Sinonasal Lesions Research Articles

Desmoid‐type fibromatosis of the head and neck region in the paediatric population: a clinicopathological and genetic study of seven cases

Desmoid-type fibromatosis (desmoid) is a locally aggressive (myo)fibroblastic lesion. It represents one of the more common fibrous tumours in children and adolescents. The head and neck region is more often involved than in adults. We investigated the clinicopathological and genetic characteristics of seven paediatric desmoids at this anatomical site, including two cases of desmoplastic fibroma located in the mandible. There were two females and five males with an age range of 1.5-8years. The sites of the soft tissue lesions were sinonasal (n=4) and paramandibular (n=1). All cases showed typical morphology and nuclear β-catenin expression. CTNNB1 gene sequencing, performed successfully in five cases, revealed mutations in three cases with one p.T41A (bone lesion), one p.S37A and one novel mutation, p.D32V (sinonasal soft tissue lesions). Six patients were treated by excision with positive margins in five cases. Follow-up, available for six patients (median 4years), showed no evidence of disease in four cases, slow progression in one case, and recurrence with stable disease in the last case. Our study provides evidence of genetic similarities in desmoid and desmoplastic fibroma. Additionally, we expanded the spectrum of mutations in CTNNB1 with one novel desmoid mutation.

English

INTRODUCTION: Nasal cavity and paranasal sinuses present a range of non-neoplastic and neoplastic lesions by virtue of their anatomic and histologic diversity. Although a presumptive diagnosis is arrived at based on presenting symptoms and imaging techniques, histopathology is the mainstay for definitive diagnosis and management of sinonasal lesions. AIMS AND OBJECTIVES: To study the incidence, mode of presentation and histologic types of sinonasal lesions in surgical pathology material. SETTING AND DESIGN: Retrospective retrieval of all sinonasal lesions and histopathologic analysis. MATERIALS AND METHODS: All sinonasal lesions, biopsied or excised over a period of three years between May 2010 and May 2013, were studied. The lesions were classified as non-neoplastic or neoplastic. Neoplasms were further categorised as benign and malignant. The histology was correlated with clinical features. Special stains and immunohistochemistry were done wherever indicated. RESULTS: In three years, sixty- one sinonasal lesions were reported, representing 0.47% of all surgical pathology specimens. Out of sixty- one cases, forty- one (67.21%) were non-neoplastic and twenty (32.79%) were neoplastic. Sinonasal lesions were common in the second and third decades of life, with male predominance. Among the non-neoplastic lesions, inflammatory polyps were the most common (80.49%). Among neoplasms, benign tumors (60%) were more frequent than malignant tumors (40%). Angiofibroma (6 cases-50%) was the commonest benign neoplasm and squamous cell carcinoma (4 cases- 50%) was the commonest malignant neoplasm. CONCLUSION: Non-neoplastic and neoplastic lesions in the sinonasal region have similar clinical manifestations. Categorization based on histopathology is necessary for differential diagnosis, management and prognosis of these uncommon lesions.

18‐FDG‐PET in the initial staging of sinonasal malignancy

The utility of fluorine-18 fluorodeoxyglucose-positron emission tomography (PET) has been gradually defined for most head and neck cancers, However, its utility in the initial diagnosis of sinonasal malignancy has not been extensively studied. The aim of this study was to determine if PET scanning accurately diagnoses and stages malignant sinonasal lesions and if maximum standard uptake value (SUV max) correlates with clinically advanced disease. Retrospective chart review. There were 51 patients with sinonasal malignancy who underwent diagnostic whole body PET or PET-computed tomography scans that were analyzed for patient and disease characteristics, SUV max, and staging. Of the 51 patients, 48 scans were positive at the primary site, with a sensitivity of 94%. Four patients were found to have intensely avid uptake, in which the numerical SUV max was not documented, and three patients did not have any uptake in the region of their tumor. Mean SUV max at the primary site was 16.1 (range, 3.1-59). Metastasis was detected in 31% (16/51) of the patients. There was a potential positive correlation between SUV max at the primary site and detection of metastasis on univariate analysis (r = 0.19, P = .09), but on multivariate analysis, SUV max was not found to correlate with T staging or metastasis. For diagnosis of sinonasal malignancy, PET scans have a high sensitivity, although false negatives occurred in 6% of cases. PET scanning detected metastasis in 31% of patients, but SUV max did not function as a marker for clinically advanced disease. The role of diagnostic PET for sinonasal malignancy is currently limited to cases with a high suspicion of metastatic disease.

