Abstract

BackgroundPilonidal disease classically presents as an abscess or soft tissue swelling which classically occurs in the intergluteal cleft, just above the anus. Risk factors for this disease include obesity, prolonged sitting, and abundance of gluteal hair. Treatment options are extensive but most often include incision and drainage with antibiotic treatment. Presentation of recurrent intergluteal pilonidal disease in a young, active female would then be unusual. We present a case of a 17-year-old female presenting with what appeared to be intergluteal pilonidal disease. Definitive excision with histopathology revealed a diagnosis of extraosseous Ewing sarcoma.Case presentationAn otherwise healthy and active 17-year-old female presented to our Pediatric Oncology clinic with a 2-month history of recurrent painful soft tissue swelling of the intergluteal cleft. At that time, she had been diagnosed with pilonidal abscess and had already undergone three incision and drainage procedures. A definitive excision with pathology was performed within weeks of her initial presentation. Immunohistochemical evaluation confirmed a diagnosis of extraosseous Ewing sarcoma.ConclusionThis unusual case underlies the importance of considering a broad differential when evaluating potential pilonidal abscess in a patient who otherwise has no risk factors. Additionally, definitive excision with pathology is critical in a patient with unusually recurrent disease as this can be crucial in the identification of an alternative, and potentially devastating, diagnosis.

Highlights

  • ConclusionThis unusual case underlies the importance of considering a broad differential when evaluating potential pilonidal abscess in a patient who otherwise has no risk factors

  • Pilonidal disease classically presents as an abscess or soft tissue swelling which classically occurs in the intergluteal cleft, just above the anus

  • This unusual case underlies the importance of considering a broad differential when evaluating potential pilonidal abscess in a patient who otherwise has no risk factors

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Summary

Conclusion

Pilonidal disease describes an inflammatory phenomenon often in the intergluteal cleft causing the formation of small open “pits,” which can become secondarily infected and can cause formation of abscesses and fulminant cellulitis [1]. This case is significant as it is the first described case of extraosseous Ewing sarcoma presenting as an intergluteal cleft mass Though this presentation is quite rare, it is important for surgeons to consider a broad differential when presented with recurrent pilonidal disease, including an unusual presentation of a neoplasm. This is especially critical in cases like ours in which patients with no classic risk factors for pilonidal disease present with an unusual gluteal swelling. This case underlies the importance of definitive excision with histological evaluation in unusual cases; our patient has made a full recovery from her Ewing sarcoma likely because it was identified and treated early in the disease course

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