Das Sumanta, Khan Adil Aziz, Jacob Sherrin, Kumar R Naveen
Department of Pathology, Fortis Memorial Research Institute, Gurugram, India.
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Indian J Surg Oncol. 2025 Feb;16(1):53-59. doi: 10.1007/s13193-024-02022-9. Epub 2024 Jul 15.
Superficial CD34-positive fibroblastic tumor (SCPFT) is an extremely rare neoplasm with an indolent behavior. It was incorporated for the first time in the fifth edition of the World Health Organization (WHO) classification in the rarely metastasizing category of soft tissue tumors with an intermediate biological potential. It is a challenging clinical entity coupled with a pathological dilemma due to moderate to marked pleomorphism in tumor cells mimicking various sarcomas. Histologically, these tumors appear to be malignant at the outset but atypical mitosis or necrosis are rarely seen. These cells show diffuse positivity for CD34 and are focally positive for Pan-cytokeratin which further aids in diagnosis. Some of the SCPFT harbor PRDM10 rearrangement; however, the diagnosis does not require molecular testing. We are discussing a series of five cases of SCPFT, highlighting the diagnostic histological and immune-histochemical features of this enigmatic entity. We pen down the series as an accurate histopathological diagnosis of SCPFT is undoubtedly a key to prevent its misdiagnosis as a malignant tumor with drastically different clinical implications.
浅表性CD34阳性成纤维细胞肿瘤(SCPFT)是一种极为罕见的肿瘤,生长缓慢。它首次被纳入世界卫生组织(WHO)第五版分类中的软组织肿瘤,属于转移罕见、具有中等生物学潜能的类别。由于肿瘤细胞存在中度至显著的多形性,类似各种肉瘤,它是一个具有挑战性的临床实体,同时也是一个病理难题。从组织学上看,这些肿瘤起初看似恶性,但很少见到非典型有丝分裂或坏死。这些细胞CD34呈弥漫性阳性,泛细胞角蛋白呈局灶性阳性,这进一步有助于诊断。部分SCPFT存在PRDM10重排;然而,诊断并不需要分子检测。我们正在讨论5例SCPFT病例系列,突出这种神秘实体的诊断性组织学和免疫组织化学特征。我们记录下该病例系列,因为SCPFT准确的组织病理学诊断无疑是防止将其误诊为具有截然不同临床意义的恶性肿瘤的关键。