Anaplastic thyroid cancer based on ATA guideline for Management of Patients with ATC. Thyroid. 2012;22: R3 이정록

Introduction Prevalence –1.3% - 9.8% (median = 3.6%) of all thyroid cancers Prognosis –Median survival : 5 months –1-year survival rate : 20% All patients are stage IV (by the AJCC TNM systems) –IVA : intrathyroidal (T4a), and N0, M0 –IVB : gross extrathyroidal extension, any N, M0 –IVC: distant metastases (M1)

Clinical presentation Rapidly growing neck mass with symptoms of dyspnea and dysphagia Flexible endoscopy commonly reveals vocal cord paralysis Over 75% patients have cervical node disease and 50~75% patients have metastasis at time of presentation Metastatic sites : Lung(80%), Bone(6-15%), brain(5-13%)

Differential diagnosis Poorly differentiated thyroid caner Squamous cell thyroid cancer Large cell lymphoma Medullary thyroid carcinoma Direct extension of a laryngeal carcinoma Metastatic carcinoma/melanoma Infectious and inflammatory lesion

Cytology and pathology procedures Limited sampling of FNA or core biopsy (e.g. extensive necrosis, paucicelluar variant)  Open biopsy should be performed Freezing of tissue compromises morphology  Intraoperative pathology consultation is not usually appropriate for definitive diagnosis Recommendation 1.Whenever possible, a definitive diagnosis should be obtained prior to surgery. 2.FNA cytology or core biopsy should play a role in the preoperative diagnosis of ATC.

Evaluation Primary surgery should not be delayed to biopsy distant metastases. If clinically indicated, such biopsies could be performed after completion of primary surgery. Only impending neurological crisis (e.g. brain tumor expansion, compromise of spinal cord) and pulmonary hemorrhage would sufficient cause for delaying primary surgery. Recommendation 1.Neck US, CT or MRI for neck/chest, PET/CT fusion scan 2.Molecular studies based on DNA/RNA analysis are not currently required for diagnosis and management. 3.Every patients should undergo evaluation of the vocal cords.

Intensity-modulated radiotherapy (IMRT) improved the tumor coverage and reduced the spinal cord dose.  high-precision radiotherapy, capable of killing all of the cancer cells, thus shrinking or eliminating tumors. Cytotoxic chemotherapy : taxane(paclitaxel or docetaxel) ± anthracyclines (doxorubicin) ± platin(cisplatin or carboplatin) Recommendation 1.Radiation started as soon as the patient is sufficiently recovered from neck surgery, usually within 2 to 3 weeks after surgery. 2.Chemotherapy begin as soon at the patient is sufficiently recovered from surgery, potentially even within 1 week of surgery. Treatment

Total/near-total thyroidectomy + central and lateral LND Extrathyroidal invasion (+)  en bloc resection R0/R1 resection improve disease-free and overall survival

Treatment

Haymart MR et al. Cancer. 2013;doi: /cncr N=2,742 (ATC, National cancer database between 1998 and 2008)

Prognostic factors Positive prognostic factors –Age < 60 years –Tumor sized < 7 cm –Less extensive disease –Greater extent of surgery –Higher dose radiotherapy –Management using multimodal therapy Negative prognostic factors Male sex Age > 60 years Tumor size > 5 cm Extrathyroidal involvement Distant metastases