[A Case Report of Rectal MiNEN Surgical Resection].

A 56-year-old man with diarrhea and bloody stools was found to have a type 2 tumor in the rectosigmoid region. A biopsy revealed well-differentiated tubular adenocarcinoma(tub1), and laparoscopic high anterior resection was performed. The final diagnosis was MiNEN(NEC:adenocarcinoma=6:4), RS, type 2, Ly0, V0, pT3(SS), pN0, M0, and pStage Ⅱa. He has been followed up without postoperative adjuvant chemotherapy and has been alive without recurrence for 1 year and 4 months after surgery. MiNEN is a rare disease, and most of them are mixed with NEC components or high- grade NET G3 and have a poor prognosis. We present an important case of rectal MiNEN.

[Surgical Resection of Gastric MANEC-Two Case Reports].

Case 1 was a 78-year-old woman with a tumor in the stomach on preoperative CT of an inguinal hernia. The patient was diagnosed with advanced gastric cancer at posterior wall of fornix and underwent total gastrectomy and splenectomy. Postoperative pathological diagnosis was gastric mixed adenoneuroendocrine carcinoma(MANEC), T1b2, N1, M0, StageⅠB. She has been alive without recurrence for 3 years without postoperative adjuvant chemotherapy. Case 2 was a 78-year-old man who was admitted to the hospital with acute pancreatitis and had a thickened wall of the lesser curvature of the gastric antrum on CT. He was diagnosed with advanced gastric cancer and underwent distal gastrectomy and D2 dissection. Postoperative pathological diagnosis was gastric MANEC, T1b2, N1, M0, Stage ⅠB. Oral administration of S-1 was started as postoperative adjuvant chemotherapy, but he was very tired and ended in 1 course at his request. Computed tomography 6 months after the operation revealed multiple liver metastases, and he was transferred to best supportive care at his request. He died 1 year after surgery. We experienced 2 valuable cases of gastric MANEC.

Dextran deposition in reticuloendothelial organs in hemodialysis patients: A forgotten adverse effect of low-molecular weight dextran.

We present two autopsy cases of refractory ascites/hydrothorax associated with systemic deposition of low-molecular weight dextran that had been used in hemodialysis over an extended period. The most striking autopsy findings in both cases were dense accumulation of dextran-laden macrophages in sinusoids of reticuloendothelial organs. These accumulated macrophages seemed to have disturbed blood and lymph flow. Based on these findings, we concluded that the persistent ascites and hydrothorax were attributable to systemic deposition of dextran, especially the accumulation of dextran-laden macrophages in the reticuloendothelial organs. We suggest that dextran solution should immediately be designated as contraindicated for hemodialysis patients.

Malignant Lymphoma of the Ovary: A Diagnostic Pitfall of Intraoperative Consultation.

Primary ovarian lymphomas are rare, but can potentially evoke diagnostic problems. We present a case of ovarian lymphoma, in which an ambiguous intraoperative pathologic report led to overtreatment (unnecessary surgery). A 73-yr-old woman with fatigue and low-grade fever was diagnosed as having a left ovarian tumor by imaging modalities. Exploratory laparotomy was carried out to confirm the diagnosis. The frozen tissue sections of the ovarian tumor showed condensed proliferation of atypical round cells accompanied with a few small lymphocytes. The pathologists could not determine whether this tumor was a lymphoma or another malignancy (eg, dysgerminoma). Hence, they reported it to gynecologists who operated as simply a malignant tumor in order to evade misdiagnosis. On the basis of the inconclusive pathologic report, the gynecologists decided to change the planned laparotomy to total hysterectomy with bilateral salpingo-oophorectomy and pelvic lymph node dissection. A postoperative paraffin section-based pathologic diagnosis was diffuse large B-cell lymphoma of the ovary, which basically does not require surgical treatments. Subsequently, chemotherapy for B-cell lymphoma was initiated, and no lymphoma recurrence has been reported to date. A more robust preoperative discussion between the gynecologists and the pathologists might have avoided the overtreatment.

