Table 2.

Characteristics of MEN 2A index cases presenting with PHPT as first manifestation.

Patient no.	Sex	RET mutation	PHPTa			MTCb		PHEOb		HSCRb	CLAb	Follow-up
			Age (yrs)	Histology	Symptoms	Age (yrs)	TNMc	Age (yrs)	Side			Age (yrs)	Status
1	F	C634Y	14	Hyperplasia	Y	14	T2N1M0		None	N	N	19	Alive
2	F	C634R	18	Adenoma	Y	18	T2N1M0	18	Bilaterald	N	N	30	Dead
3	M	C634Y	19	Adenoma	Y	19	T2N0M0	27	Unilateral	N	N	30	Alive
4	F	C634R	28	Hyperplasia	Y	28	T1N1M0	28	Unilateral	N	N	38	Alive
5	F	C634R	31	Adenoma	Y	46	T1N0N0	42	Bilateral	N	N	57	Alive
6	F	C634R	38	Hyperplasia	Y	38	T2N1M0	38	Bilateral	N	N	47	Alive
7	F	C611Y	40	Adenoma	Y	40	T1N1M0	40	Unilateral	N	N	47	Alive
8	M	C620R	61	Adenoma	Y	61	T3N1M1		None	N	N	75	Dead
9	F	E768D	61	Adenoma	Y	61	T1N1M0		None	N	N	66	Alive
10	F	C618F	68	Adenoma	NA	68	T2NxMx	80	Unilateral	N	N	90	Alive

^aDefined by biochemistry (30) and histology. ^bDefined by histology. ^cStaging was based on the American Joint Committee on Cancer seventh edition (31). ^dMalignant.

CLA, cutaneous lichen amyloidosis; HSCR, Hirschsprung disease; MEN 2A, multiple endocrine neoplasia type 2A; MTC, medullary thyroid carcinoma; N, no; NA, not available; PHEO, pheochromocytoma; PHPT, primary hyperparathyroidism; RET, rearranged during transfection; Y, yes.