RESUMO
Esthesioneuroblastoma is a tumor arising from olfactory neuroepithelium with an incidence of four per million. This case presents a rare nasal cavity neoplasm with hemorrhagic properties that may lead to significant sequelae. We present a 69-year-old male patient who presented with worsening altered mental status over the past 6-7 months. His Glasgow coma scale was a nine, showing a large mass centered at the cribriform plate with extension intracranially, sinonasal cavity, and bilateral orbits on diagnostics. Bifrontal craniotomy was performed one day after admission with resection of the tumor in the cranial fossa and intranasally, and decompression of the brainstem, frontal lobes, and third ventricle. After surgery, the patient was managed in the intensive care unit but continued to deteriorate. He was confirmed to have no meaningful brain activity and eventually deceased seven days after admission. Depending on the tumor stage, esthesioneuroblastoma can have a favorable prognosis with proper therapies. Different surgical techniques for ENB lead to the question of which therapeutic modality is the best because of the tumor's gross hemorrhagic properties. With adjunctive radiation therapy and/or chemotherapy, more research can bring light to proper surgical techniques.
RESUMO
To date, there is no definite effective target therapy or cure for nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome). Basal cell carcinoma is frequently the far most increased risk of this syndrome, including predisposition to other malignancies. In 2015, an 11-year-old female with a past medical history of sickle cell trait, oral, and unilateral knee abscesses presented with multiple visits for various nodules covering the hands and chest, as well as posterior knee cysts. Genetic testing confirmed the diagnosis. The key to treatment and surveillance relies on appropriate recognition, management of atypical presentations, and offering appropriate genetic counseling to families.
