A patient with oligometastatic hormone-sensitive prostate cancer who achieved long-term progression-free survival following cytoreductive radical prostatectomy and metastasectomy.

Introduction: Oligometastatic prostate cancer can be well-controlled through combined local and metastasis-directed therapies. However, the effects of cytoreductive radical prostatectomy and metastasectomy remain unclear. Case presentation: A 52-year-old man presented with prostate cancer and isolated bone metastasis to the thoracic spine. Six months after neoadjuvant hormonal therapy, the patient underwent cytoreductive radical prostatectomy and total en bloc spondylectomy. The postoperative course was uneventful. Hormonal therapy was terminated 5 years after surgery, and no biochemical or radiological progression was observed at 7 years postoperatively. Conclusion: Although careful patient selection is necessary, cytoreductive radical prostatectomy and metastasectomy are effective treatments for well-selected patients with oligometastatic prostate cancer.

Increased expression of ATP-binding cassette transporters in enfortumab vedotin-resistant urothelial cancer.

Introduction: In addition to reduced nectin-4 expression, the upregulation of ATP-binding cassette transporters has been suggested as a potential mechanism of resistance to enfortumab vedotin. Case presentation: A 76-year-old man previously treated with platinum-containing chemotherapy and pembrolizumab for metastatic bladder cancer was administered enfortumab vedotin because of disease progression. Subsequently, metastasectomy was performed for oligometastatic lesions (in the lung and adrenal gland) that exhibited growth during enfortumab vedotin therapy. Immunostaining analysis revealed decreased nectin-4 expression and elevated MDR1, MRP1, and BCRP expression in the metastatic lesions. Conclusion: Decreased nectin-4 expression and increased ATP-binding cassette transporter expression are potential factors in the development of enfortumab vedotin resistance in urothelial carcinoma. Immunohistochemical evaluation of these proteins may aid in predicting treatment efficacy.

A resected case of pancreatic head cancer developing 40 years after lateral pancreaticojejunostomy for chronic pancreatitis.

A 72-year-old male patient presented to our department complaining of with upper abdominal pain and jaundice. He had a history of a side-to-side pancreaticojejunostomy performed 40 years previously for chronic pancreatitis. A diagnostic workup revealed a tumor 3 cm in size in the pancreatic head as the etiology of the jaundice. Subsequently, the patient was diagnosed with resectable pancreatic cancer. Following two cycles of neoadjuvant chemotherapy, an extended pancreatoduodenectomy was performed because of tumor invasion at the previous pancreaticojejunostomy site. Concurrent portal vein resection and reconstruction were performed. Pathological examination confirmed invasive ductal carcinoma (T2N1M0, Stage IIB). This case highlights the clinical challenges in pancreatic head carcinoma following a side-to-side pancreaticojejunostomy. Although pancreaticojejunostomy is believed to reduce the risk of pancreatic cancer in patients with chronic pancreatitis, clinicians should be aware that, even after this surgery, there is still a chance of developing pancreatic cancer during long-term follow-up.

A case of adrenal undifferentiated pleomorphic sarcoma with tertiary lymphoid structures responded to pembrolizumab.

Introduction: Although undifferentiated pleomorphic sarcomas are aggressive, a subset of these tumors are immunogenic and may respond to immunotherapy. Case presentation: A 69-year-old man developed bilateral adrenal tumors and underwent bilateral adrenalectomy. Pathological examination revealed undifferentiated pleomorphic sarcoma harboring tertiary lymphoid structures and infiltration of CD8+ T cells. Genome profiling revealed PD-L1 amplification, microsatellite instability, and a high tumor mutation burden. He developed local recurrence and multiple peritoneal dissemination 2 months after surgery; adriamycin chemotherapy was ineffective for these lesions. Sustained complete remission of all lesions was achieved by administering pembrolizumab. Conclusion: Immunohistochemical analysis focusing on tertiary lymphoid structures and genome profiling to evaluate microsatellite instability and tumor mutation burden are essential for precision medicine and informed clinical decision-making when treating advanced undifferentiated pleomorphic sarcoma.

