Pediatric Surgery International

, Volume 22, Issue 2, pp 129–134 | Cite as

Clinical characteristics and surgical treatment of perianal and perineal rhabdomyosarcoma: analysis of Japanese patients and comparison with IRSG reports

  • Kaori Okamura
  • Hideki Yamamoto
  • Yuki Ishimaru
  • Hajime Takayasu
  • Yushi Otani
  • Junko Yamagishi
  • Atsushi Takahashi
  • Hiroyuki Kuwano
  • Kikuo Nagashima
  • Hitoshi Ikeda
Original Article

Abstract

Rhabdomyosarcomas of the perianal and perineal regions are uncommon. This study was performed to clarify the clinical characteristics and guidelines of surgical treatment of patients with perianal and perineal rhabdomyosarcomas younger than 20 years of age. Twenty-nine patients, 26 patients identified in the Japanese literature and three of our own, were analyzed and the results were compared with the data reported from the Intergroup Rhabdomyosarcoma Study Group (IRSG). Female predominance and a twin-peak age distribution in infancy and adolescence were characteristic findings of the Japanese patients that were not observed in the IRSG studies. The demographic differences between the two groups were attributed to the differences in demographics of patients younger than 10 years of age. Of the 29 patients, 17 were categorized into clinical groups III/IV and 21 patients into stages 3/4. Alveolar histology was diagnosed in 18 patients. In patients more than 10 years of age, the female predominance was more prominent and the incidences of advanced clinical groups/stages and alveolar histology were significantly higher than those in patients younger than 10 years of age. Inguinal lymph nodes were always involved in patients with lymph node metastases and three patients developed metastases to the breast. Information regarding the survival time was available for 18 patients and the 5-year overall survival was 20%. Two patients with a group I/stage 2 tumor and one with a group II/stage 3 tumor survived for more than 2 years with no evidence of the disease. In these patients, the tumors were excised by primary surgery or primary reexcision and they were not accompanied by lymph node metastasis. Based on these data, complete tumor resection prior to chemotherapy should be pursued and the inguinal lymph nodes should be at least sampled because nodal involvement is closely associated with the patient’s prognosis.

