Bettencourt-Silva Rita, Pereira Josué, Belo Sandra, Magalhães Daniela, Queirós Joana, Carvalho Davide
Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar São João, Porto, Portugal.
Faculty of Medicine, University of Porto, Porto, Portugal.
Front Endocrinol (Lausanne). 2018 Jun 6;9:312. doi: 10.3389/fendo.2018.00312. eCollection 2018.
The diagnosis of pituitary carcinoma is very rare, requires the evidence of metastatic disease, and has a poor overall survival. Malignant prolactinoma frequently requires dopamine agonist therapy, pituitary surgery, radiotherapy, and even chemotherapy.
A 19-year-old female presented with galactorrhea, primary amenorrhea, and left hemianopsia. Complementary study detected hyperprolactinemia and a pituitary macroadenoma with cavernous sinus invasion and suprasellar growth. She was treated with cabergoline and bromocriptine without clinical or analytical improvement. Resection of the pituitary lesion was programmed and a non-contiguous lesion of the nasal mucosa was detected during the approach. This metastasis led to the diagnosis of prolactin-producing pituitary carcinoma. After partial resection, the patient was submitted to radiotherapy for residual disease with persistent symptoms. She developed growth hormone deficiency, central hypothyroidism, hypogonadism, and permanent diabetes insipidus. Six years later she was admitted for the suspicion of secondary adrenal insufficiency and thyrotoxicosis. Physical findings, laboratory data, thyroid ultrasound, and scintigraphy achieved the diagnosis of Graves' disease and hypocortisolism. She was treated with hydrocortisone and methimazole, but central hypothyroidism recurred after antithyroid drug withdrawal. Nine years after the diagnosis of a pituitary carcinoma, she maintains treatment with bromocriptine, has a locally stable disease, with no metastases.
This report highlights an unusual presentation of a prolactin-producing pituitary carcinoma in a young female. The patient had multiple hormone deficiencies due to a pituitary lesion and treatments. The posterior development of hyperthyroidism and adrenal insufficiency brought an additional difficulty to the approach.
垂体癌的诊断极为罕见,需要有转移病灶的证据,且总体生存率较低。恶性催乳素瘤常常需要多巴胺激动剂治疗、垂体手术、放疗,甚至化疗。
一名19岁女性出现溢乳、原发性闭经和左侧偏盲。辅助检查发现高催乳素血症以及一个侵犯海绵窦并向鞍上生长的垂体大腺瘤。她接受了卡麦角林和溴隐亭治疗,但临床症状和分析检查结果均无改善。计划进行垂体病变切除术,术中发现鼻腔黏膜有一个不连续的病灶。该转移灶导致了分泌催乳素的垂体癌的诊断。部分切除术后,患者因残留病灶且症状持续而接受了放疗。她出现了生长激素缺乏、中枢性甲状腺功能减退、性腺功能减退和永久性尿崩症。六年后,她因疑似继发性肾上腺皮质功能不全和甲状腺毒症入院。体格检查、实验室检查数据、甲状腺超声和闪烁扫描确诊为格雷夫斯病和皮质醇减退症。她接受了氢化可的松和甲巯咪唑治疗,但停用抗甲状腺药物后中枢性甲状腺功能减退复发。垂体癌诊断九年后,她继续接受溴隐亭治疗,局部病情稳定,无转移。
本报告强调了一名年轻女性分泌催乳素的垂体癌的不寻常表现。该患者因垂体病变和治疗出现了多种激素缺乏。随后出现的甲状腺功能亢进和肾上腺皮质功能不全给治疗带来了额外的困难。