Nasopharyngeal Angiofibroma - Symptoms, Diagnosis, and Treatment

Introduction

Hippocrates wrote the first documented account of nasopharyngeal angiofibroma (NA) in the fifth century Before Christ (BC). The term "nasopharyngeal" may not be accurate; some sources claim it originates in the posterior nasal cavity and the sphenopalatine foramen, while others suggest it is more closely related to the choanal and nasopharyngeal regions. The consensus among researchers is that nasopharyngeal angiofibroma is a highly vascular benign lesion that accounts for 0.05 to 0.5 percent of all head and neck tumors.

Despite being histologically benign, it frequently exhibits aggressive characteristics, including local invasion into the medial pterygoid lamina, nasal septum, and nasal turbinates. Larger lesions can spread into the sphenoid, maxillary, and ethmoid sinuses. It often extends into the nasal cavity, nasopharynx, and pterygopalatine fossa. The objective of this article is to present a thorough review of this disorder, including clinical characteristics, diagnostic techniques, available treatments, and related terminology.

What Is Nasopharyngeal Angiofibroma?

Angiofibroma of the nasal cavity, also referred to as juvenile nasal angiofibroma, fibromatous hamartoma, and angiofibromatous hamartoma, is called nasopharyngeal angiofibroma. It is a vascular tumor of the nasopharynx that develops at the back of the nasal cavity and originates from the superior edge of the sphenopalatine foramen. Histologically benign, it is locally aggressive. Adolescent guys are the most typically affected since it is a hormone-sensitive tumor. Despite being benign, the tumor can spread locally and cause damage to the nose, cheek, orbit (causing a deformity similar to a frog's face), or brain.

What Are the Clinical Features of Nasopharyngeal Angiofibroma?

• Gender Predilection and Age:

  • The majority of patients with nasopharyngeal angiofibroma are male teenagers.

  • Puberty is usually when it first appears, and most instances are diagnosed between the ages of 14 and 25.

• Location:

  • The superior portion of the nasopharynx and the posterior nasal cavity are the tumor's places of genesis.

  • It frequently grows into the nasal cavity and associated tissues from the sphenopalatine foramen.

• Impact of Hormones: The growth of nasopharyngeal angiofibroma is linked to puberty and is susceptible to hormones. The tumor's androgen receptors imply that hormones have a role in its growth.

• Epistaxis (Bleeding Nose): Epistaxis can occur on its own or be brought on by trauma.

• Nasal Blockage: Nasal congestion and blockage may result from the tumor as it develops.

• Pain and Swelling in the Face: Pain and swelling in the face might be caused by the tumor's local invasion of nearby structures.

• Proptosis (The Shifting of the Eyes): The tumor could spread into the orbit, causing the eye to shift or proptosis.

• Vision-Related Symptoms: If the tumor compresses or invades adjacent tissues that impact the eyes, visual problems may result.

• Mass of the Palatal Region: A palpable lump on the palate may indicate that the tumor has spread into the nasopharynx in certain instances.

• Disturbances in Sleep and Snoring: The nasopharyngeal tumor may factor in snoring and other sleep disorders.

• Face Malformation: Large or long-standing tumors that affect the face bones can distort the face.

• Recurring Infections: People with nasopharyngeal angiofibroma may be more susceptible to recurrent upper respiratory infections.

What Are the Causes of Nasopharyngeal Angiofibroma?

The cause is unknown, likely a combination of factors:

• Embryonic remnants.

• Hormonal influences (androgens promote growth, estrogens inhibit).

• Familial adenomatous polyposis (FAP).

• Human papillomavirus (HPV).

• Environmental factors (radiation, chemicals).

• Research is ongoing, and no single cause has been identified.

What Is the Diagnosis for Nasopharyngeal Angiofibroma?

Nasopharyngeal angiofibroma diagnosis requires a thorough process:

• Clinical Evaluation:

  • Comprehensive medical history to comprehend symptoms such as face discomfort, nasal blockage, and recurrent epistaxis.

  • Physical examination to look for facial abnormalities, bleeding, or lumps.

• Imaging:

  • CT scan to evaluate the size of the tumor and the bone structures in detail.

  • MRI to assess vascularity and soft tissue involvement.

  • Angiography (specifically, digital subtraction angiography) to determine the arterial supply for embolization and surgical planning.

• Endoscopy: Using nasal endoscopy to take samples and see the tumor up close.

• Histology and Biopsy: Tissue sample for microscopic analysis to validate nasopharyngeal angiofibroma's distinctive characteristics.

• Analysis of Hormone Receptors: Androgen receptors are evaluated to comprehend hormonal responses.

What Is the Treatment for Nasopharyngeal Angiofibroma?

A multidisciplinary strategy is usually used to treat nasopharyngeal angiofibroma (NPA), considering the tumor's location, vascularity, and likelihood of recurrence. Below is a summary of the available treatment options:

• Surgical Procedure:

  • Endoscopic Surgery: In many cases of nasopharyngeal angiofibroma, endoscopic resection is the recommended surgical procedure. To reach and remove the tumor with the fewest external incisions possible, a nasal endoscope is used. This method decreases surgical complications and speeds up recovery.

  • Open Surgery: When endoscopic surgery is not an option, especially for bigger tumors, open surgical techniques may be taken into consideration. These methods require making exterior incisions to access and excise the tumor.

• Preoperative Embolization: To cut off the tumor's blood supply, embolization may be done before surgery. This promotes a safer surgical process and reduces intraoperative hemorrhage.

• Adjuvant Radiotherapy: Radiotherapy may be considered in some circumstances, especially if the tumor is persistent or recurring or if full surgical excision carries significant hazards. For most instances, it is not the main therapeutic option, though.

• Hormone Treatment:

  • Androgen Receptor Blockers: As the tumor is hormone-responsive, certain instances may require hormonal therapy, in particular, androgen receptor blockers.

• Monitoring and Sustaining: Long-term follow-up and monitoring are crucial due to the possibility of recurrence. This entails routine imaging tests and clinical evaluations to look for any indications of tumor recurrence.

• Symptomatic Treatment:

  • Management of Epistaxis: A key component of the overall care for patients with nasopharyngeal angiofibroma is symptomatic therapy, which includes steps to prevent and control recurrent epistaxis.

What Is the Prognosis for Kids With Nasopharyngeal Angiofibroma in the Long Run?

Despite the complexity of nasopharyngeal angiofibroma, long-term results are often rather favorable. Medical or surgical treatments may have adverse effects, but a skilled interdisciplinary team works to reduce them. It should not ever result in death.

Conclusion

In conclusion, teenage boys are the main population affected by nasopharyngeal angiofibroma, an uncommon but clinically important tumor. A thorough awareness of clinical characteristics, diagnostic techniques, and available treatments is necessary for its management. A team effort comprising many medical specializations is necessary to guarantee the best possible patient outcomes. Ongoing research and developments in diagnostic and therapeutic techniques can better comprehend and improve this.