Reconstrucción de aparato extensor en sarcoma fibromixoide de bajo grado en rodilla: reporte de caso

Introducción: El sarcoma fibromixoide de bajo grado es una tumoración maligna con alto riesgo de desarrollar recurrencia y metástasis, siendo la resección quirúrgica con márgenes amplios el tratamiento principal, la preservación de la extremidad y su reconstrucción es de alta demanda para los cirujanos. Reporte de caso: Presentamos el caso de una mujer de 67 años con recurrencia de tumoración en rodilla desde hace 12 meses, operado dos años antes. Se realizo resección oncológica de tumoración con reconstrucción de articulación de rodilla con prótesis Endo Model y reconstrucción de aparato extensor con injerto sintético de malla de polipropileno más autoinjerto de semitendinoso y gracilis. La patela se reconstruyó con autoinjerto de cóndilo femoral posterior. A los 16 meses de seguimiento la paciente se encuentra libre de enfermedad, con puntaje de 27 en la escala para miembro inferior de la MSTS (Musculoskeletal Tumour Society). Conclusión: La combinación de injerto sintético con autoinjerto de isquiotibiales puede disminuir la tasa de falla de la reconstrucción del aparato extensor por resecciones oncológicas.

Introduction: Low-grade fibromyxoid sarcoma is a malignant tumor with a high risk of developing recurrence and metastasis, surgical resection with wide margins is the main treatment, limb preservation and reconstruction is in high demand for surgeons. Case of report: We present the case of a 67-year-old woman with a recurrence of a knee tumor that had been operated on two year earlier. Oncological resection of the tumor was performed with reconstruction of the knee joint with an Endo Model prosthesis and extensor mechanism reconstruction with a synthetic polypropylene mesh graft plus a semitendinosus and gracilis autograft. The patella was reconstructed with posterior femoral condyle autograft. At 16 months of patient follow-up, she is free of disease, with a score of 27 on the lower limb scale of the MSTS (Musculoskeletal Tumor Society). Conclusion: The combination of synthetic graft with hamstring autograft can reduce the failure rate of extensor mechanism reconstruction due to oncological resections.

Primary orbital low-grade fibromyxoid sarcoma – A case report

An 18-year-old female presented with rapidly progressive proptosis of the left eye for one month and grade II relative afferent pupillary defect. Orbital imaging showed a well-defined homogenous extraconal mass in close relation to the lateral rectus muscle and extending up to the superior orbital fissure, associated with bony erosion. An incisional biopsy was performed, with the histopathology demonstrating stellate to spindle-shaped tumor cells (fibroblasts) embedded in a richly myxoid matrix. A diagnosis of low-grade fibromyxoid sarcoma (LGFS) was made. The patient was treated by stereotactic external beam radiotherapy. Here, we report a case of LGFS which, to the best of our knowledge, is the first at an orbital location.

Low-Grade Fibromyxoid Sarcoma Arising in Posterior Nasal Cavity: Case Report and Review of the Literature / 대한이비인후과학회지

Low-grade fibromyxoid sarcoma (LGFMS), a soft tissue tumor that has high recurrence and metastasizing potential, rarely occurs in the head and neck region. Therefore, the treatment for LGFMS in the facial area is challenging in terms of cosmetic and functional maintenance. The authors report a case of LGFMS in the posterior nasal cavity. It was completely removed in parallel with the nasal endoscopic and oral approach, but the lower margin was closed by preserving the soft palate. The patient is well without any recurrence or metastasis after 5 years of follow-up.

Mixofibrosarcoma de bajo grado de la pared torácica / A low-grade myxofibrosarcoma of the chest wall

El mixofibrosarcoma es una neoplasia maligna infrecuente, que puede originarse en los tejidos blandos, se lo ha estadificado como de alto o bajo grado y la localización más frecuente son los miembros inferiores. El reconocimiento clínico del mismo es dificultoso, en primer lugar porque la clínica de otros sarcomas de partes blandas es similar y además semejan lipomas o aún quistes. La extirpación quirúrgica amplia es el tratamiento de elección, por la propensión de este sarcoma a la recidiva local. Los catalogados como de alto grado en un 30 % de los casos, pueden originar metástasis a distancia, especialmente a hueso, pulmón y ganglios linfáticos. La histopatología asegura el diagnóstico en la mayoría de los casos. Comunicamos el caso de un mixofibrosarcoma de bajo grado, en un hombre de 43 años, que a los tres años de control evolutivo, luego de la resección quirúrgica, no presenta recaída local ni distante.

Myxofibrosarcoma is an uncommon soft tissue sarcoma that is grading as low or high malignancy. The principal sites of involvement are the lower limbs. Clinical recognition is difficult since it resembles a cyst, a lipoma, other soft tissue sarcoma or even benign conditions as panniculitis. The wide surgical excision is the main therapeutic approach because local recurrence is frequent. The high grade variant is associated in 30 % of the cases with distant metastasis, especially to the lung, bone and lymph nodes. The histopathological features allow an accurate diagnosis in most cases. A 43 year-old man with a myxofibrosarcoma of the chest wall is reported with a follow-up of two years without recurrence.

