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Modern Experiences with Aggressive Parathyroid Tumors in a High-Volume New England Referral Center
*Courtney E Quinn, *Amir H Lebastchi, *James M Healy, *Taylor C Brown, *Manju L Prasad, *Glenda G Callender, *Tobias Carling, Robert Udelsman
Yale University, New Haven, CT
Objective:
Parathyroid carcinoma (PTCA) is an exceptionally rare malignancy, often with a similar clinical presentation to benign atypical parathyroid adenoma. Its low incidence portends unclear guidelines for management. Accordingly, thorough examination of clinical and pathological variables, including intraoperative intact parathyroid hormone (iPTH) levels, was undertaken to distinguish between PTCA and atypical adenomas.
Design:
Retrospective analysis of prospective database
Setting:
Tertiary academic referral center
Patients:
Of 3,643 primary hyperparathyroidism patients referred for surgical treatment between 2001 and 2014, 52(1.4%) harbored aggressive parathyroid tumors: parathyroid carcinomas (n=18[0.49%]) and atypical adenomas (n=34[0.93%]).
Interventions:
Surgical and pathological analysis of tumor characteristics, statistical analysis
Main Outcome Measures:
Pre-operative and intra-operative variables, post-operative and pathological outcomes.
Results:
PTCA patients present with significantly increased size (p=0.002), mean serum calcium (p=0.003) and iPTH levels (p=0.04), and a higher incidence of hypercalcemic crisis compared to atypical adenomas. PTCA more frequently lacks a distinct capsule (p=0.03) and adheres to adjacent structures (p=0.017). PTCA demonstrates significantly more aggressive pathologic features including fibrous bands, vascular and lymphatic invasion. Following resection, iPTH levels fell more precipitously in PTCA (p=0.016). Additionally, there was higher loss of parafibromin expression in the PTCA group.
Conclusions:
Clinical distinction between PTCA and atypical adenomas is of critical importance to determine the appropriate extent of resection and follow-up. Presence of highly elevated serum calcium and PTH levels, coupled with a large, palpable mass, should raise suspicion for PTCA. Absence of a distinct capsule and adherence to adjacent structures should prompt parathyroidectomy with en bloc resection of involved surrounding structures. Given that PTCA patients more frequently experience hypocalcemic symptoms, clearer definition of clinic-pathological criteria for PTCA may lead to improved management.
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