A 36 year old female with symptoms of hyperparathyroidism, tumor in pancreas, adrenal cortical hyperplasia, pituitary adenomas, islet cell tumor with cutaneous angiofibromas. What is the diagnosis ?
Ans. is 'a' i.e. MEN 1 Angiofibromas are telangiectatic, skin coloured; pink or light brown papules that are 1-4 mm in diameter.They are mostly present on the face.Angiofibromas also occur in Tuberous sclerosis.The angiofibromas of MEN 1 tend to be smaller in size and less numerous than angiofibromas in patients with tuberous sclerosis.In addition angiofibromas in MEN I are common on the upper lip and vermilion border of the lip whereas angiofibromas in luberous sclerosis tends to spare the upper lip.Other features of MEN I syndrome have been discussed so many timesParathyroid adenomaPancreatic tumourPituitary tumourAdrenal cortical hyperplasiaMULTIPLE ENDOCRINE NEOPLASIA (MEN) SYNDROMES MEN IMEN 2AMEN 2BPituitaryAdenomas Q, Hyperplasia----ParathyroidHyperplasia QAdenomas QHyperplasia or adenoma Q--Pancreatic isletsHyperplasia QAdenomas QCarcinomas Q----AdrenalCortical hyperplasia QPheochromocytoma QPheochromocytoma QThyroid--C-cell hyperplasia Q Medullary carcinoma QC-cell hyperplasia Q Medullary carcinoma QExtra endocrineChangesForegut carcinoidSubcutaneous or visceralDermal angiofibroma or collagenomaCutaneous lichen amyloidosis Hirschsprung disease QMucosal and gastro intestinal neuroma, gangli o-neuromas, marfanoid habitusMutant gene locusMEN IRETRET
MEN 1
MEN 2 A
MEN 2 B
MEN 2 C
Endocrinology