Uncommon but intriguing: A pediatric primary histiocytic sarcoma case—Clinical insights and literature review

Q4 Medicine

Radiology Case Reports Pub Date : 2025-05-15 DOI:10.1016/j.radcr.2025.04.063

Sofia Elouaouch MD , Soufiane Berhili MD , Hanane Mansouri MD , Zahira El Youssi MD , Ahmed Bensghier MD , Mohammed Moukhlissi MD , Karim Bahhous PhD , Loubna Mezouar MD

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Abstract

Histiocytic sarcoma (HS) is a rare and aggressive malignancy derived from histiocytes, accounting for less than 1% of hematopoietic neoplasms. Pediatric soft tissue involvement is exceptionally rare, with limited data to guide management. We present the case of a 5-year-old girl with a painless mass on the right thigh. Imaging revealed a malignant intra-aponeurotic soft tissue lesion. Histopathological analysis confirmed HS with immunohistochemical positivity for CD68, CD31, LCA, and INI1, and a Ki-67 index of 40%. Staging PET-CT identified local lymph node involvement. Following R1 surgical resection, the patient received adjuvant radiotherapy (43 Gy to the tumor bed and 36.9 Gy to the inguinal region) and OEPAbased chemotherapy. At 20 months post-treatment, the patient remains in remission with excellent functional outcomes. This case highlights the rarity of soft tissue histiocytic sarcoma in pediatric patients and the importance of multimodal treatment to achieve long-term remission.

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罕见但耐人寻味：一例小儿原发性组织细胞肉瘤病例——临床观察及文献复习

组织细胞肉瘤（HS）是一种来自组织细胞的罕见的侵袭性恶性肿瘤，占造血肿瘤的不到1%。小儿软组织受累极为罕见，指导治疗的数据有限。我们提出的情况下，一个5岁的女孩与无痛性肿块在右大腿。影像学显示一恶性腱膜内软组织病变。组织病理学分析证实HS具有CD68、CD31、LCA和INI1免疫组化阳性，Ki-67指数为40%。分期：PET-CT发现局部淋巴结受累。R1手术切除后，患者接受辅助放疗（43 Gy至肿瘤床，36.9 Gy至腹股沟区）和oepa基础化疗。治疗20个月后，患者仍处于缓解期，功能预后良好。本病例强调了小儿软组织组织细胞肉瘤的罕见性和多模式治疗以达到长期缓解的重要性。

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

Radiology Case Reports Medicine-Radiology, Nuclear Medicine and Imaging

CiteScore

1.10

自引率

0.00%

发文量

1074

审稿时长

30 days

期刊介绍： The content of this journal is exclusively case reports that feature diagnostic imaging. Categories in which case reports can be placed include the musculoskeletal system, spine, central nervous system, head and neck, cardiovascular, chest, gastrointestinal, genitourinary, multisystem, pediatric, emergency, women''s imaging, oncologic, normal variants, medical devices, foreign bodies, interventional radiology, nuclear medicine, molecular imaging, ultrasonography, imaging artifacts, forensic, anthropological, and medical-legal. Articles must be well-documented and include a review of the appropriate literature.