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.

A Case of Myxofibrosarcoma in the Cheek / 대한이비인후과학회지

Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, mainly occurring in the lower extremities and trunk. However, head and neck myxofibrosarcoma is extremely rare. The most reliable treatment of adult soft tissue sarcoma is surgical resection with negative margin. A 79-year-old man presented with a left cheek mass first detected six months ago. The pathologic report of the mass showed that it was myxofibrosarcoma and consequently postoperative radiotherapy was done. However, distant and locoregional metastasis occurred postoperatively. We report this case with a brief review of literature.

Myxofibrosarcoma - Case report

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.

Mixofibrosarcoma de bajo grado: reporte de caso / Low grade mixofibrosarcoma: case report

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.

Mixofibrossarcoma: relato de caso / Mixofibrossarcoma: case report

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.

Myxoinflammatory fibroblastic sarcoma: a clinicopathologic study of 6 cases with review of literature / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotypes and differential diagnosis of myxoinflammatory fibroblastic sarcoma (MIFS).</p><p><b>METHODS</b>The clinical and pathologic features of 6 cases of MIFS were analyzed. Immunohistochemical study was performed using the standard EnVision method.</p><p><b>RESULTS</b>There were altogether 2 adult males and 4 adult females (median age = 47 years and mean age = 50 years). Three cases were located in the lower extremities, 2 in the upper limbs and 1 in the axillary region. Common presentation included slowly growing mass or swelling in the extremities, accompanied by mild pain or tenderness. Grossly, the tumor appeared multinodular and ranged from 2.5 cm to 4.6 cm in diameter (mean = 3.4 cm). Microscopically, there was a dense inflammatory infiltrate merging with hyaline and myxoid zones in various proportions. Spindle-shaped tumor cells were seen admixed with large atypical cells which distributed singly or in small clusters, amongst an inflammatory, hyaline or a myxoid background. These atypical cells had large nuclei and prominent nucleoli, resembling virocytes, Reed-Sternberg cells or ganglion cells. Mitotic figures were rarely identified. Extracellular mucin associated with scattered monovacuolated or multivacuolated lipoblast-like cells was noted. Immunohistochemically, these bizarre cells were consistently positive for vimentin, but negative for a panel of antibodies including LCA, CD15, CD30, CD34, CD68, S-100, HMB45, AE1/AE3, smooth muscle actin and desmin. Follow-up result was available in 4 cases; and 2 of them showed local recurrence after an incomplete excision. There was no evidence of distant metastasis.</p><p><b>CONCLUSIONS</b>MISF is a low-grade sarcoma of fibroblastic differentiation. Awareness of the clinical and pathologic characteristics is helpful in arriving at the correct diagnosis and distinction from benign inflammatory fibromyxoid lesions.</p>

High Grade Myxofibrosarcoma in the Paravertebral Muscle: A Case Report

Many different abnormalities, such as, neoplasm, infection, traumatic hematoma, or congenital or immune myopathy, may be found in the paravertebral muscles. However, neoplasms of paravertebral muscle are an uncommon cause of back pain. Such neoplasms may arise from local lesions or due to metastatic spread from a distant malignancy. The differential diagnoses of a primary soft tissue malignancy and metastatic spread from a skeletal muscle tumor are important. In cases of soft tissue sarcoma, histopathological findings and surgical margins are both related to local recurrence and metastasis, therefore, percutaneous needle biopsy may be helpful before surgical excision. A degree of surgical excision is decided based on considerations of muscular function and histopathological findings.

