[A Case of Diffuse Large B-Cell Lymphoma Which Was Diagnosed during the Best Supportive Care of Recurrent Ascending Colon Cancer].

A 70s male, who had undergone single-incision laparoscopic ileocecal resection for ascending colon cancer with pathological diagnosis of T3N3M0, Stage Ⅲc(without adjuvant chemotherapy), had enhanced-computed tomography(CT)for 3-month follow-up and a hepatic low-density area, an newly emergent nodule behind inferior vena cava and distal ileal tumor were found. Three months later, enhanced CT showed that the distal ileal tumor got exponentially larger and the diagnosis of"malignant lymphoma"was suspected. The patient became sepsis, so we planned and safely performed partial resection of the tumor. The pathological diagnosis was diffuse large B-cell lymphoma. Postoperative course was smooth except for the Clostridium difficile colitis and he was discharged on postoperative day 19. Although the regrowth of the remnant tumor was observed soon after surgery, partial response was confirmed after introduction of systemic chemotherapy. When we cope with malignant lymphoma of small intestine, we need to keep it in mind that surgery is an option for the prevention of perforation and bacterial translocation.

[A Case of Fibromatosis-Like Tumor Which Was Difficult to Differentiate from Local Recurrence of Ascending Colon Cancer].

A 60s female, who had undergone single-incision laparoscopic ileocecal resection for ascending colon cancer with pathological diagnosis of T3N1bM0, Stage Ⅲb, followed by adjuvant therapy with 8 courses CAPOX 2 years ago, had enhanced- computed tomography(CT)for follow-up and a 15-mm nodule near anastomotic site was found. 18F-fluorodeoxyglucose (FDG)-positron emission tomography(PET)CT revealed abnormal accumulation of 18F-FDG only to the lesion and diagnosis of"anastomotic recurrence"was made. We planned and safely performed resection of the anastomotic site and the nodule. The pathological diagnosis was fibromatosis-like tumor without evidence of recurrence, and margin was negative. Postoperative course was smooth and she was discharged on postoperative day 9. When we diagnose local recurrence, we need to keep it in mind that fibromatosis is one of the differential diagnoses, although its incidence rate is low.

A Case of Insulinoma with Hyperprocalcitoninemia and Hypercalcitoninemia Showing Coexpression of Insulin and Calcitonin in Its Tumor Cells.

Neuroendocrine neoplasms can produce multiple hormones that are released into the bloodstream, causing symptoms that vary depending on the type and quantity of hormones involved. We herein report a 63-year-old asymptomatic patient with pancreatic insulinoma who showed marked elevations in circulating calcitonin and procalcitonin levels that returned to normal following surgery. Immunohistochemical analyses confirmed the co-staining of calcitonin and insulin immunoreactivity in the tumor cells, suggesting a calcitonin-producing insulinoma. This insulinoma released calcitonin and a considerable amount of its precursor peptide, procalcitonin, resulting in both hyperprocalcitoninemia and hypercalcitoninemia.

KDM2B-Rearranged Soft Tissue Sarcomas Expand the Concept of BCOR-Associated Sarcoma.

