Spindle Cell Lipoma Arising from the Supraglottis: A Case Report and Review of the Literature.

Lipomas are common benign mesenchymal neoplasms. Although 13% of lipomas are found in the head and neck, only 0.6% have been reported in the larynx. Of all lipomas, the spindle cell variant is the least common. In the present study, we report a case of supraglottic spindle cell lipoma and review the literature of laryngeal spindle cell lipoma. A 35-year-old male presented with dysphagia and dyspnea and was found to have bilateral supraglottic lesions causing airway obstruction. The masses were resected endoscopically. Final pathology demonstrated mature adipocytes and spindle cells, with immunohistochemical patterns supportive of spindle cell lipoma. Spindle cell lipomas have rarely been reported in the upper airway. To our knowledge, this is the youngest patient reported to date. These lipomas are uncommon benign neoplasms and should be distinguished from aggressive mesenchymal neoplasms such as liposarcoma variants to guide appropriate conservative but curative therapy.

Immunologic mediators of outcome for irradiated oropharyngeal carcinoma based on human papillomavirus status.

PURPOSE: To investigate the prognostic value of pre-treatment immune parameters including white blood cell count (WBC) and circulating lymphocyte count (CLC) among patients with oropharyngeal carcinoma treated by radiation therapy. METHODS AND MATERIALS: A total of 136 consecutive patients were treated by radiation therapy for locally advanced (stage III/IV) squamous cell carcinoma of the oropharynx with known human papillomavirus (HPV) status. Medical records were reviewed to identify patients with documented pre-treatment laboratory bloodwork. The Kaplan-Meier method and linear regression models were used to evaluate the association between pre-treatment CBC and CLC values with survival endpoints. RESULTS: One hundred and eleven patients satisfied inclusion criteria. Median age was 62 years (range, 22-91). Eighty-four patients were HPV-positive (76%) and 27 (24%) were HPV-negative. There was no difference in WBC and CLC mean values at baseline between HPV-positive and HV-negative (p > 0.05, for both). Trends were detected in the HPV-positive cohort favoring patients with higher CLC, with respect to 2-year local-regional control (93% vs. 82%, p = 0.06) and distant control (88% vs. 82%, p = 0.10) using the median CLC as cut-off. HPV-positive patients with CLC values in the lowest quartile had inferior local-regional control compared to those in the upper 3 quartiles (69% vs. 89%, p = 0.01). CONCLUSION: Low pre-treatment CLC was correlated with local-regional recurrence and distant failure among HPV-positive patients. These associations were not observed in the HPV-negative cohort.

Prognostic significance of p16 in squamous cell carcinoma of the larynx and hypopharynx.

PURPOSE: To evaluate the prognostic significance of p16 expression among patients with squamous cell carcinoma of the larynx (LSCC) and hypopharynx (HSCC). METHODS: The medical records of all patients with locally advanced, non-metastatic LSCC/HSCC were reviewed. p16INK4A (p16) protein expression was evaluated on pathological specimens by immunohistochemistry (IHC), and the Kaplan-Meier method was used to estimate overall survival (OS) and locoregional control (LRC). In select cases, p16 expression was correlated to high-risk and low-risk HPV genotypes using in situ hybridization (ISH). RESULTS: Thirty-one patients (23 LSCC; 8 HSCC) were identified. Seventeen (54.8%) patients were p16 negative; 14 (45.2%) were p16-positive. The primary treatment modality was radiation therapy for 22 (71.0%) patients and surgery for 9 (29.0%). Nineteen (61.3%) patients were evaluated for high-risk HPV and low-risk HPV genotypes by IHC, of whom 2 (10.5%) patients were positive for high-risk HPV and 1 (5.3%) was positive for low-risk HPV. For high-risk HPV, the positive predictive value (PPV), sensitivity, and specificity of p16 was 20.0%, 100%, and 52.9%. There was no significant difference in the 2-year actuarial rates of OS (91% vs. 64%, p=0.34) or LRC (51% vs. 46%, p=0.69) between the p16-positive and p-16 negative patients. CONCLUSION: In this small cohort of 31 LSCC and HSCC patients, p16 was not a significant predictive of either LRC or OS. Furthermore, p16 was poorly correlated with HPV genotyping as identified by ISH.

Incidental finding of lymphoma after septoplasty.