Low-grade fibromyxoid sarcoma: a clinicopathological analysis of 9 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathological features, immnophenotype and differential diagnosis of low-grade fibromyxoid sarcoma (LGFMS).</p><p><b>METHODS</b>The clinical, radiological and pathological features of 9 cases of LGFMS were reviewed.</p><p><b>RESULTS</b>The patients consisted of six males and three females with ages ranging from 11 to 65 years (mean 31.4 years). Clinically, most cases presented as slowly growing painless masses located in the extremities, trunk and neck. Two cases had a history of a rapid recent enlargement. Three cases presented with recurrent diseases after incomplete resection. Ultrasound showed heterogeneous signal. Precontrast CT and T1-weighted MRI showed a nodular mass of low to isodensity, while contrast-enhanced CT and T2-weighted MRI demonstrated contrasting zonal areas of hypo/hyperintensity. The tumors measured 3 to 16 cm (mean 7.7 cm), with a fibrous to myxoid appearance on cut surface. Histologically, they were composed of alternating collagenous and myxoid areas. A transition between the two areas could be identified. Giant rosette-like structures were observed in 3 cases. The center of the giant rosettes was composed of eosinophilic collagen, which showed centrifugal in arrangement. There was also gradual transition between the giant rosettes and the fibromyxoid component. On high magnification, the tumor was composed of ovoid to spindle-shaped cells with hyperchromatic nuclei. Nuclear atypia was inconspicuous. The tumor cells were arranged mostly in interlacing fascicles or whorls. Vessels were not prominent and necrosis was absent. Immunohistochemically, the tumor cells showed unique staining for vimentin, consistent with a fibroblastic differentiation.</p><p><b>CONCLUSIONS</b>LGFMS is a distinctive low grade fibroblastic sarcoma of young adults. Recognizing the characteristics of the rare entity may help to avoid misdiagnosis. Wide local excision is recommended to avoid local recurrences.</p>

Myxoid Chondrosarcoma of the Sinonasal Cavity in a Child: a Case Report

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma.

Mixossarcoma atrial esquerdo: relato de caso / Left atrial myxosarcoma: case report

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.

Giant Cell Malignant Fibrous Histiocytoma of the Breast: A Case Report

A case of primary malignant fibrous histiocytoma of the breast is reported. The patient was a 48-yr-old woman with a huge tumor involving almost the entire left breast. The central portion of her left breast was already rotted by extensive necrosis and inflammation. She was treated by radical mastectomy and axillary lymphadenectomy to level I. Pathologic examination supported by an immunohistochemical staining confirmed the tumor as malignant fibrous histiocytoma of giant cell type. Axillary lymph nodes were free from tumor metastasis. She had not taken any postoperative adjuvant therapy. The metastasis to lungs was found 2 months after the operation, and she died within 6 months.

Primary Intracranial Myxoid Chondrosarcoma: Report of a Case and Review of the Literature

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.

Left atrial myxosarcoma with systemic metastasis: a case report

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.

Primary Left Atrial Myxosarcoma: One case Report / 대한흉부외과학회지

Primary cardiac myxosarcoma is extremely rare, which is uncommon in primary cardiac tumors. We report a primary cardiac myxosarcoma in a 40-year-old female patient who was admitted to the hospital because of exertional dyspnea and palpitation. The patient underwent emergency operation immediately after the intracardiac (left atrium) tumor was discovered by an echocardiography. Palliative tumor removal was done and final pathologic diagnosis was primary cardiac myxosarcoma. She was discharged without complications.

Mixossarcoma em mesentério de cäo / Mesenteric myxosarcoma in a dog

Myxosarcoma is an unusual neoplasm in the dog, most seen in the skin, and seldom in the heart and in the liver. The authors describe a case of a 5-years old mongrel dog showing abdominal distension, weakness, and decreased appetite. An exploratory celiotomy showed a massive non-encapsulated nodular tumour, adhered on the mesentery, measuring 30cm in diameter. Histopatology identified the tumour as a myxosarcoma. There is not previous record of mesenteric myxosarcoma

Mixosarcoma de aurícula izquierda: presentación de un caso / Myxosarcoma of left atrium: a report of a case

