Esthesioneuroblastoma with recurrent dural metastases: Long-term multimodality treatment and considerations

Background: Esthesioneuroblastoma (ENB) is a rare malignant disease and treatment protocols have not been standardized, varying widely by disease course and institutional practices. Management typically includes wide local excision through open or endoscopic resection, followed by radiotherapy, chemotherapy, and stereotactic radiosurgery. Tumor control can differ on a case-by-case basis. Herein, the complex management of a rare case of recurrent disease with multiple dural metastases is presented. Case Description: A 60-year-old patient was diagnosed with ENB after presenting with anosmia and epistaxis. The patient underwent combined endonasal and transfrontal sinus craniofacial resection, followed by proton beam radiation therapy and chemotherapy. Subsequently, he developed a total of 25 dural metastases that were controlled with repeated Gamma Knife Radiosurgery (GKRS). In spite of post-treatment course that was complicated by radiation necrosis and local vasculopathy, the patient made significant recovery to functional baseline. Conclusion: The management of ENB entails multimodality and multidisciplinary care, which can help patients obtain disease control and long-term survival. Recurrent ENB dural metastases can behave as oligometastatic disease manageable with aggressive focal GKRS. As prognosis continues to improve, chronic treatment effects of radiation in such cases should be taken into consideration.

Combined Exoscopic and Endoscopic Technique for Craniofacial Resection

We determined the feasibility of the combined exoscopic-endoscopic technique (CEE) as an alternative to the microscope in craniofacial resection (CFR). This retrospective study was conducted at a single institution and included eight consecutive patients with head and neck tumors who underwent CFR between September 2019 and July 2021. During the transcranial approach, microsurgery was performed using an exoscope in the same manner as in traditional microscopic surgery, and an endoscope was used at the blind spot of the exoscope. The exoscope provided images of sufficient quality to perform microsurgery, while the sphenoid sinus lumen was the blind spot of the exoscope during anterior (n = 3) and anterolateral CFR (n = 2), and the medial aspect of the temporal bone was the blind spot of the exoscope during temporal bone resection (n = 2). These blind spots were visualized by the endoscope to facilitate accurate transection of the skull base. The advantages of the exoscope and endoscope include compact size, ergonomics, surgical field accessibility, and equal visual experience for neurosurgeons and head and neck surgeons, which enabled simultaneous transcranial and transfacial surgical procedures. All the surgeries were successful without any relevant complications. CEE is effective in transcranial skull base surgery, especially CFR involving simultaneous surgical procedures.

Olfactory Preservation in Craniofacial Resection of Tumor Invading Hemianterior Skull Base: Operative Video

In traditional craniofacial resection of tumors invading the anterior skull base, the bilateral olfactory apparatus is resected. Recently, transnasal endoscopy has been used for olfactory preservation in resections of unilateral low-grade malignancies. However, for tumors that invade the orbita or for high-grade malignancies, the transnasal endoscopic skull base surgery has been controversial. This video demonstrates the surgical techniques of olfactory preservation during craniofacial resection of a high-grade malignancy invading the hemianterior skull base and orbita.We present the case of a 32-year-old woman with osteosarcoma in the right ethmoid sinus. The tumor invaded the ipsilateral cribriform plate, dura menta, and orbital periosteum; however, the nasal septum and crista galli were intact (Fig. 1A, B). Because the tumor was a high-grade malignancy and the orbita had been invaded, we performed craniofacial resection instead of endoscopic resection (Fig. C2A). We drilled into the right side of the crista galli, midline of the cribriform plate, and perpendicular plate of the ethmoid bone via craniotomy. As a result, we accessed the nasal cavity directly (Fig. 2B). To preserve the nasal septum, we detached the remaining right septal mucosa through the transfacial approach (Fig. 2C). Because of the high risk of cerebrospinal fluid leakage as a result of previous irradiation, we performed vascularized free flap reconstruction of the skull base instead of pericranial flap.Postoperative computed tomography revealed no evidence of tumor (Fig. 1C, D). The patient's sense of smell returned after 1 postoperative day, and she was discharged on the postoperative day 14.The link to the video can be found at: https://youtu.be/XzPABYwzkjs.

