Related Experiment Videos
[Intracranial fibrous xanthoma (xanthofibroma) in an infant: a case report]
Y Ohmori1, S Kubo, K Yasukouchi
1Department of Neurosurgery, Kyoto 2nd Red Cross Hospital, Japan.
Insights
A rare case of intracranial fibrous xanthoma (xanthofibroma) in an infant is presented. This infant fibrous xanthoma coexisted with a similar chest wall tumor, highlighting its extreme rarity.
Area of Science:
- Neuropathology
- Pediatric Oncology
- Dermatopathology
Background:
- Intracranial fibrous xanthoma (xanthofibroma) is exceptionally rare in infants under one year of age.
- This report details a unique case in an 8-month-old boy with a history of seizures and a prior diagnosis of cutaneous fibrous xanthoma.
Observation:
- A well-defined, high-density lesion in the left temporal lobe was identified via CT scan, showing significant enhancement with contrast media.
- Surgical exploration revealed a well-demarcated tumor, not attached to the dura mater, allowing for complete resection.
Findings:
- Histopathological examination showed a tumor composed of fibroblastic and foamy phagocytic cells in a storiform pattern, with multinucleated giant cells.
- Immunohistochemistry results indicated tumor cells were negative for GFAP and EMA, but positive for Vimentin and S-100 protein.
- The findings support the diagnosis of coexistent intracranial and subcutaneous fibrous xanthoma in an infant.
Implications:
- This case underscores the possibility of synchronous fibrous xanthoma in both intracranial and cutaneous locations in infants.
- The successful surgical removal resulted in a favorable outcome with no recurrence or neurological deficits at 6 months post-operation.
- Further investigation into the characteristics and differential diagnosis of infantile intracranial fibrous xanthoma is warranted.
Abstract:
A case of intracranial fibrous xanthoma (xanthofibroma) is reported. Intracranial fibrous xanthoma in infancy under the age of 1 year is extremely rare. This patient was a 8-month-old boy with a history of convulsive seizure. He had a previously known chest wall tumor which was diagnosed as fibrous xanthoma of the skin. Plain CT scan revealed a well defined high density area in the left temporal lobe. The area was well enhanced with contrast media. At operation, it was found that the tumor did not attach to dura mater and was almost well demarcated. Total removal of the tumor was performed. The patient has been doing well for these 6 months following craniotomy, with no sings of recurrence and no neurological deficits. Histologically, the tumor was composed of fibroblastic cells and foamy phagocytic cells in storiform pattern. Some multinucleated giant cells were found. Immunohistochemistry technique revealed that the tumor cells were negative for GFAP, positive for Vimentin, positive for S-100 protein and negative for EMA. Our studies support the diagnosis of intracranial fibrous xanthoma coexistent with the same tumor found in the subcutaneous space of the chest wall of a boy under 1 year of age. We regard it as a rare incidence. Differential diagnosis and the characteristics of fibrous xanthoma were discussed.