INTRODUCTION: Septoplasty, or surgical correction of the deviated septum, is an elective, routinely performed rhinologic procedure to address nasal airway obstruction. In many cases, resected septal cartilage and bone fragments are sent for pathologic review, although there is no consensus on this practice. We reported two cases of incidentally diagnosed lymphoma after elective septoplasty and discussed clinical presentation, diagnosis, and management. METHODS: Retrospective chart review of two patients who underwent septoplasty at a tertiary academic medical center and found to have incidental lymphoma based on histopathology. RESULTS: Two patients who underwent septoplasty had an incidental diagnosis of lymphoma on pathologic analysis. One patient was noted to have an S-shaped septal deviation that produced bilateral nasal obstruction. She underwent a difficult septoplasty, in which the mucoperichondrial flap was firmly adherent to the underlying septum and bone. Final pathology demonstrated diffuse large B-cell lymphoma. She was treated with chemoradiation and remained free of disease at 59 months. The other patient had a history of nasal trauma, which produced left septal deviation. He underwent an uncomplicated septoplasty, with pathology that demonstrated low-grade B-cell lymphoma. Because there was no evidence of active disease, the decision was made to not treat and to observe the patient clinically. CONCLUSIONS: This is the first reported series of septal lymphoma incidentally diagnosed on routine septoplasty. Although histopathologic review of specimens from routine nasal and sinus surgery is not routinely performed, this report highlighted the importance of this process, on a case-by-case basis, in detecting unexpected malignancies that otherwise were clinically silent.

Laryngeal oncocytic cystadenomas masquerading as laryngoceles.

OBJECTIVE: To describe an experience with laryngeal oncocytic cystadenomas and review the published literature regarding this uncommon diagnosis. METHODS AND RESULTS: A clinical review of patients presenting with cystic laryngeal masses in an urban academic medical center between January and December 2013 was performed. Three patients, two female and one male, with a mean age of 68 years, were diagnosed with oncocytic cystadenomata of the larynx. Major presenting symptoms included dysphonia, globus, and ipsilateral otalgia. Endoscopic examinations revealed a cystic structure arising from varied subsites of the larynx: laryngeal ventricle, aryepiglottic fold, and pre-epiglottic space. Cross-sectional radiographic imaging was obtained in each case. The patients were treated with transoral (CO2) laser microsurgery (TLM). In all three cases, pathological analysis revealed oncocytic cystadenoma with clear margins. CONCLUSIONS: Oncocytic cystadenoma is a rare entity of the larynx predominantly affecting elderly patients. Clinical presentation and imaging may suggest the diagnosis of an internal laryngocele. Complete excision is both diagnostic and therapeutic, and typically can be achieved using TLM. When clear margins are obtained, no adjuvant therapy is indicated. Although laryngoceles and malignancies are more commonly encountered, oncocytic cystadenomas should remain in the differential of cystic laryngeal masses.

Hepatic Fibrinogen Storage Disease in a Patient with Hypofibrinogenemia: Report of a Case with a Missense Mutation of the FGA Gene.

We report a 9-year-old patient with abnormal liver tests found incidentally during routine bloodwork as part of a preoperative evaluation for excision of a benign cyst. A liver biopsy demonstrated hepatocytes to have pale and expanded cytoplasm that contained multiple vague globular eosinophilic inclusions. Electron microscopy showed fingerprint-like structures in the dilated cisternae of the rough endoplasmic reticulum, characteristic of fibrinogen. Whole exome sequencing identified a heterozygous missense mutation at codon 35 of the fibrinogen α (FGA) gene. No mutation was identified in the ß or γ chains. His plasma fibrinogen levels were found to be decreased to 85 mg/dL (normal range 215-464). His family history was pertinent for his mother and maternal grandfather with hypofibrinogenemia. He had not had any significant bleeding episodes except for minor bruising over the shins. This case illustrates a rare etiology of storage disease that causes abnormal liver function tests.

An unusual initial presentation of mantle cell lymphoma arising from the lymphoid stroma of warthin tumor.

