A rare adult case of primary uterine rhabdomyosarcoma with mixed pattern: a clinicopathological & immunohistochemical study with literature review.

IF 2.4 3区医学 Q2 PATHOLOGY

Diagnostic Pathology Pub Date : 2024-07-17 DOI:10.1186/s13000-024-01518-w

Nehal K H Kamel, Eiman Adel Hasby

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引用次数: 0

Abstract

Background: Rhabdomyosarcomas are aggressive tumors that comprise a group of morphologically similar but biologically diverse lesions. Owing to its rarity, Mixed pattern RMS (ARMS and ERMS) constitutes a diagnostic and therapeutic dilemma.

Case: Herein is presented a very rare case of mixed alveolar & embryonal rhabdomyosarcoma in the uterus of a 68-year-old woman. The wall of the uterine corpus & cervix was replaced by multiple whitish-yellow, firm nodules, measuring up to 12 cm. Microscopically, the tumor was predominantly composed of round to polygonal cells arranged in nests with alveolar pattern intermingled with hypo- & hypercellular areas of more primitive cells with scattered multinucleated giant cells seen as well. Extensive sampling failed to show epithelial elements. Immunohistochemical staining showed positive staining for vimentin, desmin, myogenin, CD56 & WT-1. However, no staining was detected for CK, LCA, CD10, ER, SMA, CD99, S100, Cyclin-D1 & Olig-2. Metastatic deposits were found in the peritoneum. The patient received postoperative chemotherapy and radiotherapy but died of systemic metastases 3 months after surgery.

Conclusion: The rarity of this histological tumor entity and its aggressive behavior and poor prognosis grab attention to improving recognition and treatment modalities in adults.

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一例罕见的成人混合型原发性子宫横纹肌肉瘤：临床病理和免疫组化研究及文献综述。

背景：横纹肌肉瘤是一种侵袭性肿瘤，由一组形态相似但生物特性不同的病变组成。由于其罕见性，混合型横纹肌肉瘤（ARMS 和 ERMS）构成了诊断和治疗上的难题。病例：这是一例非常罕见的肺泡型和胚胎型混合型横纹肌肉瘤病例，患者是一名 68 岁的女性。子宫体壁和宫颈被多个白黄色、坚硬的结节取代，结节长达 12 厘米。显微镜下，肿瘤主要由圆形至多角形细胞组成，这些细胞排列成巢状，呈肺泡状，与原始细胞的低细胞区和高细胞区混杂在一起，还能看到散在的多核巨细胞。广泛取样未能发现上皮细胞。免疫组化染色显示，波形蛋白、desmin、肌原蛋白、CD56和WT-1呈阳性染色。但未检测到 CK、LCA、CD10、ER、SMA、CD99、S100、Cyclin-D1 和 Olig-2 染色。腹膜中发现了转移性沉积物。患者术后接受了化疗和放疗，但术后3个月死于全身转移：结论：这种组织学肿瘤的罕见性、侵袭性和不良预后引起了人们对改善成人肿瘤的识别和治疗方式的关注。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Diagnostic Pathology 医学-病理学

CiteScore

4.60

自引率

0.00%

发文量

审稿时长

1 months

期刊介绍： Diagnostic Pathology is an open access, peer-reviewed, online journal that considers research in surgical and clinical pathology, immunology, and biology, with a special focus on cutting-edge approaches in diagnostic pathology and tissue-based therapy. The journal covers all aspects of surgical pathology, including classic diagnostic pathology, prognosis-related diagnosis (tumor stages, prognosis markers, such as MIB-percentage, hormone receptors, etc.), and therapy-related findings. The journal also focuses on the technological aspects of pathology, including molecular biology techniques, morphometry aspects (stereology, DNA analysis, syntactic structure analysis), communication aspects (telecommunication, virtual microscopy, virtual pathology institutions, etc.), and electronic education and quality assurance (for example interactive publication, on-line references with automated updating, etc.).