Yong Jiang1, Shu Jiang2. 1. Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, China. 2. Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, China. Electronic address: jiangshu2000@126.com.
Abstract
BACKGROUND: Rosai-Dorfman disease (RDD) is a benign, self-limiting, and nonneoplastic histiocytic proliferative disorder with a poorly defined pathogenesis. Central nervous system involvement is extremely rare, particularly cases with multiple intracranial masses. There is no consensus on optimal treatment and management of RDD. CASE DESCRIPTION: In case 1, a 39-year-old man presented with headaches and paroxysmal convulsions of the limbs. Magnetic resonance imaging showed multiple solid masses located at the right frontoparietal and left frontal meningeal regions. The masses were surgically removed for staging, and the histologic diagnosis was consistent with RDD. In case 2, a 53-year-old man was admitted with epileptic seizures. Magnetic resonance imaging revealed multiple, homogeneously enhancing masses in the left parietal, temporal, and occipital meningeal regions. The largest mass was surgically removed, and histopathologic examination confirmed RDD. In case 3, a 9-year-old girl was admitted with bilateral exophthalmos and incomplete eyelid closure. Magnetic resonance imaging displayed multiple, heterogeneously enhancing masses in the right parietal meningeal region. All masses were surgically removed, and the histopathologic diagnosis was RDD. CONCLUSIONS: RDD with multiple isolated intracranial foci is rare but should be considered in the differential diagnosis when multiple meningiomas are suspected in children and adults. Preoperative diagnosis is challenging, and definitive diagnosis requires immunohistochemical examination. Surgical resection is the most effective therapy for rapid relief of symptoms.
BACKGROUND:Rosai-Dorfman disease (RDD) is a benign, self-limiting, and nonneoplastic histiocytic proliferative disorder with a poorly defined pathogenesis. Central nervous system involvement is extremely rare, particularly cases with multiple intracranial masses. There is no consensus on optimal treatment and management of RDD. CASE DESCRIPTION: In case 1, a 39-year-old man presented with headaches and paroxysmal convulsions of the limbs. Magnetic resonance imaging showed multiple solid masses located at the right frontoparietal and left frontal meningeal regions. The masses were surgically removed for staging, and the histologic diagnosis was consistent with RDD. In case 2, a 53-year-old man was admitted with epilepticseizures. Magnetic resonance imaging revealed multiple, homogeneously enhancing masses in the left parietal, temporal, and occipital meningeal regions. The largest mass was surgically removed, and histopathologic examination confirmed RDD. In case 3, a 9-year-old girl was admitted with bilateral exophthalmos and incomplete eyelid closure. Magnetic resonance imaging displayed multiple, heterogeneously enhancing masses in the right parietal meningeal region. All masses were surgically removed, and the histopathologic diagnosis was RDD. CONCLUSIONS: RDD with multiple isolated intracranial foci is rare but should be considered in the differential diagnosis when multiple meningiomas are suspected in children and adults. Preoperative diagnosis is challenging, and definitive diagnosis requires immunohistochemical examination. Surgical resection is the most effective therapy for rapid relief of symptoms.
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