BACKGROUND: Warthin tumors presenting concomitantly with a lymphoma is vanishingly rare with only 15 reported cases in English literature. Herein, we report an unusual initial presentation of a mantle cell lymphoma involving the lymphoid stroma of a Warthin tumor. CASE PRESENTATION: A seventy-seven year old otherwise healthy gentleman with a 50-pack year smoking history presents with a slowly enlarging left cheek mass. CT scan of the neck demonstrated a left parotid gland tumor measuring 3.4 cm in greatest dimension. He underwent a left superficial parotidectomy, with subsequent histopathologic examination revealing a Warthin tumor with extensive expansion of the lymphoid stroma. Flow cytometric, immunohistochemical, and cytogenetic studies of the stromal component of the tumor confirmed the presence of a mantle cell lymphoma. Clinical staging demonstrated stage IVa disease, and was considered to be at low to intermediate risk due to the slow growth of the parotid lesion. The patient is undergoing close follow up with repeat PET-CT scans at six months. CONCLUSION: To the best of our knowledge, this is the first well documented collision tumor between mantle cell lymphoma and a Warthin tumor. This case also brings to light the significance of thorough evaluation of the lymphoid component of Warthin tumor.

Chondroid chordoma of the sella turcica mimicking a pituitary adenoma.

We report an unusual case of a chondroid chordoma of the sella turcica that mimicked the clinical and radiologic presentation of the more common pituitary adenoma. A 50-year-old man presented with bitemporal visual field deficits. Magnetic resonance imaging (MRI) detected a sellar mass that was suggestive of a pituitary adenoma. However, the intraoperative appearance of the mass was not consistent with an adenoma, and frozen-section pathology was obtained. Pathology identified the mass as a malignant lesion. Based on this finding, the mass was treated more aggressively. Chondroid chordomas are rare and slowly growing but locally aggressive tumors. The prognosis depends on the ability to totally resect the mass, so differentiating this tumor from a benign lesion is critical. An intrasellar chordoma can be confused clinically and radiologically with a pituitary adenoma. These two lesions are nearly identical on MRI, although T2-weighted imaging sometimes demonstrates higher intensity with a chondroid chordoma. Computed tomography may be helpful in demonstrating bony destruction by these lesions, as can the presence of intralesional calcifications. Intraoperative findings of bony invasion or a purple-red color may also lead the surgeon to suspect a diagnosis other than pituitary adenoma.

Basaloid squamous cell carcinoma of the maxilla: Report of a case and literature review.

PURPOSE: Basaloid squamous cell carcinoma (BSCC) is a rare variant of squamous cell carcinoma characterized by a highly aggressive clinical course. Though typically found in the larynx, oropharynx, and hypopharynx, we report a rare case of BSCC originating in the maxillary sinus in an otherwise healthy 32-year-old male. MATERIALS AND METHODS: Single case report of a patient with BSCC of the maxillary sinus and retrospective chart review of all cases of BSCC of the maxilla at a single academic institution between January 1, 1986 and December 31, 2013. The MEDLINE database was additionally queried for all case series or reports of BSCC arising in the maxilla, and pertinent clinical data were extracted. RESULTS: The clinical presentation, disease course, and management of a patient with BSCC of the maxilla are presented. In this recent case, the patient presented with persistent alveolar pain and a nonhealing tooth infection. Radiographic studies demonstrated a large necrotic mass in the left maxillary sinus that was biopsy-proven as BSCC. The patient underwent surgical resection followed by postoperative radiation without complications. CONCLUSIONS: BSCC of the maxilla is a rare oncologic entity that may progress to late disease stage without obvious clinical signs or symptoms. Optimal treatment involves complete surgical resection followed by postoperative.

Carcinoma ex-pleomorphic adenoma of the parotid gland consisting of high-grade salivary duct carcinoma and keratinizing squamous cell carcinoma.

Carcinoma ex-pleomorphic adenoma (CXPA) is a rare salivary gland malignancy that presents diagnostic difficulties partly because of its wide range of histologic presentations. We report a case of a 77-year-old man, who presented with a 6-year history of a parotid mass that had undergone rapid growth within weeks. Magnetic resonance imaging revealed an infiltrative mass in the parotid gland, and the fine-needle aspiration (FNA) biopsy result was highly suspicious for carcinoma. Subsequent excision of the tumor demonstrated a poorly differentiated epithelial neoplasm consisting of keratinizing squamous cell carcinoma (SCC) and adenocarcinoma with regions of both ductal carcinoma in situ and invasive salivary duct carcinoma (SDC). Only focal areas exhibited a benign pleomorphic adenoma component. To our knowledge, this is the first case of a CXPA that consists of both a high-grade SDC and a keratinizing SCC in the parotid gland.

Sinonasal lymphoma: case series and review of the literature.

