Chest manifestation of the scleros tuberos complex : case report and literature review

Tuberous scleros complex (STC) is a rare autosomal dominant genetic disease responsible for an anomaly of the proteins involved in the regulation of cell proliferation and differentiation. Thus a multi-system propagation of hamartomas, benign and sometimes malignant tumors is seen in several organs. The positive diagnosis and the definition of the extent of this pathology is based on the grouping of a set of clinical and/or radiological criteria such as the presence of cortical tubercles or subependymal nodules, cardiac rhabdomyoma, renal angiomyolipoma (AML), retinal astrocytoma and many other lesions. Pulmonary involvement consists of the presence of lymphangiomyomatous (LAM) and less frequently, Micronodular Multifocal Pneumocytic Hyperplasia (MMPH), which causes cystic and nodular disease respectively. Through a case of STC with multisystem localization and late discovery, we describe the different clinical and radiological aspects of this disease, focusing on the pulmonary involvement. She is a 54-year-old woman, epileptic since the age of 12, asymptomatic, who came to consult for disgraceful skin lesions: angiofibromas of the face, Koënen tumors and in whom an AML of the left kidney was discovered on the radiological plan., calcified subependymal nodules, vertebral and costal osteocondensations a retinal hamartoma of the right eye and particularly in the lungs a MMPH of the lung made up of multiple diffuse solid, fatty and ground glass nodules associated with a hamartoma and hamartochodoma and some lesions cystic.