Author
Toshiaki Otsuki
Bio: Toshiaki Otsuki is an academic researcher from Shinshu University. The author has contributed to research in topics: Adenocarcinoma & PAX8. The author has an hindex of 3, co-authored 9 publications receiving 23 citations.
Topics: Adenocarcinoma, PAX8, Antibody, CD20, Cytology
Papers
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TL;DR: It was difficult to distinguish the present case as a pancreatobiliary-type (PB-type) intraductal papillary mucinous neoplasm (IPMN) or a phenotypic variant of ITPN, as it is possible that some cases of PB-type IPMN and ITPn overlap, the precise classification of these rare lesions may require re-evaluation.
Abstract: This report describes a unique case of intraductal tubulopapillary neoplasm (ITPN) of the pancreas in order to clarify its oncogenesis and more precisely classify pancreatic intraductal neoplasms. A 74-year-old man visited our institution for follow-up of acute pancreatitis. Imaging examinations revealed a hypovascular intraductal mass in the head of the pancreas with progressive dilation of the pancreatic duct, atrophy of the pancreatic parenchyma, and a non-mucinous appearance. A pancreatoduodenectomy was performed to identify this pancreatic intraductal neoplasm. Macroscopically, the tumor was a solid nodular mass with no visibly secreted mucin obstructing the dilated ducts. Histologically, it had a homogeneous appearance with nodules of back-to-back tubular glands and occasional papillary elements, and there were no apparent transitions to areas with less marked cytoarchitectural atypia. Although the intraductal neoplastic growth corresponded to an ITPN, immunohistochemical staining revealed partial positivity for MUC5AC, for which ITPNs are characteristically negative. Somatic mutations in KRAS, GNAS, BRAF, and PIK3CA were not detected. A loss of MUC5AC expression and mutations in KRAS and GNAS are key elements in the diagnosis of ITPN. Thus, it was difficult to distinguish the present case as a pancreatobiliary-type (PB-type) intraductal papillary mucinous neoplasm (IPMN) or a phenotypic variant of ITPN. As it is possible that some cases of PB-type IPMN and ITPN overlap, the precise classification of these rare lesions may require re-evaluation.
11 citations
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TL;DR: A 70-year-old Japanese male who underwent radical right nephrectomy for a right renal mass identified on computed tomography was found to be urothelial carcinoma and collecting duct carcinoma with sarcomatoid changes, and programmed deathligand 1 was highly expressed with a tumor proportion score of more than 10% as mentioned in this paper .
Abstract: Collecting duct carcinoma and sarcomatoid renal cell carcinoma are tumors with poor prognosis. Immune checkpoint inhibitors have been established as the standard treatment for advanced renal cell carcinoma. Some cases of remission of collecting duct carcinoma and sarcomatoid renal cell carcinoma have been reported using immune checkpoint inhibitor interventions. Specifically, sarcomatoid renal cell carcinoma expresses high levels of programmed death-ligand 1, an immune checkpoint protein, and immune checkpoint inhibitors have been reported to be highly effective for treating sarcomatoid renal cell carcinoma.We describe the case of a 70-year-old Japanese male who underwent radical right nephrectomy for a right renal mass identified on computed tomography. The pathological examination demonstrated that the renal mass was urothelial carcinoma and collecting duct carcinoma with sarcomatoid changes, and programmed death-ligand 1 was highly expressed with a tumor proportion score of more than 10%. There was no evident submucosal connective tissue invasion in the urothelial carcinoma component, and collecting duct carcinoma was diagnosed as primary cancer. The tumor-node-metastasis classification was pT3aN0, venous invasion 1, lymphovascular invasion 0, and Fuhrman nuclear grade 4. Two months after the nephrectomy, multiple metastases were observed in both lungs, the right hilar lymph node, and the S6 segment of the right liver lobe. We initiated first-line combination therapy with nivolumab (240 mg, fixed dose) and ipilimumab (1 mg/kg). One day after administration, the patient developed drug-induced interstitial pneumonia, thus we applied steroid injections. After one administration of immunotherapy, the metastatic lesion showed complete response within 6 months, which was maintained after 3 years.We report the first case of complete response to a single dose of combination therapy with nivolumab and ipilimumab for metastatic collecting duct carcinoma with sarcomatoid changes and high expression of programmed death-ligand 1. This case suggests high expectations for immune checkpoint inhibitors as treatment for sarcomatoid-transformed renal carcinoma tumors that express high levels of programmed death-ligand 1.
