Soft tissue tumors of the sinonasal tract.

Primary soft tissue tumors arising in the sinonasal tract are rare. While many mesenchymal neoplasms have been reported in the nasal cavity, sinuses, and nasopharynx, few are distinctive to this anatomic region. Some tumor types are relatively more common in this area, such as schwannoma and rhabdomyosarcoma. Nasopharyngeal angiofibroma and sinonasal hemangiopericytoma are unique entities of the sinonasal tract, as well as the recently characterized biphenotypic sinonasal sarcoma. This review discusses the clinical, morphologic, and immunohistochemical features and currently known molecular data of the more frequently encountered soft tissue tumors of the sinonasal tract.

Sinonasal small round blue cell tumors: An approach to diagnosis.

The differential diagnosis for small round cell tumors in the sinonasal tract is diverse and as the body of literature documenting not only uncommon presentations but also availability of ancillary studies grows, so does the need for a reminder to take a conservative and thorough approach before rendering a diagnosis. Small tissue samples are particularly problematic, with limitations that include volume of tumor cells available for studies, lack of architectural context and a non-specific gross description. Incorporation of patient history and presentation, radiologic findings, clinical impression and concurrent studies often guide the course of studies performed by the pathologist. If these are non-specific, the pathologist may need to perform ancillary studies, including a broad panel of immunohistochemical stains and molecular studies. If tissue is limited, a precise classification may not be achievable. Although the expectation to render a definitive diagnosis is high, the pathologist should never feel compelled to go further with a diagnosis than the tissue itself supports.

Hematolymphoid lesions of the sinonasal tract.

Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent T-lymphoblastic proliferations; and Rosai-Dorfman disease. Differentiating aggressive lymphomas from non-hematopoietic neoplasms such as poorly differentiated squamous cell carcinoma, olfactory neuroblastoma, or sinonasal undifferentiated carcinoma may pose diagnostic challenges. In addition, the necrosis, vascular damage, and inflammatory infiltrates that are associated with some hematolymphoid disorders can result in misdiagnosis as infectious, autoimmune, or inflammatory conditions. Here, we review hematolymphoid disorders involving the sinonasal tract including their key clinical and histopathologic features.

Human papillomavirus-associated neoplasms of the sinonasal tract and nasopharynx.

It is now well established that human papillomavirus (HPV) is an important causative factor in a subgroup of head and neck cancer. In the head and neck, while HPV is strongly associated with squamous cell carcinoma arising in the oropharynx, there is a growing interest in HPV-associated neoplasms of non-oropharyngeal origin including those which arise within sinonasal and nasopharyngeal mucosa. This article reviews current literature on the association of HPV with Scheiderian papillomas, sinonasal squamous cell carcinoma, sinonasal undifferentiated carcinoma, carcinoma with adenoid cystic-like features, and nasopharyngeal carcinoma. Several clinical implications of HPV detection in sinonasal and nasopharyngeal carcinomas are briefly discussed.

Recently described neoplasms of the sinonasal tract.

Surgical pathology of the sinonasal region (i.e., nasal cavity and the paranasal sinuses) is notoriously difficult, due in part to the remarkable diversity of neoplasms that may be encountered in this area. In addition, a number of neoplasms have been only recently described in the sinonasal tract, further compounding the difficulty for pathologists who are not yet familiar with them. This manuscript will review the clinicopathologic features of some of the recently described sinonasal tumor types: NUT midline carcinoma, HPV-related carcinoma with adenoid cystic-like features, SMARCB1 (INI-1) deficient sinonasal carcinoma, biphenotypic sinonasal sarcoma, and adamantinoma-like Ewing family tumor.

A simple classification of cranial-nasal-orbital communicating tumors that facilitate choice of surgical approaches: analysis of a series of 32 cases.

Cranial-nasal-orbital communicating tumors involving the anterior and middle skull base are among the most challenging to treat surgically, with high rates of incomplete resection and surgical complications. Currently, there is no recognized classification of tumors with regard to the choice of surgical approaches. From January 2004 to January 2014, we classified 32 cranial-nasal-orbital communicating tumors treated in our center into three types according to the tumor body location, scope of extension and direction of invasion: lateral (type I), central (type II) and extensive (type III). This classification considerably facilitated the choice of surgical routes and significantly influenced the surgical time and amount of hemorrhage during operation. In addition, we emphasized the use of transnasal endoscopy for large and extensive tumors, individualized treatment strategies drafted by a group of multidisciplinary collaborators, and careful reconstruction of the skull base defects. Our treatment strategies achieved good surgical outcomes, with a high ratio of total resection (87.5 %, 28/32, including 16 cases of benign tumors and 12 cases of malignant tumors) and a low percentage of surgical complications (18.8 %, 6/32). Original symptoms were alleviated in 29 patients. The average KPS score improved from 81.25 % preoperatively to 91.25 % at 3 months after surgery. No serious perioperative complications occurred. During the follow-up of 3 years on average, four patients with malignant tumors died, including three who had subtotal resections. The 3-year survival rate of patients with malignant tumors was 78.6 %, and the overall 3-year survival rate was 87.5 %. Our data indicate that the simple classification method has practical significance in guiding the choice of surgical approaches for cranial-nasal-orbital communicating tumors and may be extended to other types of skull base tumors.

