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<p>Desmoid fibromatosis is characterized by locally aggressive monoclonal fibroblast proliferation. Although these tumors do not metastasize, they demonstrate local invasion, resulting in significant morbidity and a high rate of local recurrence after excision. For the first time in Vietnam, we identified a very rare case of desmoid fibromatosis in otolaryngology. A 17-year-old male patient was diagnosed with juvenile nasopharyngeal angiofibroma (JNA) based on the presentation of typical syndromes. However, pathological analysis and immunostaining confirmed a rare case of desmoid fibromatosis of the nasopharynx and paranasal sinuses. Our report highlights the importance of considering rare differential diagnoses, even when the initial presentation is typical of JNA. Furthermore, this case raises awareness of the necessity for prompt diagnosis and timely and appropriate management.</p>

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A Case Report on the Differential Diagnosis of a Desmoid Fibromatosis Initially Diagnosed as Nasopharyngeal Angiofibroma

  • 期刊论文

  • 发表日期：

    2026年3月9日

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  • Hao Thi Nguyen,
  • Luong Trung Vu,
  • Dinh Cong Le

摘要

Desmoid fibromatosis is characterized by locally aggressive monoclonal fibroblast proliferation. Although these tumors do not metastasize, they demonstrate local invasion, resulting in significant morbidity and a high rate of local recurrence after excision. For the first time in Vietnam, we identified a very rare case of desmoid fibromatosis in otolaryngology. A 17-year-old male patient was diagnosed with juvenile nasopharyngeal angiofibroma (JNA) based on the presentation of typical syndromes. However, pathological analysis and immunostaining confirmed a rare case of desmoid fibromatosis of the nasopharynx and paranasal sinuses. Our report highlights the importance of considering rare differential diagnoses, even when the initial presentation is typical of JNA. Furthermore, this case raises awareness of the necessity for prompt diagnosis and timely and appropriate management.