Acromegaly and Cushing's Syndrome Associated with a Foregut Carcinoid Tumor*

Leveston, Steven A.; McKeel, Daniel W.; Buckley, Patrick J.; DeSchryver, Katherine; Greider, Marie H.; Jaffe, Bernard M.; Daughaday, William H.

doi:10.1210/jcem-53-4-682

Cited by 113 publications

(45 citation statements)

References 35 publications

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“…However, in two patients with metastatic lesions the primary tumour could not be discovered even after an extensive work-up. Both patients had a small primary duodenojejunal carcinoma that was detected at autopsy and laparatomy, respectively (Leveston et al, 1981;von Werder et al, 1984). Measurement of GHRH in blood specimens collected at multiple levels during selective venous catheterization failed to reveal the origin of GHRH hypersecretion in both our patients with an ectopic GHRH syndrome, one with the duodenojejunal carcinoma (von Werder et al, 1986) and the other with an intrathoracic carcinoid (unpublished observation), suggesting that this procedure may not be useful in localizing the source of ectopic secretion of GHRH.…”

Section: Radiological Findingsmentioning

confidence: 91%

“…3 years), a finding probably related to the more indolent course of bronchial carcinoids. Patients with intestinal endocrine tumours are probably younger than the other patients, as suggested by the fact that, of three such patients, two were 15 and 18 years old at the time of diagnosis (Leveston et al, 1981;von Werder et al, 1984).…”

Section: Clinical Aspectsmentioning

confidence: 99%

“…Carcinoids arising in the gastrointestinal tract were usually found in the duodenum and jejunum (Sano et al, 1988). Massive metastatic dissemination was present in two young patients (Leveston et al, 1981;von Werder et al, 1984).…”

Section: Morphology Of Ghrh-producing Tumoursmentioning

confidence: 99%

“…Sixteen patients had an already recognized primary tumour at the time of diagnosis of acromegaly and another 10 had symptoms, mainly due to airway obstruction (cough, episodic haemoptysis and recurrent pneumonia) or palpable abdominal masses, that led to the prompt discovery of the underlying neoplasm (Losa et al, 1993). Ectopic secretion of ACTH by the tumour, leading to Cushing's syndrome, was demonstrated in two patients by immunohistochemistry (Leveston et al, 1981;Southgate et al, 1988). Although mixed pituitary adenomas secreting GH and PRL or, more rarely, TSH have already been reported, at present the association of GH and ACTH hypersecretion is exceedingly rare (Blevins et al, 1992), thus the coexistence of acromegaly and Cushing's syndrome is suspicious of an ectopic syndrome.…”

Section: Clinical Aspectsmentioning

confidence: 99%

“…Second, symptoms not due to acromegaly itself, such as flushing, bleeding or obstruction of the lower respiratory airway and peptic ulcers could indicate the presence of carcinoids or other neuroendocrine tumours that are the most frequent cause of ectopic GHRH syndrome. The suspicion should be even greater when acromegalic patients manifest other uncommon diseases, such as Zollinger-Ellison syndrome (Wilson et al, 1984), Cushing's syndrome (Leveston et al, 1981), phaeochromocytoma (Roth et al, 1986) and hyperparathyroidism (Sano et al, 1987), caused by neuroendocrine tumours. Confirmation of the ectopic nature of acromegaly requires, nevertheless, the demonstration of an elevated plasma GHRH level.…”

Section: Differential Diagnosismentioning

confidence: 99%

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Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Losa¹,

Werder²

1997

Clinical Endocrinology

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Section: Radiological Findingsmentioning

confidence: 91%

Section: Clinical Aspectsmentioning

confidence: 99%

Section: Morphology Of Ghrh-producing Tumoursmentioning

confidence: 99%

Section: Clinical Aspectsmentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

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Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Losa¹,

Werder²

1997

Clinical Endocrinology

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Multihormonal carcinoid tumor of the pancreas. Secreting growth hormone-releasing factor as a cause of acromegaly