Sinonasal Respiratory Epithelial Adenomatoid Hamartomas: Series of 51 Cases and Literature Review

Respiratory epithelial adenomatoid hamartomas (REAHs) are rare, benign glandular proliferations of the nasal cavity, paranasal sinuses, and nasopharynx. This study aimed to expand our understanding of this entity by presenting a series of REAHs combined with a review of the pertinent literature. A retrospective review was performed on all patients with a diagnosis of REAH from 2002 to 2011. Data were collected with respect to age, gender, clinical presentation, imaging, histopathology, treatment, and outcome. Because olfactory cleft expansion by imaging evaluation has been reported to suggest REAH, maximum olfactory cleft (MOCs) widths were also measured. Fifty-one cases of REAH included 37 male (72.5%) and 14 female subjects (27.5%) with a mean age of 58.4 years. Headache, nasal obstruction, rhinorrhea, and hyposmia were the most common presenting symptoms. Although 35(68.6%) were associated with concurrent inflammatory pathology, 16 (31.4%) presented as isolated lesions of the nasal cavity. Enlargement of MOCs was evident on computed tomography, with mean MOCs of 8.64 and 9.4 mm, in the coronal/axial planes, respectively. There were no statistically significant differences between MOCs of isolated (7.96 mm) versus MOCs of associated (9.63 mm) lesions (p = 0.25). Forty-nine were treated with endoscopic resection without evidence of recurrence after a mean follow-up of 27.2 months. REAHs are rare sinonasal lesions that may appear as localized, isolated masses or more diffuse when in conjunction with other inflammatory processes. Irrespective of clinical presentation, endoscopic removal appears to be curative. Differentiation from more aggressive lesions is paramount to avoid unnecessarily radical surgery for an otherwise benign process.

Ruptured Internal Carotid Artery Aneurysm Presenting as a Sinonasal Organized Hematoma: Conventional Approach May Endanger Life

Sinonasal organized hematoma (SOH) is an uncommon benign entity with locally aggressive behavior. The conventional approach is complete resection. This approach, however, can cause massive hemorrhage and mortality in our patient due to a ruptured internal carotid artery (ICA) aneurysm hidden inside the SOH. To the best of our knowledge, this is the first case of a huge SOH with skull base invasion caused by a hidden ICA aneurysm, which usually would not be large enough to result in a sinonasal mass lesion. This case suggests that the possibility of an aneurysm should always be considered if a SOH is suspected, especially when it is huge and close to the ICA. Surgical resection may not be the first therapeutic choice unless the differential cause of a hidden aneurysm can be excluded.

Characteristics, management of the neck, and oncological outcomes of malignant minor salivary gland tumours in the oral and sinonasal regions

The aim of this study was to evaluate the characteristics of malignant tumours of the minor salivary glands in the oral and sinonasal regions, to make sure that the neck was managed correctly, and to identify oncological outcomes, retrospectively, at a single hospital. A total of 60 patients were reviewed. Forty-nine patients had intraoral lesions and 11 had sinonasal lesions. Of the 60 patients, 28 had stage I to II malignant tumours, and 32 had stage III to IV tumours. Treatment was almost exclusively surgical. One of the 16 patients whose nodes were clinically clear had an elective neck dissection. Adjuvant radiotherapy was given if indicated. The mean follow-up period was 52 months (range 13–190).Sinonasal lesions were all advanced T-stage at diagnosis, had more invaded resection margins, and a higher local recurrence rate than intraoral lesions. There was no regional recurrence in those patients whose nodes were clinically invaded and who had therapeutic neck dissection, or in the patient whose nodes were clinically clear and who had an elective neck dissection. Occult metastases developed exclusively in adenoid cystic carcinomas (ACC), the rate of which was 4/16. Regional recurrence developed in 4 patients who had never had elective treatment to the neck 2 of whom mixed pattern ACC and 2 who had low grade mucoepidermoid carcinomas (MEC). The overall survival was 90% at 2 years, 77% at 5 years, and 74% at 10 years.Sinonasal minor salivary gland tumours require careful follow-up because resection margins are more likely to be invaded by tumour, and they have a higher local recurrence rate than intraoral lesions. Elective neck dissection is needed, particularly for MEC and also to prevent regional recurrence in ACC.

