Surgical treatment of colonic ganglioneuromatosis in neurofibromatosis.

Gastrointestinal involvement in neurofibromatosis occurs in three forms: neurofibromatous tumours, visceral vasculopathy and ganglioneuromatosis. A case of colonic ganglioneuromatosis is reported and six similar cases from the literature are reviewed. The clinical and pathological features of this condition are summarized and guidelines for surgical therapy formulated. Subtotal colectomy is recommended for pseudo-obstructive symptoms refractory to medical management.

Multicentric gastric carcinoids complicating pernicious anemia. Origin from the metaplastic endocrine cell population.

We report three cases of multicentric carcinoid tumors of the stomach in patients with long-standing pernicious anemia and severe atrophic gastritis (type A). The tumor nodules arose in nonantral gastric mucosa showing marked intestinal metaplasia. Diffuse endocrine cell hyperplasia was present in both fundus and antrum. Antral G-cell hyperplasia was observed. A widely accepted pathogenesis of this syndrome suggests that the proliferating cell type is the argyrophilic, enterochromaffinlike cell native to the gastric body and fundus. Our findings conflict with this view, in that focal argentaffin staining was also present within tumor cells, as well as immunoreactivity for serotonin and substance P (more characteristic of small-intestinal enterochromaffin or Kulchitsky's cells and small-intestinal carcinoids). Findings in these cases at least suggest an alternative possibility: the tumors may derive from small-intestinal-type metaplastic endocrine cells within the atrophic mucosa, rather than the hypertrophic native endocrine cell population.

The osteoclast-type giant cell tumor of the pancreas.

We present a case report of osteoclast-type giant cell tumor of the pancreas and review the literature concerning this rare neoplasm, the histogenesis of which is uncertain. Electron microscopic features have suggested stromal, histiocytic, and epithelial origins to different investigators. Analysis of the present case supports and epithelial origin, with positive immunocytochemical staining for carcinoembryonic antigen and for low molecular weight keratin in the mononuclear and in some osteoclastlike giant cells. These tumor cells did not stain for mesenchymal markers (lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, S100 protein). Zymogen granules, desmosomes, and zonulae occludentes were identified ultrastructurally and further support an epithelial derivation.

Aspiration biopsy cytology of metastatic endometrial stromal sarcoma and extragenital mixed mesodermal tumor.

Aspiration biopsy from metastatic tumors in two cases of endometrial stromal sarcoma and one case of endometrial adenosarcoma revealed malignant endometrial stromal cells with ill-defined cytoplasm and round or oval hyperchromatic nuclei showing irregular chromatin clumping and conspicuous nucleoli. They were seen mainly in clusters. Aspirate from a metastatic tumor of a mixed mesodermal tumor arising from the omentum showed similar malignant endometrial stroma cells, irregular tight clusters of malignant glandular cells having scanty but well-defined cytoplasm and vesicular nuclei with conspicuous nucleoli, and fragments of atypical smooth muscle tissue. The diagnostic malignant endometrial stromal cells in those reported cases did not display any distinctive cellular features permitting their cytologic identification. They were difficult to differentiate from those of other types of sarcoma. In a clinical setting, with a known primary endometrial stromal sarcoma or mixed mesodermal tumor, however, a cytodiagnosis of its metastases may be suggested when malignant endometrial stromal-cell-like cells are seen in aspirated material, oviating an open-tissue biopsy.