Saeger W, Schulte H M, Klöppel G
Virchows Arch A Pathol Anat Histopathol. 1986;409(4):547-54. doi: 10.1007/BF00705425.
A 54 year old woman suffered from acromegaly due to a pancreatic islet cell tumour producing GHRH. The tumour was demonstrated on CT scan. The diagnosis was established from elevated plasma levels of GHRH, GH and prolactin, and by the lack of signs of a pituitary adenoma in trans-sphenoidal surgery. Acromegaly was cured by tumour removal. Light microscopically, the tumour showed a medullary and microlobular pattern. The cells were large and often cuspidal. Small granules were found in semi-thin sections. Small aggregations of amyloid fibres were seen, mostly around capillaries. Immunocytochemistry revealed GHRH, NSE, neurotensin, serotonin, VIP and PP. S 100 was positive only in nerve fibres. Staining for GH, ACTH, calcitonin, alpha-HCG, beta-HCG, insulin, glucagon, gastrin, substance P, bombesin and somatostatin was negative. Ultrastructure showed oval partly lobulated nuclei with small nucleoli, moderate amounts of rough endoplasmic reticulum, many free ribosomes, some large Golgi fields and small numbers of secretory granules measuring 150 nm or, in a few cells, 650 nm. Only 4 other cases of pancreatic endocrine tumours causing acromegaly by ectopic GHRH secretion are described in the literature and these were similar to our case in many respects.
一名54岁女性因胰腺胰岛细胞瘤分泌生长激素释放激素（GHRH）而患肢端肥大症。CT扫描显示了该肿瘤。通过血浆GHRH、生长激素（GH）和催乳素水平升高，以及经蝶窦手术中未发现垂体腺瘤的迹象而确诊。肿瘤切除后治愈了肢端肥大症。光镜下，肿瘤呈髓样和微小叶状结构。细胞大，常呈尖状。在半薄切片中发现小颗粒。可见淀粉样纤维小聚集体，主要围绕毛细血管。免疫细胞化学显示有GHRH、神经元特异性烯醇化酶（NSE）、神经降压素、5-羟色胺、血管活性肠肽（VIP）和胰多肽（PP）。S-100仅在神经纤维中呈阳性。生长激素、促肾上腺皮质激素（ACTH）、降钙素、α-人绒毛膜促性腺激素（α-HCG）、β-人绒毛膜促性腺激素（β-HCG）、胰岛素、胰高血糖素、胃泌素、P物质、蛙皮素和生长抑素染色均为阴性。超微结构显示椭圆形、部分分叶的细胞核，核仁小，有中等量的粗面内质网、许多游离核糖体、一些大的高尔基体区域和少数直径为150nm的分泌颗粒，少数细胞中为650nm。文献中仅描述了另外4例因异位分泌GHRH导致肢端肥大症的胰腺内分泌肿瘤病例，这些病例在许多方面与我们的病例相似。
