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Neoplasms of locomotive system Jongkolnee Settakorn, MD ภาพที่ใช้ประกอบการบรรยายนี้ ส่วนใหญ่เป็นภาพ ที่มาจาก internet และตำราภาษาอังกฤษ.

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Presentation on theme: "Neoplasms of locomotive system Jongkolnee Settakorn, MD ภาพที่ใช้ประกอบการบรรยายนี้ ส่วนใหญ่เป็นภาพ ที่มาจาก internet และตำราภาษาอังกฤษ."— Presentation transcript:

1 Neoplasms of locomotive system Jongkolnee Settakorn, MD ภาพที่ใช้ประกอบการบรรยายนี้ ส่วนใหญ่เป็นภาพ ที่มาจาก internet และตำราภาษาอังกฤษ

2 วัตถุประสงค์เชิงพฤติกรรม เมื่อสิ้นสุดการเรียนการสอน นักศึกษา สามารถ ระบุเซลล์ต้นกำเนิดของเนื้องอกโดย สังเกตจากชื่อเนื้องอก พยากรณ์พฤติกรรมของเนื้องอกโดย สังเกตจากชื่อของเนื้องอก บอกปัจจัยที่ใช้ในการพยากรณ์โรคของ เนื้องอกกลุ่มนี้ อธิบายคร่าวๆเกี่ยวกับระบาดวิทยา อาการ ทางคลินิก ลักษณะทางรังสีวิทยา ลักษณะทางพยาธิวิทยา และ การรักษา เนื้องอกที่พบบ่อย

3 Scope General consideration Classification Prognostic factors Soft tissue tumors Bone tumors Joint tumors ภาพที่ใช้ประกอบการบรรยายนี้ ส่วนใหญ่ เป็นภาพที่มาจาก internet และตำรา ภาษาอังกฤษ

4 General consideration Rare tumors of more than 100 subtypes

5 Incidence of soft tissue tumors: –Benign: 3000/million –Malignant: 30/million Incidence of bone tumors: –Malignant: 8-20/million Common sites of soft tissue sarcoma: –Extremities (75%) –Trunk wall (10%) –Retroperitoneum (10%)

6 Pattern of tumors: age, gender, site –Lipoma Painless, rare in hand-lower leg-foot Uncommon in children –Vascular tumors >50% occurs in patients younger than 20 years –Benign soft tissue tumors 99% are superficial 95% are < 5 cm in diameter

7 Classification Cells of origin, e.g., –Bone forming cells –Cartilage forming cell –Muscle cell Behavior –Benign –Intermediate –Malignant

8 Classification of soft tissue tumors 1.Fibrous tumors 2.Fibrohistiocytic tumors 3.Lipomatous tumors 4.Smooth muscle tumors and related lesions 5.Extragastrointestinal stromal tumors 6.Skeletal muscle tumors 7.Tumors of blood and lymph vessels 8.Perivascular tumors

9 9.Synovial tumors 10.Mesothelial tumors 11.Peripheral nerve sheath tumors and related lesions 12.Primitive neuroectodermal tumors and related lesions 13.Paraganglionic tumors 14.Extraskeletal osseous and cartilagenous tumors 15.Miscellaneous tumors

10 Classification of bone tumors 1.Cartilagenous tumors 2.Osteogenic tumors 3.Fibrogenic tumors 4.Fibrous histiocytic tumors 5.Ewing sarcoma and primitive neuroectodermal tumors 6.Hematopoietic tumors 7.Giant cell tumors

11 8.Notochordal tumors 9.Vascular tumors 10.Smooth muscle tumors 11.Lipogenic tumors 12.Neural tumors 13.Miscellaneous tumors 14.Miscellaneous lesions 15.Joint lesion

12 Benign tumor Well circumscribed border Cured by complete local excision Do not recur locally –If recur, recur in a non-destructive fashion Do not metastasize Examples: –Lipoma –Benign fibrous histiocytoma –Enchondroma

13 Intermediate tumor –Associated with an infiltrative or locally destructive growth pattern –Recur locally Locally aggressive tumor –Do not metastasize –Example: Desmoid fibromatosis Rarely metastasizing tumor –Have ability to metastasize but <2% risk –Example: Angiomatoid fibrous histiocytoma

14 Malignant tumor Local destructive growth Recurrence Significant risk of distant metastasis Examples: –Osteosarcoma –Ewing sarcoma

15 Nomenclature Benign: -oma or benign Malignant: -sarcoma or malignant Fibroblast: fibro- Fibroblast and histiocyte: Fibrous histiocyto- Fat: Lipo- Muscle: -myo- Smooth muscle: Leiomyo- Skeletal muscle: Rhabdomyo-

16 Blood vessel, endothelial cell: hemangio- or angio Lymph vessel: lymphangio-, or angio Synoviocyte: tenosynovial Nerve fiber: neuri-, neuro-, schwanno- Bone forming cell, osteoblast: osteo- or ossificans Cartilage forming cell: chondro- Myxoid: myxo- or myxoid-

