Arikan Soykan, Tatar Cihad, Emre Nayci Ali, Ersoz Feyzullah, Baki Dogan Mehmet, Gunver Feray
Department of General Surgery, Istanbul Training and Research Hospital, Istanbul, Turkey.
Department of Pathology, Istanbul Training and Research Hospital, Istanbul, Turkey.
North Clin Istanb. 2021 Dec 29;8(6):629-633. doi: 10.14744/nci.2020.37431. eCollection 2021.
A 54-year-old male was admitted to our department with neurofibromatosis and hypertension. During his examination, a mass was detected in the abdomen, and he was transferred to a surgical clinic. At the first examination of the patient, extensive café-au-lait spots and granulomas were detected on the body and the mass occupying right abdomen quadrant was palpable. The patient's medical history indicated that he had hypertension for almost a decade. The patient also stated that nodules on the body existed from his earliest recollection and he had relatives with neurofibromatosis. The patient was taken to a surgical operation. A mass with 30×23 cm in size was removed. The area of the nodular structure, with 0.5 cm in diameter, in the stomach serosa was also removed. The tumor was composed of phaeochromocytoma in the larger spaces and ganglioneuromas in the relatively narrow spaces. The nodular area removed in gastric serosa was reported as a very low-risk gastrointestinal stromal tumor. Apart from this rare combination, adrenal mass removed from the patient was considerably larger than the masses in the literature until now. Therefore, we aimed to present this rare case with a literature background.
一名54岁男性因神经纤维瘤病和高血压入住我科。在检查过程中，发现其腹部有一个肿块，遂转至外科门诊。在对患者的首次检查中，发现其身体上有广泛的咖啡牛奶斑和肉芽肿，且可触及占据右腹象限的肿块。患者病史显示其患有高血压近十年。患者还表示，自记事起身上就有结节，且他有患神经纤维瘤病的亲属。患者接受了手术。切除了一个大小为30×23厘米的肿块。胃浆膜层直径0.5厘米的结节状结构区域也被切除。肿瘤在较大区域由嗜铬细胞瘤组成，在相对狭窄区域由神经节神经瘤组成。胃浆膜层切除的结节区域被报告为极低风险的胃肠道间质瘤。除了这种罕见的组合外，从该患者身上切除的肾上腺肿块比迄今为止文献中报道的肿块要大得多。因此，我们旨在结合文献背景介绍这一罕见病例。
