RESUMEN
BACKGROUND: .-Sinonasal malignancies are rare, aggressive, deadly and challenging tumors to diagnose and treat. Since 2000, age-adjusted incidence rates average less than 1 case per 100,000 per year, male and female combined, in the United States. For the entire cohort, 2000-2017, overall median age-onset was 62.6 years. Carcinoma constitutes over 90% of these upper respiratory cancers and most cases are advanced, more than 72% (regional or distant stage) when the diagnosis is made. Composite mortality at 5 years was 108 excess deaths/1000/year with a mortality ratio of 558%, and 41% of deaths occurred in this time frame. As a consequence, observed median survival was approximately 6 years with 5-year cumulative observed survival (P) and relative survival rates (SR) 53% and 60%. This mortality and survival update study follows the World Health Organization International Classification of Diseases for Oncology-3rd Edition (ICD-O-3)1 topographical identification, coding, labeling and listing of 13,404 patient-cases accessible for analysis in the United States National Cancer Institute's Surveillance, Epidemiology and End Results program (NCI SEER Research Data, 18 Registries), 2000-2017 located in 8 primary anatomical sites: C30.0-Nasal cavity, C30.1-Middle ear, C31.0-Maxillary sinus, C31.1-Ethmoid sinus, C31.2-Frontal sinus, C31.3-Sphenoid sinus, C31.8-Overlapping lesion of accessory sinuses, C31.9-Accessory sinus, NOS. OBJECTIVES: .-1) Utilize national population-based SEER registry data for 2000-2017 to update cancer survival and mortality outcomes for 8 ICD-O-3 topographically coded sinonasal primary sites. 2) Discern similarities and contrasts in NCI-SEER case characteristics. 3) Identify current risk pattern outcomes and shifts in United States citizens, 2000-2017. METHODS: .-SEER Research Data, 18 Registries, Nov 2019 Sub (2000-2017)2,3 are used to examine the risk consequences of 13,404 patients diagnosed with sinonasal malignancies, 2000-2017, in this retrospective population-based study employing prognostic data stratified by topography, age, sex, race, stage, grade, 2 cohort entry time-periods (2000-06 & 2007-17), and disease-duration to 15 years. General methods and standard double decrement life table methodologies for displaying and converting SEER site-specific annual survival and mortality data to aggregate average annual data units in durational intervals of 0-1, 0-2, 1-2, 2-5, 0-5, 5-10, and 10-15 years are employed. The reader is referred to the "Registrar Staging Assistant (SEER*RSA)" for local-regional-distant Extent of Disease (EOD) sources used in the development of staging descriptions for the Nasal Cavity and Paranasal Sinuses (maxillary and ethmoid sinuses only) and Summary Stage 2018 Coding Manual v2.0 released September 1, 2020. Cancer staging & grading procedural explanations, statistical significance & 95% confidence levels4 are described in previous Journal of Insurance Medicine articles5,6 and other publications.7,8 Poisson confidence intervals at the 95% level based on the number of observed deaths are used in this study but not displayed here to conserve space on the mortality tables. Excluded were all death certificate only and those alive with no survival time. RESULTS: .-In the SEER 18 registries, a total of 13,404 patient cases (2000-2017) were available for analysis with an incidence of less than one patient per 100,000 people. From this group, analysis for survival and mortality totaled 10,624 patients. Males comprised 59.3% of cases and females 40.7%. Whites represented 80.3% of cases and black, others & unknown patients comprised 19.7%. The most common anatomic site of malignancy was the nasal cavity (49.7%); least common was the frontal sinus (1.2%). From diagnosis, across the span of 8 primary sites, first-year mortality rates q ranged from 14.3% (C30.0-nasal cavity) to 30.2% (C31.8-overlapping sinus) with corresponding excess death rates (EDR) of 118/1000/year and 279/1000/year. For single sites, the 5-year cumulative survival ratio (SR) was highest for the nasal cavity (69.5%) and lowest for overlapping lesions of the accessory sinuses (47.2%) with EDRs of 76 and 169 per 1000 per year respectively Overall, 5-year relative survival (SR) for all sinonasal tract malignancies combined was 60.3%, excess mortality (EDR) 108 per 1000 per year and mortality ratio 558%. CONCLUSIONS: .-The 8 sinonasal cancer primary sites are characterized by a low percentage of cases in the localized stage (28%). Since excess mortality is high even in the localized stage, overall prognosis is very poor for all patients. Excess mortality persists in cancer of the sinonasal tract as long as 10-15 years after diagnosis and treatment. EDR in the 15-year durational-interval, all sinonasal sites combined remained significant at 27.6 per 1000 per year with continuing decrease in cumulative survival ratio (SR) to 43.9%.
