Valenzuela Membrives M, Cassini Gómez de Cádiz L, Cueto Ladròn de Guevara A, García Pacheco M
San Cecilio Klinikum, Sektion Pneumologie, Granada, Spanien.
Pneumologie. 2013 Jun;67(6):340-3. doi: 10.1055/s-0033-1344079. Epub 2013 Jun 7.
The Doege-Potter Syndrome is known as a combination of intrathoracic tumor associated to symptomatic hypoglycemia. Solitary Fibrous Tumor is a mesenchymal neoplasm. Initially, it was described in the pleura, however, it starts out in any organ or tissue at the expense of a stromal CD34+ cell. Most of the patients are asymptomatic at the time of diagnosis. Symptoms such as cough, dyspnea, chest pain, and hypoglycemia due to production of IGF-2 have been reported (Doegge- Potter Syndrome). Others described paraneoplastic profiles are hypertrophic osteoarthropathy and in some cases galactorrhea. We report on a case of a 77 year old patient with solitary fibrous tumor in the pleura as well as symptomatic hypoglycemia. The primary treatment is surgical resection, and a longtime monitoring is necessary because recurrences can occur even after a long period of remission.
多伊格-波特综合征是指胸腔内肿瘤合并症状性低血糖。孤立性纤维瘤是一种间叶性肿瘤。最初它被描述于胸膜，但它起源于任何器官或组织，由基质CD34+细胞发展而来。大多数患者在诊断时无症状。已报告的症状包括咳嗽、呼吸困难、胸痛以及因IGF-2产生导致的低血糖（多伊格-波特综合征）。其他描述的副肿瘤表现为肥大性骨关节病，在某些情况下还有溢乳。我们报告一例77岁患者，患有胸膜孤立性纤维瘤以及症状性低血糖。主要治疗方法是手术切除，并且需要长期监测，因为即使在长时间缓解后仍可能复发。
