Myofibroblastoma in the suprasellar region

Abstract

Myofibroblastoma is a rare type of benign mesenchymal tumor; only two cases of intracranial myofibroblastoma have been reported in the literature. The authors report on the case of a 34-year-old woman with a myofibroblastoma in the suprasellar region who presented with the complaint of sudden onset of headache followed within 2 weeks by progressively worsening visual disturbance. Computerized tomography scanning demonstrated a mixed low- and high-density mass in the suprasellar region and contrast-enhanced magnetic resonance imaging revealed the mass to be of mixed intensity with heterogeneous enhancement. The tumor was subtotally removed via a right frontobasal translamina-terminalis approach and her vision improved immediately. Histologically, the tumor was characterized by alternating areas of spindle-shaped and round cells that were separated by collagen fibers. The diagnosis of myofibroblastoma was based on the tumor's intense immunoreactivity for alpha-smooth-muscle actin and the ultrastructural identification of myofibroblasts. The tumor was thought to have originated from the meninges in the suprasellar region.

Undifferentiated Sarcoma of the Sellar Region

“…The existence of a primary spindle cell sarcoma of the sella turcica in a patient who has not undergone previous radiotherapy is an extremely rare event [5]. Benign spindle cell lesions of the sellar region include meningioma, schwannoma, myofibroblastoma, solitary fibrous tumor, pituitary adenoma with spindle cell features, pilocytic astrocytoma, and pituicytoma to name a few [9][10][11]. These are readily distinguished from our obviously anaplastic tumor.…”

“…This prompted an unsuccessful transfrontal-transventricular biopsy at another institution; the specimen was inadequate for assessment. Prior to the present surgery, blood hormone levels were normal, including growth hormone (GH) 0.02 ng/ml (<5.0 ng/ml), insulin-like growth factor 61.4 ng/ml (48-255 ng/ml), prolactin (PRL) 6.29 ng/ml (0-15 ng/ml), luteinizing hormone (LH) 2.97 mUI/ml [2][3][4][5][6][7][8][9][10][11][12], folliclestimulating hormone (FSH) 7 mUI/ml [1][2][3][4][5][6][7][8], thyrotropic hormone (TSH) 1.67 mUI/ml (0.5-6.0 mUI/ml), free T4 1.17 ng/dl (0.8-1.8 ng/dl), and cortisol 11.8 mcg/ml . Measurements of germ cell markers including human chorionic gonadotropin, alpha fetoprotein, and lactate dehydrogenase were not detected.…”

Meningeal myofibroblastoma in the frontal lobe: A case report

“…To the best of our knowledge, only three cases of intracranial myofibroblastoma have previously been reported (7–9). The details of these case studies are summarized in Table I.…”

“…It has features of fibroblasts and smooth muscle cells, with frequent mitoses. The cells are also characterized by elongated to oval-shaped nuclei, irregular nuclear contours, finely-dispersed chromatin and poorly-defined cytoplasm (1,2,7,9,10). Immunohistochemical staining has shown that the tumor cells are strongly positive for SMA and vimentin and weakly positive for desmin and CD34 (8,9).…”

“…The majority of the masses are located in the breast, however, the number of extramammary myofibroblastoma cases being reported is increasing (2–6). Only three cases of intracranial myofibroblastoma have been reported (7–9). Histopathological and immunohistochemical staining is crucial to determine a diagnosis of intracranial myofibroblastoma.…”