Erişkinde nadir bir tümör olarak rabdomiyosarkom: Vaka serisi

Öz

Amaç: Çocuklarda daha sık ve daha iyi prognozlu olan rabdomiyosarkom, erişkinlerde çocukların aksine daha nadir ve daha kötü prognozludur. En etkili tedavi yöntemi multimodaliter yaklaşımdır. Bu çalışmamızda 14 hastalık erişkin rabdomiyosarkom hastamızın klinik, patolojik ve sağkalım sonuçlarını paylaşmayı amaçladık. Yöntemler: Çalışmamızda, Türkiye'nin üç tıbbi onkoloji bölümünde 2000 Ocak ve Ocak 2018 tarihleri arasında takip edilen ve tedavi edilen 14 RMS hastası değerlendirildi. Hastaların tamamında, küratif ve palyatif amaçlı cerrahi, kemoterapi ve radyoterapinin kullanılması multimodaliter olarak benimsenmiştir. Bulgular: Tüm hastaların ortanca yaşı 44,5 (dağılım: 16-83) yıl olarak bulundu. Hastalarımızın 10'u (%71,4) erkektir. On dört hastanın 9'unda (%64,3) lokalize, 5'inde (%35,7) metastatik hastalık mevcuttu. Lokalize hastalığı olan 9 hastanın 5 (%55,6) inde nüks gelişti. Hastaların histolojik özelliklerine bakıldığında 10 (%71,4) hasta pleomorfik, 3 (%21,4) alveoler ve 1 (%7,1) hastada pleomorfik rabdomiyosarkom (RMS )vardı. Tüm hastaların ortanca takip süresi 14,6 (dağılım; 2.3-267) aydı. Nükssüz geçen sağkalım (RFS) 15,17 aydı (%95 Güvenlik Aralığı (GA); 1,1-29,2). Metastatik hastalarda birinci basamak tedavi için progresyonsuz sağkalım süresi (PFS) 10,18 (%95 CI; 7,08-13,2) ay olarak saptandı. Hastalardan 9 unda ölüm gerçekleşmişti. Lokal evrede toplam sağkalım (OS) 29,3 ay (%95 GA; 20,8-37,9), metastatik evrede 11,2 ay (%95 GA; 9,29-13,1) ve tüm hasta grubunda 22,8 (%95 GA; 0-47) ay olarak bulundu. Beş yıllık OS %28,2 (Standart hata (SH); %13,4) ve 5 yıllık RFS %41,2 (SH; %17,3) idi. Sonuç: Multimodaliter tedavi yaklaşımı erken ve ileri evre rabdomiyosarkom için en iyi seçenektir. On dört hastalık serimizin klinik ve sağkalım sonuçları literatürle uyumludur.

Anahtar Kelimeler

• Yetişkin Rabdomiyosarkom
• Multimodalite
• Sağkalım
• Klinik ve Patoloji

Rhabdomyosarcoma as a very rare tumor in adult: Case series

Abstract

Aim: Rhabdomyosarcoma is more frequent and has a better prognosis in children. In adults, it is relatively rare and has a worse prognosis. The most effective treatment is achieved with a multimodal approach. We aimed to share the clinical, pathological and survival results of 14 patients with adult rhabdomyosarcoma. Methods: In our study, we evaluated 14 patients with RMS who were followed up and treated between January 2000 and January 2018 in three medical oncology departments in Turkey. The uses of surgery, chemotherapy and radiotherapy for curative and palliative purposes were considered multimodal in all patients. Results: The median age of all patients was 44.5 years (range: 16-83). Ten (71.4%) of our patients were male. The tumors of nine (64.3%) of 14 patients were localized and 5 (35.7%) patients had metastatic disease. Five (55.6%) of 9 patients with localized disease developed relapse. Histological examination of the patients revealed that 10 (71.4%) had pleomorphic, 3 (21.4%) had alveolar and 1 (7.1%) had undifferentiated RMS. The median follow-up period of all patients was 14.6 (range; 2.3-267) months. Relapse-free survival (RFS) was 15.17 months (95% CI; 1.1-29.2). The time to progression of disease after metastatic first-line treatment (PFS) was 10.18 (95% CI; 7.08-13.2) months. At evaluation of the data, 9 patients had died. Median overall survival (OS) at local and metastatic stages were 29.3 months (95% CI; 20.8-37.9) and 11.2 months (95% CI; 9.29-13.1), respectively, while the OS of all participants was 22.8 months (95% CI; 0-47). Five-year OS was 28.2% (Standard error (SE); 13.4%) and 5-year relapse-free survival was 41.2% (SE; 17.3%). Conclusions: The multimodal approach is the best option in early and advanced stage rhabdomyosarcoma. Among our few patient series, clinic and survival results are consistent with the literature.

