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Incidental Sphenoid Mass Found on Facial Trauma Workup

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A healthy 30-year old man presented to the emergency department with facial trauma after an assault. Physical examination was notable for tenderness over the nasal bones. No neurologic deficits were noted. Computed tomography (CT) scan of the head without contrast revealed nasal bone fractures and an incidental 7-mm tissue density within the posterior aspect of the left sphenoid sinus (Figure 1A) with an apparent dehiscence of the posterior wall and possible communication with the intracranial extra-axial spaces and brain parenchyma. A magnetic resonance imaging scan with contrast was recommended to further characterize the lesion which showed a transosseous T1 dark, T2 bright multilobulated mass measuring 2.1 × 1.4 × 1.3 cm with heterogeneous enhancement on postcontrast imaging extending into the sphenoid sinus. An endoscopic transnasal approach was used to obtain a tissue biopsy specimen. Pathological analysis revealed sheets of neoplastic, vacuolated, “soap bubble–like” cells with a myxoid background (Figure 1B).

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B. Chordoma

Chordomas are slow-growing tumors arising from the embryologic notochord. Due to their origins, these tumors occur most often along the axial skeleton with equal distribution in the skull base (32.0%), mobile spine (32.8%), and sacrum (29.2%). Mean age of occurrence is 36.9 years.1,2

Grossly, chordomas are lobulated, gelatinous, gray tumors well delineated from the surrounding tissue. Microscopically, chordomas consist of large, eosinophilic cells with multiple, round, clear vacuoles in a basophilic myxoid stroma. These vacuoles are classically referred to as physaliphorous or having a “soap-bubble” appearance (from the Greek “bubble-bearing”) (Figure 2). Vacuoles may coalesce and be mistaken for mature adipocytes. The World Health Organization recognizes 3 distinct variants of chordomas: conventional, chondroid, and dedifferentiated. Conventional chordomas are primarily composed of nests or trabeculae of eosinophilic cells with multiple clear, round vacuoles. Chondroid chordomas consist of areas resembling conventional chordomas and areas of chondroid differentiation resembling low-grade chondrosarcomas. Dedifferentiated chordomas contain high-grade sarcomatous areas and have a worse prognosis. Immunohistochemically, chordomas will stain with cytokeratin, epithelial membrane antigen, and S-100. Unlike histologic mimics, all 3 variants of chordomas will display nuclear brachyury staining with a high specificity (89.7%-100%).14

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Article Information

Corresponding Author: Edward R. Utz, MD, Department of Otolaryngology, Naval Medical Center San Diego, 34800 Bob Wilson Dr, San Diego, CA 92134 (edward.r.utz2.mil@mail.mil).

Published Online: July 3, 2019. doi:10.1001/jamaoto.2019.1662

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

Additional Information: The authors are military service members, and this work was prepared as part of official duties. Title 17, US Code §105, provides that copyright protection under this title is not available for any work of the US government. Title 17, US Code §101, defines a US government work as a work prepared by a military service member or employee of the US government as part of that person’s official duties. No funding was obtained for this study.

References
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Reith  JD. Bone and Joints. In: Goldblum  JR, ed.  Rosai and Ackerman’s Surgical Pathology. 11th ed. Philadelphia, PA: Elsevier; 2018:1740-1809.
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St. Clair  EG, McCutcheon  IE. Skull Tumors. In: Winn  RH, ed.  Youmans and Winn Neurological Surgery. 7th ed. Philadelphia, PA: Elsevier; 2017:1322-1343.
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Gui  X, Siddiqui  NH, Guo  M.  Physaliphorous cells in chordoma.  Arch Pathol Lab Med. 2004;128(12):1457-1458.PubMedGoogle Scholar
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Barresi  V, Ieni  A, Branca  G, Tuccari  G.  Brachyury: a diagnostic marker for the differential diagnosis of chordoma and hemangioblastoma versus neoplastic histological mimickers.  Dis Markers. 2014;2014:514753. doi:10.1155/2014/514753PubMedGoogle ScholarCrossref
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Jahangiri  A, Chin  AT, Wagner  JR,  et al.  Factors predicting recurrence after resection of clival chordoma using variable surgical approaches and radiation modalities.  Neurosurgery. 2015;76(2):179-185. doi:10.1227/NEU.0000000000000611PubMedGoogle ScholarCrossref
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Walcott  BP, Nahed  BV, Mohyeldin  A, Coumans  JV, Kahle  KT, Ferreira  MJ.  Chordoma: current concepts, management, and future directions.  Lancet Oncol. 2012;13(2):e69-e76. doi:10.1016/S1470-2045(11)70337-0PubMedGoogle ScholarCrossref
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Fernandez-Miranda  JC, Gardner  PA, Snyderman  CH,  et al.  Clival chordomas: A pathological, surgical, and radiotherapeutic review.  Head Neck. 2014;36(6):892-906. doi:10.1002/hed.23415PubMedGoogle ScholarCrossref
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