Researchers from the United States reported the rare case of a patient with a history of medullary thyroid carcinoma (MTC) associated with multiple endocrine neoplasia type 2A (MEN2A) who developed remote recurrent primary hyperparathyroidism in auto-transplanted tissue 18 years later.
“This case highlights the significance of indefinite vigilant surveillance in this patient population, as a recurrence of hyperparathyroidism may occur even after decades of remission,” the researchers wrote in a report they published in the journal Cureus.
Read more about the treatment of MTC
Continue Reading
MEN2A is also associated with pheochromocytoma and primary hyperparathyroidism, the current standard of treatment for which involves surgical removal of visibly enlarged glands and auto-transplantation of remnant tissue to the forearm or sternocleidomastoid muscle.
The case presented here by Melisa Esposito, MD, from Kettering Health Network and Thomas Koroscil, MD, PhD, from Wright State University both in Dayton, Ohio, is that of a 51-year-old male who underwent total parathyroidectomy with cryopreservation and subsequent autotransplantation of remnant parathyroid tissue to the left arm 18 years before.
The patient presented with asymptomatic hypercalcemia. Laboratory examination revealed elevated intact parathyroid hormone. Left arm ultrasound showed 3 areas of enlarged parathyroid tissue at the transplant site.
This raised suspicion about the development of recurrent primary hyperparathyroidism in the autotransplanted tissue.
The patient was treated with redo subtotal parathyroidectomy of autotransplanted tissue. Pathology confirmed the presence of hyperplastic parathyroid tissue.
“Given the genetic predisposition for hyperparathyroidism in patients with MEN2A, recurrence is often considered inevitable,” the researchers wrote. “Therefore, watchful vigilance is essential, and patients should be screened for recurrence with yearly serum calcium measurements following parathyroidectomy. Screening should continue indefinitely, as recurrence can occur even after decades of remission.”
MEN2A is associated with germline gain-of-function mutations in the RET protooncogene that is situated on chromosome 10q11.2.
Reference
Esposito M, Koroscil T. Remote recurrent primary hyperparathyroidism in auto-transplanted tissue. Cureus. Published online June 21, 2023. doi: 10.7759/cureus.40715
