MALIGNANT SOFT TISSUE /CONNECTIVE TISSUE TUMORS

1. MALIGNANT SOFT TISSUE /CONNECTIVE TISSUE TUMORS

2. Fibro-sarcoMa

3. Definition • A malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. • This is a type of sarcoma that is predominantly found in the area around bones or in soft tissue such as muscles, connective tissues, blood vessels, joints and fat. • Fibrosarcomas develop from fibroblasts, which produce connective tissue such as collagen.

4. The immature, proliferating fibroblasts take on an interlacing, or herringbone, pattern.

5. ETIOPATHOGENESIS • Genetic mutation • Inherited syndromes – Multiple neurofibromas • From preexisting lesions

6. TYPES Secondary Primary Soft tissue Bone From preexisting lesions/Radiotherapy INFANTILE OR ADULT FIBROSARCOMA

7. Fibrosarcomas of the bone • Fibrosarcoma can also occur in bones because it has an organic element made up of 95% collagen, similar to the collagen found in the skin. • Fibrosarcomas of the bone usually occur in long bones in the bone marrow cavity where collagen is formed. • The bones that predominantly yield fibrosarcomas are those in the legs, arms, pelvis, and hip.

8. Fibrosarcomas of the bone are sometimes connected with underlying benign bone tumors. • As a side effect from previous radiation therapy for primary cancer treatment. • Individuals with other bone diseases, such as Paget's disease and osteomyelitis, are at a higher risk for developing fibrosarcomas.

9. Clinical features • Demographics • between the ages of 25-79. (peak age 55-69 years). • equally in men and women, Site – soft tissue or bone • Though they are rare in children, there is a condition called Infantile fibrosarcoma, which is also known as congenital fibrosarcoma or juvenile fibrosarcoma, is unique. • It is similar to fibrosarcomas seen in adult, but have a more positive prognosis with a post-treatment, five-year survival rate of 83% to 94%.

10. Clinical presentations • FIBROSARCOMA OF BONE • PAIN & SWELLING • PATHOLOGIC FRACTURE • SOFT TISSUE FIBROSARCOMA • PAINLESS MASS (Large painless mass deep to fascia with ill defined margins)

13. RADIOGRAPH

14. H/F • WELL DIFFERENTIATED • INTERMEDIATE • HIGH GRADE

15. WELL DIFFERENTIATED • Multiple plump fibroblasts in a rich collagenous back ground • Normal mitotic figures

17. INTERMEDIATE • Cellular • Herring bone pattern • Slight degree of cellular pleomorphism • Moderate amounts of mature collagen • Areas of hyalinisation

19. HIGH GRADE • Very cellular • Marked cellular atypia • Increased mitotic activity • Higher grades extremely anaplastic & pleomorphic

23. Diagnosis • Patient's medical history • physical exam. • Blood tests to rule out other conditions • x raycan show the location, size, and shape of the tumor. • computed tomography (CT) scans, magnetic resonance imaging (MRI), angiograms, and biopsies.

24. Treatment & Prognosis • Radical excision, associated or not with radiotherapy, is the therapy of choice. • Adjuvant chemotherapy has also been used in high grade lesions • The local recurrence rate ranges between 18 and 79% • Distant metastases occur in 63% of the cases within 5 years after the diagnosis and the lungs and bones are the most common sites. • Currently, the overall 5-year survival rate of the adult fibrosarcoma is 39%.

25. OSTEOSARCOMA

26. Osteosarcoma • is the most common type of malignant bone cancer, accounting for 35% of primary bone malignancies affecting mainly tubular long bones. • It is a malignant connective (soft) tissue tumor of primitive bone forming cell whose neoplastic cells present osteoblastic differentiation and form tumoral bone.

