eMedicine Specialties > Dermatology > Internal Medicine

Multiple Endocrine Neoplasia Type 1: Follow-up

Author: Thomas N Darling, MD, PhD, Director of Dermatologic Research, Associate Professor of Dermatology, Department of Dermatology, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Oct 16, 2008

Follow-up

Complications

  • Endocrine tumors
    • Tumors may hypersecrete hormone, causing hypercalcemia and recurrent nephrolithiasis (hyperparathyroidism), Zollinger-Ellison syndrome (hypergastrinemia), hypoglycemia (hyperinsulinemia), amenorrhea (hyperprolactinemia), or acromegaly (excess growth hormone).
    • Tumors of the pituitary gland may cause symptoms by mass effects.
    • Pancreatic endocrine tumors, particularly gastrinomas, become malignant in about 50% of patients with multiple endocrine neoplasia type 1 (MEN1). Untreated, patients may die from peptic ulcer disease, metastatic endocrine pancreatic carcinoma, or foregut carcinoid malignancy.
  • Cutaneous tumors: Angiofibromas, collagenomas, and lipomas do not typically cause symptoms, and they are mostly of cosmetic concern.

Patient Education

 


More on Multiple Endocrine Neoplasia Type 1

Overview: Multiple Endocrine Neoplasia Type 1
Differential Diagnoses & Workup: Multiple Endocrine Neoplasia Type 1
Treatment & Medication: Multiple Endocrine Neoplasia Type 1
Follow-up: Multiple Endocrine Neoplasia Type 1
Multimedia: Multiple Endocrine Neoplasia Type 1
References

References

  1. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. Dec 2001;86(12):5658-71. [Medline].

  2. Asgharian B, Turner ML, Gibril F, Entsuah LK, Serrano J, Jensen RT. Cutaneous tumors in patients with multiple endocrine neoplasm type 1 (MEN1) and gastrinomas: prospective study of frequency and development of criteria with high sensitivity and specificity for MEN1. J Clin Endocrinol Metab. Nov 2004;89(11):5328-36. [Medline].

  3. Agarwal SK, Kennedy PA, Scacheri PC, Novotny EA, Hickman AB, Cerrato A, et al. Menin molecular interactions: insights into normal functions and tumorigenesis. Horm Metab Res. Jun 2005;37(6):369-74. [Medline].

  4. Lemos MC, Thakker RV. Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene. Hum Mutat. Jan 2008;29(1):22-32. [Medline].

  5. Pack S, Turner ML, Zhuang Z, Vortmeyer AO, Böni R, Skarulis M, et al. Cutaneous tumors in patients with multiple endocrine neoplasia type 1 show allelic deletion of the MEN1 gene. J Invest Dermatol. Apr 1998;110(4):438-40. [Medline].

  6. Vortmeyer AO, Böni R, Pack SD, Darling TN, Zhuang Z. Perivascular cells harboring multiple endocrine neoplasia type 1 alterations are neoplastic cells in angiofibromas. Cancer Res. Jan 15 1999;59(2):274-8. [Medline].

  7. Marx SJ. Molecular genetics of multiple endocrine neoplasia types 1 and 2. Nat Rev Cancer. May 2005;5(5):367-75. [Medline].

  8. Darling TN, Skarulis MC, Steinberg SM, Marx SJ, Spiegel AM, Turner M. Multiple facial angiofibromas and collagenomas in patients with multiple endocrine neoplasia type 1. Arch Dermatol. Jul 1997;133(7):853-7. [Medline].

  9. Hoang-Xuan T, Steger JW. Adult-onset angiofibroma and multiple endocrine neoplasia type I. J Am Acad Dermatol. Nov 1999;41(5 Pt 2):890-2. [Medline].

  10. Sakurai A, Hashizume K, Fukushima Y. Facial angiofibroma as an initial manifestation in multiple endocrine neoplasia type 1. Intern Med. 2008;47(11):1067-8. [Medline].

  11. Vidal A, Iglesias MJ, Fernández B, Fonseca E, Cordido F. Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1. J Eur Acad Dermatol Venereol. Jul 2008;22(7):835-8. [Medline].

  12. Sakurai A, Matsumoto K, Ikeo Y, Nishio SI, Kakizawa T, Arakura F, et al. Frequency of facial angiofibromas in Japanese patients with multiple endocrine neoplasia type 1. Endocr J. Oct 2000;47(5):569-73. [Medline].

  13. Roach ES, Sparagana SP. Diagnosis of tuberous sclerosis complex. J Child Neurol. Sep 2004;19(9):643-9. [Medline].

  14. Fuleihan Gel-H, Rubeiz N. Dermatologic manifestations of parathyroid-related disorders. Clin Dermatol. Jul-Aug 2006;24(4):281-8. [Medline].

  15. Xia Y, Darling TN. Rapidly growing collagenomas in multiple endocrine neoplasia type I. J Am Acad Dermatol. May 2007;56(5):877-80. [Medline].

  16. Bittencourt RC, Huilgol SC, Seed PT, Calonje E, Markey AC, Barlow RJ. Treatment of angiofibromas with a scanning carbon dioxide laser: a clinicopathologic study with long-term follow-up. J Am Acad Dermatol. Nov 2001;45(5):731-5. [Medline].

Further Reading

Keywords

multiple endocrine neoplasia type 1, MEN-1, Wermer syndrome, multiple endocrine adenomatosis, MEN1, endocrine tumors, cutaneous tumors, angiofibromas, collagenomas, lipomas

Contributor Information and Disclosures

Author

Thomas N Darling, MD, PhD, Director of Dermatologic Research, Associate Professor of Dermatology, Department of Dermatology, Uniformed Services University of the Health Sciences
Thomas N Darling, MD, PhD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Terry L Barrett, MD, Director, Associate Professor, Department of Dermatology, Division of Dermatopathology and Oral Pathology, Johns Hopkins University School of Medicine
Terry L Barrett, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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