FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4307860219> ?p ?o ?g. }

• W4307860219 endingPage "A93" @default.
• W4307860219 startingPage "A92" @default.
• W4307860219 abstract "Abstract Background Adrenal medullary hyperplasia (AMH) is arbitrarily defined by adrenal medullary proliferation measuring less than 1 cm while larger lesions are considered pheochromocytoma. Some patients with MEN2 present with adrenal medullary hyperplasia (AMH), which is a precursor of pheochromocytomas. Clinical Case A 22-year-old female with a past medical history of MEN2A. She is positive for germline mutation in RET proto-oncogene at 10q11.2 exon 11, position 634 from cysteine to arginine. She was evaluated for a 1cm left thyroid nodule, which required total thyroidectomy for Medullary Thyroid Cancer. Pathology revealed a 0.5cm deposit on the largest dimension, clear margins, and no angiovascular, or lymphovascular invasion, surgery was complicated by hypoparathyroidism. She complained of intermittent anxiety and fatigue but was otherwise asymptomatic. Physical examination is unremarkable except for thyroidectomy scar and multiple exostoses. On 8/5/2011 plasma-free metanephrines were elevated at 83 pg/ml (&lt;57pg/ml) and Free normetanephrine was 45 pg/ml (&lt;145pg/ml). Over the next years, her plasma-free metanephrine levels have been checked approximately every 6 months ranging between 63-106pg/ml with free plasma normetanephrine in normal ranges (30-81pg/ml). However, on 2/2021, her plasma normetanephrine level increased to 304pg/ml. More recently, on 11/30/2021 her free normetanephrine levels reached 671pg/ml and free Metanephrine 131pg/ml. She had two MRIs of the abdomen and pelvis over the years and both showed unremarkable adrenal glands. NM I-123 MIBG scintigraphy reported physiologic symmetric activity in the adrenal glands. Finally, she had GA-68 DOTATATE PET/CT with no suspicious radiotracer avid lesion in the abdominopelvic organs. Given the persistent elevation on plasma metanephrines, we decided to start the patient on Doxazosin prophylactically while we continue biochemical and imaging surveillance. Conclusion Our patient has a persistent elevation in plasma-free metanephrines with no evidence of pheochromocytoma in abdominal imaging. The earliest abnormality in the adrenal medulla in patients with MEN 2 is diffuse hyperplasia with a reduction in the cortical/medullary ratio (1). This hyperplasia is considered to be a precursor of pheochromocytoma in patients with MEN 2 and in our case the most likely explanation for the progressive elevation in plasma-free metanephrines (1). There is a strong molecular relationship between AMH and Pheochromocytoma (2), close monitoring for progression to pheochromocytoma is necessary for all patients with AMH. The presence of markedly elevated plasma metanephrines in an asymptomatic patient with no evidence of pheochromocytoma poses a therapeutic dilemma for preemptive use of alpha blockade and the potential need for a medical alert bracelet. References (1) Cho, K et al Adrenal Medullary disease in Multiple Endocrine Neoplasia II. AJR 134: 23-29. (2) Korpershoek, E et al Adrenal Medullary Hyperplasia Is a Precursor Lesion for Pheochromocytoma in MEN2 Syndrome. Neoplasia. 2014 Oct; 16(10): 868–873 Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m." @default.
• W4307860219 created "2022-11-06" @default.
• W4307860219 creator A5007305932 @default.
• W4307860219 creator A5014048388 @default.
• W4307860219 creator A5021415720 @default.
• W4307860219 date "2022-11-01" @default.
• W4307860219 modified "2023-09-26" @default.
• W4307860219 title "PSAT013 Elevated Plasma Free Metanephrines with No Evidence of Pheochromocytoma in a Patient with Multiple Endocrine Neoplasia Type 2A" @default.
• W4307860219 doi "https://doi.org/10.1210/jendso/bvac150.189" @default.
• W4307860219 hasPublicationYear "2022" @default.
• W4307860219 type Work @default.
