2025, Vol. 7, Issue 2, Part D
An Exceptionally Rare Case of Palatal Botryoid Rhabdomyosarcoma in a Child
Author(s): Khadhar Aida, Chrigui Maha, Makhlouf Nadia, Lahmar Rihab, Azouz Haifa, Helal Imen, Chelly Ines, Slim Haouet and Zehani Alia
Abstract: Background: Rhabdomyosarcoma (RMS) is the most common malignant mesenchymal tumor in children and adolescents, representing approximately 5% of all pediatric solid tumors. Although the head and neck region is a frequent site for botryoid RMS its occurrence in the oral cavity particularly on the palate is extremely rare.
Case presentation: We report the case of a 6-year-old patient who presented with a 3 cm nodular lesion on the palate. Histopathological analysis of the biopsy specimen revealed a malignant mesenchymal tumor showing a cambium layer of poorly differentiated, round or spindled cells arranged in a desmoplastic stroma. Immunohistochemical study demonstrated strong and diffuse positivity for Desmin with focal positivity for Myogenin. The Ki-67 proliferation index was estimated at 100%. These findings confirmed the diagnosis of botryoid variant of RMS.
Conclusion: This case underscores the diagnostic challenges associated with rare palatal botryoid RMS and highlights the necessity for early diagnosis using combined histopathological and immunohistochemical approaches. A personalized multimodal treatment strategy, including surgery, chemotherapy and radiotherapy when appropriate, is critical for achieving favorable outcomes. Reporting such rare cases is essential to enrich the literature and refine management protocols for these unusual presentations.
DOI: 10.22271/27081494.2025.v7.i2d.225Pages: 242-245 | Views: 300 | Downloads: 83Download Full Article: Click Here
How to cite this article:
Khadhar Aida, Chrigui Maha, Makhlouf Nadia, Lahmar Rihab, Azouz Haifa, Helal Imen, Chelly Ines, Slim Haouet, Zehani Alia.
An Exceptionally Rare Case of Palatal Botryoid Rhabdomyosarcoma in a Child. Int J Case Rep Surg 2025;7(2):242-245. DOI:
10.22271/27081494.2025.v7.i2d.225