Reconstructive Outcomes of Multilayered Closure of Large Skull Base Dural Defects Following Open Anterior Craniofacial Resection

Background Standardized reconstruction protocols for large open anterior skull base defects with dural resection are not well described. Here we report the outcomes and technique of a multilayered reconstructive algorithm utilizing local tissue, dural graft matrix, and microvascular free tissue transfer (MVFTT) for reconstruction of these deformities. Design This study is a retrospective review. Results Eleven patients (82% males) met inclusion criteria, with five (45%) having concurrent orbital exenteration and eight (73%) requiring maxillectomy. All patients required dural resection with or without intracranial tumor resection, with the average dural defect being 36.0 ± 25.9 cm2. Dural graft matrices and pericranial flaps were used for primary reconstruction of the dural defects, which were then reinforced with free fascia or muscle overlay by means of MVFTT. Eight (73%) patients underwent anterolateral thigh MVFTT, with the radial forearm, fibula, and vastus lateralis comprising the remainder. Average total surgical time of tumor resection and reconstruction was 14.9 ± 3.8 hours, with median length of hospitalization being 10 days (IQR: 9.5, 14). Continuous cerebrospinal fluid drainage through a lumber drain was utilized in 10 (91%) patients perioperatively, with an average length of indwelling drain of 5 days. Postoperative complications occurred in two (18%) patients who developed asymptomatic pneumocephalus that resolved with high-flow oxygen therapy. Conclusion A standardized multilayered closure technique of dural graft matrix, pericranial flap, and MVFTT overlay in the reconstruction of large open anterior craniofacial dural defects can assist the reconstructive team in approaching these complex deformities and may help prevent postoperative complications.

Vascularized Pericranial Flap for Endonasal Anterior Skull Base Reconstruction

Importance As the limits of advanced skull base malignancies that can be managed through an endoscopic endonasal approach continue to be expanded, the resultant anterior skull base defects are of increasing size and complexity. In the absence of nasoseptal or turbinate flaps, the vascularized pericranial flap has been employed at our institution with excellent results. Objective The study aimed to review the outcomes of patients who underwent endonasal anterior craniofacial resection with anterior skull base reconstruction using a vascularized pericranial flap. Design Retrospective chart review of patients treated by the University of California – San Francisco minimally invasive skull base service from the years 2011 to 2017. Average duration of follow-up was 16.4 months. Setting This study was conducted at Academic tertiary referral center. Participants A total of nine patients with advanced anterior cranial base malignancies were identified who were treated with a minimally invasive, endoscopic anterior craniofacial resection from the years 2011 to 2017. Due to the nature of the resection in these patients, nasoseptal flaps and inferior/middle turbinate flaps were unavailable or insufficient for anterior skull base defect repair. Each patient underwent reconstruction of the anterior cranial base defect using an anteriorly based pericranial flap harvested by bicoronal incision, and tunneled anteriorly to the nasal cavity through a frontoethmoidal incision.

Our experience with topical 5 fluoro uracil in sinonasal neoplasia

<p class="abstract">Sino-nasal neoplasms account for 3% of all head and neck cancers. Adenocarcinoma of the paranasal sinuses accounts for 9% of all sino-nasal malignancies and is the most common malignancy of the ethmoid sinus. Other neoplasms which involve the ethmoidal sinuses include inverted papilloma and squamous cell carcinoma. Traditionally, the treatment of choice for an adenocarcinoma of the ethmoidal sinuses involved craniofacial resection. This procedure is related with high rates of mortality and morbidity. Knegt et al reported greater success, both in terms of clinical outcome and survival data, with a less aggressive surgical approach coupled with repeated topical 5 fluorouracil (5FU) applications as a chemotherapeutic agent. Over the last 10 years we have treated selected cases of sinonasal neoplasia with a protocol similar to Knegt. Two (adenocarcinoma and inverted papilloma) of our four cases remain well and are on follow-up. The other two (undifferentiated carcinoma) were lost to follow-up. The rarity of sino-nasal neoplasms make them an unlikely subject for a randomised control trial. With that in mind surgical debridement and topical 5 fluorouracil seems to offer an acceptable treatment for adenocarcinoma of the sinonasal tract in properly selected cases. It may also have a role in Squamous cell carcinoma of ethmoidal or maxillary sinus and in preventing recurrence of inverted papilloma.</p>