Classical Hodgkin lymphoma type post-transplant lymphoproliferative disorder in a kidney transplant recipient: a diagnostic pitfall.

We report a case of classical Hodgkin lymphoma type post-transplant lymphoproliferative disorder (HL-PTLD) after kidney transplantation to highlight the difficulty of distinguishing this disorder from Hodgkin lymphoma-like PTLD (HL-like PTLD). Through this case report and literature review, we seek to clarify definitive pathologic features to differentiate these two conditions. A 38-year-old male kidney transplant recipient who had been receiving immunosuppressants was admitted to our hospital with unidentified high fever. Computed tomography images and blood tests indicated a lymphoproliferative disorder. Abdominal lymph node biopsy was performed, and microscopic examination revealed the presence of many large atypical cells in a background of dense T cell accumulation. The large, atypical cells were positive for Epstein-Barr Virus (EBV)-encoded small RNAs (EBER) in situ hybridization, EBV-LMP1, CD30 and PAX5, but negative for CD15, CD20 and CD45. Except for CD15-negativity, this immunohistochemical pattern was consistent with that of classical Hodgkin lymphoma. By close examination of the above immunoreactivities and the patient's subsequent chemosensitive clinical course, we finally made a diagnosis of HL-PTLD.

[A Case Report of Resectable Leiomyosarcoma of the Duodenum Origin Invading to the Inferior Vena Cava].

A 40's woman had a complaint of abdominal and back pain. Enhanced CT visualized a large retroperitoneal tumor and huge multiple myomas of the uterus. The tumor was 10cm in diameter and located in the anterior of the inferior vena cava, and progressed from the posterior of the duodenum to the abdominal aortic bifurcation. Diffusion-weighted MR image showed the tumor with high signal intensity. Upper gastrointestinal endoscopy revealed a type 2 tumor at the anal side of the Vater. The patient was performed curativly abdominal total hysterectomy and pancreaticoduodenectomy with inferior vena cava resection. Immunohistochemical examination showed that the tumor cells were negative for CD34 and c-kit, and positive for desmin and a-SMA. The tumor was histopathologically diagnosed as leiomyosarcoma originating from the duodenum.

[A Case of Double Lung and Gastric Cancer Involving Para-Aortic Lymph Node Metastasis with Difficult Preoperative Diagnosis].

A man in his 60s was admitted to our hospital with anemia. An endoscopic examination revealed advanced gastric cancer. CT revealed peri-gastric and para-aortic lymphadenopathy, and a nodular shadow(20mm)in the lower lobe of the right lung. PET-CT revealed abnormal uptake in the para-aortic lymph node and stomach wall and the nodular shadow in the right lung. A bronchoscopy revealed pulmonary adenocarcinoma. From the above, he was diagnosed with gastric cancer(cT4a, cN2, cM1, cStage IV )and lung cancer(cT2a, cN0, cM0, cStage I B). Because of gastric bleeding, we decided to operate on the gastric cancer before the lung cancer. First, total gastrectomy, splenectomy, and cholecystectomy were performed and then dissection of lymph node No. 16was performed. Histopathological examination indicated that lymph node No. 16was common to lung cancer, so the final diagnosis was gastric cancer(pT4a, pN0, cM0, fStage II A)and lung cancer(cT2a, cN0, pM1, fStage IV ). In this case, lymphadenectomy of No. 16in the first and pathological diagnosis during surgery could help us avoid splenectomy and cholecystectomy, and could reduce invasion.

[A Case of Mucinous Adenocarcinoma of the Descending Colon with Extramural Progression].