BACKGROUND: Sinonasal lymphoma is a rare rhinologic entity. We present a case series and review the literature surrounding the diagnosis and management of this disease. METHODS: A pathology database spanning 22 years at a tertiary care center was searched for a diagnosis of lymphoma in the paranasal sinuses or the nasal cavity. Seventeen cases were identified, and retrospective chart review was performed. RESULTS: Maxillary and ethmoid sinuses were affected more frequently (n = 8 patients each) than sphenoid and frontal sinuses (n = 5 patients each). Histologically, the most common type was diffuse large B-cell lymphoma (53%, 9 patients), followed by extranodal natural killer/T-cell lymphoma (ENKL, 21%, 3 patients). Presenting symptoms included nasal obstruction and rhinorrhea (53%, 9 patients) and diplopia (18%, 3 patients); and radiographic imaging demonstrated a discrete mass (59%, 10 patients), sinus opacification (53%, 9 patients), and/or bony erosion (35%, 6 patients). Treatment included chemotherapy alone (71%, 12 patients), chemotherapy and radiation (6%, 1 patient), and radiation alone (6%, 1 patient). The 2-year and 5-year overall survival rates were 75% and 53%, respectively, whereas disease-free 2-year and 5-year survival rates were 70% and 49%, respectively. CONCLUSION: Lymphoma of the nasal cavity and paranasal sinuses is extremely rare, may mimic benign processes, and may manifest either in an isolated fashion or in conjunction with systemic disease. B-cell lymphomas, a more favorable diagnosis, account for a majority of cases, whereas ENKL is associated with rapid disease progression and death. Chemotherapy and radiation are the main therapies. Histologic diagnosis is of paramount importance, and clinicians must remain cognizant of this entity to differentiate it from other sinonasal malignancies.

Tumor cells with low proteasome subunit expression predict overall survival in head and neck cancer patients.

BACKGROUND: Experimental and clinical data suggest that solid cancers contain treatment-resistant cancer stem cells that will impair treatment efficacy. The objective of this study was to investigate if head and neck squamous cell carcinoma (HNSCC) also contain cancer stem cells that can be identified by low 26S proteasome activity and if their presence correlates to clinical outcome. METHODS: Human HNSCC cells, engineered to report lack of proteasome activity based on accumulation of a fluorescent fusion protein, were separated based on high (ZsGreen-cODCneg) or low (ZsGreen-cODCpos) proteasome activity. Self-renewal capacity, tumorigenicity and radioresistance were assessed. Proteasome subunit expression was analyzed in tissue microarrays and correlated to survival and locoregional cancer control of 174 patients with HNSCC. RESULTS: HNSCC cells with low proteasome activity showed a significantly higher self-renewal capacity and increased tumorigenicity. Irradiation enriched for ZsGreen-cODCpos cells. The survival probability of 82 patients treated with definitive radio- or chemo-radiotherapy exhibiting weak, intermediate, or strong proteasome subunit expression were 21.2, 28.8 and 43.8 months (p = 0.05), respectively. Locoregional cancer control was comparably affected. CONCLUSIONS: Subpopulations of HNSCC display stem cell features that affect patients' tumor control and survival. Evaluating cancer tissue for expression of the proteasome subunit PSMD1 may help identify patients at risk for relapse.

A pregnant dilemma: primary hyperparathyroidism due to parathyromatosis in pregnancy.

OBJECTIVE: To describe an exceedingly rare case of parathyromatosis in pregnancy and the limited medical treatment options available for such cases that are refractory to surgery. METHODS: Case presentation and description of clinical course with brief review of the literature. RESULTS: A 21-year-old woman with a history of 3.5 gland parathyroidectomy presented with severe hyperemesis during her first trimester of pregnancy and was found to have primary hyperparathyroidism attributable to parathyromatosis. We describe the diagnostic and management dilemmas associated with this case, which included localization of the culprit lesions, a technically challenging surgical resection and subsequent medical management with cinacalcet when symptomatic hypercalcemia recurred during the third trimester. To our knowledge, this is only the third report of the successful use of cinacalcet during pregnancy, and the first case report of parathyromatosis presenting during pregnancy. CONCLUSION: Cinacalcet was used safely and effectively during the third trimester of pregnancy to treat symptomatic hypercalcemia due to parathyromatosis.

Infarction of papillary thyroid carcinoma after fine-needle aspiration: case series and review of literature.

