Abstract
ABSTRACT Ewing’s sarcoma is a malignant tumor that arises mainly from bone tissue, so that its extra-osseous presentation is not very common and even more unusual, in the soft tissues of the oral cavity. The objective of this case was to describe the clinical characteristics of an extra-osseous Ewing’s Sarcoma of the tongue in a pediatric patient. An 11-year-old male school patient, who attended an oral medicine consultation referring a volume increase in the tongue. Intraorally, a tumoral lesion was observed on the left lateral edge of the tongue, indurated, with the same color of the mucosa with ulcerated areas, well defined edges and symptomatic on palpation, with an approximate size of 2.5 cm. A cervical and maxillofacial MRI was indicated, observing a partially delimited hyperintense and non-infiltrating lesion in the described area. An incisional biopsy was performed with histopathological diagnosis of malignant neoplasm of blue round cells, theCD99 marker was found to be positive in the immunohistochemical study and was key to the definitive diagnosis of this tumor. A thoraco-abdomino-pelvic tomography was requested by the Pediatric Oncology service, as well as biopsy and aspiration of bone marrow, in which no neoplastic infiltrations were evidenced. After four cycles of chemotherapy, total removal of the lesion was performed with a consecutive histopathological study of the surgical piece, indicating free edges of the lesion. Ewing’s sarcoma is a tumor with aggressive behavior, so this case represents a finding of clinical and epidemiological relevance, both due to its extra-osseous appearance and its unusual behavior.
Highlights
Ewing’s sarcoma (ES) is a rare and highly malignant tumor that may arise from bone and soft tissue
It occurs most frequently in bone tissue and comprises about 4% to 6% of all primary bone tumors and represents one of the most common bone malignancies after myeloma, osteosarcoma and chondrosarcoma [1]. This family of sarcomas includes the tumor of Askim and both the osseous and extra osseous Ewing’s sarcoma [2]
An immunohistochemistry study was necessary for the definitive diagnosis, which indicated Extra osseous Ewing’s sarcoma / Primitive neuroectodermal tumor (PNET), immunoreactive to the markers of Vimentin mesenchymal differentiation, neuron-specific neuroendocrineenolase differentiation (NSA), PGP 9.50 and to the CD99 / MIC-2 marker, while the epithelial, lymphoid and desmin differentiation markers were found to be negative
Summary
Ewing’s sarcoma (ES) is a rare and highly malignant tumor that may arise from bone and soft tissue. The extra osseous presentation of this tumor represents approximately 4% of soft tissue tumors and 1.5-4% of childhood sarcomas [4] The origin of this tumor is still unclear; several studies have reported that it comes from neuroectodermal cells. An immunohistochemistry study was necessary for the definitive diagnosis, which indicated Extra osseous Ewing’s sarcoma / Primitive neuroectodermal tumor (PNET), immunoreactive to the markers of Vimentin mesenchymal differentiation (figure 2B), neuron-specific neuroendocrineenolase differentiation (NSA) (figure 2C), PGP 9.50 and to the CD99 / MIC-2 marker (figure 2D), while the epithelial, lymphoid and desmin differentiation markers were found to be negative (figure 2E, 2F). Prior to the various studies and procedures carried out, the patient’s legal guardian voluntarily signed an informed consent in which the possible risks and complications that could arise during the diagnostic process were explained, as well as the purpose and benefits of his contribution to the research
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
