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Hamartoma

Tae Kim, MD, Gemma Kim, MD and Bao T Huynh, MD Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Hamartoma was first described by Albrecht in 1904.

  • Defined as nonneoplastic disorganized growths of native cells in native tissues

  • Can occur in many different organs, including bile du...

DIAGNOSIS

HISTORY

  • Lung: Endobronchial lesions frequently cause recurrent pulmonary infections, intermittent hemoptysis, and obstructive symptoms (wheezing, stridor, atelectasis). Parenchymal lesions ar...

TREATMENT

MEDICATION

Antiseizure medication if epilepsy is present. Poor success rate for HH. mTOR inhibitors show promise in TSC and other epileptogenic mTORopathies (10). 
GnRH agonist treatment in H...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Continued surveillance for patients at higher risk of malignancy (e.g., Peutz-Jeghers, Cowden syndromes); annual follow-up postresection of HH to assess seizure c...

REFERENCES

1
Striano  S, Santulli  L, Ianniciello  M, et al. The gelastic seizures-hypothalamic hamartoma syndrome: facts, hypotheses, and perspectives. Epilepsy Behav. ...

ADDITIONAL READING

SEE ALSO

Hemangiomas and Other Vascular Lesions, Pediatric; Peutz-Jeghers Syndrome; Tuberous Sclerosis Complex; Uterine Myomas 

CODES

ICD10

Q85.9 Phakomatosis, unspecified 

SNOMED

  • 400006008 hamartoma (disorder)

  • 254644003 hamartoma of lung (disorder)

  • 277369003 Hamartoma of brain

  • 268284006 vascular hamartomas (disorder)

  • 253810002 Liver...

CLINICAL PEARLS

  • Hamartomas are disorganized growths of native cells in native tissues. Symptoms vary with tissue of origin; many are found incidentally.

  • The majority is benign; malignant transformation...

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