2012 European Thyroid Association Guidelines for Metastatic Medullary Thyroid Cancer

Abstract

Distant metastases are the main cause of death in patients with medullary thyroid cancer (MTC). These 21 recommendations focus on MTC patients with distant metastases and a detailed follow-up protocol of patients with biochemical or imaging evidence of disease, selection criteria for treatment, and treatment modalities, including local and systemic treatments based on the results of recent trials. Asymptomatic patients with low tumor burden and stable disease may benefit from local treatment modalities and can be followed up at regular intervals of time. Imaging is usually performed every 6–12 months, or at longer intervals of time depending on the doubling times of serum calcitonin and carcinoembryonic antigen levels. Patients with symptoms, large tumor burden and progression on imaging should receive systemic treatment. Indeed, major progress has recently been achieved with novel targeted therapies using kinase inhibitors directed against RET and VEGFR, but further research is needed to improve the outcome of these patients.

ENDOCRINE TUMOURS: Progressive metastatic medullary thyroid carcinoma: first- and second-line strategies

“…Although the prognosis is generally favourable when diagnosed and treated at an early localised stage, it strongly depends on disease stage, with a 10-year overall survival (OS) of 95% in patients with localised disease to 75 and 40% in patients with regional and metastasised disease respectively (9). Treatment for metastasised MTC, as for the prophylactic procedure, remains a topic of discussion, but progress has been made by American and European Taskforces (10,11). However, one of the main challenges in the treatment of MTC patients is to find effective adjuvant and palliative options for patients with metastatic disease.…”

Results of Surgical Therapy in Patients with Medullary Thyroid Carcinoma

“…According to the current guidelines of the American Thyroid Association and European Thyroid Association, the recommendations are for a modified radical neck dissection of the ipsilateral and contralateral compartment in cases with evidence of suspicious or metastatic nodes at preoperative imaging [4][5][6]. There are some authors who advocate the necessity of modified radical neck dissection (ipsilateral or bilateral) at the initial operation even in patients without any evidence of suspicious lymph nodes [7,8].…”

Is there a role for fluorine-18 fluorodeoxyglucose positron emission tomography imaging in medullary thyroid cancer?

“…In spite of aggressive primary treatment with total thyroidectomy and modified neck lymph node dissection, approximately 50 % of the patients have persistent or recurrent disease [1,2]. The recent European Thyroid Association guidelines [3] recommend surgery and other local treatment modalities (such as external beam radiation therapy, radiofrequency ablation, and chemoembolization) for the treatment of distant metastases in patients with low tumor burden (distant metastases limited to single organ) and stable disease, while patients with symptoms, significant tumor burden and progressive disease should receive novel targeted systemic therapies. However, imaging of recurrent disease in MTC is difficult and still poses a major problem in the management of the disease.…”

“…However, conventional imaging (CI) is often negative in patients with Ct concentrations \150 pg/ mL [3]. It is estimated that CI identifies metastases in about 40 % of patients with biochemical evidence of recurrence.…”

“…The meta-analysis by Treglia et al [3] highlights some of the difficulties of 18 F-FDG-PET imaging with regards to indolent course of MTC and small tumor lesions, which makes the detection rate far from optimal. Giraudet et al [11] reported that when compared to CT and MRI, 18 F-FDG-PET seemed to be inferior in detecting cervical and mediastinal lymph node metastases.…”