Pathology Quiz Case 2

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Endonasal treatment of acute invasive fungal rhinosinusitis in immunocompromised pediatric hematology–oncology patients

ObjectivesAcute invasive fungal rhinosinusitis (AIFR) is an aggressive fungal infection in immunocompromised patients with high mortality rates. The aim of this study is to present our experiences on endonasal treatment in immunocompromised pediatric hematology–oncology patients with AIFR. MethodsThirteen pediatric hematology–oncology patients treated for AIFR between March 2006 and December 2011 were analyzed retrospectively. We reviewed the following data for all patients: age, gender, predisposing disease, initial symptoms, pathological diagnosis, microbiological results, laboratory findings, surgical procedure, number of operations and treatment outcomes. ResultsNine of 13 patients with lesions confined to sinonasal cavity were operated with endoscopic approach. Open surgery was performed in four patients, three of them had palatal and buccal lesions and one had facial skin involvement. Endoscopic approach was also used for sinonasal lesions of these four patients. A total of 7 patients died: 4 patients with progression of the underlying disease, 2 patients with sepsis and 1 patient due to renal failure. Survival rate in surgically treated patients was found 46% (6/13 patients). ConclusionsEndonasal endoscopic approach is both feasible and efficient technique, also enables excellent local control with less morbidity compared to open surgery. This approach is suitable for patients who are diagnosed in the early stages of AIFR and also presents a less traumatic option for patients with poor health status. Open surgical procedure should be preferred in patients with disease extending out of the sinonasal cavity.

Analysis of KIT expression and mutations in sinonasal, genital, and acrolentiginous melanomas.

8563 Background: Melanoma is subdivided into molecular defined groups. One major subtype is characterized by a mutation in the BRAF gene. BRAF is less commonly mutated in acrolentiginous (ALM) and mucosal (MM) melanomas, instead these subtypes predominantly show aberrations in the KIT/CD117 gene. KIT aberrations predict the outcome of targeted therapies in ALM and MM patients, however the particular role of KIT expression in these melanoma subtypes remains controversial. To explore the relationship between KIT expression, mutation, and tumor stages, we investigated immunohistochemical KIT expression and mutation status in primary ALM/MM lesions and their metastases. We also compared the frequency of KIT mutations of MM in different anatomic locations. Methods: KIT immunoreactivity and mutation status was assessed in 41 ALM and 25 MM patients, using polyclonal rabbit anti-human KIT/CD117 antibody, and PCR was performed for KIT gene exons 9,11,13,17 and 18. Of these, 19 ALM and 15 MM patients had matched primary and metastatic lesions. Phosphorylated extracellular regulated kinase (P-ERK) was investigated in a subset of 8 ALM and 8 MM matched primary/metastatic pairs by immunohistochemistry. Results: Heterogeneous KIT immunoreactivity was observed in both primary and metastatic lesions. Mutations were present in four of 41 ALM (10%) and five of 25 MM (20%) patients. Only vulvar mucosal samples carried KIT mutations in contrast to sinonasal lesions (p= 0.0109). In KIT-mutated tumors, the mutations were present in KIT expressing as well as KIT negative cells, as shown by Laser Capture Microdissection (LCM). P-ERK expression was preferentially found in metastases. Conclusions: KIT expression is heterogeneous and shows no relationship with disease progression and mutation status, therefore KIT immunoreactivity is not a valuable marker for treatment decisions. In mucosal melanoma patients, KIT mutation frequency is significantly associated with anatomic location. Vulvar melanoma patients are prone to carry activating KIT mutations and thus may be more likely to benefit from KIT-targeted therapies than other subtypes. Therefore, especially vulvar melanoma patients should be screened for activating KIT mutations.

Graduated Endoscopic Multiangle Approach for Access to the Infratemporal Fossa

The infratemporal fossa (ITF) has historically been one of the most difficult regions of the skull base to access surgically. Available open approaches are complex, are associated with high morbidity, and do not always afford optimal visualization. Endoscopic access to the ITF improves visualization for management of many sinonasal and lateral skull base lesions involving this region. The purpose of this study is to evaluate a graduated multiangle approach for endoscopic access to this area using a cadaveric model. Cadaveric study at an academic medical center. Endoscopic dissection was performed on a total of 10 sides of 5 fresh cadaveric heads. Four different approaches to the ITF were studied: ipsilateral endonasal, endoscopically assisted Caldwell-Luc, contralateral endonasal via septotomy, and endoscopically assisted Gillies transtemporal. High-quality endoscopic pictures and high-definition videos of each technique were obtained in order to document the differences in access achieved with each approach. The combination of the 4 different endoscopic techniques allowed complete access to all areas of the ITF. The endoscopically assisted Caldwell-Luc improved anteroposterior access, the contralateral septotomy approach resulted in excellent far lateral access, and the endoscopically assisted Gillies approach allowed posterosuperior visualization and instrumentation. Endoscopic access to the ITF can be accomplished by each of the 4 methods described. A multiangle, graduated approach can provide surgeons the ability to customize surgical access depending on the location of a specific lesion within the ITF.

