Episodic hypertension is characteristic of:
Correct Answer: Pheochromocytoma
Description: Ans: C (Pheochromocytoma) Ref: Harrison's Principles of Internal Medicine, 18th edExplanation:"Classically, pheochromocytoma patients have episodic hypertension, but sustained hypertension is also common" Ref: Harrison.Other three options do not cause hypertension commonly.PHEOCHROMOCYTOMAPheochromocytomas are neoplasms composed of chromaffin cells, which synthesize and release catecholamines and in some instances peptide hormones.Traditionally, pheochromocytomas have been associated with a" rule of 10s"10% of pheochromocytomas are extra-adrenal, occurring in sites such as the organs of Zuckerkandl and the carotid body. Pheochromocytomas that develop in extra-adrenal paraganglia are designated paragangliomas.10% of sporadic adrenal pheochromocytomas are bilateral; this figure may rise to as high as 50% in cases that are associated with familial syndromes10% of adrenal pheochromocytomas are biologically malignant, defined by the presence of metastatic disease.10% of adrenal pheochromocytomas are not associated with hypertension. Of the 90% that present with hypertension, approximately two thirds have paroxysmal ' episodes associated with sudden rise in blood pressure and palpitations.Familial conditions assc with PheochromocytomaMultiple endocrine neoplasia, type 2 A (MEN-2a)Multiple endocrine neoplasia, type 2B (MEN-2b)Neurofibromatosis, type 1 (NF1)Von Hippel-Lindau (VHL)Familial paraganglioma 1, 2, 3 & 4- The tumors are composed of polygonal to spindle-shaped chromaffin cells or chief cells, clustered with the sustentacular cells into small nests or alveoli (zellhallen pattern) by a rich vascular network.
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