[Ganglioneuroblastoma].
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Adrenal epithelioid angiosarcomas (AEA) are rare neoplasms. We report the clinicopathologic features of nine cases of AEA. AEA occurred most frequently in the sixth and seventh decades of life (age range, 45-85 years; median, 60); five cases occurred in men and four in women. Presenting symptoms included abdominal mass with or without pain, weight loss, fever, and weakness. Two cases were asymptomatic; one was discovered during evaluation for other disease(s) and the other at autopsy. All neoplasms were nonfunctioning. Radiographic evaluation demonstrated suprarenal or retroperitoneal neoplasms ranging in size from 6 to 10 cm in greatest dimension. Histologically, the neoplasms were invasive, predominantly arranged in solid sheets or nests, and composed of epithelioid cells. Endothelial cell differentiation was suggested by the transition areas between dilated anastomotic vascular spaces and the sheet-like growth, the cytomorphologic similarity between the endothelial cells lining the discernible vascular spaces and those seen in the solid foci, and the presence of intracytoplasmic vacuolization occasionally containing red blood cells. Endothelial derivation was confirmed by immunohistochemistry including Factor VIII-related antigen (FVIII), CD-34 (hematopoetic progenitor cell antigen), and/or Ulex europaeus agglutinin-1 lectin immunoreactivity (UEA-1) and by ultrastructural findings, including rod-shaped microtubulated bodies and intracytoplasmic lumen formation. In addition, cytokeratin reactivity was seen in seven cases, and B72.3 (tumor-associated glycoprotein-72) reactivity was seen in six. Surgical resection was the treatment of choice, occasionally supplemented by chemotherapy. Three patients are presently alive, free of disease, at 13, 11, and 6 years following diagnosis. Three died with metastatic AEA of the lung, and three died of unrelated causes.
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The myxoid variant of adrenocortical carcinoma is a rare neoplasm described previously in only two case reports. Because of the rarity of these lesions, the presence of myxoid changes in adrenal cortical neoplasms usually raises the possibility of malignancy. We studied the histopathologic features of 14 cases of myxoid adrenocortical neoplasms, including six adenomas and eight carcinomas. All patients with adenomas with sufficient follow-up (n = 5) were alive with no recurrence of their tumors or evidence of metastatic disease. Four patients with carcinomas died of their disease, two were alive with metastatic disease, and one was alive with no evidence of recurrence or metastatic disease. Histologically, the 14 tumors varied in their myxoid composition, ranging from 10% to 95%. The myxoid foci stained positively with Alcian blue and were usually negative with periodic acid-Schiff and mucicarmine stains. As a group, the immunophenotype of the lesions was typical of other adrenal cortical neoplasms, with positive immunostaining for vimentin, synaptophysin, and alpha-inhibin. One tumor was focally positive for keratin. Myxoid adrenal cortical neoplasms should be included in the differential diagnosis of myxoid retroperitoneal neoplasms. Myxoid changes in adrenal cortical neoplasms may be present in both adenomas and carcinomas, and the usual clinical and histopathologic features for adrenocortical neoplasms should be used to diagnose these neoplasms.
Rhabdomyoblasts demonstrating immunoreactivity for muscle-specific actin, desmin, and myoglobin were identified in smears obtained by aspiration from a large retroperitoneal mass in a 14-mo-old girl. Following a tentative diagnosis of a rhabdomyogenous neoplasm, retroperitoneal exploration and adrenalectomy demonstrated a stromal poor neuroblastoma with extensive rhabdomyogenous differentiation. The presence of a subpopulation of rhabdomyoblasts was not diagnostic of rhabdomyosarcoma when obtained by fine-needle aspiration from a retroperitoneal tumor composed predominantly of primitive small round cells.
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Internal bleeding in patients undergoing orchiectomy for a malignant testicular tumor can cause a dissecting hematoma in the retroperitoneum. This mass may have the clinical appearance of an iliac fossa mass and may simulate metastasis on computed tomography (CT). This condition was seen in four of 486 orchiectomy patients who underwent postoperative staging with CT. One patient is described in detail.
A report is given on a 59-year-old female with retroperitoneal malignant histiocytosis and duodeno-retroperitoneal fistula. This had caused extensive autodigestion of the tumour and secondary inflammatory necrosis of the lumbar vertebral bodies. The uncommon finding and its rarity are discussed.
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We tried to divide anatomically the retroperitoneal space into three areas on computed tomography, infrahilar, suprahilar and retrocrural spaces, and investigated the relationship between frequency of lymph node swelling in each space and clinical picture in 15 patients with testicular cancer of stage II or III, particularly focusing on the lymph node swelling in the retrocrural space. Lymph node swelling was found in the infrahilar space in 11 cases (73.3%), in the suprahilar space in 7 cases (46.7%) and in the retrocrural space in 6 cases (40.0%). Retrocrural or suprahilar lymph node swelling was always accompanied by suprahilar or infrahilar lymph node swelling. Most of the patients with retrocrural lymph node swelling had bulky tumors in the infrahilar level and frequently cervical lymph node swelling. Retrocrural lymph node swelling did not necessarily affect the patient's prognosis. The retrocrural lymph node seems to be a sentinel node for retroperitoneal lymph node swelling not only in the case of testicular cancer but also in the cancer of urogenital malignancies.
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