Anaplastic thyroid cancer is an uncommon malignancy with a poor prognosis. Elderly patients are most commonly afflicted and survival past 3 years occurs in less than 5% of patients. Management of these patients is challenging, and the importance of palliation, airway protection, and aggressive resection is debated. In this report, we describe a patient with anaplastic thyroid carcinoma who presented with respiratory distress due to invasion of the tracheal cartilage. The patient was managed with cricotracheal resection, total thyroidectomy and thyrotracheal anastomosis. The patient is currently disease free 3.5 years after resection and postoperative radiation therapy with interval neck dissection.
Anaplastic carcinoma of the thyroid has a dismal prognosis, with a mean survival of less than 6 months [
The recommended aggressiveness of treatment advocated in the literature varies as some authors recommend surgery for localized tumors, followed by postoperative adjuvant radiation therapy [
In this case report, we describe a 65 year old who presented with airway obstruction due to anaplastic carcinoma of the thyroid that arose in papillary carcinoma. The patient was treated with surgical resection including the upper trachea and anterior cricoid, in continuity with total thyroidectomy. This approach was selected because the tumor could be completely resectable with negative margins by cricotracheal resection. Postoperative radiation therapy has let to control of the disease. The case illustrates that with aggressive treatment, long-term survival with an excellent quality of life can be achieved, even when the tumor presents with airway involvement.
The patient is a 65-year-old female who presented with a 7 year history of a persistent lower neck mass. She later developed a 2 month history of wheezing and dyspnea on exertion. During her third Emergency Room admission for respiratory distress, a CT scan of the neck and upper chest revealed a 1.2 cm × 8 mm subglottic tracheal mass with possible right true vocal cord involvement and a 2.2 cm right thyroid lobe mass (
Rigid bronchoscopy was performed, indicating that the intraluminal component of the tracheal mass began 1 cm inferior to the true vocal cord and extended inferiorly within the lumen for a distance of 2.5 cm. The tumor appeared to arise from the right side wall of the trachea. An 8 mm × 8 mm section of pedunculated tumor was endoscopically removed. Microscopic analysis of the intraluminal specimen indicated the presence of anaplastic thyroid carcinoma arising out of well differentiated papillary thyroid carcinoma (
Shortly after diagnosis, the patient underwent resection of the mass. The operation included a total thyroidectomy with right recurrent laryngeal nerve sacrifice and right paratracheal node dissection, resection of the upper trachea and cricoid, and anastomosis of the tracheal margin to the thyroid lamina. There were no complications and the patient was discharged on the seventh postoperative day with a tracheostomy.
Approximately 2.5 months after surgery, fiberoptic laryngoscopy indicated right vocal cord paralysis and a borderline airway with a narrow subglottis. The patient had no airway management difficulties and began successfully plugging the tracheostomy tube 10 months after surgery, and was decannulated 18 months after surgery.
Two years after the operation, a 1.5 cm enlarged level III right neck lymph node was found to have metastatic undifferentiated anaplastic carcinoma by fine needle aspiration. The patient underwent right neck dissection indicating a solitary 2 cm × 1.5 cm mass consistent with metastatic anaplastic carcinoma. Twelve other nodes in the specimen were benign. An additional course of radiation therapy was delivered to the neck following surgery. The patient is currently alive and free of disease 3.5 years following her original surgery.
Anaplastic thyroid cancer is usually a rapidly growing neoplasm with a poor prognosis. It generally occurs in the setting of previous thyroid pathology, such as goiter or papillary carcinoma, as in the present patient. The exact etiology is poorly understood and there has yet to be strong evidence identifying specific environmental or genetic factors, with the exception of iodine deficiency [
Anaplastic thyroid carcinoma can cause symptoms related to its involvement of adjacent structure such as the esophagus and trachea. Therefore, it should be considered a possible diagnosis in the setting of a thyriod mass accompanied by symptoms of airway obstruction, hoarseness and dysphagia. Diagnosis is obtained with either needle or incisional biopsy [
The role of surgical therapy for attempted cure is controversial, given the poor prognosis. Surgical intervention is indicated in anaplastic carcinoma when a complete resection is possible [
The anatomical location of anaplastic thyroid cancer and its rapid rate of growth raise the crucial issue of airway management in these patients [
A study by Pierie et al. [
Although complete surgical resection may enhance local control of the tumor or even survival, the consequential morbidity may prohibit its use. Kihara et al. [
The use of radiation therapy in the treatment of anaplastic thyroid carcinoma also has been debated [
Combining treatment types is another potential option for patients with anaplastic carcinoma. Some studies support an aggressive, multi-modality treatment protocol to increase survival rates. Haigh et al. [
If combination treatment is associated with improved survival, the impact on the quality of life must also be considered. Patients treated with surgical resection, radiation, and chemotherapy had a statistically significant increase in grade 3 toxicity. Half of the patients treated with this multi-modality protocol required tube feeding secondary to radiation mucositis and esophagitis [
Airway management is another significant issue involved in treatment of these patients. Of all malignancies, anaplastic thyroid carcinoma most commonly requires tracheostomy as part of management [
Our patient had a common presentation of anaplastic thyroid carcinoma—respiratory distress secondary to significant airway involvement. Her uncomplicated clinical course and long term survival, however, are rare. An extensive resection and lymph node dissection, including cricotracheal resection, was well tolerated by the patient despite right vocal cord paralysis. Two years after this surgery, a lymph node demonstrated metastasis and was treated with lymph node dissection and radiation with no additionally complications. Our patient is a long term survivor has no evidence of disease 3.5 years since surgery. She has had no airway complications and is without tracheostomy or tube feeding.
Part of the challenge of designing treatment for such patients is the inherent bias in many of clinical studies of anaplastic carcinoma in the literature. Selection bias plays a role in the extent of treatment that patients receive. Patients with severe disease may not be able to tolerate a multi-modality treatment plan [
While our patient responded well to aggressive surgical management, it was a somewhat unique anaplastic carcinoma that arose in a well differentiated papillary carcinoma. A larger series would be necessary to evaluate the role of cricotracheal resection for anaplastic carcinoma in general.