股骨近端平滑肌肉瘤1例并文献复习

目的探讨骨平滑肌肉瘤的临床表现、诊断和治疗。方法对1例骨原发性平滑肌肉瘤进行临床和病理资料观察。复习有关文献,就骨原发性平滑肌肉瘤发病情况、组织来源、病理、诊断、鉴别诊断、治疗和预后进行了讨论。结果骨原发性平滑肌肉瘤好发于股骨远端和胫骨近端,尤其是膝关节附近。临床最常见症状是疼痛,可伴有关节活动受限。X线显示长骨干骺端不规则低密度影。组织学、免疫组织化学标记表达具有特征。结论骨原发性平滑肌肉瘤比较少见,诊断主要靠免疫组化。宜采取手术治疗,可辅以放疗或化疗。     

原发性胫骨平滑肌肉瘤一例并文献复习

原发性骨平滑肌肉瘤（primary leiomyosarcoma of bone, PLB）是一种罕见的骨的原发性恶性梭形细胞肿瘤。结合 PLB的影像学特征、组织学诊断及免疫组化结果分析,可明确该病的诊断及鉴别诊断。
　　病例资料患者,女,24岁,右小腿疼痛7个月余,行走时疼痛加剧,于1个月前在当地诊所抗炎止痛治疗后疼痛缓解。最近3天,右小腿疼痛突然加剧就诊,体查右小腿中段压痛,局部皮温稍有升高,皮肤无红肿表现。     

原发性卵巢平滑肌肉瘤3例报告并文献复习

目的 分析原发性卵巢平滑肌肉瘤的病因、组织学特性、临床特点、治疗方法及预后.方法 回顾性分析解放军总医院收治的3例原发性卵巢平滑肌肉瘤的诊疗经过,并结合近10年来国内外的文献资料,总结原发性卵巢平滑肌肉瘤的诊治经验.结果 本组3例原发性卵巢平滑肌肉瘤均发生于>50岁的女性,发病原因不明确,临床表现无特异性,病情进展快....     

股骨原发性平滑肌肉瘤一例

患者 女,47岁.无明显诱因出现左膝部疼痛半年,逐渐加重伴膝关节活动障碍,行走受限就诊.专科检查:左膝部轻度肿胀,皮温及肤色正常,皮肤无破溃,无静脉曲张,左膝部外侧压痛明显,关节活动受限.实验室检查:血沉70 mm/h,碱性磷酸酶正常.     

股骨原发性平滑肌肉瘤1例

患者女,27岁。无明显诱因出现右膝部疼痛半年,活动稍受限,休息时明显,夜间加重。专科检查：右膝部略肿胀,皮肤无发红．皮温较健侧略高．股骨远端压痛,膝关节活动稍受限。实验室检查：肿瘤标志物、碱性磷酸酶、     

原发性前列腺滑膜肉瘤1例并文献复习

1 病例资料 患者 男,28岁.于3月前无明显诱因出现肛周疼痛,大便变细,10余天前,小便次数增多,血清前列腺特异抗原(PSA)0.05 ng/mL. MRI检查示:前列腺区不规则团块状等T1长T2信号影(图1),内可见低信号分隔,DWI扩散受限呈明显高信号,ADC图呈低信号,边界欠清,累及直肠前壁、膀胱后壁.注射Gd-DTPA后,病灶呈明显不均匀强化(图2),直肠前壁明显增厚(图3).CT检查:前列腺区不规则软组织密度团块(图4),边界不清,增强呈不均匀明显强化(图5).患者行手术治疗,术中可见盆腔巨大肿块,与膀胱壁、直肠、精囊关系密切.     

