Tumor misdiagnosis resulting in inappropriate use of biologics.

KEY POINTS: Unilateral or destructive sinonasal disease should raise suspicion for tumor. Patients receiving biologic therapy for CRSwNP should be carefully selected. Tissue diagnosis should be considered prior to starting biologics for nasal polyposis.

Sinonasal seromucinous hamartoma: a single institution case series combined with a narrative review of the literature.

PURPOSE: This study aimed to investigate the clinical and histopathological characteristics of sinonasal seromucinous hamartomas (SHs). METHODS: Eight patients with sinonasal SH and treated at a tertiary hospital between November 2005 and September 2023 were included. Additionally, a systematic review of published articles was conducted, analyzing 48 cases of SH described in the literature. RESULTS: Among the eight patients treated at our institution, tumors originated from the posterior nasal cavity in four patients and middle turbinate and middle meatus were the primary origin in two patients each. Coexistence of inflammatory nasal polyps (NPs) was observed in four cases. Histopathologically, four patients exhibited focal respiratory epithelial adenomatoid hamartoma (REAH) features, and low-grade dysplasia was found in one patient. A combined analysis with previous literature revealed that 46.3% of all cases originated in the anterior nasal cavity. The proportions of cases accompanied by NPs and those with focal REAH features were 20.5% and 39.1%, respectively. Additionally, the frequencies of cases exhibiting dysplastic features (5.4%) and recurrence (2.1%) were low. Remarkably, tumors originating from the anterior region tended to have a higher frequency of dysplasia than those originating from the posterior region, although this difference was not statistically significant (p = 0.0996). CONCLUSION: Patients with sinonasal SH showed favorable treatment outcomes following surgical resection. Focal REAH features and accompanying NPs were frequently observed. A substantial proportion of cases originate in the anterior nasal cavity, and these tumors may exhibit a high tendency for dysplasia.

Molecularly defined sinonasal malignancies: an overview with focus on the current WHO classification and recently described provisional entities.

Classification of tumors of the head and neck has evolved in recent decades including a widespread application of molecular testing in tumors of the sinonasal tract, salivary glands, and soft tissues with a predilection for the head and neck. The availability of new molecular techniques has allowed for the definition of multiple novel tumor types unique to head and neck sites. Moreover, an expanding spectrum of immunohistochemical markers specific to genetic alterations facilitates rapid identification of diagnostic molecular abnormalities. As such, it is currently possible for head and neck pathologists to benefit from a molecularly defined tumor classification while making diagnoses that are still based largely on histopathology and immunohistochemistry. This review covers the principal molecular alterations in sinonasal malignancies, such as alterations in DEK, AFF2, NUTM1, IDH1-2, and SWI/SNF genes in particular, that are important from a practical standpoint for diagnosis, prognosis, and prediction of response to treatment.

Ectopic Olfactory Neuroblastoma: Systematic Review of a Rare Clinical Entity among Sinonasal Tumors.

Objectives Ectopic olfactory neuroblastoma is an uncommon manifestation of an already rare neoplasm. We aimed to systematically review the literature for cases of ectopic olfactory neuroblastoma to better characterize this rare disease entity and to present two new case reports. Methods A search of the PubMed and Embase databases was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to identify English-language articles reporting cases of ectopic olfactory neuroblastoma, published from 1955 through November 2021. Results Sixty-six cases of ectopic olfactory neuroblastoma were identified in 62 articles including the current review. Ectopic olfactory neuroblastoma arose in a wide age range (2-89 years) without significant sex predilection. It occurred most commonly in the ethmoid (25%), maxillary (25%), and sphenoid (16%) sinuses. Seventy-three percent of cases presented with low Hyams grade (I and II). The most common symptoms were nasal obstruction (32%) and epistaxis (32%). Paraneoplastic syndromes were observed in 27% of patients. The most common treatment was surgical resection followed by adjuvant radiotherapy. Overall, 76% of all patients were disease-free at the time of last follow-up. Locoregional recurrences and distant metastases were found in 19 and 5% of cases, respectively. Conclusion This systematic review describes previously reported cases of ectopic olfactory neuroblastoma, a disease entity with poorly understood characteristics. Physicians should consider olfactory neuroblastoma in the differential diagnosis for sinonasal masses, as their ectopic presentation may present considerable diagnostic and therapeutic difficulties. Patients with olfactory neuroblastoma may benefit from long-term follow-up and routine endoscopic examinations for surveillance of ectopic recurrences.