Sinonasal, genital and acrolentiginous melanomas show distinct characteristics of KIT expression and mutations

KIT aberrations predict the outcome of targeted therapies in acrolentiginous (ALM) and mucosal (MM) melanoma patients.KIT immunoreactivity and mutation status was assessed in 41 ALM and 25 MM patients. Of these, 19 ALM and 15 MM patients had matched primary and metastatic lesions. P-ERK was investigated in a subset of 9 ALM and 7 MM matched primary/metastatic pairs by immunohistochemistry.Heterogeneous KIT immunoreactivity was observed in both primary and metastatic lesions. Mutations were present in four of 41 ALM (10%) and five of 25 MM (20%) patients. Only vulvar mucosal samples carried KIT mutations in contrast to sinonasal lesions (p=0.0109). In KIT-mutated tumours, the mutations were present in KIT expressing as well as KIT negative cells, as shown by Laser Capture Microdissection (LCM). P-ERK expression was preferentially found in metastases.KIT mutations predict treatment outcome with KIT inhibitors. Therefore, especially vulvar melanoma patients should be screened for activating KIT mutations.

Sinonasal Tract Mucoepidermoid Carcinoma: A Clinicopathologic and Immunophenotypic Study of 19 Cases Combined with a Comprehensive Review of the Literature

Primary sinonasal tract mucoepidermoid carcinomas (MEC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. The design of this study is retrospective. Nineteen cases of MEC included 10 females and 9 males, aged 15-75 years (mean, 52.7 years); males, on average were younger by a decade than females (47.2 vs. 57.7 years). Patients presented most frequently with a mass, obstructive symptoms, pain, and/or epistaxis present for a mean of 12.6 months. The majority of tumors involved the nasal cavity alone (n=10), maxillary sinus alone (n=6), or a combination of the nasal cavity and paranasal sinuses (n=3) with a mean size of 2.4 cm. Most patients presented at a low clinical stage (n=15, Stage I & II), with only 4 patients presenting with Stage III disease. Histologically, the tumors were often invasive (bone or perineural invasion), with invasion into minor mucoserous glands. Surface involvement was common. The neoplastic cells were composed of a combination of squamoid cells, intermediate cells, and mucocytes. Cystic spaces were occasionally large, but the majority were focal to small. Pleomorphism was generally low grade. Necrosis (n=5) and atypical mitotic figures (n=6) were seen infrequently. Over half of the tumors were classified as low grade (n=11), with intermediate (n=4) and high grade (n=4) comprising the remainder. Mucicarmine was positive in all cases tested. Immunohistochemical studies showed positive reactions for keratin, CK5/6, p63, CK7, EMA, and CEA in all cases tested, while bcl-2 and CD117 were rarely positive. GFAP, MSA, TTF-1, and S100 protein were non-reactive. p53 and Ki-67 were reactive to a variable degree. MEC need to be considered in the differential diagnosis of a number of sinonasal lesions, particularly adenocarcinoma and necrotizing sialometaplasia. The patients were separated into stage I (n=9), stage II (n=6), and stage III (n=4), without any patients in stage IV at presentation. Surgery occasionally accompanied by radiation therapy (n=2) was generally employed. Six patients developed a recurrence, with 5 patients dying with disease (mean, 2.4 years), while 14 patients are either alive (n=9) or had died (n=5) of unrelated causes (mean, 14.6 years). MEC probably arises from the minor mucoserous glands of the upper aerodigestive tract, usually presenting in patients in middle age with a mass. Most patients present with low stage disease (stage I and II), although invasive growth is common. Recurrences develop in about a third of patients, who experience a shorter survival (mean, 6.5 years). The following parameters, when present, suggest an increased incidence of recurrence or dying with disease: size ≥ 4.0 cm (P=0.034), high mitotic count (P=0.041), atypical mitoses (P=0.007), mixed anatomic site (P=0.032), development of recurrence (P=0.041), high tumor grade (P=0.007), and higher stage disease (P=0.027).

