Dasar diagnosa
Anamnesa :
Pada tahap dini timbul gejala cat's eye dengan bintik hitam mata menjadi putih dan bila terkena sinar mengkilat seperti mata kucing. Sering kali penderita datang dengan stadium yang sudah lanjut dalam bentuk bola mata membengkak atau menonjol, kadang menjadi juling. Dapat adanya benjolan pada kelenjar limfe leher, sakit kepala, pusing dan nyeri pada tulang.
Pemeriksaan :
Pada mata dijumpai adanya proptosis, leokoria unilateral atau bilateral.
Pada leher dapat dijumpai adanya pembesaran kelenjar limfe preaurikuler.
Laboratorium :
Kadar VMA/HMA biasanya meningkat. BMP dicari apakah adanya sel-sel ganas metastase ke sumsum tulang.
Radiologi:
Untuk mencari komplikasi dilakukan foto torak, dinilai ada/tidaknya destruksi atau klasifikasi. “ bone survey “ apakah terjadi osteolisis tulang.
Pengobatan
Penatalaksanaan Retinoblastoma meliputi operasi (enukleasi), radioterapi, dan kemoterapi.
1. Operatif /exenteratio orbita, dipertimbangkan apabila:
· Tumor meliputi > 50% bola mata
· Dicurigai keterlibatan rongga orbita atau saraf optikus
· Terdapat keterlibatan segmen anterior, dengan atau tanpa glaukoma neovaskular
2. Radioterapi :
Retinoblastoma termasuk jenis tumor yang respon terhadap radioterapi
· Stadium dini : dosis tiap hari : 150 - 200 rad (total dosis < 2 tahun : 3500 rad; total dosis > 2 tahun : 4000 rad)
· Paska operatif : pelaksanaan segera bila keadaan umum baik
· Syarat radioterapi : Hb >8 g%, leukosit >3000/mm3, trombosit >80.000/mm3
3. Sitostatika :
· Cyclopospamide 300 mg/m2 lpt/minggu I.V. selama 3 minggu, dilanjutkan oral 250 mg/m2lpt selama 5 hari berturut-turut dimulai hari 1-5.
· Methotrexate 20-25 mg/m2 lpt/minggu dimulai hari kedua.
· Vincristin 2-2,5 mg/m2 lpt/minggu, dimulai hari pertama, minimal 6 minggu.
· Prednison dapat dipertimbangkan pemberiannya dengan dosis 40-50 mg/m2 lpt/hari peroral hari 1-4.
Pemeriksaan darah tepi dilakukan tiap minggu untuk memonitor gejala toksik efek pemberian sitostatika.
PROTOKOL PENGOBATAN RETINOBLASTOMA
CPA, 150 mg/m2 po. | IIIII | IIIII | IIIII | ||||||||||
Cisplatin 100mg/m2 IV | I | I | I | I | |||||||||
VCR 1,5 mg/m2 | I | I | I | I | I | I | I | ||||||
Minggu | 0 | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 |
Operasi dilakukan pada minggu ke 8 atau ke 12.
Sumber: Standar Penatalaksanaan Bagian Ilmu Kesehatan Anak RSMH Palembang
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