• W4307860219 citedByCount "0" @default.
• W4307860219 crossrefType "journal-article" @default.
• W4307860219 hasAuthorship W4307860219A5007305932 @default.
• W4307860219 hasAuthorship W4307860219A5014048388 @default.
• W4307860219 hasAuthorship W4307860219A5021415720 @default.
• W4307860219 hasBestOaLocation W43078602191 @default.
• W4307860219 hasConcept C104317684 @default.
• W4307860219 hasConcept C126322002 @default.
• W4307860219 hasConcept C126894567 @default.
• W4307860219 hasConcept C134018914 @default.
• W4307860219 hasConcept C13514818 @default.
• W4307860219 hasConcept C142724271 @default.
• W4307860219 hasConcept C185592680 @default.
• W4307860219 hasConcept C2776512331 @default.
• W4307860219 hasConcept C2776734335 @default.
• W4307860219 hasConcept C2776783082 @default.
• W4307860219 hasConcept C2779161898 @default.
• W4307860219 hasConcept C2779505177 @default.
• W4307860219 hasConcept C2779512018 @default.
• W4307860219 hasConcept C2779761222 @default.
• W4307860219 hasConcept C2780103800 @default.
• W4307860219 hasConcept C2780120053 @default.
• W4307860219 hasConcept C501734568 @default.
• W4307860219 hasConcept C526584372 @default.
• W4307860219 hasConcept C55493867 @default.
• W4307860219 hasConcept C71924100 @default.
• W4307860219 hasConceptScore W4307860219C104317684 @default.
• W4307860219 hasConceptScore W4307860219C126322002 @default.
• W4307860219 hasConceptScore W4307860219C126894567 @default.
• W4307860219 hasConceptScore W4307860219C134018914 @default.
• W4307860219 hasConceptScore W4307860219C13514818 @default.
• W4307860219 hasConceptScore W4307860219C142724271 @default.
• W4307860219 hasConceptScore W4307860219C185592680 @default.
• W4307860219 hasConceptScore W4307860219C2776512331 @default.
• W4307860219 hasConceptScore W4307860219C2776734335 @default.
• W4307860219 hasConceptScore W4307860219C2776783082 @default.
• W4307860219 hasConceptScore W4307860219C2779161898 @default.
• W4307860219 hasConceptScore W4307860219C2779505177 @default.
• W4307860219 hasConceptScore W4307860219C2779512018 @default.
• W4307860219 hasConceptScore W4307860219C2779761222 @default.
• W4307860219 hasConceptScore W4307860219C2780103800 @default.
• W4307860219 hasConceptScore W4307860219C2780120053 @default.
• W4307860219 hasConceptScore W4307860219C501734568 @default.
• W4307860219 hasConceptScore W4307860219C526584372 @default.
• W4307860219 hasConceptScore W4307860219C55493867 @default.
• W4307860219 hasConceptScore W4307860219C71924100 @default.
• W4307860219 hasIssue "Supplement_1" @default.
• W4307860219 hasLocation W43078602191 @default.
• W4307860219 hasLocation W43078602192 @default.
• W4307860219 hasOpenAccess W4307860219 @default.
• W4307860219 hasPrimaryLocation W43078602191 @default.
• W4307860219 hasRelatedWork W1854786812 @default.
• W4307860219 hasRelatedWork W2008799564 @default.
• W4307860219 hasRelatedWork W2011397535 @default.
• W4307860219 hasRelatedWork W2070971732 @default.
• W4307860219 hasRelatedWork W3034279707 @default.
• W4307860219 hasRelatedWork W3195463543 @default.
• W4307860219 hasRelatedWork W4225165330 @default.
• W4307860219 hasRelatedWork W4307860219 @default.
• W4307860219 hasRelatedWork W2102763509 @default.
• W4307860219 hasRelatedWork W2326488753 @default.
• W4307860219 hasVolume "6" @default.
• W4307860219 isParatext "false" @default.
• W4307860219 isRetracted "false" @default.
• W4307860219 workType "article" @default.