Furlong Mary A, Motamedi Kambiz, Laskin William B, Vinh Tuyethoa N, Murphey Mark, Sweet Donald E, Fetsch John F
Department of Soft Tissue, Armed Forces Institute of Pathology, Washington, DC 20306, USA.
Hum Pathol. 2003 Jul;34(7):670-9. doi: 10.1016/s0046-8177(03)00250-8.
Synovial and tenosynovial giant cell tumors only rarely arise in close proximity to the axial skeleton; to date, fewer than 30 examples have been reported in the English-language medical literature. In this report we describe the clinical, radiologic, histopathologic, and immunohistochemical findings in 15 cases retrieved from our files. The study group comprised 7 males and 8 females, ranging in age from 17 to 44 years (mean age, 32 years). The tumors involved the cervical (n = 11), thoracic (n = 1), lumbar (n = 2), and sacrococcygeal (n = 1) regions and ranged in size from 1.0 to 6.0 cm in greatest dimension (median size, 3 cm). Symptoms were present for 2 months to at least 2 years, with the most common complaint being pain localized to the spinal region (n = 12). Ten patients also had radicular symptoms. Radiologic studies, available for 11 cases, usually demonstrated a mass involving the posterior aspect of adjoining vertebrae. Bony abnormalities (including scalloping, erosion, and destruction), facet joint and soft tissue involvement, and extradural extension were typically present. Histologically, all tumors contained a proliferation of epithelioid (histiocytoid) cells, admixed with varying numbers of osteoclast-like giant cells, siderophages, xanthoma cells, lymphocytes, and some spindled fibroblast-like cells. Only 1 tumor had the classic villiform architecture of pigmented villonodular synovitis. The remaining 14 tumors had a nodular appearance with varying amounts of collagen. Seven of these had definite histological evidence of infiltrative growth, and 6 had some features that warranted concern for possible infiltration. Only 1 tumor had findings fully compatible with a localized synovial-type giant cell tumor/nodular (teno)synovitis. All tumors had mitotic activity, with mitotic counts ranging from 1 to 21 mitotic figures per 50 high-power fields (HPFs) (mean mitotic count, 5 mitotic figures/50 HPFs). Immunohistochemistry was performed on 5 tumors, and immunoreactivity was present for CD68, CD163, and vimentin. Limited immunoreactivity for muscle actin (HUC1-1) was also noted. Follow-up information was available for 9 of the 15 patients (60%). Five patients had no evidence of recurrent or persistent disease 4 months to 9 years after undergoing either a local excision with gross total tumor removal (with or without irradiation) or a wide en bloc resection. Four patients had persistent disease after undergoing either an incomplete resection or biopsy with spinal fusion procedure. All 4 of these patients had additional surgical intervention (accompanied by irradiation in 2 instances), but only one was known to be disease-free at last follow-up (10 years after gross total tumor removal). No patient has experienced a metastasis or died of disease. The best predictor of outcome was gross total tumor removal at the surgical outset.
滑膜和腱鞘巨细胞瘤极少发生于靠近中轴骨骼的部位;迄今为止,英文医学文献报道的病例不足30例。在本报告中,我们描述了从我们的病例档案中检索出的15例病例的临床、放射学、组织病理学和免疫组化结果。研究组包括7名男性和8名女性,年龄在17至44岁之间(平均年龄32岁)。肿瘤累及颈椎(n = 11)、胸椎(n = 1)、腰椎(n = 2)和骶尾部(n = 1)区域,最大直径为1.0至6.0 cm(中位大小3 cm)。症状持续2个月至至少2年,最常见的主诉是局限于脊柱区域的疼痛(n = 12)。10例患者还伴有神经根症状。11例患者有放射学检查结果,通常显示肿块累及相邻椎体的后部。典型表现为骨质异常(包括扇贝样改变、侵蚀和破坏)、小关节和软组织受累以及硬膜外扩展。组织学上,所有肿瘤均有上皮样(组织细胞样)细胞增生,混有数量不等的破骨细胞样巨细胞、含铁血黄素巨噬细胞、黄色瘤细胞、淋巴细胞以及一些梭形成纤维细胞样细胞。只有1例肿瘤具有色素沉着绒毛结节性滑膜炎的经典绒毛状结构。其余14例肿瘤呈结节状外观,伴有不同数量的胶原。其中7例有明确的浸润性生长组织学证据,6例有一些值得关注的可能浸润特征。只有1例肿瘤的表现完全符合局限性滑膜型巨细胞瘤/结节性(腱鞘)滑膜炎。所有肿瘤均有有丝分裂活性,每50个高倍视野(HPF)的有丝分裂计数为1至21个有丝分裂象(平均有丝分裂计数为5个有丝分裂象/50个HPF)。对5例肿瘤进行了免疫组化检测,CD68、CD163和波形蛋白呈免疫反应性。还发现对肌肉肌动蛋白(HUC1-1)有有限的免疫反应性。15例患者中有9例(60%)有随访信息。5例患者在接受局部切除并完全切除肿瘤(有或无放疗)或广泛整块切除术后4个月至9年,无复发或持续性疾病证据。4例患者在接受不完全切除或活检并行脊柱融合手术后有持续性疾病。这4例患者均接受了额外的手术干预(2例伴有放疗),但最后一次随访(肿瘤完全切除后10年)时只有1例已知无病。没有患者发生转移或死于该疾病。手术开始时完全切除肿瘤是预后的最佳预测指标。