Angiomyolipoma is a hamartomatous condition which can occur as a component of the tuberous sclerosis complex. Lymphangiomyomatosis, another hamartomatous lesion occurring predominantly in the lungs, has long been suspected to be related to angiomyolipoma and tuberous sclerosis because of occasional clinical associations. We undertook this study to provide further support for the close relationship between these two entities. Five cases of lymphangiomyomatosis and 20 case of angiomyolipoma were retrieved for histological review and immunohistochemical studies. The antibodies used were anti-muscle specific actin (HHF-35), anti-desmin (D33) and anti-melanoma (HMB-45). Lesions featuring smooth muscle proliferation were used as controls. The proliferated smooth muscle cells in both lymphangiomyomatosis and angiomyolipoma were much plumper and paler or even clear, when compared with the deeply eosinophilic cytoplasm of the normal spindly smooth muscle cells and those of leiomyomas. Their nuclei were round to oval and pale rather than elongated and dark. Cells with bizarre nuclei were commoner in angiomyolipoma (18/20 cases) than lymphangiomyomatosis (1/5). In 12 cases of angiomyolipoma there were foci indistinguishable from lymphangiomyomatosis, i.e. plump spindle cells arranged in short fascicles around ramifying endothelium-lined spaces. All five cases of lymphangiomyomatosis stained for muscle-specific actin, desmin and HMB-45. For angiomyolipomas, the positivity rates for these markers were: 20/20, 17/20 and 18/20, respectively, including one case that was negative for both desmin and HMB-45. The various smooth muscle proliferations and tumours selected as controls were uniformly HMB-45 negative. The distinctive cytological features, morphological overlap and immunophenotypic profile all support a close relationship between lymphangiomyomatosis and angiomyolipoma, which probably represent different morphological manifestations of hamartomatous proliferation of a peculiar form of HMB-45-positive smooth muscle.
Background: Uterine tumor resembling ovarian sex-cord tumor (UTROSCT) is a considerably rare gynecological tumor which has undetermined pathogenesis but with distinct polyphenotypic immunohistochemical expressions. According to the limited cases and follow-up information in the relevant literature, most of the tumors exhibit indolent or low malignant clinical course and the outcomes of the patients with the tumors generally have a good ending. But for the subset of UTROSCT with aggressive characters, the outcomes of the patients with recurrent neoplasm were not always satisfactory.Case presentation: In this case report, the recurrence of neoplasm was reported in the pelvic cavity after 53 months of surgery and irregular follow-up. The recurrent neoplasm grew in an invasive manner, and the arrangement of the recurrent neoplasm cells was closer, the nucleus atypia is more pronounced, and the cells demonstrated a more briskly mitotic activity (10 mitotic figures/10 HPF). The Ki67 index increased significantly and the expression of P53 was positive. For the recurrent tumor, both the clinical characteristics and the histological morphology of the recurrent neoplastic cells showed a more malignant behavior. The patient received a palliative resection of pelvic mass and bilateral oophorectomy, and she died of intestinal obstruction caused by the recurrent disease 9 months postoperatively.Conclusions: UTROSCT should be recognized as a definite malignant potential neoplasm. Based on the clinicopathological, immunohistochemistry parameters and the reviewed previous literature, we speculated that significant mitotic activity and large tumors (≥10 cm) were of value for the aggressive characters of the tumor, and the recurrence of the tumor might lead to a poor prognosis.
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