A Case of Low-grade Fibromyxoid Sarcoma of the Colon / 대한소화기학회지

Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm that shows benign histologic features but may have clinical course of malignant disease. It has been reported to occur in the thigh, inguinal area, axilla, shoulder, neck, perineum or buttock. However, there have been few cases of abdominal organ involvement. A 21-year-old woman presented with a large palpable abdominal mass. A 7x4 cm sized round soft tissue tumor at right upper quadrant area was identified by abdominopelvic CT scan. Percutaneous ultrasound-guided biopsy revealed features of spindle cell tumor. On exploration, the tumor originated from transvers colon and was attached to gastrocolic ligament, transverse mesocolon and stomach. The tumor could be dissected with transverse colectomy and partial gastrectomy. The excised tumor, measuring 7x5x5 cm, was well demarcated and appeared as an ovoid mass with firm and myxoid cut surface. She was diagnosed with low-grade fibromyxoid sarcoma arising from transverse colon, and is currently being followed-up without recurrence or metastasis.

Primary Paravertebral Low-Grade Fibromyxoid Sarcoma

The authors report a 58-year-old woman with low-grade fibromyxoid sarcoma primarily located in the right paravertebral area with extension to L4 neural foramen. The patient complained lower back pain with radiating pain along the posterolateral aspect of the right lower leg. She underwent subtotal surgical removal and Cyber Knife therapy. Diagnosis was made by strikingly characteristic microscopic appearance of a bland spindle cell sarcoma which contained numerous giant collagen rosettes and was also supported by immunohistological findings. The diagnostic image findings and literatures are reviewed and discussed.

Tumor fibromixoide osificante simulando un cáncer anaplásico de tiroides: reporte de un caso / Ossifying fibromyxoid tumor of the neck: case report

El tumor fibromixoide osificante corresponde a una neoplasia infrecuente, de comportamiento benigno pero que presenta recurrencia en un tercio de los casos, comportándose como un sarcoma de bajo grado. Se reporta el caso de una paciente operada de tiroides en dos oportunidades en 9 años, cuyo diagnóstico final fue tumor fibromixoide osificante.

Ossifying fibromyxoid tumor of soft parts in an uncommon tumor with a benign behavior but with a tendency to relapse. We report a 76 years old female presenting with a growing mass located in the anterior portion of the neck that was excised. The pathological study disclosed an anaplastic thyroid cancer. The patient received chemotherapy and was lost from follow up. Nine years later, she presented with progressive dysphagia. A neck CT scan showed a mass in the left thyroid base that completely included the cervical esophagus. The patient was operated and the mass was excised. The pathological diagnosis disclosed an ossifying fibromyxoid tumor.

Surgical Treatment of Ossifying Fibromyxoid Tumor Invading the Lumbar Spine: A Case Report / 대한척추외과학회지

Ossifying fibromyxoid tumor is rare soft tissue neoplasm of an uncertain histogenesis, and this was first described in 1989. The majority of the reported cases have involved the soft tissue of the extremities. We present here on a case of atypical ossifying fibromixoid tumor that had invaded the spine and we report on its management and outcome. We also review the relevant literature.

Low Grade Fibromyxoid Sarcoma in Thigh

A low grade fibromyxoid sarcoma is a rare soft tissue tumor that has a tendency to develop in the deep soft tissue of young adults and the potential for local recurrence or distant metastasis. There have been several case reports and sporadic reports in the literature. However, only 1 case has been reported in Korea but without a follow-up result. We describe a 54-year-old female patient with a low-grade fibromyxoid sarcoma of the thigh that had been growing slowly for 34 years. A marginal resection of this tumor was performed. Currently, the patient is doing well without evidence of local recurrence or distant metastasis at 5 years after surgery.

Cytological Features of Low Grade Fibromyxoid Sarcoma : Report of a Case with a Review of the Literature / 대한세포병리학회지

Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor. There have been only a few prior fine-needle aspiration (FNA) cytological reports. Recognition of this tumor is important because of its potential for metastasis despite its indolent nature and its deceptively bland cytologic appearance. A 60-year-old male presented with a slowly growing mass in the left calf detected 10 years ago. The patient underwent surgical excision. FNA cytology was performed directly on the mass. The smears showed low cellularity composed of hypercellular tissue fragments, hypocellular loose aggregates, and stripped nuclei. The cytoplasm was seen as either collagenous material or very thin fibrillary collagen strands. Tumor cells had spindle, ovoid, or irregular nuclei, fine chromatin, and small nucleoli. Focally slight degree of nuclear pleomorphism is noted. There were no mitotic figures. Blood vessels were frequently seen. Immunocytochemically, tumor cells were negative for S-100 protein, desmin, smooth muscle actin, and CD34. The diagnosis of LGFMS is rarely possible by cytology alone; however, LGFMS should be included in the differential diagnosis of spindle-cell tumors consisting of hypercellular and hypocellular components with some capillary-sized vessels arising in the deep soft tissue of the lower extremities, particularly the thigh. The immunocytochemical findings are of help in the differential diagnosis.

A Case of Ossifying Fibromyxoid Tumor on the Temple Region

Ossifying fibromyxoid tumor of soft parts is a rare soft tissue neoplasm of uncertain histogenesis. It occurs commonly as a subcutaneous or intramuscular mass in the extremities and trunk, but rarely in the head and neck region. Its biologic behavior is generally benign, but a locally aggressive clinical course due to a pseudocapsule containing nests of tumor cells. Herein we report a case of a benign ossifying fibromyxoid tumor which occurred in the right temple in 34-year-old woman. The tumor was located in the temporalis muscle and completely removed with clear margin through bicoronal approach. The lesion was 2.0 x 2.5 x 3.0cm sized white to tan colored, well demarcated, firm mass. The tumor was covered by a fibrous capsule, and there was incomplete shell of lamellar bone at the periphery of the nodule. The tumor cells were uniformly round, ovoid, or spindle-shaped in collagenous and myxoid stroma. During 1 year's postoperative follow-up, the patient had no local recurrence.

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.