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.

[Bone and cartilaginous tumours in the sino-paranasal region with intracranial extension. Peculiarities of surgical treatment].

The objective of the present work was to study peculiarities of diagnostics of bone and cartilaginous tumours in the sino-paranasal region with intracranial extension, to substantiate the choice of the strategy and methods for the surgical intervention for the treatment of these neoplasms. The study included 19 patients with various bone and cartilaginous neoplasms in the craniofacial region. Diagnostics was based on computed tomography allowing for 3D reconstruction of the structures of interest, magnetic resonance imaging (with amplification whenever necessary), and angiography. It is proposed to use the microsurgical and pneumatic techniques to ensure the maximally complete removal of the tumours with a minimal injury to the surrounding tissues. The extension of fascial approach is recommended for the management of intracranially spreading tumours. This technique was used for the treatment of 9 patients (7 undergoing anterior craniofacial resection, 1 lateral craniofacial resection, and 1 subcranial resection) in combination with Moure and Denker rhinotomy. The transcranial approach was employed in 8 patients one of whom underwent transoral surgery and another transnasal intervention. Also, the histological structure of the tumours needs to be taken into consideration when planning the approach and the extent of the surgical intervention. It is concluded that preliminary courses of chemo and radiotherapy do not significantly improve the outcome of surgical treatment; in contrast, they promote the development of complications during the postoperative period. The results of this study indicate that for the management of benign tumours characterized by the slow growth rate (osteoma, chondroma, chordoma) the traditional ENT approaches can be supplemented by transcranial surgery. Extensive interventions (anterior craniofacial reseaction, lateral craniofacial resection) are needed for the management of aggressive malignant tumours (ostesarcoma, chondrosarcoma) without serious injury the healthy tissues; moreover, such approach increases the survival rate of the patients.

Sinonasal inverted papilloma: 84 patients treated by endoscopy and proposal for a new classification.

AIM: To suggest a new classification system for sinonasal papilloma based on a critical analysis of surgical indications and results obtained. METHODS: We analysed surgical data from 84 cases of sinonasal papilloma treated endoscopically. RESULTS: In 58 males and 26 females, between 25 and 85 years, the ethmoid sinus (63 cases), the maxillary sinus (43), and the nasal fossa (22) were mostly involved. No case of endocranial extension or carcinoma was reported. Complications were reported in 15.4% of patients, as well as 5 recurrences (5.9%). Median follow up was 39,5 months. To categorise the tumour for the most appropriate surgical treatment, we propose a classification based on 6 main categories that depend on the location, origin and extension of the tumour. CONCLUSION: The classification that we propose presents advantages for prognosis and surgical indication in comparison with other classifications.

Human Papillomavirus-Related Multiphenotypic Sinonasal Carcinoma-An Even Broader Tumor Entity?

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a recently defined tumor subtype with apparent favorable clinical outcome despite aggressive histomorphology. However, in recent years, additional numbers of cases, with more variable features and at locations outside the sinonasal region, have complicated the definition of HMSC. Here, we have performed a systematic review of all cases described so far in order to accumulate more knowledge. We identified 127 articles published between 2013 and 2021, of which 21 presented unique cases. In total, 79 unique patient cases were identified and their clinical and micromorphological nature are herein summarized. In our opinion, better clinical follow-up data and a more detailed tumor characterization are preferably needed before HMSC can finally be justified as its own tumor entity.

[Diagnosis, prognosis and treatment of sinonasal carcinomas (excluding melanomas, sarcomas and lymphomas)]. / Diagnostic, pronostic et traitement des carcinomes nasosinusiens (hors mélanomes, sarcomes et lymphomes).