原发性肝脏癌肉瘤1例并文献复习

目的分析总结肝脏原发癌肉瘤的影像学及组织学特征,提高对本病的认识和诊断水平。方法报道1例经病理证实的原发性肝脏癌肉瘤并复习相关文献,探讨原发性肝脏癌肉瘤临床、病理及影像学特征和有关鉴别诊断。结果CT平扫多表现为肝右叶圆形或椭圆形,无包膜,低密度或等密度或固体成分的囊性改变;增强扫描显示为巨大的混杂密度影,伴有不同程度的坏死或不规则囊变;边缘及实质部分明显强化,其内可见强化的乳头状结节。结论原发性肝脏癌肉瘤是由癌样成分和肉瘤样成分紧密混杂在一起的肝脏罕见恶性肿瘤,好发于肝右叶,肿瘤具有一定的影像学特征,但其确诊仍依赖病理诊断。     

静脉内平滑肌瘤病2例并文献复习

目的 探讨静脉内平滑肌瘤病（IVL）的影像特征。方法 回顾性分析2例经手术病理证实的IVL的临床表现及影像特征,并复习相关文献。结果 MRI平扫显示子宫及附件区不规则肿块,T₁WI呈低信号,T₂WI呈稍高信号,子宫周围多发迂曲血管影;扩散加权成像（DWI）上病变扩散受限呈不均匀高信号;增强MRI显示病变明显强化,受累静脉不同程度增宽且强化欠均匀。对比增强磁共振血管成像显示盆腔多发不规则团片状明显不均匀强化灶,受累静脉管腔增宽,内见多发条索状充盈缺损。超声检查显示子宫角不均质回声,部分呈融合状,1例病灶向上与静脉血管相连,病灶内可见丰富的血流信号;另1例病灶内有少量血流信号。结论 IVL影像表现具有一定特征,盆腔肿块伴邻近脉管增宽、管腔内充盈缺损是本病常见的影像征象。     

股骨平滑肌肉瘤一例

患者男,41岁。于5d前骑自行车时感觉左大腿中下段疼痛，无力，为阵发性隐痛。体检：左大腿下段无红肿现象，有轻压痛，生化检查无异常。     

原发性下腔静脉平滑肌肉瘤的CT与MRI表现分析(附2例报告并文献复习)

原发性下腔静脉平滑肌肉瘤（primaryleiomyosarcoma of the inferior vena cava，PIVCI。MS）是一种少见的恶性肿瘤，起源于静脉壁平滑肌。自1871年Perl首先报道，文献报道不足300例，且多数为个案报道。国内亦仅有少数报道，郑伟等。报道1986～2006年中国人民解放军总医院共收治14例为国内最多报道。笔者分析2例经病理证实的PIVCLMS的CT及MRI检查结果，旨在提高对本病的诊断与鉴别诊断水平。     

Primary leiomyosarcoma of the sigmoid colon: Case report and review of literature

Leiomyosarcomas of the sigmoid colon are aggressive and extremely rare tumors representing less than 0.1% of all colorectal malignancies. Few cases have been reported in the literature and their imaging features need to be more detailed. We report the case of a 70 year old male patient, smoker, with a history of arterial hypertension, admitted for abdominal pain and hypogastric mass. Computed tomography has shown a voluminous heterogeneously enhancing tumor process with a necrotic center, attached to the sigmoid wall, with multiple secondary peritoneal and hepatic masses. The anatomopathological examination of the biopsy sample with immunohistochemistry allowed the diagnosis of leiomyosarcoma of the sigmoid colon. Clinical presentation and radiological features of leiomyosarcomas are non-specific, and the definitive diagnosis is only established after an anatomopathological examination.     

Primary intracranial myxopapillary ependymomas: report of two cases and review of the literature

Myxopapillary ependymoma is a variant of ependymoma occurring almost exclusively in the conus medullaris or filum terminale. Myxopapillary ependymoma found primarily in the brain is extremely rare. Two such cases appearing at the 4th ventricle and cerebral falx are reported. The imaging features of such tumors are a primary cystic mass with strong enhancement at its solid part. Myxopapillary ependymoma should be a possible differential diagnosis when an intracranial cystic tumor is found.     

