Parathyroid

Multiple endocrine neoplasia

  • Autosomal dominant
MEN 1
(Wermer's)		 MEN 2A
(Sipple's)		 MEN 2B
MEN1/menin (tumor suppressor), chromosome 11 ret/tyrosine kinase
3P/diamond 2P/square 1P/triangle

Parathyroid adenoma/hyperplasia

  • Parathyroidectomy if indicated - (> 3.5 glands) with autotransplant, as usually multiple adenomas
  • Hypercalcemia - kidney stones

 

Mucosal oral/gastrointestinal ganglioneuromas
(MMMP)

Marfanoid habitus

Pancreatic, GI NET (gastrinoma (Zollinger Ellison/ulcers) > insulinoma, VIPoma, glucagonoma)

  • Gastrin levels (can be falsely elevated by hypercalcemia/PPIs; measure when normocalcemia and off PPI x > 2 weeks)
  • PPI for gastrin

Medullary thyroid cancer

  • Calcitonin
  • Prophylactic thyroidectomy
  • More aggressive in MEN 2B (thyroidectomy in 1st year of life)
Pituitary adenoma (prolactin, GH, vision problems)

Pheochromocytoma

  • Urine 24-hr fractionaed metanephrines and catecholamines
  • Plasma metanephrines (high sens, low spec)
Cutaneous tumors - angiofibroma, collagenoma, lipoma    

Indications for parathyroidectomy

  • Symptomatic hypercalcemia or Ca > 1 mg/dL over normal
  • End-organ complications (osteoporosis, CKD, stones)
  • Increased risk for complications - urine Ca > 400 mg/day
author: last edited: Oct. 21, 2018, 11:14 a.m. | pk: 137 | unpublished