Sinonasal carcinomas account for 3% of ENT cancers. They are subdivided into squamous cell carcinomas (50%), adenocarcinomas [20%, mostly of intestinal type (ITAC)], and more rarely, adenoid cystic carcinomas, olfactory neuroblastomas (=esthesioneuroblastomas), neuroendocrine carcinomas or undifferentiated sinonasal carcinomas (SNUC). The 5-year survival rates are, in descending order, 72% for neuroblastomas, 63% for adenocarcinomas, 50-60% for large-cell neuroendocrine carcinomas, 53% for squamous cell carcinomas, 25-50% for adenoid cystic, 35% for small-cell neuroendocrine carcinomas and 35% for SNUC and newly discovered histologies. Surgery is the main treatment; endoscopic approaches reduce the morbidity with equivalent tumour control. Intensity-modulated radiation therapy (IMRT) is almost systematic. Nodal involvement is rare in ethmoidal adenocarcinomas and adenoid cystic carcinomas; it is intermediate and may justify prophylactic radiotherapy for N0 necks in SNUC, neuroblastoma, squamous cell carcinomas and sinonasal neuroendocrine carcinomas. IMRT or proton therapy is the mainstay of treatment of unresectable disease. Radiotherapy optimization by carbon ion therapy for adenoid cystic carcinomas, or by chemotherapy for all carcinomas with IMRT or proton therapy, is investigated within clinical trials in France. Neoadjuvant chemotherapy is reserved for rapidly progressive disease or histologies with a high metastatic potential such as neuroendocrine carcinomas or SNUC. Given their histologic and molecular specificities and different relapse patterns, an expertise of the REFCOR network, with REFCORpath review, is likely to correct diagnoses, rectify treatments, with an impact on survival.

Medially originated inverted papilloma.

OBJECTIVES: The objective of this study was to determine the characteristics of medially originated inverted papilloma (MOIP) and compare them with laterally originated inverted papilloma (LOIP). METHODS: A retrospective review of the charts for a total of 83 patients with sinonasal inverted papilloma (IP) was conducted. Tumors originating from the nasal septum or the turbinates were categorized as MOIP, whereas tumors originating from the four sinuses were categorized as LOIP. RESULTS: Twenty-eight (34%) and 55 (66%) cases were categorized as MOIP and LOIP. MOIP from the middle turbinate behaved more aggressively than LOIP from the ethmoid sinus (P = 0.009), but less aggressively than LOIP from the maxillary medial wall (P < 0.05). Radical procedures were implemented in 14 patients with LOIP, but not in any patients with MOIP (P = 0.002). The recurrence rates were comparable in both groups (P = 0.472). CONCLUSIONS: The categorization of IP on the basis of tumor origin enabled a better surgical design and more accurate excision of the tumor. Although in some cases MOIP may behave more aggressively, radical procedures were indicated in only the late Krouse stage LOIP without compromising the recurrence rate.

[Sinonasal adenocarcinomas reviewed. Prognostic value of WHO 2005 histological classification]. / Les adénocarcinomes nasosinusiens revisités. Intérêt pronostique de la classification histologique OMS 2005.

OBJECTIVES: The WHO 2005 histological classification separates sinonasal adenocarcinoma (ADC) into three classes: intestinal-type adenocarcinoma (ITAC), low-grade sinonasal ADC and high-grade sinonasal ADC. The goal of this study was to check the relevance of this classification on the prognosis of patients treated for ADC. PATIENTS AND METHODS: All the files of patients treated consecutively in the ENT department of the Montpellier University Hospital for ADC between 1980 and 2003 were retrospectively re-examined. Each case was reviewed for anatomical and pathological data based on the immunohistochemistry results according to the WHO 2005 classification, with a study of a panel of markers: cytokeratin 7 (CK7), cytokeratin 20 (CK20), Villin, CDX2 and EGFR. The epidemiologic data, the methods of treatment and the follow-up were studied. The survival probabilities were calculated using the Kaplan-Meier method and the survival graphs were compared using a log-rank test. RESULTS: Sixty-two files were reviewed. Twelve patients were reclassified into the adenoid cystic carcinoma category and excluded from the study. In the 50 remaining cases, there were 36 ITAC cases, four low-grade ADC cases and 10 high-grade dedifferentiated carcinomas. For all of the ADC cases, the total survival at 5 years and without recurrence was 64 and 52%, respectively. The analysis of the three subgroups showed a total survival of 72.2% for ITAC, 100% for low-grade and 20% for high-grade ADC with a significant difference (p=0.044). This immunohistochemical distinction was mainly based on the expression of CK20 found in 98% of the ITAC cases and absent in low- and high-grade ADC patients. CONCLUSION: The WHO 2005 classification for sinonasal ADC provides a valuable prognosis by showing a difference in the progression profile between ITAC, low-grade ADC and high-grade ADC. Moreover, broader studies should be conducted to investigate the different subtypes of ITAC.

Sinonasal Papillomas and Carcinomas: A Contemporary Update With Review of an Emerging Molecular Classification.

