Rabu, 18 Agustus 2010

Rhabdomiosarkoma


Rhabdomiosarkoma adalah tumor ganas jaringan lunak.
Dasar diagnosa
Pemeriksaan fisik:
          Gejala umumnya tidak jelas, tergantung dari lokasi tumor. Biasanya adanya massa yang mungkin nyeri pada perabaan, berkembang cepat. Dapat timbul pada daerah leher dan kepala, ekstremitas dan sistim genitourinaria, dapat juga timbul pada orbita, intra toraks, dan retroperitoneum. Sebagian besar Rabdomiosarkoma pada anak berasal dari jaringan embrional .
Pemeriksaan lain :
Tumor dapat dilihat dengan pemeriksaan USG, Scanning, Rontgent, Tergantung dari letak tumor.


Patologi anatomi:
          Ada 4 tipe tumor secara PA. :
1.    Type embrional.
Sel-sel tumor kecil dan berbentuk kumparan.  Beberapa memiliki sitoplasma asidofilik.
2.    Type botrioid.
Sel-sel tumor berbentuk polipoid dan adanya zona tebal dari sel-sel tumor yang tidak berdiferensiasi.
3.    Type alveolar.
4.    Type pleomorfik.
Stdium penyakit:
Stadium I.: penyakit terlokalisir, dan bisa direseksi lengkap.
Substadium IA :      tumor tebatas pada otot atau organ asal.
Substadium IB :      infiltrasi ke luar struktur tanpa melibatkan kelenjar linfe regional.
Stadium II : dapat direseksi menyeluruh.
Substadium IIA :     tumor yang dapat direseksi menyeluruh dengan residual mikroskopik.
Substadium IIB :     tumor regional yang direseksi lengkap dengan keterlibatab kelenjar limfe regional.
Substadium IIC :     tumor regional dan kelenjar limfe yang terlibat direseksi menyeluruh.
Stadium III :           Tumor tidak bisa direseksi lengkap, atau hanya bisa biopsi.
Stadium IV : Tumor metastase jauh pada saat diagnosa.

 

 

 

Pengobatan

1.    Operasi untuk mengangkat tumor.
2.    Radisi dengan dosis 3.000 - 4.000 rad untuk regresi tumor, 5.000-6.000 rad untuk destruksi tumor.
3.    Kemoterapi dengan memakai Daktinomisin, siklofosfamid, vinkristin,doksorubisin ( Adriamisin ). Secara tunggal atau kombinasi.

Terapi harus disesuaikan dengan lokasi dan stadium penyakit.
Stadium  I eksisi lokal dan kemoterapi.
Stadium II dan III pembedahan lengkap, diikuti penyinaran lokal dan kemoterapi sistemik.      
Stadium IV. Pengobatan hanya dengan kemoterapi, sedangkan pembedahan dan radiasi ditujukan hanya untuk penatalaksanaan komplikasi.

PROTOKOL PENGOBATAN RHABDOMYOSARCOMA ( UICC, 1981 p 185 ).
VCR
I


I


I


I


I
ACD
IIIII





IIIII





IIIII
CPA
IIIII





IIIII





IIIII
ADR



I





I



ming.
1
2
3
4
5
6
7
8
9
10
11
12
13
VAC : VCR    1,5 mg/m2 day 1.
          ACD   15 ug/kg/days 1-5.
          CPA    300 mg/m2 days 1-5.
Curse alternated every 3 weeks with :
VAD : VCR    1,5 mg/m2 day 1.
          ADR   60 mg/m2 day 1
                   ( max. cum dose 300-500 mg/m2 )


Sumber: Standar Penatalaksanaan Bagian Ilmu Kesehatan Anak RSMH Palembang

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