Upper cervical spine chordoma of C2–C3

Abstract

Chordoma is a rare low-grade malignant neoplasm derived from the remnants of the embryonic notochord. This locally invasive neoplasm is subject to recurrence after treatment. The median survival time is estimated to be 6.3 years. Various treatment approaches have been attempted, including radical excision, radiotherapy and chemotherapy. Treatment outcome is significantly influenced by the size and site of the chordoma. Recently, Imatinib, a molecular-targeted agent, has been shown to have antitumor activity in chordoma. Proton radiotherapy, stereotactic radiotherapy and intensity-modulated radiotherapy have also been used. Surgical treatment is still the primary choice for chordoma. It has become more aggressive in recent years, evolving from intralesional or partial excision to en bloc resection. However, upper cervical localizations make such en bloc resection in most cases not possible. We present and discuss the therapeutic challenges of a young female with large retropharyngeal chordoma who presented to our institution after conventional photon beam radiotherapy. This C2/3 tumor was classified IB according to the Enneking classification. It distributed to layers A-D and sectors 1-6 according to the Weinstein Boriani Biagini Classification. The left vertebral artery (VA) was encapsulated and displaced. One stage intralesional extracapsular tumor excision and reconstruction was achieved by combined bilateral high anterior cervical approaches and posterior approach. No recurrence or metastasis was observed 3 years after the operation. She returned to her previous occupation as office worker.

Paravertebral High Cervical Chordoma

“…Although complete surgical resection is the treatment of choice for spinal chordomas 5,[25][26][27][28] , it may be obtained in less than half the cases. …”

“…They are commonly located on the midline and are associated with variable and usually marked destruction of one or more vertebral bodies [4][5][6] .…”

Cordoma en la articulación facetaria de C4: reporte de caso

“…Su aparición en la columna móvil representa el 15 %, con una distribución del 67 % en la columna lumbar; un 28 % en la columna cervical, y un 11 % en la columna torácica [3]. Dicha descripción se encuentra basada principalmente en el análisis de 262 pacientes de la Clínica Mayo [6], y coincide con registros de pequeñas series en diferentes regiones [3,4]. Sin embargo, el estudio realizado entre 1973 y 1995, de 400 pacientes en Estados Unidos por medio del Sistema de Vigilancia y Epidemiología, mostró una distribución casi igual entre la aparición en los diferentes segmentos.…”

“…Se ha visto que la recurrencia del tumor es dos veces mayor cuando la resección en bloque del tumor afecta la cápsula en aquellas intervenciones en que se logra la resección sin afectar la cápsula [6].…”

“…Idealmente, se debe hacer la escisión quirúrgica seguida de radioterapia con altas dosis, pero el lograr la resección en bloque completa con márgenes libres es suficiente. La tasa de supervivencia varía desde 6 a 29 años, con un porcentaje del 67,6 % a 5 años; del 39,9 % a 10 años, y 13,1 % a 20 años [6,14].…”