Diagnostic Challenges of Sinonasal Pleomorphic Adenoma.

Pleomorphic adenomas (PAs) are benign tumors of the salivary glands. Rarely, they arise in the sinonasal cavity, presenting as well-defined, homogeneous soft tissue masses, causing expansive bony changes. The significance of PAs is the possibility of giving rise to malignant carcinoma - "carcinoma ex-pleomorphic adenoma" (CXPA).Here, we present the case of a 64-year-old female complaining of progressive unilateral congestion and external nose deformation, mostly along the left contour of the radix, with epiphora of the ipsilateral eye. Eventually, a tumor began protruding from the left naris. The computed tomography excluded osteolysis, while the surgical procedure discovered the inferior turbinate as the origin of the tumor. In addition, the ipsilateral maxillary sinus was found to have developed secondary sinusitis. After complete surgical excision, the histological result was sinonasal melanoma, but following no progression of the disease, a second pathologist with additional immunohistochemical markers (HMB-45 (human melanoma black 45) negative, Melan-A (melanoma antigen recognized by T-cells 1) negative, S100 (protein soluble in 100% ammonium sulfate at neutral pH) positive, panCK AE1/AE3 (pan cytokeratin antibodies AE1 and AE3) negative, p63 (tumor protein 63) negative, Ki-67 (marker of proliferation Kiel 67) 10%, CD68 (cluster of differentiation 68) negative, CK7 (cytokeratin 7) negative, and CDX2 (caudal-type homeobox 2) negative) placed the definitive diagnosis of PA.PA of the inferior turbinate is an extremely rare finding, with the clinical symptoms being unspecific. Sometimes, SOX-10 (SRY-box transcription factor 10) positivity can mislead to malignant melanoma, as in our case, which is why a broad panel of immunohistochemical markers is critical for the definitive diagnosis.

Molecular pathology in diagnosis and prognostication of head and neck tumors.

Classification of head and neck tumors has evolved in recent decades including a widespread application of molecular testing in tumors of the salivary glands, sinonasal tract, oropharynx, nasopharynx, and soft tissue. Availability of new molecular techniques allowed for the definition of multiple novel tumor types unique to head and neck sites. Moreover, the expanding spectrum of immunohistochemical markers facilitates a rapid identification of diagnostic molecular abnormalities. As such, it is currently possible for head and neck pathologists to benefit from a molecularly defined classifications, while making diagnoses that are still based largely on histopathology and immunohistochemistry. This review highlights some principal molecular alterations in head and neck neoplasms presently available to assist pathologists in the practice of diagnosis, prognostication and prediction of response to treatment.

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors.

BACKGROUND: Sinonasal neoplasms, whether benign and malignant, pose a significant challenge to clinicians and represent a model area for multidisciplinary collaboration in order to optimize patient care. The International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors (ICSNT) aims to summarize the best available evidence and presents 48 thematic and histopathology-based topics spanning the field. METHODS: In accordance with prior International Consensus Statement on Allergy and Rhinology documents, ICSNT assigned each topic as an Evidence-Based Review with Recommendations, Evidence-Based Review, and Literature Review based on the level of evidence. An international group of multidisciplinary author teams were assembled for the topic reviews using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses format, and completed sections underwent a thorough and iterative consensus-building process. The final document underwent rigorous synthesis and review prior to publication. RESULTS: The ICSNT document consists of four major sections: general principles, benign neoplasms and lesions, malignant neoplasms, and quality of life and surveillance. It covers 48 conceptual and/or histopathology-based topics relevant to sinonasal neoplasms and masses. Topics with a high level of evidence provided specific recommendations, while other areas summarized the current state of evidence. A final section highlights research opportunities and future directions, contributing to advancing knowledge and community intervention. CONCLUSION: As an embodiment of the multidisciplinary and collaborative model of care in sinonasal neoplasms and masses, ICSNT was designed as a comprehensive, international, and multidisciplinary collaborative endeavor. Its primary objective is to summarize the existing evidence in the field of sinonasal neoplasms and masses.

Sinonasal Organizing Hematoma: Demographics, Diagnosis, and Treatment Outcomes of 112 patients.

