 Good evening everyone, I am Dr. Sifaliya Sati from Muzaffarnagar Medical College is going to present paper on the topic Radiological Manifestation of Tuberous Colossus. Introduction The Tuberous Colossus complex is a neurocutaneous syndrome characterized by the formation of non-malignant hematomas and neoplastic lesions in the brain, heart, skin, kidney, lungs and other organs. It has a genetically-determined multi-system disorder and the organs most commonly involved are the brain, skin, kidney, lung, retina and heart. It is the second most common inherited tumor syndrome after NF1. It is also called as bone-means disease. The incidence is 1 in 10,000 to 50,000. It is an autosomal dominant disease. Itology of the Tuberous Colossus, 50% of the cases are inherited and follow an autosomal dominant pattern while the other 50% are de novo in mutation. Two separate genes are mutated or dilated that is TSC1 and TSC2, the TSC1 is located on chromosome 9Q34 and encodes protein hematin and TSC2 is localized on chromosome 16P13.3 and encodes tuberous protein. Mutations in either gene are identified in 75% to 85% of the cases with TSC. Now we will see the diagnostic criteria of the Tuberous Colossus complex. The major features are hypomelanotic macules that is three or more macules, chagrin patch, facial angiofibroma, adenosvesium or angol or periungal fibromas, multiple retinal nodular hamatomas, cortical tubers, subependymal nodule, subependymal Jancer estrusitoma, cardiac revdomioma, single or multiple lymph angiomyomatosis and reline angiomyolipoma. So here are some of the cutaneous manifestations and the in the radiological manifestations in the cutaneous. These are the hypomelanotic macules. These are the chagrin patch on the lumbar region. These are the facial angiofibroma. These are the coenant tumors in the periphery. This is here it is only the fingers. This is a MRI brain, flare image. So in the T2 in the flare images we see the cortical and the subcortical white matter region, some hyperintensities along with the nodularities in the ventricular margin. This is a, this suggests of subependymal nodule. Next is the near the foramen of Monroe and there is there is a lesion, hypodense lesion and there is associated ventricular megalis suggestive of subependymal Jancer estrusitoma. Next are the minor features. In the minor features these are minor features are multiple pits in dental enamel, gingival fibromas, hematometrist rectal polyps, bone cysts, cerebral white matter radial migratio lines, retinal acromic patch, multiple renal cysts, non-renal hematomas and conflicted skin lesions. These are the conflicted skin lesions shown. Now on the cases I have seen are in my department. First case is of a 24 years male presented to medicine department with a complaint of scissor, scissor since 2 to 3 years on further examination patient had facial angiofibromas on the face, nose, bilateral cheek and back of the neck. This was the patient on the face that is on the nose and in the cheek area the patient had facial angiofibroma and on the nape of the neck also. Then the MRI of the patient was performed and this is the T2 weighted and this is the flare sequence. On the T2 and the flare sequence they are multiple indefined T2 flare hyper intense lesions of variable sizes and it is noted in bilateral cerebral hemisphere. But the temporal lobes are spared, suggestive of particle tubers. In the same patient in the ventricular margin also there was nodularity was seen that is multiple tiny nodules along the bilateral ventricles and posterior on the right lateral ventricle likely suggestive of subapendymal nodules. The second case witnessed was of a 20 years male came with a complaint of pain in the lumbar region. On examination the patient held pepules on her nails. These are the subpariungal fibromas that is coin and tumors in his nails. Then this the patient came for the complaint of lumbar pain in lumbar region. So the CT abdomen was performed. This is the plane scan. In the plane scan we can see the nodular soft tissue lesions in bilateral kidneys that this is the left, this is the right and these are multiple. On further the contrast study was performed and in the arterial phase we see a heterodense lesion in the right kidney and similar lesions were seen in the left kidney that is volatile kidney was involved. The same lesion was seen in the venous phase that is multiple modulately defined heterogeneously enhancing nodular soft tissue lesions with fat tissue adenation areas within is seen some of the lesions were also exhibited like this. So now the next case is case 3 of a 14 year female came with a complaint of scalp swelling. In the past history the patient had one episode of Caesar in childhood and the family history that is she had two siblings but they were normal. The patient came with a complaint of the scalp swelling in the occipital region. The patient also had facial angiofibromas and there was a ash leaf macule in the calf. The NCCT head was performed because of further swelling. So in the NCCT head we can see an oven shaped heterodense lesion in the scalp region in occipital region. Further the MRI was performed in the MRI. We can see a T2 irregular heterogeneous T2 hypo intense lesion in the scalp region in the occipital region. This is a rare cutaneous manifestation of tubular sclerosis and this is likely to be scalp fibroma. Next the patient further study, in the further study of the NCCT head these are the multiple calcified nodules in the periventricular region and it is sub-apendimental distribution suggestive of sub-apendiment nodules or radiological manifestation of tubular sclerosis. These are the similar lesions calcified nodules. Further the MRI was performed and in the MRI T1, T2 and flare images we can see the irregular margins of the ventricles. So these are the multiple sub-apendimental variable size lesions that are appearing iso intense on T1 and hypo intense on T2 and flare images. The similar lesion are shown here and these are showing blooming artifact on the, this is the SWI sequence and it is showing blooming artifact which is suggestive of calcification. So these nodules were sub-apendimental nodules or hamatomas. Next few of the sub-apendimental nodules were measuring more than 1 centimeter. So these are sub-apendimental hamatomas. So further study, further follow up can be performed and to see if these hamatomas are progressing towards the sub-apendimental giant cell astrocytoma. In the same patient they was found that in the bilateral regions there were T2 flare, hyper intense radial bands extending from the posterior aspect of the lateral ventricle up to the cortex, likely the white matter heterotopia which is a feature of tuberous cirrhosis. So in my study we found that the sub-apendimental nodules, sub-apendimental giant cell astrocytoma can also be seen. So in the sub-apendimental nodules progress, one of my patients had renal angiomyelipoma. So in the sub-apendimental nodules the tubers represent cortical glioneuromal hamatomas and consist of focal distribution in cellular organization and morphology which is extending to the sub-particle white matter. Calcification was seen in the tubers. The tubers are triangular shaped and with apex oriented inward towards the ventricle. The sub-apendimental giant cell astrocytoma arise from the sub-apendimental nodules present in the ventricle of all in the patient with tuberous cirrhosis which was multilobulated and the epidermal lining over the sub-apendimental giant cell astrocytoma was intact, making the CSA seeding very highly unlikely. The renal angiomyelipoma is the most common fat containing lesions in the kidney. It involved the cortex and there was microscopic fat. In tubers cirrhosis the lesions are larger than isolated AML and the characteristic is the multiple bilateral involvement in the tubers cirrhosis. Thank you.