11例瘤源性骨软化症的临床诊治分析

目的分析瘤源性骨软化症（tumor—induced osteomalacia,TIO）的临床特征、诊断及治疗规律,提高对TIO的识别及早期诊治能力。方法我院2010年4月-2013年4月收治11例确诊TIO患者,分析其手术前、后临床症状及血生化变化情况。结果男性5例,女性6例;年龄19—54岁,平均37．7岁。发病到确诊的时间2—15年,平均5．5年。11例均有骨痛和肌无力症状,血磷低而尿磷高,血钙正常,碱性磷酸酶（alkaline phosphatase,ALP）升高。3例发现皮下软组织包块。8例行奥曲肽扫描（99m Tc—OCT）均提示有生长抑素受体高表达病变。11例手术治疗后均获得病理确诊,术后10例血磷恢复正常,所有患者骨痛及肌无力症状明显缓解。结论TIO临床症状典型,99m Tc—OCT及其他影像学检查有助于定位肿瘤。手术切除病灶后血磷可恢复正常,症状改善明显。     

胸腺类癌导致肿瘤性低磷软骨病一例

肿瘤性低磷软骨病(tumor-induced osteomalacia，TIO)是以低磷血症、尿磷排泄增多、低1，25(OH)2D3水平、骨骼矿化障碍为特点的罕见代谢性骨病。TIO多由良性间质肿瘤引起，尚未见胸腺类癌导致的报道，本例为首次报道。     

肿瘤性低磷骨软化症一例报道并文献复习

肿瘤性低磷骨软化症（TIO）是临床上一种罕见的伴瘤综合征,由肿瘤导致肾脏排磷增加,造成的获得性低血磷骨软化症。TIO起病隐匿,临床误诊率较高,但仍具有典型的临床特点,如肌无力、进行性骨痛、身高变矮等。临床中应提高对该病的认识,早期诊断,明确肿瘤位置,手术切除一般可达到较好的治疗效果。对于疑似TIO患者,仔细的体格检查,并结合实验室检查、影像学检查等手段对肿瘤的发现和定位具有重要意义。本文回顾性报道了被诊断为TIO且得到成功诊治的患者1例,并对相关文献进行了复习,以提高临床医生对本病的认识,为临床工作提供借鉴。     

低磷软骨病的临床观察

低磷软骨病是一种发生在成人中的新形成骨基质(类骨质或骨样组织)不能正常完成矿化的代谢性骨病,由于骨样组织钙化不足和骨硬度不足,易弯曲变形。患者早期表现为腰腿疼痛和肌无力,严重时行走困难,容易发生病理性骨折,后期表现为胸廓畸形,脊柱侧弯、前凸等畸形,心肺功能严重受影响。顽固性低磷软骨病中存在肿瘤相关     

肿瘤性骨软化症的诊断及治疗

肿瘤性骨软化症(tumor induced osteomalacia,TIO)是一种由肿瘤引起肾脏排磷增加造成的获得性低血磷性骨软化症,临床表现为肌无力、骨痛,严重者出现骨骼畸形、骨折、活动障碍,显著影响生活质量,切除肿瘤后,病情可以获得明显缓解.TIO肿瘤常来源于间叶组织的良性肿瘤,多位于骨或软组织内,位置隐匿,生长缓慢,不易被发现,造成诊断困难.本研究主要探讨TIO的临床特点、早期诊断及治疗.     

肿瘤相关性低磷骨软化病13例临床分析

目的分析肿瘤相关性低磷骨软化症(T1O)患者的临床特点,以提高对本病的认识。方法分析13例TIO患者的临床资料和实验室及影像学检查特点。结果 13例患者均符合骨软化的临床诊断。均成年起病;低血磷(血磷<0.65 mmol/L时仍有尿磷排出),尿磷水平高,碱性磷酸酶升高,甲状旁腺激素(PTH)正常。除3例体表发现皮下结节,行结节彩色超声明确结节性质及血流情况,予直接切除后病理活检证实,余10例均行奥曲肽扫描提示生长抑素受体高表达,明确肿瘤体内位置后,再行CT引导下穿刺活检行病理检查以明确诊断。结论 TIO有比较典型的临床特点,明确TIO肿瘤的位置是本病治疗的前提和关键。奥曲肽显像、超声及CT对本病的发现和定位具有一定价值。     

