Gastrointestinal hemorrhage in Turner syndrome. Long-term follow-up with postmortem examination.

A 57-year-old woman with Turner syndrome had severe recurrent gastrointestinal bleeding. Exploratory laparotomy at the age of 26 showed an extensive telanglectasia of the entire small intestine. Following death due to myocardial infraction at age 57, postmortem examination revealed only a 0.2-cm residual telangiectasia in the mucosa of the distal part of the ileum. Spontaneous regression of the intestinal telangiectasia observed in Turner syndrome may occur and account for the improved prognosis with age.

Mesoblastic nephroma of adulthood. Report of three cases.

Mesoblastic nephroma is an uncommon congenital tumor of infancy that rarely occurs in adults. We report three patients (two were female, one was male) who had mesoblastic nephroma of adulthood and who presented at 45, 64, and 66 years of age with hematuria, flank mass, and pain. All underwent nephrectomy without postoperative adjuvant therapy. The tumors were solitary yellow-tan masses with solid and cystic areas involving the renal cortex (three cases) with extension into the renal pelvis and calyces (two) and ureter (one). Microscopically, all consisted of uniform spindle cell proliferations with entrapped dilated renal tubules. Focal necrosis was present in two, but no atypia or mitoses were identified in any case. The spindle cells displayed cytoplasmic immunoreactivity for vimentin, desmin, panmuscle actin (HHF-35), and alpha-smooth-muscle actin, but were nonreactive for keratin (AE1/AE3), epithelial membrane antigen, and S-100 protein. Electron microscopy revealed the presence of smooth-muscle differentiation in two cases and undifferentiated mesenchyme in one. All tumors were DNA diploid by flow cytometry. The patients were free of recurrence 8 months-2 years postoperatively. Because surgical excision may be curative, mesoblastic nephroma in adult patients must be differentiated from spindle cell neoplasms of the kidney that require additional therapy.

Primary papillary serous neoplasia of the peritoneum: a clinicopathologic and ultrastructural study of eight cases.

The well-documented but rare primary papillary serous peritoneal tumors are difficult problems for the pathologist and the clinician. Because of their unusual location, these tumors are often classified as mesothelioma or advanced ovarian carcinoma. In this study, we report the clinicopathologic features of eight primary peritoneal serous papillary tumors and compare their histologic and ultrastructural features to 12 serous ovarian tumors and 16 epithelial mesotheliomas (two peritoneal and 14 pleural). The eight peritoneal serous papillary tumors occurred in women aged 19 to 75 years; two were serous tumors of low malignant potential (borderline) and six were serous carcinomas. The tumors were located in the mesosalpinx, left pelvis, omentum, and/or surface of the ovary. The two patients with borderline neoplasms had long disease-free survival (11 years and 20 years), while three of the four patients with carcinoma with more than 1 year of follow-up died of disease. The peritoneal serous papillary tumors were morphologically identical to serous ovarian tumors of equivalent grade. Well-differentiated papillary structures with distinct fibrovascular cores and one or several layers of columnar, crowded cells, dense overlapping nuclei with a long axis perpendicular to the surface of the papillary cores, and numerous psammoma bodies were features of the peritoneal and ovarian serous tumors. In contrast, the tubulo-alveolar, solid, or poorly defined papillary structures lined by well-spaced polygonal to cuboidal cells with abundant cytoplasm, absence of nuclear polarity, and infrequent psammoma bodies characterized the mesotheliomas. Epithelial mucin and carcinoembryonic antigen (CEA) immunoreactivity, when present, supported a diagnosis of serous tumor in these generally mucin-poor and CEA-negative neoplasms. Ultrastructurally, the cells of serous tumors had slender, straight microvilli of variable length interspersed with or without cilia, while the nonciliated cells of mesothelioma had long, exuberant, wavy microvilli. The morphologic and clinical features of the peritoneal papillary serous tumors are distinctive enough to warrant their separation from mesotheliomas.

Paecilomyces javanicus endocarditis of native and prosthetic aortic valve.

A 41-year-old diabetic woman developed endocarditis of the aortic valve caused by Paecilomyces javanicus six years after insertion of a porcine mitral valve heterograft. The patient died shortly after aortic valve replacement. Autopsy revealed vegetations of the aortic heterograft, valve ring abscess and ascending aortitis due to Paecilomyces. There was no involvement of the mitral valve heterograft. Lesions due to mycotic emboli were found in the kidneys, spleen, and brain. Cultures of the surgically removed aortic valve and of the kidney at autopsy produced rapid growth of P. javanicus. The gross and microscopic pathologic and cultural characteristics of this organism are described with a review of the literature. Previously reported cases of Paecilomyces endocarditis occurred only in prosthetic heart valves. This is the first known report of P. javanicus endocarditis of a native valve and its prosthetic heart valve heterograft.

Cystadenocarcinoma of the rete testis.

We report a rare case of primary adenocarcinoma of the rete testis. The stage A lesion was managed successfully with orchiectomy and retroperitoneal lymph node dissection. The histopathological features, treatment and prognosis of this unusual malignancy are reviewed.

Diagnostic accuracy of multiple biopsies from flexible fiberoptic bronchoscopy. A comparison of central versus peripheral carcinoma.

We studied 46 patients, 26 with central visible tumors and 20 with peripheral nodules (in these, biplane fluoroscopic guidance was used), to determine the optimal number of forceps biopsies necessary to establish a diagnosis of carcinoma with the flexible fiberoptic bronchoscope. Overall yield was 96% (25 of 26) for central tumors and 75% (15 of 20) for peripheral nodules, using the combination of forceps biopsies and brushings. The combination of cytologic examinations plus one forceps biopsy produced a 92% diagnostic accuracy for central visible tumors. However, for central tumors the maximal diagnostic yield was not achieved until the fourth forceps biopsy, and for peripheral lesions accuracy continued to increase through the sixth forceps biopsy, and for peripheral lesions accuracy continued to increase through the sixth forceps biopsy in this study. Theoretically, as many as 10 biopsies may be necessary to maximize diagnostic yield for peripheral carcinomas.