CAJ | 학술논문

目的：探讨头颈部血管周上皮样细胞肿瘤( perivascular epithelioid cell tumors, PEComas)的临床特点、病理形态学特征、治疗及预后。方法收集2例头颈部PEComa的临床资料,对2例PEComa进行HE及免疫组化EnVision两步法染色,并复习相关文献。结果发生在喉部(女性,26岁)及鼻腔(男性,56岁)各1例,肿瘤边界尚清,直径2.5~3.5 cm。镜检：上皮样细胞呈巢团状、器官样排列,瘤细胞胞质丰富,嗜伊红颗粒样或透明,细胞核圆形,具轻度异型性。瘤组织富含纤细的纤维血管网。可见核分裂及局灶坏死形成。免疫表型：vimentin(2/2)、HMB-45(1/2)、Melan-A(2/2)、SMA(2/2)、Calponin(2/2)、desmin (1/2)和TFE3(1/2)呈弥漫或局灶阳性,CK、CD10、S-100、CgA、Syn和MyoD1均呈阴性。结论发生于头颈部的PEComa少见,组织形态学与副神经节瘤、恶性黑色素瘤及转移性癌有交叉,免疫组化EnVision两步法染色黑色素和肌源性标志物共表达有助于明确诊断。个别病例有局部或远处转移甚至死亡,提示头颈部PEComa的生物学行为具有恶性潜能。
목적：탐토두경부혈관주상피양세포종류( perivascular epithelioid cell tumors, PEComas)적림상특점、병리형태학특정、치료급예후。방법수집2례두경부PEComa적림상자료,대2례PEComa진행HE급면역조화EnVision량보법염색,병복습상관문헌。결과발생재후부(녀성,26세)급비강(남성,56세)각1례,종류변계상청,직경2.5~3.5 cm。경검：상피양세포정소단상、기관양배렬,류세포포질봉부,기이홍과립양혹투명,세포핵원형,구경도이형성。류조직부함섬세적섬유혈관망。가견핵분렬급국조배사형성。면역표형：vimentin(2/2)、HMB-45(1/2)、Melan-A(2/2)、SMA(2/2)、Calponin(2/2)、desmin (1/2)화TFE3(1/2)정미만혹국조양성,CK、CD10、S-100、CgA、Syn화MyoD1균정음성。결론발생우두경부적PEComa소견,조직형태학여부신경절류、악성흑색소류급전이성암유교차,면역조화EnVision량보법염색흑색소화기원성표지물공표체유조우명학진단。개별병례유국부혹원처전이심지사망,제시두경부PEComa적생물학행위구유악성잠능。
Purpose To investigate the clinicopathological features, treatment and prognosis of perivascular epithelioid cell tumor ( PEComa) of the head and neck. Methods Two cases of PEComa were analyzed by studying clinical data, histopathologic and immu-nohistochemical ( EnVision) changes and the related literatures were reviewed. Results Case 1 was a 26-year-old woman with a re-current larynx tumor, and case 2 was diagnosed in a 56-year-old man with a left nasal cavity mass. Their tumors were well-demarcated, and ranged from 2. 5 to 3. 5 cm in diameter. Microscopically, the tumors were composed of epithelioid cells arranged in nets, organoid pattern. The tumor cells had abundant pale eosinophilic granular to clear cytoplasm, vesicular nuclei, and mild atypia. The stroma was rich in capillaries, sinusoidal vasculature. There was mitotic activity and focal necrosis. By immunohistochemistry, the tumor cells were diffusely or focally positive for vimentin (2/2), HMB-45(1/2), Melan-A(2/2), SMA(2/2), Calponin(2/2), desmin(1/2), and TFE3(1/2). They were negative for CK, CD10, S-100, CgA, Syn, and MyoD1. Conclusion PEComa of the head and neck is rare. Definite diagnosis depends upon the comprehensive analysis of histopathology and coexpression of melanocytic and myoid mark-ers. The differential diagnosis include paraganglioma, malignant melanoma and metastatic carcinoma. Few PEComas of the head and neck behaved in an aggressive fashion with distant or loco-regional metastases and died of disease-related causes, it should be regarded as tumors with a malignant potential.