Pediatric radical abdominal trachelectomy for cervical clear cell carcinoma: A novel surgical approach Journal Article


Authors: Abu-Rustum, N. R.; Su, W.; Levine, D. A.; Boyd, J.; Sonoda, Y.; LaQuaglia, M. P.
Article Title: Pediatric radical abdominal trachelectomy for cervical clear cell carcinoma: A novel surgical approach
Abstract: Introduction. Clear cell carcinoma of the cervix and vagina is rare in the pediatric population. Many of these tumors are associated with prior intrauterine diethylstilbestrol (DES) exposure. All DES-associated tumors are believed to contain microsatellite instability (MI). Historically, the recommended treatment is radical hysterectomy and pelvic lymphadenectomy, which result in infertility in all cases. Radical abdominal or vaginal trachelectomy and pelvic lymph node dissection is a new technique utilized in adult women with early cervical cancer who wish to retain fertility. This novel approach is also pertinent to the pediatric patient and is described in this report. A molecular analysis is also performed to determine if these are DES-associated tumors. Methods. Due to the narrow vaginal anatomy in pediatric patients, a vaginal approach is not possible, and an abdominal approach is performed. The resection includes the cervix, upper vagina, parametrium, and paracolpos. Pelvic lymphadenectomy is performed in a similar manner to the adult patient. Matched pairs of normal and tumor DNA from both cases were examined for evidence of MI using a consensus panel of microsatellite markers. Results. Two girls aged 6 and 8 years and without history of DES exposure presented with vaginal bleeding. Vaginoscopy revealed cervical polyp in both cases. Biopsies demonstrated clear cell cancer stage IB1 in both patients. They underwent radical abdominal trachelectomy and bilateral pelvic lymph node dissection along with anastomosis of uterine isthmus to upper vagina. Intraoperative frozen-section analysis confirmed negative uterine and vaginal margins. No adjuvant treatment was given and both girls remain disease free. Neither tumor showed evidence for MI, confirming that these are not DES-associated tumors. Conclusion. To our knowledge, this is the first report of radical abdominal trachelectomy in the pediatric age group and it is likely to include the youngest patient with clear cell carcinoma of the uterine cervix not associated with DES exposure. This novel approach is feasible and appears safe in the pediatric age group. © 2004 Elsevier Inc. All rights reserved.
Keywords: child; human tissue; surgical technique; case report; cancer staging; lymph node dissection; pelvis lymphadenectomy; vagina; biopsy; histology; feasibility study; microsatellite instability; gynecologic surgical procedures; safety; anatomy; diethylstilbestrol; microsatellite marker; clear cell carcinoma; adenocarcinoma, clear cell; anastomosis; frozen section; fertility; uterine cervical neoplasms; gynecologic surgery; dna determination; polyp; trachelectomy; radical abdominal trachelectomy; uterine cervix; uterine cervix carcinoma; pediatrics; cervical cancer; fertility sparing surgery; clear cell cancer; vagina bleeding
Journal Title: Gynecologic Oncology
Volume: 97
Issue: 1
ISSN: 0090-8258
Publisher: Elsevier Inc.  
Date Published: 2005-04-01
Start Page: 296
End Page: 300
Language: English
DOI: 10.1016/j.ygyno.2004.12.050
PUBMED: 15790482
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 22" - "Export Date: 24 October 2012" - "CODEN: GYNOA" - "Source: Scopus"
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MSK Authors
  1. Wendy Tienhui Su
    11 Su
  2. Yukio Sonoda
    472 Sonoda
  3. Douglas A Levine
    380 Levine
  4. Deborah A Levine
    5 Levine
  5. Jeffrey Boyd
    112 Boyd