Malignant vaginal melanoma with metastases to the papilla of Vater in a dialysis patient: a case report.

Malignant vaginal melanoma is an extremely rare clinical condition, with less than 150 cases reported to date. A dialysis patient had primary vaginal melanoma with metastases to the papilla of Vater. Gastroduodenoscopy revealed a polypoid tumor. Histological findings revealed vimentin, S-100 protein, HMB45, MelanA-positive sarcoma-like cells. This staining pattern indicated that this tumor was a malignant melanoma of the papilla. Analysis of an autopsy specimen of the papilla of Vater revealed metastasis from the primary vaginal melanoma. Metastasis of a malignant tumor to the gastrointestinal tract, especially to the papilla, is uncommon. Melanoma should be considered in the differential diagnosis of primary gastrointestinal tract malignancy.

Widespread ischemic brain lesions caused by vasculopathy associated with neurofibromatosis type 1.

We report the autopsy findings of a 63-year-old man with neurofibromatosis type 1 (NF1), in whom widespread ischemic brain lesions caused by vasculopathy associated with the disorder were observed. The patient, who had café au lait macules, axillary freckling, and neurofibromas, was inarticulate of speech, had difficulty in maintaining a sitting position, and was hyporeactive at the age of 57 years. He then developed autonomic dysfunction, followed by consciousness disturbance and status epilepticus. Repeated MRI studies disclosed multiple, ill-defined lesions in the brain and progressive cerebral atrophy. The histopathological features of the lesions were those of ischemia that had occurred with spatiotemporal variability in the brain. Characteristically, many arteries in the subarachnoid space manifested accumulation of cells in the intimal layer: this hyperplasia had resulted in narrowing and occlusion of the lumen. Immunoblotting demonstrated a marked decrease of neurofibromin, the NF1 product, which is known to act as a functional molecule in the normal process of vascular maintenance and repair. This case provides useful information about the pathomechanisms underlying central nervous system manifestations in patients with NF1.

Malignant potential of hepatic angiomyolipoma: case report and literature review.

Hepatic angiomyolipoma (AML) is known as a rare benign tumor with invasive growth. In the past, some of these tumors were misdiagnosed as hepatocellular carcinomas, because of the similar pattern on imaging studies. Recently, correct diagnoses have been increasing, with the development of HMB-45 immunohistochemical staining, and it appears that the majority of these tumors behave as benign tumors. However, there are not a few cases which have resulted in fatal courses because of recurrence and metastasis of the tumor. The clinical features and signs of the malignant potential of this tumor are unknown; thus, the management and treatment of the tumor are still controversial. Here in this article, we report a case of hepatic AML which showed a size increase of 175% in 1 year, and portal vein thrombosis detected by angiography. During a follow up of 3 years after a curative hepatic lobectomy, no metastasis or recurrence was seen. Review of the literature suggests that portal vein thrombosis could be one of the markers of the malignant potential and transformation of this tumor. Therefore, in this paper, we recommend surgical treatment of hepatic AML in which there is a strong suspicion of portal vein thrombosis.

Eosinophilic esophagitis: a case report. Effective treatment with systemic corticosteroids for the relapse of the disease.

We report on a case of eosinophilic esophagitis in a 33-year-old Japanese woman who visited our hospital with dysphagia and esophageal food impaction in April 2006. She had had the same symptoms in May 2005, and, by endoscopic and histologic examination, she was diagnosed with eosinophilic esophagitis. Biopsy specimens from multiple locations in the esophageal mucosa had shown remarkable infiltration of eosinophils, more than 20 eosinophils per high-power field in squamous mucosa, and she had been treated with Th2 cytokine antagonist and proton pump inhibitor at the time. Her symptoms had not improved, but she had received no further medical treatment. In 2006, upper gastrointestinal endoscopy showed edematous whitish esophageal mucosa, multiple ulcerations with whitish exudates on their surfaces, and white plaques. Biopsy specimens showed the same change as before, and corticosteroid (prednisolone 20 mg/day) was administered orally. After 2 weeks of corticosteroid therapy, her symptom had effectively improved. Endoscopy after 15 weeks of the therapy revealed remarkable improvement, and biopsy specimens from esophageal mucosa revealed the disappearance of the eosinophil infiltrates. We report on a case of eosinophilic esophagitis effectively treated with systemic corticosteroids. Eosinophilic esophagitis has, as yet, no standardized treatment. However, wider recognition of its features on endoscopy may reveal more cases, thereby increasing our understanding of this disease, and will provide new therapeutic possibilities.