Tesis

Luaran Fungsional dan Analisis Kesintasan dari Rabdomiosarkoma Ekstrimitas dan Pelvis di RSUPN Dr Cipto Mangunkusumo, Periode 2005-2016 = Functional Outcome and Survival Analysis of Extremity and Pelvic Rhabdomyosarcoma in Cipto Mangunkusumo National Referral Hospital, 2005 to 2016.

Pendahuluan Rabdomiosarkoma adalah high grade malignancy sekaligus sarkoma jaringan lunak tersering pada anak dan remaja, yang tatalaksananya telah berkembang pesat. Limb salvage surgery (LSS) semakin luas dianut dan dipraktekkan sebagai prioritas pada kontrol lokal rabdomiosarkoma ekstrimitas dan pelvis, oleh karena kemajuan kemoterapi dan radioterapi. Data demografi dan evaluasi tatalaksana itu beserta kaitannya dengan analisis kesintasan belum pernah ada di Indonesia. Metode Desain penelitian ini adalah kohort retrospektif, yang dilakukan di Departemen Orthopaedi, Rumah Sakit Umum Pusat Nasional Cipto Mangunkusumo, Jakarta, Indonesia. Pada Januari 2005-Desember 2016, 43 pasien rabdomiosarkoma ekstrimitas dan pelvis dianalisis secara demografi. Dari jumlah tersebut, 28 pasien menjalani tatalaksana dan dianalisis kesintasannya terkait berbagai faktor. Dari jumlah itu, 15 pasien dibagi menjadi dua kelompok (salvage atau ablasi) dan dianalisis luaran fungsionalnya setelah satu tahun pasca operasi dengan skor MSTS. Hasil Distribusi umur menunjukkan kemaknaan terhadap jenis pleomorfik, yang hanya ditemukan pada dewasa (p=0,035). Luaran fungsional kelompok salvage (rerata = 13,4) secara bermakna (p=0,005) lebih baik dibandingkan ablasi (rerata = 22,23). Insiden metastasis secara bermakna menurunkan kesintasan (p=0,034). Angka kesintasan adalah 3,53% dan median kesintasan adalah 14 bulan. Distribusi umur berupa kurva bimodal dengan insiden dekade awal dibentuk oleh tipe embrional dan diatas 45 tahun oleh tipe plemorfik. Simpulan Fungsi ekstrimitas pasca salvage menunjang fungsi sehari-hari dan tidak terpengaruh morbiditas pasca operasi, serta tidak berpengaruh pada kesintasan juga rekurensi. Karenanya, prosedur itu menjadi prioritas dibanding ablasi. Pengananan awal agresif pada pencegahan metastasis dapat meningkatkan kesintasan. Modalitas kemoterapi multiagen, radioterapi, dan bedah memberikan kecenderungan hasil terbaik.
Kata kunci: rabdomiosarkoma, ablasi, rekurensi, kesintasan, luaran fungsional, salvage


Introduction Rhabdomyosarcoma, classified as high-grade sarcoma, comprises the most common soft tissue sarcoma in children and adolescent, in which the treatment has been advancing. Limb salvage surgery has been acknowledged and performed widely as the priority on local control of pelvic and extremity rhabdomyosarcoma, due to the advancement on radiotherapy and chemotherapy. The established data on patients’ demography and current treatments’ evaluation in Indonesia, are not available yet, especially in the concern of survival. Methods the study design is retrospective cohort, which was performed in Orthopaedics and Traumatology Department, Cipto Mangunkusumo National Primary Referral Hospital, Jakarta, Indonesia. From January 2005 to December 2016, 43 patients, diagnosed as extremity or pelvic rhabdomyosarcoma, were analyzed for demography. 28 patients of them, underwent treatment, and were analyzed for survival analysis. Subsequently, 15 patients of them were divided into two groups (ablation and salvage), and analyzed for one-year postoperative functional outcome in Musculoskeletal Tumour Society score. Result Age distribution has association on pleomorphic type, which is only found on adults (p = 0,035). Functional outcome on salvage group (mean = 22.23) has better outcome (p = 0,005) result ablation group (mean = 13.4). Of all oncologic parameters, metastasis has association with worsening 5-years survival (p = 0,034). The 5-years survival rate is 3.53 % and median survival is 14 months. Age distribution shows bimodal curve on incidence, which comprised from embryonal type on first decade and pleomorphic type after fourth decade. Conclusion The extremity function after salvage procedure reassure daily life and had not influenced by postoperative morbidity, also has no association with survival and recurrence. Therefore, salvage procedure has become priority comparing to ablation. Early aggressive management on metastatic prevention may increase survival. Combination on multiagent chemotherapy, radiotherapy, and wide excision has the most favorable survival.
Keywords: rhabdomyosarcoma, ablation, reccurence, survival, functional outcome, salvage