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Neoplasms of locomotive system

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

2 วัตถุประสงค์เชิงพฤติกรรม
เมื่อสิ้นสุดการเรียนการสอน นักศึกษาสามารถ ระบุเซลล์ต้นกำเนิดของเนื้องอกโดยสังเกตจากชื่อเนื้องอก พยากรณ์พฤติกรรมของเนื้องอกโดยสังเกตจากชื่อของเนื้องอก บอกปัจจัยที่ใช้ในการพยากรณ์โรคของเนื้องอกกลุ่มนี้ อธิบายคร่าวๆเกี่ยวกับระบาดวิทยา อาการทางคลินิก ลักษณะทางรังสีวิทยา ลักษณะทางพยาธิวิทยา และ การรักษา เนื้องอกที่พบบ่อย

3 Scope General consideration Classification Prognostic factors Soft tissue tumors Bone tumors Joint tumors ภาพที่ใช้ประกอบการบรรยายนี้ ส่วนใหญ่เป็นภาพที่มาจาก internet และตำราภาษาอังกฤษ

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., Behavior Bone forming cells
Cartilage forming cell Muscle cell Behavior Benign Intermediate Malignant

8 Classification of soft tissue tumors
Fibrous tumors Fibrohistiocytic tumors Lipomatous tumors Smooth muscle tumors and related lesions Extragastrointestinal stromal tumors Skeletal muscle tumors Tumors of blood and lymph vessels Perivascular tumors

9 Synovial tumors Mesothelial tumors Peripheral nerve sheath tumors and related lesions Primitive neuroectodermal tumors and related lesions Paraganglionic tumors Extraskeletal osseous and cartilagenous tumors Miscellaneous tumors

10 Classification of bone tumors
Cartilagenous tumors Osteogenic tumors Fibrogenic tumors Fibrous histiocytic tumors Ewing sarcoma and primitive neuroectodermal tumors Hematopoietic tumors Giant cell tumors

11 Notochordal tumors Vascular tumors Smooth muscle tumors Lipogenic tumors Neural tumors Miscellaneous tumors Miscellaneous lesions 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 Locally aggressive tumor Rarely metastasizing 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
Age Young Tumor size Large Tumor site (location) Vital organs Depth of involvement Deep Lymph node metastasis Positive Distant metastasis Positive Neurovascular involvement Positive Clinical staging Advanced Histologic grade High

18 High histologic grade Tumor differentiation Poorly differentiation
Mitotic count (mitosis) >20/10 HPF Tumor cell necrosis >50% Cellularity High Nuclear pleomorphism Positive Tumor emboli Positive

19 Normal fat Well differentiated liposarcoma
Poorly differentiated liposarcoma

20 Mitoses

21 Tumor necrosis

22 Low cellularity High 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 RCLS PMLS

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)

39

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

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

44

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 Lymphangioma Angiosarcoma

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

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

49 2.2. Osteoid osteoma (<2 cm) (benign)
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 Chondroblastoma Chodromyxoid 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 5.1. Ewing sarcoma (malignant)
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

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77 Ganglion cyst

78 Surgical excision if symptom persist despite aspiration
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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