Endocrine Cancers Flashcards Preview

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Flashcards in Endocrine Cancers Deck (14):
1

What is the epidemiology of endocrine cancers?

  • 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!

2

What is the epidemiology of thyroid cancer?

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

3

What are the major types of thyroid cancers?

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

4

What is the clinical presentation of thyroid cancer?

  • 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

5

How are thyroid cancers diagnosed?

  • 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

6

What is the treatment of thyroid cancers?

  • 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

7

What is the epidemiology of MEN?

  • MEN 1 – prevalence 1 per 30,000
  • MEN 2 – prevalence 1 per 35,000

8

What is the genetics of MEN?

  • 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

9

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

  • 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

10

What is the treatment of MEN?

  • 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

11

What is the epidemiology and pathology of adrenal neoplasms?

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

12

What is the clinical presentation of adrenal neoplasias?

  • 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

13

What is involved in the diagnosis of adrenal neoplasias?

  • 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)

14

What is the treatment of adrenal neoplasias?

  • Observation
  • Adrenalectomy
  • Adjuvant mitotane (selective use for adrenocortical carcinoma)