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Clinical Neurology and Neurosurgery Apr 2024Rosai-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... (Review)
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.
Topics: Humans; Young Adult; Adult; Middle Aged; Histiocytosis, Sinus; Spinal Cord Compression; Cervical Vertebrae; Central Nervous System
PubMed: 38461672
DOI: 10.1016/j.clineuro.2024.108206 -
Otology & Neurotology : Official... Apr 2024To determine if cochlear implant (CI) is safe and effective in patients with radiation therapy (XRT)-induced sensorineural hearing loss and to discuss considerations in... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To determine if cochlear implant (CI) is safe and effective in patients with radiation therapy (XRT)-induced sensorineural hearing loss and to discuss considerations in this population through a retrospective cohort review, systematic review, and meta-analysis.
DATABASES REVIEWED
PubMed, Cochrane Library, and Embase.
METHODS
We retrospectively reviewed all CI cases after head and neck (HN) XRT at our institution, noting intraoperative findings, postoperative complications, and hearing outcomes. Change in speech discrimination scores (SDSs) was the primary outcome measure. Systematic review was performed to identify all cases of CI after HNXRT. A meta-analysis was performed to assess SDS change.
RESULTS
The retrospective cohort review identified 12 patients who underwent CI after HNXRT. One patient with HN cancer (HNC) and one with central nervous system pathology (CNSP) received bilateral implants. Six had HNC, three had CNSP, and one had Langerhans cell histiocytosis. Eleven had abnormal findings during CI. There were no postoperative complications. Twenty articles with an additional 97 patients were suitable for systematic review inclusion. Of the 109 patients, 67 (61.5%) had HNC and 18 (16.5%) had CNSP. Abnormal intraoperative findings were common (30.3%), most frequently in the mastoid (66.7%). Postoperative complications, including wound dehiscence and infection with some requiring explantation, occurred in 10.1% of patients. Sixty-six patients were included in the meta-analysis. All demonstrated SDS improvement (mean increase, 56.2%).
CONCLUSION
Patients with prior HNXRT benefit from CI. Paying careful attention to surgical planning and technique, postoperative care, and patient expectations is imperative, as complications are not uncommon.
Topics: Humans; Cochlear Implantation; Retrospective Studies; Hearing Loss, Sensorineural; Cochlear Implants; Postoperative Complications; Speech Perception
PubMed: 38361317
DOI: 10.1097/MAO.0000000000004127 -
Child's Nervous System : ChNS :... Aug 2023Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based...
INTRODUCTION
Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited.
METHODS
Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG.
RESULTS
In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination.
CONCLUSION
Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.
Topics: Humans; Eosinophilic Granuloma; Conservative Treatment; Skull; Histiocytosis, Langerhans-Cell; Radiography; Remission, Spontaneous
PubMed: 36939904
DOI: 10.1007/s00381-023-05926-z