Esthesioneuroblastoma - Symptoms and causes

Esthesioneuroblastoma, a.k.a olfactory neuroblastoma, is a rare & aggressive cancer that originates in the upper part of the nasal cavity, particularly in the olfactory epithelium (tissue responsible for the sense of smell). 

It arises from neuroectodermal cells and is part of small round blue cell tumors. This malignancy is distinct from other nasal or sinus cancers in its origin and histology. 

It can invade nearby structures, including the sinuses, eye sockets, and brain, especially if not detected early. The tumor, though, can occur at any age, is most commonly diagnosed in adolescents and middle-aged adults.

What Are the Symptoms?

• Nasal congestion or blockage (usually on one side)
• Frequent nosebleeds
• Reduced or lost sense of smell
• Facial pain or pressure
• Swelling around the eyes or tearing
• Vision problems, such as double vision
• Headaches
• A noticeable mass inside the nasal cavity
• Loosening or pain in the upper teeth
• Neurological symptoms (in advanced cases) such as seizures or personality changes

Clinical Staging of Esthesioneuroblastoma

Esthesioneuroblastoma is commonly staged using the Kadish staging system, which classifies the tumor based on its anatomical spread. This system helps determine the severity of the disease and guides treatment planning.

• Stage A

The tumor is confined strictly to the nasal cavity without involving any surrounding structures

• Stage B

Tumor extends into one or more of the paranasal sinuses (e.g., ethmoid, maxillary, sphenoid, or frontal) but remains within the sinonasal region

• Stage C

The tumor has extended beyond the nasal cavity and sinuses into surrounding areas, including the orbit, skull base, and intracranial space. It may also involve cervical lymph nodes or show signs of distant metastasis.

Note: Some clinicians also refer to a modified Kadish system that includes a Stage D to represent cases with distant metastasis, although this is not universally adopted.

Complications Linked to Esthesioneuroblastoma

• Invasion into surrounding structures like the orbit or the brain
• Loss of vision or eye movement due to orbital involvement
• Spread to cervical lymph nodes or distant organs.
• Recurrence after treatment
• Intracranial pressure or brain damage in advanced cases
• Side effects from radiation or chemotherapy (e.g., cognitive issues, sinus damage)
• Difficulty breathing or speaking if the tumor obstructs the airways
• Long-term functional impairment depends on tumor location and treatment

How Is Esthesioneuroblastoma Diagnosed?

• Clinical examination

The process begins with a thorough examination of the head and neck. An ENT specialist checks for nasal masses, obstruction, or deformities through anterior rhinoscopy or nasal endoscopy.

• Imaging tests – CT scan

A computed tomography (or CT) scan of the head and sinuses helps assess the size of the tumor, bone involvement, and structural erosion, especially in the nasal cavity, ethmoid sinuses, and skull base.

• Imaging tests – MRI

Magnetic Resonance Imaging (or MRI) is crucial for determining soft tissue involvement, particularly to assess whether the tumor has extended into the brain, eye sockets, or other surrounding structures. It gives a clearer picture than a CT for evaluating intracranial spread.

• PET scan or whole-body imaging

A PET scan or other advanced imaging techniques may be used to detect regional or distant metastases, including those in lymph nodes or other distant organs.

• Biopsy

A tissue biopsy is mandatory for confirming the diagnosis. The sample is collected through nasal endoscopy and sent for histopathological examination.

• Histopathology and immunohistochemistry

The biopsy tissue is analyzed under a microscope. Esthesioneuroblastoma typically appears as a small, round, blue cell tumor. Immunohistochemical markers, such as synaptophysin, chromogranin, and neuron-specific enolase (NSE), aid in distinguishing it from other nasal cancers.

• Hyams' grading system

Once confirmed, the tumor is graded using the Hyams system (Grade I–IV), based on features like cell differentiation, necrosis, mitotic activity, and rosette formation. Higher grades indicate more aggressive disease.

• Spinal fluid analysis (in select cases)

If the tumor has spread near the brain, a lumbar puncture is performed to check cerebrospinal fluid (CSF) for malignant cells.

How Is Esthesioneuroblastoma Treated?

• Surgical removal

Surgery is the primary treatment. The goal is basically to remove the tumor entirely with clear margins. Depending on the extent of spread, this may involve minimally invasive endoscopic surgery or open craniofacial resection if the cancer has invaded the skull base or nearby structures.

• Radiation therapy

Post-surgical radiation is commonly recommended, especially in cases where complete removal isn't possible or if the tumor is high-grade. Radiation helps reduce the risk of recurrence by targeting any residual cancer cells.

• Chemotherapy

Chemotherapy may be used in advanced-stage cases, particularly if the tumor has reached the lymph nodes, brain, or other distant parts of the body. It's also considered when surgery and radiation alone are insufficient.

• Combined approach (multimodal therapy)

Most patients often benefit from a combination of two or more treatments. For instance, surgery followed by radiation, or a sequence of chemotherapy, surgery, and then radiation, depending on the case.

• Ongoing surveillance

After treatment, long-term follow-up is essential. Regular imaging and physical exams help monitor for recurrence, which can happen even years later.

• Re-treatment for recurrence

If the cancer returns, treatment may involve a second surgery, additional radiation, or systemic therapies, based on how and where the recurrence occurs.

Side Effects of Treatment

Treatment for esthesioneuroblastoma can lead to a range of side effects depending on the type and intensity of therapy. Surgery may result in facial swelling, sinus issues, or changes in smell and vision, especially if nearby structures are involved. 

Radiation therapy can cause fatigue, dry mouth, skin irritation, nasal dryness, and long-term damage to nearby nerves or tissues. Chemotherapy may lead to nausea, lowered immunity, and fatigue. In some cases, cognitive changes or hormonal imbalances can occur if treatment affects brain structures. 

Note: Regular follow-ups are crucial for managing and monitoring these effects.

Management

Management focuses on routine monitoring, rehabilitation, and early detection of recurrence. Patients typically require scheduled imaging, such as MRI or CT scans, every few months for the first few years, followed by annual scans. 

Endoscopic nasal exams are done to assess local recurrence. Vision, smell, and neurological functions should be regularly evaluated. Rehabilitation may include physiotherapy, speech therapy, or counseling based on the affected areas. 

If hormone-producing glands were impacted, endocrine follow-up is needed. Any new symptoms such as nasal blockage, headaches, or visual changes must be promptly investigated. A multidisciplinary team manages long-term care, comprising specialists in ENT, oncology, neurology, and endocrinology.

Sterling Hospital is Your Partner in Better Health Across Gujarat

Sterling Hospital has proven itself to be the best cancer hospital in Ahmedabad, Vadodara, and Rajkot. We provide our patients with the utmost care and comfort throughout their battle against cancer while striving to provide them with premium treatments and the best results. 

We've got the bestonco surgeons inRajkot,Vadodara, andAhmedabad, who will be your partner throughout your journey.Contact Sterling Hospital to schedule a consultation and embark on a journey towards a healthier life.