Intravascular large B-cell lymphoma in renal cell carcinoma incidentally detected by robot-assisted partial nephrectomy.

Introduction: Intravascular large B-cell lymphoma is a rare and aggressive type of extranodal large B-cell lymphoma. Although intravascular large B-cell lymphoma can invade various organs, renal involvement has been rarely reported. Synchronous occurrence of intravascular lymphoma with renal cell carcinoma is extremely rare. We herein report a case of intravascular large B-cell lymphoma in a renal cell carcinoma incidentally detected by robot-assisted partial nephrectomy. Case presentation: A 69-year-old female with recurrent fever lasting 4 years underwent robot-assisted partial nephrectomy for small renal cell carcinoma. Histological findings led to the diagnosis of intravascular large B-cell lymphoma, which involved the normal tissue of right kidney as well as clear cell renal cell carcinoma. She received six cycles of chemotherapy without major complications and achieved complete remission. Conclusion: We encountered a rare case of synchronous intravascular lymphoma with renal cell carcinoma.

A case of adenomyoepithelioma with myoepithelial carcinoma of the breast.

Adenomyoepithelioma with myoepithelial carcinoma of the breast is rare and diagnosed with histology and immunohistochemistry. We present a case of malignant transformation over 10 years, with ultrasonographic findings, highlighting the importance of an early excisional biopsy. Conservative surgery and radiation therapy were performed. There was no recurrence for 2 years.

ß-catenin alteration is rare in hepatocellular carcinoma with steatohepatitic features: immunohistochemical and mutational study.

Hepatocellular carcinoma (HCC) with steatohepatitic features (steatohepatitic HCC, SH-HCC) is a histological subset of HCC, highly associated with metabolic disease and underlying steatohepatitis. Although it has distinct clinicopathologic characteristics, little is known about the immunophenotype or genetic characteristics of SH-HCC. We conducted an immunohistochemical analysis on a tissue microarray containing 197 HCCs (70 SH-HCCs and 127 conventional HCCs (C-HCCs)), focusing on proteins associated with genetic subtypes of HCC and those associated with non-alcoholic fatty liver disease (NAFLD) or NAFLD-associated HCC. We also investigated CTNNB1 mutations in 84 HCCs (31 SH-HCCs and 53 C-HCCs) to better characterize the SH-HCC. When compared to C-HCC, SH-HCC was characterized by a significantly lower incidence of nuclear accumulation of ß-catenin (5.7 vs. 25.2 %, p < 0.001) and by a lower incidence of overexpression (H-score = 300) of glutamine synthetase (4.3 vs. 26.0 %, p < 0.001). Multivariate logistic regression analysis revealed that the low rate of nuclear ß-catenin accumulation in SH-HCC was independent of background etiology, including underlying steatohepatitis (p < 0.001). In accordance with the immunohistochemical results, CTNNB1 mutations were less frequent in SH-HCC than C-HCC (3.1 vs. 20.8 %, p < 0.048). Other notable findings included the ubiquitous expression of sonic hedgehog ligand in typical SH-HCC (100 %) and the less frequent expression of progenitor markers, such as SALL4 and EpCAM, in SH-HCC. These results indicate that SH-HCC as a subtype is not only characterized by morphology but also by distinct phenotypic and genetic traits.

Cerebral embolism through hematogenous dissemination of pulmonary mucormycosis complicating relapsed leukemia.

Invasive mucormycosis in patients with hematological diseases mostly occurs in the lungs. Invasive mucormycosis of other anatomical sites is relatively infrequent and its pathogenesis has not so far been well elucidated. Here, we describe an autopsy case of pulmonary invasive mucormycosis complicated by cerebral embolism with infarct. A 77-year-old Japanese woman with relapsed acute myeloid leukemia complained of left visual disturbance and weakness of the lower limbs. The diagnosis of leukemic infiltration to the central nervous system was made. Repeated intrathecal injection of methotrexate plus cytarabine resulted in partial amelioration of the neurologic symptoms. However, the patient then developed fever, dyspnea, and subsequent right hemiparesis. A computed tomography (CT) scan showed a consolidative shadow with halo sign in the left lung field, which was compatible with either invasive pulmonary aspergillosis or mucormycosis. These findings accounted for fever and dyspnea, but not hemiparesis. Despite antifungal therapy, the patient succumbed to death after two weeks. Autopsy revealed pulmonary invasive mucormycosis with a fungal ball in the lumina of the adjacent ascending aorta. Intriguingly, autopsy and postmortem CT scan identified left cerebral infarct due to mucormycosis, which accounted for the right hemiparesis. It is likely that the fungal ball caused the cerebral embolism through hematogenous dissemination. We should suspect hematogenous dissemination when we see a patient with pulmonary invasive mucormycosis developing neurologic symptoms.