Sarcomas with BCOR genetic alterations (BCOR-associated sarcomas) represent a recently recognized family of soft tissue and bone tumors characterized by BCOR fusion, BCOR internal tandem duplication, or YWHAE::NUTM2B fusion. Histologically, the tumors demonstrate oval to spindle cell proliferation in a variably vascular stroma and overexpression of BCOR and SATB2. Herein, we describe 3 soft tissue sarcomas with KDM2B fusions that phenotypically and epigenetically match BCOR-associated sarcomas. The cases included 1 infant, 1 adolescent, and 1 older patient. All tumors showed histologic findings indistinguishable from those of BCOR-associated sarcomas and were originally diagnosed as such based on the phenotype. However, none of the tumors had BCOR or YWHAE genetic alterations. Instead, targeted RNA sequencing identified in-frame KDM2B::NUTM2B, KDM2B::CREBBP, and KDM2B::DUX4 fusions. KDM2B fusions were validated using reverse-transcription PCR, Sanger sequencing, and in situ hybridization assays. Genome-wide DNA methylation analysis matched all 3 tumors with BCOR-associated sarcomas using the Deutsches Krebsforschungszentrum (DKFZ) classifier and t-distributed stochastic neighbor embedding analysis. One localized tumor showed a flat genome-wide copy number profile, and the patient remained disease-free after treatment. The other tumors showed multiple copy number alterations, including MDM2/CDK4 amplification and/or CDKN2A/B loss, and both tumors metastasized, leading to the patient's death in one of the cases. When tested using KDM2B immunohistochemistry, all 3 KDM2B-rearranged sarcomas showed diffuse strong staining, and all 13 sarcomas with BCOR genetic alterations also demonstrated diffuse, strong, or weak staining. By contrast, among 72 mimicking tumors, only a subset of synovial sarcomas showed focal or diffuse weak KDM2B expression. In conclusion, our study suggests that KDM2B-rearranged soft tissue sarcomas belong to the BCOR-associated sarcoma family and expand its molecular spectrum. This may be related to the known molecular relationship between KDM2B and BCOR in the polycomb repressive complex 1.1. Immunohistochemical analysis of KDM2B is a potentially valuable diagnostic tool for BCOR-associated sarcomas, including those with KDM2B rearrangement.

Immunoglobulin G4-Hepatopathy with Acute Hepatitis-Like Onset and Marked Centrilobular Necrosis: Clinicopathologically Unique Pattern of Hepatic Injury Related to Immunoglobulin G4-Related Disease.

A 69-year-old man presented with jaundice and appetite loss. Blood analyses showed elevated aminotransferase levels, hyperbilirubinemia, positivity for antinuclear antibody, elevated immunoglobulin (Ig) G4 levels, and negativity for hepatitis virus markers. Additionally, computed tomography revealed a focal enlargement of the pancreatic body and enhancement of the peripheral bile ducts. Liver biopsy showed interface hepatitis, supporting a clinical diagnosis of autoimmune hepatitis (AIH). Immunohistochemistry revealed that IgG4-bearing plasma cells accounted for more than 60% of the IgG-bearing plasma cells in the portal area. Then, we started oral prednisolone therapy. After tapering, serum transaminase levels became elevated again, and we had to adjust the dose. Azathioprine maintenance therapy was necessary to prevent relapse. We herein report a case of IgG4-hepatopathy with a clinical course similar to that of AIH with acute onset.

Mechanical Thrombectomy for Acute Middle Cerebral Artery Occlusion Caused by Tumor Embolism: A Case Report.

Objective: We report a case of acute middle cerebral artery (MCA) occlusion caused by tumor embolism. Case Presentation: A 64-year-old man with lung cancer presented with sudden onset left-sided hemiparesis and sensory disturbance. Diffusion-weighted imaging (DWI) revealed hyper-intense foci in the right MCA territory and magnetic resonance angiography (MRA) demonstrated right MCA M2 segment occlusion. Mechanical thrombectomy (MT) was performed with Thrombolysis in Cerebral Infarction 2B recanalization. On histopathology, thrombus composed of fibrin and squamous cell carcinoma was observed. We diagnosed him with tumor embolism from lung cancer that invaded the pulmonary vein and the left atrium. Conclusion: Tumor cells may be confirmed by pathological examination regardless of the morphology of the embolus. Pathological examination of the cerebral embolus is useful for the accurate diagnosis of ischemic stroke subtypes.

Lymphangioleiomyoma of the Uterus and Pelvic Lymph Nodes: A Report of 3 Cases, Including the Potentially Earliest Manifestation of Extrapulmonary Lymphangioleiomyomatosis.

