Author/Authors :
Nair، Manjusha نويسنده Division of Pediatric Oncology, Regional Cancer Centre, Trivandrum, India , , Nair، Reghu Kesavapillai Sukumaran نويسنده Division of Pediatric Oncology, Regional Cancer Centre, Trivandrum, India , , Raghavan، Rajeev Kavalakara نويسنده Department of Radiotherapy, Regional Cancer Centre, Trivandrum, India , , Parukkutty، Kusumakumary نويسنده Division of Pediatric Oncology, Regional Cancer Centre, Trivandrum, India , , Sukumaran، Renu نويسنده Department of Pathology, Regional Cancer Centre, Trivandrum, India ,
Abstract :
Ewing’s sarcoma is a common malignancy of the bone and soft tissues in pediatric
patients. It mostly affects the long bones and pelvis, and less commonly the flat bones
and vertebrae. Primary Ewingʹs sarcoma affecting the spine is very rare. The patient
has non-specific symptoms for a prolonged period of time before the correct diagnosis
is given. Patients can present with acute paraplegia due to spinal cord compression, which
needs prompt surgical intervention. Early diagnosis and treatment is important for
neurological recovery. The definitive management includes three main modalities:
surgery, radiotherapy, and combination chemotherapy. Adequate surgical excision
may not be feasible because of anatomical limitations and local control is mainly achieved
by radiotherapy. Because of the low incidence of these tumors, a multitude of therapeutic
strategies have been employed with varying success. Currently there are no clinical
guidelines outlining optimal management. We present a series of six cases of primary
Ewingʹs sarcoma of the spine, analysis of presenting complaints, examination findings,
diagnostic investigations, treatment, outcome and review of relevant literature. Five
out of the six patients had prolonged musculoskeletal symptoms before they were
correctly diagnosed and three presented with acute paraplegia. All have received
aggressive multimodality therapy with complete neurological recovery and are
surviving.