低磷骨软化症11例分析

目的分析11例低磷骨软化症患者的临床表现、实验室检查及影像学表现,以提高对低磷性骨软化症的临床诊断水平。方法回顾性分析2012年6月—2013年5月解放军总医院内分泌科住院低磷骨软化症患者11例临床资料,其中男8例,女3例,年龄20⁶4岁,平均(40.5±15.0)岁,病程164岁,平均(40.5±15.0)岁,病程1¹0年,平均(3.6±2.6)年。结果双足跟疼痛3例,双下肢乏力3例,全身疼痛2例,腰部疼痛1例,髋关节疼痛1例。血磷均降低,血钾降低3例,正常8例,24h尿钾增多5例,甲状旁腺素升高8例,3例正常,1,25二羟维生素D_3均降低。X线检查均示长骨及扁骨普遍脱钙,骨密度减低,骨皮质变薄,骨小梁稀疏模糊。诱发疾病为:右股部肿瘤1例,左股骨内侧踝肿瘤1例,右股骨头肿瘤1例,甲状旁腺亢进1例,干燥综合征1例,肿瘤相关骨软化症可疑6例。结论低磷骨软化病患者起病隐匿,易误诊,以骨痛或乏力起病,进展迅速,血生化提示低磷血症,X线提示骨骼改变,应注意肿瘤性骨软化症.需行奥曲肽扫描,并结合CT或MR定位,手术切除后症状缓解。     

低磷性骨软化症一例报道

低磷性骨软化症（hypophosphatemic osteomalacia,HO）是由于低血磷、维生素D缺乏所致的,以骨骼矿化不良、骨软化或佝偻病为主要特征的一组疾病.临床分为X连锁显性遗传性低血磷性骨软化症（X-linked hypophosphatemia,XLH）、常染色体显性遗传性低血磷性骨软化症（autosomal dominant hypophosphatemic rickets,ADHR）、肿瘤相关性低血磷性骨软化症（tumor induced osteomalacia,TIO）[1].现将重庆医科大学附属第一医院内分泌科收治的1例低磷性骨软化症报道如下.     

磷酸盐尿性间叶组织肿瘤临床与影像学特点及文献复习

目的:总结磷酸盐尿性间叶组织肿瘤(PMT)的临床与影像学特点并进行文献复习。方法:回顾分析2014年1月～2019年1月中日友好医院病理科诊断为PMT患者的临床、病理及影像学资料,进行相关文献的复习。结果:4例患者中男3例、女1例,平均年龄为47.2岁。患者均具有不同程度的乏力、骨痛、关节痛等临床表现,血磷减低,平均值为0.5mmol/L(正常值为0.81～1.78mmol/L)。病变部位:1例位于上肢,1例位于下肢,2例位于头颈部。影像学表现:CT表现为软组织密度结节或肿块,平均CT值为47Hu,病变较小时边界清楚,病变较大时可合并骨质破坏;MRI表现为T1WI等及低信号,T2WI脂肪抑制图像不均匀高信号,内部可见低信号区,增强扫描病变中度到明显强化,较大病变内部强化欠均匀。结论:PMT发病率低,其临床及影像学具有一定的特点,掌握这些特点有助于临床早期诊断和治疗。     

骨盆尤文氏肉瘤/外周型原始神经外胚层肿瘤影像征象与临床病理对照分析

目的 探讨骨盆尤文氏肉瘤/外周型原始神经外胚层肿瘤(ES/pPNET)的影像学表现,并与临床及病理对照,进一步加深对该病的认识.方法 回顾性分析经病理证实的8例骨盆原发性ES/pPNET临床、病理及影像学资料.所有病例均行常规X检查,其中4例同时行CT检查,7例行MR检查.影像学观察指标包括:发病部位、数目、骨质破坏、骨膜反应及瘤骨、软组织肿块、强化方式等.病理学观察指标包括形态学和免疫组织化学.结果 7例表现为溶骨性骨质破坏;1例表现以骨硬化为主要改变;7例见与骨质破坏不呈比例的巨大的软组织肿物,中央区多发坏死囊变;最大径大于8cm的肿瘤6例;4例跨关节浸润.MRI显示软组织肿物内可见T2WI低信号分隔影,增强扫描部分强化.6例出现转移,其中3例为肺转移.免疫组化8例患者CD99均为阳性表达.结论 原发于骨盆的ES/PNET具有一定特点,但缺乏特异性,确诊仍需组织病理学检查.对于青少年患者,结合影像学上溶骨性骨质破坏并明显软组织肿块,需考虑骨盆ES/PNET可能.     

