Mengoli M C, Bertolini F, Maur M, Barbieri F, Longo L, Gasparri P, Tiseo M, Rossi G
Unit of Pathology, IRCCS-Arcispedale Santa Maria Nuova, Reggio Emilia, Italy.
Department of Oncology, Modena University Hospital, Modena, Italy.
Pathologica. 2017 Dec;109(4):408-411.
We report an ALK-rearranged adenocarcinoma of the lung presenting as a pituitary metastasis, clinically simulating a pituitary adenoma. The patient, a 50 year-old, former-smoking woman was admitted with a Parinaud's syndrome characterized by progressive oculomotor impairment of visual verticality, bitemporal hemianopsia and nystagmus. Imaging studies showed a sellar tumor and the biopsy revealed a TTF-1 and napsin positive lung adenocarcinoma strongly expressing synaptophysin and CD56, also harboring ALK rearrangement. A subsequent CT scan disclosed the primary lung mass of the left upper lobe. The patient progressed after 4 cycles of cisplatin/pemetrexed as first line treatment, but showed a partial response and a significant clinical benefit from the combination of ceritinib and nivolumab in a phase Ib trial. Despite its central nervous system tropism, ALK-rearranged adenocarcinoma manifesting with pituitary gland involvement was never reported. Second generation ALK inhibitors seem the best therapeutic strategy.
我们报告一例表现为垂体转移的ALK重排肺腺癌，临床上酷似垂体腺瘤。该患者为一名50岁的已戒烟女性，因帕里诺德综合征入院，其特征为进行性动眼神经垂直视觉障碍、双颞侧偏盲和眼球震颤。影像学检查显示鞍区肿瘤，活检显示TTF-1和 napsin 阳性的肺腺癌，强烈表达突触素和CD56，也存在ALK重排。随后的CT扫描发现左上叶原发性肺肿块。患者一线接受4周期顺铂/培美曲塞治疗后病情进展，但在一项Ib期试验中，色瑞替尼和纳武单抗联合治疗显示出部分缓解和显著的临床获益。尽管ALK重排腺癌具有中枢神经系统嗜性，但从未有过累及垂体的报道。第二代ALK抑制剂似乎是最佳治疗策略。
