K. A. Warfel

Familial islet cell tumors in von Hippel‐Lindau's disease

Von Hippel‐Lindaus Disease is an hereditary disorder characterized by the development of hemangioblastomas of the cerebellum and retina and a variety of cystic and neoplastic lesions of other organs such as renal cell carcinoma and pheochromocytoma. In a single generation of a family with Von Hippel‐Lindaus disease, all four siblings developed lesions classically associated with the complex. Additionally, two of the four developed islet cell tumors of the pancreas, one in one patient and five in the other. While a familial incidence of islet cell tumors is known in multiple endocrine adenomatosis, type I and Zollinger‐Ellison syndrome, such a familial occurrence has been heretofore unrecorded in the Von Hippel‐Lindau complex.

Glycogen-rich Clear Cell Carcinomas of the Breast: A Clinicopathologic and Ultrastructural Study

Ten cases of glycogen-rich clear cell carcinoma of the breast are described. Only two previous case reports have been published. These neoplasms are composed of clear cells with abundant glycogen. Ultrastructurally, two cases showed large quantities of non-membrane-bound glycogen and numerous empty glycogen lakes. Neoplastic cells formed tight junctions, immature desmosomes, and occasionally had short microvilli. In nine cases the glycogen-rich carcinoma grew in a solid pattern only, while one case had both solid and papillary patterns. One case was associated with a signet-ring cell carcinoma. Seven of nine patients who underwent axillary dissections had nodal metastases. Five patients died with residual disease, and one is currently alive with local skin recurrence. These data suggest that glycogen-rich clear cell carcinoma is associated with frequent lymph node metastases and mortality.

A combined morphologic and immunologic approach to the diagnosis of gastrointestinal lymphomas. I. Malignant lymphoma of the stomach (a clinicopathologic study of 22 cases)

Twenty‐two cases of primary gastric non‐Hodgkins lymphoma, which occurred over a 20‐year period, were reviewed. Eighteen tumors were studied using an immunoperoxidase method, and the presence of intracytoplasmic monoclonal immunoglobulin (Ig) in nine (50%) suggested a B‐cell origin. Four tumors (22%) contained intracytoplasmic muramidase (lysozyme), suggesting a true histiocytic origin. Five tumors (28%) did not contain immunoglobulin or muramidase. The muramidase‐positive “true histiocytic lymphoma” could not be differentiated from histiocytic lymphoma of lymphocytic origin using light microscopic examination alone. The patients with B‐cell lymphoma survived significantly longer than patients in the other two groups. The differentiation between true histiocytic lymphoma and other conditions is discussed.

Malignant sex cord-stromal tumor of testis.

We report a case of sex cord-stromal tumor in the testis, which metastasized to the retroperitoneal space, resulting in death 13 months after orchiectomy. The primary lesion consisted of granulosa cells, theca cells, Sertoli cells and undifferentiated gonadal stroma. Biopsy of the retroperitoneal metastasis showed predominance of the Sertoli cell component.

Clear Cell Carcinoma of the Larynx A Variant of Mucoepidermoid Carcinoma

A case of clear cell carcinoma of the larynx is presented. By light microscopy the lesion was composed mainly of sheets of clear cells, containing intracytoplasmic mucin and glycogen. Glandular lumina, tonofilaments, and desmosome-tonofilament complexes were observed on electron microscopy. The presence of these features established that this unusual neoplasm was a clear cell variant of mucoepidermoid carcinoma.

Mucinous Breast Carcinomas with Abundant Intracytoplasmic Much and Neuroendocrine Features: Light Microscopic, Immunohistochemical, and Ultrastructural Study

Eight mucinous carcinomas of the breast were studied by light microscopy and immunohistochemistry; one was studied by electron microscopy. All 8 cases had abundant, relatively clear cytoplasm that contained mucin. Cells were argyrophil positive and argentaffin negative. Eight cases were positive for neuron specific enolase (NSE), 5 cases for serotonin, 1 case for serotonin and somatostatin and 2 cases for serotonin, somatostatin, and gastrin. None had clinical evidence of abnormal neuroendocrine function. Three patients had axillary lymph node metastases. Only 1 of 5 patients in whom there was clinical followup died of her disease. Electron microscopy of one case showed abundant intracytoplasmic and extracellular mucin, round and pleomorphic dense-core granules, numerous cell processes, and aggregates of intermediate filaments. These cases expand the histologic spectrum of breast carcinomas which may show neuroendocrine differentiation.

