disease | Nasopharyngeal Angiofibroma |
alias | Juvenile Nasopharyngeal Angiofibroma in Male Adolescents |
Nasopharyngeal fibroangioma commonly occurs in young males aged 10-25. Unlike ordinary fibromas, it consists of dense connective tissue, abundant elastic fibers, and blood vessels. Hence, it is also called "juvenile nasopharyngeal angiofibroma," which better reflects the characteristics of the disease. It typically stops growing after the age of 25.
bubble_chart Pathological Changes
The cause of the disease remains unclear and may be related to factors such as sex hormones, developmental abnormalities, and inflammatory stimulation. This condition originates from the fibrous and vascular tissues at the top of the nasopharynx, often presenting as round or oval-shaped masses with a broad base or pedicle. The tumor appears deep red or grayish-red, with a smooth surface covered by epithelium resembling nasal mucosa but lacking a capsule. Beneath the epithelium, markedly dilated blood vessels are visible, with thin walls composed of a single layer of endothelial cells and underlying smooth muscle, lacking elastic tissue, making them prone to significant bleeding due to poor contractility. Although histologically benign, the tumor can progressively enlarge, compressing adjacent bony structures and invading the nasal sinuses, orbits, pterygopalatine fossa, and even eroding the skull base, leading to severe consequences.
bubble_chart Clinical Manifestations
The tumor grows slowly. When confined to the nasopharynx, symptoms are often mild, with possible grade I stuffy nose, occasional hemoptysis or epistaxis, and easy misdiagnosis as fistula disease. As the tumor enlarges, recurrent bleeding of varying amounts may occur, manifesting as nasal bleeding or hematemesis. Due to massive or prolonged bleeding, patients often develop secondary anemia. Tumor enlargement obstructing the posterior nasal cavity worsens stuffy nose, progressing from unilateral to bilateral, leading to mouth breathing and hyponasal speech. Compression of the Eustachian tube orifice causes ear fullness, tinnitus, and hearing loss. Destruction of the skull base and compression of cranial nerves result in headache and cranial nerve palsy, with possible intracranial complications. Invasion into the orbit causes diplopia, tearing, and vision loss; invasion into the pterygopalatine and infratemporal fossae leads to bulging of the ipsilateral cheek and temple; anterior extension into the nasal cavity or sinuses may cause infection, hyposmia, or increased purulent discharge; downward extension may cause bulging of the soft palate, with the tumor visible in the oropharynx.
Examination: After shrinking the inferior turbinate, a red mass can be seen in the posterior nasal cavity. If protruding into the nasal cavity, the affected side may appear enlarged and filled with the tumor, with the septum pushed to the opposite side. Indirect nasopharyngoscopy reveals a round or lobulated, smooth-surfaced tumor on the nasopharyngeal roof, lateral wall, or posterior nasal aperture, with prominent vascular markings. If necessary, nasopharyngeal palpation can be performed with hemostatic preparation, revealing a hard, immobile mass prone to bleeding. Tumor compression of the Eustachian tube orifice may show corresponding signs of tympanic membrane inward invasion, effusion, and other manifestations of catarrhal otitis media. Depending on the tumor's invasion of adjacent areas, signs may include cheek bulging, eyeball displacement, restricted mouth opening, and cranial nerve palsy.Based on symptoms, examination results, gender, and age, a diagnosis can usually be made for typical patients. Due to the high tendency of this tumor to bleed, biopsy is generally not performed. X-rays and CT scans have certain diagnostic value in determining tumor size, direction of extension, and range. Foreign scholars have proposed a radiological staging system based on CT findings: Stage I, the tumor is located in the nasopharynx, posterior nasal aperture, and sphenoid sinus; Stage II, the tumor extends forward into the nasal cavity, ethmoid sinus, maxillary sinus, cheek, and medial orbit or laterally into the pterygomaxillary fossa; Stage III, the tumor extends laterally into the infratemporal fossa and temporal fossa, cheek, and lateral orbit; Stage IV, the tumor extends intracranially. This disease has characteristic clinical manifestations but must be differentiated from malignant nasopharyngeal tumors, fibrous nasal polyps, and posterior nasal polyps. Malignant nasopharyngeal tumors present differently from this disease, with rapid tumor progression, early cervical lymph node metastasis, and diagnosis confirmed by biopsy. Nasal polyps are softer, less prone to bleeding, easily movable on palpation, and often have a stalk originating from the nasal cavity or sinus.
Surgical resection is the primary treatment. Depending on the tumor's location and extent, the transpalatal, transnasal, or a combined approach is typically employed. The transpalatal approach is suitable for larger tumors extending into the oropharynx, with a base at the nasopharyngeal roof and sides, and not extending beyond the posterior nasal aperture. Common routes include midline soft palate splitting, hard palate "U" incision, or tongue-shaped incision. The transnasal approach is only suitable for tumors located at the posterior nasal aperture, extending into the nasal cavity or sinuses, with common methods including lateral rhinotomy or extended lateral rhinotomy. Due to the deep surgical cavity, limited field of view, rapid blood loss, and difficulty in tumor dissection during nasopharyngeal angiofibroma surgery, thorough preoperative preparation is essential. The goal is to completely remove the tumor while minimizing blood loss and preventing complications. Preoperative measures may include tracheostomy or external carotid artery ligation. Controlled hypotensive anesthesia can be used during tumor resection. Although radiotherapy and medications, such as diethylstilbestrol taken for 2–4 weeks, cannot cure the tumor, they may shrink it and reduce intraoperative bleeding. Intratumoral sclerosing agent injections have a similar effect. For smaller tumors, laser cauterization via fiberoptic nasopharyngoscopy can be attempted with favorable results.