A Case Of SMARCB-1 (INI-1)-deficient Sinonasal Carcinoma with Clotting Factor XI Deficiency

Autor: xinlu yu, tingyue zheng, jingpu yang
Rok vydání: 2022
Popis: BACKGROUND: SMARCB-1-deficient sinonasal carcinoma(SDSC) is a rare type of paranasal sinus malignant tumor that was newly discovered in recent years. This type of tumor is characterized by a high degree of malignancy and rapid progression, and is mostly diagnosed at an advanced stage.CASE PRESENTATION: This report serves to describe a case of an atypical imaging appearance with clotting factor XI deficiency. Computed tomography and MRI revealed that uneven soft tissue density was in the right anterior ethmoid sinus with the right lamina papyracea bone defect. The tumour was surgically removed, followed by further radiotherapy and chemotherapy. No tumor recurrence and metastasis during six months follow-up. CONCLUSION: As shown in this case, the imaging presentation is not all typical for patients, especially in the early stages. We should connect clinical symptoms with imaging features and find key clinical features to facilitate early diagnosis.Whether XI coagulation factor deficiency is related to SMARCB-1 deficiency is still unclear.
Databáze: OpenAIRE