嗜铬细胞瘤合并多发性胰岛细胞癌。

Pheochromocytoma with multiple islet cell carcinoma.

作者信息

Tamasawa N, Terada A, Kodama T, Ishigame M, Ishimaru K, Hishida R, Satoh T, Takebe K, Sasaki M, Imamura K

机构信息

Third Department of Internal Medicine, Hirosaki University School of Medicine, Aomori, Japan.

出版信息

Presse Med. 1994;23(1):32-4.

PMID:7907420

Abstract

We reported a rare case of a 17-year-old female with pheochromocytoma associated with multiple islet cell carcinoma. Pheochromocytoma was identified in the right adrenal gland. Multiple pancreas tumours were demonstrated unpredictably in the diagnostic imaging of the pheochromocytoma. No other endocrinological neoplasm was observed in the pituitary, thyroid and parathyroid gland. The patient underwent right adrenalectomy and total pancreatectomy. Pheochromocytoma was benign, however, pancreas tumours were non-functioning islet cell tumours and histologically malignant. This combination is assumed to represent a mixed form of multiple endocrine neoplasia (MEN) 1 and MEN 2.

摘要

我们报告了一例罕见病例，一名17岁女性患有嗜铬细胞瘤并伴有多发胰岛细胞瘤。嗜铬细胞瘤位于右侧肾上腺。在嗜铬细胞瘤的诊断性影像学检查中意外发现了多个胰腺肿瘤。在垂体、甲状腺和甲状旁腺未观察到其他内分泌肿瘤。患者接受了右侧肾上腺切除术和全胰腺切除术。嗜铬细胞瘤为良性，但胰腺肿瘤为无功能胰岛细胞瘤，组织学上为恶性。这种组合被认为代表了多发性内分泌肿瘤（MEN）1型和MEN 2型的混合形式。