Pendahuluan: Rabdomiosarkoma adalah high grade malignancy sekaligus sarkoma jaringan lunak tersering pada anak dan remaja. Limb salvage surgery LSS semakin luas dianut dan dipraktekkan sebagai prioritas 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.

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Introduction 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.

Method: 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.