myxofibrosarcoma
Introduction
Introduction to mucinous fibrosarcoma Mucinous fibrosarcoma includes a series of malignant fibroblastic lesions with varying degrees of mucin-like stroma, pleomorphism, and unique curvilinear vessels. Common in the elderly, males are slightly more common, most tumors are located in the limbs, about 2/3 of the cases occur in the dermis/subcutaneous tissue, and the rest are located in the lower fascia and skeletal muscle. Most of the manifestations are slow-growing painless masses with a local recurrence rate of 50% to 60%, often with multiple recurrences, and are not associated with histological grade. In addition to lung and bone metastases, a small number of lymph node metastases occur. The overall 5-year survival rate is 60% to 70%. basic knowledge The proportion of illness: 0.02% - 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: fibrosarcoma
Cause
Cause of mucinous fibrosarcoma
Causes:
Primitive mesenchymal tissue, mucinous degeneration of fibrous tissue or mucoid degeneration of tumor tissue.
Pathogenesis:
The lesion is located in the dermis, the boundary is unclear, there is no capsule, the cells are scattered in the mucinous interstitial, the mucous cells are atypical, that is, the nucleus is mostly atypical, and many, some are star-shaped, easy to see mitotic figures, capillaries Rich, signs of differentiation of fibroblasts, fat mother cells or rhabdomyoblasts are found around the lesions. If the mucinous sarcoma undergoes progressive necrosis, there are lipids in the cells.
Prevention
Mucinous fibrosarcoma prevention
The etiology of mucinous fibrosarcoma is still unclear. Understanding the risk factors of tumors and formulating corresponding prevention and treatment strategies can reduce the risk of cancer. Paying attention to regular life helps the body to improve its resistance. The focus of our current cancer prevention and treatment work should first focus on and improve those factors that are closely related to our lives, such as smoking cessation, proper diet, regular exercise and weight loss. Anyone who follows these simple and reasonable lifestyles can reduce their chances of developing cancer.
Complication
Mucinous fibrosarcoma complications Complications fibrosarcoma
The disease is characterized by primitive mesenchymal tissue, mucinous degeneration of fibrous tissue or mucoid degeneration of tumor tissue. It is a low-grade malignant tumor, which may infiltrate the surrounding skin and tissue. If the injured bone tissue can form a fracture, the nerve tissue may be damaged, which may cause abnormal feelings such as muscle weakness, numbness and acupuncture. It is also possible to perform distant metastasis, such as metastasis to the liver to form secondary liver cancer.
Symptom
Mucinous fibrosarcoma symptoms Common symptoms Nodular erythema nodules mucinous cyst
Common in the elderly, males are slightly more common, mainly involving patients aged 50 to 80 years old, patients under 20 years old are very rare. Most tumors are located in the limbs, including the limbs (lower limbs > upper limbs), which are very rare in the trunk, head and neck, hands and feet. It occurs in the retroperitoneal and abdominal cavity and is quite rare. About 2/3 of the cases occur in the dermis/subcutaneous tissue, and the rest are located in the lower fascia and skeletal muscle. Most of the manifestations are slow-growing painless masses with a local recurrence rate of 50% to 60%, often with multiple recurrences, and are not associated with histological grade. In addition to lung and bone metastases, a small number of lymph node metastases occur. The overall 5-year survival rate is 60% to 70%.
Examine
Examination of mucinous fibrosarcoma
Histopathology
The cell density, pleomorphism and value-added activity of mucinous fibrosarcoma vary widely. The morphological features of low-grade malignant tumors are characterized by few cellular components, only a few non-adhesive, obese fusiform or astrocyte cells, cytoplasmic boundaries are unclear, slightly acidophilic, and the nucleus is heterotypic, large and deeply stained. Splits are not common. The characteristic manifestation is the presence of significant elongated, curved, thin-walled blood vessels with dense tumor cells and/or inflammatory cells distributed around the blood vessels, often referred to as pseudo-fatty cells. Highly malignant tumors contain a large number of fusiform and pleomorphic tumor cell solid nests and cell-rich bundles, often with a large number of atypical mitotic, hemorrhagic and necrotic areas. Many tumors have singular multinucleated giant cells with abundant cytoplasm, eosinophilic and nuclear irregularities. However, there are also focal low-grade malignant lesions in highly malignant lesions, with significant mucin-like stroma and a large number of long capillaries. Compared with purely low-grade malignant tumors, moderate malignant tumors are more abundant and more pleomorphic. The edge of the tumor that occurs under the skin is generally markedly invasive.
2. Immunophenotype
Tumen cells are positive for Vimentin. In a few cases, some fusiform or larger eosinophilic tumor cells express specific actin and/or -SMA, suggesting that there is focal fibroblast differentiation in the fossa. The desmin and tissue cell markers (CD68, Mac387, FXIIIa) were negative.
3. Ultrastructure
Most of the cells in the mucinous zone have ultrastructural features of fibroblast differentiation and secretory activity.
4. Genetics
Comparative genomic hybridization (CGH) has been found to have genomic imbalances, often with 6p loss and redundant 9q and 12q.
Diagnosis
Diagnosis and diagnosis of mucinous fibrosarcoma
Before the diagnosis of mucinous sarcoma, other soft tissue sarcomas with significant mucus degeneration should be excluded.
Need to identify with stellate fibrosarcoma, malignant fibrous histiocytoma, extra-chondral chondrosarcoma and liposarcoma.
First, sclerosing fibrosarcoma
Protuberance cutaneous fibrosarcoma is a malignant tumor derived from the dermis. It is generally considered to be invasive in situ, reversible after treatment, but less metastatic. The so-called in-situ invasion refers to the continuous erosion and expansion of the tumor, which not only affects the scope, but also can affect the deep subcutaneous layer. Another feature of dermatofibrosarcoma is the more common in young and middle-aged people. The most common sites are the trunk and the head and face.
Second, malignant fibrous histiocytoma
It occurs mostly in deep soft tissues, first reported by O'Briea and Stoat, and is rarely seen in the bone. In 1972, Feldman and Norman proposed this tumor as an independent type of bone tumor. Previously, intramural MFH was mistaken for osteosarcoma, fibrosarcoma, giant cell tumor of bone or metastatic bone cancer. It is formed by cells that can differentiate into tissue cells and fibroblasts. Variants of this tumor have been found to be: polymorphic stratification, mucinous type, giant cell type, inflammatory type, hemangioma-like type, and the like.
Third, extra-osseous chondrosarcoma
Extracorporeal chondrosarcoma is a chondrosarcoma of soft tissue. Can be divided into mucus type, mesenchymal type and differentiation type.
