CLOVES syndrome, learning the typical from the atypical. Case report
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Abstract
Background: CLOVES syndrome is a new sporadic mosaic segmental overgrowth syndrome, currently classified under the canopy of PROS (PIK3CA-related overgrowth spectrum) disorders. The overgrowth is segmental, irregular, asymmetric, and limited to the parts of the body affected by the mutation.
Case report: Adolescent with a clinical picture characterized by lipomatous overgrowth since childhood, throughout the body, predominantly in the thorax, for which he received several surgical interventions in addition to management with chemotherapy, currently under interdisciplinary management.
Conclusion: CLOVES syndrome is a rare entity that is difficult to manage. There is no specific cure. Until recently, patients with CLOVES syndrome were treated only palliatively through multidisciplinary and stepwise cytoreductive surgeries for lipomatous and skeletal overgrowths. However, advances in molecular diagnosis have shown a contribution, using new medications that directly target the PIK3CA gene. abnormal, showing promising results in this entity.
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References
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