{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,10]],"date-time":"2025-09-10T22:27:02Z","timestamp":1757543222280,"version":"3.37.3"},"reference-count":29,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2015,10,5]],"date-time":"2015-10-05T00:00:00Z","timestamp":1444003200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2015,10,5]],"date-time":"2015-10-05T00:00:00Z","timestamp":1444003200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["World j. surg."],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Pheochromocytoma (PCC) manifests in up to 50 % of MEN2 patients. We correlated the clinico\u2010pathological features of MEN2\u2010associated PCC (MEN\u2010PCC) with <jats:italic>RET<\/jats:italic> mutations and compared them with non\u2010MEN adrenal\u2010PCCs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this retrospective single institution study on a large PCC database (<jats:italic>n<\/jats:italic> = 208, 1997\u20132014) 24\u00a0MEN\u2010PCC patients with known <jats:italic>RET<\/jats:italic> mutations were reviewed. Excluding 7 with incomplete data, the study cohort of 17\u00a0MEN\u2010PCC patients from 11 kindreds (M:F::7:10) was identified. Clinical, biochemical, pathological attributes, and outcomes in the MEN\u2010PCC group were correlated with the genotype, and further compared with non\u2010MEN, apparently sporadic adrenal\u2010PCCs (<jats:italic>n<\/jats:italic> = 132, excluding 37 extra\u2010adrenal and 15 VHL\/NF1\/SDH\u2010associated PCC).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Components of MEN2 encountered included MTC in 13(76.5 %), Marfanoid habitus in 2, and PHPT, cutaneous lichen amyloidosis and mucosal neuromas in 1 patient each. In 11(64.7 %), PCC was the first detected MEN2 component (Symptomatic:8, Incidentaloma:3). Four (23.5 %) were normotensive; 8(47.1 %) had bilateral PCC (7 synchronous, 1\u00a0metachronous). Surgery for PCC included laparoscopic adrenalectomy in 12; and cortical\u2010sparing adrenalectomy in 2 of 8 bilateral PCC patients. Mean MEN\u2010PCC tumor size was 6.9 \u00b1 3.9\u00a0cm, and 6(35 %) had additional adrenal medullary hyperplasia. Four different genotypes were encountered, commonest involving codon 634, others being 804 and 918. Mean age in MEN\u2010PCC (27.7 \u00b1 12.2 years) was lower than non\u2010MEN PCC (39.4 \u00b1 15.7, <jats:italic>p<\/jats:italic> = 0.018). Proportion of pediatric patients (35.3 % in MEN\u2010PCC vs. 12.9 % in non\u2010MEN\u2010PCC, <jats:italic>p<\/jats:italic> = 0.007), bilateral tumors (47.1 % in MEN\u2010PCC, 4.5 % in non\u2010MEN\u2010PCC, <jats:italic>p<\/jats:italic> &lt; 0.001), and adrenal medullary hyperplasia (35.2 % in MEN\u2010PCC, 0.7 % in non\u2010MEN\u2010PCC, <jats:italic>p<\/jats:italic> &lt; 0.001) were different. Median 24\u2010hour urinary metanephrines was significantly higher in index MEN\u2010PCC patients, than non\u2010MEN\u2010PCC (634 vs. 214\u00a0mcg\/24 h, <jats:italic>p<\/jats:italic> value = 0.006), but was non\u2010significantly higher in non\u2010index MEN\u2010PCC patients. Mean tumor sizes were comparable in the two groups. None of MEN\u2010PCC patients had malignant PCC, compared to 7(5.3 %) in non\u2010MEN\u2010PCC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In this cohort of MEN\u2010PCC from India, the commonest causative <jats:italic>RET<\/jats:italic> mutations for MEN\u2010PCC involved codon 634. MEN\u2010PCC patients were younger, and more frequently had bilateral PCC than non\u2010MEN disease. MEN\u2010PCC patients in India are diagnosed with large tumors and extremely high catecholamine\/metanephrine levels.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00268-015-3255-6","type":"journal-article","created":{"date-parts":[[2015,10,5]],"date-time":"2015-10-05T15:29:29Z","timestamp":1444058969000},"page":"690-696","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Genotype\u2013Phenotype Correlation in Indian Patients with MEN2\u2010Associated Pheochromocytoma and Comparison of Clinico\u2010Pathological Attributes with Apparently Sporadic Adrenal Pheochromocytoma"],"prefix":"10.1002","volume":"40","author":[{"given":"Sendhil","family":"Rajan","sequence":"first","affiliation":[{"name":"Department of Endocrine &amp; Breast Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS) Rae Bareilly Road 226014 Lucknow Uttar Pradesh 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