A 5-hydroxytryptophan-secreting carcinoid tumour

Campbell, Andy; Gowenlock, A. H.; Platt, D; Snow, P.J.D.

doi:10.1136/gut.4.1.61

Cited by 25 publications

(6 citation statements)

References 17 publications

(13 reference statements)

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“…The histamine and 5-HT contents of the tumor were determined with specific high performance liquid chromatography methods [18,19] on specimens obtained from the hepatic lesions. The histamine content was high (30/zg/g), while the 5-HT content was low (0.2 p~g/g) in accordance with earlier reports on gastric carcinoids [20,21].…”

Section: Casesupporting

confidence: 91%

Two cases of gastric carcinoids: Diagnostic and therapeutic aspects

Ahlman¹,

Dahlström²,

Enerbäck³

et al. 1988

World j. surg.

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Two cases of gastric carcinoid tumors with the foregut carcinoid syndrome are presented. Due to elevated urinary levels of 5-hydroxyindoleacetic acid (5-HIAA), primary tumors of midgut origin were suspected. Provocation with pentagastrin (PG) elicited atypical flushing and severe hronchoconstriction, most probably caused by the release of histamine. Excessive excretion of the main histamine metabolite, tele-methylimid-az01e acetic acid (MeImAA), in urine of both patients was demonstrated. Both patients had good symptomatic relief with prednisolone and blockade of histamine receptors. One of the patients had excessive secretion of serotonin (5-HT) as well as of histamine. Peripheral blockade of 5-HT2receptors was therefore added to the medical treatment. This patient had a subjective and biochemical response to cytotoxic treatment with streptozutocin and later responded well to a somatostatin analogue.On clinical suspicion of a foregut carcinoid tumor, or in patients with carcinoid tumors without anatomical localization, PG provocation should not be used until the excretion of MeImAA has been determined.

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Section: Casesupporting

confidence: 91%

Two cases of gastric carcinoids: Diagnostic and therapeutic aspects

Ahlman¹,

Dahlström²,

Enerbäck³

et al. 1988

World j. surg.

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“…Especially in foregut carcinoids, urinary serotonin excretion can be elevated, while platelet serotonin or urinary 5-HIAA is normal. The assumed mechanism is that forgut carcinoids lack AADC, as evidenced by elevated 5-HTP levels in such patients (97,98). Chromogranin A is useful as general neuroendocrine tumour marker in patients with suspected ectopic ACTH secretion.…”

Section: Laboratory Studiesmentioning

confidence: 99%

Cyclic Cushing’s syndrome: a clinical challenge

Meinardi¹,

Wolffenbuttel²,

Dullaart³

2007

eur j endocrinol

126

134

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Cyclic Cushing's syndrome (CS) is a rare disorder, characterized by repeated episodes of cortisol excess interspersed by periods of normal cortisol secretion. The so-called cycles of hypercortisolism can occur regularly or irregularly with intercyclic phases ranging from days to years. To formally diagnose cyclic CS, three peaks and two troughs of cortisol production should be demonstrated. Our review of 65 reported cases demonstrates that cyclic CS originates in 54% of cases from a pituitary corticotroph adenoma, in 26% from an ectopic ACTH-producing tumour and in about 11% from an adrenal tumour, the remainder being unclassified. The pathophysiology of cyclic CS is largely unknown. The majority of patients with cyclic CS have clinical signs of CS, which can be either fluctuating or permanent. In a minority of patients, clinical signs of CS are absent. The fluctuating clinical picture and discrepant biochemical findings make cyclic CS extremely hard to diagnose. Clinicians should therefore be aware of this clinical entity and actively search for it in all patients with suspected CS but normal biochemistry or vice versa. Frequent measurements of urinary cortisol or salivary cortisol levels are a reliable and convenient screening tool for suspected cyclic CS. Cortisol stimulation or suppression tests may give spurious results owing to spontaneous falls or rises in serum cortisol at the time of testing. When cyclic CS is biochemically confirmed, further imaging and laboratory studies are guided by the presence or absence of ACTH dependency. In cases of suspected ectopic ACTH production, specific biochemical testing for carcinoids or neuroendocrine tumours is required, including measurements of serotonin in platelets and/or urine, chromogranin A and calcitonin.European Journal of Endocrinology 157 245-254

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“…9 The liver inactivates many of the humoral factors liberated by foregut GEP-ETs; hence, the presence of the carcinoid syndrome implies presence of liver metastases, since the secretory products liberated from these hepatic lesions drain directly into the systemic circulation. 2 Foregut GEP-ETs are also capable of secreting excessive quantities of histamine, 10 which may explain the intense pruritis experienced in this patient.…”

Section: Discussionmentioning

confidence: 93%

“…2 Foregut GEP-ETs are also capable of secreting excessive quantities of histamine, 10 which may explain the intense pruritis experienced in this patient.…”

Section: Discussionmentioning

confidence: 99%

A patient with a metastatic gastroenteropancreatic endocrine carcinoma causing hyperinsulinaemic hypoglycaemia and the carcinoid syndrome

et al. 2011

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We present the case of a 57-year-old patient who initially presented with a constellation of symptoms including intense pruritis, flushing and diarrhoea. Following several months clinical deterioration, the patient was investigated radiologically, where multiple hepatic tumours were identified. Liver biopsy confirmed the presence of a well-differentiated metastatic gastroenteropancreatic endocrine carcinoma with biochemical evidence of serotonin secretion. Over a period of six months, the clinical course of the patient's disease progressed whereby severe hypoglycaemia became the major manifestation. Subsequent biochemical investigations confirmed the diagnosis of an insulinoma. Extensive radiological investigation revealed a solitary primary pancreatic tumour, indicating the presence of a metastatic pancreatic endocrine tumour (PET) secreting both insulin and serotonin. The patient was treated with a chemotherapy regimen consisting of 12 cycles of 5-fluorouracil/oxaliplatin, responding clinically -improved World Health Organization performance score from 3 to 1, biochemically -significantly reduced plasma chromogranin A and cancer antigen 19-9 concentrations and improved liver function tests, and radiologically -reduced pancreatic and hepatic tumour size. This is the first report of a primary PET secreting insulin and serotonin. Due to the association of serotonin-secreting gastroenteropancreatic endocrine tumours (GEP-ETs) with multiple endocrine neoplasia type-1 (MEN1) and biochemical evidence of an insulinoma, MEN1 should also be considered in such cases. The case provides further evidence for the biological heterogeneity of GEP-ETs and the myriad secretory humoral products and resultant clinical syndromes arising from such tumours.

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A 5-hydroxytryptophan-secreting carcinoid tumour

Cited by 25 publications

References 17 publications

Two cases of gastric carcinoids: Diagnostic and therapeutic aspects

Two cases of gastric carcinoids: Diagnostic and therapeutic aspects

Cyclic Cushing’s syndrome: a clinical challenge

A patient with a metastatic gastroenteropancreatic endocrine carcinoma causing hyperinsulinaemic hypoglycaemia and the carcinoid syndrome

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