Temporal primary cerebral Ewing sarcoma extended to skull

Sarcoma de Ewing temporal cerebral primário com extensão para o crânio

LETTERS

Temporal primary cerebral Ewing sarcoma extended to skull

Sarcoma de Ewing temporal cerebral primário com extensão para o crânio

Citlaltepetl Salinas-Lara^II; Claudia Martínez^{I, II} Martha Tena-Suck^II; Miguel Ángel Collado-Ortiz^III; Ulises Rodríguez^IV; Arturo Garcìa Marquez^V; Diana Gómez^{I, II}; Fernando Becerra^{I, II}

^IUnidad Académica de Medicina, Universidad Autónoma de Nayarit, México

^IIDepartamento de Neuropatología, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México City, DF., México

^IIIServicio de Neurología, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México City, DF., México

^IVServicio de Neuroimagen, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México City, DF., México

^VService of Neuroimagen, National Institute of Neurology and Neurosurgery, México City, DF., México

^{Correspondence}

Cerebral Ewing´s sarcoma is a very rare disease, few cases have been reported on literature^1,2. Any bone can be affected, some rare intracranial primary cases had been reported³, The peak incidence is between 10 and 20 years old. Males are more frequently affected than females, and usually presents as a solitary bony lesion^1-3. Clinically the most important and earliest symptom is pain, which is initially intermittent but becomes very intense^1-3.

CASE

A 30 year old female with a history of tonic-clonic generalized seizures and aphasia which started at 8 years old. She presented seizures characterized by loss of consciousness and tonic-clonic movements. Neurological examination showed absent corneal and nasal reflexes, right facial, central paresia, right hemiplegia, and abnormal Babinski. The MRI showed a lesion enhancement tumor with low signal heterogeneous gadolinium enhancement, whereas signal intensity on T2-weighted images varies after contrast enhancement (Fig 1A). The spectroscopy showed an increase of coline and important diminution of N-acetil-aspartate (Fig 1B).

The patient underwent left frontal craniotomy and the tumor was excised totally. Following surgery, she underwent whole brain, spine and local radiation therapy (30 Gy in total) and also received chemotherapy. She died 11 days after the surgery.

Partial autopsy was performed, grossly; the tumor was an irregular glistening gray mass of 4×4 cm, which extended all along the temporal and parietal bone on the right side of the skull (Fig 1C). Tumor surfaced poorly demarcated firm areas, with necrosis, hemorrhage and dissemination along the skull base (Fig 1D).

Histologycally tumor revealed bland spindle-shaped cells with indistinct pale eosinophilic cytoplasm and small hyperchromatic oval nuclei, ring cell likes were also observed (Fig 2A and 2B), ploemorphism and mitotic features (Fig 2C and 2D). The tumor cells were embedded in a variably fibrous or myxoid stroma that tended to alternate in different areas of the tumor. Immunohistochemical analysis of the tumor cells revealed diffuse expression of vimentin, CD99 (Fig 2E) and CD117 (Fig 2F). Ewing sarcoma was diagnosed. The tumor was resistant to different kinds of therapy.

DISCUSSION

Primitive neuroectodermal tumors are in the Ewing's sarcoma family of tumors and are composed of small round cells, belong to a family of tumors that share clinic pathologic and molecular genetic features, including the characteristic chromosomal translocation that results in the fusion of the EWS gene on 22q12 to either the FLI1 gene on 11q24 or other^2-4. One basic distinction is between primitive neuroectodermal tumors of the central nervous system (cPNETs) and primitive neuroectodermal tumors of the peripheral nervous system (pPNETs), which are clinicopathologically and genetically distinct^3,4. Among the cPNETs including medulloblastoma, pineoblastoma, cerebral neuroblastoma, ependymoblastoma, medulloepithelioma, primary rhabdomyosarcoma, and atypical teratoid/rhabdoid tumor, whereas the pPNETs comprise the more differentiated end of a spectrum of neoplasms that include skeletal and extraskeletal Ewing's sarcoma. In most instances these entities may be differentiated by a panel of antibodies that should include those with low and high molecular weight cytokeratins, epithelial membrane antigen, type IV collagen, ENE, sinatophysin, CD99, CD56, and S-100 protein³.

Appropriate treatment is based on a correct diagnosis, the surgical pathologist must be familiar both with basic characteristics of each of the numerous entities as well as the spectrum of morphologic features that each may display^3,4.

Support:

Armstrong Fundation for all support that they gave to the student Claudia Martinez during this investigation.

Received 12 May 2010

Received in final form 3 May 2011

Accepted 10 May 2011

• 1. Paulussen M, Ahrens S, Braun-Munzinger G, et al. EICESS 92 (European Intergroup Cooperative Ewing's Sarcoma Study) preliminary results. Klin Padiatr 1999; 211:276-283.
• 2. Mazur MA, Gururangan S, Bridge JA, et al. Intracranial Ewing sarcoma. Pediatr Blood Cancer 2005; 45:850-856.
• 3. Carvajal R, Meyers P. Ewing's sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am 2005;19:501-525.
• 4. Verrill MW, Judson IR, Harmer CL, et al. Ewing's sarcoma and primitive neuroectodermal tumor in adults: are they different from Ewing's sarcoma and primitive neuroectodermal tumor in children? J Clin Oncol 1997;15:2611-2621.

Publication Dates

• Publication in this collection
  27 Oct 2011
• Date of issue
  Oct 2011