Download - Neuroblastoma
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O L E H :
A I R I Z K I K H A U M A M Y
S U RT I W U L A N K H A R I S M A
P E M B I M B I N G : P R O F. D R . C H A I R U L I S M A E L , D R . , S P B , S P B A ( K )
NEUROBLASTOMA
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PENDAHULUAN
Keganasan tersering ke-3 pada anak
Insidensi 1 : 10.000 kelahiran hidup
Anak laki-laki > anak perempuan (1,2 : 1)
Puncak insidensi : usia 2 tahun
Asal: sel neural crest
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Distribusi
Tumor primer yang tersering di temukan terletak di abdomen
medula adrenal (40%) atau ganglia paraspinal (25%). Sisanya terletak di ganglia paraspinal
mediastinum (15%) ganglia paraspinal pelvis (5%) ganglia simpatis serviks (3%)dan tempat yang lain (12%).
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JARINGAN YANG BERASAL DARI NEURAL CREST
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DIFERENSIASI NEUROBLASTOMA
Jaringan normal
Undifferentiated
Neuroblastoma
Neuroblastoma(Pseudorosettes)
Ganglioneuroma
Tumor
Neuroblast Diferensiasi awal Sel ganglion
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TIPE NEUROBLASTOMA
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Etiologi
Serta kemungkinan terkait dengan abnormalitas pada lengan pendek kromosom 16.
Pada beberapa kasus dapat genetik, adanya pewarisan autosomal dominan
Etiologi neuroblastoma belum diketahui secara pasti
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Patogenesis
Sel- sel kanker yang berasal dari medula adrenal dan sistem saraf simpatik berproliferasi
Menekan jaringan di sekitarnya
Kemudian menginvasi sel sel normal sekitar
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Neuroblastoma mengalami metastasis ke korteks tulang, sumsum tulang, kelenjar getah bening, dan hati, sedangkan penyebaran ke paru atau otak jarang terjadi
Neuroblastoma dapat bermetastase ke kelenjar limfe regional maupun kelenjar limfe terjauh seperti; kelenjar supraklavikula, servikal dan inguinal
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MANIFESTASI KLINIS
Stridor
Dysphagia
A. CERVICAL
Horner’s syndrome ptosis, miosis,
Dyspnea
B. MEDIASTINUM POSTERIOR
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MANIFESTASI KLINIS
Massa abdomen yang asimptomatik
Nyeri perut, distensi abdomen, anoreksia, dan penurunan BB
Kompresi vesica urinaria nyeri suprapubis
C. ABDOMEN
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MANIFESTASI KLINIS
Diare persisten
Febris
Peningkatan tekanan darah, ↑ HR
D. PARANEOPLASTIC SYNDROME
Raddening (flushing pd kulit)
Berkeringat
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MANIFESTASI KLINIS
Rapid eyed movement (opsoclonus)
Twitch – like muscle spasm (myoclonus)
Uncoordinate when standing or walking (ataxia)
OPSOCLONUS-MYOCLONUS
ATAXIA
Dancing eyes,dancing feet
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Blueberry muffin appearance
Racoon eyes
MESTASTASIS
Cortical bone
Bone marrow
Liver
Bengkak, nyeri tekan, anak menolak utk berjalan
Anemia, lemah badan
Periorbital
Hepatomegali
Kulit
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PEMERIKSAAN PENUNJANG
Lab. darah: Hb, Ht, Leukosit, Trombosit, Diff. Count, Elektrolit serum
Blood Nitrogen Urea
Kreatinin
SGOT/SGPT
Katekolamin: Homovanilic acid (HVA), Vanillymandelic acid (VMA)LDH, Feritin serum, Neuron spesific enolase prognostic
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PEMERIKSAAN PENUNJANG
Foto thorax AP/ lateral
USG Hepar
CT Scan
Asprirasi bone marrow
MRI
Biopsi needle, insisi
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CT Scan
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Bone Marrow Aspiration
Rosette formation
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Stadium neuroblastoma menurut children cancer study group staging to neuroblastoma adalah
- Stadium I : tumor masih berada di organ asli belum bermetastase
- Stadium II: tumor menyebar dari organ asli tetapi tidak menyebrang dari garis tengah tubuh atau (metastase ipsilateral)
- Stadium III : terjadi metastase ipsilateral - Stadium IV : tumor menyebar jauh ke tulang,
organ, parenkim jaringan lunak atau kelenjar limfe - Stadium IVs : pasien dengan status stadiumm I
atau II tetapi mempunyai penyebaran jauh satu atau lebih dari organ hati, sumsum tulang dan kulit
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STAGING
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Staging
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2b1
2a 3
4
4S
INTERNATIONAL NEUROBLASTOMA STAGING SYSTEM (INSS)
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PENATALAKSANAAN
Operatif
Kemoterapi: Cyclophospamide, Cisplatin, Vincristine, Doxorubicin, Etoposide, Topotecan
Retinoid therapy
Transplantasi stem cell
Radiasi
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PROGNOSIS
25% anak dengan massa soliter prognosis baik, bila dilakukan tindakan operatif
Metastasis prognosis buruk
Diagnosis neuroblastoma ditegakkan pada usia yang lebih muda mempunyai prognosis yang lebih baik dan sebaliknya diagnosis pada usia yang lebih tua mempunyai prognosis lebih buruk
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TERIMA KASIH