Embryonal rhabdomyosarcoma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Right facial pain swelling and tenderness to palpation. History of a tooth pulled one month ago. Despite antibiotics, patient continues to have pain, swelling, and weight loss.

Patient Data

Age: 9 years
Gender: Male
ct

There is a large, heterogeneously enhancing mass which appears to be centered in the right masticator space. The mass extends anteriorly to the posterior wall the right maxillary sinus. The mass extends posteriorly to the retropharyngeal region and abuts the right longus coli muscle, but does not extend into the cervical spine. There is extension cephalad with enhancement in the right foramen ovale within a portion of the right middle cranial fossa. There is extension into the cavernous sinus, the region of the right eustachian tube, and in the right glenoid fossa/right temporomandibular joint region.

The mass extends posterolaterally into the superficial lobe of the right parotid. There is widening of the right pterygopalatine fossa and sphenopalatine foramen. The mass extends medially to cause mass effect on the right tonsillar pillar, without airway compromise.

mri

There is a large, T2/FLAIR hyperintense, mass with irregular contrast enhancement in the superior right neck. The margins of this tumor include posterior wall of right maxillary sinus, undersurface of right zygomatic arch, nasopharyngeal soft tissues, and the longus collie muscles. It extends to the midline within the nasopharynx and compresses the fossa of Rosenmuller. The eustachian tube is blocked and there is extensive fluid within the right mastoid air cell complex. Superiorly, the tumor extends through the foramen ovale and abuts the undersurface of the mesial right temporal lobe. Inferiorly, the tumor is separated from the submandibular gland by the depressed lateral pterygoid muscle.

The right common carotid artery and internal jugular vein are displaced posteriorly but appear patent.

Case Discussion

This is a case of an embryonal rhabdomyosarcoma.

Gross pathologic specimens revealed multiple small, soft, red tissue fragments. Histopathologic analysis revealed cells that were positive for myogenin, desmin, CD56, and bcl-2. The combined morphology and immunoprofile was consistent with rhabdomyosarcoma, embryonal type.

The patient received chemoradiation and has been disease-free for nearly 10 years.

Co-author:
Mason Soeder
Travis Bevington, MD

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