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Multiple Endocrine Neoplasia (MEN) I Case Presentation 62 y/o Female Pmhx - HTN & Cholecystectomy for biliary colic (1995) Presents to OSH for abd pain, N/V. Afeb ... – PowerPoint PPT presentation

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Title: Multiple Endocrine Neoplasia (MEN) I Case Presentation 6


1
Multiple Endocrine Neoplasia (MEN) I
2
Case Presentation
  • 62 y/o Female
  • Pmhx - HTN Cholecystectomy for biliary colic
    (1995)
  • Presents to OSH for abd pain, N/V. Afeb, mild
    right sided abd tenderness. Elevated LFTs.
  • ERCP performed for presumed retained stone
    sphincterotomy w/ stent placement.
  • Post ERCP cont abd pain developed MI

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  • Transferred to MSH for cardiac cath/stent
  • post cath cont abd pain GI workup begun
  • 9/4 CT

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  • Biochemical w/u for pheochromocytoma found to be
    neg (nl urine catecholamines nl normetanephrine
    / metanephrine)
  • Biochemical w/u for poss neuroendocrine
    pancreatic lesion gastrin mild elev. (172/111)
    mild elev. chromogranin A (38/18) nl glucagon
  • Pt also at this time reported FMHx of
    multiglandular hyperparathyroid in mother labs
    drawn hypercalcemic, elevated PTH elevated
    prolactin
  • Head CT negative pt not candidate for MR (metal
    stent)

10
  • 9/9 SBS negative
  • 9/11 9/12 Capsule endoscopy performed capsule
    held up in antrum of stomach EGD removal of
    capsule and placement of second capsule in
    duodenum
  • Surgery consulted N/V Afeb normal VS lower
    abdominal tenderness, nl lactate, mild levated
    WBC (12-13)
  • 9/14 repeat CT showed

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  • metallic density in RLQ capsule cont wall
    thickening
  • 9/17 Pt to OR for Small bowel resection/retrieval
    of capsule

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  • Pathology Segment of sm bowel w/ ischemic
    necrosis carcinoid tumor (1cm) extending into
    muscularis propria

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MULTIPLE ENDOCRINE NEOPLASIA MEN I SYNDROME
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Contents
  • 1. Parathyroid disease
  • 2. Anterior Pituitary Adenomas
  • 3. Entero-pancreatic tumors
  • Insulinomas
  • Gastrinomas
  • Glucagonomas
  • VIPomas
  • Somatostatinomas
  • Pancreatic Polypeptide-Producing tumors
  • Rare Islet Cell tumors
  • Surgical Therapy
  • 4. Other tumors

17
What is MEN1 ?
  • AD disorder on chromosome 11
  • Consensus recommends that MEN- 1 defined as 2/3
    main MEN-1 tumor types
  • Familial MEN-1 is at least one MEN-1case plus
    first degree relative w/ 1/3 tumors

18
Primary Hyperparathyroidism
  • 100 penetrance earliest / most common
    manifestation
  • 1-2 all hyperparathyroidism is MEN-1
  • Multiglandular involvement (unlike sporadic
    hyperPTH w/ single gland adenoma)
  • Diagnosing
  • Clinically - Stones, bones, groans, mental
    overtones
  • Diagnosis Biochemical hypercalcemia w/ hi
    serum PTH
  • Imaging sestamibi bone densitometry

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Primary Hyperparathyroidism
  • Indications for surgery
  • Symptomatic or marked hypercalcemia
  • Nephrolithiasis
  • Diminished bone density / osteopenia
  • Presence of severe gastric ulcers 20 to
    gastrinoma (hypercalcemia inc release of gastrin
    worsens hypergastrinemia and parathyroidectomy
    reduces gastrin secretion -- however PPIs work
    well