We present 3 cases of extrapulmonary lymphangioleiomyomatosis (LAM) identified incidentally in the uterine corpus and pelvic nodes resected for other reasons. One patient, a 47-yr-old female with corpus cancer, underwent a total hysterectomy and nodal dissection; the other 2 patients, aged 44 and 49 yr, underwent simple hysterectomy for corpus leiomyomas. None of the patients had evidence of tuberous sclerosis complex or any significant lesions in other organs. An area of spindle cell proliferation, intimately associated with dilated and tortuous lymphatic vessels, was found in the myometrium of all 3 patients, and nodal involvement with spindle cell proliferation was observed in the patient with corpus cancer. The spindle cells had faintly eosinophilic cytoplasm and a bland appearance. They were immunoreactive for α-SMA, gp100 (HMB45), and Melan-A. Tumor cell clusters lined with a single layer of lymphatic endothelium were floating in the lymphatic vessel lumen. These lesions were diagnosed as lymphangioleiomyoma in the uterine corpus and associated lymph nodes. Two of the cases seemed to be the earliest manifestations of extrapulmonary LAM, and the other case represents early-phase metastasis of LAM from the uterus. The present cases support the speculation that the uterus is the primary source of LAM cells.

[A Rare Case of Adenosquamous Carcinoma in the Liver with Hepatolithiasis].

This report describes a case of primary adenosquamous carcinoma of the liver with hepatolithiasis. A 70's man was followed up at a clinic for hepatolithiasis, gallbladder stone, and dilatation of the intrahepatic duct. He visited our hospital for computed tomography(CT)examination. CT showed a 30mm diameter low-density mass in the S2 liver and dilatation of the intrahe- patic duct filled with hepatolithiasis. Blood examination showed elevated levels of tumor markers(CEA 8.0 ng/mL, CA19-9 19,196 U/mL). We diagnosed the tumor as cholangiocellular carcinoma(cT2N1M0, cStage ⅣA)with hepatolithiasis and performed left hepatectomy and lymphadenectomy. In the specimen, a 39×22mm diameter solid tumor was detected and the intrahepatic duct was filled with haptolithiasis. Pathologically, a mixture of adenocarcinoma and squamous cell carcinoma was observed adjacent to the bile duct. Accordingly, a diagnosis of adenosquamous carcinoma was made(pT3N0M0, pStage Ⅲ). Multiple liver metastases were detected 8 months after the operation, and chemotherapy was started. He remains alive 11 months after the operation. We experienced a rare case of adenosquamous carcinoma in the liver with hepatolithiasis.

Follicular cholangitis mimicking cholangiocarcinoma treated with right hepatectomy: A case report and review of published works.

Follicular cholangitis is a new, rare disease that causes severe biliary stricture. We herein describe the findings from a resected case of follicular cholangitis, suggesting a distinct disease entity that causes benign biliary stricture. A 60-year-old man who was referred to our hospital due to elevated γ-glutamyl transpeptidase levels and dilatation of the B8 bile duct. Although bile juice cytology and bile duct brushing cytology showed no malignancy, the dilatation was progressive. Therefore, right hepatectomy combined with caudate lobectomy was carried out on suspicion of intrahepatic cholangiocarcinoma. The wall of the resected bile duct was markedly thickened due to severe fibrosis under the mucosal layer. Histology of the mucosal epithelium indicated no malignancy. Infiltration of plasma cells characterized by remarkable formation of lymphoid follicles with germinal centers was observed around the bile ducts. The patient was diagnosed with follicular cholangitis based on histological findings. We thus observed a rare case of follicular cholangitis. This case and review of published reports suggest that, despite its rarity, follicular cholangitis should be considered at the differential diagnosis of biliary stricture. This case report could contribute to a better understanding of how to address this disease.

[A Resected Case of Reactive Lymphoid Hyperplasia of the Liver Preoperatively Diagnosed as Hepatocellular Carcinoma].

