甲状腺髓样癌（MTC）中的Amphicrine表型是一种罕见的现象，其特征是肿瘤细胞显示内分泌分化（降钙素分泌）和外分泌分化（粘蛋白产生和分泌）。关于苯丙胺MTC的病理生物学知之甚少。本报告采用基于病例的审查方法，讨论了细胞学，组织病理学,和这个罕见的神秘实体的超微结构特征，1例28岁的MEN2B综合征患者的放射学和新的MUC免疫组织化学结果，包括C细胞增生和多灶性MTC，具有苯丙胺特征。患者出现广泛的血行转移。MUC免疫表达分析揭示了微内腔形成的证据，和MUC1，MUC5AC的独特的迄今未报告的表达模式，和MUC6在MTC的苯丙胺亚型中。对文献的审查确定了另外5例具有有据可查的苯丙胺特征的MTC病例。在这六个案例中，两个与MEN2B综合征有关，四个人患有转移性疾病。对三名患者进行了随访，两个人死于疾病。鉴于其与MEN2B综合征和生物学侵袭性的频繁联系，对这种罕见的MTC亚型的识别可能具有临床意义。
Amphicrine phenotype in medullary thyroid carcinoma (MTC) is a rare phenomenon characterized by tumor cells that show both endocrine differentiation (calcitonin secretion) and exocrine differentiation (mucin production and secretion). Not much is known about the pathobiology of amphicrine MTCs. This report undertook a case-based
review approach by discussing the cytological, histopathological, and ultrastructural features of this rare enigmatic entity, expanding on the radiological and novel MUC immunohistochemistry findings from a 28-year-old MEN2B syndrome patient with C cell hyperplasia and multifocal MTC with amphicrine features. The patient had widespread hematogenous metastases at presentation. MUC immunoexpression analysis revealed evidence of micro-lumina formation, and unique to-date unreported expression patterns of MUC1, MUC5AC, and MUC6 in an amphicrine subtype of MTC.
Review of the literature identified five other MTC cases with well-documented amphicrine features. Of these six cases, two were associated with MEN2B syndrome, and four had metastatic disease. Follow-up was available in three patients, and two died of disease. Recognition of this rare subtype of MTC may be of clinical interest given their frequent link to MEN2B syndrome and biological aggressiveness.