A case of perirenal extra-adrenal myelolipoma mimicking liposarcoma.

Myelolipoma is a benign tumor composed of mature adipose tissue and normal hematopoietic components. It usually occurs in the adrenal glands but rarely in the extra-adrenal region. However, it is difficult to differentiate extra-adrenal myelolipoma from well-differentiated liposarcoma on the basis of the radiological findings. We report the case of a 66-year-old male with perirenal and extra-adrenal myelolipoma who underwent radical tumor resection with nephrectomy after a preoperative diagnosis of liposarcoma. Intraoperative assessment by the surgeon and intraoperative pathological evaluation are important considering the divergent prognoses of myelolipoma and liposarcoma.

Usefulness of cytological diagnosis in pancreatic endoscopic ultrasound-guided fine needle aspiration or biopsy: Comparison with histological diagnosis and relationship with puncture route and sample acquisition method.

BACKGROUND: The purpose of this study was to clarify the role of cytology when using endoscopic ultrasound-guided fine needle aspiration or biopsy (EUS-FNA/FNB) for pancreatic lesions by comparison with histology, and also to examine differences in diagnostic accuracy depending on the puncture route and sample acquisition method. METHODS: We studied 146 cases in which cytology and histology were performed when undertaking pancreatic EUS-FNA/FNB and the final histological diagnosis was obtained from surgically resected samples. Cytological, histological, and combined diagnoses with cytology and histology (combined diagnosis) detected malignant including suspected malignancy, indeterminate, and benign lesions. RESULTS: The accuracy of both cytology and histology in pancreatic EUS-FNA/FNB was 80.1%, with the combined diagnosis having an improved accuracy of 88.4%. The accuracy obtained with cytology was 80.0% for trans-duodenal puncture samples and 80.3% for trans-gastric puncture samples, with no difference between them. By contrast, the accuracy obtained with histology was 76.5% for trans-duodenal samples and 85.2% for trans-gastric samples, and they differed depending on the puncture route. The cytology accuracy was 80.9% for FNA and 79.8% for FNB, while the histology accuracy was 72.3% for FNA and 83.8% for FNB. CONCLUSIONS: Combining cytological diagnosis with histological diagnosis improved the diagnostic accuracy of EUS-FNA/FNB. Compared with histological diagnosis, cytological diagnosis showed stable diagnostic accuracy without being affected by differences in the puncture route or sample acquisition method.

Abdominal wall abscess resembling urachal carcinoma caused by ileal diverticulitis.

Introduction: We report a rare case of abdominal wall abscess caused by ileal diverticulitis that developed along the midline below the umbilicus and resembled a urachal carcinoma. Case presentation: A 76-year-old woman with diabetes presented with abdominal enlargement below the umbilicus. Computed tomography revealed a well-enhanced mass, which was visualized on magnetic resonance imaging as a continuous mass connected to the restiform structure, extending from the umbilicus to the bladder. As the mass showed high uptake on 18F-fluorodeoxyglucose positron emission tomography, urachal carcinoma was suspected, and surgery was subsequently performed. As the tumor adhered to the ileum, partial resection of the small intestine was required. The pathological diagnosis was abdominal wall abscess associated with ileal pseudodiverticulitis. Conclusion: Although abdominal wall abscess caused by ileal diverticulitis is rare, it should be considered as a differential diagnosis of urachal carcinoma.

A case of obstructive jaundice caused by metastasis of breast cancer to the intra/extrahepatic bile duct.