El mixiosarcoma del corazón es un tumor extremadamente raro, representa menos de 1 por ciento de los sarcomas malignos. El mixoma es el tumor más frecuente del corazón y, en 90 por ciento de los casos de una mujer de 25 años de edad que inició con disnea de medianos esfuerzos, malestar general, tos productiva y edema de miembros inferiores. La telerradiografía de tórax mostró derrame pleural bilateral. El ECG indicó arritmias cardiacas y bloqueo ventricular, mientras que en el ecocardiograma se observó neoplasia maligna infiltrante a la aurícula izquierda. Se realizó toracotomía con toma de biopsia que resultó mixosarcoma. Recibió quimioterapia complementaria y actualmente se encuantra sin actividad tumoral. Se discute la frecuencia y el diagnóstico diferencial de esta enfermedad

A Case of Congenital Cystic Adenomatoid Malformation of the Lung Containing Fungal ball-like movable Blood clots in Healthy Young Woman / 결핵

We describe unusual manifestations of congenital cystic adenomatoid malformation ( C.C.A.M.) of the lung, such as movable fungal ball-like intracystic blood clots and hemoptysis, which were detected in previously healthy 23 years-old woman. We identified blood clots only after left upper lobectomy and could not distinguish from fungal ball with radiographic methods. CCAM of the lung, rare and lethal form of congenital pulmonary cystic disease, was initially introduced by Ch'in and Tang in 1949. The histogenesis of this lesion is characterized by polypoid glandular tissue proliferation and overgrowth of mesenchymal elements due to cessation of bronchiolar maturation which occured in after 16weeks intrauterine period. In 80-95% of reported cases, the lesion was confined to a single lobe and there was no lobe and right and left lung predilection. The clinical presentation may be widely variable, ranging from intrauterine fetal death to late discovery in childhood with recurrent pulmonary infection. But there's no reports which were misdiagnosed with intracystic fungal ball. The treatment choice is lobectomy of affected lobe. There's a few case reports with rhabdomyosarcoma, bronchiolar cell carcinoma and myxosarcoma arising in CCAM patients. Therefore, early resection is recommended even if asymtomatic cases. We experienced a rare case of CCAM of the lung in 23 years old female, and there were intracystic fungal ball-like movable blood clots in lower portion of left lung. After left upper lobectomy was performed, now she is discharged and followed up without any complications.

A Case of Congenital Cystic Adenomatoid Malformation of the Lung / 대한내과학회지

Congenital cystic adenomatoid malformation(C.C. A.M.) is a rare variant of congenital pulmonary cystic diseases. C.C.A.M. was first distinguished from other pulmonary cystic lesions by Ch`in and Tang in 1949. It is characterized by cessation of bronchiolar maturation with an overgrowth of mesenchymal elements and proliferation of polypoid glandular tissue in the absence of normal alveolar differentiation. In 80% to 95% of cases, C.C.A.M. of the lung is confinded to a single lobe and there is no lobe predilection and right and left predominance. The clinical presentation may be widely variable, ranging from intrauterine fetal death secondary to nonimmune hydrops fetalis to discovery in childhood after recurrent pulmonary infections. Definitive treatment is removal of affected lobe. In rare instances, there is cases of myxosarcoma, embryonal rhabdomyosarcoma. Bronchoalveolar carcinoma arising in C.C.A.M.. So, resection is recommended even if they are asymtomatic. We have experienced a case of congenital cystic adenomatoid malformation (type II) of the lung in 15 year old man with pneumonia. After the improvement of pneumonia, surgical operation was performed. A brief review of the related literature is presented.

O sinal dos -dentes flutuantes- / The floating teeth sign

Foram estudadas retrospectivamente as radiografias de oito pacientes com sinal do "dente flutuante" e encontrados os diagnósticos de ameloblastoma, granuloma eosinófilo, mixossarcoma, osteossarcoma, displasia fibrosa e cisto dentígeno (um caso cada) e dois casos de osteomielite. Säo revistas as principais causas deste sinal radiográfico