Asunto(s)
Neoplasias Nasales , Programa de VERF , Humanos , Estados Unidos/epidemiología , Masculino , Femenino , Persona de Mediana Edad , Anciano , Neoplasias Nasales/mortalidad , Neoplasias Nasales/diagnóstico , Neoplasias Nasales/patología , Cavidad Nasal/patología , Estadificación de Neoplasias , Oído Medio/patología , Adulto , Neoplasias de los Senos Paranasales/mortalidad , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/epidemiología , Tasa de Supervivencia , Neoplasias del Oído/mortalidad , Neoplasias del Oído/patología , Neoplasias del Oído/diagnóstico , Clasificación del Tumor , Anciano de 80 o más Años , Factores Sexuales , Análisis de Supervivencia , Factores de EdadRESUMEN
Hemangiomas of the nasal cavity are extremely rare in the practice of an otorhinolaryngologist and can be presented in various histopathological variants. Scientific data on hemangiomas of the sinonasal region are analyzed and systematized. The article describes the principles of diagnosis and choice of the method of surgical treatment of hemangiomas. An analysis of the literature data shows that with hemangiomas of the nasal cavity, a comprehensive examination of the patient is required, including collection of complaints and anamnesis, endoscopy of the nasal cavity and computed tomography of the paranasal sinuses, and with significant hemangiomas spreading to neighboring anatomical areas, magnetic resonance imaging with intravenous contrast.
Asunto(s)
Hemangioma , Humanos , Hemangioma/diagnóstico , Hemangioma/terapia , Hemangioma/cirugía , Endoscopía/métodos , Neoplasias Nasales/diagnóstico , Neoplasias Nasales/terapia , Neoplasias Nasales/cirugía , Cavidad Nasal/cirugía , Cavidad Nasal/patología , Cavidad Nasal/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Imagen por Resonancia Magnética/métodos , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/cirugía , Neoplasias de los Senos Paranasales/terapiaRESUMEN
Sinonasal tumours of varying histology are not unusual in otolaryngology surgical practice. Juvenile angiofibroma (JAs) are vascular tumours usually occurring in adolescent male population; but rare in females. But similar clinical and radiological presentations are possible in females inducing strong suspicion of JA which needs to be ruled out by detailed evaluation. Here we present a case of a young female in her 20s who presented with a bleeding nasal mass which was finally diagnosed as sinonasal glomangiopericytoma which is a very rare sinonasal tumour. Tumours resembling JA do present in the female population but rarely turn out to be JA. A strong index of suspicion along with a handful of special blood investigations to rule out androgen insensitivity syndrome is mandatory.
Asunto(s)
Angiofibroma , Hemangiopericitoma , Humanos , Angiofibroma/diagnóstico , Angiofibroma/patología , Femenino , Diagnóstico Diferencial , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/patología , Hemangiopericitoma/diagnóstico por imagen , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/diagnóstico por imagen , AdultoRESUMEN
ALK-positive anaplastic large cell lymphoma is a rare T-cell lymphoma with ALK gene rearrangement that develops in children and young adults. The disease almost always affects the lymph nodes, and extranodal areas are also frequently involved. This article describes two cases of atypical localization of ALK-positive anaplastic large cell lymphoma with involvement of the paranasal sinuses.
