Embryonal (botryoides) rhabdomyosarcoma of the uterus harboring a primitive neuroectodermal tumor component.

The association of a uterine sarcoma botryoides of the adolescence with a primitive neuroectodermal tumor is reported in a 12-year-old patient who presented with abnormal vaginal bleeding that occurred after passing per vaginam a polypoid mass. The sarcoma botryoides of the adolescence exhibited foci of cartilage and a central area of primitive neuroectodermal tumor with a trabecular, adamantiform histology and prominent angiogenesis. Primitive neuroectodermal tumor was positive for vimentin, synaptophysin, neuron-specific enolase, CD99, and SOX2 and negative for both the FLI-1 fusion protein and the rearrangement of ESWR1 gene. The neoplasm exhibited a nonaggressive behavior similar to sarcoma botryoides of the adolescence, being alive and well 3 y after its presentation. This is possibly related to its polypoid nature and the absence of invasive features at its uterine insertion level. A conservative approach without further resection and chemotherapy was indicated taking into account the patient's age.

An analysis of five clear cell papillary cystadenomas of mesosalpinx and broad ligament: four associated with von Hippel-Lindau disease and one aggressive sporadic type.

AIMS: Clear cell papillary cystadenoma (CCPC) is associated with von Hippel-Lindau disease (VHLD), but rarely involves mesosalpinx and broad ligament (M/BL). This study provides new data about its behaviour and immunophenotype. METHODS AND RESULTS: We performed an analysis of four benign cases of CCPC of M/BL with either characteristic clinical features or genetic markers [loss of heterozygosity (LOH)] of VHLD in patients ranging from 24 to 36 years and a sporadic case in a 52-year-old presenting with peritoneal metastases. All CCPCs were papillary but had solid and tubular areas. Haemorrhage, thrombosis and scarring were constant features and related to an unusual pattern of sub-epithelial vascularity. All clear or oxyphilic cells co-expressed cytokeratin 7 (CK7), CAM5.2 and vimentin, with strong apical CD10 and nuclear paired box gene 2 (PAX2) immunoreactivity. Three cases also showed positivity for VHL40, epithelial membrane antigen (EMA), Wilms' tumour suppressor gene (WT-1) and cancer antigen 125 (CA125) but only one expressed renal cell carcinoma (RCC) antigen. Vascular plexus overexpressed nuclear and cytoplasmic WT-1. CONCLUSION: The VHLD-associated cases appeared to be benign, but the sporadic case exhibited a low malignant potential. CCPCs show histological and immunophenotypical similarities with the recently reported clear cell papillary RCC, although the previously unreported apical CD10 and nuclear PAX2 expression may be related to their mesonephric origin. CCPC has a distinctive sub-epithelial vascular pattern that is consistent with its pathogenesis.

Endometrial intestinal metaplasia: a report of two cases, including one associated with cervical intestinal and pyloric metaplasia.

Intestinal metaplasia of the endometrium is extremely uncommon with only a single earlier case report. We describe 2 cases of endometrial intestinal metaplasia, one of them involving an endometrial polyp, characterized by the presence of intestinal-type epithelium containing goblet and neuroendocrine cells, which were positive with CK20, CDX2, chromogranin, and villin. In 1 case, there was concomitant intestinal and pyloric metaplasia in the endocervix. Together with the observation of the earlier reported case of endometrial intestinal metaplasia, there was also intestinal metaplasia in the cervix. This suggests a possible association between intestinal metaplasia at different sites in the female genital tract.