Endoscopic Removal of Massive Sinonasal and Skull Base Osteoblastoma

Objective: Osteoblastomas are uncommon bone neoplasms, accounting for 1% of all newly diagnosed primary bone tumors. Sinonasal and skull base osteoblastomas are exceedingly rare. We describe an endoscopic resection of a massive sinonasal/skullbase osteoblastoma, which was misdiagnosed as an ossifying fibroma. We discuss the histopathologic diagnosis of these sinonasal bony lesions. Method: Case report and current literature review. Results: A 14-year-old female presented with progressive right-sided proptosis and nasal obstruction. CT and MRI scanning demonstrated a massive sinonasal lesion with orbital and skull base involvement. An endoscopic biopsy performed by another otolaryngologist revealed a fibrous and highly vascular lesion. Histopathologic diagnosis was consistent with an ossifying fibroma. Endoscopic endonasal extended approach of this anterior skull base tumor was successfully performed with complete resection. Final histopathologic diagnosis revealed an osteoblastoma. The patient did well postoperatively without complication. Conclusion: This case aims to increase awareness of this infrequently encountered disease of the nasal cavity and paranasal sinuses and offers insight into the diagnosis and treatment of this rare entity.

Bilateral Olfactory Fossa Respiratory Epithelial Adenomatoid Hamartomas

Respiratory epithelial adenomatoid hamartoma (REAH) is a rare benign nonneoplastic sinonasal lesion that usually presents unilaterally. We report an unusual case of bilateral olfactory fossa REAHs mimicking an olfactory neuroblastoma that originated from the superior aspect of the middle turbinates. To our knowledge, only 1 similar case has been reported to date. Rhinologists and skull base surgeons should be aware of this rare entity to prevent unnecessary radical surgical interventions. Complete removal can be performed using a minimally invasive endonasal endoscopic approach.

Apparent Diffusion Coefficient Mapping for Sinonasal Diseases: Differentiation of Benign and Malignant Lesions

CT and MR imaging features of benign and malignant sinonasal lesions are often nonspecific. Therefore, we evaluated the ADC-based differentiation of these lesions. We retrospectively assessed ADCs of 61 patients with histologically proved sinonasal tumors and tumorlike lesions: 19 benign lesions, 28 malignant tumors, and 14 inflammatory lesions. Overall ADCs and percentages of total tumor area with extremely low, low, intermediate, or high ADCs (ADC mapping) were determined by using 2 b-values (500 and 1000 s/mm(2)). ADCs of malignant tumors (0.87 ± 0.32 × 10(-3) mm(2)/s) were significantly lower than those of benign (1.35 ± 0.29 × 10(-3) mm(2)/s, P < .0001) and inflammatory (1.50 ± 0.50 × 10(-3) mm(2)/s, P = .0002) lesions. On ADC mapping, percentages of total tumor area within malignant tumors having extremely low or low ADCs were significantly (P < .0001) greater than those within benign and inflammatory lesions. Cutoff points for ADC mapping (≥78% of tumor areas having extremely low or low ADCs) effectively differentiated benign or inflammatory lesions and malignant tumors with 75% sensitivity, 94% specificity, 85% accuracy, and 91% positive and 82% negative predictive values, respectively. ADCs also effectively discriminated lymphomas and SCCs from other malignant tumors. ADC mapping may be an effective MR imaging tool for the differentiation of benign/inflammatory lesions from malignant tumors in the sinonasal area.

Petrous Apex Lesions: Pictorial Review

Received January 19, 2010; accepted after revision August 4, 2010. 1Department of Radiology, Division of Neuroradiology, University of Alabama at Birmingham Medical Center, 619 19th St S, WP-150, Birmingham, AL 35249-6830. Address correspondence to A. K. Bag (abag@uabmc.edu). 2Present address: Department of Radiology, Edward Hines Jr VA Hospital, Hines, IL. AJR 2011; 196:WS26–WS37 0361–803X/11/1963–WS1 © American Roentgen Ray Society Objective The petrous apex is a pyramidal shaped, variably pneumatized structure of the skull base that forms a unique intersection between the suprahyoid neck and the intracranial compartment. Given its location, the petrous apex is susceptible to multiple pathologic processes including intrinsic lesions of bone, pneumatized air cells, or the petrous internal carotid artery; invasive “downgoing” intracranial processes; or invasive “upgoing” infiltrating nasopharyngeal or sinonasal lesions. Clinical presentations of these lesions, therefore, can be quite variable and depend largely on involvement of numerous intimately adjacent intraand extracranial structures, especially the cranial nerves. Given this variability, petrous apex lesions cannot be diagnosed accurately on the basis of clinical findings alone. Fortunately, many of these lesions have characteristic MRI and CT appearances that can often allow a precise diagnosis. The purpose of this pictorial review is to emphasize these unique imaging features in association with focused clinical presentations and brief management outlines.

