1/720. Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study. We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type iv, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract. ( info) |
2/720. Use of polyglactin 910 mesh (Vicryl) in pelvic oncologic surgery. Various synthetic materials have been shown to be useful surgical adjuncts in shielding the small intestine from pelvic radiation or in creating a new pelvic floor after major radical resections. Promising preliminary results with a polyglactin 910 (Vicryl) mesh in preventing radiation enteropathy prompted the authors to evaluate its clinical usefulness in reconstruction of the pelvic floor. Use of this mesh in pelvic exenterations (five total, one posterior) for advanced or recurrent gynecologic malignancies was associated with one enteroperineal fistula but no pelvic infection. In comparison with other pelvic reconstruction devices, this absorbable mesh significantly reduced intestinal morbidities in pelvic exenterations. This procedure appears to be feasible, reproducible, and safe, especially in patients who have undergone previous irradiation or those with an unsuitable omentum. ( info) |
| A case report of pelvic lipomatosis in a woman is presented. This condition is a benign, apparently self-limited disease in which mature adipose tissue is deposited in the pelvis. The clinical presentation is discussed. ( info) |
4/720. Low-grade fibromyxoid sarcoma: clinicopathologic case report with review of the literature. Low-grade fibromyxoid sarcoma is a rare, benign-appearing soft tissue neoplasm with an aggressive clinical course characterized by multiple local recurrences over several years, with ultimate spread to lung and occasionally to bone. Thus far, a total of 24 cases of low-grade fibromyxoid sarcoma have been reported in the literature. The authors present an additional case that grossly and microscopically emphasizes a pronounced lobular pattern of contrasting areas of cellularity showing high proliferative activity, as demonstrated by a proliferation marker, Ki 67 with MIB-1, and hypocellular areas with prominent myxoid component and abundant collagen fibrils. There was predominance of delicate capillary-sized stromal vessels with collagenized walls in both cellular and myxoid areas. The unusual features in this case were osseous metaplasia, prominent intranuclear pseudoinclusions, dna tetraploidy, and membrane-bound intracytoplasmic fat vacuoles. The immunoprofile and cytologic and ultrastructural features are described. After the excision of the tumor, the patient was treated with radiotherapy without chemotherapy. The patient has been observed for 26 months and is alive without the evidence of disease. The postoperative follow-up with axial computed tomography at 24 months showed no evidence of disease, except postsurgical fibrotic changes. ( info) |
5/720. Successful therapy in a child with a congenital peripheral medulloepithelioma and disruption of hindquarter development. PURPOSE: Medulloepithelioma is an embryonal multipotential neuroepithelial tumor with a striking potential for divergent differentiation. It is usually intraocular or intracerebral and associated with a good prognosis only if completely surgically excised. Data regarding therapy in children with incompletely resected tumors are limited. PATIENT AND methods: A girl was born with a large, peripheral, congenital medulloepithelioma associated with complete absence of the left hindquarter and anus. Plain film, ultrasonography, and magnetic resonance imaging demonstrated complete absence of the left kidney and hemipelvis. A subtotal resection of the mass and reconstruction of the tumor-related anatomical defects were performed. RESULTS: Pathologic examination showed neuroglia and pseudostratified neuroectoderm diagnostic of medulloepithelioma. She was treated with multiagent chemotherapy including vincristine, cisplatin, cyclophosphamide, carboplatin, and etoposide. She is now 50 months of age and developing normally without recurrent disease. CONCLUSIONS: A child with an incompletely resected congenital peripheral medulloepithelioma who has experienced long-term disease-free survival after treatment with chemotherapy is described. This report supports a role for adjuvant chemotherapy in the treatment of children with peripheral medulloepithelioma. ( info) |