17 Poor prognostic factors AgeYoung Tumor sizeLarge Tumor site (location)Vital organs Depth of involvementDeep Lymph node metastasisPositive Distant metastasisPositive Neurovascular involvementPositive Clinical stagingAdvanced Histologic gradeHigh

18 High histologic grade Tumor differentiationPoorly differentiation Mitotic count (mitosis)>20/10 HPF Tumor cell necrosis>50% CellularityHigh Nuclear pleomorphismPositive Tumor emboliPositive

19 Normal fat Poorly differentiated liposarcoma Well differentiated liposarcoma

20 Mitoses

21 Tumor necrosis

22 Low cellularityHigh cellularity

23 Nuclear pleomorphism

24 Tumor emboli

25

26

27 Tumor staging

28

29

30 Soft tissue tumor 1. Fat 1.1. Lipoma (benign) –Mature adipocytes –20-40 years old –A painless mass –Subcutaneous or deep soft tissue, 5% multiple –Gross and microscopic features: Well circumscribed mass of fat Lobules of mature adipocytes –Treatment: Excision

31 Prognosis –Subcutaneous lipoma: cure –Deep (intramuscular lipoma): risk of local recurrence

32 1.2. Liposarcoma (malignant) –Common sarcoma of adulthood (40-70 years) –A large mass, pain –Deep soft tissue of proximal extremities and retroperitoneum –Subtypes Well differentiated LS  dedifferentiated LS Myxoid LS  round cell LS Pleomorphic LS

33 –Gross and microscopic features: Well circumscribed or infiltrative, fat cut surface (WDLS), gelatinous surface (MXLS), solid grey white surface with hemorrhage and necrosis (PMLS) Lipoblast –Treatment: Excision, radiation, chemotherapy –Prognosis: Depend on site, tumor extension, complete excision?, histologic grade, multifocal? Local recurrence, metastasis

34 Lipoblasts

35 WDLS

36 MXLS

37 RCLSPMLS

38 2. Fibrous tumors and tumor-like lesions 2.1. Reactive pseudosarcomatous proliferation Nodular fasciitis (benign) Myositis ossificans (benign) Palmar, plantar and penile fibromatosis (benign) Desmoid (aggressive fibromatosis) (intermediate)

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40 2.2. Fibroma (benign) 2.3. Fibrosarcoma (malignant) 2.4. Fibrous histiocytic tumor Benign fibrous histocytoma Malignant fibrous histiocytoma FibrosarcomaMFH

41 3. Tumors of skeletal muscle 3.1. Rhabdomyoma (benign) 3.2. Rhabdomyosarcoma (malignant) –Most common malignant soft tissue tumor in children and teenage –Rhabdomyoblast –Subtypes: embryomal, alveolar, pleomorphic

42 4. Tumors of smooth muscle 4.1. Leiomyoma (benign) –Uterus 4.2. Leiomyosarcoma (malignant)

43 5. Tumors of peripheral nerve 5.1. Schwannoma (neurilemmoma) (benign) –Large nerve –Antoni A (cellular area, verocay body) –Antoni B (acellular area) 5.2. Neurofibroma (benign or intermediate) –Small and large nerve 5.3. Malignant peripheral nerve sheath tumor

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45 6. Tumors of vessel 6.1. Hemangioma (benign) Capillary hemangioma Cavernous hemangioma 6.2. Glomus tumor (benign) 6.3. Lymphangioma (benign) 6.4. Angiosarcoma (malignant) 6.5. Kaposi’s sarcoma (malignant)

46 LymphangiomaAngiosarcoma

47 7. Synovial sarcoma –Mass around joint –20-50 years –Histology: biphasic pattern of stroma and epithelial components

48 1. Cystic lesions in bone 1.1. Solitary bone cyst –Simple bone cyst –Long bone 1.2. Aneurysmal bone cyst –Long bone –Blood sponge Bone tumor

49 2. Bone forming tumors 2.1. Osteoma (benign) –Cortex of skull and facial bone –Mass of normal bone 2.2. Osteoid osteoma (<2 cm) (benign) 2.3. Osteoblastoma (>2 cm) (benign) –Long bone –Pain (osteoid osteoma) –Mass of osteoblasts, osteoid, trabeculae of woven bone

50 Osteoid osteoma

51 2.4. Osteosarcoma (malignant) –A primary intramedullary high grade malignant tumor in which the neoplastic cells produce osteoid. –The most common, non-hematopoietic, primary malignant tumor of bone –Incidence: 4-5 / million –Disease of the young: second decade of life –Male: female = 3:2 –Site: metaphysis of long bone, especially around the knee

52 –Clinical features: severe deep pain, mass, fracture –Radiologic features: X-ray: lytic or blastic or mixed lesion with cortical destruction, soft tissue extension and periosteal reaction (Codman’s triangle) CT and MRI –Etiology: unknown, genetic susceptibility P53 loss of function mutation MDM2 over expression Germ-line mutation in RB1 gene (hereditary retinoblastoma)

53 Periosteal reaction: Codman triangle

54 –Gross and microscopic features: Intramedullay mass at metaphysis of long bone Soft tissue extension Bone or cartilage formation Areas of hemorrhage and necrosis Pleomorphic malignant cells produce osteoid. –Treatment: surgery + chemotherapy –Prognosis: 60-70% disease free survival