Role of intraoperative CT-updates during image-guided endoscopic sinus surgery for sinonasal fibro-osseous lesions

Fibrous dysplasia, ossifying fibroma and osteomas are collectively known as fibro-osseous lesions. Occurrence in the sinonasal region is rare, and its management can be technically challenging. We report a case of an 8-year-old female with cemento-ossifying fibroma of the ethmoid sinus who underwent a two-staged, endoscopically assisted, transfacial resection of the lesion and a 31-year-old male with fibrous dysplasia of the ethmoid sinus who underwent endoscopic removal of the tumor. Both cases utilized intraoperative computed tomography (CT)-updated image-guided systems (IGS) to improve surgical precision. In the second patient, further resection was undertaken after CT-update in the same surgery. The postoperative courses were uneventful, and at 1-year follow-up no tumor recurrence was observed in either patient. Our experience suggested that the use of intraoperative CT-updated IGS in endoscopic or endoscopically assisted surgeries for sinonasal fibro-osseous lesions will lead to more optimal tumor control, which can translate into greater patient safety.

Endoscopic Graduated Multi-Angle Approach for Access to the Infratemporal Fossa: A Cadaveric Study

Introduction: The infratemporal fossa (ITF) has historically been one of the more difficult regions of the head and neck to access surgically. Available open approaches are complex, associated with high morbidity, and do not always afford optimal visualization. Endoscopic access to the ITF provides an improvement in visualization for management of the many sinonasal and lateral skull base lesions involving this region. The purpose of this study is to evaluate a graduated multi-angle approach for endoscopic access to this region using a cadaveric model.

Pediatric endoscopic skull base surgery

Pediatric endoscopic skull base surgery (ESBS) has changed the current management of pediatric sinonasal and skull base disorders. This review focuses on the recent literature exploring pediatric ESBS, including its utility and limitations. Pediatric ESBS has been established as a safe and feasible technique for a variety of disease states. Special considerations in the pediatric populations include the use of image guidance technology, potential anatomic access limitations, and skull base reconstruction with vascularized flaps to prevent cerebrospinal fluid leak. The indications for endoscopic skull base surgery continue to expand as experience and technology evolve. Pediatric endoscopic skull base surgery is a proven technique established upon adult skull base experience. Despite certain limitations, the current literature would recommend consideration for this approach for many sinonasal and skull base lesions. Further research is required to determine long-term patient outcomes.

Spindle cell carcinoma of the nasal cavity

We report an extremely rare case of spindle cell carcinoma of the sinonasal cavity. A 75-year-old man was admitted to our hospital because of right nasal obstruction. Nasal endoscopy showed a polypoid tumor measuring 3×3cm at the nasal septum in the right nasal cavity, and an excisional biopsy was performed. Computed tomography (CT) demonstrated the nasal tumor extended to the maxillary sinus. Histologically, the tumor consisted of malignant spindle cells with hyperchromatic nuclei. Mitotic figures and necrosis were recognized. In some areas, edematous changes were recognized. No apparent differentiation was noted. The tumor cells were free of keratinization and intercellular bridge formations; therefore, there were no squamous cell components. Immunohistochemically, the tumor cells were positive for pancytokeratin, cytokeratin (CK)5/6, CK18, CK19, high molecular weight CK, p63, and vimentin. The tumor cells were negative for epithelial membrane antigen, CK7, CK14, p53 protein, S100 protein, HMB45, chromogranin, synaptophysin, CD34, CD56, glial fibrillary acidic protein, neuron-specific enolase, neurofilaments, α-smooth muscle actin, neuroblastoma, myoglobin, carcinoembryonic antigen (CEA), carbohydrate antigen (CA)19-9, CD3, CD20, CD30, CD45RO, and CD45. A pathological diagnosis of spindle cell carcinoma of sinonasal lesion was made. The patient underwent resection of right maxilla followed by chemotherapy and radiation and was alive without metastasis 5years after initial presentation.