What is juvenile nasopharyngeal angiofibroma?

Juvenile nasopharyngeal angiofibroma Introduction

When it comes to “tumors,” it always freaks people out. But the key is to understand them well to catch them early and deal with them properly. nasopharyngeal angiofibroma usually pop up in young guys. These angiofibroma are full of blood vessels and tend to bleed easily, earning them the nickname “juvenile nasopharyngeal angiofibroma” .They usually stop growing after age 25. These angiofibroma bleed easily, causing nose and mouth bleeding with bright red blood, sometimes in large amounts.

What is juvenile nasopharyngeal angiofibroma?
What is juvenile nasopharyngeal angiofibroma?

Due to frequent heavy bleeding, patients can become anemic to varying degrees. As the tumor grows, it can block the nostrils, invade the nasal cavity, leading to congestion, runny nose, nasal blockage, reduced sense of smell; pressing on the Eustachian tube can cause ringing in the ears, ear fullness, and hearing loss. Because the tumor can press on and squeeze surrounding tissues, it can cause complications in the ears, eyes, and even the brain, sometimes becoming life-threatening. So, if diagnosed with juvenile nasopharyngeal angiofibroma, it’s best to get surgery done early.

Symptoms of juvenile nasopharyngeal angiofibroma:

The signs of juvenile nasopharyngeal angiofibroma can vary depending on where they are, how big they are, how fast they grow, which way they’re spreading, and if there are any other issues.

  1. Keep getting nosebleeds: This is a big one. Small angiofibroma in the nasopharynx might not cause much bleeding at first, maybe just a bit of blood in your snot. But as they get bigger, you might have nosebleeds that keep coming back, sometimes even spitting out blood. The bleeding can be a lot, sometimes hundreds of milliliters, and it’s hard to stop, even with packing. And all that bleeding can lead to anemia.
  2. Feeling more and more stuffed up: As the polyps grow and block the back of your nose, you might feel like one or both sides of your nose are all stuffed up. When it’s really bad, you might have to breathe through your mouth, snore at night, talk with a stuffy nose, and have a dry throat.
  3. Pressure on nearby organs: If the polyps press on the opening of the Eustachian tube in your throat, you could get ringing in your ears, earaches, or hearing loss. If they mess with the base of your skull or press on your cranial nerves, you might get headaches or even have problems with those nerves. And if the polyps invade your eye socket, cheekbone area, or the space under your temple, you could end up with bulging eyes, vision problems, swollen cheeks or temples, and sharp pains in your face. If a big polyp pushes into your throat, it could make your soft palate swell up and make it hard to eat.

Diagnosis key points of juvenile nasopharyngeal angiofibroma:

  1. Clinically, recurrent epistaxis and progressive nasal obstruction are two basic symptoms.
  2. Obstruction of the Eustachian tube or nasal sinuses can cause corresponding symptoms such as headache, dizziness, and decreased sense of smell.
  3. Invasion of the orbit or compression of cranial nerves can lead to exophthalmos, visual impairment, diplopia, and other symptoms.
  4. Nasal endoscopy: A pinkish mass can be seen in the nasopharynx, with dilated blood vessels on its surface, and deviation of the nasal septum to the opposite side.
  5. Other imaging examinations:
    • X-ray: Lateral cervical spine X-ray may reveal soft tissue mass in the nasopharynx, with a smooth lower border. The mass often extends upwards causing erosion of the pterygoid process, posterior nasal aperture, and the base of the sphenoid bone. Sometimes, destruction of the skull base may be visible.
    • Carotid angiography: Can show dilated blood supply arteries, vascular displacement, tumor staining, but no venous filling.
    • MRI: T1-weighted images show iso- or slightly high signal, T2-weighted images show high signal. In cases of hemorrhage, both T1-weighted and T2-weighted images show high signal.

CT findings of juvenile nasopharyngeal angiofibroma:

  1. On CT plain scan, there is a round, oval, or lobulated soft tissue mass shadow in the nasopharynx or pterygopalatine fossa, with iso-density and relatively clear borders.
  2. The tumor is located in the pterygopalatine fossa, compressing the posterior wall of the maxillary sinus, causing it to bend and deform, and moving forward, enlarging the pterygopalatine fossa, which is a characteristic feature of this condition.
  3. Due to the abundant blood vessels in the tumor, the lesion shows significant enhancement on contrast-enhanced scans, with CT values exceeding 100 HU, more than 40 HU higher than on plain scans
  4. As the tumor grows, it can invade the orbit, maxillary sinus, and entire nasal cavity, causing deformation and compression of surrounding bone structures, and even bone destruction. Coronal scans can help determine if the tumor has invaded the paranasal sinuses, orbit, or intracranial space.
  5. Differential diagnosis:
    • Nasal polyps: Soft tissue filling in the nasal cavity and nasopharynx, lower density compared to muscle tissue, and no significant enhancement on contrast-enhanced scans.
    • Nasopharyngeal carcinoma: Invasive growth of the tumor, unclear lesion borders, visible skull bone destruction, and weaker enhancement on contrast-enhanced scans compared to angiofibroma.