IMPORTANCE: Although infarction after fine-needle aspiration (FNA) is a rare occurrence, it is a known phenomenon that may lead to difficulties in interpretation for pathologists and in decision-making for head and neck surgeons. OBJECTIVE: To characterize our experience with infarction in papillary thyroid carcinomas (PTCs) after FNA and review existing cases of infarcted PTCs in the literature to better understand this phenomenon. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective case series and review of literature at a tertiary medical center (University of California, Los Angeles [UCLA], Medical Center). All patients who had a surgical pathologic diagnosis of infarcted PTC and who underwent FNA prior to surgery at UCLA from June 2006 to June 2012 were identified. There were 620 cases of PTC and 12 cases of infarcted PTC. MAIN OUTCOMES AND MEASURE: Demographic data, FNA cytologic findings, and surgical pathologic data were gathered for each patient. A comprehensive literature search for infarcted PTC was performed. RESULTS: Twelve cases of infarcted PTC were found in a total of 620 cases of PTC (1.9%). The mean (SD) time interval between the last FNA and surgery was 52 (35) days (range, 13-133 days). All patients received a diagnosis of infarcted PTC after thyroidectomy was performed. Focal infarction was found in 4 patients (33%), and near-total infarction was found in 8 patients (67%). Five patients (47%) had the follicular variant of PTC, making it the most common subtype in our series. A thorough literature search yielded 11 articles reporting a total of 26 cases of infarcted PTC after FNA. To our knowledge, our case series on infarcted PTC is the largest reported series in the literature. CONCLUSIONS AND RELEVANCE: Although infarction of PTC after FNA occurs infrequently, it may lead to difficulties in histologic diagnosis. Awareness of this phenomenon and its histologic associations, along with careful reevaluation of the FNA and surgical specimens, is important for appropriate diagnosis and subsequent treatment. At this point, infarction in PTC should not alarm a head and neck surgeon to change management, but future prospective studies with a large population of patients with infarcted PTCs are needed to establish the impact of infarction on differences in treatment outcomes for therapies that may be used in PTCs.

COX-(2) overexpression in sinonasal inverted papilloma.

BACKGROUND: Inverted papilloma (IP) is a benign, but locally aggressive neoplasm of the nasal cavity and paranasal sinuses. The mainstay of treatment of IP is surgical resection, but high rates of tumor recurrence have been reported. Cyclo-oxygenase-2 (COX-2) has been found to be overexpressed in many tumors and has been used successfully as a therapeutic target. The goal of this study is to highlight COX-2 expression in IP. METHODS: Immunohistochemistry for COX-2 was performed on IP samples obtained during surgical resection between January 2012 and June 2013. The intensity of staining was evaluated by 2 head and neck pathologists blinded to the clinical features and outcomes. A positive stain was defined as having 10% or more of tumor cells exhibiting immunoreactivity. RESULTS: The study includes 13 tumor samples from 7 females and 6 males. Mean age was 54.5 (range, 18-81) years. Tumor locations included: nasal septum (1), ethmoid (3), sphenoid (4), and maxillary (5) sinuses. No tumors demonstrated malignancy or dysplasia. Five (38%) of the 13 IP samples stained strongly positive for COX-2, and 3 of 13 (23%) stained weakly positive. There were no clinical differences in patients that stained strongly or weakly for COX-2. CONCLUSION: COX-2 overexpression was identified in 62% of cases of IP in this study, and strongly positive in 38% of cases. Larger studies are necessary to identify the true incidence of COX-2 expression for this tumor. Pharmaceuticals targeting COX-2 may eventually provide an additional therapeutic option for select cases of recurrent or unresectable IP.

Solitary fibrous tumor of the hypopharynx: case report and literature review.

Solitary fibrous tumors are rare mesenchymal neoplasms that are increasingly being described in the head and neck. Clinical presentations may include compression by these tumors on vital surrounding structures. While malignant transformation is rare, treatment entails wide local excision. We present the case of a 74 year-old female with an increasingly enlarging symptomatic hypopharyngeal solitary fibrous tumor that was found on carotid duplex ultrasound. Transoral surgical excision resulted in relief of symptoms. Treatment options are discussed and a literature review of this uncommon disorder presented.

Schneiderian papillomas: comparative review of exophytic, oncocytic, and inverted types.

