乳腺颗粒细胞瘤的临床表现、病理诊断与治疗

Scardina L, D. A, Sanchez Am, D’Archi S, Biondi E, Carnassale B, D’Alessandris N, Scaglione G, S. A, M. A, Masetti R, F. G.
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摘要

颗粒细胞瘤是一种罕见的软组织肿瘤,仅1%的病例可表现为恶性。它被认为是一种起源于周围神经或周围神经的雪旺细胞的肿瘤。材料和方法:我们回顾了2011年1月至2021年1月在意大利罗马的agagostino Gemelli IRCCS基金会接受治疗的5例乳腺颗粒细胞瘤患者。结果:所有颗粒细胞瘤均表现为孤立、无痛、坚硬的肿块,高度提示恶性。放射学表现是异质性和非特异性的。所有病变均表现为肿块,超声表现为低回声,形状不规则,轮廓模糊,边缘特征明显。肿瘤由大的多边形细胞组成,具有丰富的嗜酸性颗粒胞浆和小的中心核,免疫组织化学阳性S100, Vimentin(染色可变),CD56;HMB45、MelanA、AE1/AE3、EMA、Desmin均阴性。结论:颗粒细胞瘤是一种罕见的乳腺良性疾病,其临床和影像学特征与乳腺癌非常相似。治疗选择包括广泛切除或乳房肿瘤切除并边缘评估(肿瘤上没有墨水)。
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Granular Cell Tumor of the Breast: Clinical Presentation, Pathological Diagnosis and Treatment
Introduction: Granular cell tumor is a rare neoplasm of soft tissue and only in 1% of cases, it can shows a malignant behaviour. It is presumed to be a tumor originating from perineural or putative Schwann cells of peripheral nerves. Materials and Methods: We reviewed five patients affected by Granular cell tumor of the breast treated between January 2011 and January 2021 at the Fondazione Policlinico Universitario Agostino Gemelli IRCCS of Rome, Italy. Results: All of the granular cell tumors presented as solitary, painless and firm lump, highly suggestive of malignancy. The radiological findings were heterogeneous and non-specific. All lesions presented as masses, more clearly evident on ultrasound as hypoechoic lesions, with irregular shape, blurred contours and borderline features. The tumors were composed of large polygonal cells with abundant eosinophilic granular cytoplasm and small, central nuclei, being immunohistochemically positive for S100, Vimentin (with variable staining), CD56; negative for HMB45, MelanA, AE1/AE3, EMA, and Desmin. Conclusion: Granular cell tumor is a rare, usually benign breast disease that can have very similar characteristics to breast cancer both clinically and radiologically. Treatment of choice consists in wide resection or lumpectomy with margin assessment (no ink on tumor).
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