6/720. Late recurrence of a uterine clear cell adenocarcinoma confined to an endometrial polyp: case report. A patient with an endometrial clear cell adenocarcinoma confined to a polyp developed recurrent disease in the abdomen and pelvis four years following hysterectomy. Treatment issues related to this uncommon clinical situation are discussed. ( info) |
7/720. Case report: intrahepatic portal-hepatic venous shunts associated with a huge pelvic leiomyoma. We present a case of portal-systemic encephalopathy due to intrahepatic multiple portal-hepatic venous shunts. A 71-year-old woman was admitted to our hospital because of recurrent episodes of disturbed consciousness. She showed no clinical signs of portal hypertension. liver function was normal, except for an indocyanine green retention rate of 34% at 15 min and blood ammonia level of 282 microg/dL. Portal venography revealed dilatation of the portal vein and multiple portal-hepatic venous shunts, and a liver biopsy specimen revealed almost normal liver. Further clinical examination revealed a huge pelvic tumour. At laparotomy, two dilated veins were seen to arise from the pelvic tumour with blood flow into the mesentery. The tumour was resected successfully and a histological diagnosis of leiomyoma was made. The blood ammonia concentration decreased to the normal range postoperatively. A follow-up portal venogram demonstrated decreased portal vein dilatation and minor portal-hepatic venous shunts, considered to be congenital in origin. It is concluded that hepatic encephalopathy was produced in this patient due to an excess portal blood flow from the huge pelvic leiomyoma via the mesentery, with portosystemic shunting through pre-existent (probably congenital) intrahepatic anastomoses. ( info) |
8/720. Proximal-type epithelioid sarcoma in the pelvic soft tissues. A case of proximal-type epithelioid sarcoma arising in the pelvic soft tissues of a 46-year-old man is presented. The tumor showed a predominantly epithelioid component with rhabdoid features, cord-like arrangement, small nests, abundant eosinophilic cytoplasm containing intracytoplasmic, paranuclear, hyaline-like globules, large vesicular nuclei, and prominent nucleoli. There were foci with alveolar arrangement and a spindle-cell proliferation. The tumor cells were positive for cytokeratin CAM5.2, vimentin, epithelial membrane antigen, and CD34. flow cytometry showed a diploid dna content with high S-phase fraction. The patient had pelvic lymph-node metastases. He died of the disease 5 months after diagnosis. It is important to include proximal-type epithelioid sarcoma in the differential diagnosis of pelvic tumors. ( info) |
| OBJECTIVE: The aim of this study was to report a case of extraovarian granulosa cell tumor and to describe its relevance to the histologic origin of granulosa cell tumors and to clinical practice. methods: The clinical course and histopathology of the case were reviewed, and a literature search for other reported cases was performed. RESULTS: A 67-year-old woman presented with postmenopausal bleeding and a pelvic mass. laparotomy revealed a 16-cm mass arising from the right pelvic sidewall, filling the pelvis, and involving the bladder and rectosigmoid colon. Both ovaries appeared normal and were separate from the mass. Pathologic examination revealed granulosa cell tumor. A literature search revealed no recently reported cases of extraovarian granulosa cell tumor. CONCLUSIONS: Granulosa cell tumors can arise in locations other than the ovary and may be derived from the mesenchyme of the genital ridge. women who have undergone oophorectomy may have the potential to develop granulosa cell tumors. ( info) |
10/720. Pelvic malignant mixed mesodermal tumor of uncertain origin: a case report. Extra-uterine, and especially extragenital, malignant mixed mesodermal tumors (MMMT) are very rare. A large intrapelvic tumor resected from a 56-year-old woman was investigated with morphological and immunohistochemical methods. A large, soft and fragile tumor was located in the pelvic space. The tumor showed high cellularity and was biphasic; it consisted of an admixture of adenocarcinoma and various kinds of sarcomas. The latter were comprised of high-grade endometrial stromal sarcoma, pleomorphic sarcoma, and chondrosarcoma. The pleomorphic sarcoma showed a storiform pattern. The periodic acid-Schiff-positive eosinophilic hyaline droplets and globules in multinucleated giant cells revealed a typical ring-like or peripheral staining for alpha-1-antitrypsin and alpha-1 antichymotrypsin. We considered this case to be pelvic MMMT of uncertain origin, heterologous type. ( info) |