Long-Term Outcome of Spinal Extranodal Rosai-Dorfman Disease: A Report of Two Cases and Systematic Review.

BACKGROUND: Rosai-Dorfman disease (RDD) is a rare pathologic entity caused by sinus histiocytosis with massive cervical lymphadenopathy. Isolated spinal involvement is an infrequent presentation of extranodal RDD. The clinical and radiologic appearance of RDD represents a diagnostic challenge. We report 2 patients with paraparesis caused by RDD of the thoracic spine and a PRISMA-style systematic review. CASE DESCRIPTION: There were 2 patients with isolated extranodal thoracic spinal RDD without cervical lymphadenopathy. One patient presented with anterior thoracic RDD and a subtotal resection. The small residual disease completely responded to the postoperative course of steroids. The second patient had extradural thoracic spine RDD, which was resected completely. A 6-month postoperative follow-up magnetic resonance imaging (MRI) scan showed local recurrence, which responded to radiation therapy. Five years follow-up of both patients showed normal neurologic functions and no recurrence on MRI scan surveillance. CONCLUSIONS: RDD is a rare occurrence and should be considered in the differential diagnosis of extradural or intradural spinal lesions. Gross total resection is recommended, and long-term clinical follow-up with MRI is advised. Residual or recurrent RDD requires steroids or radiation therapy.

Endolymphatic sac tumor at the cerebellopontine angle: A case report and review of literature.

INTRODUCTION: Endolymphatic sac tumors may present as sporadic or may be associated with Von Hippel-Lindau disease. Patients generally present with hearing loss, tinnitus and vertigo. The tumor is highly vascular which may lead to erosion of the adjacent bony and vascular structures, resulting in heavy bleeding during surgery. PRESENTATION: A twenty-five year-old female presented with a five year history of chronic ear discharge, left sided facial weakness, and recent onset of ataxia. DISCUSSION: The unusual clinical presentation made management challenging, in large part due to profuse bleeding. Pre-operative embolization of the vessels supplying the tumor may reduce blood loss during surgical excision. Radiotherapy could be considered for any residual tumor. CONCLUSION: The patient was diagnosed with an endolymphatic sac tumor of sporadic origin which presented at the cerebellopontine angle and was managed with a multidisciplinary approach.