BACKGROUND: Sinonasal papillomas are benign epithelial neoplasms arising from Schneiderian mucosa. The three subtypes, exophytic, oncocytic, and inverted (inverted papilloma [IP]), should be distinguished from one another histopathologically. This study (1) highlights the histopathological and clinical differences between the Schneiderian papilloma subtypes and (2) identifies clinical features that potentially predict papilloma subtypes. METHODS: A retrospective review was performed of patients with Schneiderian papillomas over an 11-year period. RESULTS: Seventy patients with sinonasal papillomas who underwent sinus surgery were identified. There were 50 (71%) male and 20 (29%) female subjects diagnosed at an average age of 53 years (range, 13-80 years). Exophytic (n = 25), oncocytic (n = 9), and IP (n = 37) were identified. IP was associated with transformation into squamous cell carcinoma in three (8%) cases and dysplasia in three (8%) cases. Neither oncocytic nor exophytic subtypes were associated with dysplasia or malignancy. On multivariate analysis of potential predictors of papilloma subtype, history of chronic rhinosinusitis (CRS) and location of papilloma were significantly associated with papilloma subtype. Using classification and regression tree model, papilloma subtypes can be predicted based on presence or absence of CRS and papilloma location with nominal 82.4% accuracy. CONCLUSION: The inverted and exophytic type are the most common sinonasal papillomas, with the inverted type having an 8% rate of malignant transformation in this study. In contrast, the oncocytic type was not associated with dysplasia or malignancy in our series despite reports in the literature indicating malignant potential. History of CRS and papilloma location can provide clues to the histological subtype, which is important for surgical planning and patient counseling.

A Novel Exonic Splicing Mutation in the TAZ (G4.5) Gene in a Case with Atypical Barth Syndrome.

OBJECTIVE: Barth syndrome is an X-linked recessive disorder characterized by dilated cardiomyopathy, neutropenia, 3-methylglutaconic aciduria, abnormal mitochondria, variably expressed skeletal myopathy, and growth delay. The disorder is caused by mutations in the tafazzin (TAZ/G4.5) gene located on Xq28. We report a novel exonic splicing mutation in the TAZ gene in a patient with atypical Barth syndrome. PATIENT & METHODS: The 4-month-old proband presented with respiratory distress, neutropenia, and dilated cardiomyopathy with reduced ejection fraction of 10%. No 3-methylglutaconic aciduria was detected on repeated urine organic acid analyses. Family history indicated that his maternal uncle died of endocardial fibroelastosis and dilated cardiomyopathy at 26 months. TAZ DNA sequencing, mRNA analysis, and cardiolipin analysis were performed. RESULTS: A novel nucleotide substitution c.553A>G in exon 7 of the TAZ gene was identified in the proband, predicting an amino acid substitution p.Met185Val. However, this mutation created a new splice donor signal within exon 7 causing mis-splicing of the message, producing two messages that only differ in the presence/absence of exon 5; these retain intron 6 and have only 11 bases of exon 7. Cardiolipin analysis confirmed the loss of tafazzin activity. The proband's mother, maternal aunt, and grandmother carry the same mutation. CONCLUSIONS: The identification of a TAZ gene mutation, mRNA analysis, and monolysocardiolipin/cardiolipin ratio determination were important for the diagnosis and genetic counseling in this family with atypical Barth syndrome that was not found to be associated with 3-methylglutaconic aciduria.

A unique case of sclerosing polycystic adenosis of the sinonasal tract.

Sclerosing polycystic adenosis is an extremely uncommon, recently described, sclerosing lesion of the salivary glands that appears histologically similar to fibrocystic changes of the breast. The key histopathologic features of sclerosing polycystic adenosis include lobular proliferation of ductal and acinar elements, cystically dilated ducts exhibiting frequent apocrine and sebaceous metaplasia, eosinophilic intracytoplasmic granules within some acinar-type cells, intraductal epithelial hyperplasia, and dense fibrosis. Most described cases have occurred in the major salivary glands, particularly the parotid gland. Although most authorities consider sclerosing polycystic adenosis to be a pseudoneoplastic process, the occurrence of dysplasia and carcinoma in situ of ductal epithelium reported recurrence rates of up to 30%, and recent evidence of clonality suggests a possible neoplastic etiology. However, there have been no cases of metastasis. Herein, we report the first case of sclerosing polycystic adenosis of the sinonasal tract in a 79-year-old woman presenting with a sinonasal mass.