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甲状旁腺在多发性内分泌腺瘤病中的累及情况

[Parathyroid glands involvement in multiple endocrine neoplasia].

作者信息

Târcoveanu E, Niculescu D, Cotea Elena, Moldovanu R, Vasilescu A, Crumpei Felicia, Zbranca E, Zugun Fl, Rusu V, Ferariu D

机构信息

Clinica I Chirurgie, Facultatea de Medicină, Universitatea de Medicină si Farmacie Gr.T. Popa Iaşi.

出版信息

Rev Med Chir Soc Med Nat Iasi. 2009 Apr-Jun;113(2):482-96.

Abstract

UNLABELLED

Multiple endocrine neoplasias are syndromes characterized by the involvement of at least two endocrine glands. Parathyroid gland involvement is usually noted in Multiple Endocrine Neoplasia (MEN) type I and type II. Parathyroid glands tumor associated with endocrine pancreatic tumor, as well as pituitary tumors is the typical pattern of MEN I. The parathyroid gland is the most frequent abnormality in MEN I.

CASES REPORTS

We presented five cases with MEN I and parathyroid glands involvement. In three cases with young ages (28-33 years old) and familial setting, the MEN I syndrome was "complete" (parathyroid adenoma, gastrinoma or insulinoma and pituitary adenoma--prolactinoma or GH-secreting tumors), and, in the other two cases, with 57 and 68 years old respectively, the MEN I syndrome was "incomplete" with parathyroid glands and pituitary gland involvement. The cases with gastrinomas were operated in emergency for complication of peptic ulcer (perforation associated with peritonitis and gastro-intestinal bleeding); then the pancreatic tumors were diagnosed and left pancreatectomy with spleen preservation and respectively, tumor resection have been performed. Hyperparathyrodism was then diagnosed and subtotal parathyroidectomy has been performed in both cases. Unfortunately one patients died due to severe endocrine disorder. During the necropsy, the pituitary adenoma has been diagnosed. The third case with "complete" MEN was a women of 33 years old, admitted for severe hypoglycemia. The imagistic and laboratory test diagnosed a tumor situated into the pancreatic body, and an parathyroid adenoma. The resection of pancreatic tumor associated with resection of the parathyroid adenoma, in the same time, were performed. The other two cases with "incomplete" MEN were older then the first patients, and were diagnosed with hyperparathyrodism and pituitary gland tumor. The resection of parathyroid gland adenoma has been performed in both cases, with uneventful postoperative course. The literature data was also discussed.

CONCLUSIONS

The parathyroid glands involvement in MEN is common. There are two kinds of MEN associated with parathyroid gland involvement: the "complete" form, especially in young patients, with diffuse involvement of the parathyroid glands, and the subtotal parathyroidy is the best choice, and the "incomplete" form, especially in elderly, with the involvement of a single parathyroid gland; in this way, the resection of the adenoma associated with biopsy from the other parathyroid gland is the best approach. The presence of gastrinomas, complicate the disease prognosis and the surgical approach. The treatment of these patients is challenging and has to be done in multidisciplinary team.

摘要

未标注

多发性内分泌肿瘤是一类以至少两个内分泌腺受累为特征的综合征。甲状旁腺受累常见于I型和II型多发性内分泌肿瘤(MEN)。甲状旁腺肿瘤与内分泌胰腺肿瘤以及垂体肿瘤相关是MEN I型的典型模式。甲状旁腺是MEN I型中最常见的异常部位。

病例报告

我们呈现了5例MEN I型且伴有甲状旁腺受累的病例。3例年龄较轻(28 - 33岁)且有家族史的病例中,MEN I型综合征为“完全型”(甲状旁腺腺瘤、胃泌素瘤或胰岛素瘤以及垂体腺瘤——催乳素瘤或生长激素分泌性肿瘤),另外2例分别为57岁和68岁,MEN I型综合征为“不完全型”,仅累及甲状旁腺和垂体。患有胃泌素瘤的病例因消化性溃疡并发症(穿孔伴腹膜炎和胃肠道出血)进行了急诊手术;随后诊断出胰腺肿瘤并分别进行了保留脾脏的左胰腺切除术和肿瘤切除术。之后诊断出甲状旁腺功能亢进并对两例患者均进行了甲状旁腺次全切除术。不幸的是,1例患者因严重内分泌紊乱死亡。尸检时诊断出垂体腺瘤。第3例“完全型”MEN患者为一名33岁女性,因严重低血糖入院。影像学和实验室检查诊断出胰腺体部有肿瘤以及甲状旁腺腺瘤。同时进行了胰腺肿瘤切除和甲状旁腺腺瘤切除。另外2例“不完全型”MEN患者年龄比首例患者大,诊断为甲状旁腺功能亢进和垂体肿瘤。两例均进行了甲状旁腺腺瘤切除,术后恢复顺利。还讨论了文献数据。

结论

MEN中甲状旁腺受累很常见。有两种与甲状旁腺受累相关的MEN:“完全型”,尤其在年轻患者中,甲状旁腺广泛受累,甲状旁腺次全切除术是最佳选择;“不完全型”,尤其在老年患者中,仅单个甲状旁腺受累;在这种情况下,切除腺瘤并对其他甲状旁腺进行活检是最佳方法。胃泌素瘤的存在使疾病预后和手术方式复杂化。这些患者的治疗具有挑战性,必须由多学科团队进行。

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