Perianal Rhabdomyosarcoma presenting post surgery for Hirschsprungs disease: A rare presentation

  • Shailaja Shukla Lady Hardinge Medical College, New Delhi, Delhi University.
  • Manjari Kishore Lady Hardinge Medical College, New Delhi, Delhi University.
  • Sangeeta Pahuja Lady Hardinge Medical College, New Delhi, Delhi University
  • Priya Thomas Lady Hardinge Medical College, New Delhi, Delhi Unversity
Keywords: Cytopathology, Hirschsprung's, perianal, rhabdomyosarcoma

Abstract

Perianal region is an uncommon site for Rhabdomyosarcoma. Early diagnosis is essential in these cases, as 25% of patients present with metastasis at the time of diagnosis. FNAC is simple and useful procedure for making an early diagnosis and helping clinicians for prompt intervention. Previously, a single case had been reported of rhabdomyosarcoma occuring 2 years after a surgery for hamartoma at the same site. Herein, we report a case of perianal rhabdomyosarcoma which occurred 5 years after a pull through procedure done for Hirschsprungs disease and this rare association has not been reported yet in literature.

Author Biographies

Shailaja Shukla, Lady Hardinge Medical College, New Delhi, Delhi University.
Professor, Dept Of Pathology, Lady Hardinge Medical College.New Delhi
Manjari Kishore, Lady Hardinge Medical College, New Delhi, Delhi University.
Senior Resident, Dept of Pathology, Lady Hardinge Medical College, New Delhi
Sangeeta Pahuja, Lady Hardinge Medical College, New Delhi, Delhi University
Associate Professor, Dept. Of Pathology, Lady Hardinge Medical College, New Delhi
Priya Thomas, Lady Hardinge Medical College, New Delhi, Delhi Unversity
Post Graduate Student, Dept Of Pathology, Lady Hardinge Medical College, New Delhi

References

1) Piche N, Patey N, Dal Soglio D, Samson Y, Bouchard S.Perianal rhabdomyosarcoma presenting 21 months after hamartoma excision.Pediatr Surg Int. 2012 Jul;28(7):731-5.

2) Hicks J, Flaitz C. Rhabdomyosarcoma of the head and neck in children. Oral Oncol 2002;38: 450-9.

3) Rubin E, Farber EL, eds. Pathology. Vol 1. Philadelphia:. J.B. Lippincott Company. 1994: 1343-4.

4) Raney RB Jr, Crist W, Hays D, et al. Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1972 through 1984. Cancer 1990;65:2787-92.

5) Sasajima K, Okawa K, Sasamoto Y, et al. Pararectal rhabdomyosarcoma: report of a case. Dis Colon Rectum 1980;23:576-7.

6) Ali R, Ozkalemkas F, Ozan U, et al. Rhabdomyosarcoma of the perianal region presenting as acute leukemia. Ann Hematol 2004;83:729-30.

7) Hill DA, Dehner LP, Gow KW, Pappo AS, Crawford D, Pflaumer SM, Furman WL, Hayes-Jordan AA, McDermott MB.Perianal rhabdomyosarcoma presenting as a perirectal abscess: A report of 11 cases.J Pediatr Surg. 2002 Apr;37(4):576-81.

8) Marnewick J, Hulme-Moir M. Embryonal rhabdomyosarcoma of the rectum: report of a case and possible treatment option. Colorectal Dis 2010;12:e170-1.

9) Blakely ML, Andrassy RJ, Raney RB, Anderson JR, Wiener ES, Rodeberg DA, et al. 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.2003;38(3):347–353.

10) Watanabe Y, Yamaguchi A, Isogai M, Kaneoka Y, Suzuki M, Ando H, et al. Treatment strategies for perianal rhabdomyosarcoma: report of two cases. Surg Today.2004;34(8):719–724.

11) Haie-Meder C, Mazeron R, Martelli H, Oberlin O. [Brachytherapy role in pediatric rhabdomyosarcomas] Cancer Radiother. 2013;17(2):155–158.

12) Martelli H, Haie-Meder C, Branchereau S, Franchi-Abella S, Ghigna MR, Dumas I, et al. Conservative surgery plus brachytherapy treatment for boys with prostate and/or bladder neck rhabdomyosarcoma: a single team experience. J. Pediatr. Surg. 2009;44(1):190–196.
Published
2016-08-04
Section
Case Report

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