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===Medullary Thyroid Cancer===
===Medullary Thyroid Cancer===
====Stage I Follicular Thyroid Cancer====
* Several staging systems have been employed to correlate extent of disease with long-term survival in medullary thyroid cancer. The clinical staging system of the AJCC correlates survival to size of the primary tumor, presence or absence of lymph node metastases, and presence or absence of distance metastasis. Patients with the best prognosis are those who are diagnosed by provocative screening, prior to the appearance of palpable disease.[3]
* Stage I follicular carcinoma is localized to the [[thyroid gland]]. Follicular thyroid carcinoma must be distinguished from follicular adenomas, which are characterized by their lack of invasion through the [[capsule]] into the surrounding [[thyroid|thyroid tissue]]. While follicular cancer has a good [[prognosis]], it is less favorable than that of papillary carcinoma. The 10-year survival is better for patients with follicular carcinoma without vascular invasion than it is for patients with vascular invasion.
====Stage 0 medullary thyroid cancer====
====Stage II Follicular Thyroid Cancer====
* Clinically occult disease detected by provocative biochemical screening.
* Stage II follicular carcinoma is defined as either [[tumor]] that has spread distantly in patients younger than 45 years, or [[tumor]] that is larger than 2 cm but 4 cm or smaller and is limited to the [[thyroid gland]] in patients older than 45 years. The presence of lymph node metastases does not worsen the prognosis among patients younger than 45 years. Follicular thyroid carcinoma must be distinguished from follicular adenomas, which are characterized by their lack of invasion through the capsule into the surrounding thyroid tissue. While follicular cancer has a good prognosis, it is less favorable than that of papillary carcinoma; the 10-year survival is better for patients with follicular carcinoma without vascular invasion than for patients with vascular invasion.
====Stage I medullary thyroid cancer====
====Stage III Follicular Thyroid Cancer====
* Tumor smaller than 2 cm.
* Stage III is follicular carcinoma in patients older than 45 years, larger than 4 cm and limited to the thyroid or with minimal extrathyroid extension, or positive lymph nodes limited to the pretracheal, paratracheal, or prelaryngeal/Delphian nodes. Follicular carcinoma invading cervical tissue has a worse prognosis than tumors confined to the thyroid gland. The presence of vascular invasion is an additional poor prognostic factor. Metastases to lymph nodes do not worsen the prognosis in patients younger than 45 years.
====Stage II medullary thyroid cancer====
====Stage IV Follicular Thyroid Cancer====
* Tumor larger than 2 cm but 4 cm or smaller with no metastases or larger than 4 cm with minimal extrathyroid extension.
* Stage IV is follicular carcinoma in patients older than 45 years with extension beyond the thyroid capsule to the soft tissues of the neck, cervical lymph node metastases, or distant metastases. The lungs and bone are the most frequent sites of spread. Follicular carcinomas more commonly have blood vessel invasion and tend to metastasize hematogenously to the lungs and to the bone rather than through the lymphatic system. The prognosis for patients with distant metastases is poor.
====Stage III medullary thyroid cancer====
* Tumor of any size with metastases limited to the pretracheal, paratracheal, or prelaryngeal/Delphian lymph nodes.
====Stage IV medullary thyroid cancer====
* Stage IV medullary thyroid cancer is divided into the following categories:
:* Stage IVA (moderately advanced with or without lymph node metastases [for T4a] but without distant metastases).
:* Stage IVB (very advanced with or without lymph node metastases but no distant metastases).
:* Stage IVC (distant metastases).
* Medullary carcinoma usually presents as a hard mass and is often accompanied by blood vessel invasion. Medullary thyroid cancer occurs in two forms, sporadic and familial. In the sporadic form, the tumor is usually unilateral. In the familial form, the tumor is almost always bilateral. In addition, the familial form may be associated with benign or malignant tumors of other endocrine organs, commonly referred to as the multiple endocrine neoplasia syndromes (MEN 2A or MEN 2B).
* In these syndromes, there is an association with pheochromocytoma of the adrenal gland and parathyroid hyperplasia. Medullary carcinoma usually secretes calcitonin, a hormonal marker for the tumor, and may be detectable in blood even when the tumor is clinically occult. Metastases to regional lymph nodes are found in about 50% of cases. Prognosis depends on extent of disease at presentation, presence or absence of regional lymph node metastases, and completeness of the surgical resection.[4]
* Family members should be screened for calcitonin elevation to identify individuals who are at risk of developing familial medullary thyroid cancer. MEN 2A gene carrier status can be more accurately determined by analysis of mutations in the RET gene. Whereas modest el mutation is the optimal approach in evaluating MEN 2A. All patients with medullary carcinoma of the thyroid (whether familial or sporadic) should be tested for RET mutations, and, if they are positive, family members should also be tested. Family members who are gene carriers should undergo prophylactic thyroidectomy at an early age.[5-7]
 
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File:Stage M1 Thyroid cancer.png|Stage M1 thyroid cancer
File:Stage M1 Thyroid cancer.png|Stage M1 thyroid cancer
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File:Diagram showing stage T4b thyroid cancer CRUK 273.png|Stage T4b thyroid cancer
File:Diagram showing stage T4b thyroid cancer CRUK 273.png|Stage T4b thyroid cancer
</gallery>
</gallery>
==Reference==
{{Reflist|2}}
==Reference==
==Reference==
{{Reflist|2}}
{{Reflist|2}}

Revision as of 15:27, 11 November 2015

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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

According to the American Joint Committee on Cancer (AJCC)[1] 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.

