Isolated intracranial Rosai–Dorfman disease mimicking petroclival meningioma in a child

Abstract

Rationale:Rosai –Dorfman disease (RDD) is a rare, idiopathic, and non-neoplastic histioproliferative disease with distinctive entity of unknown etiology. Central nervous system (CNS) RDD is uncommon, hence, isolated intracranial RDD is extremely rare. So far only 6 cases of CNS RDD with the lesions originating from petroclival region have been reported. We present a case of isolated intracranial RDD mimicking petroclival meningioma.Patient concerns:A 14-year-old girl was admitted at our hospital with a 3-month history of dizziness, slowly progressing headache, and 2-month history of instability in walking. Cranial nerve deficits, including left facial paralysis, left facial numbness and left hearing loss, were evident on examination.Diagnoses:Initial diagnosis of petroclival meningioma was made according to preoperative magnetic resonance imaging.Interventions:The lesion was resected subtotally and pathology confirmed RDD. The patient received gamma-knife treatment for the residual lesion.Outcomes:The patient recovered well and the residual lesion significantly retrogressed on follow-up images.Lessons:Preoperative diagnosis of petroclival RDD is full of challenges. Although surgical resection of lesions is an effective treatment option, total resection is not highly recommended because the surgery-related defect must be minimal. Patient with residual lesion can be put on steroid therapy and/or radiotherapy, especially for IgG4 positive subset of RDD.

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

“…8 Other symptoms include focal neurological deficits hemiparesis, visual disturbance, gait impairment, cranial nerve deficit, hearing loss and pituitary dysfunction. 9 The present patient came with the symptoms of seizure and acute hemiparesis.…”

“…Complete surgical resection is the best therapy, but subtotal resection is recommended as a safe approach for symptoms remission and diagnosis when the lesion is located near the critical structure. 9 After surgery, patients usually improves and recurrence and development of neurological deficits have rarely been reported. Adjutant therapy such as radiotherapy, chemotherapy and steroid can be utilized in the patients whose clinical symptoms are not improved, in some cases with recurrence and when resection of mass is not possible.…”

An unusual diagnosis of a dural based intracranial lesion

“…It has a prevalence of 1:200 000 and approximately 100 new cases are diagnosed each year in the United States [2]. It is frequently diagnosed in children and young adults (mean age 20.6 years) [3], predominantly in males (M:F ratio of 1.8:1) [4]. Extranodal disease is a feature of 43% of cases, with central nervous system (CNS) involvement in <5% of cases, and over 300 cases of CNS involvement reported in the literature [5].…”

“…CNS disease most commonly presents as an isolated lesion with a dural attachment, often mistaken for a meningioma on pre-operative imaging [6], though intraparenchymal cranial disease has also been described [7]. In 20% of patients, CNS disease involves the spine [6], with isolated spinal lesions reported in 11% of cases, and combined intracranial and spinal disease in the remaining 9% [4]. The presence of KRAS and MAP2K1 mutations occurs in most cases with head and neck involvement [8].…”

Extranodal Intracranial Rosai-Dorfman-Destombes Disease in Children: a Literature Review

“…One report described recurrence few months later after total resection [3]. Total resection of the lesion with postoperative administration of corticosteroids reduces the risk of disease recurrence and the need for application of adjuvant treatment such as irradiation and immunosuppressive therapy [3,10,15,34]. Incomplete resection of RDD involving the CNS could follow even a "wait and watch" approach in case of complete neurological recovery.…”