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Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2019 Aug 07;54(8):615-617
Authors: Huang PY, Li YZ, Yang XM, Yu BQ, Guo L, Guo ZT, Li SS
PMID: 31434378 [PubMed - indexed for MEDLINE]
The patient, female, 42 years old, was admitted to the endocrinology department of our hospital on March 17, 2017 for "bone pain with height decline for 11 years and intermittent nosebleed for 2 years". The patient began to develop progressive bone pain in 2005, and there was no obvious relief for the treatment of anti-inflammatory and analgesic, accompanied by progressive decline in height. In 2011, we examined alkaline phosphatase 407 U/L in our hospital. The fluctuation of blood phosphorus was 0.4~0.6 mmol/L. The bone scan (Fig. 1) and bone density test results showed that the bone density decreased, and the diagnosis was "low-phosphorus rickets." ". After oral administration of phosphorus and calcium supplementation, the whole body bone pain can be temporarily relieved, but the drug treatment is not effective. The patient still has bone pain and height decline (161 cm in 2005, 150 cm in 2011, 138 cm in 2017). . In 2015, patients developed intermittent nosebleeds, about 5 ml each time, with bright red color, and oppression can stop bleeding. The initial frequency is 4-5 times/year, and the frequency gradually increases, gradually becoming once a week. Admission physical examination: the spine is slightly curved on the right side, the X-legs of both lower limbs, and the muscle strength of both lower limbs are 2~3. Dark red new creatures can be seen in the right middle nasal passage, with bloody adhesions and surface mucosal erosion. Blood biochemistry suggests a decrease in blood phosphorus (0.33 mmol/L), an increase in urinary phosphorus (52.82 mmol/d) at 24 h, an increase in alkaline phosphatase (203.7 U/L), and a decrease in 25-hydroxyvitamin D (18 nmol/L) ), an increase in parathyroid hormone (17.06 pmol / L). Sinus CT showed a long elliptical soft tissue shadow in the right middle nasal sinus and ethmoid sinus. The larger cross-sectional area was about 2.7 cm × 1.4 cm, and the enhanced scan showed uneven enhancement (Fig. 2). Initial diagnosis: the nature of the nasal mass to be determined; low phosphate osteomalacia. Later, she was transferred to our hospital for otolaryngology and head and neck surgery. On April 5, 2017, general anesthesia was performed under nasal endoscopic surgery for right nasal cavity and sinus tumor. Intraoperative endoscopic surgery, see the dark red mass in the right middle nasal passage, the surface is not smooth, the bleeding is easy to touch (Figure 3), after the sieving, the frontal sinus, the tumor is mostly located in the frontal sinus, the nasal septum is right Both the lateral and anterior ethmoids have destruction defects. Excision of the nasal cavity, anterior sinus and frontal sinus tumors showed dural exposure, clear fluid pulsating outflow, and reconstruction of the skull base with nasal septum mucosa after hemostasis. Tumor biopsy showed that there were more round and fusiform cell infiltration in the mucosa, some of the cells were mildly shaped, and the tumor had no capsule, involving fat tissue. Immunohistochemistry: CK(-), EMA(-), S100(-), HMB45(-), Melan-A(-), Desmin(-), CD56(+), Syn(-), CgA(-) , LCA(-), CD3(-), CD20(-), CD79a(-), SMA(-), Myogenin(-), MyoD1(-), Bcl-2(+), TiA-1(-), CD43(-), EBER(-), Ki-67(10%+), F8(-), GFAP(-), NSE(±), CD99 (focality+), HCAL(-), CD34(+) . Pathological diagnosis: low-grade malignant hemangiopericytoma (Fig. 4). The blood phosphorus level returned to normal (0.88 mmol/L) at 5 days after operation, and the symptoms of lower extremity bone pain were relieved and discharged. Final diagnosis: nasal aneurysm; tumor-induced osteomalacia (TIO). After 6 months of telephone follow-up, the patient had no nosebleeds, bone pain, and blood phosphorus levels were stable in the normal range (0.88-0.96 mmol/L).
患者女，42岁，因"骨痛伴身高进行性下降11年，间断性鼻出血2年"于2017年3月17日入住我院内分泌科。患者2005年开始出现进行性骨痛，予消炎止痛等治疗无明显缓解，伴身高进行性下降。2011年于我院检查碱性磷酸酶407 U/L，血磷波动在0.4~0.6 mmol/L，骨扫描（图1）、骨密度检查结果均提示骨密度减低，诊断为"低磷软骨病"。予以口服补磷、补钙后，全身骨痛可暂时缓解，但药物治疗效果不佳，患者仍有全身骨痛及身高进行性下降（2005年161 cm，2011年150 cm，2017年138 cm）。2015年患者出现间断性鼻出血，每次约5 ml，色鲜红，压迫可止血，初期4~5次/年，后频率逐渐增多，逐渐变为每周1次。入院体格检查：脊柱稍右侧弯，双下肢X型腿，双下肢肌力2~3级。右侧中鼻道可见暗红色新生物，有血痂附着，表面黏膜糜烂。血生化提示血磷降低（0.33 mmol/L），24 h尿磷升高（52.82 mmol/d），碱性磷酸酶升高（203.7 U/L），25-羟基维生素D降低（18 nmol/L），甲状旁腺激素升高（17.06 pmol/L）。鼻窦CT示右侧中鼻道及筛窦内长椭圆形软组织影，较大横截面积约2.7 cm×1.4 cm，增强扫描显示不均匀强化（图2）。初步诊断：鼻腔肿物性质待定；低磷骨软化症。后转入我院耳鼻咽喉头颈外科，于2017年4月5日全身麻醉下行鼻内镜下右侧鼻腔鼻窦肿物切除术。术中鼻内镜下见右侧中鼻道内暗红色肿物，表面欠光滑，触之易出血（图3），后至前筛，上达额窦，瘤体大部分位于额窦，鼻中隔右侧和前筛骨质均有破坏缺损。切除鼻腔、前筛及额窦内肿瘤，可见硬脑膜暴露，有清亮液体搏动性流出，止血后以鼻中隔黏膜瓣重建颅底。肿块送活检示：镜下可见黏膜组织内较多圆形和梭形细胞浸润，部分细胞有轻度异型，肿瘤无包膜，累及脂肪组织。免疫组织化学：CK（-），EMA（-），S100（-），HMB45（-），Melan-A（-），Desmin（-），CD56（+），Syn（-），CgA（-），LCA（-），CD3（-），CD20（-），CD79a（-），SMA（-），Myogenin（-），MyoD1（-），Bcl-2（+），TiA-1（-），CD43（-），EBER（-），Ki-67（10%+），F8（-），GFAP（-），NSE（±），CD99（灶性+），HCAL（-），CD34（+）。病理诊断：低度恶性血管外皮细胞瘤（图4）。术后5 d患者血磷水平恢复正常（0.88 mmol/L），下肢骨痛症状减轻后出院。最终诊断：鼻腔血管外皮瘤；肿瘤诱导性骨软化症（tumour-induced osteomalacia，TIO）。电话随访6个月，患者无鼻出血、骨痛，血磷水平稳定在正常范围（0.88~0.96 mmol/L）。
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