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sinonasal undifferentiated carcinoma

Alejandro López-Hernández, Blanca Vivanco, Alessandro Franchi, Elisabeth Bloemena, Virginia N Cabal, Sira Potes, Cristina Riobello, Cristina García-Inclán, Fernando López, José L Llorente, Mario Hermsen
The sinonasal cavities harbour a variety of rare tumour types. Many carry a poor prognosis while therapeutic options are limited. Histopathological classification can be difficult, especially for poorly differentiated tumours such as olfactory neuroblastoma (ONB), sinonasal neuroendocrine carcinoma (SNEC) and sinonasal undifferentiated carcinoma (SNUC). We analysed Affymetrix OncoScan genome-wide copy number profiles of these three tumour types, both as originally diagnosed and as regrouped by their cytokeratin (Ck) and neuroendocrine (Ne) expression pattern, aiming to find a relation between phenotype and genotype...
March 5, 2018: Scientific Reports
A Agaimy, F Haller, A Hartmann
The last two decades have seen significant advances in the pathology of sinonasal tract neoplasms. This was the consequence of the availability of several innovative diagnostic tools, which resulted in a dynamic evolution of entities and splitting of newly defined or conceptualized entities and subtypes that have been included in the spectrum of old heterogeneous diseases. Most of these new tumor subtypes have distinctive demographic, clinicopathologic, and biological characteristics with prognostic and therapeutic implications for individual patients...
February 2, 2018: Der Pathologe
B Sipos
Neuroendocrine neoplasms (NENs) are infrequent in sensory organs. There are well-differentiated neuroendocrine neoplasms that should be classified as neuroendocrine tumors, in analogy to their gastrointestinal counterparts, however the nomenclature is inconsistent. The best defined entities are neuroendocrine tumors in the middle ear and ectopic pituitary adenoma in the sphenoid region. Poorly differentiated NENs most often arise in the olfactory organ and nasal cavity that are represented by olfactory neuroblastomas and poorly differentiated neuroendocrine carcinomas...
February 1, 2018: Der Pathologe
Vera Comiati, Maria Luisa Scapellato, Enrico Alexandre, Tiziana Volo, Daniele Borsetto, Mariella Carrieri, Enzo Emanuelli, Diego Cazzador
BACKGROUND: Occupational exposure to chromium is carcinogenic for human respiratory system. Due to the low incidence of sinonasal malignancies, there is still a paucity of evidence to confirm that chromium(VI) exposure is a cause of nasal cancer. OBJECTIVES: To report on a sinonasal cancer (SNC) of rare occupational origin, increasing the awareness on epidemiological knowledge of occupational exposures to chromium compounds. METHODS: We describe a case of a 64-year-old chrome plater who worked in the galvanic industry in the early 1970s...
December 14, 2017: La Medicina del Lavoro
Grégoire B Morand, Nanina Anderegg, Domenic Vital, Kristian Ikenberg, Gerhard F Huber, Michael B Soyka, Matthias Egger, David Holzmann
OBJECTIVE: Sinonasal undifferentiated carcinoma (SNUC) is an aggressive malignancy first described by Frierson et al. in 1986. As the tumor is very rare, current treatment recommendations are based on institutional case reports. We thus felt the need to perform a comprehensive systematic review and meta-analysis to investigate how treatment modalities are associated with survival. DESIGN: Case-series, systematic review and meta-analysis METHODS: We searched the OvidMedline, OvidEmbase, Web of Science, Biosis, Scopus and the Cochrane Library database libraries...
December 2017: Oral Oncology
Jong Seung Kim, Ki Hwan Hong, Kyu Yun Jang, Jong Hoon Song
RATIONALE: Sinonasal inverted papilloma (IP) is a locally aggressive tumor found in the schneiderian membrane, lining the nasal cavity and paranasal sinuses. PATIENT CONCERNS: We report a case of a 63-year-old woman with undifferentiated carcinoma (UC) arising in an IP in the left maxillary sinus. DIAGNOSES: The preoperative diagnosis was IP of the maxillary sinus. INTERVENTIONS: We performed endoscopic medial maxillectomy to remove the remnant mass in the left maxillary sinus...
November 2017: Medicine (Baltimore)
Hiroshi Minato, Eriko Kobayashi, Satoko Nakada, Nozomu Kurose, Mio Tanaka, Yukichi Tanaka, Shioto Suzuki, Fumihiko Tanioka, Yutaka Saikawa, Takaki Miwa, Takayuki Nojima
Nuclear protein in testis (NUT) carcinoma is a rare malignant neoplasm with an undifferentiated morphology. Its diagnosis is often difficult, especially as the sinonasal tract gives rise to many tumors with undifferentiated morphologies. Not many cases of sinonasal NUT carcinomas have been reported, and its clinicopathological features have not been sufficiently clarified. In this study, we performed a clinicopathological study of 4 patients with sinonasal NUT carcinoma, including wide-ranging immunohistochemical tests and cytogenetic analyses using fluorescence in situ hybridization and DNA sequencing...
