Mixofibrosarcoma mandibular de bajo grado en un adulto / Low grade mandibular myxofibrosarcoma in an adult

Se describe el caso clínico de un paciente de 61 años de edad, con antecedente de buena salud, atendido en el St. Paul's Milennium Medical Hospital de Etiopía por presentar, desde hacía 2 años, una lesión en la encía marginal, con abombamiento de ambas corticales, que se extendía del primer premolar inferior derecho al izquierdo, por lo cual se decidió ingresarlo en el Servicio de Cirugía Maxilofacial para su estudio y tratamiento. Se trataba de un tumor poco frecuente y de gran tamaño, con amplia diseminación ganglionar hacia los niveles IV y V de ambas partes del cuello, de manera que se realizó el procedimiento quirúrgico. La evolución fue favorable en el posoperatorio inmediato y egresó de la institución 7 días después, pero continuó con radioterapia. Y seguimiento por consulta externa durante 24 meses después.

The clinical case of a 61-year-old patient, with a history of good health, treated at St. Paul's Milennium Medical Hospital in Ethiopia for presenting, for 2 years, a lesion in the marginal gum, with bulging of the gums, is described. both cortices, which extended from the lower right to the left first premolar, for which it was decided to admit him to the Maxillofacial Surgery Service for study and treatment. It was a rare and large tumor, with extensive lymph node dissemination to levels IV and V in both parts of the neck, so the surgical procedure was performed. The evolution was favorable in the immediate postoperative period and he was discharged from the institution 7 days later, but continued with radiotherapy. And follow-up by outpatient service for 24 months afterwards.

Factores hereditarios y síndromes de predisposición genética relacionados con sarcomas de partes blandas / Hereditary factors and syndromes of genetic predisposition related to sarcomas of soft parts

Los sarcomas de partes blandas son tumores malignos que se originan en el tejido conectivo, a partir del mesénquima embrionario. Teniendo en cuenta la existencia de nuevos y constantes cambios en la naturaleza de estos tumores, se realizó una revisión de las publicaciones más recientes para profundizar en las alteraciones genéticas, los síndromes de predisposición y su relación con los sarcomas. Se pudo concluir que aún siguen siendo muy pocos los registros que describen la identificación de dichos síndromes como principal eslabón en el desarrollo de los sarcomas.

The sarcomas of soft parts are malignancies that originate in the connective tissue, starting from the embryonic mesenchyme. Taking into account the existence of new and constant changes in the nature of these tumors, a review of the most recent publications was carried out to deepen in the genetic disorders, the predisposing syndromes and its relationship with sarcomas. It was concluded that the records that describe the identification of these syndromes are still very few as main link in the development of sarcomas.

Clear Cell Sarcoma of the Wrist: MRI Findings with Diffusion-Weighted Image and Histopathologic Correlation

Clear cell sarcoma is rare and difficult to diagnose. Herein, we present a case of clear cell sarcoma in the dorsum of the wrist with MRI findings, including diffusion-weighted imaging, and histopathologic correlation, which was initially diagnosed as giant cell tumor of tendon sheath.

Un caso inusual de rabdomiosarcoma / An unusual case of rhabdomyosarcoma

Los sarcomas de partes blandas aparecen a cualquier edad, aunque son más frecuentes en la cuarta década de la vida. Se denominan así a aquellos tumores que se originan en las estructuras que soportan el cuerpo o envuelven los órganos y tejidos, y es el rabdomiosarcoma el tumor de partes blandas más frecuente en edad pediátrica. Es rara la condición de intratabilidad, mientras que son frecuentes las recaídas tempranas y tardías después del tratamiento. Presentamos un caso de una paciente diagnosticada en etapa de recién nacida con una mala respuesta al tratamiento, con progresión en el curso de este (algo no habitual), pues, generalmente, existe una buena respuesta inicial y después puede haber recaídas