原发性甲状旁腺功能亢进合并多发棕色瘤2例报告并文献复习

棕色瘤亦称巨细胞瘤样变,系由原发性甲状旁腺功能亢进症(简称甲旁亢)引起的局限性纤维囊性骨炎,表现为单发或多发的边界清楚的溶骨性病变.临床上易误诊,本文报道2例原发性甲旁亢伴棕色瘤,以提高对本病的诊断及鉴别诊断水平.     

Apert综合征2例报告及文献复习

例1 患儿,男,6岁,因“双手足各指(趾)粘连6年”入院.患儿出生后,即发现双手足2～5指(趾)粘连.随着患儿生长发育,渐发现颜面部发育异常,头颅扁头,突眼,眼距宽,下睑睫毛呈内翻状,眼球常红肿、溢泪.入院查体:患儿身材较同龄儿童矮小,身高110 cm,体重19 kg,智力较低下.心、肺、腹部未及明显异常.专科情况:头颅扁平,前额宽而广,头颅横径16 cm,前后径15 cm.双眼眼球外凸,内眦间距约3.5 cm,内眦高于外眦约1 cm.平视时,双上眼睑缘下垂覆盖至瞳孔中央水平线,双侧下眼睑睫毛呈内翻状,眼球红肿、溢泪.患儿呈张口状,流涎,可见腭裂(图1).头颅X线片示:头颅扁平,呈舟状,脑回压迹多且深,颅缝早期闭合,上颌发育不全,低位前牙,牙列较拥挤(图2).双手足2～5指(趾)完全粘连,甲融合(图3,4).     

Primary leiomyosarcoma of bone: report of 4 cases

Primary leiomyosarcoma of bone is a very rare tumor. Four cases are reported: 3 tumors were located in the femur and 1 in the talus. Clinical and imaging findings including radiographs, CT, and MRI are described. The final diagnosis was made by histopathological, immunohistochemical, and/or ultrastructural study after biopsy.     

Robinow syndrome: report of two cases and review of the literature

We report two patients with Robinow syndrome, review the published literature and stress the importance and limitations of radiographic examination in the diagnosis of this disorder, which shows extreme clinical and radiographic variability. The radiographic differential diagnosis of Robinow syndrome is discussed.     

Rhino-orbito-cerebral mucormycosis: report of two cases and review of the literature

Rhino-orbito-cerebral mucormycosis (ROCM) is a rare, fulminant opportunistic fungal infection that is mostly seen in immunocompromised or diabetic patients. The disease should be recognised and treated immediately. We present here MR imaging findings of two patients with histopathologically proven ROCM. One of the cases had a history of corticosteroid treatment and iatrogenic diabetes mellitus and although amphotericin B was started immediately, the disease progressed and surgical debridement was necessary. The second case was a patient with diabetes mellitus type 1 in whom ROCM had occurred following an abdominal surgery; amphotericin B treatment alone was adequate in this patient.     

Posterior mediastinal epithelioid leiomyosarcoma: Case report and literature review

Epithelioid type leiomyosarcoma is rarely encountered outside of the abdomen or uterus. We present a case of posterior mediastinal leiomyosarcoma in a 45-year-old male with back pain and bilateral lower extremity weakness. Magnetic Resonance Imaging of the thoracic spine revealed a heterogeneous posterior mediastinal soft tissue mass infiltrating the vertebral body and epidural space with resultant spinal cord compression and edema. Positron Emission Tomography showed no evidence of distant metastatic spread. Histopathological characterization revealed epithelial type leiomyosarcoma. Despite multiple subtotal resections, radiotherapy, and salvage chemotherapy with successful restoration of the patient''s neurological function, the tumor burden remained significant. The patient was subsequently lost to follow up and the clinical outcome remains unknown. To our knowledge this is the first reported case of epithelioid type posterior mediastinal leiomyosarcoma presenting with spinal cord compression and edema.