Primary diffuse leptomeningeal gliomatosis as a rare cause of pain in cervical spine

Abstract

BackgroundPrimary diffuse leptomeningeal gliomatosis (PDLG) is a very rare neuro-oncological disease, with only 90 cases of PDLG described in medical literature so far.Case presentationWe present a case report of a 56-years-old female patient, who was originally hospitalized due to cervical spine pain lasting several months. Despite complex diagnostics and treatment, the neurological state of the patient progressively deteriorated. Patient died 10 months after the first reported symptom. Postmortem pathological findings resulted in the diagnosis of PDLG.ConclusionsAffection of the cervical spine in early stages of PDLG is rare and has been described in only six patients so far. PDLG is a fatal neuro-oncological disease and it must be kept in mind in the differential diagnosis of persistent back pain syndromes.

Primary and secondary leptomeningeal gliomatosis in dogs

“…9,29 PLG is rare in human medicine, with fewer than 100 docu mented cases since its first description in 1923. 25,26,29 The cell of origin of PLG remains unknown, but neoplasms are thought to arise from heterotopic glial cell nests found in the subarach noid space. 26 The age of affected patients varies (median age = 35 years), with no apparent gender predilection.…”

“…25,26,29 The cell of origin of PLG remains unknown, but neoplasms are thought to arise from heterotopic glial cell nests found in the subarach noid space. 26 The age of affected patients varies (median age = 35 years), with no apparent gender predilection. 29 Clinical signs are insidious and almost invariably lead to death a few months after their onset.…”

“…9 A clinical diagnosis is difficult due to the often subtle meningeal lesions, and although PLG can be suspected by magnetic resonance imaging (MRI), surgical biopsy is often necessary for diagnostic confirmation. 9 Although most cases consist of extensive neoplastic infiltration along the surface of the brain and/or spinal cord (hence, the occasional use of the term "diffuse leptomeningeal gliomato sis" to refer to PLG), 13,26 solitary meningeal lesions are also reported. 10,17 Early PLG is characterized by the involvement of supratentorial locations, with a predilection for the skull base.…”

“…29 However, lesions can progress and involve the spinal cord. 4,26 SLG is reported in 1% to nearly 30% of human patients with an intraparenchymal glioma. 26,30 Leptomeningeal involvement occurs following the dissemination of neoplastic glial cells from the main tumor via cerebrospinal fluid (drop metasta sis).…”

“…4,26 SLG is reported in 1% to nearly 30% of human patients with an intraparenchymal glioma. 26,30 Leptomeningeal involvement occurs following the dissemination of neoplastic glial cells from the main tumor via cerebrospinal fluid (drop metasta sis). 1,19,30 The median age of affected patients is 45 years and leptomeningeal spread is most commonly associated with highgrade gliomas.…”