Pediatric Extra-skeletal Sinonasal Ewing's Sarcoma: Report of Two Cases with Literature Review.

Ewing's sarcoma is a malignant small round cell tumor that belongs to the primitive class of neuroectodermal tumors. Ewing's sarcoma generally arises in the long bones of the extremities, and sinonasal localization of Ewing's sarcoma in pediatric population is an extremely rare event. The combination of histopathological examination and ancillary methods is extremely important to establish the diagnosis of Ewing's sarcoma. The treatment for Ewing's sarcoma includes a multidisciplinary approach with surgery, radiotherapy, and chemotherapy. Through this report, we try to communicate our experience and highlight the challenges encountered in diagnosing and approaching two extremely rare cases of pediatric sinonasal Ewing's sarcoma.

Oral and Sinonasal Tumors.

This article reviews the different types of equine non-neoplastic and neoplastic oral and sinonasal tumors and describes their known prevalence and general characteristics. The clinical and ancillary diagnostic findings (primarily radiography and endoscopy, and increasingly computed tomography) for each type of growth that can aid diagnosis are described. Most lesions require a histopathological confirmation of the diagnosed growth. The possible treatments and prognosis for these growths are briefly described.

Interpretability of operative and pathological reports for radiotherapy planning of sinonasal carcinomas: An ancillary study of the GORTEC 2016-02 SANTAL trial.

KEY POINTS: Interpretation of surgical mapping is essential for postoperative radiotherapy planning. Operative and pathological reports lack comprehensive information on margins quality and tissue block mapping. Standardizing reports is essential to reduce uncertainties, aiming for less morbid poRT.

Surgical therapy in advanced sinonasal carcinomas - retrospective study.

BACKGROUND AND AIM: Sinonasal tumors are a rare and heterogeneous group of malignant tumors with different histopathological characteristics and clinical presentation. These tumors are usually treated through surgery. The aim of this study is to present our results of surgical therapy in patients with an advanced sinonasal tumor. METHODS: This retrospective study included patients with an advanced sinonasal tumor who were surgically treated. The surgical technique combined both a frontal transbasal approach together with an endoscopic endonasal approach. The parameters used for evaluation were the histological type of tumor, the radicality of resection (complete vs. incomplete), the frequency of recurrence, the surgical and postoperative complications, the type of subsequent oncological therapy and the overall survival. RESULTS: The group consisted of ten patients seven were men and three were women. Complete resection (defined as R0) was achieved in 8 (80%) of the cases, subcomplete resection was achieved in 2 (20%) of the cases. The overall survival period was 28.7 months (95% confidence interval 15.9-41.6). CONCLUSION: The combination of the frontal transbasal approach with the endoscopic endonasal approach is a suitable surgical strategy that enables easier achievement of complete tumor resection, reconstruction of the anterior skull base and reduces the need for extensive surgical approaches.

Personalized Approach to Olfactory Neuroblastoma Care.

Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine malignancy arising from the olfactory neuroepithelium. ONB frequently presents with nonspecific sinonasal complaints, including nasal obstruction and epistaxis, and diagnosis can be obtained through a combination of physical examination, nasal endoscopy, and computed tomography and magnetic resonance imaging. Endoscopic resection with negative margins, with or without craniotomy, as necessary, is the standard of care for definitive treatment of ONB. Regional metastasis to the neck is often detected at presentation or may occur in a delayed fashion and should be addressed through elective neck dissection or radiation. Adjuvant radiotherapy should be considered, particularly in the case of high grade or tumor stage, as well as positive surgical margins. Systemic therapy is an area of active investigation in both the neoadjuvant and adjuvant setting, with many advocating in favor of induction chemotherapy for significant orbital or intracranial involvement prior to surgical resection. Various targeted immunotherapies are currently being studied for the treatment of recurrent or metastatic ONB. Prolonged locoregional and distant surveillance are indicated following definitive treatment, given the tendency for delayed recurrence and metastasis.

Long-term outcomes of modern multidisciplinary management of sinonasal cancers: The M. D. Anderson experience.