Asunto(s)
Quinasa de Linfoma Anaplásico , Linfoma Anaplásico de Células Grandes , Humanos , Linfoma Anaplásico de Células Grandes/genética , Linfoma Anaplásico de Células Grandes/patología , Linfoma Anaplásico de Células Grandes/diagnóstico , Quinasa de Linfoma Anaplásico/genética , Masculino , Femenino , Adulto , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/genética , Neoplasias de los Senos Paranasales/metabolismo , Neoplasias de los Senos Paranasales/diagnóstico , Reordenamiento GénicoRESUMEN
BACKGROUND: We report a nasal cavity unusual perivascular epithelioid cell tumor (PEComa) mimicking mucosal melanoma. METHODS: Immunohistochemistry was performed using BenchMark Ultra and panel of antibodies. The Ion Torrent platform and Ion AmpliSeq cancer hotspot panel were utilized for DNA genotyping. Target-specific RNA libraries for the detection of fusion transcripts were constructed using Archer Universal RNA Reagent Kit v2 and Archer FusionPlex Solid Tumor panel and sequenced on the MiSeqDx instrument. RESULTS: The tumor, diagnosed in 46-year-old female, was composed of spindle cells, and lacked pigmentation. Immunohistochemically, it showed a patchy HMB-45 positivity. Other melanocytic markers (S100 protein, Melan-A, SOX10) were negative. The tumor cells were weakly positive for KIT (CD117) while negative for smooth muscle actin, pancytokeratin cocktail (AE1/AE3), and synaptophysin. Diagnosis of primary sinonasal tract mucosal melanoma was favored. Additional molecular studies detected PRCC :: TFE3 fusion as the sole genetic change, and suggested the diagnosis of unusual PEComa. Previously, TFE3 fusions were reported in a subset of PEComas but not in melanomas, while PRCC involvement has only been documented once in an ocular PEComa. Immunohistochemistry revealed strong nuclear TFE3 expression concordant with the molecular findings. CONCLUSIONS: This report emphasis the importance of molecular testing in the differential diagnosis between PEComa and melanoma, especially when the tumor arises in a site typical of melanoma but showing an unusual morphology and immunophenotype. The detection of TFE3 fusion transcripts suggested the diagnosis of SNT PEComa, although it cannot be excluded that this and similar tumors represent a distinct diagnostic category.
Asunto(s)
Factores de Transcripción Básicos con Cremalleras de Leucinas y Motivos Hélice-Asa-Hélice , Melanoma , Neoplasias de Células Epitelioides Perivasculares , Humanos , Femenino , Neoplasias de Células Epitelioides Perivasculares/diagnóstico , Neoplasias de Células Epitelioides Perivasculares/patología , Neoplasias de Células Epitelioides Perivasculares/metabolismo , Neoplasias de Células Epitelioides Perivasculares/genética , Melanoma/diagnóstico , Melanoma/metabolismo , Melanoma/patología , Melanoma/genética , Persona de Mediana Edad , Factores de Transcripción Básicos con Cremalleras de Leucinas y Motivos Hélice-Asa-Hélice/metabolismo , Factores de Transcripción Básicos con Cremalleras de Leucinas y Motivos Hélice-Asa-Hélice/genética , Diagnóstico Diferencial , Inmunohistoquímica , Biomarcadores de Tumor/metabolismo , Biomarcadores de Tumor/genética , Proteínas de Fusión Oncogénica/genética , Proteínas de Fusión Oncogénica/metabolismo , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/genética , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/metabolismo , Neoplasias Nasales/diagnóstico , Neoplasias Nasales/patología , Neoplasias Nasales/genética , Neoplasias Nasales/metabolismoAsunto(s)
Neutrófilos , Rabdomiosarcoma , Humanos , Rabdomiosarcoma/diagnóstico , Rabdomiosarcoma/patología , Niño , Pronóstico , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/sangre , Linfocitos/patología , Factores de Edad , Plaquetas , Recuento de Plaquetas , Recuento de LinfocitosAsunto(s)
Neoplasias de los Senos Paranasales , Rabdomiosarcoma , Humanos , Niño , Rabdomiosarcoma/diagnóstico , Rabdomiosarcoma/patología , Pronóstico , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/sangre , Neutrófilos , Linfocitos , Recuento de Plaquetas , Factores de Edad , Recuento de LinfocitosAsunto(s)