Ultrastructure of Abnormal Collagen in Human Tumors

Abnormal collagen fibrils were identified by transmission electron microscopy in 22 human tumors of differing histogenesis. They were found in a review of 1400 electron microscopy cases. Abnormal collagen fibrils, described by others as amianthoid fibers, composite collagen, collagen flowers and intrafibrillar collagen dysplasia, have been found only rarely in human tumors but commonly in certain connective tissue diseases such as pseudoxanthoma elasticum, Ehlers-Danlos syndromes, Marfans syndrome, osteoarthritic cartilage, and emphysematous lung among others. Abnormalities in the cases described here included thickened fibrils, fibrillar degeneration of fibrils and irregular external contours. Proposed mechanisms for their formation have included degeneration possibly due to hypoxia or collagenase activity, abnormal collagen biosynthesis, and abnormal tissue levels of glycosaminoglycans. The finding of abnormal collagen fibrils in these 22 human tumors shows that their occurrence is more common than is indicated by previous published reports. Most of the tumors containing abnormal collagen fibrils were mesenchymal or soft tissue tumors. Four neuroendocrine neoplasms had abnormal collagen fibrils.

Villous papilloma of the gallbladder in association with leukodystrophy

A 2-year-old girl with metachromatic leukodystrophy had an unusual papilloma of the gallbladder. The association of metachromatic leukodystrophy and gallbladder disease in children is recognized. The striking anemone-like configuration of this tumor is remarkably unlike those of other papillomas described in the literature.

Hepatoblastomas: An Ultrastructural and Immunohistochemical Study

Seven hepatoblastomas were studied by electron microscopy, and four of these were studied by immunohistochemistry. Five tumors were purely epithelial, and two were mixed epithelial-mesenchymal. They showed a spectrum of cellular differentiation ranging from primitive epithelial cells to differentiated cells resembling adult hepatocytes. Glycogen, lipid, basal lamina, and canaliculi were present in all cases. Mitochondria with large, membrane-bound, amorphous inclusions were present in one tumor, and large, complex, basal cell processes were present in two tumors. Ultrastructural features most characteristic of hepatocytes were most common in fetal type hepatoblastomas. Immunoreactive chromogranin cells were present in two tumors, one of which also contained immunoreactive somatostatin cells. The somatostatin-positive tumor had cells with granules resembling those seen in somatostatin-containing cells of normal pancreas and somatostatin-containing neuroendocrine carcinomas. Other immunoreactive substances were present, including alpha 1-antitrypsin (four cases), vimentin (embryonal cells in four cases; fetal cells in three cases), low-molecular weight cytokeratin (embryonal cells in three cases; fetal cells in four cases), and high-molecular weight cytokeratin (embryonal cells in one case; fetal cells in two cases). Osteoidlike material was positive for epithelial membrane antigen, vimentin, and S-100 protein.

Urachal Cystadenoma with Abundant Glycogen: Ultrastructural Study

A cystadenoma arose in the urachus of a 32-year-old man. The mass was a multilocular cystic tumor filled with light yellow mucoid material. It was located in the anterior abdominal wall between the umbilicus and the dome of the urinary bladder. Overall, the mass was 14 cm x 8 cm x 5 cm, with the largest internal cyst being 6 cm in diameter. The lesion was characterized histologically by columnar cells with focal cellular and nuclear stratification. Ultrastructural examination revealed columnar cells with abundant glycogen, moderately pleomorphic microvilli without prominent filamentous cores, and apical mucin vacuoles. Nuclei had pleomorphic contours. An intact basal lamina was present. A similar urachal cystadenoma has not been described.