PURPOSE: To report long-term outcomes of modern radiotherapy for sinonasal cancers. METHODS AND MATERIALS: A retrospective analysis of patients with sinonasal tumors treated with intensity-modulated radiotherapy or proton therapy. Multivariate analysis was used to determine predictive variables of progression free survival (PFS) and overall survival (OS). RESULTS: Three hundred and eleven patients were included, with median follow-up of 75 months. The most common histologies were squamous cell (42%), adenoid cystic (15%), and sinonasal undifferentiated carcinoma (15%). Induction chemotherapy was administered to 47% of patients; 68% had adjuvant radiotherapy. Ten-year local control, regional control, distant metastasis free survival, PFS, and overall survival rates were 73%, 88%, 47%, 32%, and 51%, respectively. Age, non-nasal cavity tumor site, T3-4 stage, neck dissection, and radiation dose were predictive of PFS, while age, non-nasal cavity tumor site, T3-4 stage, positive margins, neck dissection, and use of neoadjuvant chemotherapy were predictive of OS. There was a 13% rate of late grade ≥3 toxicities. CONCLUSION: This cohort of patients with sinonasal cancer treated with modern radiotherapy demonstrates favorable disease control rate and acceptable toxicity profile.

Olfactory Neuroblastoma-A Challenging Fine Line between Metastasis and Hematology.

Developing in a limited space, rare tumors located at the nose and paranasal sinuses are sometimes difficult to diagnose due to their modest clinical presentation, which is uncorrelated with anatomopathological diversity. This limits the preoperative diagnosis without added immune histochemical study; for that reason, we present our experience with these tumors with the intention of raising awareness. The patient included in our study was investigated by our department through clinical and endoscopic examination, imaging investigations, and an anatomic-pathological study. The selected patient gave consent for participation and inclusion in this research study in compliance with the 1964 Declaration of Helsinki.

Top 10 Basaloid Neoplasms of the Sinonasal Tract.

BACKGROUND: Basaloid neoplasms of the sinonasal tract represent a significant group of tumors with histological overlap but often with different etiologies (i.e., viral, genetics), clinical management, and prognostic significance. METHODS: Review. RESULTS: "Basaloid" generally refers to cells with coarse chromatin in round nuclei and sparse cytoplasm, resembling cells of epithelial basal layers or imparting an "immature" appearance. Tumors with this characteristic in the sinonasal tract are represented by a spectrum of benign to high-grade malignant neoplasms, such as adenoid cystic carcinoma, NUT carcinoma, sinonasal undifferentiated carcinoma, SWI/SNF complex-deficient carcinomas, and adamantinoma-like Ewing sarcoma. CONCLUSION: In some instances, histology alone may be sufficient for diagnosis. However, limited biopsy material or fine-needle aspiration specimens may be particularly challenging. Therefore, often other diagnostic procedures, including a combination of histology, immunohistochemistry (IHC), DNA and RNA testing, and molecular genetics are necessary to establish an accurate diagnosis.

Preoperative Prediction of Sinonasal Inverted Papilloma-associated Squamous Cell Carcinoma (IP-SCC).

INTRODUCTION: Sinonasal inverted papillomas (IP) can undergo transformation into IP-squamous cell carcinomas (IP-SCC). More aggressive treatment plan should be established when IP-SCC is suspected. Nevertheless, inaccuracy of the preoperative punch biopsy results to detect IP-SCC from IP raises the need for an additional strategy. The present study aimed to investigate significant clinicoradiological remarks associated with IP-SCC than IP. MATERIAL AND METHODS: Postoperative surgical specimens obtained from patients with confirmed IP or IP-SCC at a single tertiary medical center from 1997 to 2018 were retrospectively evaluated. Patients' demographic and clinical characteristics, preoperative in-office punch biopsy results, and preoperative computed tomography (CT) or magnetic resonance images were reviewed. Univariate and multivariate analyses were performed to assess the odds ratio (OR) associated with IP-SCC. The area under the curve (AUC) in the receiver Operating Characteristic (ROC) curve was calculated in the prediction model to discriminate IP-SCC from IP. RESULTS: The study included 44 IP-SCC and 301 patients with IP. The diagnostic sensitivity of in-office punch biopsy to detect IP-SCC was 70.7%. Multivariate analysis showed that factors significantly associated with IP-SCC included tobacco smoking >10PY (adjusted-OR [aOR]: 4.1), epistaxis (aOR: 3.4), facial pain (aOR: 4.2), bony destruction (aOR: 37.6), bony remodeling (aOR: 36.3), and invasion of adjacent structures (aOR: 31.6) (all p < 0.05). Combining all significantly related clinicoradiological features, the ability to discriminate IP-SCC from IP reached an AUC of 0.974. CONCLUSION: IP patients with a history of tobacco smoking, facial pain, epistaxis, and bony destruction, remodeling, or invasion of an adjacent structure on preoperative images may be at higher risk for IP-SCC. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:2502-2510, 2023.

