Rare Adult-Onset Nasopharyngeal Botryoid Embryonal Rhabdomyosarcoma: A Case Report and Review of Literature


Ceylan F., Tenekecİ A. K., Ünal A. A., Kara E., AĞAÇKIRAN Y., YALÇIN B.

Journal of Oncological Science, cilt.11, sa.3, ss.276-283, 2025 (Scopus, TRDizin)

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 11 Sayı: 3
  • Basım Tarihi: 2025
  • Doi Numarası: 10.37047/jos.galenos.2025.2025-3-3
  • Dergi Adı: Journal of Oncological Science
  • Derginin Tarandığı İndeksler: Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.276-283
  • Anahtar Kelimeler: adult rhabdomyosarcoma, Botryoid embryonal rhabdomyosarcoma, nasopharyngeal tumor, radiotherapy, VAC chemotherapy
  • Sağlık Bilimleri Üniversitesi Adresli: Evet

Özet

Botryoid embryonal rhabdomyosarcoma (RMSs) is a rare malignant tumor typically seen in children, with common sites including the vagina, bladder, and nasopharynx. Its occurrence in adults, is extremely rare, with a very limited number of cases in the nasopharynx. This case report highlights the unusual presentation of the disease and its treatment follow-up. A 46-year-old male presented with a painless neck mass and weight loss. Imaging revealed a parapharyngeal mass with cervical lymphadenopathy, and biopsy confirmed nasopharyngeal botryoid embryonal RMS. Due to the tumor’s proximity to critical structures, surgical resection was not feasible. The patient received vincristine, actinomycin-D, and cyclophosphamide, chemotherapy followed by radiotherapy, achieving complete radiological remission. However, regional recurrence was detected three months post-treatment, necessitating a switch to ifosfamide and etoposide chemotherapy. This case highlights the challenges of diagnosing and treating adult-onset nasopharyngeal botryoid embryonal RMS, emphasizing the importance of vigilant follow-up and tailored treatment strategies. Given its rarity, this report provides valuable insights into the management of adult RMS.