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Extrarenal Wilms' tumor: an analysis of four cases.

During a review of Wilms' tumor, four located external to the kidney were identified. Patient age ranged from 7 months to 4 years; three were female. One neoplasm was located in the parametrial connective tissue to the left of the uterus; both kidneys were radiographically normal. Three neoplasms were located in the right retroperitoneum adherent to the surface of the ipsilateral kidney, but separated from the parenchyma by a thick fibrous capsule. Two were attached to the upper pole, while the third was attached to the midportion of the kidney. Radiologic studies showed displacement of all three kidneys, but intravenous pyelogram (IVP) revealed no calyceal distortion. All four neoplasms were favorable histology Wilms' tumor: one was monophasic epithelial type, one was monophasic blastemal type, and two had a mixture of stromal, epithelial, and blastemal tissue. No teratomatous elements were present. Immunoperoxidase staining for cytokeratin (AE-1, AE-3, CAM 5.2), vimentin, and epithelial membrane antigen (EMA) showed the strongest focal positive staining of tubular structures with CAM 5.2, and slight staining with EMA. Staining reaction to vimentin was variable, but negative in most areas. Three tumors extracted from paraffin were diploid by quantitative flow cytometric DNA analysis; in one case, flow cytometry could not be performed. Clinical follow-up from 2 years to 6 years showed all children to be alive without evidence of disease. Based on the similarity to conventional renal Wilms' tumor, these findings support the hypothesis of displaced mesonephric/metanephric rests for the origin of extrarenal Wilms' tumor.

Child, Preschool↗

Familial mesothelioma: a report of two families.

Five reports of familial mesothelioma in which mesotheliomas occurred in two or more family members have been recorded in the medical literature. In this report, we describe two examples of familial mesothelioma. In one family, three brothers who worked in the asbestos insulation industry developed mesothelioma. In the second family, the father, who was occupationally exposed to asbestos, died from a tubulopapillary peritoneal mesothelioma 11 years before his son died from an identical histologic type of peritoneal mesothelioma. Our report, as with those previously recorded, suggests that genetic factors may be important in the genesis of some mesotheliomas.

Adult↗

Unusual radiologic features of metastatic prostatic carcinoma confirmed by immunohistochemical study.

The clinical application of an immunohistochemical technique for prostatic acid phosphatase allowed the accurate diagnosis of metastatic prostatic carcinoma in 17 patients whose condition was not recognized by either clinical or histologic observations. The cardinal manifestations in these 17 patients included supraclavicular lymphadenopathy in 7, hilar lymphadenopathy in 1, pulmonary infiltration in 2, simulating carcinoma of the rectum in 4, fracture of femur in 1, orbital tumor in 1, and brain tumor in 1. A retrospective analysis of the radiologic features of these patients showed that the unusual manifestations were due to the route of metastasis, that is, direct invasion, lymphatic, and hematogenous. Radiologic studies, even in these unusual cases, are both useful in establishing the diagnosis and helpful in delineating the mode of metastasis of prostatic carcinoma.

Acid Phosphatase↗

Benign retroperitoneal schwannoma.

A case of benign retroperitoneal Schwannoma is presented, with special attention to clarification of often confused pathologic nomenclature. The radiologic diagnosis and the literature on this rare tumor are reviewed.

Adult↗

Castleman disease. Unusual retroperitoneal location indistinguishable from malignant tumor in preoperative angiographic appearance.

Castleman disease (angiofollicular lymph node hyperplasia) has been reported rarely as a solitary retroperitoneal mass radiographically indistinguishable from retroperitoneal malignancies. We report a case of a hyaline-vascular variant of Castleman disease presenting as a pararenal mass. The computerized tomographic and angiographic appearances of this lesion are presented, and the clinical importance of the differential diagnosis of retroperitoneal tumors is discussed.

Adult↗

Lipoplastic lymphadenopathy presenting as an ovarian mass: a case report.

Lipoplastic lymphadenopathy is a pathological condition wherein accumulations of intranodal fat result in lymph node enlargement and may mimic abdominal, pelvic, and retroperitoneal neoplasms, particularly lymphomas. The pathology appears to be different from lipomatosis in that benign, mature adipocytes and lipids are located within lymph nodes, rather than deposited in body cavities. The case of a 49-year-old woman presenting as an ovarian neoplasm is presented as an example of the pathology and its ability to masquerade as other neoplasms. The etiology of lipoplastic lymphadenopathy is unclear although associated causes are suggested. The difficulties of radiological examination of this case and others makes open lymph node biopsy important for the final diagnosis.

Abdomen↗

Body computed tomography in the evaluation of pediatric malignancy.

Fifty-eight body CT scans were obtained in 33 children with malignant disease over a 2-year period at the Medical College of Virginia Hospitals. When compared with conventional radiography, excretory urography, nuclear medicine, and ultrasound, CT provided additional information in 26 patients (78%). In 17 patients CT was useful for monitoring response to surgery, radiation therapy, and/or chemotherapy.

Abdominal Neoplasms↗

Computed tomography of invasive pleural mesothelioma.

The computed tomography (CT) findings in three patients with invasive pleural mesothelioma are presented, and the invasive route and metastatic extension of the disease in the abdomen and retroperitoneum are discussed. These findings indicate the value of CT in detecting the extrathoracic extension of mesothelioma. Abdominal CT studies should be obtained in evaluating mesothelioma patients for extrathoracic extension of the disease.

Abdominal Neoplasms↗

Molecular characterization of a recurring complex chromosomal translocation in two human extragonadal germ cell tumors.

The molecular characterization of a recurring complex chromosomal translocation involving 6p21, 6p22, 6q23, and 11q13 in two independent but similar extragonadal human germ cell tumors was initiated using fluorescence in situ hybridization (FISH) and pulse field gel electrophoresis (PFGE) techniques. By using a series of specific probes from the 11q13 region, the translocation breakpoint in this chromosomal band could be located within a long-range restriction enzyme map in between the markers D11S457 and D11S546. In addition, aberrantly hybridizing restriction fragments were revealed by PFGE in both tumors, indicating that the breakpoint region must be located within a distance of at maximum 200 kilobase pairs (kbp) from the nearest DNA marker (D11S546).

Blotting, Southern↗