Intraosseous Cavernous Hemangioma of the Middle Turbinate: A Case Report.

BACKGROUND: We report a case of an intraosseous cavernous hemangioma originating from the middle turbinate that expanded into the anterior skull base, without traversing the cribriform plate. METHODS: The mass was found incidentally after a computed tomography head was ordered for unrelated reasons. On questioning, the patient denied any nasal symptoms. Magnetic resonance imaging showed an enhancing mass and the radiological imaging supported a broad differential. RESULTS: The lesion was removed by endoscopic image-guided surgery, and the pathology was that of a benign intraosseous cavernous hemangioma. There was no residual hemangioma on postoperative imaging and the nasal mucosa healed well. This is the first report of an intraosseous cavernous hemangioma of the middle turbinate showing superior expansion to the anterior skull base. CONCLUSION: This case demonstrates the extent to which cavernous hemangiomas may expand into surrounding tissues. While these lesions are uncommon, they can be considered as part of a broad differential diagnosis of sinonasal tumors.

Nasal Cavity Squamous Cell Carcinoma: Factors Associated With Treatment Outcomes and Potential Organ Preservation.

BACKGROUND: Squamous cell carcinoma of the nasal cavity (NCSCC) is a rare, challenging malignancy. Surgical resection of this tumor can cause significant facial deformity, and indications for adjuvant or organ preservation therapies are not well-described. OBJECTIVE: To examine the impact of treatment regimen on survival outcomes in NCSCC and to compare surgical to non-surgical based therapies. METHODS: The National Cancer Database was queried for NCSCC from 2004 to 2014. Patient demographics, tumor characteristics, and treatment regimen were compared for the entire cohort. Multivariable Cox proportional hazards regression was performed for statistical analysis of treatment regimen and surgical margins on overall survival (OS) for early and late-stage disease. RESULTS: A total of 1883 NCSCC patients were identified. The OS for the cohort was 83 months, and median age at diagnosis was 65 years. NCSCC patients who underwent surgery followed by adjuvant radiation therapy (RT) had a better OS compared to definitive RT (HR: 0.58, P < .001). In early stage NCSCC (T1/T2, N0), there was no significant difference in OS between patients treated with surgery only or surgery with adjuvant RT compared to definitive RT. In advanced stage NCSCC, surgery with adjuvant RT had a better OS compared to definitive chemoradiation. Having positive margins was shown to predict a worse OS when compared to negative margins in surgical patients despite adjuvant RT or chemoradiation. CONCLUSIONS: NCSCC appears to be best treated with surgery followed by adjuvant RT in advanced-stage disease whereas in early-stage disease, surgery does not improve OS compared to definitive RT.

A congenital extranasal glioma in a newborn.

Nasal gliomas are extremely rare in neonates with an incidence of 1 in 20,000 to 40,000. They often are asymptomatic but can present with respiratory distress depending on the size and location of the tumor. A newborn female was prenatally diagnosed with a left nasal mass. After her birth, she was transferred to a local children's hospital for subspecialty evaluation and for diagnostic imaging. The mass was resected at 1 year of age. Pathology confirmed a nasal glioma. Several weeks after surgery, a nasal prosthetic device was applied to correct the nasal deformity caused by the pressure effect of the tumor. At almost 1 year of age, there was no evidence of metastasis or recurrence of the nasal glioma. The prognosis and outcome tend to be favorable. The rare case of a neonate with a congenital nasal glioma is presented.

Interdisciplinary challenges and aims of flap or graft reconstruction surgery of sinonasal cancers: What radiologists and radiation oncologists need to know.

