ABSTRACT
Mesenchymal chondrosarcoma is a highly malignant and extremely rare tumor of the orbit: only 18 cases have been reported to date. We report a case of spheno-orbital mesenchymal chondrosarcoma in a 36-year-old woman presented with a 4-month history of progressive left exophthalmia and temporal mass. A CT-scan of the orbit and MRI showed a spheno-orbital mass, with temporal fossa extension, fed by the internal maxillary artery visible on cerebral angiography. Surgery via a transcranial, left frontotemporozygomatic approach after selective embolization enabled subtotal removal. Definitive histologic examination revealed mesenchymal chondrosarcoma. Postoperatively, exophthalmia spectacularly regressed. We report our clinical findings and present a review of the literature.
Subject(s)
Bone Neoplasms/surgery , Chondrosarcoma/surgery , Skull/surgery , Adult , Bone Neoplasms/diagnostic imaging , Chondrosarcoma/diagnostic imaging , Chondrosarcoma/radiotherapy , Combined Modality Therapy , Exophthalmos/etiology , Female , Humans , Magnetic Resonance Imaging , Radiotherapy/methods , Skull/diagnostic imaging , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Liponeurocytoma (lipomatous medulloblastoma) is an uncommon clinicopathologic entity. We report a case of cerebellar liponeurocytoma in an adult and attempt to better characterize this uncommon lesion. A 43-year-old woman presented with symptoms and signs of increased intracranial pressure and cerebellar dysfunction. CT and MRI showed a heterogeneous well-circumscribed mass in the left cerebellar hemisphere with hydrocephalus. Complete surgical excision of the tumor was accomplished through a suboccipital craniotomy. Pathological examination with immunohistochemical study revealed a medulloblastoma with neuronal, astrocytic and lipomatous differentiation. Postoperative radiation therapy was given. After surgery, the patient was followed up for a 16 month-period; symptoms improved dramatically and no evidence of tumor recurrence was found. Careful analysis of the histopathological and immunohistochemical studies correctly identifies liponeurocytoma (lipomatous medulloblastoma). To our knowledge, only 16 cases have been reported. Although this lesion appears to have a better prognosis than the conventional form of adult medulloblastoma, complete surgical removal with postoperative radiation therapy is recommended.
