Elevated basal serum levels of calcitonin and simultaneous surgery of MEN2A-specific tumors

Multiple endocrine neoplasia type 2A (MEN2A) is a rare syndrome caused almost by germline RET mutation, and characterized medullary thyroid carcinoma (MTC), in combination or not with pheochromocytoma (PHEO), hyperparathyroidism (HPTH), cutaneous lichen amyloidosis (CLA), Hirschsprung's disease (HD). The basal serum calcitonin (Ctn)/carcinoembryonic antigen (CEA) levels are significantly correlated the MTC stage. Metachronous surgery of MEN2A-specific tumors routine procedure. We aimed to explore clinical significance pro-gastrin-releasing peptide (proGRP) elevated Ctn simultaneous tumors. retrospectively investigated 8 mutation carriers 2 Chinese pedigrees MEN2A. Clinical profiles, imaging examinations, preoperative postoperative biochemical data, surgical procedures, follow-up records were evaluated. Three patients showed but normal proGRP. Among them, one patient (FAIII-6) Family A (one for C634R mutation), diagnosed bilateral MTC, left PHEO, HPTH, CLA, classified as MEN2A-related CLA subtype, underwent successfully adrenal-sparing (ASS), total thyroidectomy (TT), parathyroidectomy, while TT other two (FBII-3 FBIII-7) B (all C618R mutation) performed. Unexpectedly, absence neck lymph node metastasis was indicated histopathological examination. Postoperatively, all had consistently undetectable Ctn/CEA during follow-up. Patients proGRP, despite high Ctn, might have no regional metastasis, dissection should be avoided. Moreover, coexistent PHEO either HPTH an approach choice use alternative treatment pattern. Recognition subsequently early screening may favorable timely management