Keywords

Perianal Perineal rhabdomyosarcoma 

References

  1. 1.
    Crist W, Gehan EA, Ragab AH et al (1995) The third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 13:610–630PubMedGoogle Scholar
  2. 2.
    Newton WA Jr, Gehan EA, Webber BL et al (1995) Classification of rhabdomyosarcomas and related sarcomas: pathologic aspects and proposal for a new classification—an Intergroup Rhabdomyosarcoma Study. Cancer 76:1073–1085PubMedCrossRefGoogle Scholar
  3. 3.
    La Quaglia MP, Heller G, Ghavimi F et al (1994) The effect of age at diagnosis on outcome in rhabdomyosarcoma. Cancer 73:109–117PubMedCrossRefGoogle Scholar
  4. 4.
    Sorensen PHB, Lynch JC, Qualman SJ et al (2002) PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the Children’s Oncology Group. J Clin Oncol 20:2672–2679PubMedCrossRefGoogle Scholar
  5. 5.
    Baker KS, Anderson JR, Link MP et al (2000) Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol 18:2427–2434PubMedGoogle Scholar
  6. 6.
    Crist WM, Anderson JR, Meza JL et al (2001) Intergroup Rhabdomyosarcoma Study-IV: results for patients with nonmetastatic disease. J Clin Oncol 19:3091–3102PubMedGoogle Scholar
  7. 7.
    Hays DM, Lawrence W Jr, Wharam M et al (1989) Primary reexcision for patients with ‘microscopic residual’ tumor following initial excision of sarcomas of trunk and extremity sites. J Pediatr Surg 24:5–10PubMedCrossRefGoogle Scholar
  8. 8.
    Neville HL, Andrassy RJ, Lobe TE et al (2000) Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991–1997). J Pediatr Surg 35:317–321PubMedCrossRefGoogle Scholar
  9. 9.
    Andrassy RJ, Wiener ES, Raney RB et al (1999) Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group. J Pediatr Surg 34:731–735PubMedCrossRefGoogle Scholar
  10. 10.
    Corpron CA, Andrassy RJ, Hays DM et al (1995) Conservative management of uterine pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III and IV pilot. J Pediatr Surg 30:942–944PubMedCrossRefGoogle Scholar
  11. 11.
    Breneman JC, Wiener ES (2000) Issues in the local control of rhabdomyosarcoma. Med Pediatr Oncol 35:104–109PubMedCrossRefGoogle Scholar
  12. 12.
    Raney RB Jr, Crist W, Hays D et al (1990) Soft tissue sarcoma of the perineal region in childhood. Cancer 65:2787–2792PubMedCrossRefGoogle Scholar
  13. 13.
    Blakely ML, Andrassy RJ, Raney RB et al (2003) Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: a report of Intergroup Rhabdomyosarcoma Studies I through IV, 1972 through 1997. J Pediatr Surg 38:347–353PubMedCrossRefGoogle Scholar
  14. 14.
    Yokoyama T, Ishii T (1974) Two cases of childhood rhabdomyosarcoma (titled in Japanese). J Jpn Soc Pediatr Surg 10:683Google Scholar
  15. 15.
    Hasegawa J, Tsujimoto M, Kin M et al (1976) Two cases of rhabdomyosarcoma successfully treated with intraarterial infusion chemotherapy (titled in Japanese). J Jpn Soc Pediatr Surg 12:878–884Google Scholar
  16. 16.
    Oshio T, Saito T, Hirai T et al (1979) A case of rhabdomyosarcoma of the anal region. Jpn J Pediatr Surg 11:725–729Google Scholar
  17. 17.
    Asano M, Ishida J, Miwa M et al (1979) A case of embryonal rhabdomyosarcoma of the perineal region (titled in Japanese). Jpn J Urol 70:472–473Google Scholar
  18. 18.
    Mihara S, Yano H, Matsumoto H et al (1982) Perianal alveolar rhabdomyosarcoma in childhood. Jpn J Pediatr Surg 14:1109–1113Google Scholar
  19. 19.
    Murakami T, Yano Y, Yamamoto R et al (1984) A case of alveolar rhabdomyosarcoma metastasizing to the breast, bone marrow, inguinal lymph nodes, perianal and pelvic regions (titled in Japanese). J Jpn Soc Int Med 73:1074Google Scholar
  20. 20.
    Matsumoto Y, Harada Y (1987) A case of perianal rhabdomyosarcoma with metastasis to the breast (title in Japanese). J Jpn Soc Pediatr Surg 23:655Google Scholar
  21. 21.
    Oishi M, Kuroki N, Kuwagata Y et al (1987) A case of rhabdomyosarcoma of the anal sphincter muscle (titled in Japanese). J Jpn Soc Pediatr Surg 23:1331Google Scholar
  22. 22.
    Hirobe S, Yokoyama J, Hoshino K et al (1988) A case of perianal rhabdomyosarcoma (titled in Japanese). J Jpn Soc Pediatr Surg 24:1532Google Scholar
  23. 23.
    Uchiyama T, Hanaoka H, Endo K et al (1989) Perianal rhabdomyosarcoma in childhood. Jpn J Pediatr Surg 21:695–700Google Scholar
  24. 24.
    Komatsu K, Harada Y (1990) A case of perianal rhabdomyosarcoma (titled in Japanese). J Jpn Soc Pediatr Surg 26:181–182Google Scholar
  25. 25.
    Sotoyanagi H, Anbo J, Sasaki S et al (1991) A case of perianal alveolar rhabdomyosarcoma (titled in Japanese). J Jpn Soc Clin Cytol 30:947Google Scholar
  26. 26.
    Morinaga S, Kawasaki H, Matsushige Y et al (1991) Four cases of perineal rhabdomyosarcoma (titled in Japanese). Jpn J Pediatr Oncol 28:823–824Google Scholar
  27. 27.
    Sakaguchi T, Suita S, Zaizen Y et al (1991) A case of perineal rhabdomyosarcoma (titled in Japanese). Jpn J Pediatr Oncol 28:827Google Scholar
  28. 28.
    Suenobu S, Shukei T, Takahashi K et al (1993) A 15-year-old female patient with perianal rhabdomyosarcoma (titled in Japanese). Jpn J Pediatr Oncol 30:166–168Google Scholar
  29. 29.
    Ishikawa S, Imura K, Yoneda M et al (1993) A case of rhabdomyosarcoma mimicking periproctal abscess (titled in Japanese). Jpn J Pediatr Oncol 30:960Google Scholar
  30. 30.
    Yamada R, Shimizu T, Hatori S et al (1997) A case of perianal rhabdomyosarcoma (titled in Japanese). J Jpn Surg Assoc 58 suppl:563Google Scholar
  31. 31.
    Sumi K, Kitsuke H, Maruyama R et al (1997) Two cases of perianal rhabdomyosarcoma (titled in Japanese). J Jpn Soc Colo-Proctol 50:526Google Scholar
  32. 32.
    Nakano T, Numata M, Onoda N et al (1998) The cytological features of alveolar rhabdomyosarcoma arising from the perianal region in a female juvenile: a case study. J Jpn Soc Clin Cytol 37:61–64Google Scholar
  33. 33.
    Yanase T, Nakamura T, Takahashi H (1998) A case of rhabdomyosarcoma presented with leukemia-like symptoms with massive bone marrow metastasis at the onset. Jpn J Pediatr Hematol 12:369–373Google Scholar
  34. 34.
    Uetake H, Sugihara K, Enomoto M et al (1998) A case of perineal rhabdomyosarcoma. J Jpn Soc Colo-Proctol 51:177–182Google Scholar
  35. 35.
    Sugiyama M, Sakanashi S, Ogawa H et al (2000) Two cases of perianal rhabdomyosarcoma. J Jpn Surg Assoc 61:1842–1847Google Scholar
  36. 36.
    Suzuki Y, Hazama K, Ishikawa K et al (2001) A case report of perianal rhabdomyosarcoma. J Jpn Soc Colo-Proctol 54:403–409Google Scholar

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Kaori Okamura
    • 1
    • 2
  • Hideki Yamamoto
    • 1
    • 2
  • Yuki Ishimaru
    • 1
  • Hajime Takayasu
    • 1
  • Yushi Otani
    • 1
  • Junko Yamagishi
    • 1
  • Atsushi Takahashi
    • 2
  • Hiroyuki Kuwano
    • 2
  • Kikuo Nagashima
    • 3
  • Hitoshi Ikeda
    • 1
  1. 1.Department of Pediatric SurgeryDokkyo University School of Medicine, Koshigaya HospitalKoshigaya, SaitamaJapan
  2. 2.Department of General Surgical ScienceGunma University School of MedicineGunmaJapan
  3. 3.Department of SurgeryFujioka General HospitalGunmaJapan

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