A 63-year-old man was admitted to our hospital with a cough. Based on imaging studies, the patient was diagnosed with locally advanced descending colon cancer with extensive infiltration into peripheral organs by extramural progression. There was no distant metastasis. Therefore, he received left hemicolectomy with splenectomy, partial gastrectomy, and partial diaphragm resection. Histopathological examination showed a mucinous adenocarcinoma, pT4b, pN1, cM0, fStage III A. We report a rare case of extramural progression colon cancer with invasion of various organs and review 12 previous case reports.

[A Case of Synchronous Multi-Centric Invasive Ductal Carcinomas of the Pancreas].

A 69-year-old man with left upper abdominal pain visited our hospital. Abdominal CT revealed a hypovascular mass(66× 57mm)in the pancreatic tail. MRI revealed hemorrhage and cysts in the caudal lesion of the tumor. The tumor involved the posterior wallof the stomach. Based on a diagnosis of pancreatic tailcancer with gastric posterior wallinvasion, distalpancreatectomy and partialgastrectomy were performed. Histopathologicalexamination indicated 2 adjacent tumors through a capsule in the pancreas tail. These tumors were diagnosed as synchronous invasive ductal carcinomas of the pancreas, which were anaplastic and papillary carcinomas. We report a rare case of synchronous multi-centric invasive ductalcarcinomas of the pancreas.

[A Case of Mixed Adenoneuroendocrine Carcinoma(MANEC)of the Duodenum].

A 79-year-old man was admitted to our hospital because of a positive fecal occult blood test. Gastrointestinal endoscopy revealed a tumor in the duodenal bulb. Histologically, biopsy specimens indicated adenocarcinoma. The patient underwent distal gastrectomy. Histopathological examination showed a neuroendocrine carcinoma with an adenocarcinoma. According to the 2010WHO Classification of Tumours of the Digestive System, this case was diagnosed as mixed adenoneuroendocrine carcinoma(MANEC). There was no metastatic lesion in the resected lymph node. The patient has been followed up for 2 years without evidence of recurrence. We report a rare case of MANEC in the duodenum.

Cytomegalovirus-Associated Gastroduodenal Ulcers in a Patient With Functional Hypercortisolism: A Case Report.

Cytomegalovirus (CMV)-associated gastroduodenal ulcers (GDU) are a rare digestive disease, which principally affect immunocompromised patients. We recently experienced CMV-associated GDU occurring in a seemingly immunocompetent patient. The rarity of such a condition was inimical to a correct clinical diagnosis.A 77-year-old woman with Alzheimer's disease was admitted to our hospital because of vomiting and anorexia. Her general condition was extremely poor due to severe dehydration. Any invasive procedures including gastroduodenal endoscopy could not be performed. Laboratory test results showed electrolyte imbalance, hyperglycemia, and hypercortisolemia. The plasma adrenocorticotropic hormone level was rather low. On her 11th day in hospital, she suddenly fell into shock status. Despite intensive care, the patient could not be rescued. An autopsy was performed and revealed that she had suffered from CMV-associated GDU and died of candidemia that invaded through the ulcer. Her adrenal glands showed neither neoplasm nor hyperplasia, suggesting that her hypercortisolism was a purely functional disorder. We concluded that the severe opportunistic infections were developed in association with functional hypercortisolism.This case suggests that functional hypercortisolism, even though transient, can cause a patient to be immunocompromised.

Significance of liver biopsy for the evaluation of methotrexate-induced liver damage in patients with rheumatoid arthritis.

It is well recognized that long-term administration of methotrexate (MTX) in patients with rheumatoid arthritis (RA) can induce liver fibrosis via a steatohepatitis-like inflammatory process. Several non-invasive tests have been investigated as alternatives to liver biopsy, which is, however, still recognized as a final diagnostic modality to detect the MTX-induced liver damage. To clarify whether there is a significant discrepancy between clinical estimations and pathologic findings of this hepatic condition, we performed a following comparative study. Four RA patients (4 women, age 67-80 yr) with MTX-induced liver damage were reviewed. The severity of hepatic damage estimated clinically was compared with histopathologic findings. Consequently, the liver biopsies showed the relatively earlier stages of and milder degrees of hepatic damages than the clinical estimations. The histopathologic findings were more reliable and useful than any other clinical examinations, to plan and modify the treatment strategies, especially in cases of liver damages with multiple etiologies besides MTX. These findings suggest that liver biopsy is an unavoidable examination to assess precisely MTX-induced liver damage. Non-invasive tests may be useful to monitor the hepatic condition of RA patients receiving MTX but do not constitute an acceptable alternative to liver biopsy.

