Surgical approach of synchronous medullary thyroid carcinoma and pheochromocytoma in MEN 2 syndrome.

Henry Ford Hospital medical journal Pub Date : 1992-01-01
G F Scheumann, H Dralle
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Abstract

In cases with concurrent medullary thyroid carcinoma (MTC) and pheochromocytoma, discussion regarding a one-stage versus two-stage treatment strategy approach remains open. From 1975 to 1990, 11 of 25 multiple endocrine neoplasia type 2 (MEN 2) patients presented with biendocrinopathies or triendocrinopathies synchronously. All patients were treated surgically and followed subsequently in our hospital. Of the group of nine patients with concurrent MTC and pheochromocytoma, five were treated in one-stage and four in two-stage procedures. No patient had major complications intraoperatively. For the two-stage group, the total hospital stay (preoperatively and postoperatively) averaged 35 days. For the one-stage group, the total hospital stay averaged 25 days. In patients with increased operative risks (patients with higher age and impaired physical condition or if neck surgery includes transsternal cervicomediastinal lymphadenectomy), two-stage procedures should be selected. However, in young patients with the MEN 2 syndrome or syndromes with small tumors detected by family screening, thyroidectomy, cervical lymphadenectomy, and adrenalectomy may be performed in a one-stage procedure without increasing surgically related morbidity.

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甲状腺髓样癌伴发嗜铬细胞瘤的手术治疗。
对于并发甲状腺髓样癌(MTC)和嗜铬细胞瘤的病例,关于一期与两期治疗策略方法的讨论仍然开放。1975 ~ 1990年,25例多发性内分泌瘤2型(MEN 2)患者中有11例同时出现双内分泌或三内分泌病变。所有患者均在我院接受手术治疗并随访。在9名同时患有MTC和嗜铬细胞瘤的患者中,5名患者接受一期治疗,4名患者接受两期治疗。术中无重大并发症发生。对于两阶段组,总住院时间(术前和术后)平均为35天。一期组的总住院时间平均为25天。对于手术风险增加的患者(年龄较大、身体状况受损的患者或颈部手术包括经胸骨颈纵隔淋巴结切除术的患者),应选择两期手术。然而,在患有MEN 2综合征的年轻患者或经家庭筛查发现有小肿瘤的综合征患者中,甲状腺切除术、宫颈淋巴结切除术和肾上腺切除术可以在一期手术中进行,而不会增加手术相关的发病率。
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Extracellular Ca2+ sensing in C-cells and parathyroid cells. Proceedings of the Urban Health Care Symposium II. June 2-4, 1991. Characterization of the clinical features of five families with hereditary primary cutaneous lichen amyloidosis and multiple endocrine neoplasia type 2. Unusual features of multiple endocrine neoplasia. Long-term follow-up in four large MEN 2 families in The Netherlands.
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