Medullary Thyroid Cancer (MTC)
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Questions and Answers

In medullary thyroid cancer (MTC), what is the principal origin of the tumor cells, and what substance do they produce?

  • Develops from parathyroid cells, secreting parathyroid hormone.
  • Derives from follicular cells, generating triiodothyronine (T3).
  • Originates from parafollicular C cells, synthesizing calcitonin. (correct)
  • Arises from follicular cells, producing thyroglobulin.

What genetic mutation is most strongly associated with the development of medullary thyroid cancer (MTC)?

  • EGFR
  • BRAF
  • RET proto-oncogene (correct)
  • TP53

A patient presents with a neck mass and chronic diarrhea. Which biochemical marker would be most indicative of medullary thyroid cancer (MTC)?

  • Elevated serum calcitonin (correct)
  • Elevated serum thyroglobulin
  • Elevated serum parathyroid hormone
  • Elevated serum TSH

Why is fine-needle aspiration (FNA) with calcitonin staining considered a crucial step in the diagnostic evaluation of suspected medullary thyroid cancer (MTC)?

<p>To confirm the diagnosis of MTC based on calcitonin production. (B)</p> Signup and view all the answers

In patients with medullary thyroid cancer (MTC), what is the rationale behind performing a total thyroidectomy with central neck lymph node dissection?

<p>To completely remove the tumor and prevent locoregional recurrence. (C)</p> Signup and view all the answers

Which of the following is a primary indication for using tyrosine kinase inhibitors (TKIs) like cabozantinib or vandetanib in the management of medullary thyroid cancer (MTC)?

<p>To target and inhibit the growth of advanced or metastatic MTC. (D)</p> Signup and view all the answers

In the context of Multiple Endocrine Neoplasia type 2 (MEN 2) and its association with medullary thyroid cancer (MTC), what is the significance of prophylactic thyroidectomy in RET mutation carriers?

<p>To reduce the risk of developing MTC later in life (C)</p> Signup and view all the answers

A patient with known medullary thyroid cancer (MTC) undergoes surgical resection. Postoperatively, what role does calcitonin monitoring play in their ongoing management?

<p>To monitor for residual or recurrent disease. (B)</p> Signup and view all the answers

Which clinical manifestation is specifically associated with MEN 2B and not typically seen in MEN 2A?

<p>Marfanoid habitus and mucosal neuromas (D)</p> Signup and view all the answers

In cases of advanced medullary thyroid cancer (MTC) where surgical resection is not feasible, what is the primary goal of radiation therapy?

<p>To provide palliation and alleviate symptoms (C)</p> Signup and view all the answers

A patient diagnosed with sporadic medullary thyroid carcinoma (MTC) has a 10-year survival rate of approximately 75%. Which factor would most significantly alter this prognosis?

<p>The patient's MTC is found to be metastatic at the time of diagnosis. (D)</p> Signup and view all the answers

In the context of Multiple Endocrine Neoplasia type 2 (MEN 2), which of the following genetic characteristics is most indicative of an aggressive form of medullary thyroid carcinoma (MTC) and a correspondingly poorer prognosis?

<p>The presence of a RET M918T mutation, particularly in MEN 2B. (B)</p> Signup and view all the answers

A 25-year-old patient is diagnosed with MEN 2B. Besides aggressive medullary thyroid carcinoma (MTC), which clinical finding would be most indicative of this specific subtype rather than MEN 2A?

<p>Presence of Marfanoid habitus and mucosal neuromas. (A)</p> Signup and view all the answers

A patient is suspected of having MEN 2A syndrome. Which combination of clinical findings would provide the strongest basis for this diagnosis?

<p>Medullary thyroid carcinoma (MTC), pheochromocytoma, and hypercalcemia due to primary hyperparathyroidism. (B)</p> Signup and view all the answers

A newly identified RET mutation in exon 11 is found in a patient with a family history of MEN 2A. How does this information most directly influence the patient's clinical management?

<p>Confirms the diagnosis of MEN 2A, suggesting the need for comprehensive endocrine screening for pheochromocytoma and hyperparathyroidism. (B)</p> Signup and view all the answers

A patient with confirmed MEN 2A is undergoing evaluation for pheochromocytoma. Which of the following aspects regarding pheochromocytoma in MEN 2A is most critical for surgical planning?

<p>The risk of hypertensive crisis during surgery necessitates careful preoperative management with alpha-adrenergic blockade. (C)</p> Signup and view all the answers

What is the underlying genetic mechanism behind Multiple Endocrine Neoplasia (MEN) syndromes?

<p>Autosomal dominant disorders caused by mutations in genes controlling cell growth, affecting multiple endocrine organs. (B)</p> Signup and view all the answers

A child is diagnosed with MEN 2B due to an aggressive presentation of medullary thyroid carcinoma (MTC). What is the most crucial next step in managing this patient’s care?

<p>Performing a prophylactic thyroidectomy to prevent further aggressive tumor growth. (B)</p> Signup and view all the answers

In managing a patient with MEN 2A, what is the rationale for prioritizing the surgical removal of a pheochromocytoma before addressing medullary thyroid carcinoma (MTC)?

<p>To reduce the risk of life-threatening hypertensive crises during thyroid surgery. (C)</p> Signup and view all the answers

A patient with MEN 2A is found to have primary hyperparathyroidism. Which of the following pathological findings would you most expect to observe during parathyroid surgery?