Staging of juvenile nasopharyngeal angiofibroma:

There are several staging systems for juvenile nasopharyngeal angiofibroma, including Close staging, Radkowski staging, Chandle staging, Sessions staging, and Fisch staging. Among them, Radkowski staging is a newly recommended staging system that evolved from Sessions staging, emphasizing the importance of posterior invasion into the pterygoid plates and skull base.

The staging is as follows: Stage I (A) located in the nasopharynx and nasal cavity, Stage I (B) tumor invading the nasal sinuses; Stage II (A) minimal invasion of the pterygopalatine fossa, Stage II (B) complete invasion of the pterygopalatine fossa, with or without orbital bone destruction; Stage II (C) extending posteriorly to the pterygoid plates; Stage III (A) skull base destruction, limited intracranial invasion, Stage III (B) skull base destruction, extensive intracranial invasion, with or without involvement of the cavernous sinus. The staging system for nasopharyngeal angiofibroma plays an important role in the selection of surgical approach and prognosis assessment.

Differential diagnosis of juvenile nasopharyngeal angiofibroma:

  1. Posterior nasal polyps in the maxillary sinus: This condition is more common in adolescents, with no gender difference in incidence. Patients may experience recurrent unilateral nasal bleeding accompanied by nasal congestion, with a long course and slow progression. Inside the nasal cavity, there may be dark red, easily bleeding masses, sometimes with swelling in the cheek area. However, soft tissue shadows can be seen in the maxillary sinus, with sinus wall showing expansive changes. The edges of the mass are usually smooth, and there may be signs of compressive bone absorption, destruction, or sclerosis, with mild or no enhancement on enhanced scans.
  2. Malignant tumors in the nasopharynx: Patients with this condition may also have progressive nasal congestion and intermittent nasal bleeding, but they are generally older, and the surface of the mass is often not smooth. CT scans show unclear boundaries between the mass and surrounding tissues, indicating infiltrative growth, associated with surrounding bone destruction and often with cervical lymph node metastasis, with generally only mild enhancement.
  3. Chordoma: This condition can also present with progressive nasal congestion and intermittent nasal bleeding, often accompanied by headaches. As the tumor grows, eye and cranial nerve symptoms may appear. On CT scans, round or irregular slightly high-density masses are visible, with scattered punctate or patchy high-density calcifications. The lesion borders are clear, with evident bone destruction. After contrast enhancement, the tumor shows homogeneous or heterogeneous enhancement.

DSA examination ,Complications and prevention of embolization:

1.Why perform preoperative embolization?

The blood supply of nasopharyngeal angiofibroma mostly comes from the external carotid artery system, with the maxillary artery being the most common supplier. Blood supply can also come from the ascending pharyngeal artery, facial artery palatal branch, middle meningeal artery, and branches of the posterior cerebral artery in the internal carotid artery system, sometimes from multiple vessels. As the disease progresses, intraoperative bleeding significantly increases. Preoperative angiography helps understand the tumor’s location, size, extent, and blood supply, providing direct guidance for further embolization and serving as an important basis for selecting surgical approaches and plans. Surgery should be performed 1-3 days after embolization to prevent the establishment of collateral circulation, which can increase surgical bleeding.

These symptoms typically do not require special treatment and usually resolve after surgery. The severe complications of external carotid artery embolization mainly involve embolus reflux or misembolization into the internal carotid artery system, leading to cerebral embolism, facial paralysis, and blindness.

To prevent such serious complications, during the procedure: perform bilateral carotid and vertebral artery angiography before vascular embolization, understand the blood supply characteristics of the head and neck, analyze pathological collateral or anastomotic branches; choose embolic agents rationally; inject embolic agents slowly, evenly, in small amounts, and multiple times to prevent embolus reflux, superflow, or the opening of pressure-induced anastomotic branches; immediately stop injection if slow embolus migration, opening of “dangerous anastomotic” branches, or neurological symptoms in the patient are observed.

Treatment of nasopharyngeal angiofibroma:

Surgical treatment is necessary for angiofibroma to completely remove the tumor, prevent major bleeding, and local complications. Surgical approaches include external nasal routes (such as transpalatal route for nasopharyngeal angiofibroma resection) and endoscopic surgery. For those temporarily ineligible for surgery, treatment options may include radiation therapy, sclerosing agent injection, and oral steroids.

Unlike malignant tumors such as lung cancer, nasopharyngeal angiofibroma is usually not fatal.Patients can choose to receive treatment at the nearest hospital.

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