Cheung Kitty Kit Ting, Chow Francis Chun Chung, Lo Anthony Wing Ip
Departement of Medicine and Therapeutics, The Chinese University of Hong Kong, Shatin, Hong Kong.
Division of Anatomical Pathology, Queen Mary Hospital, Pokfulam, Hong Kong.
BMJ Case Rep. 2016 Jun 9;2016:bcr2016215183. doi: 10.1136/bcr-2016-215183.
Ectopic-acromegaly is rare. Diagnosing ectopic-acromegaly is challenging given that the clinical and biochemical manifestations can be almost indistinguishable from those of patients with growth hormone secreting pituitary adenomas. This case report highlights the importance of clinical vigilance in differentiating between the two conditions. A 41-year-old Caucasian man presented with typical features of acromegaly with an enlarged pituitary and a lung lesion. Although excision of the lung mass showed a carcinoid tumour, normalisation of growth hormone factors did not occur soon enough. This led to a presumed diagnosis of a pituitary adenoma. However, no pituitary tumour was identified during trans-sphenoidal surgery. Postoperatively, the patient improved clinically and biochemically. Retrospective histological examination of the excised lung lesion showed a small proportion of tumour cells containing growth hormone releasing hormone (GHRH), suggesting ectopic-GHRH production from the lung neuroendocrine tumour. An open-and-close trans-sphenoidal surgery could have been avoided in this patient with ectopic-GHRH acromegaly.
异位性肢端肥大症很罕见。鉴于异位性肢端肥大症的临床和生化表现几乎与生长激素分泌型垂体腺瘤患者的表现无法区分,因此诊断异位性肢端肥大症具有挑战性。本病例报告强调了临床警惕性在区分这两种疾病方面的重要性。一名41岁的白种男性表现出典型的肢端肥大症特征,垂体增大且肺部有病变。尽管切除肺部肿块显示为类癌肿瘤,但生长激素因子并未足够快地恢复正常。这导致初步诊断为垂体腺瘤。然而,经蝶窦手术期间未发现垂体肿瘤。术后,患者在临床和生化方面均有改善。对切除的肺部病变进行回顾性组织学检查发现,一小部分肿瘤细胞含有生长激素释放激素(GHRH),提示肺部神经内分泌肿瘤产生异位GHRH。对于这名患有异位GHRH型肢端肥大症的患者,本可避免进行经蝶窦开放-闭合手术。