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Malignant transformation of craniopharyngioma with detailed follow-up
Wei Wang1, Xiao-Dong Chen, Hong-Min Bai
1Department of Pathology, Guangzhou Liuhuaqiao Hospital, Guangzhou, China.
Abstract:
A 29-year-old male patient was admitted into hospital with the main complaint of progressive visual disturbance. Both CT SCAN and MRI demonstrated a cystic-solid contrast-enhancing sellar-suprasellar mass with obvious calcification. Histopathological examination of the first resected specimen showed a typical appearance of adamantinomatous craniopharyngioma. The patient received gamma knife therapy after his first operation because of partial tumor removal. He experienced two relapses in the subsequent 2 years, for which only surgical resection was performed. The later histopathology presented malignant appearance with tumor cells moderate to severe pleomorphism, hyperchromasia, increased nuclear cytoplastic ratio, high mitotic activity (30/10 high power fields) and focal coagulative necrosis. The patient died 9 months after identification of histologic malignancy. Clinical and histopathological features, biological behavior of one case of malignant craniopharyngioma were discussed, with a brief review of the relevant literature.
Insights
Malignant transformation of adamantinomatous craniopharyngioma is rare but aggressive. This case highlights the challenges in managing aggressive pituitary tumors and the need for further research into malignant craniopharyngioma.
Area of Science:
- Neuro-oncology
- Endocrinology
- Pathology
Background:
- Craniopharyngiomas are rare tumors arising from Rathke's pouch remnants.
- Adamantinomatous craniopharyngioma is the most common subtype, typically benign.
- Malignant transformation is exceptionally rare, posing diagnostic and therapeutic challenges.
Observation:
- A 29-year-old male presented with progressive visual disturbance.
- Imaging revealed a cystic-solid, calcified sellar-suprasellar mass.
- Initial histopathology confirmed adamantinomatous craniopharyngioma.
Findings:
- The patient underwent surgery and gamma knife therapy for recurrent tumors.
- Subsequent resections revealed malignant transformation with high-grade features.
- The patient succumbed to the disease 9 months after diagnosis of malignancy.
Implications:
- This case underscores the potential for malignant behavior in craniopharyngiomas.
- Aggressive tumor biology necessitates careful monitoring and tailored treatment strategies.
- Further investigation into the pathogenesis and management of malignant craniopharyngioma is warranted.
