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Hydrocephalus and Papilledema in Spinal Cord Tumors: A Report of Two Cases.

ABSTRACT: Background The association between spinal cord tumors and hydrocephalus is a rarely reported phenomenon. Diagnosis in this group of patients is difficult as they present with findings of an intracranial pathology and the symptoms of a spinal lesion may be absent. Case Report We report two cases of spinal cord tumors presenting with visual disturbance and findings of increased intracranial pressure. Discussions Mechanisms describing the relationship between spinal cord tumors and increased intracranial pressure have been explained. Most of the literature reported marked regression of these manifestations after tumor excision. Conclusions Spinal cord tumors associated with hydrocephalus and papilledema are rare conditions. The diagnosis of these conditions may be difficult or confusing because the symptoms referable to the spinal lesion may be minimal. Meticulous history taking, examination, and investigations are mandatory to diagnose this entity.

SUBMITTER: Marzban AN

PROVIDER: S-EPMC5553482 | biostudies-other | 2016 Apr

REPOSITORIES: biostudies-other

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Project description:BackgroundDiffuse midline glioma with H3K27-altered (DMG-H3K27a) is a novel tumor entity of the pediatric-type diffuse high-grade tumor in the latest WHO CNS 5. It mostly affects children and is only rarely found in adults. The tumor has a high level of aggressiveness, with a rapid progression and bad prognosis. In adults, the spinal cord is the most common site of DMG-H3K27a. Rare adult cases of primary DMG-H3K27a in the spinal cord were reported in this study, together with clinico-histopathologico-radiographic data.MethodsFrom January 2016 to December 2020, we conducted a retrospective study of five adults with primary DMG-H3K27a in the spinal cord, analyzing their clinical, pathohistological, and radiographic datasets from the first diagnosis to follow-up.ResultsAll five patients were diagnosed for the first time and were given full treatment. In three of the five patients, post-operative follow-up revealed tumor recurrence. The longest survival of the five patients was 45 months at the time of report submission, while the longest progression-free survival (PFS) following surgery was 20 months. Immunohistochemical studies showed the tumors featured aggressive behavior (grade 4) and were positive for the H3K27M mutation. The radiographic appearances were varied, but they were all initially mistaken as benign. DMG-H3K27a in the spinal cord was characterized by isointense/hyperintense on T1WI and isointense/hyperintense on T2WI, as well as cystic necrosis and peripheral spinal cord edema, as well as central canal enlargement and other types of enhancement.ConclusionThis is the first case report focusing on adult DMG-H3K27a of the pediatric-type diffuses high-grade gliomas in the spinal cord. In our cases, we discovered the following: 1) adults had a better prognosis with a longer PFS compared with prior pediatric reports; 2) despite aggressive behavior under the microscope, radiographic appearances of the tumors were less aggressive; and 3) adjuvant treatment, including TCM, may have played a role in the prognosis.

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Hydrocephalus and Papilledema in Spinal Cord Tumors: A Report of Two Cases.

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