Sassolas G, Chayvialle J A, Partensky C, Berger G, Trouillas J, Berger F, Claustrat B, Cohen R, Girod C, Guillemin R
Ann Endocrinol (Paris). 1983;44(6):347-54.
In exceptional cases, acromegaly develops as the clinical expression of an ectopic secretion of Growth Hormone (GH) or Growth Hormone-Releasing Factor (GRF), tumorous in origin. In the present report, we describe an instance of acromegaly caused by the secretion of GRF from a voluminous pancreatic tumor. The resection of this tumor resulted in a temporary disappearance of the biological and clinical symptoms of acromegaly, which then reappeared in conjunction with a rise in plasma GRF. From this pancreatic tumor, substances displaying a potent GRF activity were isolated and characterized. Amino acid analyses revealed that they were related to 3 peptides containing respectively 44, 40 and 37 aminoacids. The largest (hp GRF (1-44)-NH2) referred as hp GRF or somatocrinin is considered to be the primary molecule. The pancreatic tumor was multisecreting as proved by high plasma levels of somatostatin, pancreatic polypeptide and glucagon, normalized after the tumor removal, taken together with the immunocytochemical demonstration of the presence of these peptides in the tissue and with the isolation of somatostatin. In contrast hypercalcemia associated with an elevated plasma level of IR-PTH was unmodified by tumor removal. Diagnosis of acromegaly as ectopic endocrine syndrome will probably be facilitated by plasma GRF radioimmunoassay, as a result of production of anti synthetic GRF antibodies.
在特殊情况下，肢端肥大症表现为生长激素（GH）或生长激素释放因子（GRF）异位分泌的临床症状，起源于肿瘤。在本报告中，我们描述了一例由巨大胰腺肿瘤分泌GRF导致的肢端肥大症病例。切除该肿瘤后，肢端肥大症的生物学和临床症状暂时消失，但随后随着血浆GRF水平升高而再次出现。从该胰腺肿瘤中分离并鉴定出具有强大GRF活性的物质。氨基酸分析表明，它们与分别含有44、40和37个氨基酸的3种肽有关。最大的肽（hp GRF (1-44)-NH2），称为hp GRF或生长激素释放素，被认为是主要分子。胰腺肿瘤具有多种分泌功能，血浆中生长抑素、胰多肽和胰高血糖素水平升高证明了这一点，肿瘤切除后这些水平恢复正常，同时组织中这些肽的免疫细胞化学证明以及生长抑素的分离也证实了这一点。相比之下，与血浆中免疫反应性甲状旁腺激素（IR-PTH）水平升高相关的高钙血症在肿瘤切除后未得到改善。由于抗合成GRF抗体的产生，血浆GRF放射免疫测定可能有助于将肢端肥大症诊断为异位内分泌综合征。
