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Soft tissue sarcoma

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Soft tissue sarcoma

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Soft tissue sarcoma

  1. 1. Lec 9&10 Lec 9&10
  2. 2. Definition Mesenchymal proliferations that occur in the extraskeletal, nonepithelial tissues of the body, excluding the viscera, coverings of the brain, and lymphoreticular system. - Soft tissue benign tumours outnumber malignant Tumours 100:1 -They are aggressive if malignant
  3. 3. Histologic type Benign Malignant Adipose Tissue Lipoma Liposarcoma Fibrous tissue Fibromatosis Nodular fasciitis Fibrosarcoma Fibrohistiocytic tumours Fibrous histiocytoma Dermatofibroma Malignant Fibrous Histiocytoma????? Skeletal Muscle Rhabdomyoma Rhabdomyosarcoma Smooth Muscle Leiomyoma Leiomyosarcoma Vascular Haemangioma Lymphangioma Angiosarcoma Peripheral nerve Neurofibroma Schwannoma Malignant peripheral nerve sheath tumour Uncertain histogenesis Granular cell tumour Synovial Sarcoma Alveolar soft part sarcoma Epithelioid sarcoma
  4. 4. CAUSES • MOSTLY UNKNOWN • RADIATION association • CHEMICAL BURN association • THERMAL BURN association • TRAUMA association • VIRUS association (HHV8 for Kaposi) • GENETICS • Parts of many SYNDROMES • MANY TRANSLOCATIONS
  5. 5. TUMORS OF ADIPOSE TISSUE LIPOMA Lipomas most common soft tissue tumors. Site: anywhere in the body subcutaneous tissues of adults. Clinical: slowly enlarging masses. Most lipomas are solitary , sporadic. Rare familial multiple lipomas.
  6. 6. TUMORS OF ADIPOSE TISSUE LIPOMA cont…. Morphology: Gross: soft, yellow encapsulated mass. Superficial are well circumscribed, deep lesions be less demarcated. Microscopically: composed of mature adipose tissue. Variants: - Vasculare tissue (angiolipoma) give local pain - Smooth muscle (myolipoma) - Bone marrow (myelolipoma of adrenal gland) - Angiomyolipoma, containing a mixture of adipose tissue, smooth muscle, and blood vessels. Occur in kidneys of patients with tuberous sclerosis. Treatment: Complete excision is usually curative.
  7. 7. Lipomas Conventional lipomas are soft, yellow, well- encapsulated masses of mature adipocytes; they can vary considerably in size.
  8. 8. Lipomas Histologically, they consist of mature white fat cells with no pleomorphism.
  9. 9. Liposarcoma • Liposarcomas are malignant neoplasms of adipocytes. • AGE: They occur most commonly in the fifth and sixth decades. • SITE: Most liposarcomas arise in the deep soft tissues or in visceral sites. • The prognosis: • is greatly influenced by the histologic subtype; • well-differentiated types have a more favorable outlook than do the more aggressive poorly differentiated tumors. • Metastasis: metastasize to lungs.
  10. 10. Liposarcoma morphology • Several different histologic subtypes are recognized, • 1- Low-grade variants; • the well-differentiated liposarcoma • the myxoid/round cell liposarcoma, [ abundant, mucoid extracellular matrix]. • Some well-differentiated lesions can be difficult to distinguish histologically from lipomas, • 2- Poorly differentiated tumors;Pleomorphic variant • resemble various other high-grade malignancies. • In most cases, cells indicative of fatty differentiation are present. Such cells are known as lipoblasts; they recapitulate fetal fat cells with cytoplasmic lipid vacuoles that scallop the nucleus
  11. 11. Liposarcoma morphology Large relatively well-circumscribed lesions. soft tissue mass. The mass is uncapsulated, soft, yellow, lobulated,with marked areas of hemorrhage and necrosis.
  12. 12. Liposarcoma morphology
  13. 13. TUMORS AND TUMOR-LIKE LESIONS OF FIBROUS TISSUE • Nodular fascitis, reactive, self-limited lesion. • Fibromatoses, aggressive local growth that may defy surgical excision. • Fibrosarcomas, highly malignant, recur locally, and metastasize.
  14. 14. TUMORS OF FIBROUS TISSUE Nodular Fascitis Nodular fascitis is a self-limited, reactive fibroblastic proliferation mistaken for a sarcoma. Age: young adults. Clinically: rapidly enlarging, painful mass of several weeks duration. 10% to 15% of cases, a history of local trauma. Site: Any part of the body, most common in the upper extremities(Forearm) and trunk. Treatment: excision
  15. 15. TUMORS OF FIBROUS TISSUE Nodular Fascitis Morphology Grossly: unencapsulated lesion in the subcutaneous tissue, muscle, or deep fascia. Superficial locations, well circumscribed, nodular with poorly defined margins . Microscopically, composed of immature fibroblastic cells with bland cytology in myxoid matrix containing lymphocytes and extravasated red blood cells. Mitotic figures present, but not abnormal.
  16. 16. Nodular fasciitis • Characteristically, the lesion is several centimeters in greatest dimension and nodular with poorly defined margins. • Histologically, it is richly cellular and consists of plump, randomly arranged, immature-appearing fibroblasts in an abundant myxoid stroma . • The cells vary in size and shape (spindle to stellate) and have conspicuous nucleoli and numerous mitoses.
