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Background Primary inverted papillomas of the middle ear are extremely rare tumors, with only few cases published in the literature. They have a characteristic epithelium surface inversion to the inside of the stroma. Inverted papillomas are benign lesions but have a high propensity of recurrence, local aggressiveness, and association with malignancy. Methods We present a patient with an inverted papilloma originating in the middle ear producing chronic otorrhea and facial nerve palsy treated with a subtotal petrosectomy. Results During the follow-up, the patient developed an intracranial invasion of the tumor causing his death. Conclusion Primary inverted papillomas of the middle ear can have an extremely aggressive behavior despite their benign characteristics; therefore, an aggressive treatment must be performed in order to minimize the possibility of recurrence. © 2015 Wiley Periodicals, Inc. Head Neck, 2015

Background Despite therapeutic improvements, patients with sinonasal squamous cell carcinoma (SCC) still face an unfavorable prognosis and there is great need for alternative treatments. Methods SCCNC4 cells, originally derived from a T2N1M0 primary and untreated sinonasal SCC, were inoculated in the maxillary sinus of immunodeficient mice. Histology, invasive behavior, and genetic features were evaluated and compared with the original primary tumor. Results The mice developed tumors that invaded bone, surrounding tissues, and brain, showing the same poor differentiation as the original primary tumor. Genetic analysis revealed an almost identical pattern of copy number alterations, except for the deletion and loss of expression of the genes CDKN2A and PTEN. Conclusion This article shows the feasibility of an orthotopic mouse model of SCC of the maxillary sinus. Completed by genome-wide genetic profiling data, this model will be useful for preclinical testing of specific gene-targeted anticancer drugs. © 2014 Wiley Periodicals, Inc. Head Neck, 2014

Background Patients with head and neck squamous cell carcinoma (HNSCC) have an unfavorable prognosis, with a 5-year survival rate of approximately 40%. Genetic analyses have revealed that the majority of HNSCCs carry complex, aneuploid karyotypes, showing numerical and structural chromosomal imbalances. New compounds are being developed that target chromosomal instability in general, specifically affecting cells with aneuploid karyotypes. Methods Two such compounds, 5-aminoimidazole-4-carboxamide riboside (AICAR) and 17-allylamino-17-demethoxygeldanamycin (17-AAG), were tested using a panel of stable diploid and unstable aneuploid HNSCC cell lines, and short-term cultures of normal keratinocytes as control. Results A significant growth inhibitory effect by both compounds was observed in the aneuploid compared to diploid HNSCC cell lines and to the normal keratinocytes. This effect was independent from the TP53 mutation status. Combination treatment with AICAR and 17-AAG demonstrated the strongest inhibition. Conclusion Aneuploidy-targeted therapy may be a viable addition to the treatment options for HNSCC. © 2014 Wiley Periodicals, Inc. Head Neck 37: 884–888, 2015

Background Nevoid basal cell carcinoma syndrome (NBCCS) is a rare, inheritable, multisystem disorder characterized by numerous basal cell carcinomas (BCCs), maxillary keratocyst, and musculoskeletal malformations. Occasionally, it is associated with malignancies like rhabdomyoma, melanoma, and sinonasal undifferentiated carcinoma, to name a few. Methods A patient presented with NBCCS with a medullary thyroid carcinoma. Clinical, surgical details, and germline genetic analysis are herein described. Results A 32-year-old woman was referred to our department with suspicion of medullary thyroid carcinoma, which was confirmed by histopathological examination. The patient was diagnosed as also having NBCCS. Germline mutation analysis indicated wild-type genes PTCH1 and RET. DNA copy number analysis by high resolution microarray comparative genomic hybridization (CGH) revealed a small interstitial loss at chromosomal band 2q37.3. Conclusion To our knowledge, this is the first described patient with NBCCS carrying a medullary thyroid carcinoma and a 2q37 deletion, which confirms that this syndrome can be associated with many different malignancies. © 2012 Wiley Periodicals, Inc. Head Neck, 2013

