NEOPLASMA MUSCULOSKELETAL TUMOR JINAK TULANG LUGINDO KEPANITRAAN KLINIK ILMU BEDAH FK UNSWAGATI RSUD WALED CIREBON
PENDAHULUAN
Jaringan tulang & Jaringan lunak ( otot, pembuluh darah, saraf, tendon, lemak )
JINAK
Tumor muskuloskeletal GANAS
0,2 % dari seluruh neoplasma yang diderita manusia 65,8 % Tumor tulang primer jinak 34,2% Tumor tulang primer ganas
ANATOMI & FISIOLOGI
TULANG ORGAN VITAL Matriks tulang : serat-serat kolagen dan protein non-kolagen sel tulang : osteoblas, oisteosit, dan osteoklas
Pembentuk tubuh Menahan dan menegakkan tubuh Gerak pasif Tempat perlekatan otot
Proteksi alat-alat di dalam tubuh Metabolisme kalsium dan mineral
Organ hemopoetik
TUMOR TULANG • Tumor adalah pertumbuhan sel baru, abnormal, progresif, dimana sel-sel tersebut tidak pernah menjadi dewasa.
• Tumor Tulang yaitu pertumbuhan abnormal pada tulang yang bisa jinak atau ganas
ETIOLOGI Penelitin diduga
• Penyebab pasti terjadinya tumor tulang tidak diketahui
• Radiasi sinar radio aktif dosis tinggi. • Keturunan. • Beberapa kondisi tulang yang ada sebelumnya seperti penyakit Paget (akibat pajanan radiasi).
Manifestasi klinis
Pemeriksaan fisik Teraba massa tulang dan peningkatan suhu kulit diatas massa serta adanya pelebaran vena.
Nyeri dan / atau pembengkakan Fraktur patologik Teraba massa tulang
Pembengkakan pada / di atas tulang atau persendian serta gerakan yang terbatas.
pergerakan yang terbatas Gejala-gejala penyakit metastatik
Nyeri tekan atau nyeri lokal pada sisi yang sakit.
PEMERIKSAANA PENUNJANG • Roentgen • CT-Scan • MRI • Skintigrafi (radionuclide bone scan) • PET ( Positron emission tomography)
KLASIFIKASI TUMOR TULANG MENURUT WHO TAHUN 2002
GANAS Tumor asal jaringan tulang JINAK
(Osteogenik):
Tumor asal jaringan tulang (Osteogenik):
Osteosarkoma
Osteoid osteoma Osteoblastoma .
Tumor asal jaringan tulang rawan (Kondrogenik):
Tumor asal jaringan tulang rawan (Kondrogenik): Osteokondroma (Enkondroma,
Kondrosarkoma
TUMOR TULANG JINAK • Sering ditemukan secara kebetulan pada foto Roetgen yang dimaksudkan untuk penyakit lain. • Jarang menimbulkan keluhan.
• Langkah untuk menentukan jenis tumor : 1.Lokasi predileksi 2.Agresivitas tumor dengan menilai tepi tumor dengan tulang dehat. 3.Reaksi tubuh terhadap tumor ( zona transisi) 4.Mineralisasi
OSTEOID • Osteoid Osteoma is a benign osteoblastic OSTEOMA (bone forming) tumor that is usually less than 2cm in size Age: • Osteoid Osteoma is most common in second decade of life • 75%-80% of patients < 25 years • Rarely over 30 years Sites: • Femoral neck most common but can occur in any bone and any site within a bone (metaphyseal, diaphyseal, epiphyseal; cortical, medullary and periosteal) • 50% occur in long bones of lower extremities •Most osteoid osteomas are intracortical in origin but can also occur in the medullary canal or subperiosteal
• Signs/Symptoms: • Progressive pain that is significantly relieved by aspirin or an NSAID (very rarely, less than 1%, may be painless) • Tumors next to growth plates may increase growth and cause skeletal asymmetry • The pain is often the worst an night • Epiphyseal lesions may cause a t effusion and clinical picture similar to rheumatoid arthritis • Vertebral lesions may cause a scoliosis due to muscle spasm • Prevalence: Males more affected than females ~ 3:1
commonly
• Treatment • Today, most osteoid osteomas are amenable to CT guided percutaneous radiofrequency ablation (RF Ablation). • Some patients may require open surgical excision or "Burr Down Resection" of the osteoid osteoma. • Prognosis • This is a benign tumor and there is no risk of metastasis; • RF Ablation is effective over 90% of the time;