[A case of multiple skin metastases from cancer of the descending colon responding to FOLFIRI/cetuximab therapy].

A 64-year-old woman presented to our hospital with subcutaneous tumors in the right thoracic region. After undergoing a thorough medical evaluation, she was diagnosed with multiple skin metastases arising from cancer of the descending colon. Surgical resection of the primary lesion was performed and FOLFIRI (5-fluorouracil, levofolinate calcium, irinotecan) and cetuximab chemotherapy for the metastases was initiated. The patient subsequently entered remission and did not experience any major side effects. This case report details an effective therapy for colon cancer with multiple skin metastases and presents a discussion of the expression profiles of epidermal growth factor receptor in both the primary and metastatic lesions.

Adenocarcinoma arising from vaginal stump: unusual vaginal carcinogenesis 7 years after hysterectomy due to cervical intraepithelial neoplasia.

Primary vaginal adenocarcinomas are one of the rarest malignant neoplasms, which develop in the female genital tract. Because of the extremely low incidence, their clinical and pathologic characteristics are still obscure. Recently, we experienced a case of vaginal adenocarcinoma that appeared 7 yr after hysterectomy because of cervical intraepithelial neoplasia. The patient, a 65-yr-old obese woman, was diagnosed as having adenocarcinoma in the vaginal stump and was treated by simple tumor excision and radiation. Immunohistochemical and molecular biologic examinations indicated a potential association with human papilloma virus infection in the development of the vaginal adenocarcinoma. There has been no evidence of recurrence for 3 yr after the operation.

The significance of platelet consumption in the development of thrombocytopenia in patients with cirrhosis.

INTRODUCTION: Thrombocytopenia in cirrhosis is mainly explained by accelerated platelet destruction/sequestration because of hypersplenism or by decreased thrombopoietin levels. Excessive platelet consumption because of cirrhosis-related hypercoagulability has also been assumed to be an etiopathologic factor in thrombocytopenia. To clarify whether excessive platelet consumption (eg, venous thrombosis and disseminated intravascular coagulation) contributes to thrombocytopenia in cirrhosis, the following autopsy-based study was performed. METHODS: Ninety-nine autopsies of chronic liver disease (80 cirrhosis and 19 noncirrhosis) were examined retrospectively. Platelet count, weight of spleen and thrombotic complications were checked in autopsy protocols. Megakaryocytes in bone marrow were counted under high-power microscopic view. Univariate and multivariate analyses were performed to evaluate significances of these factors in thrombocytopenia. RESULTS: The platelet count was significantly lower in the cirrhosis cases (88 ± 51 × 10/L) than in the noncirrhosis cases (150 ± 120 × 10/L). The megakaryocyte count was also lower in the cirrhosis cases (1.5 ± 0.6 per high-power field) than in the noncirrhosis cases (1.9 ± 0.5 per high-power field). The weight of the spleen was greater in the cirrhosis cases (264 ± 179 g) than in the noncirrhosis cases (142 ± 82 g). Thrombotic complications had been recorded in 29 cases, whose platelet count (70 ± 41 × 10/L) was lower than that of those without these complications (113 ± 80 × 10/L). Multivariate analysis revealed that these 3 factors (megakaryocyte count, weight of spleen, and thrombotic complications) were independently correlated with the platelet count. CONCLUSIONS: These results suggest that the imbalance of platelet production-destruction/sequestration-consumption contributes to thrombocytopenia in cirrhosis. Excessive platelet consumption cannot be ignored to explain this complex condition, especially in patients with major thrombotic events.

