[Cavernous sinus syndrome as the initial symptom of Burkitt's lymphoma: a case report and literature review]

Rev Neurol. 2019 Sep 16;69(6):249-254. doi: 10.33588/rn.6906.2019191.
[Article in Spanish]

Abstract

Introduction: The cavernous sinus is a structure in the base of the skull that houses several nerve and vascular structures. Its compromise leads to cavernous sinus syndrome, which is a combination of oculomotor disorders and others affecting the first two trigeminal branches, often accompanied by pain or proptosis. Infiltration due to Burkitt's lymphoma is a rare cause of this syndrome.

Case report: A 43-year-old male, carrier of human immunodeficiency virus, with good control of the disease, who developed a clinical picture consisting of progressive painful ophthalmoplegia in the presence of a laterocervical adenopathy. Complementary tests allowed a diagnosis of Burkitt's lymphoma with extranodal extension to the cavernous sinus. A review of the cases published in Medline was also carried out: a total of 15 cases were detected and their epidemiological characteristics, form of presentation, extracranial involvement at the time of diagnosis and clinical progression were described.

Conclusions: Burkitt's lymphoma is a high-grade lymphoproliferative syndrome. Its form associated with immunodeficiency is an important cause of morbidity and mortality in this subgroup of patients. In the cases analysed in the literature, the age of presentation varied and the form of onset was a progressive painful ophthalmoplegia or numb chin syndrome. Exclusive involvement of the cavernous sinus was infrequent, but in that case it entailed a poor prognosis. It is important to rule out a primary extracranial origin and not to confuse it with an idiopathic Tolosa-Hunt syndrome that would delay the beginning of antitumour treatment.

Title: Sindrome del seno cavernoso como inicio de un linfoma de Burkitt: a proposito de un caso y revision de la bibliografia.

Introduccion. El seno cavernoso es una estructura de la base craneal que alberga varias estructuras nerviosas y vasculares. De su afectacion se deriva el sindrome del seno cavernoso, una combinacion de alteraciones oculomotoras y de las dos primeras ramas del trigemino, frecuentemente acompañadas de dolor o proptosis. La infiltracion por un linfoma de Burkitt es una causa infrecuente de este sindrome. Caso clinico. Varon de 43 años, portador del virus de la inmunodeficiencia humana, con buen control de la enfermedad, que desarrolla un cuadro consistente en oftalmoplejia dolorosa progresiva en presencia de adenopatia laterocervical. Las pruebas complementarias permiten el diagnostico de linfoma de Burkitt con extension extranodal al seno cavernoso. Tambien se realiza una revision de los casos publicados en Medline: se recogen un total de 15 casos y se describen sus caracteristicas epidemiologicas, forma de presentacion, afectacion extracraneal en el momento del diagnostico y evolucion clinica. Conclusiones. El linfoma de Burkitt es un sindrome linfoproliferativo de alto grado. Su forma asociada a inmunodeficiencia es una importante causa de morbimortalidad en este subgrupo de pacientes. En los casos analizados en la bibliografia, la edad de presentacion fue variable y la forma de inicio fue una oftalmoplejia dolorosa progresiva o un sindrome de numb chin. Fue infrecuente una afectacion exclusiva del seno cavernoso, pero en ese caso conllevaba un mal pronostico. Es importante descartar un origen primario extracraneal y no confundirlo con un sindrome de Tolosa-Hunt idiopatico que retrasaria el inicio del tratamiento antitumoral.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Burkitt Lymphoma / complications*
  • Burkitt Lymphoma / diagnosis
  • Cavernous Sinus*
  • Humans
  • Male
  • Tolosa-Hunt Syndrome / diagnosis
  • Tolosa-Hunt Syndrome / etiology*
  • Trigeminal Nerve Diseases / diagnosis
  • Trigeminal Nerve Diseases / etiology*

Supplementary concepts

  • Ophthalmoplegia, painful