OSTEOBLASTOMA • Benign osteoblastic neoplasm with aggressive growth pattern (considered a benign aggressive tumor) • Histologically it is similar to osteoid osteoma but is a larger size and grows progressively Age: • Patients are young, Median age 18 • 80% of patients are between 10 and 30 years old Sites: • Spine (40% of cases; usually posterior elements) • Long Bones (30%; Most arise from diaphysis or metaphysis; Epiphyseal lesions are rare but may occur more often in the tubular bones of the hands or feet)
• Signs/Symptoms: • Pain is the most common presenting symptom • Pain is usually less severe and less pronounced at night and may or may not be relieved by aspirin compared to an osteoid osteoma in which night pain is particularly severe and the pain is usually reliable relieved with an aspirin or NSAID • Spinal lesions may be accompanied by muscle spasms, scoliosis and neurological manifestations • Males: Females 2-3:1
OSTEOCHONDROMA • Osteochondroma is an outgrowth of medullary and cortical bone • Pedunculated (with a stalk) • Sessile (flat without a stalk)
Age: Usually presents clinically by the third decade of life Sites: Appendicular skeleton: Femur (30%) Tibia (20%) Humerus (2-%) Hand and Foot (10%) Pelvis (5%) Scapula (4%) Surface of metaphyseal portions of long tubular bones : Knee area 35% of cases
• Signs/Symptoms: • Hard swelling for many years • Symptoms dependent on location/size • May cause mechanical symptoms from compression of adjacent structures such as tendons, nerve or blood vessels • Malignant Transformation: Solitary osteochondroma <1% • Prevalence: • Male>Female 1.8:1
• Treatment • Simple excision: • Cosmetic reasons • Impingement on tendons, nerves or blood vessels • Pain and limitation of motion • For multiple exostoses, corrective surgery may be necessary due to secondary deformities • Prognosis • Recurrence after excision is rare • Rarely, osteochondromas may give rise to malignant chondrosarcoma • Solitary osteochondromas 1%-2% • Multiple osteochondromas 5%-25%
ENCHONDROMA • Enchondroma is a benign indolent intramedullary hyaline cartilage neoplasm • Limited growth, most lesions are less than 5 cm in maximal dimension
Age: Range: Wide distribution; 5-70 years 60% of enchondromas are discovered in patients between 15 to 40 years of age Sites: 50% involve hands and feet (mostly phalanges) Proximal Humerus, Femur most common long bones Enchondromas of the pelvis, vertebrae and ribs are uncommon
• Signs/Symptoms: • Depends on location • Most long bone enchondromas are asymptomatic and found incidentally • Phalangeal tumors may be painful due to stress fractures • Prevalence: • No clear sex predilection
• Treatment • Enchondromas are benign, indolent (not growing) tumors • Indications for surgery: • Digits: Impending or actual pathological fracture • Intralesional curettage and bone graft or cement
• Long bones: Rare to fracture—usually observe • If grows it is considered chondrosarcoma and would recommend surgery accordingly
• Prognosis • Recurrence rate following curettage is <5% • Recurrence of an enchondroma suggests malignancy
CHONDROBLASTOMA • Benign neoplasm of immature cartilage cell (chondroblast) proliferation • Rare; 1-2% all bone tumors Age: Range 3 - 72 years 95% of cases occur between the ages 5 and 25 Most cases occur in adolescents between 10 and 20 years of age Sites: Predilection for distal femur, proximal tibia & humerus 98% located in epiphysis, 30% in knee area May also occur in calcaneus, talus and temporal bone
• Signs/Symptoms: • Mild Pain lasting from months to several years • 33% of patients have a t effusion and swelling with limitations in range of motion • Often confused with a sports injury • Sex Predilection: Male > Female 1.4:1
• Treatment • Intralesional curettage resection and bone grafting is the most common treatment. • Prognosis • Chondroblastomas are benign aggressive tumors that grow and destroy the bone and t as it grows. • There is a significant risk of local recurrence (up to 30% with an intralesional curettage alone without an additional local adjuvant such as cryosurgery).