Background
Rosai- Dorfman Disease (RDD) is a benign condition of unknown aetiology which is characterized by non-neoplastic proliferation of histiocytes. Pathophysiology and natural history remain obscure due to the low prevalence of disease. It is known to present with nodal or extranodal involvement and occurrence in the genitourinary system could lead to dreadful complications. RDD is diagnosed by demonstrating emperipolesis on histology and supported by S100 positivity in immunohistochemistry. Treatment is tailored individually and includes expectant monitoring, steroids, surgery, chemotherapy and radiotherapy. Prognosis will be poor if there is involvement of vital organs. We report a rare case of renal Rosai-Dorfman Disease in a 12-year-old girl which also associated with cold type autoimmune haemolytic anaemia (AIHA).
Case presentation
A previously healthy, 12-year-old girl presented with low grade fever and cough over one month. On examination, she was pale, mildly icteric and had a firm mass in the left hypochondrial region.
Her blood count revealed significant eosinophilia, normocytic normochromic anaemia and thrombocytosis. Further laboratory investigations revealed reticulocytosis, positive urine urobilinogen, positive direct antiglobulin test and red blood cell agglutination on blood picture suggestive of autoimmune haemolytic anaemia.
Ultrasound scan of abdomen revealed paraaortic and left side retroperitoneal lymphadenopathy with left renal mass. It was further evaluated by Contrast Enhanced Computed Tomography (CECT). Biopsy was done and that concluded sinus histiocytosis with massive lymphadenopathy (SHML) with positive S100 and CD1a in immunohistochemistry. Child was treated with steroids however there was no significant response as assessed by repeat CT and has been commenced on chemotherapy.
Conclusion
RDD is believed to be due to host immune dysregulation and precise diagnosis is imperative. It should be considered as differential diagnosis in a child presenting with massive lymphadenopathy and AIHA. Association between RDD and AIHA may possibly be explained by abnormal immune response of the host.
Background
Ganglioneuroma is a rare neurogenic tumor which could grow into massive size in the thorax with minimal or no symptoms. Resection of these tumors will be challenging in view of large size and vascular encasement. Here, we report resection of a thoracic ganglioneuroma in a child with infiltration of spinal canal and lymph node metastasis, which is a very rare occurrence.
Case presentation
A 3-year and 10-month-old girl presented with a left side thoracic mass with minimal symptoms and signs. After the initial imaging with ultrasound scan and cross-sectional imaging, she had a true cut biopsy of the lesion confirming the diagnosis of ganglioneuroma. On exploration, she had a paraspinal thoracic mass extending from level of arch of aorta down to diaphragm with evidence of tumor infiltration of the intercostal spaces and spinal canal in multiple vertebral levels. Pathologically, she had infiltrated lymph nodes which were encircling the lower thoracic aorta. The tumor was resected successfully, and she recovered with a good functional status.
Conclusion
Thoracic ganglioneuroma has a good prognosis after complete resection even with spinal canal and lymph node infiltration. The resection could be arduous in case of massive size and vascular encasement; hence, benefit versus risk of complete resection should be carefully weighed.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.