<p>Parathyroid hyperplasia affecting multiple glands. (D)</p> Signup and view all the answers

Flashcards

Medullary Thyroid Cancer (MTC)

A neuroendocrine tumor from parafollicular C cells, producing calcitonin.

Pathophysiology of MTC

Originates from neural crest C cells; associates with RET mutations; metastasizes to lymph nodes and other organs.

Clinical Presentation of MTC

Includes neck mass, dysphagia, diarrhea, flushing, and endocrine symptoms like hyperparathyroidism.

Biochemical Markers for MTC

Serum calcitonin and carcinoembryonic antigen (CEA) help in diagnosis and monitoring tumor burden.

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RET Proto-oncogene Mutation

A genetic marker often tested in MTC to detect hereditary forms and risks.

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Total Thyroidectomy

Surgical removal of the entire thyroid; standard treatment for localized MTC.

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Adjuvant Treatment for MTC

Includes targeted therapy with tyrosine kinase inhibitors and radiation for metastatic disease.

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Familial Syndromes of MTC

MTC can occur as part of MEN 2A and MEN 2B syndromes, which have distinct features.

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Calcitonin Monitoring

Used post-surgery to check for residual or recurrent disease in MTC patients.

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Imaging in MTC Diagnosis

Ultrasound is first-line; FNA confirms MTC; CT/MRI and PET scans assess metastasis.

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Annual screening for MTC

Includes neck ultrasound, serum calcitonin, and CEA levels for monitoring.

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Sporadic MTC prognosis

Approximately 75% 10-year survival rate for sporadic medullary thyroid carcinoma.

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Familial MTC prognosis

Worse prognosis than sporadic, particularly in MEN 2B due to aggressive nature.

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Stage-dependent survival

Survival outcomes vary by disease stage; localized is better than metastatic.

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MEN syndromes

Autosomal dominant disorders affecting multiple endocrine organs due to gene mutations.

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MEN 2A

Classical MEN 2 with 100% MTC, approximately 50% pheochromocytoma.

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Pheochromocytoma

Tumor often leading to hypertensive crises, seen in MEN 2 syndromes.

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MTC genetics

RET mutations at specific exons (10, 11, 13-16) are linked to MEN 2.

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MEN 2B characteristics

Aggressive medullary thyroid carcinoma, pheochromocytoma, and marfanoid habitus.

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Prophylactic thyroidectomy

Preventive surgery recommended in high-risk patients with MEN 2.

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Study Notes

Medullary Thyroid Cancer (MTC)

  • MTC arises from C cells in the thyroid, producing calcitonin.
  • It's distinct from differentiated thyroid cancers (papillary and follicular).
  • About 75% of cases are sporadic; 25% are familial, linked to MEN 2 syndromes.
  • Originates from neural crest-derived C cells.
  • Associated with RET proto-oncogene mutations.
  • Commonly metastasizes to lymph nodes (cervical, mediastinal), lungs, liver, and bones.
  • Typical presentation includes a painless, firm neck mass, potentially causing dysphagia or hoarseness.
  • Diarrhea, flushing, and endocrine symptoms (e.g., hyperparathyroidism, pheochromocytoma) might occur.

Diagnostic Evaluation

  • Biochemical Markers: Serum calcitonin (highly sensitive, linked to tumor burden), CEA (often high in advanced disease), & RET proto-oncogene mutation testing.
  • Imaging: Thyroid ultrasound (initial), FNA with calcitonin staining (confirmation), CT/MRI for metastasis, PET-CT (advanced cases), MIBG/Octreotide scans (considering other neuroendocrine tumors).

Management

  • Surgical Treatment (Localized): Total thyroidectomy with central neck lymph node dissection.
  • Prophylactic thyroidectomy: For MEN 2 carriers (age dependent on mutation risk).
  • Lateral neck dissection: For node-positive disease.
  • Adjuvant Treatment (Metastatic/Advanced): Targeted therapy (tyrosine kinase inhibitors like cabozantinib, vandetanib), radiation therapy (palliation), calcitonin monitoring (residual/recurrent disease).
  • Prognosis Sporadic MTC: ~75% 10-year survival. Familial MTC (esp. MEN 2B): poorer prognosis given early onset and aggressive nature.

Multiple Endocrine Neoplasia (MEN)

  • MEN syndromes are hereditary, caused by mutations affecting cell growth, impacting multiple endocrine glands.

MEN 2

  • MTC is a hallmark of MEN 2.

MEN 2A

  • 100% MTC risk
  • Pheochromocytoma (approx. 50%) – potentially life-threatening.
  • Primary hyperparathyroidism (approx. 20-30%).
  • RET mutations (exons 10, 11, 13, 14, 15, 16)

MEN 2B

  • 100% MTC risk (often aggressive, early-onset)
  • Pheochromocytoma (approx. 50%)
  • Marfanoid habitus, mucosal neuromas (lips, tongue, etc.)
  • No hyperparathyroidism.
  • RET mutations (exons 15, 16); M918T mutation associated with poorer prognosis.
  • Prophylactic thyroidectomy at an appropriate age.

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Description

This lesson covers medullary thyroid cancer (MTC), which originates from C cells in the thyroid and produces calcitonin. It discusses the genetic and sporadic forms of MTC, common sites of metastasis, clinical presentation, and diagnostic evaluation using biochemical markers and imaging techniques.

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