Sarcomas are tumours which are generally malignant and they originate in connective tissue; they metastasize via the circulatory system. Sarcomas are rare types of neoplasia with a particularly grave prognosis. In the oral and maxilla-facial areas they represent less of 1% of malignant cancers.
- Fibrosarcoma. Fibrosarcoma is a malignant tumour of fibroblasts, occurring in the head/neck area in 10% of cases. It is common in young adults and children and typified by a slowly growing mass which can become enlarged before presenting as symptomatic. Fibrosarcoma is treated surgically with a 50% relapse rate and a survival rate at 5 years of between 40% and 70%.
- Malignant fibrous histiocytoma. A malignant fibrous histiocytoma is a very aggressive sarcoma with double fibroblast/histiocytoma components. It occurs in old age, presenting as a slowly growing mass, which can be ulcerated in a few cases. It can be totally asymptomatic, treatment is by surgical resection and it relapses in 40% of cases.
- Liposarcoma. Liposarcoma is a neoplasia, occurring in fat cells. It is particularly rare in the head-neck region, mainly affecting those between 40 and 60 years of age. It typically presents as a soft, slowly growing mass, which is yellowish in colour. Prognosis varies on the basis of three histo-pathological variants: well differentiated liposarcoma, myxoid/round cell liposarcoma or pleomorphic liposarcoma. The well differentiated variant more typically occurs in the oral cavity with a favourable prognosis. Treatment is with surgery with relapses in 15-20% of cases. Metastasis is rare.
- Malignant peripheral nerve sheath tumour (MPNST). An MPNST is a sarcoma of the peripheral nerves, affecting the head/neck area in 10-15% of cases. It occurs mainly in young adults, often with neurofibromatosis. Common sites in the oral cavity are the jaw, lip and buccal mucosa. Treatment is with surgery with radio- and chemo-therapy adjuvants. Prognosis is very poor.
- Angiosarcoma
- Leiomyosarcoma. Leiomyosarcoma is a malignant neoplasia of smooth muscle tissue. It is common in the uterine wall and gastro-intestinal tract and rare in the oral cavity; it mainly affects adults. Its pathology is non-specific, with growths which increase with the possible reoccurrence of pain. Relapses and metastases are possible; the survival rate at 5 years is 62%.
- Rhabdomyosarcoma (RMS). RMS is a neoplasia, occurring in muscular, skeletal tissue. It is very common in infancy (representing 60% of infant sarcomas) and rare in adults. It often occurs in the maxillo-facial area (35% of cases). The tumour presents as a painless, infiltrating and quickly growing mass. The most frequent intra-oral site is the palate. Treatment is with surgery, followed by post-operative chemotherapy and radiotherapy. The survival rate at 5 years varies between 50% and 60% on the basis of histological variants.
. Angiosarcoma is a rare sarcoma of the vascular endothelium. In more than 50% of cases it presents in the head-neck area. Intra-oral lesions are rare and they are located on the tongue and jaw. Angiosarcoma affects patients in old age with lesions which tend to enlarge, terminating as a nodular or ulcerated surface. Prognosis is generally poor with improved results in cases with intra-oral or salivary gland lesions.