27. ETIOLOGY • Genetic predisposition • Bone dysplasias (Pagets disease,Fibrous dysplasia) • Children with inherited one of the rare syndromes also are at higher risk for osteosarcoma. • 1. retinoblastoma (a malignant tumor that develops in the retina, usually in children younger than age) • 2. Li-Fraumeni syndrome (a kind of inherited genetic mutation). • Exposure to radiation is another trigger for DNA mutations, children who have received radiation treatments for a prior episode of cancer are also at increased risk for osteosarcoma

28. Because osteosarcoma usually develops from osteoblasts (the cells that make growing bone), it most commonly affects teens who are experiencing a growth spurt. • Boys are more likely to have osteosarcoma than girls, and most cases of osteosarcoma involve the knee. • Most osteosarcomas arise from random and unpredictable errors in the DNA of growing bone cells during times of intense bone growth.

29. VARIANTS • CONVENTIONAL - OSTEOBLASTIC - CHONDROBLASTIC - FIBROBLASTIC • MULTIFOCAL • TELANGIECTATIC • SMALL CELL • INTRA OSSEOUS WELL DIFFERENTIATED • INTRACORTICAL • PERIOSTEAL • PARAOSTEAL • HIGH GRADE • EXTRAOSSEOUS

30. C/F • Incidence - 3RD most common cancer • Site – Long bone of extremities (There is a preference for the metaphyseal region of tubular long bones.50% of cases occur around the knee.) • Also involve Skull or Jaw • Gender predilection - M>F • Age - 10-25 yrs ( related to growth spurts)

31. C/F • The most common symptoms are SWELLING & PAIN in a child's leg or arm. • Pain may be worse during exercise or at night, • A lump or swelling may develop in the affected area up to several weeks after the pain starts. • Pain that persistently wakes the child up at night and pain • In osteosarcoma of the leg, the child may also develop an unexplained limp. • In some cases, the first sign of the disease is pathological fracture because the cancer has weakened the bone to make it vulnerable to a break.

32. Oral Manifestations Tooth ache Bleeding Nasal obstruction Mand > Max

34. Diagnosing Osteosarcoma • detailed medical history • a physical exam, • X-rays to detect any changes in bone structure. • magnetic resolution imaging(MRI) scan

35. R/F • SUNRAY PATTERN • WIDENING OF PERIODONTAL SPACE • CODMANS TRIANGLE – Acute angle b/w periosteum and bone surface

36. Types - OSTEOBLASTIC - CHONDROBLASTIC - FIBROBLASTIC

37. H/P • Proliferation of atypical osteoblasts and its precursors. • Tumor Osteoid • Stromal cells spindle shaped, atypical, irregular nuclei • Conventional type classified based on predominant cell

42. Treatment & Prognosis • Surgery • Chemotherapy • osteosarcoma is one of the few that actually begin in bones and sometimes spread (or metastasize) elsewhere, usually to the lungs or other bones.

43. KAPOSI SARCOMA/ ANGIORETICULOENDOTHELIOMA

46. CLINICAL FEATURES • 4 clinical presentation of the lesion: • CLASSIC • ENDEMIC [LYMPHADENOPATHIC] • IMMUNOSUPPRESSION – ASSOCIATED • AIDS RELATED FLAT ,BLUE, RED, PURPLE PLAQUES LATER EXOPHYTIC, ULCERATED LYMPH NODE & SALIVARY GLAND ENLARGEMENT

47. CLASSIC (CHRONIC) • Cutaneous blue red nodule develops on lower extremities , with regressing and newly forming. • Oral lesion appear as bluish nodule of the palatal mucosa.

48. ENDEMIC: • Young African children are affected with local/generalized enlargement of lymph nodes. • Minimal skin & mucosal involvement.

49. TRANSPLANTATION(IMMUNOSUPPRESSION) • Renal transplant patients become manifested 1 or 2 years later the transplantation. • Sarcomatous involvement occurs on the skin as well as internal organs, but oral lesions are rare.

50. AIDS related Kaposi sarcoma • It affects cutaneous and oral part. • Oral lesions appear as red or purple color plaque, either focal or diffuse. • Over a period of time lesion becomes nodular, ulcerated and exophytic. • Salivary gland and lymph nodes are enlarged.