CONTEXT.­: Sinonasal papillomas and carcinomas are uncommon head and neck neoplasms that comprise a broad clinicopathologic and morphologic spectrum, and thus frequently represent a diagnostic challenge for surgical pathologists. Recent molecular interrogation of these tumors has delineated a number of recurrent alterations that correspond to distinct entities with potential diagnostic and/or therapeutic clinical utility. OBJECTIVE.­: To summarize the salient clinicopathologic, morphologic, and molecular features of sinonasal papillomas and carcinomas. DATA SOURCES.­: Review of pertinent literature regarding sinonasal papillomas and sinonasal carcinomas. CONCLUSIONS.­: Despite their relative rarity in many surgical pathology practices, sinonasal papillomas and carcinomas frequently demonstrate characteristic morphologic features that are important for accurate diagnosis. Given our emerging understanding of the molecular basis for these tumors, judicious use of available ancillary tools-including immunohistochemistry and in situ hybridization-may be helpful in subsets of cases, whereas additional molecular testing may be useful for diagnostically challenging and/or clinically aggressive sinonasal tumors.

Sinonasal undifferentiated carcinoma: clinicopathological spectrums and diagnosis reappraisal.

Sinonasal undifferentiated carcinoma (SNUC) is defined as undifferentiated carcinoma of the sinonasal tract without glandular or squamous features and not otherwise classifiable. SNUC is a rare tumor, with a long list of differential diagnoses, and often poses a considerable diagnostic challenge. In addition, recent advances in molecular and immunohistochemistry techniques have recognized several new entities that were previously included in the SNUC category. These include SMARCB1 (INI-1)-deficient carcinoma, NUT (nuclear protein in testis) carcinoma, adamantinoma-like Ewing sarcoma, and the most recently described and rarer SMARCA4 (BRG)-deficient carcinoma. In this study, we retrospectively reviewed 11 cases with an original diagnosis of SNUC. We found that a significant portion of those cases can be reclassified into specific entities, with potential impact on therapy and prognosis because of misclassification in 2 of these cases.

High-Grade Sinonasal Carcinoma: Classification Through Molecular Profiling.

High-grade sinonasal carcinomas are a cohort of malignant epithelial neoplasms arising in the sinonasal cavities with distinct, ominous morphologic features or lacking well-differentiated features that might otherwise classify them as less biologically worrisome. Recent advances in molecular profiling have led to the identification of several distinct tumor entities previously grouped together. These molecularly distinct lesions include NUT (midline) carcinoma, INI1 (SMARCB1)-deficient carcinoma, SMARCA4-deficient sinonasal carcinoma, and novel IDH-mutant sinonasal undifferentiated carcinoma, in addition to the previously described lymphoepithelial carcinoma that may also be included in the differential diagnosis. The discovery of these distinct molecular tumor profiles may have significant clinical impact as targeted molecular-based therapeutics continue to evolve, and they may offer some respite for patients who have these highly aggressive cancers.

Primary cutaneous B cell lymphoma--leg type (NEW EORTC--WHO classification), with nasal sinuses involvement.

Primary Cutaneous lymphomas of B cell origin are rare, there remains a controversy in truly classifying these lymphomas and an updated EORTC classification divides them on the basis of their distinct histopthological grounds rather than on the basis of their anatomic location as in WHO classification, while the new WHO- EORTC joint classification maintains some characteristics of both systems, We report an elderly gentleman who primarily had a typical Leg dominant Cutaneous lymphoma of B cell origin uniquely with involvement of nasal Sinusues, bearing the Immunohistochemical staining features of "Cutaneous lymphoma - Leg Type" befitting the new joint WHO-EORTC classification of Cutaneous B cell Lymphoma.

[WHO classification of head and neck tumours 2017: Main novelties and update of diagnostic methods]. / Classification de l'OMS 2017 des tumeurs de la tête et du cou : principales nouveautés et mise à jour des méthodes diagnostiques.

The publication of the new WHO classification of head and neck tumours in 2017 brought major modifications. Especially, a new chapter is dedicated to the oropharynx, focusing on the description of squamous cell carcinoma induced by the virus Human Papilloma Virus (HPV), and new entities of tumors are described in nasal cavities and sinuses. In this article are presented the novelties and main changes of this new classification, as well as the updates of the diagnostic methods (immunohistochemistry, cytogenetics or molecular biology).

Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumors of the Nasal Cavity, Paranasal Sinuses and Skull Base.

The sinonasal tract remains an epicenter of a diverse array of neoplasia. This paper discusses changes to the WHO classification system of tumors involving this area. In particular, seromucinous hamartoma, NUT carcinoma, biphenotypic sinonasal sarcoma, HPV-related carcinoma with adenoid cystic features, SMARCB1-deficient carcinoma, and renal cell-like adenocarcinoma are discussed.