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原发性皮肤组织细胞肉瘤:1例罕见病例

Primary Cutaneous Histiocytic Sarcoma: A Rare Case.

作者信息

Ntalakos Nektarios, Arnaouti Maria

机构信息

Department of Pathology, Saint Savvas Anticancer Hospital of Athens, Athens, GRC.

出版信息

Cureus. 2025 Aug 2;17(8):e89241. doi: 10.7759/cureus.89241. eCollection 2025 Aug.

DOI:10.7759/cureus.89241
PMID:40901224
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12400779/
Abstract

Histiocytic sarcoma is a rare malignancy composed of neoplastic cells that resemble macrophages in morphology and immunoprofile. It manifests in lymph nodes or extranodal sites, with the majority occurring in the gastrointestinal tract, central nervous system, spleen, skin, and soft tissues. Clinical manifestations depend on organ involvement, with systemic symptoms, including fever, fatigue, night sweats, weight loss, and weakness. A significant proportion of patients are diagnosed at an advanced stage, which is associated with high mortality rates. In this report, we highlight the case of an 80-year-old who displayed a subcutaneous forearm lesion. Histological evaluation confirmed the diagnosis of cutaneous histiocytic sarcoma, with tumor cells expressing CD163 and CD68. A multidisciplinary team decided on supplementary adjuvant radiotherapy, considering the unifocal presentation and complete surgical excision of the lesion. The patient remains disease-free at one-year follow-up. This case emphasizes that a unifocal and localized manifestation of the malignancy has a more favorable outcome. Although treatment protocols have not yet been standardized, recent advances in genomic profiling may offer opportunities for targeted therapies.

摘要

组织细胞肉瘤是一种罕见的恶性肿瘤,由形态和免疫表型类似于巨噬细胞的肿瘤细胞组成。它表现于淋巴结或结外部位,大多数发生在胃肠道、中枢神经系统、脾脏、皮肤和软组织。临床表现取决于受累器官,伴有全身症状,包括发热、疲劳、盗汗、体重减轻和虚弱。相当一部分患者在晚期被诊断出来,这与高死亡率相关。在本报告中,我们重点介绍了一名80岁患者,其前臂出现皮下病变。组织学评估确诊为皮肤组织细胞肉瘤,肿瘤细胞表达CD163和CD68。考虑到病变呈单灶性且已完全手术切除,多学科团队决定进行辅助性放疗。患者在一年的随访中仍无疾病复发。该病例强调,恶性肿瘤的单灶性和局限性表现预后更有利。尽管治疗方案尚未标准化,但基因组分析的最新进展可能为靶向治疗提供机会。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/d827fde096a1/cureus-0017-00000089241-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/bb42595a624d/cureus-0017-00000089241-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/c5c93984800f/cureus-0017-00000089241-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/d827fde096a1/cureus-0017-00000089241-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/bb42595a624d/cureus-0017-00000089241-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/c5c93984800f/cureus-0017-00000089241-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7aee/12400779/d827fde096a1/cureus-0017-00000089241-i03.jpg

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本文引用的文献

1
Case Report: A case series on histiocytic sarcoma - various clinical features and patient outcomes.病例报告:组织细胞肉瘤病例系列——各种临床特征及患者预后
Front Oncol. 2025 Feb 24;15:1505737. doi: 10.3389/fonc.2025.1505737. eCollection 2025.
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Genomic profiling of primary histiocytic sarcoma reveals two molecular subgroups.原发性组织细胞肉瘤的基因组分析揭示了两个分子亚群。
Haematologica. 2020 Apr;105(4):951-960. doi: 10.3324/haematol.2019.230375. Epub 2019 Aug 22.