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Cushing Syndrome in Children: Symptoms, Etiology, and Localization of Pituitary Adenoma

This article discusses the symptoms, etiology, and localization of pituitary adenoma in children with Cushing Syndrome. It also explores the sensitivity and specificity of octreoscan in pituitary adenoma, as well as the use of desmopressin stimulation test for diagnosis.

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Cushing Syndrome in Children: Symptoms, Etiology, and Localization of Pituitary Adenoma

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  1. Problem list weight gain ,lack of height gain, cushingoid festurered purple wide striae,muscle weakness,easy bruisinglab: ACTH dependent cushing syndrom HDDST 》90% supression pituitary MRI :NL Chest & abdominopelvic ct : nodule (10 mm ) in left adrenal octreoscan :high uptake in pituitary gland

  2. QUESTIONS●Children and adult symptoms difference ●Etiology of CS in children●localization of Pituitary adenoma : Desmopression test IPSS●Ectopic pituitary adenoma ●sensitivity & specifity of octreoscan in pituitary adenoma

  3. ●approximately 10% of the new cases each year occur in children●As in adult patients, in children with Cushing syndrome there is a female-to-male predominance●The most common cause of Cushing syndrome in children is exogenous or iatrogenic Cushing syndrome●common cause of endogenous CS in children is CD(75% of all CS)●Adrenal causes are the most common causes in infant and young toddler ●In most children ,the onset of CS is incidious

  4. ●in childhood,lack of height gain concomitant with weight gain is the most common presentation of CS ●Growth failure and weight gain are frequently observed in subjects with ACTH -dependent CS , while in ACTH independent CS the secretion of adrenal androgens may lead to an acceleration of bone age and eventually compromise growth potential●other common problems reported in children include faciaL plethora,headaches ,hypertension,hirsutism,glucose intolerance ,kidney stone,fractures,delayed sexual development ●compared with adult patients with CS ,symptoms that are less commonly seen in children include sleep disruption , proximal myopathy

  5. ● Causes of short stature (GH deficiency, hypothyrodism ,malabsorption) should be considered● first step is to confirm or rule out the presence of CS ●LDDST in children weighing 》40 kg is same as adult ●in patients 《 40 kg a doseage of 30mcg/kg/day is recommended

  6. Desmopressin stimulation test●measure of plasma ACTH 0, 10, 20, 30 min after administration of 10 micg Desmopressin●patients with ACTH dependent CS show an increase in ACTH, but those with other causes of Cushing’s syndrome or those without Cushing’s syndrome do not respond●sensitivity for Cushing’s disease was 82–87%● sensitivity for Cushing’s syndrome was 63–75%● specificity ranged from 85 -91%

  7. some early data indicating that this test may also have a role in distinguishing between Cushing’s disease and ectopic ACTH secretion, subsequent studies failed to confirm this observationColombo et al in 1997 demonstrated a positive cortisol & ACTH response in 14 of 17 of patients with CD but not in 1 patient with suspected EASThis study suggested that a positive response to desmopressin might indicate CD, in analogy to the response to CRH, and led to studies aiming to evaluate the role of desmopressin in differential diagnosis of ACTH-dependent CS

  8. ROC curves; optimal cut-off for the percentage increase in ACTH after the CRH and DDAVP test for the purpose of identifying CD

  9. ●In fact, ROC curve analysis showed that there was not a good criterion that can distinguish between the two groups of CD and EAS patients●Overall, the available data do not support a role of the DT in the differential diagnosis of ACTH-dependent Cushing’s syndrome

  10. Ectopic pituitary adenoma●Pituitary adenomas are the most common lesion found in the sellar space, and ectopic pituitary adenomas (EPAs) are extremely rare●Ectopic pituitary adenomas are defined by the presence of adenomatous pituitary tissue outside the sella and distinctly separate from the pituitary ●from 1909, about 100 cases have been reported in the literature ●sphenoid sinus and suprasellar region are predominant locations

  11. ●sphenoid sinus : 36.9% ●suprasellar region : 27.7% ●clivus : 7.7% ●nasal cavity : 6.2% ●cavernous sinus : 6.2% ●parasellar region : 6.2% ●sphenoid wing : 3.1%●EPAs occur most frequently in the fourth to the seventh decades●EPAs in the early decades were found to be predominantly ACTH -secreting; those that occurred in the middle decades were prolactin-secreting; and those that presented in later decades were endocrineinactive

  12. ●Sphenoid sinus ectopic pituitary adenomas can manifest like a mass effect such as headache and nasal obstruction if large enough, or with symptoms hormonal excess●sphenoid sinus must be carefully screened if pituitary MRI is negative in the setting of hypercortisolism ●The adenohypophysis originates from the floor of the nasopharynx –Rathke’s pouch that is partly located in precursor tissue of the sphenoid bone●Ectopic pituitary tissue arise from remnants of Rathke’s pouch, and ectopic adenomas are believed to originate from remaining cells resting along the path of the embryological formation of the pituitary gland