A 38-year-old woman was admitted to our hospital for a detailed examination of jaundice. Three years before, she had undergone a right total mastectomy and axillary lymph node dissection of her right breast because of cancer. Histopathological evaluation revealed invasive ductal carcinoma. Postoperatively, because multiple bone metastases were found, she underwent chemoradiotherapy. Endoscopic retrograde cholangiopancreatography was performed, which revealed widespread multiple stenoses with a smooth surface from the intrahepatic to the extrahepatic bile duct. A transpapillary biliary biopsy was performed. Histopathological examination revealed adenocarcinoma extending into the subepithelium of the bile duct. The obtained cancer cells were similar to those of the earlier invasive breast cancer. This rare case demonstrates bile duct metastasis of breast cancer with specific endoscopic retrograde cholangiopancreatography findings.

Castleman disease and mimickers: Clinicopathological findings of atypical lymphoproliferative disorders associated with autoimmune disease.

Atypical lymphoproliferative disorders (LPDs) related with autoimmune disease (AID) show marked clinicopathological diversity, which are defined as three distinct clinicopathological subtypes such as those resembling Castleman disease (CD), atypical paracortical hyperplasia with lymphoid follicles (APHLF), and atypical lymphoplasmacytic and immunoblastic proliferation (ALPIB). We studied excisional biopsy specimens from 31 patients with atypical LPDs associated with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren syndrome (SjS). The lesions in these 31 cases were classified into 6 (19.4%) cases resembling CD, 14 (45.2%) cases of APHLF, and 11 (35.5%) cases of ALPIB. Five cases (83.3%) resembling CD were in the active stage with systemic symptoms and multicentric lymphadenopathy. Thirteen cases (92.9%) of APHLF showed systemic symptoms, multicentric lymphadenopathy and abnormal laboratory findings. Histologic findings for cases resembling CD were rare in patients with RA and SjS. In AID patients, histologic findings for cases resembling CD or APHLF findings correlated with disease activity and multicentric lymphadenopathy. Six cases (54.5%) of ALPIB were in the active phase with systemic symptoms and multicentric lymphadenopathy. ALPIB tended to be unrelated to AID activity, especially in the majority of patients with no abnormal laboratory findings. Atypical LPDs associated with AID is a group of diseases that may be overdiagnosed and overtreated. The diagnosis of atypical LPDs associated with AID requires an understanding of the histological findings as well as a comprehensive assessment of the presence of systemic symptoms, the distribution of lymphadenopathy, and abnormal laboratory findings.

Presurgical avelumab plus axitinib in an immunosenescent octogenarian with renal cell carcinoma invading the vena cava.

Application of immune checkpoint inhibitors (ICIs) in elderly patients remains challenging due to the scarcity of safety and efficacy data. An 84 year-old female with a right renal cell carcinoma invading the vena cava received two cycles of avelumab plus axitinib. As the thrombus showed a marked reduction, right nephrectomy and vena cava thrombectomy were performed. Pathological examination revealed intra-tumor infiltration of CD8+ T cells suggesting the efficacy of immunotherapy. Although immune function deteriorates with age (immunosenescence), our findings suggest that older patients may not necessarily be excluded from ICI therapy.

Mixed adenoneuroendocrine carcinoma of the non-ampullary duodenum with mismatch repair deficiency: a rare case report.

A non-ampullary duodenal mixed adenoneuroendocrine carcinoma (MANEC), consisting of a conventional adenocarcinoma and a neuroendocrine carcinoma (NEC), is exceedingly rare. Moreover, mismatch repair (MMR) deficient tumors have recently attracted attention. The patient, a 75-year-old woman with epigastric pain and nausea, was found to have a type 2 tumor of the duodenum, which was diagnosed on biopsy as a poorly differentiated carcinoma. A pancreaticoduodenectomy specimen showed a well-defined 50 × 48 mm tumor in the duodenal bulb, which was morphologically composed of glandular, sheet-like, and pleomorphic components. The glandular component was a tubular adenocarcinoma, showing a MUC5AC-positive gastric type. The sheet-like component consisted of homogenous tumor cells, with chromogranin A and synaptophysin diffusely positive, and a Ki-67 index of 72.8%. The pleomorphic component was diverse and prominent atypical tumor cells proliferated, focally positive for chromogranin A, diffusely positive for synaptophysin, and the Ki-67 index was 67.1%. The sheet-like and pleomorphic components were considered NEC, showing aberrant expression of p53, retinoblastoma, and p16. Notably, all three components were deficient in MLH1 and PMS2. We diagnosed a non-ampullary duodenal MANEC with MMR deficiency. This tumor has a unique morphology and immunohistochemical profile, and is valuable for clarifying the tumorigenesis mechanism of a non-ampullary duodenal MANEC.

