多发性平滑肌肉瘤病变引起的肠系膜在一个年轻的女性:一个不寻常的罕见病例介绍

K. Akhtar, Aprajita Sinha, S. Usman, Zohra Naheed
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摘要

理由:原发性肠系膜平滑肌肉瘤是一种非常罕见的发现。它主要出现在70年代,在年轻人中非常罕见。细针抽吸细胞学检查是软组织病变的初步诊断程序,随后是切除活检。患者关注点:在此,我们报告了一例罕见的原发性肠系膜多发平滑肌肉瘤病例,患者为19岁女性,自过去4个月以来出现腹部肿块和疼痛。诊断:腹部CT扫描显示肝下间隙和膈下区域有多个大型多房肿块,似乎来自肠系膜。子宫和宫颈正常。该肿块的细针抽吸细胞学检查显示,有细长的、钝端的纺锤形细胞核的纺锤状细胞束与分散的细胞或剥脱的细胞核混合。一些细胞表现出多形性,偶尔有核内液泡。大体上,切除的标本是多个结节,棕色,软到硬,最大的尺寸为8.5x8x2.5厘米,最小的尺寸为2.2x2.2x1厘米。在显微镜检查中,切片显示非典型纺锤体细胞的束状模式,其雪茄状细胞核显示多形性,嗜酸性纤维细胞质具有1-2个有丝分裂图/10HPF。SMA免疫组化显示肿瘤细胞弥漫性强胞浆阳性,结蛋白显示轻度局灶性胞浆阳性。诊断为原发性多发性肠系膜平滑肌肉瘤。干预措施:对肿块进行了广泛的手术切除。患者随后接受了6个周期的5-氟尿嘧啶化疗。结果:经过2个月的随访,她恢复得很好。经验教训:我们的病例将拓宽医生和病理学家的视野,让他们考虑年轻女性原发性肠系膜平滑肌肉瘤的可能性,这种肉瘤表现为不涉及女性生殖道,从而指导对此类病变的正确诊断和管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Multiple leiomyosarcomatous lesions arising from the mesentery in a young female: An unusual rare case presentation
Rationale: Primary mesenteric leiomyosarcoma is a very rare finding. It is seen predominantly in the seventh decade and is very rare in the young. Fine needle aspiration cytology is the initial diagnostic procedure in soft tissue lesions, followed by excision biopsy. Patient Concerns: Herein, we present a rare case of primary multiple mesenteric leiomyosarcoma in a 19 year old female, who presented with abdominal lump and pain since the last 4 months. Diagnosis: CT scan of abdomen showed multiple large multiloculated masses in the sub-hepatic space and sub-diaphragmatic region, which seemed to be arising from the mesentery. The uterus and cervix were normal. Fine needle aspiration cytology of the mass revealed fascicles of spindled cells with elongated, blunt ended fusiform nuclei admixed with dispersed cells or stripped nuclei. Some cells showed pleomorphism with occasional intranuclear vacuoles. Grossly the resected specimen was multiple nodular, brown, soft to firm, with the largest measuring 8.5x8x2.5 cm and the smallest 2.2x2.2x1 cm in size. On microscopic examination, sections showed fascicular pattern of atypical spindle cells having cigar shaped nuclei showing pleomorphism and eosinophilic fibrillary cytoplasm with 1-2 mitotic figures/10HPF. Immunohistochemistry with SMA showed strong diffuse cytoplasmic positivity and desmin showed mild focal cytoplasmic positivity in the tumor cells. A diagnosis consistent with primary multiple mesenteric leiomyosarcoma was given. Interventions: Wide surgical excision of the mass lesion was performed. The patient was subsequently administered 6 cycles of 5- Fluorouracil based chemotherapy. Outcomes: She is recovering well after 2 month of follow up period. Lessons: Our case will widen the vision of both physicians and pathologists to consider the possibility of primary mesenteric leiomyosarcoma in young females, presenting without any involvement of female genital tract, thus guide in the proper diagnosis and management of such lesions.
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