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A Large Tonsillar Mass in a Healthy Teenager

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

A previously healthy 18-year-old woman presented with a growth in the right oropharynx of 4 months’ duration. At the time of presentation, it had begun to cause changes in her voice, globus sensation, and dysphagia. The patient reported a 20-pound weight loss over the prior 3 months and decreased appetite. She denied any associated difficulty breathing, pain, fever, chills, rash, or night sweats. She was a nonsmoker and nondrinker.

On examination, she appeared well. Despite a muffled voice, she was not in respiratory distress. Fiberoptic laryngoscopy revealed a large, partially obstructing mass in the right oropharynx protruding from the right tonsil. It descended inferiorly and was partially compressing the epiglottis. The true and false vocal cords, although partially obstructed, appeared normal and mobile. The lesion was without ulceration, exudate, or erythema. The contralateral tonsil was small and normal in appearance. The remainder of her physical examination findings were unremarkable, including no neck masses.

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C. Mixed lymphatic and venous malformation

Surgical pathologic analysis determined that the lesion was a mixed lymphatic and venous malformation. The lesion had large, macrocystic channels mostly devoid of red blood cells (Figure 2A and B). Occasional valvelike structures were noted, consistent with both lymphatic and venous features (Figure 2C and D). It was also noted that many of these vascular structures were associated with smooth muscle, a characteristic more closely associated with veins. Immunohistochemical analysis of the lesional specimen demonstrated strongly positive CD-31 staining (Figure 2E), an endothelial cell marker, and weak to strongly positive D2-40 (podoplanin) staining, an endothelial cell marker specific to lymphatics.1 It was noted that both D2-40–positive and D2-40–negative structures were present in the specimen, suggesting a mixed lymphatic and venous origin of the lesion (Figure 2F). There was no evidence of lymphoma within the specimen.

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Article Information

Corresponding Author: Todd E. Falcone, MD, University of Connecticut Health Outpatient Pavilion, 263 Farmington Ave, Eighth Floor, Farmington, CT 06030 (tfalcone@uchc.edu).

Published Online: January 31, 2019. doi:10.1001/jamaoto.2018.3891

Conflict of Interest Disclosures: Dr Falcone serves as a consultant to GI Reviewers, LLC. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Enrique Ballesteros, MD, Department of Pathology and Laboratory Medicine, University of Connecticut School of Medicine, for helping to interpret and review the pathology, providing photomicrographs, and helping to edit the manuscript. He received no compensation for his contributions.

References
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International Society for the Study of Vascular Anomalies. ISSVA classification. http://www.issva.org/classification. Accessed October 29, 2018.
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Pansky  B.  Review of Medical Embryology. New York, NY: Macmillan; 1982.
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Perkins  JA, Manning  SC, Tempero  RM,  et al.  Lymphatic malformations: review of current treatment.  Otolaryngol Head Neck Surg. 2010;142(6):795-803, 803.e1.PubMedGoogle ScholarCrossref
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Stal  S, Hamilton  S, Spira  M.  Hemangiomas, lymphangiomas, and vascular malformations of the head and neck.  Otolaryngol Clin North Am. 1986;19(4):769-796.PubMedGoogle Scholar
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Mardekian  S, Karp  JK.  Lymphangioma of the palatine tonsil.  Arch Pathol Lab Med. 2013;137(12):1837-1842. doi:10.5858/arpa.2012-0678-RSPubMedGoogle ScholarCrossref
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