In children with medulloblastoma, spinal metastases must be screened for even if there is no clinical evidence of myelon compression. How self-evident this must be is shown, among other things, by a Case history described by a team led by Dr. John Ross Crawford (Pediatrics, University of California Irvine) in the «British Medical Journal»
The patient and his history
As the authors report, the child (there is no exact age, Editor’s note) with an ataxia that had existed for several weeks.The child was known to have delayed development (speech and fine motor skills).
The findings
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The neurological examination revealed only slight difficulties in tandem walking.
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Magnetic resonance imaging: Tumor in the posterior cranial fossa that was accompanied by obstructive hydrocephalus.
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Spinal MRI showed an obvious extramedullary and intradural tumor at levels C1 and C2 with similar signaling features to the Brain tumor; in addition, an extended central channel (C1 — C3) was detected.
The neurological examination revealed only slight difficulties in tandem walking.
Magnetic resonance imaging: Tumor in the posterior cranial fossa that was accompanied by obstructive hydrocephalus.
Spinal MRI showed an obvious extramedullary and intradural tumor at levels C1 and C2 with similar signaling features to the Brain tumor; in addition, an extended central channel (C1 — C3) was detected.
Diagnosis and therapy
The operation revealed intramedullary tumor tissue, which was removed in the same way as the brain tumor.mHistological examination of the cerebral and spinal tumor tissue confirmed the suspected diagnosis of metastatic medulloblastoma (WHO grade IV). Postoperatively, the child received radiation and chemotherapy.For 5 years, according to the authors, it has been in remission.
Discussion
About 20–40 percent of all primary brain tumors in children are medulloblastomas. In adults, however, medulloblastoma accounts for less than 1% of all primary brain tumors. Medulloblastomas are usually located in the posterior cranial fossa. Boys are affected 2 to 4 times more often than girls. About 40% of medulloblastomas occur within the first 5 years of life and about 75% within the first 10 years. The clinical presentation is often non-specific and includes headache, vomiting and gait disturbances.
Ten to 50 percent of aggressive tumors have already spread at the time of diagnosis. Medulloblastomas are prone to leptomningal seeding and drop metastasis by the cerebrospinal fluid. The most common are such drip metastases. Intramedullary metastasis of medulloblastoma is rare. According to the authors, only six cases of intramedullary metastasis of medulloblastoma in children have been reported. Four children would have had clinical symptoms of myelon compression, for example, a Urinary incontinence or progressive muscle weakness.Intramedullary metastasis was usually detected after the intracranial tumor or at its initial diagnosis, when there were signs of spinal cord compression.
What is unusual about their patient’s medical history is the intramedullary location of the medulloblastoma metastasis without clinical signs of myelon compression, Crawford and his colleagues report.
The standard therapy for medulloblastoma consists of surgery in combination with radiation and chemotherapy, which are determined individually.
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Source — https://www.univadis.de/viewarticle/ein-kind-mit-ataxie-und-hydrocephalus-2023a10009zd