Cryptococcal Prostatitis Forming Caseous and Suppurative Granulomas Diagnosed by Needle Biopsy: A Case Report.

Cryptococcal granulomatous prostatitis is extremely rare, and there have been few reports of its diagnosis by prostate needle biopsy. The patient, an 81-year-old man, was receiving immunosuppressive treatment for rheumatoid arthritis. He had an oropharyngeal ulcer, and it was diagnosed alongside a methotrexate-related diffuse large B-cell lymphoma. A systemic imaging examination revealed a prostatic tumor-like mass clinically suspected to be prostatic cancer, and a needle biopsy was performed. The biopsy specimen showed various types of inflammatory cell infiltration, and suppurative granuloma and caseous granuloma were observed. Both granulomas showed multiple round and oval organisms that were revealed with Grocott methenamine silver staining. Acid-fast bacilli were not detected by Ziehl-Neelsen staining. We histologically diagnosed granulomatous prostatitis caused by Cryptococcus infection. Caseous granulomas often develop in the prostate after bacillus Calmette-Guerin immunotherapy for bladder cancer, although the possibility of cryptococcal granulomatous prostatitis should also be considered.

A case of clear cell renal cell carcinoma with vena cava thrombus responding to presurgical avelumab, and axitinib.

INTRODUCTION: We report a case of renal cell carcinoma with vena cava thrombus showing a marked reduction with presurgical avelumab plus axitinib, facilitating nephrectomy with thrombectomy. CASE PRESENTATION: A 50-year-old man was taken to emergent care unit due to spontaneous renal rupture and was diagnosed to have left-sided renal cell carcinoma with level IV tumor thrombus. After hemostasis was obtained via transcatheter arterial embolization, avelumab plus axitinib was introduced because upfront surgery was deemed unfeasible due to poor performance status and possible retroperitoneal tumor dissemination. After four treatment cycles, thrombus was reduced to level II, and nephrectomy with thrombectomy was performed. Histological analyses revealed massive CD8+ T cell infiltration in the thrombus, suggesting immunotherapy efficacy. He has remained recurrence-free without any additional treatment for eight months. CONCLUSION: For locally advanced renal cell carcinoma with vena cava thrombus, presurgical combination therapy with avelumab plus axitinib could be an option to facilitate curative surgery.

A case of estrogen-secreting adrenocortical carcinoma: Comprehensive immunohistochemical analysis of disorganized steroid genesis.

INTRODUCTION: Adrenocortical carcinoma rarely secretes estrogens, and little is known regarding the mechanism of intra-tumor estrogen production. We report an estrogen-secreting adrenocortical carcinoma in a postmenopausal woman, where comprehensive immunohistochemical analyses of the resected tumor revealed disorganized steroidogenesis. CASE PRESENTATION: A 68-year-old woman presented with postmenopausal vaginal bleeding and was found to have a left adrenal tumor. Serum estradiol and testosterone were elevated but they normalized after resection of the tumor, suggestive of adrenocortical carcinoma with disorganized steroidogenesis. Immunohistochemical analyses revealed that the tumor expressed aromatase which converts androgens into estrogens. Furthermore, the tumor lacked 17ßHSD2, which converts estradiol to estrone, suggesting that estradiol accumulated as the final product of the tumor's steroidogenic pathway. CONCLUSION: The capability of adrenocortical carcinoma to produce estrogen can be demonstrated by comprehensive immunohistochemical analyses of steroidogenic enzymes, such as those reported here.

