Open AccessVon Recklinghausen's Disease Associated with Somatostatin‐rich Duodenal Carcinoid (Somatostatinoma), Medullary Thyroid Carcinoma and Diffuse Adrenal Medullary Hyperplasia
Author(s) -
Yoshida Aichi,
Hatanaka Shingo,
Ohi Yasuyo,
Umekita Yoshihisa,
Yoshida Hiroki
Publication year - 1991
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1991.tb01629.x
Subject(s) - psammoma body , pathology , medicine , neurofibromatosis , medullary cavity , medullary carcinoma , calcitonin , hyperplasia , thyroid , thyroid carcinoma , multiple endocrine neoplasia , biology , immunohistochemistry , biochemistry , gene
This report describes the concomitant occurrence of a somatostatin‐rich duodenal carcinoid, a medullary thyroid carcinoma and a diffuse adrenal medullary hyperplasia in a patient with von Recklinghausen's disease. A 50‐year‐old Japanese man died from lung metastasis of a malignant schwannoma. In addition to extensive viscero‐cutaneous neurofibromatosis, two different types of neuroendocrine tumors were found in the duodenum and thyroid gland at autopsy. The duodenal tumor, which was located in the second portion, showed the histologic appearance of a carcinoid tumor with glandular differentiation and psammoma‐bodies. Immunohistochemically the tumor cells were intensely positive for somatostatin. The thyroid tumor was composed of nests of tumor cells arranged in an endocrine pattern, and showed immunoreactivity for calcitonin. A review of the literature revealed no previously reported case of concomitant occurrence of duodenal somatostatinoma and medullary thyroid carcinoma in a single patient with von Recklinghausen's disease. Morphometric analysis of adrenal glands disclosed the presence of diffuse medullary hyperplasia. Thus, the present case exhibited a similarity in some respects with multiple endocrine neoplasia (MEN) syndrome, Type Ila or IIb.