Extraosseous Ewing Sarcoma in a Pelvic Region: A Case Report.

JNMA J Nepal Med Assoc

Department of Paediatric Haematology and Oncology, Bhaktapur Cancer Hospital, Dudhpati, Bhaktapur, Nepal.

Published: July 2022

Unlabelled: Ewing sarcoma is the second most common malignant bone tumour in children. It rarely originates from extraskeletal soft tissue sites such as the upper thigh, buttocks, upper arm and shoulder. Primary extraosseous Ewing sarcoma located in the pelvic region is rare. We report a 17-year-female who had gradual onset of progressive lower abdominal mass and pain. A computed tomography scan revealed well defined lobulated heterogeneously enhancing lesion noted in the pelvic region measuring approximately 12.9 x 9.8 x 9.3 cm. Incisional biopsy showed a small round blue cell tumour which was strongly positive for Cluster of Differentiation 99, vimentin, Friend Leukaemia Integration 1 with 40% Ki-67. Following treatment with chemotherapy, surgery and radiotherapy, there was complete resolution of the tumour. Although extraosseous Ewing sarcoma is rare, it can occur virtually in any soft tissue site. Therefore, clinicians need to distinguish it from soft tissue sarcoma because rapid progression, early diagnosis and timely treatment are crucial for a favourable prognosis.

Keywords: Ewing sarcoma; neoadjuvant chemotherapy; radiation therapy.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9297356PMC
http://dx.doi.org/10.31729/jnma.7523DOI Listing

Publication Analysis

Top Keywords

ewing sarcoma
20
extraosseous ewing
12
pelvic region
12
soft tissue
12
sarcoma
6
sarcoma pelvic
4
region case
4
case report
4
report unlabelled
4
ewing
4

Similar Publications

Extra-skeletal Ewing sarcoma is a rare tumor, most commonly occurring in the paravertebral region, extremities, retroperitoneum, and very rarely in visceral locations. We present two primary visceral Ewing sarcomas confirmed at the molecular level: one located in the stomach and the other in the bladder. Both tumors demonstrated strong and diffuse membranous CD99 expression, accompanied by variable KIT immunoreactivity.

View Article and Find Full Text PDF

International benchmarking of stage at diagnosis for six childhood solid tumours (the BENCHISTA project): a population-based, retrospective cohort study.

Lancet Child Adolesc Health

February 2025

Developmental Biology and Cancer Research & Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK. Electronic address:

Background: International variation in childhood cancer survival might be explained by differences in stage at diagnosis, among other factors. As part of the BENCHISTA project, we aimed to assess geographical variation in tumour stage at diagnosis through the application, by population-based cancer registries working with clinicians, of the international consensus Toronto Childhood Cancer Stage Guidelines.

Methods: This population-based, retrospective cohort study involved 67 cancer registries from 23 European countries, Australia, Brazil, Japan, and Canada.

View Article and Find Full Text PDF

Background And Aims: Chemotherapy with alternating cycles of vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide, along with primary tumor treatment with surgery or radiotherapy or both, constitute the usual treatment of Ewing sarcoma. The AEWS0031 study demonstrated survival benefits after interval-compressed chemotherapy without significant toxicity. The aim of this study was to assess the tolerability of dose-intensified chemotherapy in developing countries like India.

View Article and Find Full Text PDF

Bone sarcomas and cancer predisposition syndromes.

Bull Cancer

January 2025

Department of Paediatric Oncology, Institut d'Haematologie et d'Oncologie Pédiatrique, Centre Léon-Bérard, Lyon, France. Electronic address:

Bone sarcomas, constituting less than 1% of malignant neoplasms across all age groups, are rare tumours possibly associated with genetic susceptibility syndromes. This review aims to provide recommendations for the detection of cancer predisposition syndromes associated with bone sarcomas and managing affected patients. Recommendations were formulated by a multidisciplinary working and reviewing group from GROUPOS and SFCE oncogenetic's group, including geneticists, oncologists, and radiologists.

View Article and Find Full Text PDF

Incidence and survival of European adolescents and young adults diagnosed with sarcomas: EUROCARE-6 results.

Eur J Cancer

January 2025

Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

Background: Epidemiological data for sarcoma in adolescents and young adults (AYAs) and across age groups are limited. We aim to: 1) update sarcoma incidence, survival, and changes over time in European AYAs; 2) provide an updated comparison of sarcoma survival in AYAs versus children and mature adults.

Methods: We calculated crude incidence rates (IR) per 100,000 European population per year from 2006 to 2013.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!