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"Pendahuluan: Tumor Wilms juga dikenal sebagai nephroblastoma, pertama kali dijelaskan oleh Thomas F. Rance pada tahun 1,814 adalah salah satu neoplasma ganas padat yang paling umum pada anak-anak. Modalitas terapi untuk tumor Wilms adalah pembedahan, kemoterapi, dan radioterapi. Pembedahan adalah perawatan yang paling penting dan prosedur pembedahan yang dilakukan secara tepat dapat menentukan stadium tumor Wilms dan perencanaan perawatan yang lebih akurat. Manajemen multidisiplin tumor Wilms telah menghasilkan peningkatan yang mencolok dalam kelangsungan hidup lebih dari 85% saat ini dan telah menjadi paradigma untuk terapi kanker yang sukses. Studi ini bertujuan untuk mengevaluasi karakteristik penyakit dan hasil pengobatan pasien tumor Wilms di RSUP Adam Malik selama 5 tahun.Metode: Kami meninjau secara retrospektif pasien Tumor Wilms yang telah menjalani operasi dan terapi lain di rumah sakit rujukan nasional kami dalam interval antara Januari 2013 dan Desember 2018. Fitur demografi, riwayat obstetrik, stadium tumor, hasil histopatologi dan radiografi, terapi dan hasil dievaluasi. Analisis deskriptif menggunakan frekuensi digunakan untuk menggambarkan variabel penelitian.Hasil: Total 50 pasien (54% perempuan) dengan tumor Wilms dengan usia rata-rata 56 bulan diidentifikasi. Frekuensi stadium kasus kami adalah: stadium I (24%), stadium II (12%), stadium III (28%) dan stadium IV (36%). Nefrektomi dilakukan pada 16 pasien (32%). Mayoritas orang tua (72%) memiliki tingkat pendidikan yang rendah. Enam puluh delapan persen orang tua menghadiri perawatan antenatal kurang dari empat kali selama kehamilan. Rerata usia kehamilan dari ibu adalah 24,96 ± 4,370. Rata-rata berat badan lahir dan panjang lahir berada dalam kisaran normal; 2878±415, masing-masing 50,32±3,50. Ditemukan korelasi yang signifikan antara tingkat pendidikan stadium tumor (p=0,002) dengan pelayanan antenatal (p=0,011). Sementara faktor lain seperti usia, jenis kelamin, pengobatan pilihan, usia ibu saat melahirkan, berat badan lahir, dan panjang lahir tidak menunjukkan korelasi yang signifikan.Kesimpulan: Luaran tumor wilms pada penelitian lebih buruk daripada center lain di Asia dan dunia. Studi ini menunjukkan bahwa latarbelakang penddikan dan asuhan antenatal adalah dua faktor yang berhubungan signifikan terhadap staging tumor pada studi kami

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Introduction and Objectives: Wilms tumor also known as nephroblastoma, first described by Thomas F. Rance in 1.814 is one of the most common solid malignant neoplasms in children. Therapy modalities for Wilms tumor are surgery, chemotherapy, and radiotherapy. Surgery is the most important treatment and surgical procedure that is done precisely can determine the staging of Wilms tumor and the treatment planning more accurately. The multidisciplinary management of Wilms tumor has resulted in striking improvement in survival of more than 85% nowadays and has become a paradigm for successful cancer therapy. We try to evaluate outcome treatment for patient with wilms tumor

Method: : We retrospectively reviewed the Wilms Tumor patient who had undergone surgery and other therapy in our national referral hospital in the interval between January 2013 and December 2018. Demographic feature, obstetric history, tumor stage, histopathologic and radiographic result, therapy and outcome were evaluated. Descriptive analysis using frequencies was used to describe the study variables.

Results: Total of 50 patients (54% female) with Wilms tumor with a median age of 56 months were identified. The stage frequencies of our cases were: stage I (24%), stage II (12%), stage III (28%) and stage IV (36%). Nephrectomy was performed in 16 patients (32%). The majority of parents (72%) have low educational level. Sixty-eight percent parent attended antenatal care less than four times during pregnancy. Mean of pregnancy age from the mother is 24,96 ± 4,370. The average birth weight and birth length were within the normal range; 2878±415, 50.32±3.50 respectively. Significant correlations were found between tumor stage educational level (p=0,002), and antenatal care (p=0,011). While other factors such as age, gender, treatment of choice, mother’s age at delivery, birth weight, and birth length failed to show any significant correlations.

Conclusion: Outcome of Wilms tumor in our center were poorer than worldwide Wilms Tumor, even in Asia. Furthermore, our study showed that maternal educational background and antenatal care were the only two factors significantly associated with tumor stage in our study."

