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原发性皮肤局限性/明显界限的真性组织细胞肉瘤:两例长期随访病例。

Primary cutaneous localized/clearly-outlined true histiocytic sarcoma: Two long-term follow-up cases.

机构信息

Departments of, Department of, Diagnostic Pathology, Saitama Medical University International Medical Center, Hidaka, Japan.

Department of, Skin Oncology/Dermatology, Saitama Medical University International Medical Center, Hidaka, Japan.

出版信息

J Dermatol. 2020 Jun;47(6):651-653. doi: 10.1111/1346-8138.15315. Epub 2020 Mar 16.

DOI:10.1111/1346-8138.15315
PMID:32180261
Abstract

Histiocytic sarcoma (HS) is a rare hematopoietic tumor that mainly involves extranodal sites, including the intestinal tract, skin, soft tissues and other organs. It is well known as an aggressive neoplasm that shows a poor response to therapy. However, a subset of patients with resectable disease has shown a favorable outcome with surgical treatment. Primary cutaneous HS is exceedingly rare and, to date, its long-term prognosis has thus not been well described. Here, we highlight two cases of primary cutaneous HS that showed long-term survival. Case 1 was a healthy 47-year-old woman who found a 12-mm tumor on her forehead. Case 2 was a 66-year-old woman, under follow up of a myxoid liposarcoma in her leg, who presented with a 25-mm tumor in her hypothenar eminence. Histologically, the tumors in both cases had a smooth outline with proliferating atypical tumor cells that showed histiocytic differentiation as revealed by immunohistochemistry with antibodies to CD68 (KP-1) and lysozyme in case 1; and CD68, lysozyme and CD163 in case 2. Tumor cells in case 1 had a monotonous appearance. After complete resection, cases 1 and 2 have survived for 10 and 4 years, respectively, without recurrence. To date, such patients are relatively long follow-up cases of survival from HS and highlight how a clear outline of the primary cutaneous HS tumor may be associated with its resectability and be an important factor in the assessment of its curability.

摘要

组织细胞肉瘤(HS)是一种罕见的造血系统肿瘤,主要涉及结外部位,包括肠道、皮肤、软组织和其他器官。它是一种众所周知的侵袭性肿瘤,对治疗反应不佳。然而,一部分可切除疾病的患者通过手术治疗显示出良好的预后。原发性皮肤 HS 极为罕见,迄今为止,其长期预后尚未得到很好的描述。在此,我们强调两例原发性皮肤 HS 的长期生存病例。病例 1 是一位健康的 47 岁女性,她发现额头上有一个 12mm 的肿瘤。病例 2 是一位 66 岁女性,因腿部黏液样脂肪肉瘤接受随访,在手小鱼际处出现一个 25mm 的肿瘤。组织学上,两个病例的肿瘤均具有平滑的轮廓,肿瘤细胞增生且具有非典型性,免疫组织化学显示组织细胞分化,病例 1 中 CD68(KP-1)和溶菌酶阳性,病例 2 中 CD68、溶菌酶和 CD163 阳性。病例 1 中的肿瘤细胞呈单调形态。完全切除后,病例 1 和 2 分别存活了 10 年和 4 年,无复发。到目前为止,这些患者是 HS 中相对长期的生存随访病例,突出了原发性皮肤 HS 肿瘤的清晰轮廓可能与其可切除性相关,并成为评估其可治愈性的重要因素。

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