The soft parts sarcomas appear in any age, although are more frequent in the fourth decade of life. Are called in this way, those tumors originating in structures supporting the body or surrounding the organs and tissues and it is the rhabdomyosarcoma the more frequent soft parts tumor present in children. It is uncommon the irritability whereas the early and late relapses are frequent. This is the case of a patient diagnosed in the newborn stage with a poor response to treatment and progression during its course (something unusual), since generally, there is a good initial response and afterwards may be relapses

Sinoviosarcoma de tornozelo: quarta recidiva / Ankle's synoviosarcoma: fourth relapse

Esse relato apresenta o caso de uma paciente com quarta recidiva de sinoviosarcoma, um sarcoma de partes moles que possui estreita relação com bainhas tendinosas, bursas e cápsulas articulares. A paciente apresentou uma quarta recidiva, sendo necessária a realização da amputação de perna, visto que as cirurgias conservadoras já não aliviam o sofrimento da paciente. O principal objetivo deste relato de caso é apresentar um caso clássico de neoplasia rara, expondo a doença e fazer uma breve revisão da literatura e mostrar sua importância, visto que corresponde a apenas 5% das neoplasias de partes moles.

This report presents the case of a female patient with fourth synoviosarcoma recurrence, a sarcoma of soft parts that have close relationship with tendom sheaths, bursas and articular capsule.The patient present her fourth recurrence, in wich the perfomance of amputation was required, since the conservative surgeriesno longer relieved the patient suffering. The main objective of this case report is to present a classic case of a rare cancer, exposing the disease, and to make a brief review of the literature to show its importance, since it corresponds to only five percent of the cancers of soft parts.

Ossifying Fibromyxoid Tumor of Soft Parts

An ossifying fibromyxoid tumor of soft parts is a rare, recently described, fibro-osseous neoplasm of uncertain histogenesis. It occurs most frequently within the subcutis or skeletal muscle of the extremities. Its biologic behavior is generally regarded as benign with at worst a locally aggressive clinical course. But, atypical and malignant variants have been recently reported. Herein we report a case of a benign ossifying fibromyxoid tumor which occurred in the left upper back of 41-year-old man. The tumor is composed of uniformly round or polygonal cells arranged in cords or nests which are separated by myxoid and hyalinzed fibrous matrix and associated with irregular bony trabeculae. The tumor cells are strong positive for vimentin. Ultrastructural findings and a review of literatures are added.

Ossifying Fibromyxoid Tumor of Soft Parts

An ossifying fibromyxoid tumor of soft parts is a rare, recently described, fibro-osseous neoplasm of uncertain histogenesis. It occurs most frequently within the subcutis or skeletal muscle of the extremities. Its biologic behavior is generally regarded as benign with at worst a locally aggressive clinical course. But, atypical and malignant variants have been recently reported. Herein we report a case of a benign ossifying fibromyxoid tumor which occurred in the left upper back of 41-year-old man. The tumor is composed of uniformly round or polygonal cells arranged in cords or nests which are separated by myxoid and hyalinzed fibrous matrix and associated with irregular bony trabeculae. The tumor cells are strong positive for vimentin. Ultrastructural findings and a review of literatures are added.

A Case of Malignant Melanoma of Soft Parts with Unusual Histopathologic Findings

We report a case of malignant melanoma of soft parts presented with a hard mass on the dorsum of the left second toe. Malignant melanoma of soft parts has also been known as clear cell sarcoma of tendons and aponeuroses, which indicates the histologic feature of clear cell predominance and the origin of tumor cells, tendinous or aponeurotic structures. Although our case presented with many characteristic clinicopathologic features of malignant melanoma of soft parts, diagnosing this case as malignant melanoma of soft parts was made with great difficulty because of a rather unusual finding that the major cell component of neoplasm was spindle shaped cells in stead of clear cells. Cases featuring this distinctive histopathologic finding have not been reported individually to our knowledge, but described briefly in the literature. We think this case deserves special attention because of its close resemblance to various sarcomas.