Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, 400038, China.
Department of Internal Medicine, University Hospital Laquintinie and Faculty of Medicine, Douala, Cameroon.
Diagn Pathol. 2019 Aug 14;14(1):89. doi: 10.1186/s13000-019-0869-2.
Follicular dendritic cell sarcoma (FDCS) is a rare malignancy. In addition to the classical histopathologic features, it has also some special morphological variants that can present a challenge in the diagnosis of this disease.
A 45-year-old male who presented with a left supraclavicular mass was given a final diagnosis of FDCS after lymph node biopsy. The specimen obtained during radical resection revealed five different morphologies, including the classical histological appearance and atypical areas resembling desmoplastic infiltrative carcinoma, anaplastic large cell lymphoma (ALCL), hemangiopericytoma and classical Hodgkin's lymphoma (CHL). Immunohistochemistry was notable for positive CD21 and CD23 expression across all morphologies. Given the atypical appearance and location, the specimen was initially misdiagnosed as a metastatic carcinoma based on histology alone at an outside institution. The patient eventually underwent surgical resection followed by adjuvant chemotherapy and radiation. Despite treatment, the disease progressed, and the patient passed away 36 months after surgery.
This unusual case of FDCS contains four types of atypical histomorphologies within a single tumor specimen, including those resembling ALCL and hemangiopericytoma which are described here for the first time. Our report further expands the histopathologic spectrum of FDCS and may help assist in the diagnosis of other such challenging cases.
滤泡树突状细胞肉瘤(FDCS)是一种罕见的恶性肿瘤。除了经典的组织病理学特征外,它还有一些特殊的形态变异,这在诊断这种疾病时可能会带来挑战。
一名 45 岁男性因左锁骨上肿块就诊,经淋巴结活检后最终诊断为 FDCS。根治性切除标本显示了五种不同的形态,包括经典的组织学外观和类似于促结缔组织增生性浸润性癌、间变性大细胞淋巴瘤(ALCL)、血管外皮细胞瘤和经典霍奇金淋巴瘤(CHL)的非典型区域。免疫组织化学显示所有形态均有 CD21 和 CD23 表达阳性。鉴于非典型的外观和位置,标本最初在一家外部机构仅根据组织学被误诊为转移性癌。患者最终接受了手术切除,随后进行了辅助化疗和放疗。尽管进行了治疗,但疾病仍在进展,患者在手术后 36 个月去世。
这个不寻常的 FDCS 病例在单个肿瘤标本中包含四种类型的非典型组织形态,包括在这里首次描述的类似于 ALCL 和血管外皮细胞瘤的形态。我们的报告进一步扩展了 FDCS 的组织病理学谱,并可能有助于辅助诊断其他具有挑战性的病例。