Background Juvenile nasopharyngeal angiofibromas (JNAs) with significant skull base involvement and intracranial extension are challenging tumors. We evaluated our experience in the treatment of extensive JNAs through resection followed by radiosurgery. Methods From 1999 to 2007, 10 advanced JNAs (Andrews grade IV) were treated by primary surgical resection followed by gamma knife radiosurgery of residual tumor. Tumor control and treatment morbidity were evaluated. Results Six months after radiosurgery and annually thereafter, an imaging study was done that revealed decrement in tumor size in 3 patients and no change in 7 patients, after a 3-year minimum follow-up. Clinically, patients are asymptomatic. Conclusion An efficient strategy in the management of extensive JNAs is the use of a multimodality approach, in which surgical resection is followed by the treatment with radiosurgery in critical locations. These therapeutic schemes are safe and offer long-term tumor control. © 2012 Wiley Periodicals, Inc. Head Neck, 2013

Item does not contain fulltext Adenocarcinomas of various types account for 10% to 20% of all primary malignant neoplasms of the nasal cavity and paranasal sinuses. There is a general consensus that the optimal treatment of adenocarcinoma is surgery and postoperative radiotherapy. The purpose of this report was to review the results of this combined treatment as well as other treatment strategies and their outcome. Most series present outcome data from a heterogeneous group of patients, with a wide variety of tumor subtypes presenting at differing stages, who received a variety of treatment strategies. Surgical excision remains the treatment of choice. The choice of approach is determined by what will best allow complete excision of the disease. Endoscopic techniques, if feasible for complete removal of the tumor, offer results comparable to those of external approaches with lower morbidity. Although clear evidence to support the use of radiotherapy in sinonasal adenocarcinoma is difficult to obtain, local control rates of combined treatment strategies for advanced cases are comparable to less advanced cases with surgery alone, suggesting a positive role for postoperative radiotherapy. However, the importance of thorough surgical resection should be stressed. (c) 2011 Wiley Periodicals, Inc. Head Neck, 2012. 01 augustus 2012

Background. Paragangliomas (PGLs) are rare tumors arising either from sympathetic or parasympathetic-associated chromaffin tissue. PGLs can occur either sporadically or as part of a hereditary syndrome. Sympathetic head and neck PGLs are extremely rare tumors and only a few cases have been reported to date. Methods. We report the pedigree of a patient with a head and neck PGL arising from the right sympathetic trunk. SDHD mutation analysis was performed using standard sequencing, multiplex ligation-dependent probe amplification, chromosome 11-specific comparative genome hybridization, and long-range/short-range polymerase chain reaction (PCR) approaches. Results. A previously unreported chromosome 11q deletion encompassing 5 annotated genes (SDHD, DLAT, PIH1D2, C11Orf57, and TIMM8B) was detected in the proband. Conclusion. PGL families considered “mutation-negative” may be attributable to large gene deletions not detectable by standard sequencing methods. Therefore, deletion analysis should be offered to families or individuals at risk for hereditary PGLs. © 2010 Wiley Periodicals, Inc. Head Neck, 2011

Malignant ethmoid and maxillary sinus tumors frequently involve the orbit. Orbital involvement is an important prognostic predictor of recurrence-free, disease-specific, and overall survival. Most authors agree that orbital preservation as opposed to orbital exenteration or clearance does not result in significant differences in local recurrence or actuarial survival. The eye can be safely preserved in most patients with ethmoid or maxillary sinus cancer invading the orbital wall, including malignancies that invade the orbital soft tissues with penetration through the periorbita provided that they can be completely dissected away from the orbital fat. Malposition of the globe and nonfunctional eyes frequently result when patients have not had adequate rigid reconstruction of the orbital floor, particularly if they have received postoperative radiotherapy. This underscores the importance of such reconstruction. Isolated defects following orbital exenteration may be reconstructed with a temporalis muscle flap. Microvascular free-tissue transfer is the best option for repair of defects following orbital exenteration and total maxillectomy, although an obturator still has a role in selected patients.