A60s woman was followed-up regularly for primary biliary cholangitis and a solitary enlarging hepatic mass in the S6 segment of her liver was discovered by ultrasonography. We diagnosed the mass as hepatocellular carcinoma by contrast ultrasonography, contrast computed tomography, and ethoxbenzyl magnetic resonance imaging and laparoscopic partial hepatectomy of S6 segment was performed. The resected specimen was histopathologicaly diagnosed as liver-reactive lymphoid hyperplasia(RLH). The patient is alive without recurrence 17 months after the surgery. Although liver RLH is a rare disease, it should be considered in the differential diagnosis of small liver tumors.

[Long-Term Survival after Surgical Treatment of Neuroendocrine Carcinoma of the Ampulla of Vater-A Case Report].

A 60s man underwent upper gastrointestinal endoscopy at a regular medical check-up without symptoms, which showed an ulcerative region in the duodenal ampulla, measuring 3 cm in diameter. He was diagnosed with poorly differentiated adenocarcinoma on biopsy and referred to our hospital. Abdominal contrast-enhanced CT scan revealed an enhanced-ulcerative tumor, measuring 3 cm, at the duodenal ampulla. After the preoperative diagnosis of adenocarcinoma of the duodenal ampulla, subtotal stomach-preserving pancreatoduodenectomy with regional lymph node dissection was performed. The final diagnosis was neuroendocrine carcinoma(NEC)of the duodenal ampulla. He has been alive for 9 years with no recurrences. NEC of the duodenal ampulla is rare, and its prognosis is poor. We report a case of long-term survival after resection of NEC of the duodenal ampulla.

Alveolar Echinococcosis Mimicking a Hepatic Neoplasm with Lymph Node Metastasis: A Case Report.

A 37-year-old man had an asymptomatic 17-mm mass in the liver by health check with ultrasonography. Five years later, he was referred to our hospital because the mass was slightly enlarged with a peripancreatic lymph node. We performed endoscopic ultrasonography fine-needle aspiration (EUS-FNA) to evaluate a lymph node, but it showed amorphous eosinophilic material and eosinophilic infiltrate in necrotic tissue of toothpaste-like white specimen. However, we diagnosed as potentially malignant liver mass with lymph node metastasis because of 2-deoxy-2-(fluorine-18) fluorodeoxyglucose uptake. We then performed hepatectomy and enucleation of the pancreas. DNA polymerase chain reaction analysis revealed Echinococcus multilocularis infection. Retrospectively, we could find a part of Echinococcus in the specimens of EUS-FNA.

[A Case of Unresectable Advanced Gastric Neuroendocrine Cell Carcinoma Treated with Nivolumab].

A 69-year-old man was administered S-1/oxaliplatin/trastuzumab as induction chemotherapy for advanced gastric cancer (cT4b[liver, pancreas], N2M1(H1P0CYX), cStage IV). After 4 courses, because contrast-enhanced computed tomography showed remarkable reduction of the tumor, distal gastrectomy, partial hepatectomy, and radiofrequency ablation for the liver metastasis were performed. The patient was histopathologically diagnosed with gastric neuroendocrine carcinoma(NEC). S- 1/oxaliplatin/trastuzumab was continued after surgery; however, recurrence in the remnant liver was observed after 4 cours- es. For recurrence, cisplatin/irinotecan as first-line and paclitaxel/ramucirumab as second-line treatment were administered, but progression of liver metastasis and ascites due to peritoneal dissemination were observed. As third-line treatment, nivolumab was initiated. Ascites decreased after 3 courses, but after 2 more courses, progression of ascites, liver recurrence, and multiple metastasis in the lumbar vertebra were observed.

A case of intraductal papillary-mucinous neoplasm of the pancreas penetrating into the stomach and spleen successfully treated by total pancreatectomy.

