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罕见部位的蓝色细胞瘤:腹膜后尤因肉瘤

Blue Cell Tumour at Unusual Site: Retropritoneal Ewings Sarcoma.

作者信息

Javalgi Anita P, Karigoudar Mahesh H, Palur Katyayani

机构信息

Assistant Professor, Department of Pathology, Shri B M Patil Medical College , Sholapur Road Bijapur, Karnataka, India .

Professor, Department of Pathology, Shri B M Patil Medical College , Sholapur Road Bijapur, Karnataka, India .

出版信息

J Clin Diagn Res. 2016 Apr;10(4):ED19-20. doi: 10.7860/JCDR/2016/18302.7618. Epub 2016 Apr 1.

Abstract

Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations. Haematological profile, renal function test and liver function test were in normal limits. USG abdomen was normal, MRI showed a mass in pelvis retroperitoneum measuring 10x10cms, bilateral ovaries and tubes were normal. Because of retroperitoneal nature of tumor and suspicion of uterine sarcoma, laparotomy was performed. The large retroperitoneal mass adherent to posterior of uterus was excised and send for histopathological diagnosis. On gross and microscopy examination the diagnosis of blue cell tumor with PAS positivity, possibility of extraskeletal Ewing's sarcoma/primitive neuro-ectodermal tumor was made which was further confirmed by immunohistochemistry, positive for S100, Vementin and CD99 and negative for desmin and CK. Confirmed diagnosis help in accurate management and improves survival rate.

摘要

尤因肉瘤是一种起源于骨或非骨的高度恶性肿瘤,如果起源于软组织则称为骨外尤因肉瘤。它是一种罕见的肿瘤,最常见于椎旁区域、胸壁、头颈部和腹膜后。报告一例39岁女性腹膜后尤因肉瘤的有趣病例。患者自2个月前开始出现腹部不适和隐痛,随后出现下肢无力和体重减轻。经过详细的病史询问和检查后,她被进一步转诊进行详细的病理和放射学检查。血液学检查、肾功能检查和肝功能检查均在正常范围内。腹部超声检查正常,磁共振成像显示腹膜后盆腔有一个10×10厘米的肿块,双侧卵巢和输卵管正常。由于肿瘤位于腹膜后且怀疑为子宫肉瘤,遂进行了剖腹手术。切除了附着于子宫后部的巨大腹膜后肿块,并送去进行组织病理学诊断。经过大体和显微镜检查,诊断为具有PAS阳性的蓝细胞瘤,可能为骨外尤因肉瘤/原始神经外胚层肿瘤,免疫组织化学进一步证实,S100、波形蛋白和CD99呈阳性,结蛋白和细胞角蛋白呈阴性。确诊有助于准确治疗并提高生存率。

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