Gökyer Ali, Sayın Sezin, Küçükarda Ahmet, Çelik Mehmet, Güldiken Sibel, Çiçin İrfan
Trakya University School of Medicine, Department of Internal Medicine, Division of Medical Oncology, Edirne, Turkey.
Trakya University School of Medicine, Edirne, Turkey.
Curr Probl Cancer. 2021 Jun;45(3):100704. doi: 10.1016/j.currproblcancer.2020.100704. Epub 2021 Jan 8.
Sinonasal type hemangiopericytoma is a rare soft tissue tumor. Oncogenic osteomalacia (tumor-induced osteomalacia) is a rare syndrome that develops especially due to benign mesenchymal tumors. Nonspecific general bone pain and weakness delay the diagnosis and treatment of oncogenic osteomalacia, and it is difficult to determine the localization of the primary tumor causing oncogenic osteomalacia. A 43-year-old male patient with nasal hemangiopericytoma with symptoms of oncogenic osteomalacia is presented. The patient had musculoskeletal complaints at first and was diagnosed with lumbar disc herniation and surgery was performed. When his complaints recurred 1 year later, he was re-evaluated and diagnosed with hypophosphatemic osteomalacia. Despite the various treatments he received, his complaints did not decrease but increased, so a detailed examination was decided. When the positive PHEX mutation and very high fibroblast growth factor 23 level were detected, PET-CT imaging was performed with a pre-diagnosis of possible oncogenic osteomalacia, but no finding was found. Then he was evaluated with Ga-68 DOTATATE, and the soft tissue mass filling the right ethmoidal sinus was detected. Due to the relation of the mass with surrounding structures, it was considered unsuitable for total excision and incomplete surgical excision was performed. Pathologic evaluation revealed sinonasal type hemangiopericytoma (glomangiopericytoma). A significant remission in the patient's complaints was observed after the operation. Young patients with osteomalacia with unknown causes should be evaluated for malignancy, and screening and further examinations should be performed.
鼻窦型血管外皮细胞瘤是一种罕见的软组织肿瘤。致癌性骨软化症（肿瘤性骨软化症）是一种罕见的综合征，尤其由良性间充质肿瘤引起。非特异性的全身骨痛和乏力会延误致癌性骨软化症的诊断和治疗，且难以确定导致致癌性骨软化症的原发肿瘤的位置。本文介绍了一名43岁患有鼻窦血管外皮细胞瘤并伴有致癌性骨软化症症状的男性患者。该患者最初有肌肉骨骼方面的主诉，被诊断为腰椎间盘突出症并接受了手术。1年后他的症状复发，再次接受评估后被诊断为低磷性骨软化症。尽管接受了各种治疗，他的症状并未减轻反而加重，因此决定进行详细检查。当检测到PHEX基因突变阳性和成纤维细胞生长因子23水平非常高时，进行了PET-CT成像，初步诊断可能为致癌性骨软化症，但未发现异常。随后对他进行了Ga-68 DOTATATE评估，发现右侧筛窦有软组织肿块。由于肿块与周围结构的关系，认为不适合进行全切，于是进行了不完全手术切除。病理评估显示为鼻窦型血管外皮细胞瘤（血管球性血管外皮细胞瘤）。术后观察到患者的症状有明显缓解。对于病因不明的年轻骨软化症患者，应评估是否存在恶性肿瘤，并进行筛查和进一步检查。
