Hamartoma

Reviewed 06/2018

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 (7).

ISSUES FOR REFERRAL

Management...

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

Striano S, Santulli L, Ianniciello M, et al. The gelastic seizures-hypothalamic hamartoma syndrome: facts, hypotheses, and perspectives. Epilepsy Behav. 2012;24(1):7–13. [View Abstra...

ADDITIONAL READING

OMIM: http://www.ncbi.nlm.nih.gov/omim
Rosenfeld JV. The evolution of treatment for hypothalamic hamartoma: a personal odyssey. Neurosurg Focus. 2011;30(2):E1.

CODES

ICD10

Q85.9 Phakomatosis, unspecified

ICD9

759.6 Other hamartoses, not elsewhere classified

SNOMED

400006008 hamartoma (disorder)
254644003 hamartoma of lung (disorder)
277369003 Hamartoma of brain
26...

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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