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Updated: Aug 24, 2026
Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
Published on: September 13, 2019
Primary diffuse leptomeningeal sarcoma with rhabdomyoblastic differentiation. A case report and immunohistochemical
Marcelo José de Oliveira1, Ana Maria de Oliveira Ramos, Alexandre de Oliveira Sales
1Department of Infectology, Federal University of Rio Grande do Norte (UFRN), Rua Governador José Varela, 2887, Cidade Jardim, Natal, RN, CEP 59078-300, Brazil. marcelojoliveira@uol.com
Abstract:
Primary meningeal neoplasias are rare, and their diffuse growth within the subarachnoid space may simulate inflammatory processes or metastatic neoplastic dissemination. We report here the case of a boy with an 18-day history of fever and progressive neurological deterioration. While in the hospital, the patient showed neurological deterioration and did not respond to antituberculosis treatment. His cerebrospinal fluid (CSF) cytology disclosed an elevated white blood cell count accompanied by a mildly elevated protein level and a slightly decreased glucose level. The child died, and pathology revealed that his meningeal process was a sarcoma. The immunophenotype of the neoplastic cells showed expression of a sarcomeric actin marker, characterizing rhabdomyoblastic differentiation of the cells that occupied the subarachnoid space and invaded superficially the encephalon. Rhabdomyoblastic differentiation in leptomeningeal diffuse primary sarcoma (LDPS) is an aspect rarely observed in this malignant meningeal neoplasia, with few reported cases. The present case is the first reported in the Portuguese literature and the fifth reported in the English literature.
Insights
This case report details a rare rhabdomyoblastic primary sarcoma of the leptomeninges (LDPS) in a child. The rare tumor presented with fever and neurological decline, highlighting the need for accurate diagnosis of meningeal sarcomas.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
Background:
- Primary meningeal neoplasias are rare and can mimic inflammatory or metastatic conditions.
- Diffuse leptomeningeal disease presents diagnostic challenges.
Observation:
- A pediatric case with an 18-day history of fever and progressive neurological deterioration is presented.
- Cerebrospinal fluid (CSF) analysis revealed elevated white blood cells, protein, and decreased glucose.
- The patient did not respond to antituberculosis treatment.
Findings:
- Post-mortem pathology confirmed a diffuse primary sarcoma of the leptomeninges (LDPS).
- Neoplastic cells exhibited rhabdomyoblastic differentiation, confirmed by sarcomeric actin marker expression.
- The tumor occupied the subarachnoid space and superficially invaded the brain.
Implications:
- Rhabdomyoblastic differentiation is a rare feature in LDPS, with few cases reported globally.
- This case is the first reported in Portuguese and fifth in English literature.
- Accurate diagnosis of rare meningeal sarcomas is crucial for understanding and managing these aggressive neoplasms.
