Daniele Falaguasta, Silvia Garelli, Marta Mazzucato, Fabio Presotto, Silvia Tresso, Carlo De Riva
Unit of Endocrine, Metabolic, and Nutritional Diseases, Department of Clinical Medicine, Ospedale dell'Angelo, Mestre-Venice, Italy.
Unit of Internal Medicine, Ospedale Dell'Angelo, Mestre-Venice, Italy.
Endocr Metab Immune Disord Drug Targets. 2024 Jul 23. doi: 10.2174/0118715303320875240625070226.
Adenomas of the pituitary gland, predominantly prolactinomas, can exhibit aggressive behavior. Aggressive prolactinomas are characterized by radiographic invasion, rapid growth, clinically significant progression despite standard therapies, and recurrence after surgery or radiotherapy. Pituitary carcinoma is rare (0.1-0.2% of pituitary tumors) [1, 2]. Case Presentation: In 2005, a 50-year-old man presented with bitemporal hemianopsia and severe asthenia due to a large pituitary tumor. Hormonal tests revealed hyperprolactinemia and panhypopituitarism; he received hormonal replacement and dopamine agonists (DA) therapy with a reduction in prolactin levels. Ten years later, he experienced tumor regrowth consistent with pituitary apoplexy and VI cranial nerve palsy. MRI showed a macroadenoma with suprasellar extension and compression of the optic structures. The patient underwent transsphenoidal surgery in view of the partially resistant disease. Histopathology showed a pituitary macroadenoma, and immunohistochemistry showed a high mitotic index (Ki-67 80%). In 2016, the patient developed a partial deficit of the left sixth cranial nerve. He underwent a new surgery but with incomplete resection. In view of the aggressive and resistant nature of the disease, he received radiotherapy. In 2020, prolactin levels began to increase again. MRI showed an occipital- temporal lesion. Subsequently, he underwent radiotherapy and started chemotherapy with temozolomide, resulting in the normalization of prolactin levels in the absence of DA therapy. The patient is currently in remission, with no evidence of tumor recurrence. Conclusion: It was found that 15% of prolactinomas are resistant to DAs, and resistance to DA may signal malignant transformation. Therefore, multimodality therapy and molecular analysis are critical for aggressive prolactinomas and pituitary carcinoma.
垂体腺瘤,主要是泌乳素瘤,可表现出侵袭性。侵袭性泌乳素瘤的特征为影像学上的侵袭、快速生长、尽管接受标准治疗仍有具有临床意义的进展以及手术或放疗后复发。垂体癌很罕见(占垂体肿瘤的0.1 - 0.2%)[1, 2]。病例报告:2005年,一名50岁男性因巨大垂体肿瘤出现双颞侧偏盲和严重乏力。激素检测显示高泌乳素血症和全垂体功能减退;他接受了激素替代和多巴胺激动剂(DA)治疗,泌乳素水平降低。十年后,他出现与垂体卒中及第六颅神经麻痹一致的肿瘤复发。磁共振成像(MRI)显示一个鞍上扩展并压迫视结构的大腺瘤。鉴于部分耐药性疾病,该患者接受了经蝶窦手术。组织病理学显示为垂体大腺瘤,免疫组化显示有高有丝分裂指数(Ki - 67为80%)。2016年,该患者出现左侧第六颅神经部分功能缺损。他接受了再次手术,但切除不完全。鉴于该疾病的侵袭性和耐药性,他接受了放疗。2020年,泌乳素水平再次开始升高。MRI显示枕颞部病变。随后,他接受了放疗并开始使用替莫唑胺化疗,在未使用DA治疗的情况下泌乳素水平恢复正常。该患者目前处于缓解期,无肿瘤复发迹象。结论:发现15%的泌乳素瘤对DA耐药,对DA耐药可能预示着恶性转化。因此,多模式治疗和分子分析对于侵袭性泌乳素瘤和垂体癌至关重要。