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Les tératomes sacrococcygiens. Expérience d’un service de chirurgie pédiatrique nord-africain

Sacrococcygeal teratomas. Experience of a North African department of pediatric surgery

Résumé

Objectif

Étudier les aspects épidémiologiques, anatomopathologiques, cliniques et évolutifs des tératomes sacrococcygiens (TSC) chez l’enfant dans le sud et le centre tunisiens.

Matériel et méthodes

Il s’agit d’une étude rétrospective de 24 observations d’enfants opérés pour TSC entre 1984 et 2004. Le diagnostic de TSC a été confirmé dans tous les cas par l’examen histologique de la pièce opératoire.

Résultats

L’âge de nos patients a varié de la naissance à cinq ans. Des malformations associées ont été retrouvées dans 12,5% des cas. Il s’agissait d’une exstrophie vésicale associée à une ambiguïté sexuelle, d’une ectopie testiculaire bilatérale et d’un spina-bifida. Le traitement chirurgical a été entrepris de façon rapide. Il a consisté en une exérèse en monobloc de la tumeur et du coccyx. La voie d’abord était toujours périnéale associée à une voie abdominale dans deux cas. L’étude anatomopathologique a montré qu’il s’agissait d’un tératome mature dans 66,6% des cas, immature dans 16,7% et malin dans 16,7%. La mortalité a été de 16,7% et a concerné dans tous les cas des tératomes initialement malins.

Discussion

Les TSC sont les plus fréquents des tératomes de l’enfant. Des malformations associées au TSC sont rapportées dans 18% des cas. Elles sont dominées par les anomalies musculo-squelet-tiques, génito-urinaires, digestives, cardiaques et chromosomiques. Le traitement du TSC est essentiellement chirurgical. Il doit être instauré le plus précocement possible et doit comporter une exérèse totale de la masse emportant le coccyx. Un complément thérapeutique par chimiothérapie est indispensable dans les formes malignes. Le pronostic des TSC est généralement bon. En effet dans les tératomes bénins, la chirurgie précoce et totale emportant le coccyx donne de bons résultats avec un taux de guérison de 90 à 100 %.

Conclusion

Une surveillance clinique, biologique et radiologique est indispensable et doit s’étaler sur au moins trois à cinq ans pour détecter à temps les récidives.

Abstract

Objective

The aim of this study is to review the different aspects of sacrococcygeal teratomas (SCT) in the Tunisian south and centre.

Material and Methods

This is a retrospective study of 24 SCT observations operated between 1984 and 2004. The diagnosis of SCT was confirmed in all cases by the histological exam.

Results

The age of our patients varied from the birth to 5 years. Associated malformations were found in 12.5% of cases and consisted on bladder exstrophy associated to a sexual ambiguity, bilateral cryptorchidism and spina bifida. The surgical treatment consisted on complete resection of the tumour and the coccyx. The approach was perineal in all cases associated to an abdominal approach in two cases. Histological study showed that the teratoma was mature in 66.6% of cases and immature in 16.7%. A yolk sac tumour was encountered in 16.7%. Mortality rate was 16.7% and concerned the cases of yolk sac tumour.

Discussion

The SCT is the most frequent of teratomas of the child. Associated malformations are reported in 18% of cases and are dominated by musculo-scrawny, genito-urinary, digestive, cardiac and chromosomic abnormalities. The SCT treatment is essentially surgical. It must be established the most prematurely and consists on a total resection of the mass taking the coccyx. A therapeutic complement by chemotherapy is indispensable for malignant teratomas and yolk sac tumour. The outcome of the SCT is generally good with a cure rate ranged from 90 to 100%.

Conclusion

A clinical, biologic and radiological follow-up is indispensable and has to spread out over at least 3–5 years to discover at time the recurrences.

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Correspondence to M. Mekki.

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Mekki, M., Jallouli, M., Krichene, I. et al. Les tératomes sacrococcygiens. Expérience d’un service de chirurgie pédiatrique nord-africain. Oncologie 9, 864–868 (2007). https://doi.org/10.1007/s10269-007-0727-9

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  • DOI: https://doi.org/10.1007/s10269-007-0727-9

Mots clés

  • Tératome sacrococcygien
  • Tumeur du sac vitellin
  • Enfant
  • Chirurgie
  • Chimiothérapie

Keywords

  • Sacrococcygeal teratoma
  • Yolk sac tumour
  • Children
  • Surgery
  • Chemotherapy