PMC4392567_01
Diagram showing the clinical course of the tumor including surgical procedures and pathological findings
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F1
The clinical course and corresponding figures are shown in Figure 1.
Successive gadolinium-enhanced magnetic resonance images (MRIs) showing the clinical course of the craniopharyngioma during a 6-year period. (a) Tumor regrowth in the left cavernous sinus at 31 years of age. (b) MRI after removal of the lesion and gamma knife surgery. (c) Tumor regrowth at 36 years and 3 months of age. The tumor grew around the left optic nerve. (d) MRI after transsphenoidal biopsy. (e, f) MRIs at 31 years and 8 months of age. Tumor size increased drastically, and the tumor invaded the cavernous sinus and the internal carotid artery
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F2
When he was 31 years old, tumor regrowth occurred in the left cavernous sinus [Figure 2a]. We removed this lesion [Figure 2b], which appeared to be an adamantinomatous craniopharyngioma, and performed gamma-knife surgery (GKS) because of frequent cyst formations. When the patient was 36 years and 3 months of age, the tumor recurred in the cavernous sinus and surrounded the left optic nerve [Figure 2c]. Transsphenoidal biopsy was performed; the pathological findings showed squamous metaplasia, and CyberKnife surgery was performed on the residual lesion [Figure 2d]. However, the residual tumor regrew rapidly in the irradiated area during the next 4 months [Figure 2e and f], suggesting malignant transformation of the tumor.
Computed tomography scans with contrast enhancement before (a and b) and after (c and d) the operation of transcranial and transsphenoidal dual surgeries with an extracranial-intracranial bypass. The tumor was totally removed
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F3
Transcranial and transsphenoidal surgeries with an extracranial-intracranial (EC-IC) bypass using the radial artery. The postoperative course was uneventful, and the tumor was completely removed [Figure 3a-d].
Pathological findings. (a and b) Histopathology at 31 years of age. The tumor is covered with prickle cells, and a single layer of nonatypical basal cells and cholesterin crystals is present in the interstitial tissue. The diagnosis was adamantinomatous craniopharyngioma. (c and d) Histopathology at 36 years and 3 months of age. Densely packed squamous cells and stratification of basal cells with an atypical appearance are seen. The features of adamantinomatous type craniopharyngioma are no longer apparent. (c-d) Hematoxylin and eosin staining at the original magnification
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F4
Serial pathological changes are shown in Figures 4 and 5. In the specimen taken at 31 years of age, the tumor was covered with prickle cells, and a single layer of nonatypical basal cells and cholesterin crystals were present in the interstitial tissue Internal layer of stellate cells in loose connective matrix was found [Figure 4a and b]. The features of adamantinomatous craniopharyngioma were no longer apparent [Figure 4c, d].
Pathological findings of a specimen from the transcranial and transsphenoidal dual surgeries at 36 years and 9 months of age. (a) The number of squamous cells has increased. (b) The lamina propria has collapsed, and infiltration of atypical cells is seen. (c) Tumor cells have enlarged nuclei and clarification of the nucleolus. (d) Parakeratosis and intercellular bridges are present in the tissue. Hematoxylin and eosin staining at the original magnification
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F5
Serial pathological changes are shown in Figures 4 and 5. A specimen from the transcranial and transsphenoidal dual surgeries consisted chiefly of squamous cells [Figure 5].
Immunohistochemistry of p40 (a-c) and p53 (d-f). The specimens were obtained at 31 years of age (A, D), 36 years and 3 months of age (b and e), and 36 years and 9 months of age (after the transcranial and transsphenoidal dual surgeries)
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F6
As shown in immunohistochemistry, the abundance of p40 (a specific marker of squamous cells) and p53 (the indicator of malignancy) progressively increased between the ages of 31 and 36 years and 9 months [Figure 6].