Esthesioneuroblastoma

A rare form of cancer called esthesioneuroblastoma starts in the upper part of the nasal cavity. A bone that separates the beginning of the esthesioneuroblastoma from the brain has tiny holes in it that the olfactory nerves, which control smell, can pass through. Olfactory neuroblastoma is another name for esthesioneuroblastoma.

Adults of any age can develop esthesioneuroblastoma, which typically starts as a nasal tumor and can spread or grow into the sinuses, eyes, and brain. As the tumor grows, people with esthesioneuroblastoma may lose their sense of smell, experience frequent nosebleeds, and have trouble breathing through their nostrils.

Moreover, parotid glands and neck lymph nodes may become affected by esthesioneuroblastoma.

Esthesioneuroblastoma: Causes

Esthesioneuroblastoma's actual etiology is yet unknown. Cancer typically starts with a genetic mutation that allows healthy, normal cells to keep growing despite signals to stop, in contrast to what healthy cells normally do. Unchecked growth and proliferation occur in cancer cells. A mass of aberrant cells develops as they accumulate (tumor).

Esthesioneuroblastoma: Symptoms

In terms of clinical presentation, esthesioneuroblastoma frequently mimics benign inflammatory or viral diseases, and delays in diagnosis are not unusual. Early indications such as nasal obstruction and epistaxis are normal; however, depending on the location and size of the tumor, additional, more specialized symptoms may also appear.

The diagnosis of esthesioneuroblastoma may be made several years before anosmia. Additional symptoms include signs of incorrect antidiuretic hormone production, expansion into the paranasal sinuses (facial edema, medial canthus mass), the orbit (orbital discomfort, proptosis, epiphora, visual field abnormalities), or anterior cranial fossa (headache).

Esthesioneuroblastoma complications might involve −

• A tumor that spreads to surrounding structures. If advanced neuroblastoma enlarges to the point where it invades neighboring structures like the brain or eyes, difficulties may result.

• Cancer's spread (metastasis). Esthesioneuroblastoma can spread (metastasize) to the lymph nodes, bone marrow, lungs, liver, skin, and bones, among other organs.

• Loss of smell perception. The nasal channel may become blocked by the tumor, which may stop airflow via the nose.

• Problems following surgery. These side effects might include abscess, infection, visual issues, and spinal fluid leaks.

Esthesioneuroblastoma: Risk Factors

Several factors play an important role in the development of esthesioneuroblastoma which includes −

An esthesioneuroblastoma cannot be predicted to develop due to any known risk factors.

Esthesioneuroblastoma: Diagnosis

The diagnosis of esthesioneuroblastoma is mainly done based on history and some of the tests may be required for confirmation and to rule out underlying causes −

• Inspection of the body. Doctors can determine the size of the tumor and assist in the diagnosis by carefully noting your signs and symptoms and having your eyes, nose, head, and neck examined.

• Seeing into your nose using a small, bendable camera. An endoscope, a small, flexible tube, is put inside your nose during an endoscopic examination. Doctors can examine the size of the tumor in the nasal cavity's sinus region, as well as in the back of the nose (nasopharynx), thanks to the tube's attachment to a camera.

• Image-based exams. Your esthesioneuroblastoma's location, extent, and potential spread can all be determined with the aid of imaging tests like MRI and positron emission tomography (PET).

• Removing a tissue sample for analysis. An operation called a biopsy is used to take a sample of tumor tissue for pathological examination. A little bit of the malignancy may be removed during a biopsy by introducing specialized equipment via your nose. Most of the time, a doctor's office can do this treatment.

• Lab examination of a tissue sample. To differentiate between esthesioneuroblastoma and other cancers that could have a similar appearance, accurate testing and analysis of the biopsy sample are required. Although esthesioneuroblastoma is extremely uncommon and can resemble other tumors that develop in the head, neck, or nasal regions, diagnosing it can be challenging. The aggressiveness (grade) of the tumor is also determined by the pathological investigation.

Esthesioneuroblastoma: Treatment

The treatment is based on the severity of the symptoms. Your doctor may advise conservative or surgical treatment.

Conservative Treatment

The use of radiation − To destroy cancer cells, radiation treatment employs high-energy beams like X-rays or protons. After surgery, patients with epithelioblastoma frequently get radiation therapy to eradicate any tiny cancer cells that could have survived in the head and neck.

If surgery is not an option because of other health issues or if the cancer is too advanced to be eliminated by surgery, radiation treatment may be performed instead of or in addition to chemotherapy.

Chemotherapy − Chemotherapy uses medication to destroy cancer cells. After surgery, chemotherapy and radiation therapy are given to patients with esthesioneuroblastoma to eradicate any cancer cells that could have survived, particularly if the tumor was extremely aggressive or widespread.

Surgical Treatment

Treatment for esthesioneuroblastoma often includes surgery to remove the malignancy. Other treatments include chemotherapy using potent medicines and radiation using high-powered energy beams.

Surgical methods vary based on where the tumor is located and often involve actions taken by −

• Removing the tumor’s nasal portion. Typically, endoscopic surgery is used for this. To examine cancer, the surgeon inserts an endoscope via the nose, which is a long, thin tube with a camera on one end. To see the region and help with the excision of cancer and surrounding tissue, special surgical instruments are passed via the endoscope.

• Accessing the tumor by opening the skull. To remove the tumor and separate it from the brain, craniotomy is a technique that involves removing a tiny part of the skull.

Experts from a variety of disciplines, including neurosurgeons, head and neck surgeons, radiation oncologists, and medical oncologists, are typically involved in the treatment of esthesioneuroblastoma.

Esthesioneuroblastoma: Prevention

Some of the measures that can help to prevent esthesioneuroblastoma include −

• Stop smoking

• Limit alcohol use

• Limit weight gain

• Exercise

• Using antioxidants

Conclusion

Esthesioneuroblastoma is a rare condition that necessitates international and multi-institutional cooperation. For the greatest results, an interprofessional team approach is used. The best chance of recruiting more patients and using a standardized database for information collection and outcome reporting would be provided by centralizing the processing of instances of embryo-neuroblastoma.

Because of the aggressiveness of esthesioneuroblastoma, early identification is essential to choose the best course of therapy. Every patient with a suspected nasal mass should be quickly sent to an otolaryngologist for further testing by the primary care physician and nurse practitioner.

Otolaryngologists, neurosurgeons, radiation oncologists, pathologists, radiologists, and oncologists are the team members needed for the complicated management of esthesioneuroblastoma, with support from nursing and pharmacy as needed as therapy progresses. Pharmacists and specialty care nurses can be crucial members of the team. Pharmacy professionals evaluate prescriptions, look for drug-drug interactions, and educate patients. Oncology and otolaryngology specialty-trained nurses participate in the initial assessment, monitor patients, educate patients, and provide the team with up-to-date information.