Keywords

• ,Multimodality,
• Survival
• Adult Rhabdomyosarcoma
• Clinic and pathology

Kaynakça

1. 1. Wibmer C, Leithner A, Zielonke N, Sperl M, Windhager R. Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review. Ann Oncol. 2010 May;21(5):1106-11.
2. 2. Allouche F, Mazti̇ A, Terrab F, Ghammad S, Bouhafa T, El Mazghi̇ A, Hassouni̇ K. Kidney clear cell sarcoma: About one case. J Surg Med. 2018;2(3):380-2.
3. 3. Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A. Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol. 2009 Jul 10;27(20):3391-7.
4. 4. Nagar SP, Mytelka DS, Candrilli SD, Dyachkova Y, Lorenzo M, Kasper B, et al. Treatment patterns and survival among adult patients with advanced soft tissue sarcoma: a retrospective medical record review in the United Kingdom, Spain, Germany, and France. Sarcoma, 2018 May;2018:5467057. 10.1155/2018/5467057
5. 5. Demi̇rli̇ Atıcı S, Yeşi̇lyurt D, Di̇kmeer E, Sali̇moğlu S, Ayaz D, Aydın C. Biphasic synovial sarcoma: A rare cause of axillary mass. J Surg Med. 2019; 3(7): 533-535.
6. 6. Bompas E, Campion L, Italiano A, Le Cesne A, Chevreau C, Isambert N, et al. Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study. Cancer Med. 2018 Aug;7(8) 4023–35.
7. 7. Elsebaie MA, Amgad M, Elkashash A, Elgebaly AS, Shash E, Elsayed Z. Management of low and intermediate risk adult rhabdomyosarcoma: a pooled survival analysis of 553 patients. Sci Rep. 2018 Jun 19;8(1):9337. doi: 10.1038/s41598-018-27556-1.
8. 8. Kojima Y, Hashimoto K, Ando M, Yonemori K, Hirakawa A, Kodaira M, et al. Clinical outcomes of adult and childhood rhabdomyosarcoma treated with vincristine, d-actinomycin, and cyclophosphamide chemotherapy. J Clin Oncol. 2009 Nov 1;27(31):5182–8.

9. 9. Ferrari A, Dileo P, Casanova M, Bertulli R, Meazza C, Gandola L, et al. Rhabdomyosarcoma in adults: a retrospective analysis of 171 patients treated at a single institution. Cancer. 2003 Aug 1;98(3):571-80
10. 10. Schöffski P, Cornillie J, Wozniak A, Li H, Hompes D. Soft tissue sarcoma: an update on systemic treatment options for patients with advanced disease. Oncol Res Treat. 2014;37(6):355-62
11. 11. Garcia del Muro X, de Alava E, Artigas V, Bague S, Brana A, Cubedo R, et al. Spanish Group for Research on Sarcoma. Clinical practice guidelines for the diagnosis and treatment of patients with soft tissue sarcoma by the Spanish group for research in sarcomas (GEIS). Cancer Chemother Pharmacol. 2016 Jan;77(1):133-46.
12. 12. Dumont SN, Araujo DM, Munsell MF, Salganick JA, Dumont AG, Raymond KA, Linassier C, Patel S, Benjamin RS, Trent JC. Management and outcome of 239 adolescent and adult rhabdomyosarcoma patients. Cancer Med. 2013 Aug;2(4):553-63.