Facial disfigurement due to olfactory neuroblastoma: beauty regained with chemotherapy

Abstract Background Olfactory neuroblastoma (ONB) is a sinonasal malignancy seldom seen in clinical practice. It is also known by various other names like esthesioneuroblastoma, esthesioneuroepithelioma, esthesioneurocytoma, and esthesioneuroma. Surgery and radiation therapy are considered as standard treatment modalities for ONB; however, the role of chemotherapy is not well established. Aims We aim to define the role of chemotherapy in the neoadjuvant setting in a case of ONB. Methods and Results We report a young female patient presenting with a naso‐facial swelling causing facial disfigurement, proptosis, decreased visual acuity, and poor performance status. She was diagnosed with advanced‐stage ONB. Prompt administration of chemotherapy led to the improvement in the symptoms and rapid regression of the tumor mass. Later on, the tumor mass was excised completely without any neurological deficit. Conclusion This report justifies the role of neoadjuvant chemotherapy in the management of ONB.


| INTRODUCTION
Olfactory neuroblastoma (ONB) is an uncommon malignancy of neuroectodermal origin. It usually arises from the sensory neuroectodermal olfactory cells situated in the nasal cavity. The estimated incidence is 0.4 cases per million populations. 1 The paucity of cases does not allow a prospective clinical trial in search of a standard treatment protocol. Moreover, the biology of the disease is highly variable ranging from slow-growing indolent tumors to locally aggressive, and metastatic lesions. This leads to inconsistency in the recommended treatment protocols. Surgical resection followed by radiation therapy has been considered as the optimum treatment protocol for most of the cases of ONB; however, the role of chemotherapy is not clearly established. We report a case of aggressive ONB in a young female. The aggressive nature of the disease led to her facial disfigurement with an imminent threat to loss of vision. Expedient administration of chemotherapy led to regaining her normal facial features and restoration of normal vision.

| Case
A 32-year-old female from a remote hilly village presented with an 8-week history of progressive swelling around the nose, and severe bouts of headaches. For the last 2 weeks, she was also experiencing episodes of recurrent epistaxis, diminution of vision, and double vision. According to her husband, the disease had rapidly progressed in the last 4 weeks leading to severe facial disfigurement and now it had become difficult to recognize her. She was unable to do household chores and self-care, being mostly confined to bed. On examination, the patient had a swelling involving the whole of the nose with facial puffiness and proptosis of the right eye. The right vestibule of the nose was full of some necrotic appearing mass. Bilateral level I and II neck nodes were enlarged. She had marked anhedonia and was chair-bound, not responding to any queries. Her case was discussed in the multidisciplinary tumor board.
Since it was very difficult to achieve an R0 resection without much morbidity and neurological deficits, neoadjuvant chemotherapy (NACT) was planned. However, in a patient with poor performance status (PS) and severe depression, it was difficult to administer chemotherapy. The patient's husband was counseled in detail regarding the guarded prognosis and pros and cons of chemotherapy and he consented for the treatment.
Tumor lysis prophylaxis in the form of allopurinol was given. We administered a 3-day protocol of cisplatin (25 mg/m 2 ) and etoposide (75 mg/m 2 ); however, the dose was reduced by 15% keeping in view The dose of chemotherapy was optimized and she received four cycles of the same protocol without any significant adverse effects. MRI done for response evaluation after four cycles was suggestive of a very good response to therapy (Figure 1, panel B). She was referred to the head and neck surgeon for surgical management. The patient underwent an endoscopic craniofascial resection of the tumor via modified Denker's approach. Intraoperatively, pinkish, polypoidal soft tissue mass in the right nasal cavity and b/l maxillary, ethmoid, sphenoid sinus with obliteration of frontal recess with mucocele formation was noted. Bony dehiscence was noted over ipsilateral lamina papyracea, cribriform plate, planum sphenoidale, and optic nerve and suprachiasmatic region, however, dura and periorbita were found intact. The tumor was excised in piece meal using debrider and coblation. The soft tissue adherent to the cribriform region and planum was sent for the frozen section, which was negative.
The peri-operative period was uneventful. There was no neurological deficit. Post-operative histopathological examination of the resected tumor mass was suggestive of pathological complete response. She received two more cycles of the same protocol as the adjuvant therapy and subsequently referred to the radiation oncologist for adjuvant radiation therapy. however, not well defined. 13,14 Our case has several salient features. The patient had a poor PS and she was in severe depression. Administration of chemotherapy under such circumstances is difficult as it may do more harm than benefit. However, a cautious decision was taken as biology and history of the tumor suggested that the tumor might be responsive to chemotherapy. The patient regained her facial features which were otherwise lost due to disfigurement caused by the expansile tumor.

| DISCUSSION
Her vision was restored. She achieved a PCR which has not been reported in ONB.

| CONCLUSIONS
1. ONB can present like a locally aggressive tumor, and prompt evaluation and treatment is necessary to prevent disease-related morbidities.
2. The tumor is highly sensitive to chemotherapy and NACT should be administered to downsize the tumor amenable to R0 resection. 3. A cautious trial of chemotherapy can be done in patients with highly chemosensitive tumors who are otherwise unfit for systemic anti-cancer therapy due to poor PS or comorbidities.

PATIENT'S PERSPECTIVE
My wife was very sick and in great depression for the last 1 month. I had lost all hopes. I was very skeptical about chemotherapy as I had many myths regarding the ill-effects of chemotherapy. The treating team explained to me about the treatment and I showed faith in them.
Her condition started improving remarkably within a few days after starting chemotherapy. Her vision has become normal. She has now completed her chemotherapy and surgery and we are thankful to the treating team.