Staging


Thyroid TNM staging
Stage Description
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor ≤2 cm in greatest dimension limited to the thyroid
T1a Tumor ≤1 cm, limited to the thyroid
T1b Tumor >1 cm but ≤2 cm in greatest dimension, limited to the thyroid
T2 Tumor >2 cm but ≤4 cm in greatest dimension, limited to the thyroid
T3 Tumor >4 cm in greatest dimension limited to the thyroid or any tumor with minimal extrathyroid extension (e.g., extension to sternothyroid muscle or perithyroid soft tissues)
T4a Moderately advanced disease
Tumor of any size extending beyond the thyroid capsule to invade subcutaneous soft tissues, larynx, trachea, esophagus, or recurrent laryngeal nerve
T4b Very advanced disease
Tumor invades prevertebral fascia or encases carotid artery or mediastinal vessel


Regional Lymph Nodes (N)
Stage Description
NX Regional lymph node cannot be assessed
N0 No regional lymph node metastasis]]
N1 Regional lymph node metastasis
N1a Metastases to Level VI (pretracheal, paratracheal, and prelaryngeal/Delphian lymph nodes)
N1b Metastases to unilateral, bilateral, or contralateral cervical (Levels I, II, III, IV, or V) or retropharyngeal or superior mediastinal lymph nodes (Level VII)
Distant Metastasis (M)
Stage Description
M0 No distant metastasis
M1 Distant metastasis


Anatomic Stage/Prognostic Groups
Stage T N M
Medullary carcinoma (all age groups)
I T1 N0 M0
II T2 N0 M0
T3 N0 M0
III T1 N1a M0
T2 N1a M0
T3 N1a M0
IVa T4a N0 M0
T4a N1a M0
T1 N1b M0
T2 N1b M0
T3 N1b M0
T4a N1b M0
Stage IVb T4b Any N
IVB T4b Any N M0
IVC Any T Any N M1

Medullary Thyroid Cancer

  • Several staging systems have been employed to correlate extent of disease with long-term survival in medullary thyroid cancer. The clinical staging system of the AJCC correlates survival to size of the primary tumor, presence or absence of lymph node metastases, and presence or absence of distance metastasis. Patients with the best prognosis are those who are diagnosed by provocative screening, prior to the appearance of palpable disease.[3]

Stage 0 medullary thyroid cancer

  • Clinically occult disease detected by provocative biochemical screening.

Stage I medullary thyroid cancer

  • Tumor smaller than 2 cm.

Stage II medullary thyroid cancer

  • Tumor larger than 2 cm but 4 cm or smaller with no metastases or larger than 4 cm with minimal extrathyroid extension.

Stage III medullary thyroid cancer

  • Tumor of any size with metastases limited to the pretracheal, paratracheal, or prelaryngeal/Delphian lymph nodes.

Stage IV medullary thyroid cancer

  • Stage IV medullary thyroid cancer is divided into the following categories:
  • Stage IVA (moderately advanced with or without lymph node metastases [for T4a] but without distant metastases).
  • Stage IVB (very advanced with or without lymph node metastases but no distant metastases).
  • Stage IVC (distant metastases).
  • Medullary carcinoma usually presents as a hard mass and is often accompanied by blood vessel invasion. Medullary thyroid cancer occurs in two forms, sporadic and familial. In the sporadic form, the tumor is usually unilateral. In the familial form, the tumor is almost always bilateral. In addition, the familial form may be associated with benign or malignant tumors of other endocrine organs, commonly referred to as the multiple endocrine neoplasia syndromes (MEN 2A or MEN 2B).
  • In these syndromes, there is an association with pheochromocytoma of the adrenal gland and parathyroid hyperplasia. Medullary carcinoma usually secretes calcitonin, a hormonal marker for the tumor, and may be detectable in blood even when the tumor is clinically occult. Metastases to regional lymph nodes are found in about 50% of cases. Prognosis depends on extent of disease at presentation, presence or absence of regional lymph node metastases, and completeness of the surgical resection.[4]
  • Family members should be screened for calcitonin elevation to identify individuals who are at risk of developing familial medullary thyroid cancer. MEN 2A gene carrier status can be more accurately determined by analysis of mutations in the RET gene. Whereas modest el mutation is the optimal approach in evaluating MEN 2A. All patients with medullary carcinoma of the thyroid (whether familial or sporadic) should be tested for RET mutations, and, if they are positive, family members should also be tested. Family members who are gene carriers should undergo prophylactic thyroidectomy at an early age.[5-7]

Reference

  1. 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

Reference