ATA Guidelines Tools

9 Î Patients with MEN2A or MEN2B and a histological diagnosis of MTC regardless of age and presenting symptoms must have a PHEO excluded prior to any interventional procedure. The presence of a PHEO must be excluded in women with MEN2A or MEN2B who are planning a pregnancy or are pregnant. If a PHEO is detected it should be removed preferably during pregnancy. (C). Î If they coexist, a PHEO should be removed prior to surgery for either MTC or HPTH. (B). Î After appropriate preoperative preparation a PHEO should be resected by laparoscopic or retroperitoneoscopic adrenalectomy. (B) Note: Subtotal adrenalectomy to preserve adrenal cortical function should be considered as an alternative procedure. Î Patients with no adrenal glands require glucocorticoid and mineralocorticoid replacement therapy and should be carefully monitored to assure that their steroid levels are adequate. Patients should be educated regarding the risk of adrenal crisis and wear a bracelet or a necklace indicating that they have no adrenal glands and are on corticosteroid replacement therapy. Glucocorticoid supplementation will be required if they become severely ill or are injured. (B) Î Patients in the ATA-H and ATA-MOD categories should be screened for HPTH at the time of screening for PHEO (by age 11 years in patients in the ATA-H category and by age 16 years in patients in the ATA-MOD category). (C). Î In patients with HPTH, only the visibly enlarged parathyroid glands should be resected. (C) Note: If all four glands are enlarged, surgical options include subtotal parathyroidectomy with a piece of one gland left in situ on a vascular pedicle, or total parathyroidectomy with a heterotopic autograft. Î Patients who develop HPTH subsequent to thyroidectomy for MTC should have localization studies performed prior to repeat neck surgery. At reoperation all enlarged parathyroid glands should be removed, and parathyroids of normal size should be left in situ. If only one enlarged parathyroid gland is identified, and there is histological documentation that three parathyroid glands have been removed previously, a portion of the enlarged gland should either be left in situ with an adequate blood supply, or grafted to a heterotopic site. (C) Î Clinicians should consider the American Joint Committee on Cancer TNM classification (Table 3A&B), the number of lymph node metastases, and postoperative serum Ctn levels in predicting outcome and planning long-term follow-up of patients treated by thyroidectomy for MTC. (C)

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