De Los Santos-Aguilar Ramón G, Chávez-Villa Mariana, Contreras Alan G, García-Herrera Juan S, Gamboa-Domínguez Armando, Vargas-Sánchez Joel, Almeda-Valdes Paloma, Reza-Albarrán Alfredo A, Iñiguez-Ariza Nicole M
Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Department of Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
J Endocr Soc. 2019 Jan 8;3(3):537-543. doi: 10.1210/js.2018-00281. eCollection 2019 Mar 1.
Doege-Potter syndrome with acromegaloid facial changes is extremely rare. Uncooked cornstarch along with glucocorticoids have been used as supportive care in patients with non-islet cell tumor hypoglycemia (NICTH). Preoperative embolization of hepatic solitary fibrous tumors (SFT) with NICTH has yielded unsatisfactory results. Herein we present the case of a 61-year-old man with a 3-month history of severe frequent hypoglycemic episodes and acromegaloid facial changes. During a spontaneous hypoglycemia (26 mg/dL), laboratory values showed a hypoinsulinemic pattern with low levels of GH, IGFPB3, and an IGF2/IGF1 ratio of 8.5:1. Cross-sectional imaging revealed a large (16 × 13 × 11 cm) hepatic tumor, and cytology was consistent with SFT. A preoperative right portal embolization was performed in an effort to induce normal remnant liver hypertrophy to allow for safe tumor resection. After the procedure, uncooked starch treatment followed by prednisone was started, achieving complete remission of hypoglycemic episodes in the preoperative setting. He subsequently underwent partial hepatectomy. The histologic diagnosis was compatible with a potentially malignant SFT. The patient had an excellent outcome with complete remission of hypoglycemia, improvement of facial acromegaloid changes, and no further evidence of disease. To our knowledge, this is the first case of a patient with Doege-Potter syndrome with acromegaloid facial changes induced by a potentially malignant liver SFT, treated successfully with a multimodal approach consisting of uncooked cornstarch, low-dose prednisone, preoperative embolization, and complete surgical resection. The use of cornstarch and low-dose glucocorticoids may be an adequate treatment in advance of undergoing surgery.
伴有肢端肥大样面部改变的多伊格-波特综合征极为罕见。生玉米淀粉与糖皮质激素已被用作非胰岛细胞瘤低血糖症（NICTH）患者的支持性治疗。对伴有NICTH的肝脏孤立性纤维瘤（SFT）进行术前栓塞治疗效果不佳。在此，我们报告一例61岁男性患者，有3个月严重频繁低血糖发作及肢端肥大样面部改变的病史。在一次自发性低血糖发作（血糖水平为26mg/dL）期间，实验室检查结果显示为低胰岛素血症模式，生长激素（GH）、胰岛素样生长因子结合蛋白3（IGFPB3）水平较低，胰岛素样生长因子2（IGF2）与胰岛素样生长因子1（IGF1）的比值为8.5:1。横断面成像显示肝脏有一个大的肿瘤（16×13×11cm），细胞学检查结果与SFT一致。为促使剩余肝脏正常肥大以确保安全切除肿瘤，进行了术前右门静脉栓塞。术后，开始采用生淀粉治疗，随后使用泼尼松，术前低血糖发作完全缓解。患者随后接受了部分肝切除术。组织学诊断符合潜在恶性的SFT。患者预后良好，低血糖完全缓解，面部肢端肥大样改变有所改善，且无疾病复发迹象。据我们所知，这是首例由潜在恶性肝脏SFT引起的伴有肢端肥大样面部改变的多伊格-波特综合征患者，采用生玉米淀粉、低剂量泼尼松、术前栓塞及完整手术切除的多模式方法成功治疗。在手术前使用玉米淀粉和低剂量糖皮质激素可能是一种合适的治疗方法。