BACKGROUND: Intraductal papillary-mucinous neoplasms (IPMNs) are potentially malignant intraductal epithelial neoplasms that sometimes penetrate into other organs. To the best of our knowledge, no report has yet described a case with penetration into the spleen. We recently encountered a case of IPMN with penetration of the stomach and spleen that was successfully treated by total pancreatectomy. CASE PRESENTATION: A 70-year-old female visited our hospital with a complaint of fever and abdominal pain. Contrast-enhanced computed tomography (CT) revealed dilatation of the main pancreatic duct in the entire pancreas and penetration into the stomach and spleen. Upper gastrointestinal endoscopy revealed mucin extruding from four openings of the fistula in the stomach. No malignancy was detected based on cytology of the mucin. Inflammation markers and tumor markers (CEA, CA19-9) were elevated in the blood. The pre-operative diagnosis was IPMN of main pancreatic duct type penetrating into the stomach and spleen. A total pancreatectomy and splenectomy were performed, combined with distal gastrectomy including resection of the fistulas between the pancreas and stomach. No postoperative complications were noted. Histopathological examination of the resected specimen revealed atrophy of the pancreatic parenchyma, and the main duct of the pancreas was filled with mucin. Mucin-producing malignant tumor cells were detected in the epithelium of the main pancreatic duct with no signs of invasion. No malignancy was found at the fistulas between the pancreas and stomach or spleen. The patient was finally diagnosed with non-invasive intraductal papillary-mucinous carcinoma (IPMC) of main pancreatic duct type. Mechanical penetration was suspected as a mechanism of the penetration. The patient remained disease-free without evidence of recurrence more than 15 months after the operation. CONCLUSION: Though IPMNs sometimes penetrate into other adjacent organs, penetration into two organs, including the spleen, is rare. The rare case of IPMC penetrating into the stomach and spleen presented here was treated successfully by total pancreatectomy.

Extracranial meningiomas concurrently found in the lung and vertebral bone: a case report.

BACKGROUND: Primary pulmonary meningiomas are very rare, and primary intraosseous meningiomas outside the head and neck region have not yet been reported. We report an extremely unusual case of concurrent meningiomas arising in the pulmonary parenchyma and vertebral bone. CASE PRESENTATION: A 40-year-old Asian woman presented with a destructive lesion of the lumbar vertebral bone and a small nodule in the right lung. Five years later, both lesions slightly increased in size. To evaluate both the pulmonary and vertebral lesions, video-assisted thoracic surgery and curettage of the lytic lesion were performed. Both lesions showed similar histopathological findings corresponding to an intracranial meningioma of World Health Organization grade 1. The patient made good postoperative progress and remained free from disease at 41 months after the operation. CONCLUSIONS: Our patient presented with almost synchronous pulmonary and lumbar vertebral intraosseous meningiomas. Regarding the relationship between the two lesions, there are two possibilities: Independent tumors occurred coincidentally or the primary pulmonary meningioma metastasized to the vertebral bone despite its bland morphology. It is important to keep in mind the exceptionally rare condition of extracranial meningioma.

Mesonephric adenocarcinoma of the uterine corpus with intracystic growth completely confined to the myometrium: a case report and literature review.

BACKGROUND: Mesonephric adenocarcinoma (MA) is a rare tumor believed to arise from mesonephric remnants occurring mostly in the uterine cervix and, to a lesser extent, the corpus. Since the first case report of MA in the corpus in 1995, only 16 cases have been reported in the English literature. A recent report suggested that MA originates in Müllerian tissue and exhibits the mesonephric differentiation phenotype. CASE PRESENTATION: An asymptomatic 61-year-old woman was referred to our hospital because of elevated levels of tumor markers. Imaging revealed an intramural lesion of the uterine corpus exhibiting fluorodeoxyglucose uptake. A total hysterectomy and bilateral salpingo-oophorectomy were performed. The tumor was completely confined to the corpus wall and was composed of an intracystic bulky component and an invasive component in the myometrial layer. The tumor exhibited a variety of growth patterns, including a characteristic tubular pattern with dense eosinophilic secretion reminiscent of the thyroid, as well as a variety of morphologies, such as acinar, papillary, and ductal structures. The structures were immunoreactive for CK7, vimentin, CD10, calretinin, PAX8, and GATA3 and almost completely negative for ER/PgR. CA125 and CA19-9 antigen expression was also detected. CONCLUSION: A case of MA with a unique growth pattern of an intracystic mass within the corpus wall is presented. The histogenesis and differential diagnoses are discussed. The histogenesis of MA is not yet clear. We hypothesize two different pathways involved: 1) direct development from the mesonephric remnants and/or 2) mesonephric transformation of Müllerian adenocarcinoma.