October 24, 2017: Human Pathology
Carolyn A Orgain, Terry Y Shibuya, Lester D Thompson, David B Keschner, Rohit Garg, Jivianne T Lee
INTRODUCTION: Inverted papillomas (IP) are benign sinonasal neoplasms, which account for 0.5-4% of all nasal tumors. IPs have been known to transform into squamous cell carcinoma in 5-15% of cases. Rarely, transformations to other malignancies have been reported. Here we report a unique case of malignant transformation of an IP into sinonasal undifferentiated carcinoma (SNUC). METHODS: A case report with a literature review; institutional review board exempted. The clinical presentation, radiographic features, surgical intervention, histopathologic analysis, treatment, and outcome of the case were examined...
October 1, 2017: Allergy & Rhinology
Aanchal Kakkar, Vijay Mariadas Antony, David Victor Kumar Irugu, Narayan Adhikari, Deepali Jain
NUT midline carcinomas (NMCs) are rare, poorly differentiated tumors with aggressive biological behavior and a characteristic molecular signature. Availability of NUT antibody has facilitated diagnosis of NMC without molecular testing. We report a series of head and neck NMCs diagnosed using NUT IHC at our institute, including one case with an unusual course. Immunohistochemistry for NUT was performed in nasal and sinonasal tumors with diagnoses of undifferentiated carcinoma, poorly differentiated squamous cell carcinoma and malignant neoplasm, not otherwise specified, to identify cases of NMC...
September 25, 2017: Head and Neck Pathology
Arthur Wang, John V Wainwright, Anubhav G Amin, Eric Vail, Rachel Silverstein, Kaushik Das
Sinonasal undifferentiated carcinoma (SNUC) is a rare malignancy of the upper airways and anterior skull base that carries a poor prognosis. The tumor is known to be invasive into the surrounding structures of the skull base and brain. To date, there is only one existing case report documenting drop metastasis to the intradural extramedullary spinal cord. To the best of our knowledge, we present the second case of metastatic SNUC to the spine. This report describes a 59-year-old male with a history of head and neck SNUC who presented with thoracic back pain and bilateral lower extremity paresis...
May 2017: Case Reports in Oncology
Morten Jørgensen, Steffen Heegaard
Tumors that invade the orbit are uncommon. The majority are meningiomas arising from the sphenoid ridge (66%). Others are bone and cartilage tumors arising from the surrounding bones of the orbit, pituitary adenomas, and epithelial tumors arising from the paranasal sinuses and nasal cavity. Meningiomas occur more often in women, whereas epithelial tumors have a predilection for men. Meningiomas and epithelial tumors typically present in the sixth decade of life, whereas bone tumors tend to affect individuals in their third decade of life...
July 22, 2017: Survey of Ophthalmology
Austin McCuiston, Justin A Bishop
NKX2.2 is a new immunohistochemical marker that has been reported to be sensitive and specific for Ewing sarcoma (ES). It has not, however, been investigated specifically in the sinonasal small round blue cell tumor (SRBCT) differential diagnosis which includes many tumors specific to that site. It has also not been investigated in the newly recognized "adamantinoma-like" variant of ES. Immunohistochemistry for NKX2.2 was performed on 170 poorly differentiated sinonasal neoplasms: 73 squamous cell carcinomas (67 poorly differentiated, non-keratinizing, or basaloid types and 6 nasopharyngeal carcinomas), 46 olfactory neuroblastomas, 8 sinonasal undifferentiated carcinomas (SNUCs), 6 melanomas, 7 Ewing sarcomas, 6 SMARCB1-deficient carcinomas, 6 teratocarcinosarcomas, 5 alveolar rhabdomyosarcomas, 4 solid adenoid cystic carcinomas, 4 NK/T cell lymphomas, 3 NUT carcinomas, and 2 small cell carcinomas...
