Smith Steven C, Gooding William E, Elkins Matthew, Patel Rajiv M, Harms Paul W, McDaniel Andrew S, Palanisamy Nallasivam, Uram-Tuculescu Cora, Balzer Bonnie B, Lucas David R, Seethala Raja R, McHugh Jonathan B
*Departments of Pathology and Surgery, VCU School of Medicine, Richmond, VA †Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA §Biostatistics Facility, University of Pittsburgh Cancer Institute **Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA ∥Department of Pathology, SUNY Upstate Medical University, Syracuse, NY Departments of ‡Pathology ¶Dermatology ††Oral and Maxillofacial Surgery, University of Michigan Health System, Ann Arbor #Department of Urology, Henry Ford Health System, Detroit, MI.
Am J Surg Pathol. 2017 Dec;41(12):1642-1656. doi: 10.1097/PAS.0000000000000940.
Solitary fibrous tumors (SFTs) of the head and neck are uncommon. Lesions previously diagnosed in the head and neck as hemangiopericytomas (HPCs), giant cell angiofibromas (GCAs), and orbital fibrous histiocytomas (OFHs) are now recognized as within the expanded spectrum of SFTs. To better understand the clinicopathologic profile of head and neck SFTs, we performed a multi-institutional study of 88 examples. There was no sex predilection (F:M ratio 1.2), and the median patient age was 52 years (range: 15 to above 89 y). The sinonasal tract and orbit were the most common sites involved (30% and 25%), followed by the oral cavity and salivary glands (15% and 14%). Original diagnoses included HPC (25%), SFT (67%), and OFH (6%), with 1 SFT and 1 OFH noted as showing GCA-like morphology. On review, the predominant histologic pattern was classic SFT-like in 53% and cellular (former HPC-like) in 47%; lipomatous differentiation (8%) and GCA-like pattern (7%) were less prevalent. Subsets demonstrated nuclear atypia (23%), epithelioid morphology (15%), or coagulative necrosis (6%). Infiltrative growth (49%) and osseous invasion (82%) were prevalent among evaluable cases. Of the 48 SFTs with follow-up (median: 43 mo), 19 showed recurrence (40%). Of these, 4 patients were alive with disease and 4 dead of disease. Size and mitotic rate were negative prognosticators using a joint prognostic proportional hazards regression model. Three patients experienced metastasis, to lungs, parotid, bone, and skull base, including one case showing overtly sarcomatous "dedifferentiation." As a group, SFTs present in a wide anatomic and morphologic spectrum in the head and neck. Only rare examples metastasize or cause death from disease. However, the fairly high local recurrence rate underscores their aggressive potential and highlights the importance of prospective recognition.
头颈部孤立性纤维性肿瘤(SFT)并不常见。以往在头颈部诊断为血管外皮细胞瘤(HPC)、巨细胞血管纤维瘤(GCA)和眼眶纤维组织细胞瘤(OFH)的病变,现在被认为属于SFT的扩展范畴。为了更好地了解头颈部SFT的临床病理特征,我们对88例病例进行了多机构研究。该肿瘤无性别倾向(女性与男性比例为1.2),患者中位年龄为52岁(范围:15至89岁以上)。鼻窦和眼眶是最常受累的部位(分别为30%和25%),其次是口腔和唾液腺(分别为15%和14%)。最初诊断包括HPC(25%)、SFT(67%)和OFH(6%),其中1例SFT和1例OFH显示出GCA样形态。复查时,主要组织学模式为经典SFT样的占53%,细胞性(以前的HPC样)占47%;脂肪化生(8%)和GCA样模式(7%)较少见。部分病例表现为核异型性(23%)、上皮样形态(15%)或凝固性坏死(6%)。在可评估病例中,浸润性生长(49%)和骨质侵犯(82%)较为常见。在48例有随访的SFT中(中位随访时间:43个月),19例复发(40%)。其中,4例患者带瘤生存,4例死于疾病。使用联合预后比例风险回归模型分析,肿瘤大小和有丝分裂率是不良预后因素。3例患者发生转移,转移至肺、腮腺、骨和颅底,其中1例表现为明显的肉瘤样“去分化”。总体而言,头颈部SFT在解剖和形态学上具有广泛的表现。只有极少数病例发生转移或因疾病死亡。然而,较高的局部复发率强调了其侵袭性潜能,并突出了前瞻性识别的重要性。