eMedicine Specialties > Oncology > Carcinomas of Endocrine Organs

Thyroid, Anaplastic Carcinoma

Author: Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Brigham and Women's Hospital, Harvard University
Coauthor(s): Debra J Graham, MD, Director of Surgical Specialties Service, Cleveland Louis Stokes VA Medical Center, Associate Program Director, Assistant Professor, Department of Surgery, Case Western Reserve University
Contributor Information and Disclosures

Updated: Apr 18, 2006

Introduction

Background

Anaplastic carcinoma of the thyroid (ATC) is the most aggressive thyroid gland malignancy. Although ATC accounts for less than 2% of all thyroid cancers, it causes up to 40% of deaths from thyroid cancer.

The aggressive nature of ATC makes treatment studies difficult to perform.

Pathophysiology

ATC generally occurs in people in iodine-deficient areas and in a setting of previous thyroid pathology (eg, preexisting goiter, follicular thyroid cancer, papillary thyroid cancer). Local invasion of adjacent structures (eg, trachea, esophagus) commonly occurs.

ATC has a rapid course and early dissemination. The most common sites of distant spread include (in descending order) lung, bone, and brain. Metastases (particularly in the lung) are likely to be present at diagnosis more than 50% of the time.

Frequency

United States

ATC comprises less than 2% of all thyroid malignancies. Fortunately, the incidence appears to be declining.

International

Worldwide frequency likely approximates that in the United States.

Mortality/Morbidity

ATC typically has a rapidly progressive course. The overall 5-year survival rate is reportedly less than 10%, and most patients do not live longer than a few months after diagnosis.

Sex

The female-to-male ratio is approximately 3:1.

Age

Peak incidence occurs during the sixth to seventh decades of life. The age range of affected patients reportedly is 15-90 years.

Clinical

History

Patients with anaplastic thyroid carcinoma (ATC) typically present with a rapidly growing neck mass. Patients with metastases may also note bone pain, weakness, and cough. Neurologic deficits may be observed with brain metastases. The rapidly growing neck mass may produce the following symptoms:

  • Dysphagia
  • Cough
  • Neck pain
  • Dyspnea

Physical

Physical examination typically reveals a dominant neck mass. More than 40% of affected patients have lymph node enlargement, indicating local metastases. Pleural effusions may lead to decreased breath sounds on auscultation. With metastases, the physician may note bone pain and neurologic deficits.

Causes

ATC is believed to occur from a terminal dedifferentiation of previously undetected long-standing thyroid carcinoma (eg, papillary, follicular). ATC has a genetic association with oncogenes C-myc, H-ras, and Nm23.

More on Thyroid, Anaplastic Carcinoma

Overview: Thyroid, Anaplastic Carcinoma
Differential Diagnoses & Workup: Thyroid, Anaplastic Carcinoma
Treatment & Medication: Thyroid, Anaplastic Carcinoma
Follow-up: Thyroid, Anaplastic Carcinoma
References
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References

  1. Ain KB. Anaplastic thyroid carcinoma: a therapeutic challenge. Semin Surg Oncol. 1999;16:64-69. [Medline].

  2. Austin JR, el-Naggar AK, Goepfert H. Thyroid cancers. II. Medullary, anaplastic, lymphoma, sarcoma, squamous cell. Otolaryngol Clin North Am. 1996;29:611-27. [Medline].

  3. Goutsouliak V, Hay JH. Anaplastic thyroid cancer in British Columbia 1985-1999: a population-based study. Clin Oncol (R Coll Radiol). Apr 2005;17(2):75-8. [Medline].

  4. Kapp DS, LiVolsi VA, Sanders MM. Anaplastic carcinoma following well-differentiated thyroid cancer: etiological considerations. Yale J Biol Med. 1982;55:521-8.

  5. Kebebew E, Greenspan FS, Clark OH, et al. Anaplastic thyroid carcinoma. Treatment outcome and prognostic factors. Cancer. Apr 1 2005;103(7):1330-5. [Medline].

  6. Schott M, Scherbaum WA. Immunotherapy and gene therapy of thyroid cancer. Minerva Endocrinol. Dec 2004;29(4):175-87. [Medline].

  7. Udelsman R, Lakatos E, Ladenson P. Optimal surgery for papillary thyroid carcinoma. World J Surg. 1996;20:88-93. [Medline].

  8. Xing M. BRAF mutation in thyroid cancer. Endocr Relat Cancer. Jun 2005;12(2):245-62. [Medline].

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Further Reading

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Keywords

undifferentiated thyroid carcinoma, anaplastic carcinoma of the thyroid, anaplastic thyroid carcinoma, ATC, thyroid gland malignancy, anaplastic thyroid carcinoma, thyroid cancer, thyroid tumor, metastases, thyroid malignancy

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Contributor Information and Disclosures

Author

Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Brigham and Women's Hospital, Harvard University
Disclosure: Nothing to disclose.

Coauthor(s)

Debra J Graham, MD, Director of Surgical Specialties Service, Cleveland Louis Stokes VA Medical Center, Associate Program Director, Assistant Professor, Department of Surgery, Case Western Reserve University
Debra J Graham, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Association for Academic Surgery, Association of VA Surgeons, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

Lodovico Balducci, MD, Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

John S Macdonald, MD, Professor of Medicine, New York Medical College; Chief, Division of Medical Oncology, St Vincent's Hospital and Medical Center; Medical Director, Saint Vincent's Comprehensive Cancer Center
Disclosure: Nothing to disclose.

 
 
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