June 14, 2017: Head and Neck Pathology
Zia-Us-Salam Qazi, Sarfraz Latif, Sadia Maqsood Awan
BACKGROUND: Orbital involvement in sinonasal diseases can present as proptosis, ophthalmoplegia or even as blindness due to optic nerve damage. There are a number of sinonasal diseases which can involve eyes. The purpose of this study was to enlist diagnoses of all the patients with sinonasal disease, in which orbit was also involved unilaterally or bilaterally and to analyse the management strategy and final outcome in all the cases. METHODS: Hundred consecutive patients having orbital symptoms along with sinonasal complaints that presented in ENT department of Shaikh Zayed federal postgraduate medical institute were included in our prospective study...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
Mauricio E Gamez, Devyani Lal, Michele Y Halyard, William W Wong, Carlos Vargas, Daniel Ma, Stephen J Ko, Robert L Foote, Samir H Patel
BACKGROUND: Sinonasal undifferentiated carcinoma (SNUC) is a rare aggressive disease arising in the nasal cavity and paranasal sinuses with poor prognosis and unclear optimal management. METHODS: Forty patients were analyzed. Nasal cavity was the most common primary site. Most patients presented with T4 disease, received trimodality therapy, and were treated with intensity-modulated radiotherapy (IMRT). RESULTS: Median follow-up was 6.9 years...
September 2017: Head & Neck
Snjezana Dogan, Deborah J Chute, Bin Xu, Ryan N Ptashkin, Raghu Chandramohan, Jacklyn Casanova-Murphy, Khedoudja Nafa, Justin A Bishop, Simion I Chiosea, Edward B Stelow, Ian Ganly, David G Pfister, Nora Katabi, Ronald A Ghossein, Michael F Berger
Sinonasal undifferentiated carcinoma (SNUC) is a high-grade malignancy with limited treatment options and poor outcome. A morphological spectrum of 47 sinonasal tumours including 17 (36.2%) SNUCs was analysed at genomic level. Thirty carcinomas (cohort 1) were subjected to a hybridization exon-capture next-generation sequencing assay (MSK-IMPACT(TM) ) to interrogate somatic variants in 279 or 410 cancer-related genes. Seventeen sinonasal tumours (cohort 2) were examined only for the presence of IDH1/2 exon 4 mutations by Sanger sequencing...
August 2017: Journal of Pathology
Abbas Agaimy, Arndt Hartmann, Cristina R Antonescu, Simion I Chiosea, Samir K El-Mofty, Helene Geddert, Heinrich Iro, James S Lewis, Bruno Märkl, Stacey E Mills, Marc-Oliver Riener, Thomas Robertson, Ann Sandison, Sabine Semrau, Roderick H W Simpson, Edward Stelow, William H Westra, Justin A Bishop
To more fully characterize the clinical and pathologic spectrum of a recently described tumor entity of the sinonasal tract characterized by loss of nuclear expression of SMARCB1 (INI1), we analyzed 39 SMARCB1-deficient sinonasal carcinomas collected from multiple medical centers. The tumors affected 23 males and 16 females with an age range of 19 to 89 years (median, 52). All patients presented with locally advanced disease (T3, n=5; T4, n=27) involving the sinuses (mainly ethmoid) with variable involvement of the nasal cavity...
April 2017: American Journal of Surgical Pathology
I S S Alves, L G S Berriel, R T Alves, M B Pinto, C F P Oliveira, A C Cazzotto, W V Moura
Purpose. Sinonasal malignant mucosal melanoma is a rare, aggressive tumour. Nasal obstruction and epistaxis are the most commonly reported symptoms, although symptomatology may develop late and be nonspecific, which tends to delay diagnosis, resulting in a poorer prognosis. Case Report. This report describes a 64-year-old male patient with nasal obstruction and epistaxis. Computed tomography of the facial sinuses revealed a large lesion in the right nasal cavity, with infiltration into the left cavity, ethmoidal cells, and erosion of the cribriform plate...
2017: Case Reports in Oncological Medicine
Abbas Agaimy, Wilko Weichert
The term "sinonasal undifferentiated carcinoma (SNUC)" has been coined in 1986 for a highly aggressive sinonasal tract epithelial neoplasm showing distinctive morphology, but lacking any specific line of differentiation. Recent developments resulted in a dynamic splitting of new entities traditionally included in the spectrum of SNUC. Sinonasal NUT-midline carcinoma, adamantinoma-like Ewing family tumors and most recently, SMARCB1(INI1)-deficient sinonasal carcinoma are the main entities defined by specific genetic aberrations...
February 7, 2017: Head and Neck Pathology
Lester Dr Thompson
One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases. Specific tumor site of origin, imaging findings, and clinical findings must be combined with the histology and pertinent ancillary studies if the correct diagnosis is to be reached...
January 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Farzan Siddiqui, Richard V Smith, Sue S Yom, Jonathan J Beitler, Paul M Busse, Jay S Cooper, Ehab Y Hanna, Christopher U Jones, Shlomo A Koyfman, Harry Quon, John A Ridge, Nabil F Saba, Francis Worden, Min Yao, Joseph K Salama
The American College of Radiology (ACR) Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios...
March 2017: Head & Neck
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