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Background: Primary intraosseous hemangioma (PIH) of the skull base, when localized in the sella, is a rare, benign lesion that can mimic other common sellar tumors. Such tumors may be asymptomatic incidental radiologic findings or present with nonspecific symptoms (e.g., headaches). Case Description: We present a case of a primary intraosseous hemangioma of the body of sphenoid bone extending into the sellar cavity, clinicoradiographically mimicking an atypical pituitary adenoma. Conclusions: PIH should be included as a rare differential diagnosis in cases of space-occupying sellar lesions with atypical features. Radiologic and intraoperative findings may be suited to entertain a probable diagnosis; however, a definite diagnosis can only be obtained via histopathologic analysis. Surgical excision may be chosen under the assumption of dealing with a primary pituitary lesion, but extent of resection depends on the accessibility, extent, involvement of surrounding structures (such as the internal carotid artery/cavernous sinus), and control of intraoperative bleeding. When facing inoperable or residual lesions, radiotherapy can be a viable option. Key words: Mixed hemangioma, Pituitary, Primary intraosseous hemangioma, Sella turcica, Skull base, Sphenoid