Abdulqader Al Hebshi has completed his General Pediatrics at Jordanian and Arab Board in 2010. Then, he did a Clinical Fellowship in Pediatric Hematology Oncology
for three years from King Hussein Cancer Centre in Jordan. After that, he joined The Hospital for Sick Children in Toronto to do one year Clinical Fellowship 2014-2015. Currently, he is a Consultant of Hematology & Oncology
and the Clinical Supervisor of Medical Student & Medical Interns at Prince Mohammed Bin Abdul Aziz Hospital-National Guard Hospital Affair in Saudi Arabia. He is an active Member in American Society of Hematologic Oncology
Introduction: Association of osteosarcoma with certain syndromes is well known, but the incidence varies from one report to another, and from one syndrome to another, Ruthmond syndrome is the most common syndrome reported to be associated while others like Blackfan Diamond anemia and osteogenesis imperfect are very rarely associated, and others like Osteopoikilosis are never reported to be associated with osteosarcoma.
Purpose: Our aim from this review is to report our experience and frequency of association of osteosarcoma with syndromic features, and to try to see if these syndromes have effects in the prognosis of osteosarcoma.
Methods: Retrospectively, we reviewed files of all patients diagnosed to have osteosarcoma during the period from January 2003 till December 2011, information regarding presence of syndromic features, current condition of the patient whether alive or death or lost and whether had localized or metastatic disease at diagnosis were recorded.
Results: During the study period, a total of 69 patients were diagnosed to have osteosarcoma, 6 of them were having syndromes: 2 were having Ruthmond syndrome, 1 Blackfan Diamond anemia, 1 Cockyne syndrome, 1 osteogenesis imperfecta and 1 Osteopoikilosis, constituting 8.7% of all cases. From the 63 non-syndromic patients, 41 (65%) were having localized disease, 22 (35%) were metastatic, and from the 6 syndromic patients 2 (33.3%) were localized and 4 (66.6%) were metastatic at diagnosis. Regarding prognosis, from the non-syndromic patients, 14 were lost for follow up, from the reminder 49 patients, 34 (69.3%0) were alive and 15 (30.6%) dead, from the syndromic patients 1 lost for follow up, 1 alive only (20%) and 4 died (80%). Conclusions: Syndromic features were present in 8.7% of our osteosarcoma patients. Number is small, but gives some evidence about the bad prognosis of osteosarcoma when associated with syndromes. Further studies are needed.