Clival chordoma: A clinical case report

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Monsalve B, Millan Y, Gomez M and Pereira A

Children´s Hospital Jose Manuel Hospital of Los Rios, Venezuela

: J Forensic Toxicol Pharmacol

Abstract

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The clival chordoma are slow-growing embryonic tumors, originate from remains of the primitive notochord, in the center of the base of the skull with different patterns of extension in all directions, which limits the extent of tumor removal. An estimated 75% of global survival is 5 years after being treated surgically. The incidence is 0.08- 0.5 cases per year. Six variants have been described: classic chordoma, chondroid, undifferentiated, sarcomatoid, intradural and extrarenal. The standard treatment is surgery; adjuvant radiotherapy is used in cases of incomplete resection; conventional chemotherapy has not proven to be effective. Studies have confirmed the efficacy of targeted therapies such as tyrosine kinase inhibitors (anti-PDG, anti-VEGF and mTOR inhibitors). Clinical case: 3-year-old girl who presented with cervical hyperextension and functional limitation. Subsequently, weight loss and swallowing disorder; MRI showed retro-pharyngeal tumor with extension to the cervical spine and foramen magnum with atlo-axoid erosion. She is referred to our Center, and evaluated by Neurosurgery and Oncology. Incisional biopsy reports classic chordoma notochordal tumor; the girl receives Imatinib with progression disease given by increase in size of the lesion, medullary bulb compression and retroclival extension, radiant treatment is decided which is rejected, patient dies 4 months later. The Imatinib indication based on clinical evidence, the tumor could not be surgically resected due to extension and compromise of the medullary bulb; the objective was to reduce tumor volume to subsequently apply radiotherapy, considering the age group, extensive field of radiation and sequelae after treatment

Biography

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E-mail: belkisandreinamonsalve@gmail.com