20
Therapy for Primary Hyperparathyroidism
  • Subtotal parathyroidectomy (removal of 3.5) with
    transcervical near-total prophylactic thymectomy
  • Parathyroid remnants in situ OR autograft
    implanted into forearm
  • Endoscopic parathyroidectomy not appropriate b/c
    difficulty in identifying all four glands

21
Primary Hyperparathyroidism (cont)
  • Complications of Parathyroid Surgery
  • Temporary hypocalcemia common
  • 4 risk permanent hypoPTH, 30 risk
    persistent/recurrent hyperPTH
  • Graft function successful in gt90 of
    autotransplantations
  • High rate of recurrence after successful surgical
    therapy unlike sporadic 67 _at_ 8yrs

22
Anterior Pituitary tumors
  • Prevalence in MEN-1 ranges 15-60 in different
    series
  • First clinical manifestation in up 25 of cases
  • Two-thirds are microadenomas (lt1cm)
  • Prolactinomas 60, somatotrophinomas 20,
    corticotrophinomas and null cell tumors 15
  • Pt p/w sx of amenorrhea, galactorrhea,
    infertility, impotence in men acromegaly or
    cushings disease
  • Monitor PRL, IGF-1
  • CT/MRI
  • Trans-sphenoidal resection of pituitary tumor

23
Entero-pancreatic tumors
  • Gastrinomas
  • Insulinomas
  • Glucagonomas
  • VIPomas
  • Somatostatinomas
  • Pancreatic Polypeptide-Producing tumors

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Entero-pancreatic tumors
  • Prevalence ranges from 30-75
  • Lesions tend to be multicentric
  • Micro/macroadenomas -gt invasive -gt metastaic
    carcinoma
  • As a result of the effective rx available for
    parathyroid pituitary maifestations in MEN-1
    the malignant potential of these
    entero-pancreatic lesions is now the primary life
    threatening manifestation of the syndrome.

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Gastrinoma
  • Most common represent 50 of all
    entero-pancreatic tumors in MEN1 tend to be small
    multiple lesions
  • MEN1 gastrinomas mostly in duodenum, 50
    metastasized at Dx
  • Poor prognosis w/ pancreatic (not duodenal)
    mets higher gastrin level
  • Biochemical diagnosis incl hypergastrinemia,
    mult peptic ulcers hi gastric acid output
  • Imaging octreotide scan, EGD U/S, CT/MRI
    portal venous sampling
  • MEDICAL TREATMENT
  • PPIs - Omeprazole

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Gastrinoma (cont)
  • SURGICAL TREATMENT
  • For solitary sporadic gastrinoma - complete
    resection
  • MEN1 gastrinomas freq mult/mets controversial
    role of surgery
  • Intraop US
  • Gastrinoma triangle pancreas/duodenum/CBD
  • Duodenotomy
  • Tumors head pancreas-enucleated
  • Tumors body/tail pancreas distal pancreatectomy
    - splenectomy
  • Liver mets- wedge resection

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Norton et al. 1999 NEJM
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Gastrinoma (cont)
  • METASTATIC GASTRINOMA
  • 25-90 of ZE patients
  • ChemoRx streptozocin 5 FU
  • Hepatic arterial chemoembolization
  • Octreotide
  • Interferon ?
  • Aggressive debulking surgery

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Insulinoma
  • Pt p/w hypoglycemia after fast/exertion sx
    improve w/ glucose intake inappropriately
    elevated insulin
  • 90 solitary and benign
  • head, body and tail pancreas
  • Malignancy - size gt 6cm or liver/nodal mets
  • MEDICAL TREATMENT
  • Control Sx prior to surgery
  • Frequent small CHO meals until operated on
  • Octreotide

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Insulinoma (cont)
  • SURGICAL TREATMENT
  • Mainstay
  • Precise identification w/ intraop ultrasound
  • Large malignant tumors or adjacent to vital
    structures - distal pancreatectomy or Whipples
    pancreaticoduodenectomy