  17. 17. TUMORS OF FIBROUS TISSUE FIBROMATOSES Definition: Is a fibroblastic proliferation with aggressive local growth and infiltrative margins. recur after surgical excision and do not metastasize. Two major groups:  Superficial fibromatoses, include palmar fibromatosis (Dupuytren’s contracture) and penile fibromatosis (Peyronie’s disease), occur in superficial fascia and are benign, have abundant collagen and result in deforming contractures in palms or soles.  Deep fibromatoses, called desmoid tumors, arise in the abdomen, muscle of the trunk and extremities, they recur and show local aggressive growth.
  18. 18. TUMORS OF FIBROUS TISSUE FIBROMATOSES cont. MORPHOLOGY Gross: either well-defined nodules, infiltrative masses without margins. Microscopically: composed of proliferating Fibroblasts have uniform appearance. Superficial fibromatoses, contain abundant collagen.
  19. 19. TUMORS OF FIBROUS TISSUE FIBROSARCOMAS Fibrosarcomas are malignant neoplasms composed of malignant fibroblasts. Age: adults Site: deep tissue of the thigh, knee and trunk. They grow slowly many years before diagnosis.
  20. 20. TUMORS OF FIBROUS TISSUE FIBROSARCOMAS MORPHOLOGY Gross: solitary lesions either infiltrative or fairly circumscribed. Histologically: interlacing fascicles of fibroblasts, arrayed in a herringbone pattern. Nuclear atypia and mitotic activity present. Prognosis: recur locally after excision and may metastasize by blood to the lungs.
  21. 21. Fibrosarcoma
  22. 22. Lecture 10
  23. 23. TUMORS OF SMOOTH MUSCLE Leiomyomas are benign smooth muscle tumors, are common, well-circumscribed neoplasms. Site: uterus, anywhere in the body. Leiomyosarcomas are malignant smooth muscle tumors. Site:most often in uterus and gastrointestinal tracts, or any area of the body. Most arise de novo, than in a preexisting leiomyoma.
  24. 24. Uterine Leiomyoma • Characteristic whorled like
  25. 25. Leiomyoma: Section of tumour shows: A well demarcated tumour mass in the muscle coat of uterus without a definite capsule. Tumour consists of interlacing bundles of smooth muscle and fibrous tissue. The muscle cells are spindle shaped with elongated nuclei and eosinophilic cytoplasm.
  26. 26. TUMORS OF SMOOTH MUSCLE LEIOMYOSARCOMA Morphology: Have infiltrative growth, greater cellularity and pleomorphism, greater mitotic activity (mitotic activity used to differentiate benign SMC tumor from malignant).
  27. 27. Cut surface of this leiomyosarcoma showing ill defined pale and soft large fleshy mass with hemorrhage and necrosis.
  28. 28. TUMORS OF SKELETAL MUSCLE Rhabdomyoma Is a benign skeletal muscle tumor. Is an uncommon tumor. The cardiac rhabdomyoma associated with tuberous sclerosis.
  29. 29. TUMOR OF SKELETAL MUSCLE RHABDOMYOSARCOMA Rhabdomyosarcoma is a malignant aggressive skeletal muscle tumor. Age: infancy, childhood, and adolescence The most common soft tissue sarcoma in pediatric age.
  30. 30. TUMORS OF SKELETAL MUSCLE MORPHOLOGY Gross: is variable. Tumor (sarcoma botryoides) arising near the mucosal surfaces of the lower genitourinary tract and in the head and neck. Are soft, gelatinous, grape-like masses, other variant may present as poorly defined mass or as infiltrating mass.
  31. 31. TUMORS OF SKELETAL MUSCLE Microscpically: *Embryonal variants and sarcoma botryoides, are composed of malignant small round cells and eosinophilic large cells with evidence of myoblastic differentiation. They are subclassified into the embryonal, alveolar, and pleomorphic variants.
  32. 32. TUMORS OF SKELETAL MUSCLE Diagnosis: Depend of the demonstration of skeletal muscle differentiation, by the electron microscope (sarcomeres), or immunocytochemical (desmin). DDX: to be distinguished from other small round cell tumors of childhood.
  33. 33. SKELETAL MUSCLE • RHABDOMYOMA • RHABDOMYOSARCOMA
  34. 34. Rhabdomyosarcoma
  35. 35. SYNOVIAL SARCOMA Accounts for 10% of all soft tissue sarcomas. Synovial sarcoma does not arise from synovial cells, rather, it arises form mesenchymal cells about joint cavities or away from the joint especially around the knee and thigh
  36. 36. SYNOVIAL SARCOMA MORPHOLOGY Gross: can be small, circumscribed lesions or infiltrative sarcomatous masses. Histologically: biphasic pattern, epithelial component forming glands, mixed with spindle cells similar to fibroblast.. Less frequent monophasic, either composed of epithelial elements that resemble a carcinoma or, of spindle cells sarcoma.
  37. 37. Assessment –Which of the following statements is TRUE for Liposarcoma? • A. Usually show benign behavior • B. The most common site is subcutaneous tissue • C. Histologically shows mature fat cells arranged in lobules. • D. Grossly, shows invasive growth with hemorrhage and necrosis.
  38. 38. Assessment –The most common soft tissue sarcoma of infancy and childhood is: • A. Rhabdomyosarcoma • B. Liposarcoma • C. Myositis ossificans • D. Malignant fibrous histiocytoma

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