Pathology Unit, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Sant'Andrea Hospital, Rome, Italy.
Clinical Pathology Department, Army hospital of Rome "Celio", Roma, Italy.
Acta Chir Belg. 2023 Dec;123(6):687-690. doi: 10.1080/00015458.2022.2102358. Epub 2022 Jul 20.
Interdigitating dendritic cell sarcoma (IDCS) represents an extremely rare neoplasm frequently originating from T-cell- associated dendritic antigen-presenting cells normally populating the paracortex of lymph nodes. Diagnosis is challenging due to the rarity of this neoplasm and the overlapping features with the other primary and metastatic spindle cell neoplasm, even more, when localized in an extra-nodal site.
Herein we report a case of 48 Years old woman with a six-month history of centimetric, quiet painful mass of the philtrum without other significant comorbidity.
Histological report showed a proliferation of quiet bland spindle to plump cell, scattered multinucleated giant cell in a subtle background of lymphocytes. IHC study displays only positivity for S-100 and fine, granular scattered cytoplasmatic stain for CD68; all dendritic IHC markers were negative. Morphological and immunohistochemical analyses were consistent with extra-nodal Interdigitating Dendritic Cell Neoplasm.
Interdigitating Dendritic Cell Sarcoma is a rare and challenging entity with a variable prognosis. We present a case of extra-nodal IDCS whit low worrisome histological features, emphasizing the need for further efforts to better definitely this rare neoplasm ad its potential for malignancy.
指状突细胞肉瘤（IDCS）是一种非常罕见的肿瘤，通常起源于 T 细胞相关的树突状抗原呈递细胞，这些细胞通常存在于淋巴结的副皮质区。由于这种肿瘤非常罕见，与其他原发性和转移性梭形细胞肿瘤的特征重叠，因此诊断具有挑战性，尤其是当肿瘤位于淋巴结外部位时。
本文报告了一例 48 岁女性的病例，她有 6 个月的病史，表现为鼻唇沟处有厘米大小的无痛性肿块，无其他重要合并症。
组织学报告显示，存在安静的、温和的梭形至肥胖细胞的增殖，在微妙的淋巴细胞背景中散布有多核巨细胞。免疫组化研究仅显示 S-100 阳性和 CD68 阳性的细颗粒状细胞质染色；所有树突状免疫标志物均为阴性。形态学和免疫组化分析与结外指状突细胞肿瘤一致。
指状突细胞肉瘤是一种罕见且具有挑战性的实体瘤，其预后存在差异。我们报告了一例结外 IDCS 的病例，其组织学特征不具有明显的担忧性，强调需要进一步努力以更好地确定这种罕见肿瘤及其恶性潜能。