In sinonasal cancer surgery, a fundamental challenge is to understand the postoperative imaging changes after reconstruction. Misinterpretation of post-operative imaging may lead to a misdiagnosis of tumor recurrence. Because radiotherapy planning is based on imaging, there are many gaps in knowledge to be filled in the interpretation of postoperative imaging to properly define radiotherapy tumor volumes in the presence of flaps. On the other hand, radiotherapy may be responsible for tissue fibrosis or atrophy, the anatomy of the reconstructed region and the functional outcomes may change after radiotherapy compared to surgery alone. This narrative review illustrates the interdisciplinary aims and challenges of sinonasal reconstructive surgery using flaps or grafts. It is particularly relevant to radiologists and radiation oncologists, at a time when intensity modulated radiotherapy and proton therapy have the potential to further contribute to reduction of morbidity.

High-Grade Biphenotypic Sinonasal Sarcoma: A Case Report.

Introduction Biphenotypic sinonasal sarcoma (BSNS) is a recently found entity that first described by Lewis et al. It was then added to the 4th edition of the World Health Organization (WHO) of head and neck tumors in 2012. BSNS has been described as a rare low-grade sarcoma arising in the upper sinonasal tract. It is believed that in the past, BSNS was, likely, previously diagnosed as other low-grade or benign malignancies. Fibrosarcoma, leiomyosarcoma, and peripheral nerve sheath tumors, all fall within the differential diagnosis of BSNS. However, BSNS is unlike other mesenchymal sinonasal tumors, as it displays both neural and myogenic differentiation. BSNS has thus far been recognized in only a hand full of case reports, all of which have reported similar morphologic features of a low-grade soft tissue tumor with neural involvement arising from the nasal cavity or ethmoid air cells in middle aged individuals. In fact, being low-grade sarcoma became such a hallmark characteristic of this tumor that it even received the name low-grade sinonasal sarcoma with neural and myogenic features or LGSSNMF. Case Presentation We present, however, for the first time, a high-grade differentiation of BSNS in an otherwise healthy 72-year-old female. The patient was referred from an outside ENT (ear, nose, and throat) after pathology from a presumed polypectomy returned positive for a BSNS. Initial imaging revealed erosion through the bilateral lamina papyracea, anterior cranial fossa floor, and posterior table of the frontal sinus. She then underwent a combined endoscopic and bicoronal open approach for resection of the skull base lesion that was found to encompass the entirety of the sinonasal cavities bilaterally. Postoperatively, the patient underwent significant complications including infection of the pericranial flap, pneumocephalus, and eventually death. Discussion As BSNS is a fairly new entity, currently there has only been four case series conducted, each identifying features of a low-grade sarcoma with both myogenic and neural differentiation. Histologically, BSNS has monophasic spindle cells with uniform, elongated nuclei with scant cytoplasm between benign proliferations of surface-type respiratory epithelium, with a low mitotic rate. Our case, however, revealed pleomorphic hyperchromatic cells with high mitotic activity and necrosis with invasion of bone, staging it as high grade. Immunohistochemistry also differed from the previously reported standards. This case describes a new category for BSNS which may change the differential diagnosis, management, and surgical recommendations that are currently utilized for this skull base neoplasm.

Rare presentation of angiomatosis in the paranasal sinuses mimicking juvenile nasopharyngeal angiofibroma in a 16 year old male.

Rare presentation of pediatric angiomatosis of the paranasal sinus and skull base presenting mimicking juvenile nasopharyngeal angiofibroma (JNA). This is a 16-year-old male who presented to the emergency room with acutely worsening headaches, decreased visual acuity, subjective diplopia on lateral gaze, and a skull base mass centered in the sphenoid cavity. Endoscopic biopsy at an outside facility was aborted due to profuse bleeding. Upon transfer to a tertiary care center, contrast MR demonstrated a heterogeneously and avidly enhancing vascular mass centered around the sphenoid and skull base originating from the internal maxillary artery with significant bilateral extension into the adjacent paranasal sinuses, sella, and cavernous sinus. History of presentation and imaging was suggestive of JNA. Patient underwent preoperative embolization followed by endoscopic endonasal transphenoidal resection with a skull base trained otolaryngologist and neurosurgeon. Final pathology confirmed angiomatosis. This is only the second reported case of paranasal sinus angiomatosis in the literature. Angiomatosis has a high rate of recurrence and failure of timely diagnosis can lead to requirement of repeated surgical intervention. Re-operations are associated with increased costs, patient dissatisfaction, and poorer surgical/clinical outcomes. Because angiomatosis can mimic JNA, hemangiomas, or other vascular tumors, it is essential to maintain a broad differential diagnosis that includes angiomatosis when evaluating sinonasal tumors.

