LAMB syndrome

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Classification according to ICD-11
5A70.Y Other specified Cushing syndrome
ICD-11 ( WHO version 2019)

The LAMB syndrome was first described in 1984 and belongs to the group of "familial myomas". The combination of moles and arterial circulation disorders - such as strokes - in young people should bring this rare syndrome to mind. The tumors of the heart can usually be detected with an echocardiography . About 10% of all myxomas are part of this syndrome.

"LAMB" is an acronym for

L entigines (liver spots),
A trial (i.e. atrial ) myxomas ,
M ukocutaneous myxomas (myxomas of the skin and mucous membrane ) and
B tepid nevi .

The therapy consists of an early surgical removal of the heart tumors .

Patients are on average 20 years old and, unlike other patients with myxomas of the heart, they are more likely to have myxomas in atypical locations and develop multiple tumors that are more likely to recur after removal .

The LAMB syndrome and also the NAME syndrome are possibly variants of the Carney complex with only latent overactive endocrine glands.

See also

Individual evidence

  1. ^ AR Rhodes, RA Silverman, TJ Harrist, AR Perez-Atayde: Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi: The "LAMB" syndrome. In: J Am Acad Dermatol. 1984; 10, pp. 72-82.
  2. M. Dietel, N. Suttorp, M. Zeitz et al. (Eds.): Harrisons internal medicine. German edition in cooperation with the Charité . 16th edition. McGraw Hill, ABW-Wissenschaftsverlag, Berlin 2005, ISBN 3-936072-29-9 .
  3. DP Zipes, P. Libby, RO Bonow, E. Braunwald (Eds.): Braunwald's Heart Disease, A Textbook of Cardiovascular Medicine. Elsevier Saunders, Philadelphia 2005, ISBN 0-8089-2305-6 .