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目的探讨成人型外阴孤立性肥大细胞瘤的临床病理特点。方法报道1例成人型外阴孤立性肥大细胞瘤,回顾分析其临床和影像学表现及病理组织学特点,应用免疫组化及特殊染色进一步确认诊断,探讨其鉴别诊断。结果患者女性,23岁。发现外阴肿物长达18年。CT提示阴蒂海绵体异常增大;MRI示外阴部肿物表现为与周边肌肉信号相似,界清。核医学MIBG显像显示外阴部放射性增强,提示异位嗜铬细胞瘤;奥曲肽显像示生长抑素受体(-);核医学血池显像示外阴部肿物处未见示踪剂(99mTcRBC)异常摄取。巨检:单一灰白色结节,切面质地细腻,无包膜。镜检:相对一致的肿瘤细胞在真皮层纤细的纤维间隔间呈结节状生长,围绕血管和神经,瘤细胞胞质丰富,呈红色、颗粒状,胞核圆形、染色质致密,核分裂象罕见,肿瘤局部可见小淋巴细胞浸润。免疫组化:肿瘤细胞CD117、CD68,CD43和LCA弥漫强(+),α-AT和calretinin部分(+),Ki-67<1%,瘤细胞胞质甲苯胺蓝染色呈深紫红色。术后随诊98个月,肿瘤无复发和转移。结论成人型孤立性肥大细胞瘤是一种罕见的肥大细胞良性肿瘤,组织病理学诊断关键在于认识肥大细胞,结合免疫组化和甲苯胺蓝染色可以确诊。
Objective To investigate the clinicopathological features of adult vulvar solitary mastocytoma. Methods One adult adult genital solitary mastocytoma was reported. The clinical and radiographic features and histopathological features were retrospectively analyzed. Immunohistochemistry and special staining were used to confirm the diagnosis and to explore the differential diagnosis. Results Female patient, 23 years old. Found vulvar tumor for up to 18 years. CT prompted abnormal clitoral enlargement; MRI showed genital tumors showed similar to the surrounding muscle signal, clear. Nuclear medicine MIBG imaging showed increased genitourinary radioactivity, suggesting ectopic pheochromocytoma; octreotide imaging showed somatostatin receptor (-); nuclear medicine blood pool imaging showed no signs of vulvar tumor 99mTcRBC) abnormally uptake. Giant check: a single gray-white nodules, cut texture delicate, non-enveloped. Microscopic examination: relatively consistent tumor cells in nodular dermis slender fiber compartment growth, around the blood vessels and nerves, tumor cells rich in cytoplasm, red, granular, round nucleus, chromatin dense, mitotic Rare, locally visible small lymphocytic infiltration of the tumor. Immunohistochemistry showed that the tumor cells were diffusely strong (+), α-AT and calretinin (+), Ki-67 was less than 1% in the tumor cells, and the tumor cells were dark purple in toluidine blue staining. Follow-up after 98 months, no recurrence and metastasis of the tumor. Conclusion Adult solitary mastocytoma is a rare benign tumor of mast cells. The key of histopathological diagnosis is to recognize mast cells, which can be confirmed by immunohistochemistry and toluidine blue staining.