"Tujuan: Mengetahui pengaruh mutasi patogenik BRCA1/2 tumor terhadap kesintasan pasien advanced stage-high grade serous epithelial ovarian cancer di RSUPN Dr. Cipto Mangunkusumo, RSUP Persahabatan, dan RS MRCCC Siloam Jakarta.Metode: Sejumlah 68 sampel dari 144 pasien diagnosis high-grade serous epithelial ovarian cancer (HGSOC) stadium FIGO IIB-IV, periode 1 Januari 2015 sampai 31 Maret 2021, di RSUPN Dr. Cipto Mangunkusumo, RSUP Persahabatan, dan RS MRCCC Siloam Jakarta, menjalani pemeriksaan NGS mutasi patogenik BRCA1/2 tumor, dilibatkan dalam penelitian kohort historikal ini. Kami membandingkan karakteristik klinikopatologis pasien, dan hasil luaran kesintasan, setelah pasien menjalani tatalaksana primer, berdasarkan status mutasi patogenik BRCA1/2 tumor. Faktor terkait tatalaksana, yang diperkirakan berpengaruh terhadap hasil luaran kesintasan pasien, juga turut dianalisis dalam penelitian ini.Hasil: Angka kejadian mutasi patogenik BRCA1/2 tumor diketahui sebesar 27,94% (19/68). Antara kelompok mutasi patogenik BRCA1/2 tumor, dengan kelompok tanpa mutasi patogenik, tidak terdapat perbedaan statistik signifikan berdasarkan usia, paritas, indeks massa tubuh (kg/m2), riwayat kanker payudara, stadium FIGO 2014, kadar CA125 serum pre operatif (U/mL), volume cairan ascites intra operatif (mL), lesi residual pasca laparotomi debulking, pemberian neoadjuvant chemotherapy (NACT), pemberian kemoterapi adjuvant. Riwayat kanker keluarga terkait HBOC, merupakan variabel paling berpengaruh terhadap mutasi patogenik BRCA1/2 tumor. Kelompok dengan riwayat kanker keluarga terkait HBOC, berisiko 5,212 kali lebih besar mengalami mutasi patogenik BRCA1/2 tumor, dibandingkan dengan kelompok tanpa riwayat kanker tersebut (RR adjusted 5,212; 95%CI 1,495-18,167; nilai p=0,010).Pada kelompok mutasi patogenik BRCA1/2 tumor, kemungkinan meninggal 86% lebih rendah (RR adjusted 0,149; 95%CI 0,046-0,475; nilai p=0,001), dan median survival yang lebih baik (median 46 bulan; 95%CI 34,009-57,991; nilai p=0,001), apabila dibandingkan dengan kelompok tanpa mutasi patogenik (median 23 bulan; 95%CI 15,657-30,343; nilai p=0,001). Analisis multivariat menunjukkan mutasi patogenik BRCA1/2 tumor merupakan faktor prognostik independen yang baik terhadap hasil luaran kesintasan (RR adjusted 0,149; 95%CI 0,046-0,475; nilai p=0,001).Kesimpulan: Pasien advanced stage-high grade serous epithelial ovarian cancer, dengan mutasi patogenik BRCA1/2 tumor, memiliki kesintasan lebih baik, dibandingkan pasien tanpa mutasi patogenik BRCA1/2 tumor.

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Objective: To evaluate the impact of pathogenic BRCA1/2 tumor mutational status on advanced stage- high grade serous epithelial ovarian cancer survival outcome at RSUPN Dr. Cipto Mangunkusumo, RSUP Persahabatan, and RS MRCCC Siloam Jakarta.

Methods: A total 68 of 144 patients diagnosed with FIGO 2014 stage IIB-IV high grade serous epithelial ovarian cancer (HGSOC) between January 1st, 2015 until March 31st, 2021, at RSUPN Dr. Cipto Mangunkusumo, RSUP Persahabatan, and RS MRCCC Siloam Jakarta, underwent NGS tumor BRCA1/2 gene testing, and were included in this cohort hystorical study. We compared patients clinicopathological characteristics, and survival outcomes after primary treatment, according to pathogenic BRCA1/2 tumor mutational status. Treatment-related factors that might affect patients’ survival outcome were also investigated.

Results: The BRCA1/2 pathogenic tumor mutations prevalence was observed in this study 27.94% (19/68). There were no significant statistical differences in age, parity, body mass index (kg/m2), previous breast cancer history, FIGO 2014 staging, pre-operative serum CA 125 level (U/mL), intra operative ascites volume (mL), post cytoreductive surgery residual lesion, neoadjuvant chemotherapy (NACT), and adjuvant chemotherapy administration, between the pathogenic tumor BRCA1/2 mutation, and no pathogenic tumor BRCA1/2 mutation groups. The hereditary breast ovarian cancer family history (HBOC) variable has the strongest correlation with pathogenic tumor BRCA1/2 mutation. The group with a family history of HBOC-related cancer had a 5.212 times greater risk of developing pathogenic BRCA1/2 tumor mutations, compared with the group without a history of those cancer (RR adjusted 5.212; 95%CI 1.495-18.167; p value=0.010).

The pathogenic BRCA1/2 tumor mutation group displayed better survival outcome. In the pathogenic BRCA1/2 tumor mutation group, the likelihood of dying was 86% lower (RR adjusted 0.149; 95%CI 0.046-0.475; p-value=0.001), and the median survival was better (median 46 months; 95%CI 34.009- 57.991; p value=0.001), than without pathogenic BRCA1/2 tumor mutations group (median 23 months; 95%CI 15.657-30.343; p value=0.001). The multivariate analyses identified pathogenic BRCA1/2 tumor mutation as an independent favorable prognostic factor for survival outcome (RR adjusted 0.149; 95%CI 0.046-0.475; p-value=0.001).

Conclusions: In advanced stage-HGSOC, patients with pathogenic BRCA1/2 tumor mutations have a better prognosis with longer survival outcome than those without pathogenic BRCA1/2 tumor mutations."