The existence of angiosarcoma of the thyroid gland and its relation to angiomatoid carcinoma have been debated. The authors reviewed eight angiomatoid thyroid neoplasms. Controls consisted of six sarcomatoid thyroid carcinomas without angiomatoid features and an angiosarcoma metastatic to the thyroid gland. All eight angiomatoid neoplasms consisted of epithelioid cells with prominent eosinophilic cytoplasm lining vascularlike spaces. All eight expressed vimentin. Four tumors were predominantly angiosarcomalike neoplasms, based on staining for factor VIII-related antigen (three of four), CD31 (four of four), CD34 (one of four), and Ulex europaeus I lectin (four of four); they lacked epithelial markers other than cytokeratin (two of four). Four tumors were designated as angiomatoid carcinomas, based on staining for multiple epithelial markers: cytokeratin (four of four), epithelial membrane antigen (three of four), thyroglobulin (three of four). Three angiomatoid carcinomas also expressed or labeled with one or more vascular markers: CD34 (one of four), CD31 (two of four), Ulex europaeus I lectin (one of four), factor VIII-related antigen (one of four). The metastatic angiosarcoma to the thyroid gland labeled for factor VIII-related antigen, vimentin, CD31, and with Ulex europaeus I lectin. It did not express CD34. The six sarcomatoid carcinomas without angiomatoid features stained for cytokeratin (four of six), epithelial membrane antigen (one of six), and vimentin (six of six). None labeled for thyroglobulin, factor VIII-related antigen, CD31, CD34, or with Ulex europaeus I lectin. Angiomatoid carcinomas of the thyroid gland exhibit both epithelial and endothelial features. "Angiosarcoma" may represent the extreme in this spectrum of endothelial differentiation. All tumors behaved in a similar clinical fashion characterized by persistent local disease, widespread metastases and poor prognosis.

We report on an infant with minor anomalies and a de novo 1q duplication. The chromosomal abnormality was diagnosed prenatally after sonographic detection of cerebral ventriculomegaly and bilateral choroid plexus cysts in the fetus. The amniocentesis showed an abnormal male karyotype, 46,XY,dup(1)(q32q44), subsequently confirmed by fluorescence in situ hybridization using whole chromosome paint 1 and comparative genomic hybridization. The baby, born at 37 weeks of gestation, had wide cranial sutures and large fontanelles, sloping forehead, hypertelorism, short and downward-slanting palpebral fissures, a high-arched and narrow palate, malformed ears, and long feet with overriding second and third toes. This is the sixth case of known duplication involving the 1q32q44 segment; the physical findings in the case reported herein are similar to those of other patients reported previously, providing further evidence of the existence of the "distal 1q trisomy" phenotype.

Studies suggest that E-cadherin is useful to classify epithelial breast lesions as ductal or lobular, but extensive experience with this antibody is lacking. We studied reactivity of lesions with classic and indeterminate morphologic features. We reviewed 95 lesions and divided them into unanimous and nonunanimous diagnosis groups; the unanimous group served as benchmark lesions to which E-cadherin reactivity could be standardized and compared. All 37 ductal lesions in the unanimous group had strong, diffuse E-cadherin reactivity. Two of 22 classic lobular carcinoma in situ (LCIS) lesions had sparse E-cadherin-reactive lobular cells within a few terminal duct lobular units. Neither displayed transition from nonreactive to reactive cells. Of 36 lesions in the nonunanimous group, 19 had insufficient morphologic features for definitive classification. Only 6 of 19 were E-cadherin reactive, including several minimally proliferative lesions. The other 17 lesions in the nonunanimous group had LCIS and ductal carcinoma in situ (DCIS) features. All had no E-cadherin, or strong membrane reactivity of constituent cells in varying proportions, without a transition between reactive and nonreactive cells. Results suggest that the majority of morphologically nondiagnostic atypical lesions are lobular, including those associated with DCIS. E-cadherin seems to be absent in most lobular lesions.

Isolated ischemic necrosis of the cecum is an infrequently described entity. We report three cases seen at our institution within a three-year period. All three patients had been hospitalized for congestive heart failure in the past, but none was in failure at the time of the most recent hospitalization. All three patients presented with clinical and laboratory findings consistent with acute appendicitis. At surgery the cecum was ischemic in each case, while the appendix and the remainder of the intestine appeared normal. There was no evidence of major vascular occlusion or embolization at the time of original operation. We propose that the cecum, like the splenic flexure, is a "watershed area," with poor blood supply relative to that of the adjacent intestine. While cecal ischemia has been described in association with a variety of clinical entities, we propose a newly recognized association with poor myocardial function. In such patients, isolated ischemic necrosis of the cecum should be considered in the differential diagnosis of right lower quadrant pain.

The syndrome of hereditary thrombocytopenia, deafness, and renal disease was manifest in at least eight members in three generations of a family. They had a lifelong history of bleeding, usually as epistaxis, bilateral sensorineural deafness starting in late childhood or the teenage years, and persistent proteinuria with varying degrees of renal dysfunction. Two members died at a young age, one from central nervous system hemorrhage, the other from chronic renal failure. Splenectomy and steroid therapy have been of transient benefit. There was dominant inheritance of the syndrome. Hematologic studies showed thrombocytopenia, large platelets, and megakaryocytic hyperplasia of the bone marrow. In contrast to a previous report, our studies showed that affected members had normal in-vitro platelet function and normal ultrastructural platelet morphology. At autopsy, histologic changes in the kidney of one affected family member were indistinguishable from those reported in classic hereditary nephritis with nerve deafness (Alport's syndrome).