THROAT - Burkitt’s tumour

Synonym : Malignant Lymphoma of Africa.

Geographic distribution:
1) Tropical Africa
2) New Guinea
3) Nigeria
4) South America where malaria is endemic

What is the Aetiology of   Burkitt's tumour?
 
1) Sex: Males predominantly affected
2) Age: Young children affected more. Over 80% of cases occur
between ages of 3 years and 12 years.
3) Seen in areas where malaria is endemic suggesting a possible
insect vector of an infective agent.
4) The Epstein- Barr virus which causes glandular fever.
5) Infectious mononucleosis in young adults in western societies
is also closely associated with Burkitt's lymphoma.

Clinical presentation:
1)      Tumor multifocal grows rapidly, soft and painless.
2)      In the typical African form, the disease presents primarily as an extra      lymphatic tumor arising in bone of jaw 60% of patient.
3)      Maxillary lesions may present as oral or orbital tumors.
4)      Second commonest presentation is an abdominal tumor involving kidneys, ovaries , retroperitoneal nodes or gastrointestinal tract.
5)      Other sites include thyroid, testicles, skeleton and breast.
6)      Bone marrow and CSF involvement present in 1/3rd of patients

Histopathology:
Presence of dense lymphoblasts interspersed with large, clear histocyles containing debris derived from rapid cell turn over contributing 'Starry Sky' appearance.

What is the treatment of  Burkitt's  tumour?

a.       Sensitive to both radiotherapy and chemotherapy such as     Cyclophosphamide ,Methotrexate .
b.       Spontaneous remission has been seen.




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