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Pheochromocytomas not linked to lower survival, medullary thyroid cancer stage
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Results from a retrospective chart review have characterized the presentation of pheochromocytoma in patients with multiple endocrine neoplasia type 2 as well as its relationship with survival and stage of medullary thyroid cancer in patients with RET 634 mutations.
Sonali Thosani, MD, assistant professor in the division of endocrine neoplasia and hormonal disorders at The University of Texas MD Anderson Cancer Center, and colleagues explored the natural history of multiple endocrine neoplasia type 2 (MEN2) pheochromocytoma. They evaluated data from 85 patients with MEN2-associated pheochromocytoma who were seen at the MD Anderson Cancer Center from 1960 to 2012. Patients with RET codon 634 mutations (n=59) were compared with patients with RET codon 634 mutations but without MEN2-associated pheochromocytoma (n=48).
Sonali Thosani
According to data, 70 patients had MEN2A and 15 had MEN2B, and median age at diagnosis of pheochromocytoma was 32 years. Sixty percent of patients initially presented with medullary thyroid cancer vs. 34% who initially presented with synchronous medullary thyroid cancer and pheochromocytoma, and 6% with pheochromocytoma alone. Results showed that 72% of patients had bilateral pheochromocytoma, 82% of whom had synchronous presentation.
Of the 59 patients with RET codon 634 mutations, medullary thyroid cancer and pheochromocytoma, data on stage of medullary thyroid cancer at initial surgery were available for 46 patients. Five patients had stage I, four had stage III and eight had stage IV cancer, according to data. For patients without pheochromocytoma, 17 patients had stage I, nine had stage III and three had stage IV medullary thyroid cancer. There were no significant differences in stage at diagnosis, the researchers wrote.
According to survival analyses of patients with pheochromocytoma, 100% were alive at 5 years, 98% at 10 years and 90% at 15 years after MEN2 diagnosis. For patients without pheochromocytoma, 98% were alive at 5 years, 85% at 10 years and 78% at 15 years. Median survival time for patients with pheochromocytoma was 499 months vs. 444 months for patients with medullary thyroid cancer but without pheochromocytoma (P<.05). Survival time did not appear shorter for patients with pheochromocytoma among those with RET 634 mutations. Median follow-up time was 249 months for patients with pheochromocytoma vs. 67 months for those without pheochromocytoma (P<.01), according to data.
“For the first time, we are presenting objective data describing how the epidemiology of [pheochromocytoma] and [medullary thyroid cancer] in the setting of MEN2 has changed over time, which is perhaps a reflection of the historical impact of RET testing and the MEN guidelines,” the researchers wrote. They added that these results suggest that patients with pheochromocytoma and medullary thyroid cancer may represent a “different spectrum of disease” compared with those without pheochromocytoma.
The researchers said larger prospective studies are warranted to confirm these data.
Disclosure: The researchers report no relevant financial disclosures.
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