Berger

Trouillas

Bloch

et al. 1984

Cancer

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This report describes the histologic, immunocytochemical, and ultrastructural study of a multihormonal carcinoid tumor of the pancreas, secreting a growth hormone releasing factor (GRF) which provoked acromegaly. The patient presented a nonfamilial multiple endocrine neoplasia, type 1. The absence of radiologic signs of a pituitary adenoma in conjunction with elevated plasma levels of pancreatic polypeptide, glucagon, somatostatin, as well as growth hormone (GH), led to the discovery of the tumor. Its surgical excision produced a rapid disappearance of most of the clinical and biologic disorders. No immunoreactive GH was found in the tumor using radioimmunoassay and immunocyto‐chemistry. In contrast, three peptides with GH‐releasing activity were extracted and characterized. Immunocytochemistry showed that the GRF‐reactive cells, together with rare somatostatin‐storing cells, made up areas which demonstrated a medullary pattern of growth with extracellular amyloid deposits. Under electron microscopic examination, actively secreting cells were observed which carried endocrine granules of 100 to 150 nm in diameter. The other regions of the tumor presented a different type of growth and were composed of pancreatic polypeptide‐, glucagon‐, or somatostatin‐reacting cells. Cells immunostained with antisera raised against β‐endorphin were also noted. These data suggest that GRF may be a new biologic marker for pancreatic endocrine tumors.

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Acromegaly from ectopic growth hormone-releasing hormone secretion by a malignant carcinoid tumor.Successful treatment with long-acting somatostatin analogue SMS 201–995

Barkan

Shenker

Grekin

et al. 1988

Cancer

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A 26-year-old man with acromegaly secondary to ectopic growth hormone-releasing hormone (GHRH) secretion by a metastatic carcinoid tumor is the subject of this study. He previously failed to respond to conventional therapeutic modalities (partial hypophysectomy, pituitary irradiation, highdose bromo-criptine and a combination of streptomtwin and 5-fluorouracil) and was treated with long-acting so-matostatin analogue SMS 201-995 (Sandoz, East Hanover, NJ). Growth hormone and somatomedin C concentrations became normal, and GHRH-LI (GHRH-like immunoreactivity) was suppressed by more than 60%. The growth hormone response to exogenous GHRH 1-40 was stopped and growth hormone rise to thyrotropin-releasing hormone (TRH) was significantly attenuated. A significant shrinkage of the pituitary gland was observed. Similarly, the size of the metastatic carcinoid lesions decreased dramatically and was accompanied by a normalization of liver function. After almost 2 years of SMS 201-995 therapy, the patient was well and had no clinical signs of acromegaly. Thus, SMS 201-995 appears to be a remarkably effective agent for treatment of acromegaly secondary to ectopic GHRH secretion. Cuncer 61:221-226, 1988. HE CLINICAL AND BIOCHEMICAL syndrome of acro-T megaly usually stems from hypersecretion of growth hormone (GH) by benign pituitary somatotroph adenomas. This disease also rarely may result from ec-topic secretion of growth hormone-releasing hormone (GHRH) by malignant islet cell or carcinoid tumors.'d In these cases chronic stimulation by ectopic GHRH leads to the development of pituitary somatotroph hy-perplasia and GH hypersecretion. Removal of ectopic GHRH-producing tumors is followed by normalization of pituitary GH secretion and cure of acromegaly.' Unfortunately , by the time of diagnosis, most of the GHRH-producing tumors are already metastatic and not suitable for surgical resection. Standard chemother-From the *Endocrinology Section, VA Medical Center and the University of Michigan, Ann Arbor, Michigan, and the ?Clayton Founda-apy aimed at the ectopic GHRH-producing tumor is usually ineffe~tive,~-~ and attempts to control excessive GH secretion by "pituitary" approaches (surgery, radiation , or bromocriptine) are also unsuccessful because they cannot overcome the continuous stimulatory drive of ectopic GHRH.'*3*5 Thus, until recently, acromegaly secondary to metastatic GHRH-producing tumors was regarded as an untreatable disease. A long-acting somatostatin analogue, SMS 20 1-995, has a powerful suppressive effect on pituitary somato-trophs7s8 and has been shown to normalize plasma GH and to shrink pituitary tumors in patients with acro-megaly.' Additionally, this drug was found to suppress the hormone secretion by carcinoid" and gastrointesti-tumors. In four patients with unresectable GHRH-producing tumors treated thus far,49S*15*16 SMS 201-995 suppressed both GH and ectopic GHRH secretion. However, the GHRH-producing neoplasms did not shrink. We report the first case of ectopic GHRH-induced acromegaly successfully tr...

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Acromegaly and Cushing's Syndrome Associated with a Foregut Carcinoid Tumor*

Cited by 113 publications

References 35 publications

Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Pathophysiology and clinical aspects of the ectopic GH‐releasing hormone syndrome

Multihormonal carcinoid tumor of the pancreas. Secreting growth hormone-releasing factor as a cause of acromegaly

Acromegaly from ectopic growth hormone-releasing hormone secretion by a malignant carcinoid tumor.Successful treatment with long-acting somatostatin analogue SMS 201–995

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