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Bae YJ, Kim H, Cha W, et al. Primary pharyngeal synovial sarcoma in a pediatric patient: A case report. Medicine (Baltimore). 2021;100(52):e28411doi: 10.1097/MD.0000000000028411.
Bae, Y. J., Kim, H., Cha, W., & Choi, B. S. (2021). Primary pharyngeal synovial sarcoma in a pediatric patient: A case report. Medicine, 100(52), e28411. https://doi.org/10.1097/MD.0000000000028411
Bae, Yun Jung, et al. "Primary pharyngeal synovial sarcoma in a pediatric patient: A case report." Medicine vol. 100,52 (2021): e28411. doi: https://doi.org/10.1097/MD.0000000000028411
Bae YJ, Kim H, Cha W, Choi BS. Primary pharyngeal synovial sarcoma in a pediatric patient: A case report. Medicine (Baltimore). 2021 Dec 30;100(52):e28411. doi: 10.1097/MD.0000000000028411. PMID: 34967377.
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Medicine (Baltimore). 2021 Dec 30;100(52):e28411. doi: 10.1097/MD.0000000000028411.

Primary pharyngeal synovial sarcoma in a pediatric patient: A case report.

Medicine

Yun Jung Bae, Hyojin Kim, Wonjae Cha, Byung Se Choi

Affiliations

  1. Department of Radiology, Seongnam, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Republic of Korea.
  2. Department of Pathology, Seongnam, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Republic of Korea.
  3. Department of Otorhinolaryngology-Head and Neck Surgery, Seongnam, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Republic of Korea.

PMID: 34967377 DOI: 10.1097/MD.0000000000028411

Abstract

RATIONALE: Synovial sarcoma is a rare malignant tumor that typically originates from the soft tissue of the extremities. The occurrence of primary pharyngeal synovial sarcoma is even rarer, and few studies have reported its radiological features. Here, we report a case of pediatric primary pharyngeal synovial sarcoma and describe the conventional and advanced magnetic resonance imaging (MRI) findings with pathologic correlation.

PATIENT CONCERNS: An 11-year-old girl presented to the otolaryngologic clinic with dysphagia.

DIAGNOSIS: Laryngoscopy revealed a large mass in the oropharynx. MRI revealed a well-defined soft tissue mass with a maximal diameter of approximately 5 cm originating from the submucosal space of the oropharynx. The mass was primarily solid and showed homogeneous contrast-enhancement. The mass was hypointense on T1-weighted images and hyperintense on T2-weighted images. The mass showed a homogeneously low apparent diffusion coefficient value on diffusion-weighted imaging, which indicated high tumor cellularity. Dynamic contrast-enhanced MRI revealed a hypovascular tumor with low values of the volume transfer constant between the extracellular extravascular space and blood plasma and blood plasma volume per unit tissue volume. Amide proton transfer-weighted MRI revealed a relatively high amide proton transfer signal in the tumor, indicating a high protein/peptide component. The patient underwent partial surgical resection of the tumor, and the diagnosis of biphasic synovial sarcoma was confirmed on postoperative pathological examination.

INTERVENTION: The patient was started on chemotherapy with vincristine, ifosfamide, doxorubicin, and etoposide.

OUTCOMES: The tumor did not respond to the 3 cycles of the chemotherapy. Thus, the patient underwent second surgery and subsequent radiation therapy. The patient is now under ifosfamide/carboplatin/etoposide chemotherapy.

LESSON: Synovial sarcoma should be considered in the differential diagnosis of pediatric oropharyngeal submucosal tumors. Multimodal MRI may aid diagnosis, although the final diagnosis should be based on the postoperative pathological examination findings.

Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

Conflict of interest statement

The authors have no funding and conflicts of interest to disclose.

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