Department of Endocrinology, Bellvitge University Hospital, Barcelona, Spain.
Department of Pathology; Bellvitge University Hospital, Barcelona, Spain.
Exp Clin Endocrinol Diabetes. 2020 Dec;128(12):835-844. doi: 10.1055/a-1120-8277. Epub 2020 Apr 14.
Phenotype transformation in pituitary adenomas (PA) is a little known and unexpected clinical phenomenon. We describe two illustrative cases and performed a systematic review of cases reported in literature.
: A 24-year-old woman underwent surgery because of Cushing's disease. A complete tumor resection and hypercortisolism resolution was achieved. Two years later, tumor recurred but clinical and hormonal hypercortisolism were absent. : A 77-year-old woman underwent surgery due to acromegaly. A complete tumor resection and GH excess remission was achieved. Four years later, tumor recurred but clinical and hormonal acromegaly was ruled out. : From 20 patients (including our cases), 75% were female with median age 45 (19) years. Ten patients (50%) had initially functioning PA: 8 switched to NFPA (5 ACTH-secreting PA, 2 prolactinomas and 1 acromegaly) and 2 exchanged to acromegaly from TSH-secreting PA and microprolactinoma. One patient developed a pituitary carcinoma from ACTH-secreting PA. Ten patients (50%) initially had NFPA; 9 developed Cushing's disease (4 silent corticotroph adenomas, 4 null cell PA and 1 managed conservatively). One patient with silent somatotroph PA changed to acromegaly. Treatments before transformation were surgery (80%), radiotherapy (40%), pharmacological (40%) and in 2 patients switching happened without any treatment. Median follow-up until transformation was 72 months (range 12-276).
PA can change from functioning to (NF) non-functioning (vice versa) and even exchange their hormonal expression. Clinicians should be aware and a careful lifelong follow-up is mandatory to detect it.
垂体腺瘤(PA)的表型转化是一种鲜为人知且出乎意料的临床现象。我们描述了两个说明性病例,并对文献中报道的病例进行了系统回顾。
一名 24 岁女性因库欣病接受手术。肿瘤完全切除,皮质醇增多症得到缓解。两年后,肿瘤复发,但无临床和激素性皮质醇增多症。一名 77 岁女性因肢端肥大症接受手术。肿瘤完全切除,生长激素过多得到缓解。四年后,肿瘤复发,但排除了临床和激素性肢端肥大症。从 20 名患者(包括我们的病例)中,75%为女性,中位年龄为 45(19)岁。10 名患者(50%)最初患有功能性 PA:8 名患者转变为 NFPA(5 例 ACTH 分泌性 PA、2 例泌乳素瘤和 1 例肢端肥大症),2 例从 TSH 分泌性 PA 和微泌乳素瘤转为肢端肥大症。1 例 ACTH 分泌性 PA 发展为垂体癌。10 名患者(50%)最初患有 NFPA;9 例发生库欣病(4 例无功能促肾上腺皮质激素腺瘤、4 例无功能细胞 PA 和 1 例保守治疗)。1 例无功能生长激素腺瘤患者转变为肢端肥大症。转变前的治疗方法为手术(80%)、放疗(40%)、药物治疗(40%),2 例患者在无任何治疗的情况下发生转变。转变前的中位随访时间为 72 个月(范围 12-276)。
PA 可以从功能性变为(NF)非功能性(反之亦然),甚至改变其激素表达。临床医生应该意识到这一点,需要进行仔细的终身随访以检测到这种情况。