Ewing sarcoma - extraskeletal

Presentation

Female pediatric patient, with a history of trauma injury secondary to a fall from his own height, leading to acute urinary retention.

Patient Data

Age: 10 years
Gender: Female
CT

Bilateral ureteropyelocaectasia with bladder distension. Hypodense mass with presacral peripheral enhancement in the region of the right piriformis muscle and displacement of the rectum to the left.

Annotated image

Hypodense mass with presacral peripheral enhancement in the region of the right piriformis muscle (arrows). 

MRI

MRI

A presacral mass located in the right piriformis muscle, T1 hypointense and T2 hyperintense with a hypointense wall. Diameters of 4x2.8x2.6 mm go from S2 right sacral foramen and into the spinal canal and move the rectum to the left.

Annotated image

A presacral mass located in the right piriformis muscle (arrows).

Photo

3D PRINTING

Pictures of the case on 3D printing

Ewing Sarcoma corresponds to the green mass

Pathology

H&E: Small cell sarcoma, findings consistent with primitive neuroectodermal tumor and/or Ewing's sarcoma with extensive necrosis of the soft tissues of the presacral region.

Immunohistochemical: morphological and immunohistochemical findings consistent with primitive neuroectodermal tumor and/or Ewing's sarcoma of the soft parts of the sacral region.

Case Discussion

Ewing's sarcoma is a malignant, aggressive, relatively frequent tumor that occurs predominantly in adolescent age, affecting the metadiaphyseal layer of bone, occasionally with intramedullary extension.

Within the Ewing family of tumors is the variety of soft tissue involvement that manifests radiologically as a heterogeneous mass of extraskeletal extension, which generally does not affect bone tissue, although occasionally it can cause erosion or periosteal reaction due to mass effect on the bone structure.

Clinically, if it is in relation to the spinal column, as in this case, it is presacral in location. it can manifest as radicular pain, bladder or intestinal dysfunction.

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Case information

rID: 60720
Published: 7th Jul 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Nucleo diagnóstico avanzado

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