[Long-term survival of a patient with extrahepatic cholangiocarcinoma treated with adjuvant radiotherapy for positive ductal margins after surgical resection].

A 57-year-old man with abdominal discomfort visited our hospital. Abdominal computed tomography (CT) revealed no tumorous lesions. Endoscopic retrograde cholangiography (ERC) revealed a tumorous lesion in the middle bile duct. Peroral cholangioscopy-assisted biopsy was performed, and the lesion was diagnosed as a papillary adenocarcinoma. Extra bile duct resection and regional lymph node dissection were performed; however, residual carcinoma in situ was detected at the ductal margins by intraoperative frozen section analysis. Therefore, external radiation therapy at a dose 50.4 Gy/28 Fr was administered after the operation. No complication due to radiotherapy occurred. The patient remains alive and recurrence -free for more than 7 years after the operation. We report the long-term disease-free survival of a patient with extrahepatic cholangiocarcinoma who was treated with external radiation therapy for positive ductal margins after surgical resection.

[Spontaneous cerebrospinal fluid rhinorrhea associated with long-standing overt ventriculomegaly in adults (LOVA)].

OBJECTIVE: Spontaneous cerebrospinal fluid rhinorrhea associated with aqueductal stenosis is rare. CSF diversion is reported to be a failure in the majority of cases. The combination of the repair of the skull base and CSF diversion is reported to be successful. We describe a case successfully treated by intradural repair with ventricular drainage followed by endoscopic third ventriculostomy. CLINICAL PRESENTATION: A 28-year-old woman presented with rhinorrhea, and occasional attacks of headache, vomiting, and unconsciousness for two years. She had been diagnosed as arrested hydrocephalus for 10 years. Magnetic resonance imaging revealed triventriculomegaly with ballooning of the floor of the third ventricle, tonsilar herniation, right anterior horn herniation into the cribriform plate, and bilateral temporal lobe herniation into the temporal base. INTERVENTION: A ventricular drain was inserted followed by dissection of the herniated brain and repair of the enlarged cribriform foramen with periosteal flap. Make sure that the bacterial culture negative, endoscopic third ventriculostomy has been performed. There is no recurrence of hydrocephalus and rhinorrhea for two years. CONCLUSION: Direct communication between the lateral ventricle and the nasal/paranasal sinus is a rare complication of aqueductal stenosis and LOVA. Surgical repair of the skull base followed by cerebrospinal fluid diversion with endoscopic third ventriculostomy was a safe and reliable method.

Pulmonary tumor thrombotic microangiopathy caused by lung adenocarcinoma: Case report with review of the literature.

Pulmonary tumor thrombotic microangiopathy (PTTM) is an uncommon cancer-related complication characterized by intimal proliferation in pulmonary small arteries and arterioles with or without tumor emboli. In the majority of cases, the causative lesion is gastric poorly differentiated adenocarcinoma. In the present study, an autopsy case of PTTM caused by lung adenocarcinoma is reported and the pathogenesis of this complication is discussed. Multiple nodular lesions in the bilateral lungs were found in a 62-year-old Japanese man. Transbronchial biopsy revealed non-small cell carcinoma. Chemotherapy was performed; however, the patient succumbed to sudden dyspnea. Autopsy revealed poorly differentiated adenocarcinoma with multiple intrapulmonary metastases and intimal proliferation of pulmonary small arteries and arterioles with or without tumor emboli, which were characteristic of PTTM. Tumor cells were immunohistochemically positive for vascular endothelial growth factor (VEGF) and osteopontin (OPN), which are endothelial proliferative factors. This case indicates the possible involvement of VEGF and OPN in the pathogenesis of PTTM caused by lung adenocarcinoma.