INTRODUCTION: Sinonasal organizing hematoma (OH) is a rare, nonneoplastic lesion that often presents with epistaxis, a reddish mass, and destruction of the involved sinonasal structures. Due to its rarity, the demographics, diagnostic modalities, treatment strategies, and outcomes have not yet been studied in a large, long-term study. MATERIALS AND METHODS: Retrospect cohort of 112 sinonasal OH patients treated between 1997 and 2020 in a tertiary, university hospital were evaluated. Demographics, systemic comorbidities, sinonasal surgery history, serum laboratory tests, radiological findings, and treatment results were collected. The present study aimed to assess the accuracy of preoperative computed tomography (CT), Gadolinum-enhanced magnetic resonance (MR), and punch biopsies in detecting sinonasal OH as the most likely diagnosis. In addition, incidental differences by age and year of diagnosis were calculated using the Poisson log-linear regression model. RESULTS: The median age was 44, and 58% were male. Fewer than 20% of these cases had a chronic systemic comorbidity, bleeding tendency, or sinonasal surgery history. MR had the highest accuracy of (87%) to detect sinonasal OH as the most likely diagnosis, compared with contrast-enhanced-CT (53%), punch biopsy (49%), and non-enhanced-CT (16%) (all <0.05). Sinonasal OH incidence did not vary by age, but the yearly rate significantly increased by 1.05 times over 23 years (p < 0.05). Notably, 84% of 112 patients received surgical removal through the assistance of an endoscope, and none had substantial bleeding without preoperative embolization. CONCLUSION: Sinonasal OH was observed regardless of age, sex, systemic comorbidities, bleeding tendency, prior sinonasal surgery, or trauma. Preoperative MR gives the highest accuracy for detecting this disease. Sinonasal OH may be safely managed with endoscopic-assisted surgery removal without embolization. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1581-1590, 2024.

A Rare Case of Sinonasal Seromucinous Hamartoma Developing from the Nasal Septum.

A 45-year-old man presented with a history of chronic left nasal congestion. Nasal endoscopy revealed a pedunculated polypoid mass with glandular epithelium surface on the posterior nasal septum. Computed tomography revealed a 25-mm mass-like growth in the left posterior nasal cavity attached to the nasal septum with a stalk. The patient underwent transnasal endoscopic surgery, and the tumor was removed under a block with safety margin. The final pathological diagnosis was sinonasal seromucinous hamartoma (SH). Sinonasal SH is a rare tumor with only 31 reported cases. Transnasal endoscopic surgery is currently the first-line treatment for sinonasal SH. Differential diagnoses of this lesion include inflammatory polyps, respiratory epithelial adenomatoid hamartoma, and adenocarcinoma. Although SH is a benign tumor, its progression to adenocarcinoma has been reported. Therefore, unilateral posterior nasal tumors must be diagnosed precisely.

A malignant peripheral nerve-sheath tumor: A CARE case report.

INTRODUCTION: Malignant peripheral nerve-sheath tumor (MPNST) is an extremely rare sarcoma of the nasal cavity and paranasal sinuses. Non-specific clinical and radiological presentation and challenging histological diagnosis make it little known by physicians. We describe a case of maxillary sinus MPNST, following CARE guidelines. CASE REPORT: A 62-year-old woman consulted for swelling of the right cheek and hard palate with several months' progression. CT and MRI revealed a tissue mass in the right maxillary sinus with osteolysis of the orbital and maxillary floors, hard palate and lateral and medial walls of the maxillary sinus. Biopsy confirmed diagnosis of low-grade MPNST. After total resection and 60Gy adjuvant radiotherapy, 2-year follow-up showed no signs of recurrence. CONCLUSION: MPNST in the nasal cavity and paranasal sinuses is very rare. Because of a high risk of recurrence, wide resection should be implemented, possibly completed by radiotherapy if resection cannot be complete because of proximity to at-risk structures.

A Rare Case of Sinonasal Ewing Sarcoma With Radiologic-Pathologic Correlation.

