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[A case of an intracranially located esthesioneuroepithelioma]
H Manabe1, T Moriyama, K Saitoh
1Department of Neurosurgery, Hirosaki University School of Medicine, Japan.
Insights
A rare esthesioneuroepithelioma in a young child was successfully removed and treated with adjuvant therapies. This pediatric brain tumor case highlights the importance of comprehensive diagnostic methods for accurate diagnosis and effective management.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
- Surgical Neurology
Background:
- Esthesioneuroepithelioma, also known as olfactory neuroblastoma, is a rare malignant tumor arising from the olfactory epithelium.
- Pediatric cases are exceptionally rare, presenting unique diagnostic and therapeutic challenges.
Observation:
- A 1-year-8-month-old boy presented with nausea, vomiting, and consciousness disturbance.
- CT revealed a right frontal cystic mass with homogeneous enhancement; angiography showed no hypervascularity.
- Surgical removal demonstrated an extracerebral tumor in the anterior cranial fossa with dural invasion at the crista galli.
Findings:
- Microscopy showed small, undifferentiated cells with rosette formation and high mitotic activity.
- Ultrastructural analysis revealed cilia, microvilli, and junctional complexes, but no dense-cored granules.
- Immunohistochemistry was positive for NSE, S-100, vimentin, and keratin, and negative for NF and GFAP, supporting an esthesioneuroepithelioma diagnosis.
Implications:
- Complete surgical resection combined with adjuvant radiotherapy and chemotherapy led to an uneventful recovery.
- This case underscores the successful management of a rare pediatric brain tumor.
- Accurate diagnosis through integrated pathological and imaging studies is crucial for effective treatment strategies.
Abstract:
A 1-year-8-month-old boy was admitted to our service on September 30, 1986, complaining of nausea, vomiting and consciousness disturbance lasted for about 2 weeks. In CTs, right frontal cystic mass which was homogeneously enhanced by contrast media was revealed. Neither hypervascularity nor tumor staining were seen angiographically. On October 9, 1986, total removal of the tumor was performed. The tumor was located extracerebrally in the right anterior cranial fossa, but was covered with arachnoid membrane. The tumor showed tight adhesion with falx cerebri, particularly at crista galli where an invasive infiltration was seen. Light microscopic examination demonstrated oval or spherical small cells arranged multilobularly with rosette like formation and numerous mitoses. Ultrastructurally, cilia, microvilli and junctional complexes were observed. No dense-cored secretory granules were found in the tumor cells. Immunohistochemical study on this tumor showed negative NF and GFAP; positive NSE, S-100, vimentin and keratin. From these findings, the tumor was diagnosed as esthesioneuroepithelioma. Postoperatively, irradiation and chemotherapies were also performed, and the patient showed uneventful course. On January 31, 1987, he was discharged on his foot, and no recurrent or metastatic signs could be found until the end of March of 1990.