磷酸盐尿性间叶瘤继发低磷骨软化症1例

This case report concerns a 34-year-old woman who had been diagnosed with ankylosing spondylitis (AS), fibromyalgia syndrome (FMS), osteoarthritis (OA), lumbar disc herniation and the like in different hospitals during the past 18 months. She had progressive osteoarthrosis, significant muscle weakness, gait abnormalities in weight-bearing areas, however without typical inflammatory low back pain, while the treatment with non-steroidal anti-inflammatory drugs (NSAIDs) was invalid, with normal inflammation index, negative results for rheumatic factor (RF) and human leukocyte antigen (HLA)-B27, and normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). She had hyphosphatemia, normal serum calcium, 1, 25-(OH)2-D3 reduction, elevated alkaline phosphatase (ALP) and normal parathyroid hormone (PTH), however with elevated urinary phosphorus. Finally, the medial thigh nodule was found in the subcutaneous of her inner leg by careful examination and imaging scans including B-ultrasound and PET/CT. The final pathology confirmed that the nodule was phosphate urinary mesenchymal tumors. After the tumor was removed, the patient was treated with anti-osteoporosis and phosphorus supplementation. The symptoms of bone pain and muscle weakness were alleviated, and hypophosphatemia was corrected. It was confirmed that the patient had low-phosphorus osteomalacia due to tumor. Tumor-induced hypophosphatemia osteomalacia (TIO) was a rare paraneoplastic syndrome which was caused by excessive phosphorus excretion induced by the tumor, and was thus categorized as an acquired hypophosphatemic osteomalacia. TIO had an occult onset and was associated with a high rate of misdiagnosis, although TIO has some typical clinical features. Early diagnosis, correctly positioning of the tumor, and surgical resection can achieve good outcomes.     

Clinical features and detection of osteomalacia in the elderly

Fifteen elderly patients with osteomalacia were investigated in 2½ years in a geriatric unit. Although they all had bone pain the diagnosis in some cases was overshadowed by other common diseases with which they were referred to hospital, such as impaired balance or mental confusion. The bone pain had been present for weeks or months and was often attributed to arthritis. Biochemical investigations showed a low serum calcium and low or low-normal serum phosphate levels and a moderate to large increase in alkaline phosphatase above 15 KA units/100 ml. Only six patients had radiological signs of osteomalacia but all had histological evidence on bone biopsy.     

Osteoid osteoma of a scapula: a case report in a 34 years old woman

Osteoid osteoma is a benign bone tumor and accounts for 10% of benign tumors. Almost any bones can be involved but half of cases involving femur or tibia. Osteoid osteoma is a rare tumor of the scapula with only 18 reported cases in the literature. We presented a case of osteoid osteoma of the scapula in right shoulder in angle of coracoid and geloneid in a 34- year old woman. She had right radicular shoulder pain increased at night for 4 years. An important point about this case is that, patient was treated in long-term with miss diagnosis of cervicospinal discopathy. The key of diagnosis in this patient was paying attention to the nature of night increasing pain and performing bone scan. After the operative curettage of the tumor, the pain dramatically stopped and a few weeks of physical therapy led to full range of motion in her shoulder.     

十二指肠神经内分泌癌8例临床分析

目的探讨十二指肠神经内分泌癌（duodenal neuroendocrine carcinoma,DNEC）的临床病理学特征与外科治疗效果。方法回顾性分析2009-2012年于我院治疗的8例DNEC的临床病理学特征与外科治疗效果并复习文献。结果 8例DNEC中,男性5例,女性3例;发病年龄28~80岁,平均年龄53岁;主要临床表现为皮肤巩膜黄染5例,上腹及腰背部疼痛5例,黑便1例,1例无症状;肿瘤位于十二指肠降段4例,乳头3例,水平段1例;腹部影像呈多样性,CT可呈十二指肠乳头或相应部位肿物。4例行胰十二指肠切除术,2例行内窥镜逆行胆总管造影并胆管支架置入术（ERCP）,2例行胃肠吻合并超声引导经皮经肝胆道造影置管引流术（PTCD）;术后随访3~40个月,4例行根治性切除患者均长期存活,4例行姑息性治疗患者均已死亡。结论十二指肠神经内分泌癌呈浸润性生长,应积极手术治疗以改善患者预后。     

Primary leiomyoma of the ovary--a report of 2 resected cases

Leiomyoma of the ovary is relatively rare and its origin is still controversial. Here, we report 2 cases of ovarian leiomyoma. Case 1, a 59-year-old woman who complained of abdominal distention. Ultrasonography and computed tomography revealed a large solid mass in the lower abdomen. The mass was diagnosed as uterine myoma preoperatively, but it turned out to be a left ovarian tumor at laparotomy. The tumor was about an adult-head size, grayish, elastic hard, and solid. In case 2, a fist-sized mass was accidentally detected in the right ovary of a 84-year-old woman during laparotomy for rectal cancer. Histologically both tumors were composed of solid proliferation of short spindle-shaped cells, that formed interlacing fascicles and showed varying degrees of hyalinization. Both tumors were diagnosed as leiomyoma. Although this tumor is rather infrequent, it is necessary to consider leiomyoma for differential diagnosis, when a solid tumor is detected in the ovary.     

