SMARCB1 deficient sinonasal carcinoma: An emerging entity with a diagnostic challenge

SMARCB1 deficient sinonasal carcinomas are rare neoplasms, classified under sinonasal undifferentiated carcinomas by the fourth edition of the World Health Organization (WHO) classification of head and neck tumors. It is characterized immunohistochemically by loss of SMARCB1(INI1) expression. We are reporting the case of a 63-year-old man who was evaluated for nasal stuffiness of 3 months duration in another hospital where a radiological evaluation showed a polypoidal soft tissue lesion in the right maxillary sinus extending to the right nasal cavity and spheno-ethmoidal sinus. He underwent excision biopsy which was reported as non- keratinizing nasopharyngeal carcinoma. He was referred to our center with residual disease in spheno-ethmoidal recess for which radiotherapy was given. After completion of radiotherapy, the primary site had no residual disease, but while on follow-up he developed left sided neck nodes within 4 months of completion of treatment. Excision of the lesion was done and histopathological and immunohistochemical analysis revealed it to be metastasis from SMARCB1 deficient sinonasal carcinoma and not nasopharyngeal carcinoma as diagnosed from the other center. This case is being reported to highlight the diagnostic challenge associated with this rare entity.

Nonkeratinizing squamous cell carcinoma: A rare case report with oral involvement

Transitional cell carcinoma also known as nonkeratinizing carcinoma (NKCa) of sinonasal tract comprises 15%–20% of malignant sinonasal carcinoma. We are reporting the case of 48-year-old male with a history of tooth extraction. A computed tomography was done which showed opacity in the right nasal cavity. Incisional biopsy was taken which revealed NKCa (transitional type). Very few reported cases of this type of malignancy were found. A possible reason could be multiple synonyms such as cylindrical cell carcinoma, Schneiderian carcinoma, and transitional cell carcinoma

Large Cell Poorly Differentiated Neuroendocrine Carcinoma of the Maxilla: An extremely Rare Tumor.

Neuroendocrine carcinomas are epithelial neoplasm with predominant neuroendocrine differentiation. Mostly they are found in gastrointestinal and respiratory system. In the nasal and paranasal sinus regions, squamous cell carcinoma is the most common tumor, followed by adenocarcinoma, malignant lymphoma, sinonasal undifferentiated carcinoma, and olfactory neuroblastoma. Large cell neuroendocrine carcinoma of head and neck region is extremely rare. Until now, very few cases of neuroendocrine tumors of paranasal sinuses have been documented. Compared to other organ systems, the neuroendocrine carcinoma involving sinuses are much more aggressive and with poor prognosis. The rarity of this carcinoma has restricted the understanding of its etiology and clinical outcome. We herein reporting a case of large cell poorly differentiated neuroendocrine tumor of maxilla with orbital extension with the purpose of analysis of the available information of this rare malignancy.

Cáncer de senos paranasales y sus manifestaciones orales: reporte de dos casos clínicos / Paranasal sinus cancer and oral manifestations: report of two cases

Los carcinomas de senos paranasales son entidades poco frecuentes y cuando se diagnostican, generalmente ya se encuentran en estados avanzados de la enfermedad, dado que el proceso neoplásico en las etapas iniciales crece invadiendo las cavidades paranasales, para luego en etapas avanzadas producir la deformación de las corticales. Dentro de las manifestaciones clínicas se pueden presentar epistaxis, obstrucción nasal, dolor en zona maxilar, dolor o movilidad dentaria. Se presentan dos casos clínicos que dentro de las primeras manifestaciones cursaron con dolor de la zona maxilar y movilidad anormal de un diente. Al momento de realizar el diagnóstico definitivo los procesos tumorales se encontraban muy avanzados, comprometiendo diversas estructuras como seno maxilar, etmoidal, esfenoidal, y piso de órbita, lo que obliga a realizar grandes resecciones de la zona maxilofacial. El rol del odontólogo en el diagnóstico de la enfermedad podría ser de gran importancia, al sospechar que un aumento de volumen del reborde, la movilidad dentaria anormal o un dolor de la zona maxilar no siempre corresponden a causas odontogénicas.

Paranasal sinus cancer are rare entities, but when diagnosed, often already in advanced stages of the disease, because the neoplastic process in the initial stages grows invading the paranasal sinuses and then in advanced stages, produces deformation of cortical bone. Among the clinical manifestations epistaxis, nasal obstruction, pain in maxillary area, pain or tooth mobility may occur. We present two clinical cases in which within the first manifestations are the maxillary pain and abnormal tooth mobility. When making the final diagnosis, tumor processes were well advanced, engaging various structures as maxillary sinus, ethmoid, sphenoid, and orbital floor, forcing large resections perform maxillofacial area. The role of the dentist in the diagnosis of the disease could be of great importance, suspecting that an increase maxillary volume, abnormal tooth mobility or pain of the maxillary not always correspond with odontogenic causes.