Undifferentiated pleomorphic sarcoma of the mandible.

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm that is usually located in the extremities and retroperitoneum. In the past, UPS was considered the most common soft tissue sarcoma in adults; due to improvements in diagnostic techniques, most cases have been reclassified as other lineage-specific tumors. Gnathic bones are rarely affected, and the clinicopathological characteristics of this neoplasm when diagnosed in the jaw remain to be better described. In this report, we present a rare case of mandibular UPS affecting an 88-year-old female who demonstrated a painful swelling on the right side of the mandible that was accompanied by a pathological fracture. Microscopic examination revealed a pleomorphic spindlecell neoplasm with mitotic figures and necrosis. The patient underwent surgery and adjuvant radiotherapy but experienced metastasis after 12 months of follow-up and died. Diagnosis of UPS is challenging, and oral pathologists must be aware of this entity when dealing with aggressive undifferentiated neoplasms.

Rapidly Progressive Malignant Fibrous Histiocytoma of Right Atrium: a Rare Case Report.

We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

Rapidly progressive malignant fibrous histiocytoma of right atrium: a rare case report

Abstract We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

Sarcoma pleomórfico. Presentación de un caso / Pleomorphic sarcoma. Case presentation

Se presenta un paciente del sexo masculino de 36 años de edad, que en el momento del inicio del cuadro clínico, a partir de un trauma leve, debutó con fiebre, dolor y aumento de temperatura a nivel del muslo derecho, se valoró como un posible hematoma abscedado por lo que requirió ingreso y tratamiento quirúrgico. Posterior al alta y dos meses de evolución, regresa al centro hospitalario por presentar una evolución tórpida con un aumento de volumen de tipo tumoral se ingresó, estudió y se valoró por un equipo de especialistas multidisciplinarios decidiendo una nueva intervención quirúgica en base a los resultados de laboratorio y estudios imagino lógicos, se le realizó exéresis de la tumoración (a nivel del vasto externo) con margen oncológico y enviado para anatomía patológica, informado como un sarcoma pleomórfico, llevó posteriormente tratamiento adyuvante de quimioterapia, contando con 10 años de sobrevivencia en la actualidad y totalmente asintomático.

The case of a male patient aged 36 years is presented. The clinical characteristics are the following: after a light trauma, he had fever, pain, temperature increase in the right thigh. It was diagnosed as a possible abscessed hematoma and the patient was admitted in the hospital and surgically treated. After discharging and two-months evolution, he came back to the hospital presenting a torpid evolution with a tumoral-kind volume increase. He was readmitted, studied and evaluated by a team of multidiscipline specialists, who decided a new surgical intervention based on the laboratory and imaging studies. Tumor was removed (at the level of external vastus) with oncologic margin and send to pathologic anatomy. It was informed as a pleomorphic sarcoma. An adjuvant chemotherapeutic treatment was applied later. Currently, the patient has survived 10 years and is totally asymptomatic.

Sarcoma pleomórfico gigante de la mama / Giant pleomorphic breast sarcoma

Abstract It is reported the case of a female patient 50 years old who, after failed attempts at diagnosis, arrives at breast surgery service of the E.S.E. Hospital Universitario del Caribe with history of five months consistent inthe excessive growth of a mass in right breast; patient received radical mastectomy plus reconstruction withlatissimus dorsi muscle flap. Following excision of tumor mass of 6,500 g is performed immunohistochemicaldiagnosis of a giant pleomorphic sarcoma.

Resumen introducción: Los tumores derivados del tejido conectivo interlobulillar de la mama son en mayor proporción malignos y, en virtud a su ubicación, en la actualidad reciben el nombre de "sarcomas" de la mama. Caso clínico: Se reporta el caso de una paciente femenina de 50 años de edad quien, posterior a fallidosintentos de diagnóstico, acude al servicio de cirugía de mama de la E.S.E. Hospital Universitario del Caribecon un cuadro de cinco meses de evolución consistente en el crecimiento desmedido de una masa en mamaderecha; la paciente recibió mastectomía radical más reconstrucción con colgajo de músculo dorsal ancho.Posterior a exéresis de masa tumoral de 6.500 g de peso se lleva a cabo diagnóstico inmunohistoquímico deun sarcoma pleomórfico gigante de la mama.

Sarcoma Pleomórfico Indiferenciado de Maxilar de Alto Grado: Diagnóstico, Tratamiento y Pronóstico / Undifferentiated Pleomorphic Sarcoma of the Maxillary: Diagnosis, Treatment and Prognosis

Los sarcomas son neoplasias poco vistas en el territorio maxilofacial, donde la baja frecuencia de casos y los cambios de nomenclatura, han dificultado su estudio. Una de las variables de este tipo de lesiones corresponde a un tumor conocido como sarcoma pleomórfico indiferenciado, del cual se sabe muy poco pues se describe una incidencia de 1:1.000.000. Corresponde a un tumor maligno con características clínicas e imagenológicas de crecimiento invasivo y destructivo; la confirmación diagnóstica se realiza por medio de una biopsia en base al estudio histológico e inmunohistoquímico. Aparece principalmente en el tracto nasosinusal y su manejo terapéutico se basa en la cirugía con amplios márgenes de seguridad y radioterapia o quimioterapia según corresponda. No presenta metástasis locales pero si a distancia con gran afinidad por el pulmón. Se reporta un caso de sarcoma pleomórfico indiferenciado de alto grado de maxilar diagnosticado en la Unidad de Patología Oral y Maxilofacial del Hospital las Higueras de Talcahuano durante el año 2014, que a diferencia de la mayoría de los casos reportados, producto de su extensión, fue tratado con radioterapia paliativa. Además, se realizó una revisión de la literatura disponible en diversas bases de datos con lo cual se obtuvo información de casos ya reportados. Actualmente resulta difícil producto de la baja cantidad de casos descritos poder hacer proyecciones en cuanto a su pronóstico o evolución, sin embargo hay consenso de que el manejo interdisciplinario, diagnóstico precoz y la confirmación de este por medio de exámenes complementarios son fundamentales para su adecuado tratamiento.

Sarcomas are neoplastic lesions rarely observed in the maxillofacial region. Due to the few cases reported and to changes in nomenclature of these tumors their study has been difficult. One of the variants of this kind of lesion corresponds to a tumor currently known as undifferentiated pleomorphic sarcoma, which there is scarce knowledge of. In fact the incidence described is about 1:1.000.000. This one corresponds to a malignant tumor with image and clinical features as invasive and destructive growth, the confirmation of diagnosis is carried out by performing biopsy based on histologic and immunohistochemical studies. They appear mostly in the nose and sinusal tract. Its therapeutic management is based on surgery with wide security margins and radio or chemotherapy when warranted. No local metastases but distant ones are found and highly affined with lungs. We report a case of an advanced undifferentiated pleomorphic sarcoma diagnosed at the Oral and maxillofacial pathology unit of Hospital Las Higueras in Talcahuano at 2014, which in spite of most of the reported cases in literature; because of the extent it was treated with palliative radiotherapy. Also has been made review of the available literature in different databases with which information of the reported cases was obtained. Nowadays, due to the low number of cases described it is difficult to establish projections in terms of prognosis or evolution. However, there is consensus that interdisciplinary management, early diagnosis and confirmation with complementary exams are essential for an appropriate treatment.