Medullary Thyroid Carcinoma Follow-up

Updated: Feb 09, 2017
  • Author: Anastasios K Konstantakos, MD; Chief Editor: Neetu Radhakrishnan, MD  more...
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Follow-up

Further Outpatient Care

Measure calcitonin and carcinoembryonic antigen (CEA) levels after thyroidectomy. Patients with undetectable calcitonin—or, in patients with sporadic MTC who have undergone hemithyroidectomy, calcitonin levels within the normal reference range—should have follow-up testing every 6-12 months.

Detectable CEA levels after total thyroidectomy, or above-normal levels after hemithyroidectomy, mandate further assessment with imaging studies, as per American Thyroid Association guidelines. If calcitonin becomes detectable after total thyroidectomy but imaging studies do not identify disease or if calcitonin levels rise after hemithyroidectomy, doubling time of calcitonin and CEA levels may be used to assess tumor progression. [1] In one study, 94% of patients with doubling times shorter than 25 months had progressive disease and 86% of patients with doubling times longer than 24 months had stable disease. [25]

Further intervention may include the following:

  • Perform reoperative cervical exploration for isolated recurrent cervical disease (without distant metastases) identified by ultrasonography or CT scanning
  • Identification of distant metastatic disease may require laparoscopy with probe ultrasonography to detect liver surface lesions and bone scanning to detect osseous disease
  • Selective hepatic venous sampling for liver metastases is an experimental procedure that is used to detect intrahepatic lesions with greater sensitivity
  • If metastatic workup findings are negative in a patient with elevated plasma calcitonin levels, elective cervical lymph node dissection or modified radial neck dissection may be performed.

For patients with undetectable calcitonin and normal CEA levels, post-surgical followup may include the following:

  • Physical examination twice yearly for 2 years and then yearly thereafter
  • Measurement of serum calcitonin and CEA levels twice yearly for 2 years and then yearly thereafter
  • Neck ultrasound 3 to 12 months postoperatively (depending on the extent of lymph node involvement prior to surgery) to establish a baseline; additional imaging is not required unless the calcitonin or CEA values rise during follow-up

Calcitonin values that remain ≥150 pg/mL 2 to 6 months after surgery increase the likelihood that the patient may have distant metastases. These patients should undergo neck ultrasound and additional imaging (eg, CT or MRI of neck, chest, and abdomen; bone scan or bone MRI in patients suspected of having skeletal metastases) to identify possible distant metastases. Adjuvant radiation has not been shown to influence 10 yr survival rates, however.

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Further Inpatient Care

Thyroid hormone therapy and radiotherapy are not as effective as surgical treatment for medullary thyroid carcinoma (MTC). However, positive surgical margins or mediastinal extension may be an indication for adjuvant radiotherapy.

External beam radiotherapy may provide a palliative benefit in controlling symptoms from bony metastases.

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Deterrence/Prevention

Management guidelines from the American Thyroid Association (ATA) recommend prophylactic thyroidectomy for individuals with documented RET mutation who are at risk for aggressive medullary thyroid carcinoma. [1] The ATA has proposed schedules for the recommended age of RET testing, first ultrasound, serum calcitonin level, and prophylactic surgery, depending on the level of risk; in those at highest risk, surgery is recommended within the first year of life.

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Complications

Permanent hypoparathyroidism and recurrent laryngeal nerve palsy reportedly occur in less than 2% of virgin neck dissections. Reoperation is associated with a considerably higher risk of these injuries.

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Prognosis

Prognosis depends on patient age, histologic grade, and status of surgical resection. Patients with a worse prognosis tend to be older, have higher-grade lesions, and have undergone incomplete surgical resection of the lesion.

A study by Rohmer et al concluded that disease-free survival (DFS) in younger patients (>21 y) with hereditary MTC was best predicted by TNM staging and preoperative basal CT level of less than 30 pg/mL. [26] Basal CT findings, class D genotype, and age were the key factors in deciding peroperatively timely surgery.

In a meta-analysis of 27 studies involving 984 MTC patients who underwent reoperation, Rowland and colleagues found that normalization of calcitonin after reoperation occurred in 16.2% of patients overall. Patients who underwent targeted selective lymph node removal procedures showed normalization of calcitonin in 10.5% of cases, while normalization was seen in 18.6% of those who underwent compartment-oriented procedures. [27]

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Patient Education

For patient education resources, see the Endocrine System Center, as well as Thyroid Problems and Anatomy of the Endocrine System.

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