Kimura's disease: clinical, histological and immunohistochemical studies.

In the present study, 14 cases of Kimura's disease were clinicopathologically studied. The disease occurred at ages ranging from 5 to 75 years. The average age was 37.8 years. Sexes were about equally affected. The most common sites were the subcutis of head and neck, and parotid gland. Simultaneous involvement of lymph nodes occurred in 5 cases. Laboratory findings revealed eosinophilia in almost all the patients, but serum IgE levels were not elevated in 2 patients. Lesions were surgically removed and the clinical course thereafter was favorable for all but one case. Histologically, lesions were characterized by lymphoid follicles, granulation tissue with infiltration by many eosinophils, lymphocytes, plasma cells, mast cells and histiocytes, proliferation of blood vessels and fibrosis. Immunohistochemically, IgE reacted strongly in germinal centers, showing a reticular pattern. IgG-, IgA- and lysozyme-positive cells were scattered mainly in interfollicular granulomatous areas. Pathogenesis of this disease is briefly discussed.</p

Early stages of development of rat brain tumors induced by JC virus: a sequential histological and immunohistochemical study.

In order to clarify the origin of JC virus-induced brain tumors in rats, the development of tumors was sequentially analyzed histologically and immunohistochemically. Twenty-two of 30 rats (73%), which were intracerebrally inoculated with JC virus within 24 h of birth (group 1), developed, as a group, 45 brain tumors after 12 to 26 weeks. Seventeen of 27 rats (63%), which were inoculated on the 7th day after birth (group 2), developed 37 brain tumors as a group after a time 12 to 40 weeks. The tumors were found exclusively in the cerebrum. The microtumors, which were defined as tumors less than 2 mm in diameter, were located in the subependymal plate around the ventricular system. The microtumors and most part of the macrotumors consisted of cells of undifferentiated neuroectodermal nature, showing nuclear palisades and Homer-Wright-pseudorosette-like structures. Some tumor cells of macrotumors had an astrocytic nature and were positive for glial fibrillary acidic protein, S-100, Leu 7, and vimentin. In conclusion, the target cells of JC virus in rats may be undifferentiated subependymal cells of the cerebrum. The tumor cells show partial glial differentiation as they grow.</p

Mallory Bodies in Hepatocytes of Alcoholic Liver Disease and Primary Biliary Cirrhosis Contain Nε-(Carboxymethyl)lysine-Modified Cytokeratin, but not those in Hepatic Carcinoma Cells

Mallory bodies (MBs) are intracytoplasmic bodies seen in hepatocytes of alcoholic liver disease, primary biliary cirrhosis and hepatocellular carcinoma. However, the mechanism of MB formation has not been fully understood. Proteins could be modified to advanced glycation end products (AGEs) after long-term incubation with reducing sugar. AGEs are known to accumulate in several tissues in aging and age-enhanced disorders. To study the possible glycation process in the formation of MBs, hepatocytes of 80 human liver tissues with MBs were subjected to immunohistochemical analyses with five AGEs, two markers for oxidative stress proteins (OSPs) and four stress-response proteins (SRPs). MBs in hepatocytes of primary biliary cirrhosis and alcoholic liver disease were strongly positive for Nε-(carboxymethyl)lysine (CML) and weakly positive for pyrraline. MBs in hepatocellular carcinomas were negative for both CML and pyrraline. No significant immunoreactivity was detected in MBs for other AGEs, such as Nε-(carboxyethyl)lysine, pentosidine, and 3DG-imidazolone, or for OSPs and SRPs. Stainings for cytokeratin, a major protein component of MBs, and CML were co-localized. Furthermore, immunoblot analysis suggested that cytokeratin of MBs was modified to AGE, since a single protein band detected by a monoclonal anti-CML had a molecular weight identical to cytokeratin. The absence of the CML signal in MBs of hepatocellular carcinoma cells could be explained by scarce content of cytokeratin in carcinoma MBs

