Malignant craniopharyngioma; case report and review of the literature

Abstract

A case of malignant craniopharyngioma in a 46-year-old woman presenting clinically with visual disturbance and bifrontal headache is reported. Histopathologic examination of the suprasellar mass showed a lesion characterized by nests of epithelial cells with a basaloid appearance, round-to-oval nuclei, moderate pleomorphism, hyperchromasia, increased nuclear cytoplastic ratio and high mitotic activity. Immunohistochemically, the tumor cells were positive for Ki-67 (44.3%), p53 (98%), and p63 (100%), but negative for estrogen and progesterone receptors. Clinical and pathologic features with a brief review of the relevant literature for malignant craniopharyngioma as a novel member of tumors of the suprasellar region, is discussed.

Malignant Transformation of Craniopharyngioma Associated With Moyamoya Syndrome -Case Report-

“…18) Other de novo tumors contain components of adamantinomatous craniopharyngioma. 3,24) The current case presented similar diagnostic problems. No adequate specimens could be obtained for definitive diagnosis before malignant transformation because of the hardness of the tumor and the difficulty in hemostasis.…”

“…This immunohistochemical finding may be an effective surrogate marker of malignant transformation. 3,7,17,18) Positive immunostaining for p63, a homologue of p53 protein, was also observed in the present case, although this issue remains to be elucidated.…”

“…3,18,24) Such tumors should possibly be considered as a World Health Organization grade 3 tumor, a new variant of malignant craniopharyngioma, 3,10) although whether the tumors are derived from the remnant of Rathke's pouch is debatable. De novo tumor is histologically similar to odontogenic tumors, so that the tumor is an odontogenic ghost cell carcinoma.…”

“…1,3,7,14,17,18,20,23,24) The patients were aged 11 to 62 years (median 31 years), and the male to female ratio is 6:7. Radiotherapy was administered in all but the three patients with de novo tumor, and may have been one of the causes of the subsequent malignant transformation.…”

“…Almost all craniopharyngiomas have benign features, and histologically malignant craniopharyngiomas are extremely rare. 1,3,7,14,17,18,20,23,24) Moyamoya disease is an idiopathic disease entity characterized by progressive intracranial occlusion of the anterior circulation followed by the appearance of an abnormal collateral vascular network around the circle of Willis. 15,21) Moyamoya vessels may also arise in patients with predisposed disorders such as parasellar tumors and cranial irradiation, 10,13) which is known as moyamoya syndrome, quasi moyamoya disease, akin moyamoya disease, and moyamoya-like vasculopathy.…”