Reconstructive challenges of sinonasal tumors: A case report.

Sinonasal tumors are an uncommon pathological entity and applying the optimal treatment may represent a challenge, even for experienced physicians. A various number of techniques and materials may be used in the reconstruction of craniofacial defects following surgery for extensive sinonasal cancer. The aim of the present study was to present the case of a 33-year-old male patient diagnosed with a large sinonasal tumor and discuss the challenges faced while selecting the most suitable rehabilitation technique. In the present case, it was decided that the optimal solution was to use a craniofacial prosthesis in order to cover the entire defect, as well as a temporoparietal flap. In summary, reconstructive interventions must always be adapted to each individual patient and a multimodal approach may lead to a highly satisfactory outcome, for both the patient and the surgical team. All the reconstructive solutions available must always be kept in mind and adapted to the individual requirements of each case, taking into consideration both the extent of the tumor and the comorbidities of the patient, as there is no one solution that is considered as optimal for all patients.

Augmented Reality and Intraoperative Navigation in Sinonasal Malignancies: A Preclinical Study.

OBJECTIVE: To report the first use of a novel projected augmented reality (AR) system in open sinonasal tumor resections in preclinical models and to compare the AR approach with an advanced intraoperative navigation (IN) system. METHODS: Four tumor models were created. Five head and neck surgeons participated in the study performing virtual osteotomies. Unguided, AR, IN, and AR + IN simulations were performed. Statistical comparisons between approaches were obtained. Intratumoral cut rate was the main outcome. The groups were also compared in terms of percentage of intratumoral, close, adequate, and excessive distances from the tumor. Information on a wearable gaze tracker headset and NASA Task Load Index questionnaire results were analyzed as well. RESULTS: A total of 335 cuts were simulated. Intratumoral cuts were observed in 20.7%, 9.4%, 1.2,% and 0% of the unguided, AR, IN, and AR + IN simulations, respectively (p < 0.0001). The AR was superior than the unguided approach in univariate and multivariate models. The percentage of time looking at the screen during the procedures was 55.5% for the unguided approaches and 0%, 78.5%, and 61.8% in AR, IN, and AR + IN, respectively (p < 0.001). The combined approach significantly reduced the screen time compared with the IN procedure alone. CONCLUSION: We reported the use of a novel AR system for oncological resections in open sinonasal approaches, with improved margin delineation compared with unguided techniques. AR improved the gaze-toggling drawback of IN. Further refinements of the AR system are needed before translating our experience to clinical practice.

Long-term Outcomes from Proton Therapy for Sinonasal Cancers.

PURPOSE: To report long-term disease control, survival, and toxicity after proton therapy for sinonasal cancer. PATIENTS AND METHODS: We reviewed 143 cases of adults with nonmetastatic sinonasal cancers treated with primary (18%; n = 26) or adjuvant (82%; n = 117) proton therapy. The most common histologies were squamous cell carcinoma (29%; n = 42), olfactory neuroblastoma (23%; n = 33), and adenoid cystic carcinoma (16%; n = 23). Patients had predominantly advanced-stage disease (T3, 24%, n = 35; T4, 66%, n = 94) and high-grade histology (52%; n = 74). Surgery included endoscopic resection alone (50%) with craniotomy (10%) or open resection (40%), and 31% had gross disease present at radiotherapy. Most (91%) received high-dose (median, 73.6 Gy radiobiological equivalent [GyRBE]; 84% >70 GyRBE) passive-scatter proton therapy using accelerated hyperfractionation (1.2 GyRBE twice daily) and concurrent chemotherapy (70%). Univariate and multivariate models assessed prognostic factors. Grade 3+ toxicities were recorded per Common Terminology Criteria, version 4. Median follow-up was 3.4 years (range, 0.1-12.5 years) overall and 4.9 years (range, 0.9-12.5 years) for living patients. RESULTS: The 5-year outcomes were as follows: local control (LC), 80%; neck control, 96%; local-regional control, 78%; freedom from distant metastases, 71%; and disease-free survival, 62%; cause-specific survival, 64%; and overall survival, 59%. Surgery improved LC, but only with gross total resection (5-year LC 87% versus subtotal resection 62.9%, and biopsy alone 55% (P < 0.001). Gross residual disease was the only significant prognostic factor for local-regional control on multivariate analysis. High-grade, T4, and local recurrence were associated with decreased overall survival. Late (G3+) toxicity occurred in 22% (32 of 143), including central nervous system necrosis and vision loss in 6% (9 of 143) and 3.5% (5 of 143), respectively. CONCLUSION: Proton therapy after gross-total resection provides excellent long-term LC in patients with locally advanced, high-grade sinonasal cancer. Moreover, LC remains strongly associated with long-term survival. With gross disease, about 60% of patients had long-term LC with proton therapy and induction or concurrent chemotherapy.

