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Heme/Onc > Endocrine Cancers > Flashcards

Endocrine Cancers Flashcards

1. List risk factors for the development of thyroid cancers of follicular cell origin (MKS1b) 2. Describe the components of the treatment of differentiated thyroid cancers of follicular cell origin (MKS3d) 3. List the genes that, when mutated, lead to multiple endocrine neoplasia (MEN) 1 and MEN 2 (MKS1b). 4. Describe the endocrine tumors and non-endocrine manifestations of MEN 1 and MEN 2 (MKS1d) 5. Describe the tumors that arise from each layer of the adrenal cortex and medulla, the h

1
Q

What is the epidemiology of endocrine cancers?

A
  • 64,860 new cases in 2015
    • 2890 deaths in 2015 (4.4%)
  • 62,450 – thyroid cancer (96%)
    • 1950 deaths (3%)
  • 2,410 – other endocrine cancers (3.7%)
    • 940 deaths (39%)
  • Other endocrine cancers are more deadly!
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2
Q

What is the epidemiology of thyroid cancer?

A
  • 62,450 new cases in 2015
  • 47,230 cases in women
  • 15,220 cases in men
  • 5th most common cancer in women
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3
Q

What are the major types of thyroid cancers?

A
  • Follicular cells
    • Papillary thyroid cancer
    • Follicular thyroid cancer
    • Hurthle cell cancer
    • Anaplastic cancer
  • Parafollicular cells
    • Medullary thyroid cancer
      • 75% sporadic
      • 25% familial
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4
Q

What is the clinical presentation of thyroid cancer?

A
  • Thyroid nodule
  • Screening
    • Presentation with another diagnosis known to be associated with thyroid cancer
    • Genetic testing for a syndrome known to be associated with thyroid cancer
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5
Q

How are thyroid cancers diagnosed?

A
  • Assessment of risk factors
    • Radiation exposure (thyroid cancers of follicular cell origin only)
    • Family history
    • Syndromic associations
  • Physical exam
  • Diagnostic tests
    • Evaluation of hormone excess
    • Ultrasound
    • Biopsy
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6
Q

What is the treatment of thyroid cancers?

A
  • Differentiated thyroid cancers of follicular cell origin
    • Thyroidectomy
    • Radioactive iodine (selective use)
    • TSH suppression
  • Thyroid cancer of parafollicular cell origin (medullary thyroid cancer)
    • Thyroidectomy + lymph node dissection
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7
Q

What is the epidemiology of MEN?

A
  • MEN 1 – prevalence 1 per 30,000
  • MEN 2 – prevalence 1 per 35,000
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8
Q

What is the genetics of MEN?

A
  • MEN 1
    • MEN1 gene, a tumor suppressor gene
    • Encodes a protein called menin
    • Autosomal dominant pattern of inheritance
    • No genotype-phenotype correlation
  • MEN 2
    • (REarranged during Transfection) (RET) protooncogene
    • Encodes a membrane receptor tyrosine kinase that is expressed by neuroendocrine and neuronal cells
    • Autosomal dominant pattern of inheritance
    • Genotype-phenotype correlations exist
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9
Q

What is the clinical presentation of the various types of MEN?

A
  • MEN 1
    • Primary hyperparathyroidism
      • Usually the first manifestation of the syndrome
    • Pancreatic/duodenal neuroendocrine tumors
      • Islet hyperplasia
      • Microadenomas/Macroadenomas
      • Islet cell carcinomas
    • Pituitary tumors
    • Less common manifestations
      • Adrenal tumors
      • Foregut carcinoid tumors
      • Others
  • MEN 2A
    • Medullary thyroid cancer
      • Usually the first manifestation of the syndrome
    • Pheochromocytoma
    • Primary hyperparathyroidism
    • Less common manifestations
      • Cutaneous lichen amyloidosis
      • Hirschsprung disease
  • MEN 2B
    • Medullary thyroid cancer
    • Pheochromocytoma
    • Marfanoid body habitus
    • Mucosal neuromas
    • Intestinal ganglioneuromas
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10
Q

What is the treatment of MEN?

A
  • Primary hyperparathyroidism
    • Parathyroidectomy
  • Pancreatic neuroendocrine tumors
    • Treatment depends on hormone produced, type of tumor, and results of evaluation for metastases (about 50% will have metastasized to regional lymph nodes/liver at the time of diagnosis)
  • Pituitary tumors
    • Treatment depends on hormone produced and size/resectability of tumor (surgery done endonasal/transsphenoidal)
  • Medullary thyroid cancer
    • Thyroidectomy + lymph node dissection
    • Prophylactic thyroidectomy in patients with a known RET mutation
  • Pheochromocytoma
    • Adrenalectomy
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11
Q

What is the epidemiology and pathology of adrenal neoplasms?

A
  • Epidemiology
    • Incidental adrenal masses exist in approximately 5% of people
  • Pathology
    • Adrenal Cortex
      • Cortical adenomas
      • Adrenocortical carcinomas
    • Adrenal Medulla
      • Pheochromocytoma
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12
Q

What is the clinical presentation of adrenal neoplasias?

A
  • Incidental adrenal mass
  • Symptoms of hormone excess
  • Screening
    • Presentation with another diagnosis known to be associated with an adrenal tumor
    • Genetic testing for a syndrome known to be associated with an adrenal tumor
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13
Q

What is involved in the diagnosis of adrenal neoplasias?

A
  • Assessment of risk factors
    • Family history
    • Syndromic associations
  • Physical exam
  • Diagnostic tests
    • Evaluation of hormone excess
      • Hormones from the adrenal cortex
      • Hormones from the adrenal medulla
    • Additional imaging (selective use)
    • Biopsy (selective use)
    • Adrenalectomy (selective use)
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14
Q

What is the treatment of adrenal neoplasias?

A
  • Observation
  • Adrenalectomy
  • Adjuvant mitotane (selective use for adrenocortical carcinoma)
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Heme/Onc (50 decks)

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