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In this article, we describe unilateral gynecomastia and pseudoangiomatous stromal hyperplasia (PASH) in a case of type-1 neurofibromatosis (NF-1). It is important to distinguish PASH from fibroadenoma clinically, and from true blood capillaries and angiosarcoma histologically. In the present case, giant multinucleated cells lined the pseudovascular spaces, which was markedly different from that of conventional breast PASH. The origin of PASH has been reported to be either the fibroblast or the myofibroblast phenotype and may be affected by endocrine signaling because many cases have been reported in premenopausal women, and cases are often estrogen (ER) and progesterone receptor (PR) positive. However, previous reports have identified PASH in NF-1 in juvenile males only, and the cases were negative for α-SMA, ER and PR. The cause and prognosis of PASH in NF-1 may be distinguished from that of conventional PASH, and mast cells, histiocytes and CD54 may play roles., (Copyright © 2012 Elsevier GmbH. All rights reserved.)

A case of non-functioning adrenocortical adenoma combined with myelolipoma and endothelial cysts is reported. A 72-year-old Japanese female was noticed to have right renal and left adrenal tumors by an abdominal CT scan. At surgery, the mildly enlarged left adrenal gland contained a well-demarcated tumor. Macroscopically, it was yellowish to dark red or grayish in color, and was characterized by geographic appearance on the cut surface. Histopathological examination revealed a solid proliferation of clear or compact cells and a normal rim of adrenal gland, coexisting with vascular multiple cysts and myelolipomas. The cysts were filled with clotted blood, fibrinous material, or thrombi, and were partially lined with flattened endothelial cells with focal papillary hyperplasia, which were immunohistochemically positive for CD31 and CD34. These cystic walls were often thickened with hyalinized fibrosis and calcification, and were connected to myelolipomatous elements. To our knowledge, this is the first case report of adrenocortical adenoma associated with myelolipoma and endothelial cysts. It is probable that the extensive degeneration in adenoma might induce myelolipomatous metaplasia and cystic vascular formation., (Copyright © 2010 Elsevier GmbH. All rights reserved.)

Although intralesional calcification is a common finding of synovial sarcoma, ossification is an unusual phenomenon in such a soft tissue sarcoma. Here we report a case of ossifying synovial sarcoma arising in the back of a young adult man. Microscopically, the tumor was composed of spindle or oval cells together with foci of atypical polygonal cells in small nests or cord-like structures, displaying an epithelial appearance. In addition, extensive osteoid or woven bone formation was present in the tumor, resembling extraskeletal osteosarcoma. However, an SS18-SSX1 fusion gene transcript was detected by reverse transcription-polymerase chain reaction, supporting the diagnosis of biphasic synovial sarcoma. The osteogenic phenotype of the tumor cells was further demonstrated by an intense immunohistochemical expression of Runx2, a key transcription factor involved in the regulation of osteoblastic differentiation. The current case suggests the diagnostic utility of the molecular detection of a tumor type-specific fusion gene and expands the phenotypic plasticity of the soft tissue sarcoma of uncertain differentiation.