 Good morning everyone. I am Dr. M.D. Kursilalum, second year post-graduate tenure of NRS Medical College Kolkata, going to present a rare case report of tuberous cirrhosis. This case being done under the guidance of Dr. Ramchandra Badra, associate professor of NRS Medical College and Dr. Supanna Saab, senior resident of Department of Radiology of NRS Medical College. Now let's see how the case is presented to our department. Tuberous cirrhosis is a multi-system neurocuteness in genetic condition, exhibiting a wide range of manifestations. It's also known as epilovia or pingle bone-bell fecomatosis and was initially described in 19th century by virtual wound reclamation who identified hematomas in brain and heart during the nectopsy of patient with seizure and mental retardation. However, the correlation with clinical manifestation and description of syndrome was made by bodily in 20th century. Inheritance is autosomal dominant with new mutation accounting of two-third of cases. A great diversity of mutations are found in TSC1 and TSC2 genes including contiguous gene deletion of TSC2 PKD on causing tuberous cirrhosis with severe polycystic kidney disease. The clinical manifestation of the tuberous cirrhosis is associated with variable expression and complete penetration. Both sexes are equally affected. Cutaneous manifestations include facial angiofibroma which is known as adenoma civicius, hypopigmented macules, known as acylic macules, and saccharine patches and periangular and subangular fibromas. These are known as quinine tumor and fibrocephalic plaque. Neurological manifestation includes include epilepsy, mental retardation, and behavioral problems. Neuroimaging studies can show cortical and sub-cortical tubers, subependermal nodules in the all of the literal venticles, and giant cell subependermal astrocytoma. Renal manifestation include angiomyelarcoma and rarely renal cell carcinoma. Pulmonary manifestation include linfangioleomyomatosis, cardiac manifestation include raptomyoma. Other dental enamel pits, internal fibroma, retinal acromic fats, and non-enamel hematomers. In our case, a 40 years old male came to the department of the medic medicine of NRS medical college with complaint of pain abdomen for three months and skin lesion and depression. Here you can see the patient presented with adenoma cibacium. This is the adenoma cibacium present in the face of the patient and here we can see in the axilla there is axillary fetus. Here you can see very unwell fibromas in both upper limb and lower limbs. Here you can see this. Here in the forehead we can see there is a huge plugs known as fibroscephalic plugs and sagreen patches mainly present in the dorsum of the back, dorsum in the thorax, mainly lumbar regions. And aslip macules, here you can see aslip macules in the periaxial region. Insistency of the whole abdomen source, there is a bilateral enlarged kidneys with multiple fat density areas. The hospital unit of this area is minus 80 hospital units intermixed with softened density which will have hospital unit of 38 with no areas of calcification, suggestive of bilateral angiomyelipoma. You might have the brain of this patient's source, there is subependymal nodules in the lateral all of the lateral metrical which is hyperintense on T1 and hyperintense in T2 and axial T2 flare source hyperintensity areas in the left posterior left occipital parietal region. Here there is a hyperintensity area in the left frontal parietal regions. These are mainly cortical stevers. CT scan of the brain shows there is a hyper dense nodule noted in the lateral all of the left lateral ventricle, suggestive of subependymal nodule. Tuberous complex is a multi-system neurocutaneous genetic condition with autosomal dominance inheritance characterized by hematomas that affect multiple organs including skin, central nervous system, heart, lung and kidney. It occurs due to deletion, rearrangement and inactivation, inactivating mutation of tumor suppressants in TSC1 and TSC2 that lead to abnormal protein, haematine and tuberonin codified in the loci 9p34 and 16p13 respectively. The complex haematine and tuberonin is an important inhibitor of tumor growth. This protein suppresses the activity of the inter pathway responsible for cellular proliferation and inhibition of cellular apoptosis. In TSC patient, in tuberous closest patient, changes in this protein lead to permanent activation of the inter pathway and therefore to the formation of the haematomas in multiple organs. Familial cases of the conditions are due to germline mutation and despite being able to transmit hereditary, 70% of the tuberous closest patient are the result of somatic mutation, configuring sporadic cases. Most patients affected by the tuberous closest seek medical attention due to the seizure of or skin lesion. Cutaneous manifestations represent the most common finding. Neurological and renal complications are the main cause of morbidity and mortality associated to the condition. The genetic basis of the disease is now well understood and genetic testing is available for the majority of the families. Tuberculosis treatment is consist above all the management of the symptom caused by haematomas and prophylactic measure to avoid loss of function of the affected organs. Because it is a systemic disease, it is a multidisciplinary follow-up is mandatory within the need of assessment and follow-up in conjunction with terms of genetics, neurology, ophthalmology, nebulogy, nephrology, and odontology. Thank you.