Metastatic mucinous tubular and spindle cell carcinoma of the kidney responding to nivolumab plus ipilimumab.

INTRODUCTION: Mucinous tubular and spindle cell carcinoma is a rare subtype of renal cell carcinoma. Little is known regarding the efficacy of systemic therapy on its metastatic form because of its rarity. CASE PRESENTATION: We present the case of a patient with metastatic mucinous tubular and spindle cell carcinoma who achieved durable complete remission of multiple osseous metastases after undergoing cytoreductive nephrectomy followed by combination immunotherapy (ipilimumab plus nivolumab). Immunohistochemical analyses of the primary tumor revealed the presence of the tumor-infiltrating immune cells, including activated CD8+ T cells and PD-L1 expression, suggesting an immunologically hot tumor. CONCLUSION: Combination immunotherapy was a viable treatment option for this disease. Immunohistochemical analyses of the tumor-infiltrating immune cells predicted the efficacy of immune checkpoint inhibitors against rare renal cancers.

A case of 18 years disease-free survival after combined pancreatoduodenectomy and hemicolectomy for carcinosarcoma of the transverse colon.

BACKGROUND: Ascertaining the origin of large tumors located in the region of the pancreas head and adjacent mesocolon can pose a challenge preoperatively. En bloc pancreatoduodenectomy with hemicolectomy is often required towards curative tumor resection (R0) of malignant tumors in this region. CASE PRESENTATION: Herein we report a case of a 48-year-old man with two contiguous masses each 5 cm in size, located in the pancreatic head. The masses were detected incidentally by abdominal ultrasonography at an annual health check. Endoscopic biopsies revealed inflammation with no malignancy. Cross-sectional imaging showed the tumor direct invasion of the uncinate process of the pancreas, and the third portion of the duodenum. Based on imaging, a malignant submucosal tumor originating from mesenchymal cells in the mesentery of the transverse colon was made preoperatively. The mass required en bloc pancreatoduodenectomy, right hemicolectomy, and resection of the superior mesenteric vein. The final pathology was carcinosarcoma of the transverse colon. The patient survived 18 years after surgery without recurrence. CONCLUSIONS: Malignant tumors located in the region of the pancreas head should be considered for an en bloc curative tumor resection and adjuvant chemotherapy treatments offered that might be beneficial for carcinosarcoma.

Plasmacytoid urothelial carcinoma of renal pelvis with positive zinc finger E-box-binding homeobox 1: a case report.

BACKGROUND: The tumor transformation mechanism of a plasmacytoid urothelial carcinoma remains unexplained. We describe the case of a plasmacytoid urothelial carcinoma of the renal pelvis in which the expression of zinc finger E-box-binding homeobox 1 (ZEB1), a key nuclear transcription factor in an epithelial-mesenchymal transition, is involved in tumor transformation. CASE PRESENTATION: The patient had a left nephrectomy with the clinical diagnosis of left pelvic renal cancer. The resected specimen showed that the tumor surface comprised a noninvasive papillary urothelial carcinoma with the carcinoma in situ, and the invasive area comprised a plasmacytoid urothelial carcinoma characterized by the presence of single dyscohesive malignant cells that resembled plasma cells in a loose myxoid stroma. The noninvasive urothelial carcinoma was positive for cytokeratin and E-cadherin, and negative for vimentin and ZEB1. In contrast, the invasive plasmacytoid urothelial carcinoma was positive for cytokeratin and also vimentin and ZEB1, and negative for E-cadherin. Additionally, this component was immunoreactive for CD138 and CD38 that are immunohistochemical markers for plasma cells. CONCLUSION: We suggest that ZEB1 is involved in the plasmacytoid transformation by repressing the E-cadherin in a plasmacytoid urothelial carcinoma.