Nelson tumor

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Classification according to ICD-10
E24.1 Nelson tumor
ICD-10 online (WHO version 2019)

The Nelson-tumor , and Nelson's syndrome , is a fast growing, aggressive pituitary tumor with ACTH production after bilateral adrenalectomy due to Cushing's syndrome .

The first description comes from 1958 by the American endocrinologist Don H. Nelson and colleagues

The syndrome should not be confused with Emery Nelson Syndrome or the English Dermal Ridges, Nelson Syndrome

distribution

The frequency is reported to be around 40% of patients after bilateral adrenalectomy.

Clinical manifestations

Clinical criteria are:

  • Condition after bilateral adrenalectomy due to Cushing's syndrome
  • Hyperpigmentation of the skin and mucous membranes
  • Mass in the Sella turcica
  • Visual field impairment, headache
  • massively increased ACTH in blood serum

literature

  • TD Azad, A. Veeravagu, S. Kumar, L. Katznelson: Nelson Syndrome: Update on Therapeutic Approaches. In: World Neurosurgery . Volume 83, number 6, June 2015, pp. 1135–1140, doi: 10.1016 / j.wneu.2015.01.038 , PMID 25683128 (review).
  • TM Barber, E. Adams, JA Wass: Nelson syndrome: definition and management. In: Handbook of clinical neurology. Volume 124, 2014, pp. 327-337, doi: 10.1016 / B978-0-444-59602-4.00022-8 , PMID 25248597 (review).
  • AL Arregger, EM Cardoso, OB Sandoval, EG Monardes Tumilasci, R. Sanchez, LN Contreras: Hormonal secretion and quality of life in Nelson syndrome and Cushing disease after long acting repeatable octreotide: a short series and update. In: American journal of therapeutics. Volume 21, number 4, 2014 Jul – Aug, pp. E110 – e116, doi: 10.1097 / MJT.0b013e318255bc07 , PMID 22820717 .
  • T. Bianda, C. Schmid: [Nelson syndrome: 20 years after bilateral adrenalectomy for Cushing's disease]. In: Swiss medical weekly. Volume 128, Number 13, March 1998, p. 508, PMID 9583102 .

Individual evidence

  1. ↑ a b Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  2. ↑ a b Nelson syndrome. In: Orphanet (Rare Disease Database).
  3. ↑ emedicine
  4. ^ DH Nelson, JW Meakin, JB Dealy, DD Matson, K. Emerson, GW Thorn ACTH-producing tumor of the pituitary gland. In: The New England Journal of Medicine . Volume 259, Number 4, July 1958, pp. 161-164, doi: 10.1056 / NEJM195807242590403 , PMID 13566440 .
  5. ↑ Emery-Nelson Syndrome. In: Orphanet (Rare Disease Database).
  6. ↑ DERMAL RIDGES, NELSON SYNDROMS.  In: Online Mendelian Inheritance in Man . (English)
  7. ↑ Encyclopedia Dermatology