Hepatocellular carcinoma with steatohepatitic features: a clinicopathological study of Japanese patients.

AIMS: The aim of this study was to investigate the clinicopathological significance of steatohepatitic features in hepatocellular carcinomas (HCCs) using a large-scale analysis. METHODS AND RESULTS: Retrospective clinicopathological analysis was performed on HCCs treated surgically at the University of Tokyo Hospital between 2005 and 2010. The diagnosis of HCC with steatohepatitic features (SH-HCC) was made if the tumour fulfilled four of the following five criteria: steatosis (>5% tumour cells), ballooning or Mallory-Denk body formation, interstitial fibrosis and inflammatory infiltrates. There were 120 HCCs (31.4%) from 106 patients (36.3%) that met the criteria of SH-HCC. Patients with SH-HCC were characterized by a higher frequency of diabetes mellitus and hypertension, along with higher serum levels of cholesterol and triglycerides, than those with conventional HCC (P < 0.01). The background liver of SH-HCC patients showed steatosis and steatohepatitis more frequently (P < 0.01). SH-HCCs were smaller, relatively more differentiated and had a higher frequency of bile duct invasion (P < 0.05). Multivariate analysis failed to show prognostic significance of steatohepatitic features in HCCs. CONCLUSIONS: SH-HCC is a subcategory of HCC associated with the patient's metabolic condition and the presence of steatosis or steatohepatitis in the background liver. Steatohepatitic features were not a significant prognostic factor for HCCs.

Rhabdomyosarcoma of the duodenum: report of a case.

This report presents a case of primary pleomorphic rhabdomyosarcoma arising in the duodenum. A 63-year-old male with persistent melena was referred for a solid tumor in his right upper abdomen detected using ultrasonography. Gastrofiberscopy revealed a protrusion in the upper part of the duodenum, with a large ulcer on the top of it. Enhanced computed tomography showed that the tumor extended to the pancreas. Pancreaticoduodenectomy was performed, despite the absence of malignant cells in the biopsy specimen, with a preoperative diagnosis of duodenal cancer. The tumor consisted of multiple cell types, and immunohistochemical staining was positive for desmin, HHF-35 and alpha smooth muscle actin. Electron microscopy revealed primitive Z-band structures in the tumor. The final diagnosis was pleomorphic rhabdomyosarcoma of the duodenum. This is the first report of primary rhabdomyosarcoma occurring in the duodenum, confirmed by immunohistochemical staining and electron microscopy.

Clinicopathologic characteristics of SALL4-immunopositive hepatocellular carcinoma.

The aim of this study was to investigate the clinicopathologic characteristics of sal-like protein 4 (SALL4)-immunopositive hepatocellular carcinoma (HCC). Solitary HCCs that were surgically treated at the University of Tokyo Hospital between 2000 and 2008 were the subject of this study. Diffuse, non-punctate nuclear immunoreactivity to SALL4 was observed in 47 of 337 HCCs (13.9%). Compared to patients with SALL4-negative HCC, patients with SALL4-positive HCC were younger (mean 59.2 years vs. 65.2 years), more frequently female (44.7% vs. 18.3%) and positive for hepatitis B virus angigen (42.6% vs. 18.6%). They had much higher serum levels of alpha-fetoprotein (median 3976.5 ng/ml vs. 14.0 ng/ml) (P < 0.001). Liver function tended to be favourable, as was shown by less indocyanine green retention at 15 minutes (ICG15), in patients with SALL4-positive HCCs (P < 0.001). Histologically, SALL4-positive HCCs exhibited less histological differentiation (P < 0.001) and had a higher frequency of micro- or macrovascular invasion (72.3% vs. 54.1%, P = 0.019) and intrahepatic metastasis (34.0% vs. 19.3%, P = 0.022) than SALL4-negative HCCs. SALL4-positive HCCs were more frequently immunoreactive for cytokeratin 19 (42.6% vs. 11.7%, P < 0.001) and EpCAM (51.1% vs. 8.3%, P < 0.001). The log-rank test indicated short-term disease-free survival (< 1 year) of patients with SALL4-positive HCC was worse than those with SALL4-negative HCC (P = 0.019). Multivariate analyses, however, failed to show the prognostic significance of SALL4 immunoreactivity in HCCs. In conclusion, SALL4-immunopositive HCCs constitute a subset with characteristic patient backgrounds and somewhat aggressive behavior, as was manifested by frequent vascular invasion and intrahepatic metastasis. There was little prognostic significance of SALL4 immunoreactivity in HCCs.