
Pulmonary choriostoma in a case of tuberous sclerosis complex
Author(s) -
Sonam Spalgais,
Dipti Gothi,
Amita Verma
Publication year - 2015
Publication title -
journal of postgraduate medicine/journal of postgraduate medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.405
H-Index - 52
eISSN - 0972-2823
pISSN - 0022-3859
DOI - 10.4103/0022-3859.159426
Subject(s) - medicine , tuberous sclerosis , dermatology , medline , pediatrics , pathology , law , political science
A 52 years old lady was diagnosed to have Tuberous Sclerosis Complex (TSC) on the basis of 2 major and one minor criterion. She had family history of similar complaints in her sister and two sons. There was involvement of kidney in the form of angiomyolipoma, skin in the form of facial angiofibroma and teeth with a dental pit. She had an unusual lung involvement in the form of multiple small choristomas. Choristoma was diagnosed on transbronchial lung biopsy and was present in the form of disorganised striated muscles. The reported pulmonary manifestations of TCS i.e. lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH) are types of hamartomas. Hamartomas and choristomas are both types of disorganized tissue. 'Choristoma'of lung in TSC however is not reported. Clinopathological correlation of pulmonary hamartoma and choristoma, and treatment in TSC has been discussed.