Medullary thyroid cancer overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Medullary thyroid cancer from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

CT

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

Medullary thyroid cancer was first discovered by John Beach Hazard, an American pathologist, in 1959.[1] Medullary thyroid cancer may be classified according to mode of occurrence into 2 groups: sporadic medullary thyroid cancer and inherited medullary thyroid cancer. Development of medullary thyroid cancer is the result of genetic mutation of RET proto-oncogene. On gross pathology, well circumscribed, gray, white, or yellow in color mass is a characteristic finding of medullary thyroid cancer. On microscopic histopathological analysis, polygonal to spindle to small cells, interstitial edema, and vascular hyalinized stroma are characteristic findings of medullary thyroid cancer. Medullary thyroid cancer is caused by a mutation in the RET proto-oncogene. If left untreated, patients with medullary thyroid cancer may progress to develop metastasis. Common complications of medullary thyroid cancer include vocal cord compression, dysphagia, and dyspnea. The presence of metastasis is associated with a particularly poor prognosis among patients with medullary thyroid cancer. The 5 year event free survival rate is 80%. The hallmark of medullary thyroid cancer is lump in the neck. A positive family history of medullary thyroid cancer or multiple endocrine neoplasia is suggestive of medullary thyroid cancer. The most common symptoms of medullary thyroid cancer include diarrhea, flushing, and dysphagia. Laboratory findings consistent with the diagnosis of medullary thyroid cancer include decreased thyroid stimulating hormone, elevated calcitonin, and decreased calcium. On biopsy, medullary thyroid cancer is characterized by trabecula, interstitial edema, and coarse calcifications. Surgery is the mainstay of treatment for medullary thyroid carcinoma.

Historical Perspective

Medullary thyroid cancer was first discovered by John Beach Hazard, an American pathologist, in 1959.[1]

Classification

Medullary thyroid cancer may be classified according to mode of occurrence into 2 groups: sporadic medullary thyroid cancer and inherited medullary thyroid cancer.

Pathophysiology

Development of medullary thyroid cancer is the result of genetic mutation of RET proto-oncogene. On gross pathology, well circumscribed, gray, white, or yellow color mass is a characteristic finding of medullary thyroid cancer. On microscopic histopathological analysis, polygonal to spindle to small cells, interstitial edema, and vascular hyalinized stroma are characteristic findings of medullary thyroid cancer.

Causes

Medullary thyroid cancer is caused by a mutation in the RET proto-oncogene.

Differential Diagnosis

Medullary thyroid cancer must be differentiated from anaplastic thyroid carcinoma, papillary thyroid carcinoma, and Hurthle cell carcinoma.

Epidemiology and Demographics

The incidence of medullary thyroid cancer is approximately 1000 per 100,000 individuals in United States per year. The incidence of medullary thyroid cancer increases with age; the median age at diagnosis peaks in the 3rd to 4th decades.

Risk Factors

Common risk factors in the development of medullary thyroid cancer are family history of medullary thyroid cancer and family history of multiple endocrine neoplasia.

Screening

According to the American Society of Clinical Oncology, screening for medullary thyroid cancer by RET gene testing is recommended for children with increased risk of medullary thyroid cancer.

Natural history, Complications and Prognosis

If left untreated, patients with medullary thyroid cancer may progress to develop metastasis. Common complications of medullary thyroid cancer include vocal cord compression, dysphagia, and dyspnea. The presence of metastasis is associated with a particularly poor prognosis among patients with medullary thyroid cancer. The 5 year event free survival rate is 80%.

Staging

According to the American Joint Committee on Cancer (AJCC)[2] there are 4 stages of medullary thyroid cancer based on the clinical features and findings on imaging. Each stage is assigned a letter and a number that designate the tumor size, number of involved lymph node regions, and metastasis.

History and Symptoms

The hallmark of medullary thyroid cancer is lump in the neck. A positive family history of medullary thyroid cancer or multiple endocrine neoplasia is suggestive of medullary thyroid cancer. The most common symptoms of medullary thyroid cancer include diarrhea, flushing, and dysphagia.

Physical Examination

Patients with medullary thyroid cancer usually appear thin and cachectic. Physical examination of patients with medullary thyroid cancer is usually remarkable for thyromegaly, lymphadenopathy and anxiety.

Laboratory Findings

Laboratory findings consistent with the diagnosis of medullary thyroid cancer include decreased thyroid stimulating hormone, elevated calcitonin, and decreased calcium.

CT

CT scan may be helpful in the diagnosis of medullary thyroid cancer.

Echocardiography or Ultrasound

Neck ultrasound may be performed to detect medullary thyroid cancer.

Other Diagnostic Studies

Other diagnostic studies for medullary thyroid cancer include nuclear imaging, which demonstrates increased uptake of radioactive iodine at the areas of metastases.

Biopsy

On biopsy, medullary thyroid cancer is characterized by trabecula, interstitial edema, and coarse calcifications.

Medical Therapy

The predominant therapy for medullary thyroid cancer is surgical resection. Adjunctive chemoradiation may be required. The optimal therapy for medullary thyroid cancer depends on the stage at diagnosis.

Surgery

Surgery is the mainstay of treatment for medullary thyroid carcinoma.

Prevention

According to the American Society of Clinical Oncology, surveillance for medullary thyroid cancer by annual measurement of serum calcitonin, serum calcium, serum parathyroid hormone, and catacholamines is recommended postsurgically to monitor for recurrence and complications for medullary thyroid cancer.

Reference

  1. 1.0 1.1 HAZARD JB, HAWK WA, CRILE G (1959). "Medullary (solid) carcinoma of the thyroid; a clinicopathologic entity". J. Clin. Endocrinol. Metab. 19 (1): 152–61. doi:10.1210/jcem-19-1-152. PMID 13620740.
  2. Stage Information for Thyroid Cancer Cancer.gov (2015). http://www.cancer.gov/types/thyroid/hp/thyroid-treatment-pdq#link/stoc_h2_2- Accessed on October, 29 2015

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