Endoscopic versus open surgery in patients with malignant sinonasal tumors and brain invasion. A case series study. / Cirugía endoscópica vs. cirugía abierta en pacientes con neoplasias nasosinusales malignas con invasión cerebral. Estudio de serie de casos.

OBJECTIVES: To determine the safety, effectiveness and perioperative costs of endonasal endoscopic approach in brain invasive malignant sinonsal tumors patients. MATERIALS AND METHODS: This was a case series bidirectional study; that included 30 brain invasive malignant sinonsal tumors patients treated by endonasal endoscopic approach (2015-2017) and 53 by open surgery (2010-2015). Propensity score matching was used to compensate the prognostic factors; in a sample of 50 patients (25 per group). Primary response variables was local control and 3-years overall survival. Perioperative cost variables were analyzed. RESULTS: A number of 50 patients were included after matching (25 in each therapeutic group). The age average was 55 years and male proportion was 62%. Squamous cell carcinoma and grade II lesions were the most represented in the sample. Endonasal endoscopic approach reduced surgical time in 1 hour 20 minutes, transfusion needs in 5.5 fold and hospitalization in 19 days; in comparison with open technique. Oncologic control based on surgical free margins, local control, overall survival and progression free survival after three years was higher when the resection was performed endoscopically. Functional status was enhanced and complications diminished by using endoscopic approach. Saving was estimated in $7 355.18 per patient. CONCLUSIONS: Endonasal endoscopic approach represents a safe, effective and economic procedure in selected patients with malignant sinonasal tumors and brain invasion.

Radiooncological View on Therapy Outcome after Multidisciplinary Treatment of Sinonasal Tumors.

PURPOSE: We report the outcome of a mono-institutional retrospective study of sinonasal carcinoma with the primary focus on GTV (gross tumor volume) and the effect of radiotherapy. METHODS: 53 patients with sinonasal carcinoma and that of the nasal cavity, paranasal sinus or both except lymphoma were included. All patients were treated between 1999 and 2017. For tumor volume delineation, all pre-therapeutic images were fused to the planning CT (computed tomography). RESULTS: The median follow-up was 17 months [0.3-60], the median age 60 years, 35 males and 18 females were included. Squamous cell carcinoma (SCC) (60.4%) was the predominant histology, followed by adenocarcinoma (15.1%). The mean composite OS (overall survival) time was 33.3 ± 3.5 months. There was no significant difference in the 5 y composite OS between tumor localization or radiotherapy setting. The simultaneous integrated boost concept showed a trend towards improving five-year composite OS compared to the sequential boost concept. The only factor with a significant impact on the 5 y composite OS rate was the pre-therapeutic GTV (cutoff 75 cm3; p = 0.033). The GTV ≥ 100 cm3 has no effect on the 5 y composite OS rate for SCC. CONCLUSIONS: The pre-therapeutic GTV is a prognostic factor for five-year composite OS for the entire group of patients with sinonasal tumors, influencing the outcome after completion of all treatment strategies. The GTV seems to not influence five-year composite OS in SCC. For this rare tumor entity, an intensive, multidisciplinary discussion is essential to finding the best treatment option for the patient.