Glomangiopericytoma, three cases in nine days; case reports and Brief review

Glomangiopericytoma is a rare sinonasal tumor. We report three casesof this rare disease that presented in nine days to one subspecialt ypractice. Histochemical and DNA sequencing confirmed the diagnosisineachcase.Endoscopicsinonasalsurgeryprovidedcompleteresectionin each case. The occurrence of three cases to one practice withina 2-week timeframe may suggest GPC is under-recognized. Routinesequencing to detect CTNNB1 mutations and staining for smooth muscleactin of suspicious sinonasal tumorsmayincrease the frequencyof GPC detection

Proton re-irradiation of Sinonasal Esthesioneuroblastoma: a Case Report

"Head and neck (H&N) cancers are in the 7-8th place among all types of cancer. Despite novel approaches in cancer treatment, most of the patients have a high risk of loco-regional recurrence. Esthesioneuroblastoma (ENB) is a very rare H&N neoplasm, accounting for only 3-6% of all intranasal tumors. These tumors usually are presented with locally advanced stages and required radiotherapy as a part of the comprehensive treatment. Loco-regional failures represent a challenge, and re-irradiation can be effective for some groups of patients. We present a case of re-irradiation with protons of sinonasal tumor in a 49-years old patient with recurrent ENB, involving visual structures. We achieved a 5-year positive local control after the proton therapy (PT), without significant toxicity.The case shows an example of re-irradiation as an essential area for PT application,given the need to maximize the treatment's efficacy and minimize the risk of severetoxicity."

PROFILE OF SINONASAL TUMOR PATIENT IN ADAM MALIK GENERAL HOSPITAL 2016-2018

Sinonasal tumor is a neoplasm that arises from nasal and paranasal sinus tissue. It is rarely found, only <3% of all malignancy in head and neck, and 1% of all malignancy in the body. Although it is rarely found, it can cause serious complication in adult. Unspecific sign and symptoms of sinonasal tumor often make the patients ignore the disease. It leads to the diagnosis of this disease becomes late and the patients come to the doctor in advanced stage. This research was conducted to know about the profile of sinonasal tumor patients in H. Adam Malik Medan Hospital on 2016-2018. The study was a descriptive study with retrospective approach. The data used are secondary data taken from medical records. The data were analyzed using a statistical application programs. Sinonasal tumor patients in RSUP HAM Medan are 158 patients, where 95 patients met the inclusion criteria, consisting of 24 benign tumors (25,3%) and 71 malignant tumors (74,7%). The majority of patients are male (71,6%), and the highest age group in in 51-60 years old (31,6%). The main complaints felt by the patients were nasal symptoms (64,2%), the location of tumors mostly found in nasal cavity (50,5%), the most histopathological type is NKSCC (43,2%), the most occupation is self-employment (26%), and the treatment of the patients are surgery (29,5%). Keyword: Sinonasal Tumor, Profile, Sinonasal Cancer

Orbital decompression for inverted papilloma – Case report

Inverted papilloma is a rare benign sinonasal tumor with a still unclear etiology. In the last decades, HPV implication in inverted papilloma etiopathogeny has been studied and demonstrated in some scientific research papers, but with no causality proven. Imagistic investigations and the Krouse classification establish the surgical approach. Endoscopic surgery represents the golden standard treatment for most cases of inverted papilloma, while external or combined approaches present limited indications. The authors present a case and therapeutic plan of an inverted papilloma located in the ethmoid sinus, with origin in the lamina papyracea and high-risk HPV implication.

Biphenotypic Sinonasal Sarcoma: A Review and Update

Context.— Biphenotypic sinonasal sarcoma (BSNS) is a rare, slow-growing soft tissue sarcoma of the sinonasal tract, typically presenting with nonspecific obstructive nasal symptoms. Although recurrences are common, no metastases have been reported, and only 1 patient has died of disease thus far. It characteristically demonstrates rearrangements of PAX3 with multiple fusion partners, the most common of which is MAML3. Objectives.— To highlight the most important diagnostic features, including morphologic, immunohistochemical, and molecular findings, and to provide comparisons to other entities in the differential diagnosis. We also aim to provide a summary of the clinical features and outcomes in cases reported to date. Data Sources.— Recently published literature encompassing BSNS and its synonym, low-grade sinonasal sarcoma with neural and myogenic differentiation. Conclusions.— BSNS is a sinonasal tumor that is important to recognize because its biologic behavior differs from most of the entities in the differential diagnosis. The diagnosis can typically be rendered through a combination of morphology, immunohistochemical stains, and ancillary testing for characteristic PAX3 rearrangements.