Rare incidence of three consecutive primary tumors in the maxillofacial region: retinoblastoma, leiomyosarcoma, and choriocarcinoma: case report

J Craniofac Surg. 2001 Sep;12(5):464-8. doi: 10.1097/00001665-200109000-00012.


Multiple primary tumors occur more commonly in the region of the head and neck than elsewhere in the body. The chance of this is particularly high in patients treated for retinoblastoma, in part because of a genetic predisposition, and in part because of the possibility of irradiation treatment. However, triple tumors occur in only 0.5% of multiple tumors. A rare case of a triple (metachronous) tumor is reported: 12 years after the treatment of bilateral retinoblastoma (enucleation and irradiation), secondary leiomyosarcoma developed in the maxillofacial region, followed 5 years later by choriocarcinoma. Surgery was performed on all three types of tumor. As a result, the female patient (currently 21 years old) is now free of complaints and has married. It is extremely rare for either leiomyosarcoma or choriocarcinoma (CHC) to appear in the maxillofacial region. The long-term, systematic control of such patients is absolutely necessary, for the multiple tumors tend to develop only after a long latency period of 10 to 20 years.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Choriocarcinoma / drug therapy
  • Choriocarcinoma / pathology*
  • Choriocarcinoma / surgery
  • Cranial Irradiation / adverse effects
  • Eye Enucleation
  • Female
  • Head and Neck Neoplasms / pathology*
  • Head and Neck Neoplasms / radiotherapy
  • Head and Neck Neoplasms / surgery
  • Humans
  • Leiomyosarcoma / etiology
  • Leiomyosarcoma / pathology*
  • Leiomyosarcoma / surgery
  • Neoplasms, Multiple Primary / pathology*
  • Neoplasms, Radiation-Induced / etiology
  • Neoplasms, Radiation-Induced / pathology*
  • Neoplasms, Radiation-Induced / surgery
  • Neoplasms, Second Primary / etiology
  • Neoplasms, Second Primary / pathology*
  • Retinoblastoma / pathology*
  • Retinoblastoma / radiotherapy
  • Retinoblastoma / surgery