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一例罕见的分泌催乳素垂体癌伴硬膜外和脑脊膜转移

A Rare Prolactin-secreting Pituitary Carcinoma With Epidural and Thecal Metastases.

作者信息

Liu Anna, Van Uum Stan, Lee Donald, Hammond Robert R, Ezzat Shereen, Clemens Kristin K

机构信息

Schulich School of Medicine and Dentistry, Western University, London, ON N6A 3K7, Canada.

Center for Diabetes, Endocrinology and Metabolism, St. Joseph's Health Care London, London, ON N6A 4V2, Canada.

出版信息

JCEM Case Rep. 2024 Apr 24;2(5):luae047. doi: 10.1210/jcemcr/luae047. eCollection 2024 May.

Abstract

Pituitary carcinomas are rare but associated with significant morbidity and mortality. They remain challenging to diagnose and manage. In this case, we describe a 56-year-old man who presented with erectile dysfunction and binocular vertical diplopia. He had central hypogonadism, secondary adrenal insufficiency, and central hypothyroidism on biochemical testing. His serum prolactin was 1517 mcg/L (1517 ng/mL; reference range 4-15 mcg/L), and his sellar magnetic resonance imaging showed a 2.0 × 2.2 × 3.1 cm pituitary tumor. Pathology revealed a prolactin-secreting carcinoma. Despite treatment with a high-dose dopaminergic, 2 transsphenoidal resections, and 1 course of radiation, prolactin levels continued to rise. He developed metastases to the epidural space and thecal sac from the thoracic to sacral spine, for which he received 12 cycles of temozolomide chemotherapy with initial clinical and biochemical response. This was followed by disease escape and progression. We discuss the clinical and imaging features that warrant a high index of suspicion for pituitary carcinoma and review contemporary treatment.

摘要

垂体癌罕见,但与显著的发病率和死亡率相关。其诊断和管理仍然具有挑战性。在此病例中,我们描述了一名56岁男性,他出现勃起功能障碍和双眼垂直复视。生化检查显示他存在中枢性性腺功能减退、继发性肾上腺功能不全和中枢性甲状腺功能减退。他的血清催乳素为1517微克/升(1517纳克/毫升;参考范围4 - 15微克/升),蝶鞍磁共振成像显示有一个2.0×2.2×3.1厘米的垂体瘤。病理检查显示为分泌催乳素的癌。尽管接受了高剂量多巴胺能药物治疗、2次经蝶窦切除术和1个疗程的放疗,催乳素水平仍持续升高。他出现了从胸椎至骶椎硬膜外间隙和硬膜囊的转移,为此他接受了12个周期的替莫唑胺化疗,最初有临床和生化反应。随后疾病复发并进展。我们讨论了需要高度怀疑垂体癌的临床和影像学特征,并回顾当代治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/453f/11040273/ab3268d74ca8/luae047f1.jpg

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