An 89-year-old male presented with syncope and worsening difficulty in breathing through the left nostril. Computed tomography demonstrated a tumor in the anterior ethmoid air cells and maxillary sinus, which extended into the frontal lobe. Magnetic resonance imaging similarly demonstrated an aggressive lesion. This mass was difficult to differentiate from more commonly seen lesions at this location such as an esthesioneuroblastoma or nasopharyngeal carcinoma. Direct visualization, biopsy, and subsequent pathologic analysis eventually confirmed the diagnosis of malignant Ewing sarcoma (EWS). Our case explores the radiological findings of EWS originating from the ethmoid sinus, compares EWS with other common carcinomas in the same location, confirms the diagnosis through pathological correlation, and investigates the prognosis and treatment of these lesions. This case highlights the importance of a multidisciplinary approach to diagnose EWS when it occurs in an atypical location. The clinical team relied on input from the radiology, surgery, ENT, neurology, and pathology departments to make an accurate diagnosis and plan treatment for this aggressive tumor.

Anterior Skull Base Osteoradionecrosis in the Age of Intensity-Modulated Radiation Therapy: A Case Series.

Background Despites advances in radiation technology, skull base osteoradionecrosis (ORN) continues to be a rare, devastating, and hard to treat complication of radiotherapy. We present three cases of anterior skull base ORN in a cohort treated with intensity-modulated radiation therapy (IMRT). Case Series Three patients developed anterior skull base ORN after receiving at least one round of IMRT. ORN was diagnosed through either nasal endoscopy or imaging findings. The first was a 59-year-old woman with a sinonasal squamous cell carcinoma. Her chemoradiation history was notable for reirradiation and a high dose of radiation (143.3 Gy). The second was a 55-year-old man with recurrent nasopharyngeal carcinoma, whose history was notable for a high dose of radiation (∼140 Gy) and for being reirradiated. The final patient was a 37-year-old woman with an unremarkable history who received radiotherapy (65.0 Gy) for an esthesioneuroblastoma. One patient was asymptomatic and did not receive ORN-specific therapy. The other two were treated with a combination of medical and surgical intervention with successful short-term outcomes (no evidence of infection). Conclusion Anterior skull base ORN can be treated through conservative and surgical means to achieve successful short-term outcomes. Further investigation of long-term outcomes is warranted.

Sinonasal hemangiopericytoma: A rare sinonasal tumor.

Hemangiopericytoma is a rare vascular tumor arising from pericytes and occurs very rarely in sinonasal region. A 48-year-old man with sinonasal mass presented with nasal obstruction and occasional epistaxis. Nasal endoscopy showed a readily bleeding mass in the left nasal cavity. Endoscopic removal of the mass was done. The histopathology came out to be hemangiopericytoma. The patient was kept on follow-up, and no metastasis or recurrence was seen in last 1 year. Hemangiopericytoma is a very rare vascular tumor. Surgery is the mainstay treatment of choice. A long-term follow-up is needed after surgery to rule out recurrence and metastasis.

The Latest Craniofacial Reconstructive Techniques Using Anchored Implants after Surgical Treatment of Nasal and Paranasal Sinuses Tumors.

Reconstructive surgery after surgical treatment of neoplasms in the head and neck region is always a challenge. Many factors are responsible for the success of reconstruction. The anatomy of the facial region is complex, which significantly influences the aesthetic effect of the reconstruction. Moreover, many patients undergo postoperative radiotherapy after surgical treatment, which affects the range of reconstructive techniques. The aim of this study is to review current reconstructive methods in the craniofacial region, using bone-anchored implants to attach nasal prostheses. The article also comprises the authors' own experience with successful single-stage, Vistafix 3 osseointegrated implants for the attachment of an external nasal prosthesis in a 51-year-old man after surgical removal of squamous cell carcinoma of the nose and paranasal sinuses. The literature search for articles regarding implants in craniofacial reconstructions was performed using the three following databases: Scopus, Web of Science and MEDLINE (through PubMed), and follows the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Statement (PRISMA). A systematic literature search was set for 2018-2023 and retrieved 92 studies. From them, 18 articles were included in the review.

Nasopharyngeal Respiratory Epithelial Adenomatoid Hamartoma-Master of Masquerade.