  13. ●Type I are SPAs originating from pars distalis, the superior portion of the anterior pituitary tissue, and extending superiorly through the diaphragma sella ●Type II are SPAs originating from pars tuberalis, an elevatedportion of anterior pituitary tissue and mainly constitute pituitary stalK (Type IIa andType IIb)●Type III are originating from residual cells of Rathke’s pouch

  14. ●A54-year-old man presented with vertebral compression fracture and physical stigmata of Cushing syndrom(Cushingoid feature ,proximal muscle weakness,plethorea,easy bruising…)●Biochemical testing confirmed hypercortisolemia responsive to high dose dexamethasone suppression test

  15. ●MRI of the head demonstrated an enhancing mass in posterior aspect of the sphenoid sinus not involving the sella turcica●Endoscopic biopsy followed by resection confirmed this mass to be a pituitary adenoma with unusual pathologic features●Immunohistochemical profile of the tumor was typical of an ACTH secreting tumor, notably with positivity for ACTH●The patient did well from his surgery●Post-operatively his serum cortisol level normalized and he remains in chemical remission one year after surgery

  16. ACTH secretion in patients with untreated Cushing disease caused by pituitary microadenomas is not affected by octreotide The most important clinical use of somatostatin analogs in patients with cushing syndrom is in patients with ectopic ACTH secretion

  17. ●Wehave not carried out somatostatin receptor scientigraphy in patients with cushing disease ●application of this procedure in 4 patients with cushing syndrom caused by ectopic ACTH secretion allowed visualization of a medullary thyroid carcinoma as well as 3 lymph node metastasis , 2 small islet cell tumor with ACTH secretion●somatostatin receptor scientigraphy seems of no value in the diagnosis of pituitary Cushing disease ●It will be considerable value for differential diagnosis,allowing visualization of ectopic sources ofACTH secretion in most patients

  18. BIPSS●BIPSS generally is reserved for patients who have ACTH-dependent CS and anegative MRI or a positive MRI but inconsistent with CD data ●BIPSS has also been used for the purpose of lateralization of a pituitary adenoma ●Sensitivity for lateralization by BIPSS reported 60–90% before and after CRH stimulation but Information on BIPSS in children is limited

  19. ●94 children who admitted for CD between 1982- 2004 recorded●BIPSS procedure was done and Bilateral central and peripheral samples were taken for of ACTH levels before and after iv administration of 1g/kg CRH

  20. ●A central to peripheral (C/P) ACTH level ratio was calculated ●ratio of pre-oCRH administration of 2.0 or more and/or a C/P ratio of 3.0 or more post-CRH were considered evidence for the CD ●The ACTH levels were considered to indicate lateralization, if the interpetrosal sinus gradient(IPG)of the highest ACTH levels at the same time point was morethan 1.4●An IPG ratio less than 1.4 was considered as evidence of lack of lateralization and was used as evidence of a midline lesion●All 94 subjects underwent TSS and in 70 patients a tumor was clearly identified and selectively excised during surgery and In the 24patients,anadenoma was not identified at surgery; even though all of them were eventually cured

  21. ●lateralization of ACTH was frequent in the right than left(66% vs34%)●Lateralization on RS correctly identified the location of a tumor in78% , whereas on LS was correct in 56% of the patients After CRH stimulation(P=0.09)●After CRH , there were no differences in the sensitivity of the two lateralizing sides(67% vs 70% , P=0.14)

  22. ●In conclusion, BIPSS in children is a safe and well-tolerated procedure in experienced hands and remains the standard for the exclusion of ectopic ACTH production in the diagnostic work -up of CD●it is not a reliable test for lateralization of a pituitary adenoma●If BIPSS is needed for the definite exclusion ectopic ACTH -producing tumor in childhood, it may be used only in conjunction with imagings tudies, both pre-and intraoperatively,to guide the surgeon with regard to the location of the tumor

  23. ●Fifty-twoconsecutivepatientswithCDandnormalMRIwere enrolledbetween2009 -2015●SelectivecatheterizationofpetrosalsinusesforIPSSwasperformed●Allpatientsunderwentmicrosurgicaldissection,andifalesionwasfound,underwentlesionresection

  24. ●IPSS was performed using the same technique in all patients●Patients were considered to have Cushing’s disease when a threefold difference found between peripheral and central ACTH after CRH-induction●Patients whohada50%differencebetweensides were considered to have a significant lateralization

  25. ●This study showed a positive predictive value of 71%, These findings are similar to other studies published in the literature●However, in patients with a high burdenofdiseaseinwhomnolesionwas found,BIPSSandavery thorough microsurgical dissection were shown to have a 71% cure rate at 12 months●it hasspecificutilityinpreoperativeevaluation,especiallyifno radiologic abnormalities are found in high-power MRI.●patients confirmed to have Cushing’s disease without clear radiologic evidence of an ACTH-producing lesion should be taken to BIPSS to guide the best treatment course both in preoperative planning and intraoperative decision making

  26. Thanks for your attention

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