Acromegalic gigantism and tuberous sclerosis

Hoffman, William H.; Perrin, J; Halac, Eduardo; Gala, Richard R.; England, Barry G.

doi:10.1016/s0022-3476(78)81170-6

Cited by 27 publications

(8 citation statements)

References 8 publications

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“…There are a few case reports addressing pituitary adenomas in TSC patients, producing GH-, prolactinor ACTH-secreting tumours (Galaction-Nitelea et al 1978, Hoffman et al 1978. Somatotroph tumours were reported in two cases (Galaction-Nitelea et al 1978, Hoffman et al 1978.…”

Section: Pituitarymentioning

confidence: 99%

“…Somatotroph tumours were reported in two cases (Galaction-Nitelea et al 1978, Hoffman et al 1978. In one of them the clinical diagnosis of acromegalic gigantism was reported in a boy who, at the age of 6 years, was diagnosed with TSC.…”

Section: Pituitarymentioning

confidence: 99%

“…One of the patient's siblings, a 10-year-old sister, was as mentally retarded with the typical skin findings of TSC; her height was appropriate to her age and her mean GH level on three separate fasting sera was 'within the normal range'. Interestingly, her serum prolactin was elevated, but her plain skull radiology and tomograms of the sella were normal (Hoffman et al 1978). In another case report, a female with TSC presenting with hyperprolactinaemia with amenorrhoea and galactorrhoea was reported; she was unresponsive to any treatment then available (including protirelin (TRH), chlorpromazine, levodopa, bromocriptine mesylate or oestrogens).…”

Section: Pituitarymentioning

confidence: 99%

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Are neuroendocrine tumours a feature of tuberous sclerosis? A systematic review

Dworakowska¹,

Grossman²

2009

Endocrine-Related Cancer

109

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Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem disorder characterised by the development of multiple hamartomas in numerous organs. It is caused by mutations of two tumour suppressor genes, TSC1 on chromosome 9q34 and TSC2 on chromosome 16p13.3, which encode for hamartin and tuberin respectively. The interaction between these two proteins, the tuberin-hamartin complex, has been shown to be critical to multiple intracellular signalling pathways, especially those controlling cell growth and proliferation. TSC may affect skin, central nervous system, kidneys, heart, eyes, blood vessels, lung, bone and gastrointestinal tract. Small series and case reports have documented that in tuberous sclerosis patients many endocrine system alterations might occur, affecting the function of the pituitary, parathyroid and other neuroendocrine tissue. There have been scattered reports of the involvement of such tissue in the pathological process of TSC, but no systematic review as to whether this is a true association. We have therefore systematically assessed all available published literature in this area. We conclude that there may be an association with pituitary and parathyroid tumours, and two recent descriptions of Cushing's disease are especially intriguing. However, the evidence seems more firm in the case of islet cell tumours, particularly insulinomas. As these latter may cause changes in mental state that may be confused with the cerebral manifestations of TSC per se, it is particularly important for physicians working with these patients to be aware of the putative and indeed likely association.

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Section: Pituitarymentioning

confidence: 99%

Section: Pituitarymentioning

confidence: 99%

Section: Pituitarymentioning

confidence: 99%

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Are neuroendocrine tumours a feature of tuberous sclerosis? A systematic review

Dworakowska¹,

Grossman²

2009

Endocrine-Related Cancer

109

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“…There is a growing body of evidence, though anecdotal, that TSC patients develop hormone-secreting tumors involving the neuroendocrine system at higher frequency than the general population. Cushing's disease,[12] hypoglycemia secondary to insulinomas,[3–7] precocious puberty,[89] thyrotoxicosis,[10] hypercalcemia secondary to parathyroid adenomas,[10–12] hyperprolactinemia,[13] and acromegaly[14] have all been reported in TSC patients. The circulating prolactin of our patient may be of pituitary origin or may possibly be secreted ectopically by a hamartoma.…”

mentioning

confidence: 99%

Endocrinopathy complicating a case of Tuberous sclerosis

Kota

Meher

Rao

et al. 2013

Indian J Endocr Metab

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“…Pituitary gigantism is a rare cause of tall stature in childhood, and reports are usually limited to sporadic cases (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). More comprehensive studies of acromegaly have been reported from adult centres and most of the available methods for dealing with growth hormone (GH) secreting pituitary adenomas have been evaluated in adults ( [14][15][16].…”

mentioning

confidence: 99%

Management of Pituitary Gigantism

et al. 1985

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True gigantism with overproduction of growth hormone (GH) and prolactin (PRL) was diagnosed in two boys, aged 13 years (case I) and 7 1/2 years (case II). Both had shown increased growth rates since early childhood (from 4 years and 1 1/2 years, respectively), but no skeletal acromegalic features were noted. However, both showed increased sweating and both had advanced pubic hair relative to testis volume. No other pituitary dysfunction was recorded. Case I underwent transsphenoidal surgery with only incomplete and temporary suppression of GH and PRL levels. However, in both patients bromocriptine administration promptly suppressed PRL levels. Following combined irradiation and bromocriptine treatment, GH also gradually normalized over a period of 2 years. Both boys are still on treatment, and both showed an increase in plasma GH concentrations when the dose of bromocriptine was reduced or discontinued, indicating that even 3 1/2-5 years after irradiation therapy (and during continuous treatment with bromocriptine) the disease was controlled but not cured. However, in these two boys bromocriptine has proved effective in controlling the PRL/GH oversecretion.

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Acromegalic gigantism and tuberous sclerosis

Cited by 27 publications

References 8 publications

Are neuroendocrine tumours a feature of tuberous sclerosis? A systematic review

Are neuroendocrine tumours a feature of tuberous sclerosis? A systematic review

Endocrinopathy complicating a case of Tuberous sclerosis

Management of Pituitary Gigantism

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