Imagen Multimodal , Papiloma Invertido , Neoplasias de los Senos Paranasales , Humanos , Persona de Mediana Edad , Imagen por Resonancia Magnética , Papiloma Invertido/diagnóstico por imagen , Papiloma Invertido/patología , Papiloma Invertido/diagnóstico , Neoplasias de los Senos Paranasales/diagnóstico por imagen , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/diagnóstico , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Nonintestinal adenocarcinoma of the nasal cavity and paranasal sinuses (nonITAC) is a heterogeneous tumour that has rarely been reported in previous studies. We compared and analysed the symptoms, radiographic and pathological features, treatment methods, and prognosis of patients with low-grade (G1) and high-grade (G3) tumours. METHODS: This was a retrospective study included 22 patients with pathologically confirmed non-ITAC of the nasal cavity and paranasal sinuses who were treated between January 2008 and December 2021 at a single centre. Of these, 11 patients had G1 tumours, and 11 patients had G3 tumours. Clinicopathological features, treatment methods, and survival outcomes were analysed. RESULTS: The median follow-up period was 48.5 months. Nasal congestion was the most common initial symptom, and the nasal cavity was the most frequently involved site. For G1 tumours, the main treatment was simple surgery, 1 and 3year overall survival (OS) rates were 100 and 88.9%, while the 1 and 3year local control (LC) rates were 100 and 100%, respectively. For G3 tumours, the main treatments were surgery combined with radiotherapy and/or chemotherapy,1 and 3year OS rates were 72.7 and 72.7%, while the 1 and 3year LC rates were 100 and 90.91%, respectively. G3 tumours was associated with significantly shorter overall survival than G1 tumours (P = 0.035). Patients with stage III-IV showed shorter overall survival compared to stage I-II patients (P = 0.035). CONCLUSIONS: Non-ITAC of the nasal cavity and paranasal sinuses may frequently occur in the nasal cavity. The main treatment modality is surgery, supplemented by radiotherapy and chemotherapy. Pathological grade and tumour stage were poor prognostic factors for the disease.
Asunto(s)
Adenocarcinoma , Cavidad Nasal , Neoplasias Nasales , Neoplasias de los Senos Paranasales , Humanos , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Neoplasias de los Senos Paranasales/terapia , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/mortalidad , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias Nasales/terapia , Neoplasias Nasales/patología , Neoplasias Nasales/mortalidad , Neoplasias Nasales/diagnóstico , Anciano , Cavidad Nasal/patología , Pronóstico , Adenocarcinoma/terapia , Adenocarcinoma/patología , Adenocarcinoma/mortalidad , Adenocarcinoma/diagnóstico , Adulto , Tasa de Supervivencia , Clasificación del Tumor , Estadificación de NeoplasiasRESUMEN
OBJECTIVES: To present a case report of sinonasal glomangiopericytoma (GPC) in a female patient in her thirties and to highlight the importance of collecting pathology specimens even in routine sinus surgery cases. METHODS: A case report detailing the diagnosis of GPC in a female in her thirties, including her initial presentation, treatment, and follow-up, along with a brief review of the literature. RESULTS: Pathology of the collected specimen revealed sinonasal GPC along with chronic rhinosinusitis. Immunohistochemistry was positive for SMA, beta-catenin, and cyclin D1; and negative for STAT6, ERG, pankeratin, SOX10, and S100. CONCLUSION: This diagnosis expands the knowledge around the demographic profile of GPC patients. GPC should be included in the differential diagnosis of sinonasal masses, even in younger patients. The case highlights the importance of collecting the entire pathology specimen in all cases, even of ones that seem routine and benign.
