Article Text

Rare disease
PNET/Ewing’s sarcoma of the rectum: a case report and review of the literature
  1. Omar M Aboumarzouk1,
  2. Robert Coleman2,
  3. John R Goepel3,
  4. Andrew J Shorthouse4
  1. 1
    West Wales General Hospital, Carmarthen, Carmarthenshire SA31 2AF, UK
  2. 2
    Weston Park Hospital, Academic Unit of Clinical Oncology, Sheffield, Yorkshire S10 2SJ, UK
  3. 3
    Royal Hallamshire Hospital, Glossop Road, Sheffield, Yorkshire S10 2JF, UK
  4. 4
    Northern General Hospital, General/Colorectal Surgery, Herries Road, Sheffield, Yorkshire S5 7AU, UK
  1. Andrew J Shorthouse, Shorthouse{at}doctors.org.uk

Summary

A 34-year-old female presented with anorectal pain and rectal bleeding due to an extensive rectal tumour. A trephine loop ileostomy was fashioned and biopsies were initially reported to show a poorly differentiated cloacogenic carcinoma. CT revealed numerous liver metastases. A histological review and immunohistochemical studies subsequently favoured a primitive neuroectodermal tumour (PNET). Stem-cell supported chemoradiotherapy resulted in complete resolution of her primary tumour and liver metastases. Serial CT scanning and endoscopy revealed no recurrence after 7 years of follow-up, when she presented with a malignant anal fissure. Imaging and subsequently abdominoperineal resection revealed no evidence of metastases from either the anal cancer or the PNET tumour. Histopathology showed a T1N0R0 basaloid squamous carcinoma originating from grade III squamous intraepithelial neoplasia with no obvious wart viral infection.

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Footnotes

  • Competing interests: none.

  • Patient consent: Patient/guardian consent was obtained for publication.

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