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Updated: Dec 26 2021

Multiple Endocrine Neoplasias

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  • Snapshot
    • A 35-year-old women presents to her primary care physician with 2 months of severe episodes of headache, tremulousness, palpitations, and anxiety. The patient has noted a recent change in her voice, and she has difficulty swallowing solids. On physical exam there is a palpable, nontender swelling in the front of her neck that moves with swallowing. There is no cervical lymphadenopathy. Lab studies show hypercalcemia. An ultrasound of the neck shows irregular calcifications within a mass, and an MRI of the abdomen shows the presence of bilateral adrenal lesions. (Multiple endocrine neoplasia [MEN] type IIA)
  • Introduction
    • Overview
      • cancer syndrome with several associated endocrine neoplasias
      • 3 MEN types
        • MEN I
        • MEN IIA
        • MEN IIB
      • all have autosomal dominant inheritance
  • Epidemiology
    • Prevalence
      • MEN I: 1 in 50,000
      • MEN II: 1 in 30,000
  • Classification
    • MEN I (Wermer syndrome)
      • MEN1 gene mutation
      • clinical definition: presence of 2 of the 3 P's
        • parathyroid tumors
          • increased PTH causes hypercalcemia
            • results in kidney stones
        • pituitary tumors (prolactin or GH)
        • pancreatic islet cell/endocrine tumors
          • Zollinger-Ellison syndrome
            • causes gastric ulcers
          • insulinomas
          • VIPomas
            • part of watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome
          • glucagonomas
    • MEN II
      • divided into MEN IIA and IIB
      • common features
        • medullary thyroid carcinoma
        • pheochromocytoma
        • associated with RET proto-oncogene mutation
          • gain of function mutation
      • MEN IIA (Sipple syndrome)
        • medullary thyroid carcinoma
          • secretes calcitonin
        • pheochromocytoma
        • parathyroid tumors
      • MEN IIB
        • medullary thyroid carcinoma
        • pheochromocytoma
        • oral/intestinal ganglioneuromatosis
          • associated with marfanoid habitus
  • Presentation
    • Symptoms
      • dependent on which type of MEN syndrome
      • MEN I
        • flank pain
          • secondary to kidney stones
        • abdominal pain and dark stools
          • secondary to gastric ulcers
        • watery diarrhea
      • MEN II
        • neck pain, dysphagia, and hoarseness
          • secondary to thyroid mass
        • episodic headaches, palpitations, tremulousness, and anxiety
          • secondary to pheochromocytoma
    • Physical exam
      • MEN I
        • costovertebral angle (CVA) tenderness
        • abdominal tenderness
      • MEN II
        • hypertension
        • tachycardia
        • neck mass or palpable thyroid nodule
        • cervical lymphadenopathy
  • Imaging
    • Ultrasound of neck
      • indications
        • patient with dysphagia or hoarseness
      • findings
        • thyroid nodule
          • follow up with thyroid scintigraphy (radioactive iodine uptake scan)
    • Magnetic resonance imaging (MRI) or computed tomography (CT) scan of abdomen
      • indications
        • patient with symptoms consistent with pheochromocytoma
      • findings
        • adrenal gland mass
  • Studies
    • TSH levels
      • to evaluate thyroid function
        • decreased TSH more concerning for malignancy
    • Serum calcitonin
      • tumor marker for medullary thyroid cancer
    • Free serum metanephrine level
      • elevated in pheochromocytoma
      • follow up with 24-hour urin collection
        • increased urine metanephrines
        • elevated vanillyl mandelic acid (due to breakdown of norepinephrine and epinephrine)
    • Serum PTH and calcium levels
      • elevated in parathyroid tumor
    • Serum glucose
      • elevated in glucagonoma
      • decreased in insulinoma
    • Serum glucagon
      • elevated in glucagonoma
    • Serum insulin and C-peptide
      • elevated in insulinoma
    • Serum gastrin
      • increased in gastrinoma
  • Treatment
    • Medical
      • proton pump inhibitor
        • indications
          • Zollinger-Ellison syndrome
        • modalities
          • omeprazole
          • lansoprazole
    • Surgical
      • thyroidectomy
        • indications
          • medullary thyroid cancer
            • MEN IIA or IIB
        • follow with thyroid hormone supplementation
      • parathyroidectomy
        • indications
          • parathyroid tumor
            • MEN I
      • duodenal-pancreatic surgery
        • indications
          • Zollinger-Ellison syndrome refractory to medical management
          • pancreatic neuroendocrine tumors
      • adrenalectomy
        • indications
          • pheochromocytoma
        • preoperative medications to prevent hypertensive crisis
          • must start with alpha blockade
          • follow with beta blockade
        • modalities
          • complete bilateral adrenalectomy is recommended to reduce risk of recurrence
  • Prognosis
    • Dependent on MEN type and which neoplasias occur in individual patient
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