ETIOPATHOLOGY
OF PRIMARY HYPERPARATHYROIDISM IN MEN2 A SYNDROME
Primary
hyperparathyroidism (PHPT) is a manifestation in 20-40 % of patients with MEN2
A syndrome. The etiopathology of PHPT is not as predictable as in MEN1 where PHPT
is due to diffuse parathyroid hyperplasia. Indeed, in MEN2 A multiglandular
disease is less frequent and the volume of the affected glands tends to be
smaller. PHPT in MEN2 A syndrome is due to:
(a) Hyperplasia
of all four parathyroid glands, potentially with associated adenomas (~ 50 %)
(b) Single
adenoma (30 – 50 %)
(c) Double
adenomas (1 – 17 %)
Interestingly,
ectopic and supernumerary parathyroid glands are found in 15 % and 9 % of
patients with MEN2 A syndrome, respectively.
Parathyroid
carcinoma is a very rare cause of PHPT in MEN2A syndrome, with only one case
reported in the literature
Understanding
the etiopathology of PHPT in MEN2A has clinical importance since allows selection
of the optimal surgical strategy in the management of these patients. Based on
the above presented data, a more conservative surgical approach (selective
parathyroidectomy instead of total or subtotal parathyroidectomy) is justified.
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