31
Insulinoma (cont)
  • SURGERY FOR MALIGNANT INSULINOMA
  • Curative
  • Whipples or subtotal pancreatectomy-splenectomy,
    excision of liver metastases
  • Palliation for metastatic disease
  • ChemoRx
  • Chemo-embolization
  • Alcohol injection, cryotherapy, radiofrequency
    ablation
  • COMPLICATIONS of SURGICAL TREATMENT
  • Abscess
  • Fistula
  • Pseudocyst
  • Wound infection

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Glucagonoma
  • Necrolytic migratory erythema
  • Hypoaminoacidaemia, Type II DM, muscle wasting,
    cachexia, DVT/PE
  • Tail of Pancreas most common site
  • Surgical removal is treatment of choice
  • However, 50-80 w/ mets at time of diagnosis
  • Optimize medical conditions, TPN, octreotide
  • ChemoRx - 5FU, streptozotocin, dacarbazine

33
VIPoma
  • Vasoactive Intestinal Peptide tumors
  • Watery diarrhoea, hypokalaemia, achlorhydria
    (WDHA), hypovolaemia, dehydration
  • 5-10 litres stool/day
  • correct dehydration
  • Surgical management w/ distal pancreatectomy
    curative

34
Somatostatinoma
  • Duodenum/pancreas
  • Steatorrhoea, cholelithiasis, type II DM,
    hypochlorhydria
  • Medical problems hyperglycaemia, malnutrition
  • Resect primary and debulk hepatic mets
  • Cholecystectomy

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Pancreatic Polypeptide-Producing tumors
  • Non-functioning
  • Malignant and large at time of Dx
  • Extrahepatic bile duct obstruction, GI bleeding,
    GI obstruction
  • Surgical resection
  • chemoRx for residual disease

36
Other tumors
  • CARCINOID
  • 3 of MEN1 bronchi, gi tract, pancreas, or
    thymus
  • Mostly asymptomatic no signs of carcinoid
    syndrome
  • ADRENOCORTICAL LESIONS
  • 20-40 MEN1
  • Usually bilateral, hyperplastic, non-functional
  • No consensus on management of these lesions

37
Other tumors
  • BENIGN THYROID tumorS
  • LIPOMAS
  • Cutaneous and visceral
  • Up to 1/3 of MEN1 patients
  • Tend not to recur once excised
  • CNS EPENDYMOMAS
  • COLLAGENOMAS, ANGIOFIBROMAS

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MULTIPLE ENDOCRINE NEOPLASIA MEN I SYNDROME
  • - Autosomal dominant disorder caused by loss of a
    tumor suppressor gene on chromosome 11.
  • 1. Parathyroid hyperplasia or adenoma ? ? Ca
    renal calculi.
  • 2. Pituitary adenoma MC prolactinoma, also GH
    ACTH (Cushings
  • disease)
    producing tumors
  • 3. Entero-pancreatic tumors ? excessive secretion
    of
  • - gastrin ? peptic ulcers (Zollinger-Ellison
    syndrome),
  • - insulin ? hypoglycemia
  • - serotonin (carcinoid syndrome)
  • - VIP (vasoactive intestinal polypeptide) ?
    watery diarrhea

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References
  • Guideline for Diagnosis and Therapy of MEN Type 1
    and Type 2 Consensus. Journal of Clinical
    Endocrinology Metabolism. 86(12)5658-71, 2001
    Dec.
  • Gauger PG, Thompson NW. Early Surgical
    Intervention and strategy in patients with
    multiple endocrine Neoplasia Type 1. Best
    Practice and Research Clinical Endocrinology
    Metabolism. 15(2)213-223, 2001
  • Veldhuis et al. Therapeutic Controversy. Surgical
    Versus Medical Management of Multiple Endocrine
    Neoplasia Type 1. Journal of Clinical
    Endocrinology and Metabolism. 82(2)357-364
  • Burgess et al. The outcome of subtotal
    thyroidectomy for treatment of hyperparathyroidism
    in MEN-1. Arch Surg 1998133126.

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