Asunto(s)
Hemangiopericitoma , Humanos , Femenino , Hemangiopericitoma/patología , Hemangiopericitoma/diagnóstico , Hemangiopericitoma/cirugía , Adulto , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/cirugía , Neoplasias de los Senos Paranasales/diagnóstico , InmunohistoquímicaAsunto(s)
ADN Tumoral Circulante , Neoplasias Nasofaríngeas , Infecciones por Papillomavirus , Humanos , Neoplasias Nasofaríngeas/virología , Neoplasias Nasofaríngeas/sangre , Neoplasias Nasofaríngeas/diagnóstico , Infecciones por Papillomavirus/diagnóstico , Infecciones por Papillomavirus/virología , Infecciones por Papillomavirus/sangre , ADN Tumoral Circulante/sangre , ADN Tumoral Circulante/genética , Neoplasias de los Senos Paranasales/virología , Neoplasias de los Senos Paranasales/sangre , Neoplasias de los Senos Paranasales/diagnóstico , Papillomaviridae/genética , Papillomaviridae/aislamiento & purificaciónRESUMEN
PURPOSE: This study aimed to evaluate the diagnostic value of image-enhanced endoscopy (IEE) in detecting sinonasal inverted papilloma (SNIP). METHODS: Overall, 86 patients with unilateral nasal papillary or lobulated neoplasms were included between July 2018 and June 2019. All patients underwent IEE examinations, and the diagnosis of all neoplasms was confirmed through postoperative pathology. Logistic regression analysis was conducted to screen for independent predictors of various types of vascular patterns of SNIP. Furthermore, a prognostic nomogram was constructed using the independent predictors screened by logistic regression analysis to evaluate its usefulness in distinguishing SNIP from nasal polyp (NP) and papillary mucosa folds (PMF). RESULTS: In total, 86 consecutive cases were observed, including 37 with SNIP, 40 with NP, and 9 with PMF. Logistic regression analysis showed that spot, corkscrew, and multilayered vascular patterns were independent predictors of SNIP diagnosis. Furthermore, a nomogram comprising the three independent risk factors was constructed with scores of 5, 2, and 3. The area under the receiver operating characteristic curve for predicting SNIP was 0.954, 0.66, 0.71, and 0.76 for the nomogram model, spot vascular pattern, corkscrew vascular pattern, and multilayered vascular pattern, respectively. CONCLUSION: The nomogram model based on spot, corkscrew, and multilayered vascular patterns in SNIP observed using IEE can be a useful diagnostic tool for predicting and distinguishing between NP and PMF.
