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RB1-associated tumor predisposition syndrome

Friday 6 November 2015

rb-associated tumor predisposition syndrome

Tumors

- retinoblastoma
- post-radiation osteosarcoma

- secondary tumors arising in patients with retinoblastoma

  • Almost two-thirds of the secondary tumors arising in patients with retinoblastoma are mesenchymal in origin.
  • Over 60% of the mesenchymal tumors are osteosarcomas.
  • The soft tissue sarcomas include:
    • fibrosarcoma,
    • leiomyosarcoma,
    • liposarcoma

- homozygous deletions of the RB1 locus in sporadic cases of:

  • leiomyosarcoma,
  • malignant fibrous histiocytoma,
  • undifferentiated sarcoma in the absence of any history of retinoblastoma.