Hamartoma

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Classification according to ICD-10
Q85.9 Phacomatosis, unspecified
ICD-10 online (WHO version 2019)

A hamartoma is a congenital or later manifesting malformation of a body tissue in which an abnormal composition of commonly occurring tissue structures is present. As a major difference to neoplasia , the growth of a hamartoma is not autonomous , but goes hand in hand with the growth of the surrounding tissue.

etymology

The term hamartom is a loan word from Hamartie or Hamartia (Greek ἁμαρτία ), which in medical terminology describes a local tissue defect as a result of an embryonic malformation of the germinal tissue. Actually the word hamartia from the language of the native Greek tragedy and the New Testament , where the importance of not hit , miss , miss the mark or misconduct has.

properties

Hamartia is initially a collective term for tissue changes that are understood as malformations with local excess tissue. Such tissue changes are considered benign . In contrast to conventional benign tumors , they have no growth autonomy. In rare cases, may result from a hamartoma however malignant tumor form of Hamartoblastom is called.

Hamartomas are developmental disorders that develop in the germinal tissue and lead to a scattered localization of body tissue as a tumor-like malformation during embryonic blastogenesis . As a rule, they have parts of cartilage, connective tissue or adipose tissue. Hamartomas can in principle occur anywhere - frequent locations are in particular the ovaries , skin , liver , lungs and brain .

A special form are hamartoma-like neoplasms due to a fetal inclusion of a former twin fruit possibly decades ago : embryonic pluripotent stem cells still preserved in the surviving twin begin to multiply and give rise to relatively highly differentiated neoplasms such as milk teeth or hair in extremely atypical locations, such as in the abdominal cavity or in the Skull.

Tissue-specific occurrence

Section through a spleen (light tissue on the right) with a hamartoma (dark tissue on the left)

In the eye , hamartomas of the iris are called Lisch nodules . They are particularly observed in neurofibromatosis .

A hamartoma in the hypothalamus (hypothalamic hamartoma) can trigger epileptic fits. In some cases these hamartomas produce the hormone gonadoliberin (GnRH, English gonadotropin-releasing hormone ), which triggers an early onset of puberty ( precocious puberty ) in children .

A hamartoma of the skin is called a nevus (colloquially also "birthmark"). There are many different types of nevi such as: B. pigment nevi or verrucous nevi.

Hamartomas of the bone tissue are called exostoses . Hamartomas that occur in the lungs are known as cystic adenomatoid malformations .

Multiple occurrences

A multiple occurrence of hamartomas is called hamartosis. A synonym is the term multiple hamartosis syndrome, to which Cowden syndrome is counted. Sometimes hamartomas occur in multiple organ systems. The diseases in which this is the case are called phakomatoses for historical reasons according to the Greek word for lens ( φακός , phakós ) and because of lens-shaped changes in the fundus of the eye in some of these diseases .

literature

  • Max Eder, Peter Gedik (ed.): Textbook of general pathology and pathological anatomy . Springer, Berlin 1974

Web links

Individual evidence

  1. Alphabetical directory for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 348
  2. ^ Peter Altmeyer: "Therapy lexicon Dermatology and Allergology: Therapy compact from A to Z". Springer, Berlin 2005, page 348
  3. Duden: The foreign dictionary. Mannheim 2007, Lemma Hamartie.
  4. ^ Roche Lexicon Medicine Online (see web links.)
  5. Peter Reuter: Springer Lexicon Medicine. Springer, Berlin a. a. 2004, ISBN 3-540-20412-1 (Lemma nevus).
  6. Peter Reuter: Springer Lexicon Medicine. Springer, Berlin a. a. 2004, ISBN 3-540-20412-1 (Lemma Hamartose).