Asunto(s)
Endoscopía , Papiloma Invertido , Neoplasias de los Senos Paranasales , Humanos , Papiloma Invertido/diagnóstico , Papiloma Invertido/diagnóstico por imagen , Papiloma Invertido/patología , Masculino , Femenino , Persona de Mediana Edad , Endoscopía/métodos , Adulto , Anciano , Neoplasias de los Senos Paranasales/diagnóstico por imagen , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Nomogramas , Aumento de la Imagen/métodos , Estudios Retrospectivos , Diagnóstico Diferencial , Neoplasias Nasales/diagnóstico , Neoplasias Nasales/diagnóstico por imagen , Neoplasias Nasales/patologíaRESUMEN
Background: Human papillomavirus (HPV)-related multi phenotypic sinonasal carcinoma (HMSC) is a recently described tumor subtype with an unknown prognosis, often misdiagnosed with other sinonasal carcinomas, and associated with high-risk HPV (HR-HPV). The present study aimed to evaluate the expression of vascular endothelial growth factor (VEGF), Bcl-2-associated X protein (BAX), epidermal growth factor receptors (EGFR), ProExTMC, and human telomerase reverse transcriptase (hTERT) and assess their association with survival and clinicopathological characteristics. Methods: Between 2017 and 2022, 40 HMSC patients underwent surgical resection at the School of Medicine, Zagazig University Hospitals (Zagazig, Egypt). Tissue samples were examined for the presence of HR-HPV; absence of myeloblastosis (MYB), MYB proto-oncogene like 1 (MYBL1), and nuclear factor I/B (NFIB) fusions and the presence of myoepithelial proteins (calponin, S100, SMA), squamous differentiation markers (p63, p40, calponin), VEGF, BAX, ProExTMC, and hTERT by immunohistochemistry. All patients were followed up for about 54 months until death or the last known survival data. Data were analyzed using the Chi square test and Kaplan-Meier method. Results: The expression of VEGF, hTERT, and ProExTMC was significantly associated with age, advanced tumor stages, lymph node metastasis, tumor size, mortality, relapse, poor disease-free survival (DFS), and overall survival (OS) (P<0.001). BAX expression was significantly associated with tumor size, age, poor DFS, and relapse (P=0.01, P<0.001, P=0.035, and P=0.002, respectively). Conclusion: HMSC is strongly associated with HR-HPV. The expression of VEGF, EGFR, BAX, hTERT, and ProExTMC is associated with aggressive malignant behavior, poor survival, and poor prognosis, making them novel prognostic biomarkers for targeted therapeutics in HMSC.
Asunto(s)
Carcinoma , Infecciones por Papillomavirus , Neoplasias de los Senos Paranasales , Humanos , Factor A de Crecimiento Endotelial Vascular , Proteína X Asociada a bcl-2 , Virus del Papiloma Humano , Pronóstico , Infecciones por Papillomavirus/complicaciones , Infecciones por Papillomavirus/diagnóstico , Papillomaviridae , Recurrencia Local de Neoplasia/complicaciones , Carcinoma/diagnóstico , Carcinoma/patología , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Receptores ErbB , Recurrencia , BiomarcadoresRESUMEN
OBJECTIVES: Fungal tissue invasion in the setting of sinonasal malignancy has been rarely described in the literature. Only a handful of studies have discussed cases of suspected chronic and acute IFS (CIFS and AIFS, respectively), having an underlying undifferentiated sinonasal carcinoma, sinonasal teratocarcinosarcoma, and NK/T-cell lymphoma. METHODS: Here, we describe 3 cases of carcinoma mimicking IFS from a single institution. RESULTS: Each of our patients presented with sinonasal complaints as an outpatient in the setting of immunosuppression. Intranasal biopsies consistently were predominated by necrotic debris, with and without fungal elements, ultimately leading to a delay of oncologic care. The final pathologies included NK/T-cell lymphoma and SNEC. All patients were followed by radiation and chemotherapy, with 1 case of mortality. CONCLUSIONS: We aim to emphasize the importance of obtaining viable tissue as pathology specimens as the presence of necrosis with fungal elements may limit the diagnosis and ultimately delay the care of an underlying sinonasal carcinoma.
Asunto(s)
Neoplasias de los Senos Paranasales , Sinusitis , Humanos , Diagnóstico Diferencial , Sinusitis/diagnóstico , Sinusitis/microbiología , Masculino , Persona de Mediana Edad , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/patología , Femenino , Anciano , Infecciones Fúngicas Invasoras/diagnóstico , Carcinoma/diagnóstico , Carcinoma/patología , Biopsia , Tomografía Computarizada por Rayos X , Neoplasias del Seno MaxilarRESUMEN
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.
