A 53-year-old woman turned to NYU Langone after sinus pain and diplopia led to the diagnosis of a sinonasal undifferentiated carcinoma (SNUC), a rare and high-grade cancer. A multidisciplinary team was assembled to develop a carefully targeted treatment plan with trimodality therapy—preoperative radiation and chemotherapy to reduce the size of the tumor and a targeted surgical approach to enable a successful resection.
Complex Pathology Demands a Unique, Patient-Centered Solution
This patient’s initial symptoms of nasal congestion, facial pain, headaches, and photophobia persisted despite prescribed antibiotics, and the development of double vision led her to a neuro-ophthalmologist, who performed a CT scan revealing a mass invading her orbit.
Upon referral to NYU Langone, an MRI revealed a massive tumor originating in the superior nasal cavity bilaterally, with intracranial and intraorbital extension. A biopsy performed two days later revealed a sinonasal undifferentiated carcinoma.
This diagnosis would typically warrant surgical resection, if feasible, followed by radiation, but the advanced stage of the tumor and its involvement with a number of nearby structures, including the carotid artery, complicated the treatment plan—adding to the life-threatening risk of resection as a first step.
“The tumor involved both orbits and a wide area of dura, so the only way to achieve negative margins would be bilateral orbital exenterations and a large dural resection—clearly not a reasonable option,” recalls Seth M. Lieberman, MD, assistant professor in the Department of Otolaryngology—Head and Neck Surgery. Upfront treatment with chemoradiation to shrink the tumor was identified as the best option for the patient, and a multidisciplinary team was assembled to plan the optimal approach.
Balancing Tumor Eradication with Preservation of Critical Structures
Despite the risks of poor response, continued tumor progression, and poor wound healing, presurgical radiation was the only way to balance tumor eradication with preservation of the patient’s quality of life.
“For patients unable to undergo resection, the dose needed to eradicate the tumor would put the patient at significant risk of visual loss and even brain necrosis,” explains Kenneth S. Hu, MD, associate professor of radiation oncology and otolaryngology—head and neck surgery. “Working in conjunction with a highly skilled skull base surgical team allowed us to use a lower dose of preoperative radiation, which would shrink the tumor while also protecting the eye, brain stem, and brain. Additionally, an involved lymph node was treated with full-dose radiation rather than surgery, negating risk to the carotid artery.”
To help further mitigate risks and preserve critical structures, the team implemented an image-guided approach using daily cone-beam CT imaging as well as mid-treatment adaptive replanning to account for tumor shrinkage. The patient began an aggressive course of titrated chemoradiation for six weeks, followed by six weeks of recovery prior to surgery.
Building Expertise Through Practical Teamwork to Optimize Outcomes
With post-radiation imaging confirming tumor regression from the orbit and nasal cavity, Dr. Lieberman and Donato R. Pacione, MD, assistant professor of neurosurgery, began preoperative planning, opting for an endoscope-assisted craniofacial resection, which would shorten recovery time and avoid a disfiguring transfacial incision, potential mid-facial degloving, and facial osteotomies associated with a traditional open approach.
Dr. Lieberman began the endoscope-assisted craniofacial resection by evaluating both nasal cavities. With no evidence of gross tumor present, the anterior cranial fossa was exposed via bilateral extended sphenoethmoidectomies and frontal sinusotomies. Dr. Lieberman exposed the dural base from orbit to orbit, and crista galli to planum sphenoidale. He also obtained periorbital specimens, which were negative for tumor.
Cognizant that extensive resection of the dural margins would be necessary, Dr. Pacione prepared for a bifrontal craniotomy, to be performed in conjunction with the endonasal resection in order to gain access to the entire region of dural involvement. The olfactory nerves, which were also involved with the tumor, were dissected from the frontal lobe and incised. The dura was then incised, followed by removal of the crista galli and en bloc removal of the entire anterior skull base dura, olfactory nerves, olfactory bulbs, and remaining attachments, with all specimens negative for tumor.
A fascia lata graft and fat were then harvested from the patient's thigh, and the skull base was reconstructed in a multilayer fashion. The endonasal approach was again used to adjust the inlay graft of fascia lata posteriorly over the bony ridge, before Dr. Lieberman raised a vascularized flap from the nasal floor and inferior septum to reinforce the repair.
Although no viable tumor remained after chemoradiation, the decision to perform an open craniotomy allowed excision of wider dural margins and avoided a potentially complicated post-radiation skull base reconstruction. At the same time, the endoscopic approach to the nasal portion of the tumor allowed the patient to avoid a facial incision.
Collaborative Effort Optimizes Surgical Outcome with Preservation of Function
With margins in full view, surgeons performed a successful resection, avoiding injury to the carotid artery and optic nerves—a remarkable outcome considering the typical trajectory of this tumor type.
“Without our team approach, which engaged this aggressive cancer from a number of angles, we have given this patient a chance at life, though she will require vigilant surveillance to ensure that the cancer does not recur at the primary site or from metastatic disease,” notes Dr. Lieberman. “We were able to pool our expertise and develop a multifaceted plan that circumvented the limitations of conventional treatment, resulting in total eradication of the tumor without functional compromise.”