以转移灶为首发临床表现的肾癌患者临床特征分析

目的分析以转移灶为首发临床表现肾癌患者的临床特征。方法回顾性分析了32例以转移灶为首发临床表现肾癌患者的临床资料,男性24例,女性8例,中位年龄56岁。结果患者以骨转移灶(下肢痛4例、胸痛3例、背痛3例、枕部结节3例、胸壁肿物2例、肩疼1例、髂区痛1例、下肢麻木1例)、肺转移灶(咳嗽7例、咳血2例、体检发现肺占位2例)、脑转移灶(头痛1例、头晕1例)及淋巴结转移灶(颈部肿物1例)的症状和体征为首发临床表现。多发转移27例、单发转移5例。透明细胞癌31例、乳头状癌1例。肾肿瘤中位直径7.5cm。有肾原发灶病理资料的28例患者中,T1期7例、T2期12例、T3a期9例,G12例、G211例、G312例、G43例,11例伴脉管瘤栓。全身治疗为干扰素-α+白细胞介素-Ⅱ(24例)、索拉非尼(4例)及舒尼替尼(4例)。28例行减瘤肾切除术,11例行转移灶姑息放疗或切除。本组30例获随访,23例死亡,患者的中位生存期10个月(4个月～7年)。2例单发骨转移的患者,经全身治疗联合减瘤肾切除及转移灶切除或放疗,分别无瘤生存7年及19个月。结论以转移灶为首发临床表现的肾癌患者,骨转移造成的临床表现最为常见。患者多为多发转移、伴不良病理预后因素,预后差、生存期短。对孤立骨转移的患者,全身治疗辅以原发灶及转移灶的姑息治疗,部分患者可能长期生存。     

外周型原始神经外胚层肿瘤的影像学表现

目的探讨外周型原始神经外胚层肿瘤（p PNET）的影像学表现,以提高对该病的进一步认识。方法回顾性分析4例经病理证实的p PNET的X线平片、CT及MRI表现,其中4例均行X线平片检查,3例行CT检查,2例行MRI检查。结果胸壁软组织2例,髂骨1例,股骨1例。发生在软组织的2例p PNET,X线及CT表现为分界不清的等或略低密度软组织肿块,破坏相邻骨质;MRI表现为软组织内边界不清的肿块,其内信号不均匀,以等或稍长T1、等或稍长T2信号为主,明显不均匀强化,邻近骨质破坏。发生在骨的2例p PNET,X线及CT表现为溶骨性骨质破坏,周围伴有较大的软组织肿块;MRI在T1WI为等或低信号,T2WI为等或高信号,不均匀强化。结论 p PNET有一定的影像学特点,X线平片、CT与MRI检查有助于术前了解病变的性质、范围等,CT和MRI能很好地显示肿瘤的内部结构、有无侵犯毗邻组织器官或远处转移,具有重要的临床应用价值。     

外周性原始神经外胚层肿瘤的影像学表现

目的：探讨外周性原始神经外胚层肿瘤（pPNET）的临床和影像学表现，以提高对该病的进一步认识。方法：分析6例经手术病理证实pPNET的临床和X线、CT及MRI表现。结果：6例中，男2例，女4例，发生于胸壁2例，纵隔、肾脏、脊柱、肩背部各1例。临床以局部疼痛（4例）伴肿块（3例）为主诉，年龄（15～39）岁，X线检查4例，均见软组织肿块，伴溶骨性骨质破坏3例。CT扫描5例，软组织肿块边缘模糊4例，边界清楚l例，3例伴溶骨性骨质破坏，均未见骨膜反应。MRI扫描2例，在T1WI病变为中等信号，T2WI为高信号。结论：pPNET影像学检查以软组织肿块，常伴溶骨性骨质破坏为主要表现，缺乏特征性，但有助于了解病变范围、良恶性鉴别、远处转移和评价治疗效果。