Ewing's sarcoma, also called primitive neuroectodermal tumour of the adrenal gland, is extremely rare.
Only a few cases have been reported in the literature. We report on a woman with adult-onset primitive neuroectodermal tumour of the adrenal gland presenting with progressive flank pain. Computed tomography confirmed an adrenal tumour with invasion of the left diaphragm and kidney. Radical surgery was performed and the pain completely resolved; histology confirmed the presence of primitive neuroectodermal tumour, for which she was given chemotherapy. The clinical presentation of this condition is non-specific, and a definitive diagnosis is based on a combination of histology, as well as immunohistochemical and cytogenic analysis. According to the literature, these tumours demonstrate rapid growth and aggressive behaviour but there are no well-established guidelines or treatment strategies. Nevertheless, surgery remains the mainstay of local disease control; curative surgery can be performed in most patients. Adjuvant chemoirradiation has been advocated yet no consensus is available. The prognosis of patients with primitive neuroectodermal tumours remains poor.
Written by:
Tsang YP, Lang BH, Tam SC, Wong KP. Are you the author?
Division of Endocrine Surgery, Department of Surgery, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong.
Reference: Hong Kong Med J. 2014 Oct;20(5):444-6.
doi: 10.12809/hkmj134127
PubMed Abstract
PMID: 25307073