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妊娠期双侧嗜铬细胞瘤预示多发性内分泌腺瘤病IIA型:一例报告

Bilateral pheochromocytoma in pregnancy heralding multiple endocrine neoplasia syndrome IIA. A case report.

作者信息

Tewari K S, Steiger R M, Lam M L, Rutgers J K, Berkson R A, DiSaia P J

机构信息

Divisions of Gynecologic Oncology and Maternal-Fetal Medicine and Department of Obstetrics and Gynecology, University of California, Irvine, Medical Center, Orange, 101 The City Drive, Orange, CA 92868, USA.

出版信息

J Reprod Med. 2001 Apr;46(4):385-8.

Abstract

BACKGROUND

Multiple endocrine neoplasia syndrome type IIA (MEN IIA) has rarely been encountered in pregnancy.

CASE

A 22-year-old, nulliparous woman developed bilateral pheochromocytomas during pregnancy. This finding aroused suspicion for MEN IIA, and close endocrinologic follow-up was arranged. Four years later, hyperparathyroidism developed, and the diagnosis was established. The patient underwent prophylactic total thyroidectomy with parathyroid exploration.

CONCLUSION

This was the first case of MEN IIA in pregnancy in which the diagnosis was established prior to the development of medullary thyroid cancer, thereby allowing prophylactic thyroidectomy. The presence of bilateral neoplastic disease in young patients may be indicative of a hereditary predisposition to malignancy.

摘要

背景

妊娠期间很少遇到IIA型多发性内分泌肿瘤综合征(MEN IIA)。

病例

一名22岁未生育女性在妊娠期间发生双侧嗜铬细胞瘤。这一发现引发了对MEN IIA的怀疑,并安排了密切的内分泌随访。四年后,发生甲状旁腺功能亢进,确诊为MEN IIA。患者接受了预防性全甲状腺切除术并探查甲状旁腺。

结论

这是妊娠期间MEN IIA的首例病例,在甲状腺髓样癌发生之前确诊,从而得以进行预防性甲状腺切除术。年轻患者出现双侧肿瘤性疾病可能提示存在恶性肿瘤的遗传易感性。

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