Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome

Arzamendi, Audrey E.; Shahlaie, Kiarash; Latchaw, Richard E.; Lechpammer, Mirna; Arzumanyan, Hasmik

doi:10.1055/s-0036-1585091

Cited by 9 publications

(4 citation statements)

References 23 publications

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“…In fact, an alteration of PRL circadian rhythm has been described in PES, associated with a chronic or intermittent altered intracranial pressure: the nightly PRL increment is reduced or completely lacked in patients with intracranial hypertension, even just during the REM phase (47,48). Rarely, pituitary hypersecretion condition was described in patients with PES associated to ectopic GH-and ACTHsecreting pituitary adenomas (49,50), located within the midline intersphenoidal septum. dynamics and possible immune system reactions.…”

Section: Endocrinological Picturementioning

confidence: 99%

DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review

Chiloiro¹,

Giampietro²,

Bianchi³

et al. 2017

European Journal of Endocrinology

120

149

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Primary empty sella (PES) is characterized by the herniation of the subarachnoid space within the sella, which is often associated with variable degrees of flattening of the pituitary gland in patients without previous pituitary pathologies. PES pathogenetic mechanisms are not well known but seem to be due to a sellar diaphragm incompetence, associated to the occurrence of upper sellar or pituitary factors, as intracranial hypertension and change of pituitary volume. As PES represents in a majority of cases, a neuroradiological findings without any clinical implication, the occurrence of endocrine, neurological and opthalmological symptoms, due to the above describes anatomical alteration, which delineates from the so called PES syndrome. Headache, irregular menses, overweight/obesity and visual disturbances compose the typical picture of PES syndrome and can be the manifestation of an intracranial hypertension, often associated with PES. Although hyperprolactinemia and growth hormone deficit represent the most common endocrine abnormalities, PES syndrome is characterized by heterogeneity both in clinical manifestation and hormonal alterations and can sometime reach severe extremes, as occurrence of papilledema, cerebrospinal fluid rhinorrhea and worsening of visual acuity. Consequently, a multidisciplinary approach, with the integration of endocrine, neurologic and ophthalmologic expertise, is strongly advocated and recommended for a properly diagnosis, management, treatment and follow-up of PES syndrome and all of the related abnormalities.

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Section: Endocrinological Picturementioning

confidence: 99%

DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review

Chiloiro¹,

Giampietro²,

Bianchi³

et al. 2017

European Journal of Endocrinology

120

149

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“…However, acromegaly in patients with PES is exceedingly rare and limited to a handful of case reports and a few retrospective studies. In a single retrospective study of 76 patients with ES, only 3 patients were diagnosed with acromegaly ( 9 , 15 , 16 , 17 , 18 ). Following this, three retrospective studies conducted between 2004 and 2017 reported acromegaly with ES in 20.3% ( 19 ), 22% ( 20 ), and 15.4% ( 21 ) of cases, respectively.…”

Section: Discussionmentioning

confidence: 99%

Acromegaly with empty sella syndrome

Daya

Seedat

Purbhoo

et al. 2021

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Acromegaly is a rare, chronic progressive disorder with characteristic clinical features caused by persistent hypersecretion of growth hormone (GH), mostly from a pituitary adenoma (95%). Occasionally, ectopic production of GH or growth hormone-releasing hormone (GHRH) with resultant GH hypersecretion may lead to acromegaly. Sometimes localizing the source of GH hypersecretion may prove difficult. Rarely, acromegaly has been found in patients with an empty sella (ES) secondary to prior pituitary radiation and/or surgery. However, acromegaly in patients with primary empty sella (PES) is exceeding rarely and has only been described in a few cases. We describe a 47-year-old male who presented with overt features of acromegaly (macroglossia, prognathism, increased hand and feet size). Biochemically, both the serum GH (21.6 μg/L) and insulin-like growth factor 1 (635 μg/L) were elevated. In addition, there was a paradoxical elevation of GH following a 75 g oral glucose load. Pituitary MRI demonstrated an ES. In order to exclude an ectopic source of GH hypersecretion, further biochemical tests and imaging were done, which were unremarkable. Notably, increased uptake in the sella turcica on the 68Gallium DOTATATE PET/CT confirmed the ES as the likely source of GH secretion. As no overt lesion was noted, medical treatment (octreotide acetate) was initiated with a good clinical and biochemical response. At his 3 month follow-up, he reported an improvement in symptoms (fatigue and headache), however he still complained of low libido. Due to a persistently low testosterone level at follow-up, a long-acting testosterone was initiated. His GH level normalised, and IGF-1 has significantly reduced. Learning points The commonest cause of acromegaly is due to GH hypersecretion from pituitary adenomas (95%). Acromegaly has rarely been found in patients with ES. It is important to exclude a past history suggestive of pituitary apoplexy. Extra-pituitary source of GH such as ectopic production of GHRH with resultant GH hypersecretion needs to be excluded. In such cases, since there is no resectable mass, medical therapy is the primary treatment option.

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“…There was consensus to assess the ectopic production of GH-releasing hormone (GHRH) in case of biochemical diagnosis of acromegaly and an image of empty sella (S12). Primary empty sella syndrome is frequently associated to GH deficiency; [18] thus, the coexistence of this image with acromegaly might be explained by the existence of GHsecreting pituitary microadenomas or an ectopic pituitary adenoma [19][20][21], or more likely, by an ectopic secretion of GHRH, usually by a bronchial carcinoid tumor [22][23][24]. Both of these explanations might apply in the case of a biochemical diagnosis of acromegaly and no tumor image in the pituitary gland MRI [22,25,26].…”

Section: Diagnosismentioning

confidence: 99%

Diagnosis, treatment and follow-up of patients with acromegaly in a clinical practice setting in Spain: the ACROPRAXIS program Delphi survey

et al. 2019

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Aim The ACROPRAXIS program aims to describe the management of acromegaly in Spain and provide guidance. Methods Ninety-three endocrinologists were organized into 13 panels to discuss the practical issues in managing acromegaly. Based on the key learnings, an online Delphi survey with 62 statements was performed, so those statements achieving consensus could be used as guidance. Statements were rated on a 9-point scale (9, full agreement; consensus > 66.6% of response in the same tertile). Results Ninety-two endocrinologists (98.8%) answered two rounds of the survey (mean age 47.6 years; 59.8% women; median 18.5 years of experience). Consensus was achieved for 49 (79%) statements. Diagnosis: The levels of insulin-like growth factor I (IGFI) is the preferred screening test. If IGFI levels 1–1.3 ULN, the test is repeated and growth hormone (GH) after oral glucose tolerance test (OGTT) is assessed. A pituitary magnetic resonance is performed after biochemical diagnosis. Treatment: Surgery is the first treatment choice for patients with microadenoma or macroadenoma with/without optical pathway compression. Pre-surgical somatostatin analogues (SSA) are indicated when surgery is delayed and/or to reduce anaesthesia-associated risks. After unsuccessful surgery, reintervention is performed if the residual tumor is resectable, while if non-resectable, SSA are administered. Follow-up First biochemical and clinical controls are performed 1–3 months after surgery. Disease remission is considered if random GH levels are < 1 µg/L or OGTT is < 1 or ≤ 0.4 µg/L, depending on the assay’s sensitivity. Conclusion Current clinical management for acromegaly is homogeneous across Spain and generally follows clinical guidelines.

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Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome

Cited by 9 publications

References 23 publications

DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review

DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review

Acromegaly with empty sella syndrome

Diagnosis, treatment and follow-up of patients with acromegaly in a clinical practice setting in Spain: the ACROPRAXIS program Delphi survey

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