5 citations
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TL;DR: Sjögren syndrome-related plasma cell disorder may be responsible for the formation of this unique multifocal nodular AL amyloidosis.
Abstract: Background: Localized nodular deposits of AL amyloid are seen in different tissues/organs; however, the pathogenesis of this form of amyloidosis remains unclear. Recently, Sjogren syndrome combined with localized nodular AL amyloidosis has been noted. Here, we report Sjogren syndrome cases showing multifocal nodular AL amyloidosis and the followed benign course. Materials and methods: We investigated the clinical pictures and histopathological findings of three cases with both presence of Sjogren syndrome and localized nodular AL amyloidosis, paying a special attention to the distribution of amyloidoma. Results: All three cases were middle-aged females. In two of three cases localized deposits of AL amyloid preceded Sjogren syndrome. Amyloidoma was detected in scalp, eyelid, cheek, larynx, trachea, lung and breast, and around these amyloid-deposited lesions infiltration of plasma cells was seen. Pulmonary amyloidosis was consistently accompanied with parenchymal cystic lesions, but this amyloidosis did not produce any significant respiratory symptoms. Some of large pulmonary amyloidomas showed cavity formation and subsequent shrinkage. In two cases amyloid deposition was found on gastric mucosa. Two cases received small doses of oral prednisone, with no further appearance of amyloidoma. Conclusion: Sjogren syndrome-related plasma cell disorder may be responsible for the formation of this unique multifocal nodular AL amyloidosis.
5 citations
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TL;DR: The immunohistochemical analysis showed that gastric mucin (MUC5AC) was strongly positive and intestinal mucin(MUC2) was weakly and partially positive, and the final diagnosis of Hp-negative well-differentiated early gastric cancer was made.
Abstract: A 58-year-old Japanese man was diagnosed with differentiated adenocarcinoma of the stomach. Histological findings of the resected specimen revealed well- to moderately-differentiated tubular adenocarcinoma (tub1, tub2), 13 mm in diameter, which invaded into the submucosa (SM1, 300 μm) and lymphovascular lumen (ly1). Serum antibody against Helicobacter pylori (Hp) and the (13)C-urea breath test were negative, and there were no atrophic changes in the tumor-adjacent mucosa. The immunohistochemical analysis showed that gastric mucin (MUC5AC) was strongly positive and intestinal mucin (MUC2) was weakly and partially positive. According to these results, the final diagnosis of Hp-negative well-differentiated early gastric cancer was made.
4 citations
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TL;DR: A 32‐year‐old woman who was referred to the authors' hospital because of severe psychosis and was found to have an ectopic ACTH‐producing thymic neuroendocrine tumor with positive anti‐ACTH immunohistochemical staining is presented.
Abstract: A 32-year-old woman was referred to our hospital because of severe psychosis and was found to have an ectopic ACTH-producing thymic neuroendocrine tumor. Laboratory data revealed an elevated serum cortisol and plasma ACTH level, hypokalemia, and metabolic alkalosis. Chest computed tomography (CT) revealed an anterior mediastinal mass and multiple pulmonary nodules. As the patient was unable to communicate because of her consciousness disturbance, she was managed with artificial ventilation and deep sedation. Metyrapone and potassium supplementation were administered, and steroid psychosis gradually improved. Thoracic surgery was performed and the histopathological diagnosis was thymic neuroendocrine tumor with positive anti-ACTH immunohistochemical staining. Here we present details of the case and review the literature.
