Rathore Rita, Ahuja Sana, Gautam Akansha, Chohan Shikhar, Zaheer Sufian
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Indian J Otolaryngol Head Neck Surg. 2025 Apr;77(4):1903-1906. doi: 10.1007/s12070-025-05405-8. Epub 2025 Mar 3.
Plexiform neurofibroma in the salivary glands is a rare condition, comprising only 0.4% of all salivary gland tumors. This benign but locally invasive tumor, commonly associated with neurofibromatosis type 1 (NF-1), arises from Schwann cells of the peripheral nerve sheath. In this report, we present a case of a 20-year-old male with a massive plexiform neurofibroma involving both the left parotid and submandibular glands. The patient had a painless swelling in the left preauricular region since 2 years, which had gradually enlarged over the last one year. Imaging revealed extensive involvement of the tumor, extending into the parapharyngeal space and infratemporal fossa. A surgical excision was performed, and a histopathological examination confirmed the diagnosis. The extensive size and involvement of multiple salivary glands posed significant challenges in management, emphasizing the need for careful surgical planning and thorough clinical evaluation. This case highlights the rare occurrence of large plexiform neurofibromas in the salivary glands and underscores the complexities involved in treating such extensive tumors.
涎腺丛状神经纤维瘤是一种罕见疾病,仅占所有涎腺肿瘤的0.4%。这种良性但具有局部侵袭性的肿瘤通常与1型神经纤维瘤病(NF-1)相关,起源于周围神经鞘的施万细胞。在本报告中,我们呈现了一例20岁男性患者,患有累及左侧腮腺和下颌下腺的巨大丛状神经纤维瘤。患者自2年前起左侧耳前区出现无痛性肿胀,在过去一年中逐渐增大。影像学检查显示肿瘤广泛累及,延伸至咽旁间隙和颞下窝。进行了手术切除,组织病理学检查确诊。肿瘤的巨大尺寸和累及多个涎腺给治疗带来了重大挑战,强调了仔细的手术规划和全面临床评估的必要性。该病例突出了涎腺中大型丛状神经纤维瘤的罕见性,并强调了治疗此类广泛肿瘤所涉及的复杂性。