Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast

Ikhwan, S M; Kenneth, V K T; Seoparjoo, Azmel; Zin, Anani Aila Mat

doi:10.1136/bcr-2013-009584

Cited by 3 publications

(2 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Metastatic disease at the time of diagnosis is a negative prognostic factor ( 9 , 10 , 21 ) and recurrent Ewing’s sarcoma, both localized and metastatic, is almost always fatal ( 10 , 20 , 32 ). As in soft tissue sarcomas, involvement of the lymph node stations in the axilla is very rare, as they mainly spread by the hematogenous route.…”

Section: Discussionmentioning

confidence: 99%

Ewing’s Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature

et al. 2022

View full text Add to dashboard Cite

Ewing’s Sarcoma Family Tumors (ESFT) include classic Ewing’s sarcoma of bone, extra-skeletal Ewing’s sarcoma (EES), malignant small cell tumor of the chest wall (Askin tumor), and soft tissue-based Peripheral Primitive Neuroectodermal tumors (pPNET). The t(11;22)(q24;q12) translocation is associated with 85% of tumors and leads to EWS-FLI-1 (Ewing’s Sarcoma–Friend Leukemia Integration-1) formation. This is a potent transforming gene that encodes a chimeric protein that plays a role in the genesis of Ewing’s Sarcoma and Primitive Neuroectodermal Tumors. The breast location of ESFT remains exceptional. The prognosis is among the poorest of all subtypes of breast cancer and even poorer than other extraosseous Ewing’s sarcomas. We describe the case report of a 23-year-old patient with a growing breast lump, who required an accurate and challenging diagnostic estimation and who ultimately resulted in a peripheral primary neuroectodermal tumor (pPNET). Through this case description and a brief narrative review of the literature, we aim to highlight the rarity of ESFT located in the breast. Histopathological confirmation is mandatory for all growing masses of the breast to reach a conclusive diagnosis and plan the correct treatment. Patients with rare diagnoses should always be centralized in breast units, conducting multidisciplinary meetings and, when necessary, the diagnosis should be shared through wider national or international registries.

show abstract

Section: Discussionmentioning

confidence: 99%

Ewing’s Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature

et al. 2022

View full text Add to dashboard Cite

show abstract

“…Extraosseous Ewing's sarcoma (EES)/PNET is an extremely rare primary malignant tumor with rapid progression and poor prognosis (3). EES/PNET has been reported mainly in the chest wall, lower extremities and paravertebral regions, followed by the upper extremities, hip and pelvis, and rarely in the epidural space, especially at the cervicothoracic junction (4,5).…”

Section: Introductionmentioning

confidence: 99%

A case of giant Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the cervicothoracic junction in children with incomplete paralysis of both lower limbs: Case report and literature review

Zhang

Lin

et al. 2023

Front. Surg.

View full text Add to dashboard Cite

BackgroundExtraosseous Ewing's sarcoma/primary neuroectodermal tumor (EES/PNET) is a rare, malignant, small round blue cell tumor, which usually involves the larynx, kidneys, and esophagus. The most common metastatic sites are lung and bone. The incidence of epidural EES/PNET was 0.9%, and a detailed search of the PubMed literature found only 7 case reports of epidural ESS/PNET at the cervicothoracic junction in children.Case descriptionWe report a case of epidural ESS/PNET at the cervicothoracic junction in a child with chest and back pain as the first symptom, which worsened after half a year and developed incomplete paralysis of both lower extremities and urinary incontinence. She underwent emergency surgery, chemotherapy and radiotherapy, and died of lung metastases 8 months after surgery.ConclusionPrimary epidural tumors are mostly benign, such as spinal meningiomas and neuromas. Contrary to what has been previously thought, we report a case of malignant epidural EES/PNET at the cervicothoracic junction without bone destruction; The rarity of epidural EES/PNET at the cervicothoracic junction in children has led to a lack of data, particularly on prognostic factors and recurrence patterns. Due to the difficulty of early diagnosis and high mortality, spine surgeons must explore and increase their awareness of this disease.

show abstract

Primary Ewings Sarcoma/Primitive Neuroectodermal Tumor (PNET) of the Breast: a Rare Tumor

2019

View full text Add to dashboard Cite

Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast

Cited by 3 publications

References 10 publications

Ewing’s Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature

Ewing’s Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature

A case of giant Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the cervicothoracic junction in children with incomplete paralysis of both lower limbs: Case report and literature review

Primary Ewings Sarcoma/Primitive Neuroectodermal Tumor (PNET) of the Breast: a Rare Tumor

Contact Info

Product

Resources

About