Bargellini T, Cantelli G, Bruscino A, Falchetti A, Giusti F, Brandi M L, Valeri A
Azienda Osperdaliero-Universitaria Carregi, Firenze.
G Chir. 2011 Mar;32(3):132-4.
Multiple endocrine neoplasia syndromes (MEN) are genetic disease with many pathologic models. MEN 2B is a autosomal dominant syndrome characterized by medullary thyroid carcinoma, pheochromocytoma, mucosal ganglioneuromatosis and marfanoid habitus. Laparoscopic surgery is the gold standard for the treatment of pheochromocytoma.
Woman 25 years old, suffering from medullary thyroid carcinoma in MEN 2B syndrome, underwent total thyroidectomy, with emptying of the lymphonodal central and lateral cervical (bilaterally) compartments and radioreceptor therapy. The patient was then submitted to four plastic surgery for cervical keloid. In January 2010, follow-up blood and instrumental tests show, in the lower portion of left adrenal gland, a round lesion, with smooth margins 17 mm diameter, attributable to pheochromocytoma. In March 2010 the patient underwent laparoscopic surgery for left adrenalectomy.
The benefits of laparoscopic adrenalectomy for a single lesion are well documented in the literature; this type of intervention is indicated in cases of pheochromocytoma in patients with MEN 2B. Our case demonstrates the feasibility of this technique.
多发性内分泌肿瘤综合征(MEN)是具有多种病理模式的遗传性疾病。MEN 2B是一种常染色体显性综合征,其特征为甲状腺髓样癌、嗜铬细胞瘤、黏膜神经节瘤病和类马凡氏体型。腹腔镜手术是嗜铬细胞瘤治疗的金标准。
一名25岁女性,患有MEN 2B综合征的甲状腺髓样癌,接受了甲状腺全切术,双侧颈部中央和外侧淋巴结清扫以及放射性受体治疗。该患者随后因颈部瘢痕疙瘩接受了四次整形手术。2010年1月,随访的血液和器械检查显示,左肾上腺下部有一个圆形病变,边界光滑,直径17毫米,归因于嗜铬细胞瘤。2010年3月,该患者接受了腹腔镜下左肾上腺切除术。
腹腔镜肾上腺切除术治疗单一病变的益处已在文献中得到充分证明;这种干预方式适用于MEN 2B患者的嗜铬细胞瘤病例。我们的病例证明了该技术的可行性。