INTRODUCTION: Rosai-Dorfman Disease (RDD) is a benign histiocytic disorder that commonly presents with massive lymphadenopathy. Central nervous system (CNS) involvement is only 5%; isolated CNS involvement is much rarer. The patient presented here has been diagnosed with isolated pontine RDD. CASE REPORT: A 5-year-old child was admitted to the hospital with the history of double vision and ptosis of the left eye. Magnetic resonance imaging revealed presence of a mass at the pontomesencephalic junction. Subtotal tumor excision was performed. Microscopic examination of the lesion showed histologic features consistent with RDD. The patient was followed up without medication, with a small residue. CONCLUSION: RDD is rare in childhood, and isolated CNS involvement is also quite rare. The tumor is located in pons in our case. The treatment of choice is gross total resection. Even total excision is not possible, follow up without medication may be an option because of the indolent and self-limited course of the disease.
INTRODUCTION:Rosai-Dorfman Disease (RDD) is a benign histiocytic disorder that commonly presents with massive lymphadenopathy. Central nervous system (CNS) involvement is only 5%; isolated CNS involvement is much rarer. The patient presented here has been diagnosed with isolated pontine RDD. CASE REPORT: A 5-year-old child was admitted to the hospital with the history of double vision and ptosis of the left eye. Magnetic resonance imaging revealed presence of a mass at the pontomesencephalic junction. Subtotal tumor excision was performed. Microscopic examination of the lesion showed histologic features consistent with RDD. The patient was followed up without medication, with a small residue. CONCLUSION: RDD is rare in childhood, and isolated CNS involvement is also quite rare. The tumor is located in pons in our case. The treatment of choice is gross total resection. Even total excision is not possible, follow up without medication may be an option because of the indolent and self-limited course of the disease.
Authors: P Purav; K Ganapathy; V S Mallikarjuna; S Annapurneswari; S Kalyanaraman; J Reginald; P Natarajan; K R Suresh Bapu; M Balamurugan Journal: J Clin Neurosci Date: 2005-08 Impact factor: 1.961
Authors: Oussama Abla; Eric Jacobsen; Jennifer Picarsic; Zdenka Krenova; Ronald Jaffe; Jean-Francois Emile; Benjamin H Durham; Jorge Braier; Frédéric Charlotte; Jean Donadieu; Fleur Cohen-Aubart; Carlos Rodriguez-Galindo; Carl Allen; James A Whitlock; Sheila Weitzman; Kenneth L McClain; Julien Haroche; Eli L Diamond Journal: Blood Date: 2018-05-02 Impact factor: 22.113
Authors: Rafael Trindade Tatit; Paulo Eduardo Albuquerque Zito Raffa; Giovana Cassia de Almeida Motta; André Alexandre Bocchi; Júlia Loripe Guimaraes; Paulo Roberto Franceschini; Paulo Henrique Pires de Aguiar Journal: Surg Neurol Int Date: 2021-06-21