The spectrum of malignancy in craniopharyngioma.
Craniopharyngiomas are low-grade epithelial neoplasms occurring almost exclusively in the sellar/suprasellar region. Histologic malignancy is extremely rare; the literature consists mostly of isolated case reports. Herein, we report 3 patients with craniopharyngiomas exhibiting histologic malignancy, 2 of which received radiation therapy before its appearance. Hematoxylin and eosin-stained slides and selected immunohistochemical stains were reviewed in all cases. Microvessel density analysis was performed in case 2. The patients included 2 men and 1 woman, age 14, 31, and 58 years at presentation, respectively. All patients expired 3 months to 9 years after first resection and 3 to 9 months after identification of histologic malignancy. The latter developed after multiple recurrences and radiation therapy in 2 cases, but seemed to arise de novo in 1 case resembling odontogenic ghost cell carcinoma and lacking any definite low-grade craniopharyngioma precursor. The malignant component of the other 2 cases resembled squamous cell carcinoma and low-grade myoepithelial carcinoma, respectively. The MIB-1 labeling index was markedly increased in the malignant component in comparison with the low-grade precursor. Malignant transformation in craniopharyngiomas, although rare, does exist. It assumes varied histologic appearances, usually after multiple recurrences and radiation therapy, and has a near uniformly fatal outcome. De novo malignancy in odontogenic tumors of the sella is even more unusual, but also has an ominous prognosis.
Rodriguez
Fausto J
FJ
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
Scheithauer
Bernd W
BW
Tsunoda
Shigeru
S
Kovacs
Kalman
K
Vidal
Sergio
S
Piepgras
David G
DG
D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002294:Carcinoma, Squamous Cell / Q000473:pathology; D002471:Cell Transformation, Neoplastic; D003131:Combined Modality Therapy; D003397:Craniopharyngioma / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D016609:Neoplasms, Second Primary / Q000473:pathology*; D010911:Pituitary Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D018714:Radiotherapy, Adjuvant; D044767:Ubiquitin-Protein Ligases / Q000032:analysis
D014408:Biomarkers, Tumor; C513496:MIB1 ligase, human; D044767:Ubiquitin-Protein Ligases