OBJECTIVES: To highlight a case of nasopharyngeal Respiratory Epithelial Adenomatoid Hamartoma (REAH) in a female patient with chronic nasal block. METHODS: A single case report of a female patient with the diagnosis of REAH, detailing her presenting symptoms, clinical findings, management and follow up. RESULTS: Histopathological assessment of the excised nasopharyngeal polyp was consistent with a diagnosis of REAH with a discussion on the disease and its current literature reviews. CONCLUSION: The incidence of REAH within the nasopharynx remain rare with only few cases described in literature, especially in females.

Adenofibromatous Solitary Fibrous Tumor: An Unusual Morphologic Variant Occurring in the Sinonasal Tract.

BACKGROUND: Solitary fibrous tumor can exhibit a broad morphologic spectrum, such as presence of epithelioid tumor cells, adipose cells and multinucleated giant cells. This report describes an unusual morphologic variant characterized by adenofibromatous features, all occurring in the sinonasal region. METHODS: Four cases of the adenofibromatous variant of solitary fibrous tumor were retrieved from the surgical pathology and consultation files in Queen Elizabeth Hospital, Hong Kong. Histologic examination, immunohistochemical study and reverse-transcription polymerase chain reaction (RT-PCR) were performed. RESULTS: The patients were adults who presented with an obstructive mass of the nasal septum, nasal cavity or nasolacrimal sac. Histologic examination showed a circumscribed biphasic tumor with intermingling of glandular structures and spindle cells, reminiscent of mammary fibroadenoma. Bland-looking spindle cells formed short, irregularly oriented fascicles, admixed with variable amount of collagen fibers. The glandular component comprised ducts and seromucinous acini with a lobular architecture, indicating that it represented exuberant hyperplasia of indigenous glands rather than part of the neoplastic process. Demonstration of CD34 and STAT6 immunoreactivity in the spindle cells and NAB2::STAT6 gene fusion by polymerase chain reaction supports the diagnosis of solitary fibrous tumor. CONCLUSION: This study reports four cases of sinonasal solitary fibrous tumor with adenofibromatous features, furthermore expanding the morphologic spectrum of this tumor.

Navigating Fibrous Dysplasia of the Middle Turbinate: A Decade-Long Journey and Comprehensive Management Strategies.

The authors report an unusual case of monostotic fibrous dysplasia involving the middle turbinate. Only four cases of fibrous dysplasia (FD) involving the middle turbinate had previously been documented globally, all of which entailed severe presentation and were treated surgically. We report the first case of asymptomatic monostotic FD of the middle turbinate in the Middle East, followed over a span of 10 years.  Fibro-osseous lesions are rare, benign tumors of the sinonasal and orbital regions. Craniofacial FD most commonly affects the maxilla and mandible, followed by the frontal, parietal, and occipital bones. The prevalence of most patients presenting with monostotic craniofacial FD is between 10% and 29%. It is typically found incidentally, and is most likely to be asymptomatic. The risk of skull base damage and cerebrospinal fluid leaks is increased in this case due to the involvement of the middle turbinate and the expansion of FD into the lateral lamella and the cribriform plate.  Our patient was managed conservatively with regular follow-up due to the minor clinical symptoms despite the extent of the disease. We aim to elucidate the specific challenges associated with middle turbinate involvement and contribute to the growing body of knowledge in this field, ultimately improving the care and quality of life for individuals affected by this condition.

Orbital Apex Syndrome Secondary to SMARCB1-Deficient Invasive Sinonasal Carcinoma.

Orbital apex syndrome (OAS) is a clinical entity defined by ophthalmoplegia and optic nerve dysfunction due to local disruption of the orbital apex. The causes of OAS are extensive and include infectious, inflammatory, traumatic, iatrogenic, and neoplastic conditions. Thus, appropriate management is dependent on an accurate and timely diagnosis of the underlying etiology. We present a case of a 58-year-old female who presented to the emergency department with ophthalmoplegia of subacute onset and diminished visual acuity in the setting of two weeks of headache, ocular pain, and facial swelling. She was ultimately diagnosed with OAS and admitted to the hospital for five days for further evaluation. She was found to have an incurable primary SMARCB1-deficient sinonasal carcinoma with an invasion of her orbital apex. A multidisciplinary management approach involving chemotherapy, radiation, and surgical intervention was performed, and the patient responded well. Nearly two years after her diagnosis, she continues to have stable residual carcinoma without evidence of recurrence or metastatic disease. Her visual acuity has returned to normal limits, and her oculomotor function has returned to near-normal levels.