53例软组织颗粒细胞瘤的临床病理分析

Granular cell tumor: a clinicopathologic analysis of 53 cases

目的探究颗粒细胞瘤的临床病理特点及良恶性诊断标准。方法收集解放军总医院第一医学中心病理科2007-05—2019-12期间经手术切除的53例颗粒细胞瘤,分析其临床病理特点,并复习相关文献。结果男性23例,女性30例。年龄范围978岁,平均年龄43岁。27例发生于消化道,头颈部9例,皮下10例,骨骼肌内3例,乳腺4例。临床表现多为缓慢生长的无痛结节。病程数天至5年。肿瘤最大径0.36 cm,平均1.54 cm。大体呈结节状或多结节状,边界尚清晰,切面灰黄至灰白色,质软至质中。光镜下,肿瘤边界欠清,呈片状、梁状、束状或巢状排列;瘤细胞大,呈圆形、多角形或梭形,胞质丰富,嗜酸或淡染,呈颗粒状,细胞核小,多居中,染色质细腻,可见小核仁形成。每例胞质内均见嗜酸性小体,小体周围见空晕围绕。部分病例可见衬覆鳞状上皮的假上皮瘤样增生12/26例。3例肿瘤内可见固有腺。36例可见瘤内及瘤旁的淋巴组织浸润。11例伴间质纤维化,10例伴玻璃样变。1例见肿瘤包绕神经束。按照FS标准,良性为19例,非典型为33例,1例为恶性。按Nasser H标准,50例为良性,3例为恶性潜能未定。免疫组化表达情况:S-100(50/50,100%,弥漫中等至强阳);CD56(21/21,100%);Syn(19/23,82.6%);CD68(26/31,83.8%),TFE-3(11/12,91.6%);CD34(肿瘤细胞阴性,呈包绕肿瘤细胞巢或单个肿瘤细胞的阳性模式12/18,66.7%);Ki-67(表达均低于10%,39/39)。41例获随访结果,其中有1例复发两次。结论 FS标准结合影响预后的不良因素(包括局部复发、转移、肿瘤直径>5.5 cm等),可较好地判断颗粒细胞瘤的生物学行为。本组病例提示梭形细胞形态为肿瘤细胞正常形态之一,与预后无明显相关性。CD34免疫组化染色显示包绕肿瘤细胞的巢状或单个肿瘤细胞的阳性模式对鉴别诊断可能有提示意义。

Objective To investigate the clinicopathological features and diagnostic criteria of benign and malignant granular cell tumor( GCT). Methods Fifty-three cases of GCT surgically removed from the archive of Department of Pathology,the First Medical Center of PLA General Hospital from May 2007 to December of 2019 were collected,and their clinical data,microscopic morphology and immunohistochemical results were retrospectively analyzed. Results There were 23 males and 30 females. The age range was from 9 to 78 years,with an average age of 43 years. The affected organs were the digestive tract( 27 cases),subcutaneous or intramuscular tissue( 13 cases),head and neck( 9 cases) and breast( 4 cases). The clinical manifestations were mostly slow-growing painless nodules with a course of several days to 5 years.Ultrasound and MRI of breast cases suggested malignancy,BIRADS category 5. The largest diameter of the tumor ranged from 0. 3 cm to 6 cm,with an average of 1. 54 cm. Oval bodies with surrounding halos were formed in each case.Pseudoepithelial neoplasia was found in 12 cases. Intratumoral and peritumoral lymphoid infiltration was emerged in 36 cases. Nerve invasion was noted in 1 case. According to the Fanburg-Smith criteria,19 cases were classified as histological malignant,33 as atypical( 22 of them only showed spindle cells),and 1 case as malignant. Immunohistochemical results showed positive staining for S-100( 50/50),CD56( 21/21),Syn( 19/23),CD68( 26/31),and TFE-3( 11/12).Surprisingly,CD34 staining showed that small nest tumors or single tumor cells were wrapped. Follow-up information was available in 41 patients,and one patient showed local recurrence twice. Conclusions Fanburg-Smith criteria combined with adverse factors that affect prognosis( including local recurrence,metastasis,tumor diameter exceeding 5. 5 cm,etc.)can better predict the biological behavior of GCT. Spindle cell morphology is one variant of tumor cells,regardless of prognosis. In addition,CD34 immunohistochemical staining is shown to surround small nested tumors or single tumor cells.