain reaction (RT-PCR) to determine whether aberrant WT1 gene expression was present. This tumor was found to lack 5'-regional expression of the WT1 gene, as well as immunoreactivity with the N-WT1 antibody. Finally, fluorescence in situ hybridization (FISH) and RT-PCR analyses revealed the presence of a gene showing fusion between exon 7 of EWSR1 and exon 8 of WT1. The tumor was diagnosed as a DSRCT of the right parotid gland. The patient has been followed for 3 years without recurrence or metastasis.

Conclusions: Although DSRCT in the salivary gland is extremely rare, it should be included in the differential diagnosis of poorly differentiated salivary gland neoplasms, especially with a fibromyxoid background. Pathologists should bear in mind that DSRCT may occur in major salivary glands and should perform immunohistochemistry with appropriate antibodies, not only those against keratin and desmin, but also one detecting the C-terminal region of WT-1. Furthermore, molecular detection of EWSR1-WT1 fusion gene conclusively confirmed the diagnosis of DSRCT in this uncommon location.
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http://dx.doi.org/10.1186/s13000-019-0825-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6525968PMC
May 2019

Combination of eribulin plus AKT inhibitor evokes synergistic cytotoxicity in soft tissue sarcoma cells.

Sci Rep 2019 04 8;9(1):5759. Epub 2019 Apr 8.

Department of Medical Oncology, Sapporo Medical University School of Medicine, Sapporo, Japan.

An activated AKT pathway underlies the pathogenesis of soft tissue sarcoma (STS), with over-expressed phosphorylated AKT (p-AKT) correlating with a poor prognosis in a subset of STS cases. Recently, eribulin, a microtubule dynamics inhibitor, has demonstrated efficacy and is approved in patients with advanced/metastatic liposarcoma and breast cancer. However, mechanisms of eribulin resistance and/or insensitivity remain largely unknown. In this study, we demonstrated that an increased p-AKT level was associated with eribulin resistance in STS cells. We found a combination of eribulin with the AKT inhibitor, MK-2206, synergistically inhibited STS cell growth in vivo as well as in vitro. Mechanistically, eribulin plus MK-2206 induced G1 or G2/M arrest by down-regulating cyclin-dependent kinases, cyclins and cdc2, followed by caspase-dependent apoptosis in STS cells. Our findings demonstrate the significance of p-AKT signaling for eribulin-resistance in STS cells and provide a rationale for the development of an AKT inhibitor in combination with eribulin to treat patients with STS.
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http://dx.doi.org/10.1038/s41598-019-42300-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6453888PMC
April 2019
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