Pancreatic intraductal tubulopapillary neoplasm with associated invasive cancer successfully treated by total pancreatectomy: A case report.

A 74-year-old male was admitted to Departments of Surgery, Toyonaka Municipal Hospital (Osaka, Japan) for treatment of a pancreatic tumor. Contrast enhanced computed tomography (CT) revealed a mass with small cystic lesions in the pancreatic head and body. Fluorodeoxyglucose-positron emission tomography/CT revealed an abnormal uptake of fluorodeoxyglucose, corresponding to the mass lesions. Upper gastrointestinal endoscopy revealed rough mucosa near the opening of the accessory pancreatic duct, and the mucosa biopsy exhibited adenocarcinoma with no mucin observed. The preoperative diagnosis was pancreatic intraductal tubulopapillary neoplasm (ITPN) with cancerous lesions, and a total pancreatectomy with splenectomy was performed. The resected tissue specimen revealed a solid tumor occupying the entire pancreas with intraductal growth into the main pancreatic duct. Histological examination revealed high-grade dysplastic cells in a tubulopapillary growth pattern without overt mucin production beyond the pancreatic duct. Immunohistochemical staining analysis of the tumor was positive for cytokeratin (CK)7, CK19 and mucin (MUC)1, and negative for MUC2, MUC5AC, MUC6 and caudal type homeobox 2. The tumor was finally diagnosed as pancreatic ITPN with associated invasive cancer. The patient remains well without evident recurrence nine months post-surgery. ITPN is a rare type of epithelial neoplasm of the pancreas, and is characterized by intraductal tubulo-papillary growth, ductal differentiation, limited intracellular mucin production, and cellular dysplasia. The present case report may contribute to improved understanding of how to effectively treat patients with ITPN.

Esophageal carcinoma originating in the surface epithelium with immunohistochemically proven esophageal gland duct differentiation: A case report.

A case of esophageal carcinoma exclusively composed of adenocarcinoma simulating an esophageal gland duct in a 61-year-old man is presented. The tumor arose as a slightly elevated lesion in the middle intrathoracic esophagus. It was almost completely overlaid with non-neoplastic stratified squamous epithelial cells. Beneath the overlying surface epithelium, an adenocarcinoma that was bilayered in structure diffusely invaded both the mucosal and submucosal layers. Although the tumor consisted exclusively of adenocarcinomatous cells, a keratinizing squamous cell carcinoma component was focally observed. The invasive carcinoma was focally continuous with the small area of the surface squamous epithelial layer, which was confirmed to be neoplastic by immunohistochemistry. Morphological and immunohistochemical examinations suggested that the adenocarcinomatous component arose from the esophageal surface epithelium and clearly differentiated into an esophageal gland duct. It is important to consider the possibility of this type of adenocarcinoma when diagnosing a ductal or glandular lesion of the esophagus in small biopsy specimens.

A giant periosteal chondroma of the distal femur successfully reconstructed with synthetic bone grafts and a bioresorbable plate: a case report.

Periosteal chondromas are rare benign cartilaginous tumors that arise adjacent to the cortex beneath the periosteum. These lesions are usually slow-growing and rarely exceed 3 cm in the greatest dimension. Here, we describe a 17-year-old boy who had a giant periosteal chondroma of the right distal femur, which was treated with intralesional resection and intensive curettage. In addition, we report a novel application of a bioresorbable plate in the management of the large bone defect after resection of a benign bone tumor.