PROFIL KLINIS DAN PROGNOSIS NEUROBLASTOMA ANAK DI RSUP DR. SARDJITO, YOGYAKARTA

ANGGRAENI PUSPITA, dr. Bambang Ardianto, M,Sc., Ph.D., Sp.A ; dr.Eko Purnomo, Ph.D

2017 | Skripsi | S1 PENDIDIKAN DOKTER

Latar Belakang Neuroblastoma salah satu tumor padat yang berasal dari sel krista neuralis primodial yang terdapat disepanjang sistem saraf simpatis retroperitonial atau di dalam kelenjar adrenal. Saat ini neuroblastoma adalah salah satu keganasan yang sering terjadi pada anak - anak dan menduduki urutan ketiga setelah ALL dan tumor otak. Perjalanan penyakit neuroblastoma sangat bervariasi, mulai dari regresi spontan hingga penyebaran secara menyeluruh namun, seringkali tidak bergejala dan lebih dari 70% kasus terdiagnosis sudah metastasis. Angka mortalitas pasien neuroblastoma mencapai 15% dari kasus kanker anak. Beberapa faktor yang mempengaruhi prognosis dari neuroblastoma seperti usia saat terdiagnosis, jenis kelamin, metastasis, diameter tumor, dan terapi. Identifikasi gambaran klinis dan faktor prognosis neuroblastoma diperlukan dalam rangka menurunkan angka mortalitas pasien. Tujuan Mengetahui gambaran klinis dan prognosis pasien neuroblastoma anak di RSUP DR. Sardjito, Yogyakarta. Metode Penelitian retrospektif dengan desain case control dengan mengambil data sekunder dari rekam medis pasien anak dengan neuroblastoma di RSUP DR. Sardjito dari 2011 hingga 2016. Hubungan antar faktor prognosis akan dianalisis dengan fisher's exact test. Dengan metode kaplan-meier dan log rank test dilakukan untuk mengetahui tingkat kelangsungan hidup dari beberapa sub-kelompok pasien dengan neuroblastoma. Hasil Dua puluh pasien neuroblastoma dimasukkan dalam penelitian, dimana terdapat 13 laki-laki dan 7 perempuan. Sebagian besar pasien terdiagnosis saat berusia >18 bulan (90%). Diantara semua pasien neuroblastoma, terdapat 45% pasien mendapat kemoterapi, 15% mendapat multiterapi, 35% tidak mendapatkan terapi dan 5% dilakukan pembedahan. Berdasarkan metastasis, 75% didapatkan metastasis negatif. Diameter dari massa 58,3% <= 10 cm sedangkan 41,7% >10 cm. Dengan menggunakan fisher's exact test tidak didapatkan hubungan antar faktor prognosis seperti jenis kelamin, usia terdiagnosis, metastasis, terapi dan diameter massa (p>0,05) tetapi dengan metode kaplan meier dan log rank test terdapat hubungan antara kelangsungan hidup pasien dengan terapi yang didapatkan (p=0,025).

Background Neuroblastoma is one of solid tumor that derived from crista neuralis primodial along the retroperitoneal nervous system symphatetic or in the adrenal. Neuroblastoma currently one of common maglinancy in children, ranked in third place after ALL and brain tumors. Pathogenesis neuroblastoma starting form spontaneous regression to thorough spreading of lession, but it is usually asymptomatic with more than 70% cases diagnosed after metastasic. Mortality rate patient neuroblastoma reached 15% of pediatric cancer. Several factors affecting prognosis of neuroblastoma such as it ages when diagnosed, sex, metastatic, diameter of tumor, and treatment. Identification manisfestation clinic and prognostic factor of neuroblastoma are required to decrease mortality rate patients. Objective This study aims to identify clinical profile and prognostic children with neuroblastoma in RSUP DR. Sardjito, Yogyakarta. Method This study was a retrospective with case control study using medical records from patients with neuroblastoma in RSUP DR. Sardjito from year 2011 until 2016. Correlation between each prognostic factor were analyzed by fisher's exact test. Kaplan-meier method was applied to measure the survival rate of patients from different subgroup of neuroblastoma. Result Twenty neuroblastoma patients were ascertained in this study, of whom 13 males and 7 females. Most patients were diagnosed at the age >18 months (90%). Among them, they were showing 45% patient received chemotherapy, 15% received multitherapy, 35% untreatened and 5% received surgery. Based on metastatic, 75% defined negative metastatic. As much as 58,3% of patients had lesion with diameter <= 10 cm, whereas 41,7% > of the patients had lesion with diameter 10 cm. There were no significant association found using fisher's exact test between prognostic factor such as it ages when diagnosed, sex, metastatic, diameter of tumor, and treatment (p>0,05). However analysis using kaplan meier method and log rank test showed correlation between survival rate patient and treatment (p=0,025).

Kata Kunci : pediatric neuroblastoma, clinical profile, prognostic factors, mortality.