Department of Otolaryngology-Head and Neck Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.
Otolaryngology Institute of Shanghai Jiao Tong University, Shanghai, China.
Otolaryngol Head Neck Surg. 2020 May;162(5):674-682. doi: 10.1177/0194599820904055. Epub 2020 Mar 3.
To describe our clinical experience with surgical treatments for sinonasal phosphaturic mesenchymal tumors diagnosed at our institution.
Retrospective case series.
Affiliated Sixth People's Hospital, Shanghai Jiao Tong University.
We retrospectively reviewed the medical records of 10 patients diagnosed with phosphaturic mesenchymal tumors associated with tumor-induced osteomalacia between December 2014 and October 2019.
There were 4 men and 6 women with a disease course of 1 to 19 years. All patients exhibited hypophosphatemia and tumor-induced osteomalacia. The tumor was located in the sinonasal region, frontal bone, and temporal bone in 8 patients, 1 patient, and 1 patient, respectively. Technetium-99m octreotide scintigraphy was used for tumor localization in 4 cases. Six patients underwent endoscopic resection; the remaining 4 underwent unilateral transorbital anterior and posterior ethmoid artery ligation + endoscopic resection, endoscopic resection + skull base repair, internal carotid artery stenting + transcatheter arterial embolization + temporal bone tumor excision + adipose tissue plugging, and endoscopic resection + transfrontal craniotomy (n = 1 each). Two patients had a history of incomplete endoscopic resection. All patients achieved clinical remission and normalized biochemical indices after surgery. Only 1 patient developed recurrence and died of a brain hernia.
A diagnosis of sinonasal phosphaturic mesenchymal tumors should be based on a combination of clinical, imaging, and pathological findings. Technetium-99m octreotide scintigraphy helps in locating the tumor. Complete surgical excision guarantees clinical remission, and preoperative transcatheter arterial embolization or feeding artery ligation may reduce intraoperative bleeding in cases of highly vascularized tumors.
描述我们在本机构诊断的鼻窦磷酸盐尿性间充质肿瘤的手术治疗经验。
回顾性病例系列。
上海交通大学附属第六人民医院。
我们回顾性地审查了 2014 年 12 月至 2019 年 10 月期间诊断为与肿瘤诱导性骨软化症相关的磷酸盐尿性间充质肿瘤的 10 例患者的病历。
患者 4 男 6 女，病程 1 至 19 年。所有患者均表现为低磷血症和肿瘤诱导性骨软化症。肿瘤位于 8 例患者的鼻窦区、额骨和颞骨，1 例患者位于眼眶内，1 例患者位于颞骨。4 例患者行锝-99m 奥曲肽闪烁扫描定位肿瘤。6 例患者行内镜切除术；其余 4 例患者行单侧眶额前后动脉结扎+内镜切除术、内镜切除术+颅底修复、颈内动脉支架置入+经导管动脉栓塞+颞骨肿瘤切除+脂肪组织填塞、内镜切除术+经额颅切除术（各 1 例）。2 例患者有内镜切除不完全史。所有患者术后临床缓解，生化指标恢复正常。仅 1 例患者复发并死于脑疝。
鼻窦磷酸盐尿性间充质肿瘤的诊断应结合临床、影像学和病理学表现。锝-99m 奥曲肽闪烁扫描有助于定位肿瘤。完全手术切除可保证临床缓解，术前经导管动脉栓塞或供血动脉结扎可能减少高度血管化肿瘤术中出血。
