Department of Disease Control and Homeostasis, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan.
Endocr J. 2012;59(9):791-6. doi: 10.1507/endocrj.ej11-0324. Epub 2012 May 31.
A 67-year-old woman with familial clustering of thyroid papillary adenocarcinoma was diagnosed with acromegaly due to pituitary macroadenoma. She had multiple skin vegetations, but had no parathyroid and pancreas diseases. Before transsphenoidal surgery, she was further diagnosed as having a duodenal tumor and multiple hypervascular liver nodules. Biopsy specimens from the duodenal tumor and liver nodules were diagnosed histologically as moderately differentiated adenocarcinoma. Immunohistochemically, the tumor cells were positive for chromogranin, synaptophysin and somatostatin receptor 2a, suggestive for neuroendocrine features. After surgery, the patient was not in biochemical remission, and octreotide treatment was initiated. The duodenal cancer was treated with chemotherapy (neoadjuvant cisplatin and S-1). After 24 months, the patient's insulin-like growth factor I level had been normalized, and her liver tumors had not progressed macroscopically. This is a rare case of acromegaly associated with multiple endocrine tumors, not being categorized as conventional multiple endocrine neoplasia. Octreotide treatment might have had beneficial effects on our patient's duodenal adenocarcinoma and liver metastases, both directly via SSTR2a and indirectly via GH suppression, thereby contributing to their slow progression.
一位 67 岁的女性因垂体大腺瘤而被诊断为肢端肥大症，她有家族性甲状腺乳头状腺癌聚集。她有多处皮肤赘生物，但没有甲状旁腺和胰腺疾病。在经蝶窦手术前，她进一步被诊断为十二指肠肿瘤和多个富血管性肝结节。十二指肠肿瘤和肝结节的活检标本组织学诊断为中分化腺癌。免疫组化染色显示肿瘤细胞对嗜铬粒蛋白、突触素和生长抑素受体 2a 呈阳性，提示具有神经内分泌特征。手术后，患者未达到生化缓解，开始使用奥曲肽治疗。对十二指肠癌采用化疗（新辅助顺铂和 S-1）进行治疗。24 个月后，患者的胰岛素样生长因子 I 水平已恢复正常，其肝肿瘤在宏观上没有进展。这是一例罕见的肢端肥大症伴多发性内分泌肿瘤的病例，不符合常规多发性内分泌肿瘤的分类。奥曲肽治疗可能对我们患者的十二指肠腺癌和肝转移有有益的影响，其直接通过 SSTR2a，间接通过 GH 抑制，从而有助于它们的缓慢进展。