Juvenile Granulosa Cell Tumor with Elevated Peripheral Interleukin-6 Level Shows Prolonged Fever and Delayed Puberty

Juvenile granulosa cell tumor (JGCT), classified as a sex cord-stromal tumor, is a rare neoplasm. This is an instructive case report of JGCT accompanied by augmented interleukin (IL)-6 secretion. A 13-year-old girl with prolonged fever and delayed puberty was diagnosed with JGCT of the left ovary based on an imaging study and pathological investigation. Although it was not clear whether IL-6 was secreted from the tumor cells, her serum level of IL-6 was very high. After tumorectomy, the patient’s symptoms immediately disappeared, her IL-6 level decreased, and she entered puberty. Therefore, augmented IL-6 secretion production induced by tumors should be considered a potential cause of prolonged fever and/or delayed puberty

High Efficacy of Preoperative Low-Dose Radiotherapy with Sanazole (AK-2123) for Extraskeletal Ewing's Sarcoma: A Case Report

Extraskeletal Ewing's sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from Ewing's sarcoma of bone. We report a case of extraskeletal Ewing's sarcoma with several systemic problems. A 69-year-old man presented with a 5-month history of a rapidly enlarging mass in the right thigh. Because preoperative radiotherapy with sanazole (AK-2123) contributed the tumor mass reduction down to 40% in size, the tumor was successfully resected with clear surgical margins and repaired with a musculocutaneous flap. The high efficacy of pre-operative low-dose radiotherapy with sanazole was histologically confirmed that the resected tumor specimen involved no viable tumor cells and showed 100% necrosis. Based on clinical outcomes in this case, the combined modality of pre-operative low-dose radiotherapy with hypoxic cell radiosensitizer and adequate surgical resection might provide for the useful clinical application of extraskeletal Ewing's sarcoma treatment

Chronic Active Epstein–Barr Virus Infection Indistinguishable from Autoimmune Hepatitis: A Case Report

Chronic active Epstein–Barr virus (CAEBV) infection is a rare disease, mainly affecting children, typically characterized by persistent infectious mononucleosis (IM)-like symptoms. We describe an adult case of CAEBV without IM-like symptoms, which was indistinguishable from autoimmune hepatitis (AIH). A 60-year-old woman with liver damage was diagnosed with AIH (International Diagnostic Score: 16 points). She had been treated with prednisolone for three years; however, her transaminases had never normalized. She was admitted for another liver biopsy due to repeated high fevers and worsening of her liver damage over two months. Her EBV-DNA copy number was 2.9 × 104 copies/μg DNA, and EBV-encoded small RNA1-positive lymphocytic infiltration was observed in both the present and previously collected (three years ago) liver tissue samples. This case implies that hepatic involvement in a CAEBV without IM-like symptoms is difficult to distinguish from AIH and may be misdiagnosed. In some steroid resistant AIH cases, evaluating for CAEBV may be valuable

Cerebellar Ganglioglioma in Childhood: Histopathologic Implications for Management During Long-term Survival: A Case Report

We report the case of a 19-year-old female with cerebellar ganglioglioma that was diagnosed at 4 years of age. Despite treatment with partial resection, radiation, and chemotherapy, residual tumor slowly expanded into the brainstem and upper cervical cord, resulting in nocturnal hypopnea, progressive tetraparesis, and feeding difficulty during 8?10 years of age. Initiation of temozolomide and bevacizumab was effective in preventing further expansion of the tumor, and the patient has been treated at home and in school with noninvasive positive pressure ventilation and gastrostomy. Histopathologic examination of the resected tumor tissue revealed phospho-S6-positive tumor cells of either neuronal or astroglial appearance. This suggests that a higher proportion of cells of glial lineage could be linked to the progression of cerebellar ganglioglioma in childhood. Possible treatment options with mammalian target of rapamycin inhibitors are discussed