PDF(Pubmed)
Abstract：
Follicular dendritic cell sarcoma (FDCS) is a rare, mesenchymal neoplasm that may be nodal or extranodal in location. Lung involvement is rare. It is a slow-growing, painless tumor with a frequent capacity to recur and metastasize. We present a case of extranodal FDCS of the lung with an unusual presentation. A 34-year-old man presented with the complaints of haemoptysis and chest pain. A large left perihilar mass with endobronchial component was found on radiological evaluation. On endobronchial biopsy and mediastinal tru-cut biopsy, differential diagnoses of an inflammatory myo-fibrohistiocytic lesion and leiomyosarcoma were provided on the basis of morphological features of bipolar spindled cells arranged in intersecting fascicles and storiform patterns and immunophenotyping. A pneumonectomy was performed for the mass on which further immunohistochemical evaluation with CD21, CD35, and D2-40 finally helped form a diagnosis of FDCS. The patient recovered well from the surgery and has been on follow-up ever since. Owing to the rarity of this condition and its non-specific clinical features, FDCS is often misdiagnosed in the absence of appropriate immunohistochemistry. An of awareness of its morphological features and immunophenotype is, thus, necessary to provide early treatment and follow-up in order to prevent its recurrence and metastasis.
摘要：
滤泡树突状细胞肉瘤（FDCS）是一种罕见的，间充质肿瘤，可能是结节或结外。肺部受累是罕见的。这是一个缓慢增长的，无痛性肿瘤，经常复发和转移。我们介绍了一例肺结外FDCS的异常表现。一名34岁的男子出现咯血和胸痛的抱怨。在放射学评估中发现了带有支气管内成分的大的左肺门周围肿块。在支气管内活检和纵隔切开活检中，根据呈交叉束状排列的双极纺锤状细胞的形态学特征和免疫表型，提供了炎性肌纤维组织细胞病变和平滑肌肉瘤的鉴别诊断。对肿块进行了肺切除术，对CD21，CD35和D2-40进行进一步的免疫组织化学评估最终有助于诊断FDCS。患者从手术中恢复良好，此后一直在随访。由于这种情况的罕见性及其非特异性临床特征，在缺乏适当的免疫组织化学的情况下，FDCS经常被误诊。对其形态特征和免疫表型的认识是，因此，必须提供早期治疗和随访，以防止其复发和转移。
