ABSTRACT
Abstract Myxofibrosarcoma is recognized as a malignant neoplasm of fibroblastic origin with increased prevalence in the elderly, presenting as nodules or tumors that may extend to the dermis and skeletal muscle, preferably in the lower limbs. Histologically it is characterized by a proliferation of spindle cells in a myxoid stroma. Myxofibrosarcoma has a high potential for local recurrence and metastasis, mainly when it presents a high or intermediate histological grade. We report the case of an eighty-four year old patient with a difficult diagnosis of a highly aggressive tumor.
Subject(s)
Humans , Male , Aged, 80 and over , Skin Neoplasms/pathology , Fibroma/pathology , Myxosarcoma/pathology , Skin/pathology , Biopsy , Immunohistochemistry , Magnetic Resonance Imaging , Lymphatic Metastasis , Neoplasm Recurrence, LocalABSTRACT
El mixofibrosarcoma es un subtipo de sarcoma que comúnmente se presenta en las extremidades de personas ancianas. La presentación clínica no es característica y el aspecto histológico es altamente heterogéneo, lo que frecuentemente retrasa el diagnóstico o conduce a uno equivocado. Técnicas de histoquímica e inmunohistoquímica son mandatorias para establecer el diagnóstico de MFS. Presentamos el caso de un hombre de 57 años para ilustrar lo poco sugerente de este diagnóstico dada la presentación clínica. El manejo de este tumor es con cirugía, eventualmente radioterapia y seguimiento estricto.
Myxofibrosarcoma is a subtype of sarcoma commonly found in the extremities of elderly people. The clinical presentation is not characteristic and the histological aspect is highly heterogeneous, which often delays the diagnosis or leads to the wrong one. Histochemistry and immunohistochemistry techniques are required to establish the diagnosis of SFM. We present the case of a 57-year-old man to illustrate the unimpressive nature of this diagnosis given the clinical presentation. The management of this tumor is with surgery, eventually radiotherapy and strict follow-up.
Subject(s)
Humans , Male , Middle Aged , Skin Neoplasms/pathology , Fibrosarcoma/pathology , Myxosarcoma/pathology , Physical Examination , Immunohistochemistry , Upper Extremity , Fibrosarcoma/surgery , Fibrosarcoma/diagnosis , Myxosarcoma/diagnosisABSTRACT
O mixofibrossarcoma, previamente conhecido como histiocitoma fibroso maligno, variante mixoide, é um tumor raro, de origem mesenquimal, composto por células fusiformes e estroma mixoide. Acomete mais idosos, envolvendo extremidades inferiores e estendendo-se, em sua maior parte, até a derme e o subcutâneo. Apresenta altas taxas de recorrência e para seu diagnóstico é fundamental a realização de uma biópsia profunda. Relataremos o caso de um mixofibrossarcoma de alto grau, caracterizado por lesão tumoral de crescimento rápido e pela presença de marcado pleomorfismo celular e componente mixoide em abundância.
Myxofibrosarcoma, previously known as malignant fibrous histiocytoma, myxoid variant, is a rare tumor of mesenchymal origin, composed of spindle cells and myxoid stroma. It mainly affects elderly people, involving the lower extremities and frequently extending to the dermis and subcutaneous tissue. The tumor presents high rates of recurrence, and a deep biopsy is required to obtain the correct diagnosis. We report a case of high-grade mixofibrossarcoma characterized by a rapidly growing tumor and the presence of marked cellular pleomorphism and an abundant myxoid matrix.
Subject(s)
Adult , Humans , Male , Knee/pathology , Myxosarcoma/pathology , Skin Neoplasms/pathologyABSTRACT
O mixossarcoma é uma forma rara de neoplasia cardíaca primária de difícil diferenciação clínica e patológica com o mixoma. Até onde os autores têm conhecimento, este é o primeiro relato de caso na literatura nacional indexada com o tratamento cirúrgico do mixossarcoma atrial esquerdo, em paciente do sexo feminino, de 36 anos de idade, cuja evolução pós-operatória tem sido satisfatória, encontrando-se em classe funcional I (NYHA) e em remissão do processo tumoral há mais de 180 dias.
Subject(s)
Female , Adult , Humans , Heart Atria/pathology , Myxoma/pathology , Myxosarcoma/pathology , Sarcoma/pathology , Cardiac CatheterizationABSTRACT
The authors present a case of primary intracranial extraosseous myxoid chondrosarcoma without any attachment to the cranium or the meninges. The clinical and radiological findings of the primary intraparenchymal tumor are described with a review of the literature concerning cranial and intracranial myxoid chondrosarcoma.
Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Middle Aged , Bone Neoplasms/diagnosis , Bone Neoplasms/pathology , Bone Neoplasms/surgery , Chondrosarcoma/diagnosis , Chondrosarcoma/pathology , Chondrosarcoma/surgery , Magnetic Resonance Imaging , Myxosarcoma/diagnosis , Myxosarcoma/pathology , Myxosarcoma/surgery , Biomarkers, TumorABSTRACT
The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. Echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.