![]() Describimos el caso de un varón joven diagnosticado de síndrome de Noonan en el que se resecó un condroma gigante de la convexidad. Se han relacionado con la encondromatosis múltiple de Ollier y con el síndrome de Maffuci. Excepcionalmente, puede crecer a partir de restos cartilaginosos en la duramadre de la convexidad o en la hoz. Los condromas intracraneales son tumores extremadamente raros que suelen surgir de la sincondrosis de la base craneal. Association of a dural convexity chondroma with Noonan's syndrome is unique as far as the literature is concerned. No predisposition of Noonan's syndrome for tumoural development is reported in the literature. Noonan's syndrome (also known as pseudo-Turner syndrome) is a complex familial genetic disorder with a phenotype that resembles that of Turner's syndrome but exhibits no chromosomal defect. ![]() Radiation therapy is currently not recommended for residual tumours or inoperable patients due to risk of malignization. Complete tumour resection including its dural attachment is the treatment of choice. Angiogram is clue to differentiate from meningiomas since chondromas are completely avascular. MRI studies show a well-circumscribed lesion without surrounding tissue oedema, that exhibit heterogeneous signal with intermediate to low intensity on T1-weighted images and mixed intensity on T2-weighted images with minimum enhancement. CT scan imaging shows a mass of variable density due to different degrees of calcification with minimum to moderate contrast enhancement. Chondromas present clinical features similar to meningiomas. Several hystopathogenetic theories have been proposed: metaplasia of meningeal fibroblasts and perivascular meningeal tissue, traumatic or inflammatory cartilaginous activation of fibroblasts and growth of aberrant embryonal cartilaginous rests in the dura mater. Only twenty-five cases of intracranial dural convexity chondromas are reported in the literature. Chondromas comprise less than 0.3% of intracranial tumours. Subsequent brain MRI studies have shown no evidence of recurrence after 33 months of follow up.ĭiscussión. The pathological study revealed a mature chondroma. Postoperative seizures required additional anticonvulsant therapy. The tumour was firm, white-greyish, avascular and could be finely dissected away from the cortex. The patient was initiated on valproic acid and underwent craniotomy and complete excision of the tumour. Brain Magnetic Resonance image showed a large well-circumscribed intracranial mass in the dural convexity of the left frontal-parietal lobes, with heterogeneous contrast enhancement and no peritumoural oedema. Physical and neurological examinations were unremarkable. He harboured mild psychomotor retardation. The patient was previously diagnosed of Noonan's syndrome on the basis of his special phenotype (Turner-like), low stature, cardiac malformation, retarded sexual and bone development and normal karyotype. An 18-year-old male presented with a single generalized seizure. We describe the case of a young male diagnosed of Noonan's syndrome that underwent resection of a large intracranial chondroma arising from the dural convexity.To our best knowledge this is the first report of suchassociation.Ĭase report. They may be part of Ollier's multiple enchondromatosis or Maffuci's syndrome. Exceptionally, they may grow from cartilage rests within the dura mater of the convexity or the falx. Intracranial chondromas are extremely rare intracranial tumours that usually arise from the skull base synchondrosis. Servicios de Neurocirugía y de *Anatomía Patológica. Case report and review of the literatureĬondroma gigante de la convexidad en un paciente con síndrome de Noonan. Large chondroma of the dural convexity in a patient with Noonan's syndrome.
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