Isolated Rosai-Dorfman disease of the spine: A systematic literature review.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis involving the central nervous system in 5% of cases. Spinal location occurs in less than 1% of extranodal RDD and can be responsible for neurological manifestations. We present a systematic review of cases of isolated spinal RDD. We also report a new case of isolated spinal RDD revealed by spinal cord compression. MATERIALS AND METHODS: The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing isolated RDD of the spine. RESULTS: There were 53 patients with isolated spinal RDD (including our case). The mean age was 35.85±16.48 years. Neurological deficit was the most frequent clinical presentation (89%). RDD lesions were mainly located in the thoracic spine (51%), then the cervical spine (32%). The lesion was reported to be extradural (57%), intradural extramedullary (26%), intramedullary (7%), and in the vertebral body (10%). Histological examination showed emperipolesis in 73%. Histocytes were positive for S-100 protein in 83%. Treatment was based on surgery 96%), radiotherapy, chemotherapy, and adjunctive steroid therapy were indicated in four, one, and eight cases. After a mean follow-up period of 14.84±13.00 months, recurrence of RDD was noted in 15%. CONCLUSION: Spinal RDD is a rare condition, requiring meticulous histological examination for accurate diagnosis. Complete surgical resection is the treatment of choice. Adjuvant chemotherapy and radiotherapy can also be indicated in patients demonstrating partial improvement following surgery.

Surgical perspective on perianal Bowen's disease: A rare case report.

INTRODUCTION AND IMPORTANCE: Bowen's Disease (BD) stands out as a dermatologic entity known for its rarity and diagnostic intricacies. While BD is recognized for its diverse clinical presentations, its occurrence in the perianal region is particularly exceptional. Our case contributes to the limited body of knowledge regarding perianal BD, shedding light on its distinctive characteristics and guiding clinicians in navigating the intricacies associated with this uncommon presentation. CASE PRESENTATION: We present the case of a 67-year-old female who sought medical attention for persistent itching in the perianal region. A thorough examination revealed a solitary BD lesion, a notable rarity in this anatomical site. Considering the distinctive characteristics and location, surgical excision was chosen as the preferred treatment strategy. The postoperative course was straightforward, yielding favorable aesthetic outcomes and no recurrence. CLINICAL DISCUSSION: The clinical discussion explores the unique challenges associated with perianal BD, emphasizing the rarity of its presentation and its potential to mimic other dermatologic conditions. Factors contributing to the diagnosis, including clinical indicators and risk factors, are scrutinized. Furthermore, the discussion delves into the evolving landscape of diagnostic tools and treatment modalities, especially relevant in the context of perianal BD. CONCLUSION: This case illuminates the rarity of BD in the perianal region, serving as a valuable addition to the limited body of knowledge on this unusual presentation. By unraveling the complexities associated with perianal BD, this report contributes to a deeper understanding of the disease and provides insights that can guide clinicians in navigating similar cases.

Myxoid Liposarcoma of the Parotid Gland.

Liposarcoma is extremely rare in the parotid gland. In this article, we report the case of an 87-year-old man who presented to our department with swelling in the left parotid region. Magnetic resonance imaging (MRI) revealed a tumor in the superficial lobe of the parotid gland. Total parotidectomy and ipsilateral lymph node dissection were performed. Histologic examination confirmed the diagnosis of myxoid liposarcoma of the parotid gland. On imaging, there was no evidence of nodal or distant metastasis. Radiation therapy was planned, but the patient refused to receive treatment. After a follow-up of 3 years, MRI showed no sign of tumor recurrence and the patient remained symptom-free. The case is original by the tumor's location. Only 8 cases of primary parotid liposarcoma have been reported in the literature. The purpose of this article was to discuss, through our case, clinical and anatomopathological features of parotid gland liposarcomas as well as its treatment options and prognosis.