背景:颗粒细胞瘤(GCT)仍然是一个诊断性的临床病理问题,因为其详细的形态学和临床特征的确切频率是未知的,因为大多数观察是从小系列或孤立病例收集的。在这里,我们的目的是根据我们的系列文章和现有的医学(PubMed)文献,强调这种罕见肿瘤的所有临床病理特征的发生频率.
方法:42例被评估为:肿瘤累及的组织层(皮肤和粘膜),增长模式,核多态性,有丝分裂指数,坏死,纺锤,钙化,透明质化,还有米莲的脓疱卵圆形身体,以及神经周和血管侵犯,以及邻近上皮的改变,淋巴细胞和嗜酸性粒细胞浸润。,随访进行了分析。肿瘤被分为良性,根据Fanburg-Smith标准,非典型和恶性,并根据Nasser标准进入良性或恶性潜力不确定的GCT。根据PRISMA指南对1499例进行了相同的特征分析。
结果:在当前系列中,诊断时的平均年龄为45.8岁(范围6~69岁).大多数患者是女性(60%),受累器官的频率下降:皮肤和皮下组织,支气管,食道,乳房,舌头,喉部,咽部,牙龈,气管,右结肠,外阴,和下咽。未见复发或进展,尽管32例切除不完全,除了一个恶性肿瘤。生长模式为渗透型(85.71%)或有限型(7.14%)。16个肿瘤有囊泡核。在两个肿瘤中发现有丝分裂活性。在14个肿瘤中发现淋巴细胞浸润。6例存在嗜酸性粒细胞。右结肠的一个GCT显示广泛的钙化和透明化。在6个病变中发现了神经周围浸润。未发现血管侵犯。一个肿瘤是临床上恶性的,患者在诊断后2年死亡。医学文献综述显示,在报告的临床和形态学特征的频率方面,结果相似。在有随访的病例中,近20%显示切缘阳性,20%出现局部复发.根据范堡-史密斯标准,72%是良性的,17%的非典型和11%的恶性肿瘤,而根据纳赛尔的说法,93%是良性的,7%是不确定的恶性潜力。然而,真正的恶性肿瘤,正如GCT转移所证实的那样,在近2.5%的病例中发现。
结论:GCT通常是良性肿瘤,影响任何解剖位置。坏死和有丝分裂活动似乎是检测侵袭性肿瘤的最有效的组织学标准。但是转移的存在(占病例的2.5%)仍然是诊断恶性GCT的最公认的确定标准。
BACKGROUND: Granular cell tumor (GCT) remains a diagnostic clinicopathologic problem because the exact frequency of its detailed morphological and clinical characteristics is unknown as most observations are collected from small series or isolated cases. Herein, our aim is to highlight the frequency of all clinicopathological characteristics of this rare tumor based in our series and the available medical (PubMed) literature.
METHODS: 42 cases were evaluated for: tissue layers involved by the tumor (in skin and mucosae), growth pattern, nuclear pleomorphism, mitotic index, necrosis, spindling, calcification, hyalinization, and pustule-ovoid bodies of Milian, as well as perineural and vascular invasion, and the presence of adjacent epithelium changes, and lymphocytes and eosinophils infiltration., Follow-up was analyzed. The tumors were subclassified into benign, atypical and malignant according to Fanburg-Smith criteria and into benign or GCT of uncertain malignant potential according to Nasser criteria. The same characteristics were analyzed for 1499 cases reviewed according to PRISMA guidelines.
RESULTS: In the current series, the mean age at diagnosis was 45.8 years (range 6-69 years). Most patients were females (60 %) and the involved organs were by descending frequency: skin and subcutaneous tissue, bronchus, esophagus, breast, tongue, larynx, pharynx, gingiva, trachea, right colon, vulva, and hypopharynx. No recurrence or progression was seen, despite 32 cases were incompletely excised, with the exception of one malignant tumor. The growth pattern was either infiltrative (85.71 %) or well limited (7.14 %). Sixteen tumors had vesicular nuclei. Mitotic activity was found in two tumors. Lymphocytic infiltration was found in 14 tumors. Eosinophils were present in 6 cases. One GCT of the right colon showed extensive calcification and hyalinization. Perineural invasion was noted in 6 lesions. No vascular invasion was found. One tumor was clinically malignant and the patient died 2 years after diagnosis. Medical literature review showed similar results in terms of frequency of the reported clinical and morphological features. Among cases with available follow up, almost 20 % showed positive margins and of those 20 % developed local recurrence. According to the Fanburg-Smith criteria, 72 % would be benign, 17 % atypical and 11 % malignant tumors, while according to those of Nasser, 93 % would be benign and 7% of uncertain malignant potential. However, true malignancy, as affirmed by metastasis of GCT is found in almost 2.5 % of the cases.
CONCLUSIONS: GCT is a usually benign tumor, affecting any anatomic location. Necrosis and mitotic activity seem to be the most effective histologic criteria for detecting aggressive tumors, but the presence of metastasis (2.5 % of the cases) remains the most accepted definitive criterion for diagnosis of malignant GCT.