Undifferentiated high-grade pleomorphic sarcoma is a tumor of mesenchymal cells without clear cell differentiation and represents a diagnosis of exclusion. It is located mainly in extremities, so its location in the head and neck is extremely rare. We report the case of an undifferentiated high-grade pleomorphic sarcoma located on the hard palate.
We report the case of a patient who presented a pineal tumor. A partial resection was performed. The biopsy showed a glioneuroma rosette forming tumor, considered as WHO grade I. The patient was observed for 13 months and experienced significant tumor growth. Radiotherapy was performed. Instead of scarce evidence of the topic and treatment modalities in this type of tumors; radiotherapy was performed. The patient has been stable and asymptomatic.Keywords: pineal, tumor, partial, resection, radiotherapy
IntroducationThe rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle tumor of the glioneuronal family (Komori, 2002). The clinical presentation, natural history, and treatment response of these tumors has been unclear as there are no significant series of a sizeable population with long-term follow-up (Solis, 2011;Marhold, 2008). Classically, has been described as WHO I grade neoplasms with indolent biologic behaviour. The optimal management is surgical resection, mainly in symptomatic patients cases where tumor is causing mass effect over neighboring structures (Zhang, 2013). Adjuvant treatments are supported by few data and their role is unknown. We present the case of a rosette forming glioneuroma tumor with aggressive behavior that doubled size after 16 months of follow up after partial resection achieving decompression of neural structures.
Case ReportThe present case refers to a previously healthy 48 years old woman who began with mild headache for 2 weeks. Brain MRI showed an expansive cystic-solid heterogeneous contrast enhancement mass of 2,4 cm in the pineal region with mass effect over mesencephalon and cuadrigeminal plate. Tumor markers, including beta-human chorionic gonadotropin, and alpha-fetoprotein were not significantly elevated in both the cerebrospinal fluid (CSF). A partial resection of the tumor by endoscopic approach and III cisternoventriculostomy was approached because of thalamic invasion.The tumor specimen was analyzed by pathology department of Universidad de Concepcion and the case was consulted to neuropathologists of the MD Anderson cancer center. The final biopsy report was Rosette forming glioneuronal tumor, Ki67 negative, GFAP positive. The patient was evaluated then by an interdisciplinary oncology committee (neurosurgery, radiation oncology, pathology and medical oncology) and the management decision based on the evidence reported was observation, mainly due to the indolent behavior reported in the literature.
Abstract:We report on a patient who presented to the ENT service with right side nasal obstruction. Imaging studies revealed an aggressive non-calcified solid heterogeneous mass centered in the right naso ethmoidal region. The mass was hyper enhanced following contrast media administration. The patient underwent partial tumor resection and a biopsy was performed confirming the presence of Solid Alveolar Rhabdomyosarcoma. The patient was treated with chemo-radiation therapy.
We report on a patient who presented to the ENT service with right side nasal obstruction. Imaging studies revealed an aggressive noncalcified solid heterogeneous mass centered in the right naso ethmoidal region. The mass was hyper enhanced following contrast media administration. The patient underwent partial tumor resection and a biopsy was performed confirming the presence of Solid Alveolar Rhabdomyosarcoma. The patient was treated with chemo-radiation therapy.
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