Asunto(s)
Biomarcadores de Tumor , Neoplasias de los Senos Paranasales , Humanos , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/genética , Neoplasias de los Senos Paranasales/clasificación , Neoplasias de los Senos Paranasales/diagnóstico , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/análisis , Organización Mundial de la SaludRESUMEN
A case is presented of a 43-year-old male with a chronic history of progressive nasal obstruction and epiphora. MRI confirmed a heterogeneous mass involving the middle and superior turbinates with T2 hyperintense and calcified components, with extension into the inferomedial orbit. Tissue biopsy revealed a grade 2 chondrosarcoma of the conventional subtype. Endonasal wide local resection of the lesion was performed with clear margins. The patient had no functional sequelae and will undergo routine surveillance.
Asunto(s)
Condrosarcoma , Imagen por Resonancia Magnética , Neoplasias Orbitales , Neoplasias de los Senos Paranasales , Humanos , Masculino , Condrosarcoma/diagnóstico , Condrosarcoma/cirugía , Condrosarcoma/patología , Adulto , Neoplasias Orbitales/diagnóstico , Neoplasias Orbitales/cirugía , Neoplasias Orbitales/patología , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/diagnóstico , Neoplasias de los Senos Paranasales/cirugía , Invasividad Neoplásica , Biopsia , Tomografía Computarizada por Rayos X , Órbita/patología , Órbita/diagnóstico por imagenRESUMEN
ABSTRACT: Sinonasal teratocarcinosarcoma (SNTCS) is an extremely rare and aggressive malignant tumor arising in the sinonasal tract, having a combined clinicopathological feature of teratoma and carcinosarcoma. It shows a male predominance and affects adults with an age range of 18-79 years and a mean age of 60 years. Here, we report a case of SNTCS in a 14-year-old male patient who presented with swelling over the upper right alveolus and pain in the right jaw for 2 months. The tumor was completely removed by right total maxillectomy with orbital mess reconstruction, and postoperative radiotherapy with chemotherapy was given. The follow-up of the patient for 2 years has shown evidence of recurrence and is now on palliative care.
Asunto(s)
Carcinosarcoma , Teratoma , Humanos , Masculino , Adolescente , Carcinosarcoma/patología , Carcinosarcoma/diagnóstico , Teratoma/patología , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/diagnóstico , Histocitoquímica , Neoplasias Nasales/patología , Neoplasias Nasales/diagnóstico , Microscopía , Resultado del Tratamiento , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Poorly differentiated sinonasal small round cell tumors (SRCTs) are rare and heterogeneous, posing challenges in diagnosis and treatment. METHODS: Recent advances in molecular findings and diagnostic refinement have promoted better understanding and management of these tumors. RESULTS: The newly defined and emerging sinonasal entities demonstrate diverse morphologies, specific genomic signatures, and clinical behavior from conventional counterparts. In this review of SRCTs, emphasis is placed on the diagnostic approach with the employment of a pertinent panel of immunohistochemistry studies and/or molecular tests, fine-tuned to the latest WHO 5 classification of sinonasal/paranasal tumors and personalized treatment. CONCLUSION: Specifically, this review focuses on tumors with epithelial and neuroectodermal derivation.
Asunto(s)
Neoplasias de los Senos Paranasales , Sarcoma , Humanos , Neoplasias de los Senos Paranasales/diagnósticoRESUMEN
Sinonasal carcinomas represent a rare and diverse group of tumors, presenting diagnostic complexities due to their varied histological and molecular features. To ensure accurate differentiation among these malignancies, a systematic and stepwise approach is paramount. Even with the morphological similarities between poorly differentiated (non) keratinizing sinonasal squamous cell carcinoma (SNSCC) and DEK::AFF2 SNSCC, the two lesions are distinguishable using the surrogate immunohistochemical marker AFF2 or molecular testing for DEK::AFF2 mutation. We report a rare case of SMARCB1-retained DEK::AFF2 papillary non-keratinizing SNSCC in a 53-year-old female, who presented with a polypoid mass corresponding to the left middle turbinate. Following the surgical resection of the tumor and locoregional lymph nodes, adjuvant radiotherapy was administered to eradicate any residual cancer cells that may have remained after surgery.