55 A 5 year old girl with a painful mass at left leg for 4 month Knee disarticulation and chemotherapy

56

57 3. Cartilage forming tumors 3.1. Osteochondroma (benign) –A cartilage capped bony projection arising on the external surface of bone, containing marrow cavity that is continuous with that of the underlying bone

58 –The most common bone tumor (35% of benign, 8% of all) –Age <30 years –Multiple OC: 15% –Etiology: growth plate arrest, EXT1 gene –Site: metaphysis-diaphysis of long bone (distal femur, proximal humerus, proximal tibia and fibula) –Clinical features: most asymptomatic, mass

59 –Radiologic features: X-ray = pedunculated or sessile mass (a projection of the cortex in continuity with the underlying bone) –Gross: mushroom shape bone mass with cartilagenous cap –Histology: 3 layers (perichondrium, cartilagenous cap, and bone) –Treatment: excision –Prognosis: Cure, rare recurrence, rare malignant change (chondrosarcoma) in case with thick cap

60 A 17 years old man with mass on right thigh Excision

61 3.2. Chondroma, enchondroma (benign) –Diaphysis of long bone (finger) –Mass of cartilage 3.3. Chondroblastoma (intermediate) –Mass of chondroblast 3.4. Chondromyxoid fibroma (benign) –Mass of chondroid, myxoid, and fibroblast

62 ChondroblastomaChodromyxoid fibroma

63 3.5. Chondrosarcoma (malignant) –Axial bone –30-60 years –Malignant cells produce cartilage

64 4. Fibrous and fibro-osseous tumors 4.1. Fibrous cortical defect and non-ossifying fibroma (benign) –Cortex –Mass of fibroblast 4.2. Fibrous dysplasia (benign) –Medullar of long bone –Fibroblasts and trabeculae of woven bone 4.3. Fibrosarcoma and malignant fibrous histiocytoma (malignant)

65 Non-ossifying fibroma

66 Fibrous dysplasia

67 5. Miscellaneous tumors 5.1. Ewing sarcoma (malignant) –Malignant small round cell tumor –Diaphysis of long bone –<15 years

68 5.2. Giant cell tumor (intermediate) –Epiphysis of long bone (around knee) –X-ray: soap-bubble appearance –Huge multinucleated giant cells

69 6. Metastatic tumor –The most common malignant tumor affecting the skeleton –2/3 of the patients age between years –Primary sites: breast, lung, prostate, kidney, thyroid, bowel, stomach, cervix –Site ~ location of the primary tumor and blood flow Vertebrae (lumbar spine), proximal femur, rib, sternum, pelvis, skull, shoulder girdle Axial bone (45%), appendicular bone (30%), multiple bone (25%)

70 –Clinical features: pain, swelling, fracture, neurologic symptom –Radiologic features: X-ray: lytic lesion (thyroid, kidney), blastic lesion (prostate), or mixed (breast, lung) Bone scan: very sensitive CT, MRI –Histology: as primary tumor Adenocarcinoma > squamous cell carcinoma –Prognosis and treatment: poor, palliative

71 A 70 years old man with left shoulder pain An underlying history of colonic cancer (adenocarcinoma) Treatment: curettage, cementation, and internal fixation with T-plate

72 Metastatic adenocarcinoma

73 Joint Solid joint Cavitated joint –Articular cartilage –Synovial membrane (synoviocytes) –Synovial fluid –Fibrous capsule, ligament, muscle

74 1. Ganglion or ganglion cyst A small cyst (1-1.5 cm) near a joint capsule or tendon sheath, especially near the wrist joint (dorsal aspect) Most common mass in hand Adult (20-40 years) Subside in most cases Etiology: cystic or myxoid degeneration of connective tissue Symptom: freely moveable mass, local pain or weakness Sign: more prominent with wrist flexed

75 Gross and microscopic features: –Firm, fluctuant, translucent nodule –Stalk attaches cyst to tendon sheath or joint –No communication with joint space –Cyst contains mucinous material –The cyst wall lacks a true cell lining

76

77 Ganglion cyst

78 Management –Aspiration Compression dressing for hours Splinting wrist for several days –Surgical excision if symptom persist despite aspiration Course –Spontaneous resolution (53%) –Recurrence within 2-5 years after treatment After surgical excision (42%) After aspiration (47%)

79 2. Synovial cyst Herniation of synovium through a joint capsule or massive enlargement of a bursa Baker cyst = synovial cyst in popliteal space

80 Clinical features: mass, pain, interfering of movement Ultrasonography, CT, MRI Gross and microscopic features: –Synovial lining cyst (synoviocytes) –Synovial fluid may contain inflammatory cells and fibrin Treatment: –Medication (NSAID), physical therapy –Excision

81 A 40 years old woman with a mass at popliteal fossa Excision

82 3. Tumors of synoviocyte (intermediate) 3.1. Localized: Giant cell tumor of tendon sheath 3.2. Diffuse: Villonodular tenosynovitis

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