Types of malignant soft tissue sarcomas

Types of malignant soft tissue sarcomas


    • Epidemiology. The most frequently observed type of soft tissue sarcomas (20%). It affects more often women aged 30-40 years
    • Histology. The tumor consists of fibroblast-type cells with different numbers of collagen and reticular fibers.
    • Clinical picture
    • Localization – soft tissues of the limbs (thigh, shoulder girdle), less often the trunk, head, neck
    • The most important sign is the absence of skin lesions over the tumor.
    • Metastases to regional lymph nodes are noted in 5-8%. Hematogenous metastases (most often in the lungs) appear in 15-20%
    • Surgical treatment – wide excision of the tumor
    • Forecast. With adequate treatment, the 5-year survival rate is 77%. Liposarcoma
    • Epidemiology. Liposarcomas are registered in 15% of cases of malignant soft tissue tumors. Occur more often between 40 and 60 years of age
    • Histology. The tumor consists of anaplastic fat cells and areas of myxoid tissue.
    • Clinical picture
    • Most often, the tumor is located on the lower extremities and in the retroperitoneal space.
    • It is extremely rare for liposarcomas to develop

from single and multiple lipomas

    • Typically early hematogenous metastasis to the lungs (30-40%)
    • Surgical treatment – wide excision, with large tumors, preoperative radiation therapy is possible
    • Forecast. In patients with differentiated tumors, the 5-year survival rate is 70%, with poorly differentiated tumors – 20%. Angiosarcoma
    • Epidemiology. The frequency of angiosarcomas is within 12% of all soft tissue sarcomas. The disease is more often observed in young people (up to 40 years old)
    • Histology
    • Hemangioendothelioma is formed from many atypical capillaries with proliferation of atypical endothelial cells filling the lumen of the vessels
    • Hemangiopericytoma, which develops from modified cells of the outer shell of capillaries
    • Clinical picture
    • Tumors have infiltrating rapid growth, are prone to early ulceration and fusion with surrounding tissues.
    • Early metastasis to the lungs and bones, dissemination to the soft tissues of the trunk is quite common
    • Treatment is combined: surgical with pre- or postoperative radiation therapy. Lymphangiosarcoma is a specific tumor that develops in places of permanent lymphatic edema (upper limb in women with postmastectomy syndrome, especially after a course of radiation therapy). The prognosis is unfavorable. There are no effective treatments. Rhabdomyosarcoma (rhabdosarcoma, rhabdomyoblastoma, malignant rhabdomyoma). There are 3 forms: pleomorphic, with a primary lesion of the limbs in adults; alveolar, with damage to the muscles of the limbs, trunk and orbital region; embryonic, observed mainly in young children with lesions of the head and neck, lower urinary tract, pelvis and extremities. The course is traditionally progressive
    • Epidemiology. In terms of frequency, rhabdomyosarcoma ranks third among malignant soft tissue neoplasms. Register at any age, but more often in adolescents and in the middle age group. Women get sick twice as often
    • Histology. The tumor consists of spindle-shaped or rounded cells with longitudinal and transverse striation of the cytoplasm.
    • Clinical picture
    • Most often, tumors are localized in three anatomical regions of the body: limbs, head and neck, small pelvis
    • Tumors grow quickly, without pain and dysfunction of organs
    • Infrequently sprout the skin with the formation of exophytic bleeding tumors
    • Characterized by early recurrence
    • Treatment is surgery; for large tumors, preoperative radiation therapy is advisable
    • During the combined (surgical and chemotherapy) treatment of localized forms of embryonic rhabdomyosarcoma (develops up to 15 years), the 5-year survival rate is 70%. In the presence of metastases, survival is 40%
    • With pleomorphic rhabdomyosarcoma (adult tumor), the 5-year survival rate is 30%. Leiomyosarcoma
    • Epidemiology. The frequency of leiomyosarcomas is 2% of all sarcomas
    • Histology. The tumor consists of various degrees of differentiation of elongated cells with rod-shaped nuclei.
    • Clinical picture
    • Tumors do not often develop in the internal organs (uterus, gastrointestinal tract), localized on the extremities in the projection of the vascular bundle
    • The tumor is always solitary
    • Treatment is surgical.

synovial sarcoma

    • Epidemiology. Synovial sarcoma ranks 3rd-4th in frequency among soft tissue sarcomas (8%). Registered mainly in people under 50 years of age
    • Histology. The tumor is composed of succulent spindle-shaped and rounded cells.
    • Clinical picture
    • Characterized by localization in the area of ​​the hand and foot
    • 25-30% of patients indicate a history of trauma
    • Tumors in 20% of cases give regional metastases, in 50-60% – hematogenous metastases to the lungs
    • Treatment is surgical, with regional lymphadenectomy.

Malignant neuromas

    • Epidemiology. Malignant neuromas are a fairly rare pathology (within 7%)
    • Histology. The tumor consists of elongated cells with elongated nuclei.
    • Clinical picture
    • Most often located on the lower extremities
    • The primary multiplicity of the tumor is characteristic
    • Possible recurrence
    • Treatment – operative
    • Forecast. The main prognostic factors are the degree of histological differentiation and tumor size.
    • The degree of histological differentiation depends on the mitotic index, nuclear polymorphism and other signs of cellular atypism, as well as on the prevalence of necrosis. A less differentiated tumor has a less favorable prognosis
    • Tumor size is an independent prognostic factor. Small (less than 5 cm) completely resected, highly differentiated tumors do not often recur and metastasize. Kaposi’s sarcoma.

See also Tumor, radiotherapy, Tumor, markers; Tumor, methods of treatment; Tumor, stages; Kaposi’s sarcoma ICD

    • C45-C49 Malignant neoplasms of mesothelial and soft tissues
    • C46 Kaposi’s sarcoma
    • C47 Malignant neoplasm of peripheral nerves and autonomic nervous system
    • C49 Malignant neoplasm of other types of connective and soft tissues



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