4 citations
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TL;DR: The diagnosis and therapeutic strategies including the modern, currently available tools and emphasizing the operational effectiveness of care are discussed.
Abstract: Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.
102 citations
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TL;DR: Despite the difficulties of determining the extent of invasive carcinoma in many cases, the overall outcome seems to be relatively favorable and substantially better than that of conventional pancreatic ductal adenocarcinoma, even when only the cases with invasive carcinomas are considered.
Abstract: Intraductal tubulopapillary neoplasm (ITPN) is a relatively recently described member of the pancreatic intraductal neoplasm family. Thus, the literature on its histologic and immunohistochemical features, clinical behavior, and its similarities and differences from other pancreatic neoplasms is limited. Thirty-three cases of ITPN, the largest series to date, were identified. Immunohistochemical labeling for cytokeratins, glycoproteins, pancreatic enzymes, markers for intestinal and neuroendocrine differentiation, and antibodies associated with genetic alterations previously described in pancreatic neoplasms was performed. Clinicopathologic features and survival was assessed. Seventeen patients were female and 14 were male. Mean age was 55 years (range, 25 to 79 y). Median overall tumor size was 4.5 cm (range, 0.5 to 15 cm). Forty-five percent of the tumors occurred in the head, 32% in the body/tail, and 23% showed diffuse involvement. Microscopically, the tumors were characterized by intraductal nodules composed of tightly packed small tubular glands lined by cuboidal cells lacking apparent mucin. Although it was often challenging to determine its extent, invasion was present in 71%. Almost all tumors labeled for CAM5.2, CK7, and CK19; most expressed CA19.9, MUC1, and MUC6. CDX2, MUC2, trypsin, chymotrypsin, chromogranin, and synaptophysin were not expressed. SMAD4 expression was retained in 100%; p16 expression and p53 overexpression was seen in 33% and 27%, respectively. Follow-up information was available for 22 patients (median follow-up, 45 mo; range, 11 to 173 mo). Two patients with invasive carcinoma died of disease at 23 and 41 months, respectively. One patient died of unrelated causes at 49 months. Twelve patients were alive with disease. Seven patients were alive with no evidence of disease. The overall 1-, 3-, and 5-year survival rates were 100% in patients without an invasive component and 100%, 91%, and 71%, respectively, in patients with an invasive component (P=0.7). ITPN is a distinct clinicopathologic entity in the pancreas. Despite the difficulties of determining the extent of invasive carcinoma in many cases, the overall outcome seems to be relatively favorable and substantially better than that of conventional pancreatic ductal adenocarcinoma, even when only the cases with invasive carcinoma are considered.
62 citations
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TL;DR: The results suggest that intraductal tubulopapillary neoplasm has distinguishing genetic characteristics and some of these mutated genes are potentially targetable.
52 citations
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TL;DR: Despite the recent progress, more studies are necessary to assess the biology and genetics of ITPN for a better understanding of the prognostic factors and treatment options.
Abstract: Context.—Intraductal tubulopapillary neoplasm (ITPN) is a rare intraductal epithelial neoplasm of the pancreas recently recognized as a distinct entity by the World Health Organization classification in 2010. It is defined as an intraductal, grossly visible, tubule-forming epithelial neoplasm with high-grade dysplasia and ductal differentiation without overt production of mucin. The diagnosis can be challenging owing to morphologic overlap with other intraductal lesions and its rarity. While recent advances in molecular genetic studies of ITPN have provided new tools to facilitate clinical diagnosis, the limited number of cases has yielded limited follow-up data to guide management. Objective.—To provide a clinical, pathologic, and molecular update on ITPN with respect to clinical presentation, imaging findings, histopathologic features, differential diagnosis, biological behavior, molecular characteristics, and treatment options. Data Sources.—Analysis of the pertinent literature (PubMed) and authors' re...
26 citations