Extra-renal rhabdoid tumor in an adult presenting as cervical adenopathy with spondylodiscitis.

INTRODUCTION AND IMPORTANCE: Rhabdoid tumor (RT) is among the most aggressive and lethal types of cancer. It most commonly arises in the central nervous system, kidney, or soft tissues. It typically affects young children. CASE PRESENTATION: We present a case of a 21 years old man with an extrarenal rhabdoid tumor (ERRT) in the neck extending to the vertebra and epidural space. Clinical presentation was at first misleading as it presented as infectious spondylodiscitis with cervical adenopathies. The patient developed quadriplegia a few days after biopsy was taken and succumbed to his disease a week later. CLINICAL DISCUSSION: The diagnosis of MRT is mainly based on histopathology, immunohistochemistry and molecular studies. Loss of INI1 expression due to biallelic mutation of the SMARCB1 gene is characteristic. Because of the lack of standardized treatment protocols for MRT, various combinations of chemotherapeutic drugs are used. However, prognosis remains poor especially for cervical vertebral localization as it results in a rapid fatal outcome secondary to respiratory failure. CONCLUSION: SMARCB1-related biology and potential therapeutic targets are the main focus of preclinical investigations that aim to expand treatment options and improve patient's survival.

Cervical approach to cervico-mediastinal goiters: Experience of a Moroccan ENT tertiary center - Case series.

BACKGROUND: The purpose of the study was to analyze and discuss the demographic, clinical, radiological, therapeutic and postoperative findings of the Cervico-mediastinal goiters (CMG) treated through a cervical approach admitted in the ENT department of Ibn Rochd university hospital, Casablanca, Morocco between January 2014 and January 2020. MATERIELS AND METHODS: Over a period of 6 years, 116 patients underwent surgical treatment for CMG. It was defined as a goiter extending below the plane of superior thoracic aperture on CT scan. All our patients had clinical, biological and radiological assessment before surgery. A nasofibroscopy was carried out pre and postoperatively. All the CMG have been extracted trough a cervical approach by an experimented ENT surgeon. RESULTS: 84,48% of the CMG was diving into the anterior mediastinum and 15.52% into the posterior. The CMGs extended above, at, and below the level of the aortic arch respectively in 76.72%, 18.10% and 5.17% of the patients. All of 116 goiters were successfully removed through a cervical approach. No patient required a sternotomy. Postoperatively, vocal cord paralysis was transient in 3 patients (2.58%) and permanent in 2 patients (1.72%). Hypocalcemia was transient in 10 patients (8.62%) and permanent in 2 patients (1.72%). Final histology found 106 benign multinodular goiters (91.37%), 7 papillary carcinomas (6.03%) and 3 vesicular carcinomas (2.58%). No death was noted. CONCLUSIONS: With expertise in thyroid surgery, cervical approach for CMGs is safe and sufficient in the majority of the cases with low morbidity rate and no mortality.