MULTIGLANDULAR
PARATHYROID DISEASE (MGPD) IN SPORADIC PRIMARY HYPERPARATHYROIDISM (PHPT)
MGPD is a
typical manifestation of inherited PHPT (observed in MEN syndromes [mainly type
1, but also in type 2A], and occasionally hyperparathyroidism-jaw tumor
syndrome [HPT-JT syndrome]). In clinical practice, however, most cases of MGPD are
encountered in patients with sporadic PHPT. Sporadic PHPT is due to MGPD in
10-15 % of patients and to single adenoma in 85 % of them (parathyroid cancer
is very rare, < 1 %). In the majority of patients, MGPD is caused by
hyperplasia involving all parathyroid glands, whether in the remaining patients
by double or (very rarely) triple adenomas. Interestingly, even in patients
with concordant imaging (ultrasonography + sestamibi scan) suggestive of a single
adenoma, further enlarged parathyroids could be encountered in a small but
significant percentage (10-15 %) of patients with sporadic PHPT, if those
patients undergo bilateral neck exploration instead of focused (selective) parathyroidectomy.
The clinical significance of these additional enlarged glands remains
questionable. Double adenomas could in some cases represent asymmetric or asynchronous
hyperplasia. These data emphasize the importance of the experience of the
radiologist performing preoperative ultrasound, who should be able to recognize
all pathologically enlarged parathyroid glands. Unfortunately, frozen biopsy
cannot accurately differentiate between hyperplasia and adenoma. Intraoperative
parathormone monitoring is a useful diagnostic tool to estimate success of
surgery. Long-term follow up of patients is mandatory to document permanent
cure.
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