Histopathological examination revealed undifferentiated small round and oval tumoral cells. Immunohistochemical staining was positive for vimentin, desmin, and myogenin. The final diagnosis was alveolar RMS. She received radiotherapy and chemotherapy by vincristine, actinomycin-D, doxorubicin, and ifosfamide. The patient was in good condition with no tumor recurrence or metastasis at the 1-year follow-up.
Chondromyxoid fibroma (CMF) is a rare bone tumor of cartilaginous derivation representing less than 1% of all benign bone neoplasms. It is a slow-growing, locally destructive tumor that usually affects the metaphyseal region of long bones of patients in their second or third decade of life. Involvement of the skull base and orbit is extremely rare. We present the first case of histopathology-proven CMF originating in the greater sphenoid wing and extending into the orbit in a 14-year-old boy. He complained of pain and swelling of his left lateral orbit for 4 months and had 3-mm nonaxial proptosis. Magnetic resonance imaging of the orbit revealed a well-defined lesion in the lateral wall of the left orbit, hypointense on T1-weighted images, and heterogenous hyperintense on T2-weighted images and enhanced after contrast media injection. Through a left semicoronal skin incision, the mass was removed totally and the lateral orbital wall was reconstructed. The clinical manifestations, imaging findings, and surgical treatment of the lesion are discussed with a review of the literature.
Brown tumor is a bone lesion secondary to hyperparathyroidism of various etiologies. Skeletal involvement in primary hyperparathyroidism secondary to parathyroid adenoma is very uncommon and brown tumor has become extremely a rare clinical entity. Hyperparathyroidism is usually associated with high levels of serum calcium. Brown tumor as the only and initial symptom of normocalcemic primary hyperparathyroidism is extremely rare. Moreover, involvement of the skull base and the orbit is exceedingly rare. The authors would report three cases of brown tumor of the anterior skull base that were associated with true normocalcemic primary hyperparathyroidism. Clinical manifestations, neuroimaging findings, pathological findings, diagnosis and treatment of the patients are discussed and the relevant literature is reviewed.
KeywoRds: Brown tumor, Skull base, Normocalcemia, Primary hyperparathyroidism
ÖZKahverengi tümör çeşitli etiyolojiler nedenli hiperparatiroidiye sekonder bir kemik lezyonudur. Paratiroid adenomuna sekonder primer hiperparatiroidide iskelet tutulumu çok nadirdir ve kahverengi tümör çok nadir bir klinik olay haline gelmiştir. Hiperparatiroidi genellikle yüksek serum kalsiyum düzeyleriyle ilişkilidir. Normokalsemik primer hiperparatiroidinin tek ve başlangıç belirtisi olarak kahverengi tümör çok nadirdir. Ayrıca kafatası tabanı ve orbitanın tutulması son derece nadirdir. Yazarlar gerçek normokalsemik primer paratiroidi ile ilişkili anterior kafatası tabanının üç kahverengi tümör vakasını sunmaktadır. Klinik bulgular, nörogörüntüleme bulguları, patolojik bulgular ve bu hastaların tanı ve tedavisi sunulmakta ve ilgili literatür gözden geçirilmektedir.
Glioblastoma occurs rarely in pediatric patients (0.6-7.9% of all glioblastomas). Symptom duration is about 3-5 months prior to diagnosis with a dismal prognosis (median survival of 50 weeks). The authors describe two pediatric age patients with histopathologically confirmed glioblastoma multiforme whose lesions appeared within just 1 week of normal computed tomography scan and magnetic resonance imaging. Both patients had long-term survival (one of them 6 years and the other 3 years and 7 months) following tumor resection. The present report serially illustrates the very rapid development of glioblastoma in childhood and emphasizes the importance of serial neuroimaging as well as paying attention to sudden onset headaches in pediatric patients with inconclusive imaging findings.
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