- Department of Neurosurgery, School of Medicine, Keio University, Tokyo, Japan
- Department of Neurosurgery, Kawasaki Municipal Hospital, Kanagawa, Japan
Department of Neurosurgery, Kawasaki Municipal Hospital, Kanagawa, Japan
DOI:10.4103/2152-7806.92161Copyright: © 2012 Tomio R. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Tomio R, Katayama M, Takenaka N, Imanishi T. Complications of surgical treatment of Rosai–Dorfman disease: A case report and review. Surg Neurol Int 21-Jan-2012;3:1
How to cite this URL: Tomio R, Katayama M, Takenaka N, Imanishi T. Complications of surgical treatment of Rosai–Dorfman disease: A case report and review. Surg Neurol Int 21-Jan-2012;3:1. Available from: http://sni.wpengine.com/surgicalint_articles/complications-of-surgical-treatment-of-rosai-dorfman-disease-a-case-report-and-review/
Background:Rosai–Dorfman disease (RDD) was first described in 1969 as an idiopathic histiocytic proliferative disorder. It commonly presents as a massive and painless adenopathy. Until 1990, extranodal involvement of the central nervous system (CNS) was rare and reported in less than 5% of the total number of patients with extranodal RDD. Complete removal of CNS RDD has been achieved in many cases.
Case Description:We report a case of an isolated intracranial RDD in a 53-year-old man. The patient had an episode of generalized seizures. Imaging studies of the brain were compatible with a meningioma en plaque. The mass was exposed by a right frontotemporal craniotomy. The tumor was adhered tightly to the adjacent cerebral cortex and was permeated by pial arteries of the brain surface. The sacrificing of these arteries was inevitable in order to achieve the total removal of the tumor. The patient had incomplete left hemiparesis after the surgery. Brain computed tomography (CT) imaging revealed a postoperative hemorrhage and a low-density lesion in the right frontal lobe. The patient was postoperatively diagnosed with isolated central nervous system RDD.
Conclusion:Although the complete removal of dural-based lesions without any neurological deficits has been performed in many cases, the treatment of cases with high risks, such as the present case, indicates conservative excisions and adjuvant radiotherapy with or without chemotherapy.
Keywords: En plaque meningioma, intracranial, meningioma, Rosai–Dorfman disease
Rosai–Dorfman disease (RDD) was first described in 1969 as an idiopathic histiocytic proliferative disorder.[
However, there has been a significant increase in the number of isolated CNS RDD cases that have been reported in the last decade. Over 100 cases of CNS RDD have now been reported.[
A 53-year-old homeless man was admitted to the hospital due to a right femoral neck fracture. His medical history included noninsulin-dependent diabetes mellitus. A physical examination performed at admission was unremarkable. There was no fever, lymphadenopathy, or other neurological defects. Laboratory tests revealed the following results: white blood cells, 4330 cells/mL; hemoglobin, 9.2 g/dL; and platelet count, 29,900 platelets/mL. C-reactive protein was negative, and only normochromic-normocytic anemia was present. He successfully underwent surgery for the femoral neck fracture, which was performed by an orthopedic surgeon. However, he had an episode of generalized seizures during the postoperative course, and phenytoin was administered. After this episode of generalized seizures, he was referred to our department for further examination and treatment.
A computed tomography (CT) examination of the head that was conducted without contrast showed a high-density and extraaxial mass in the right parietal convexity, and peritumoral brain edema was clearly observed [
A frozen section of the lesion showed inflammatory cell infiltration, which mainly consisted of lymphocytes and plasma cells, and the presence of these cells was initially interpreted as some kind of hematologic disorder or inflammatory pseudotumor [
(a) Photomicrographs of the frozen sections showing inflammatory cell infiltration, which consisted of lymphocytes and plasma cells, which was initially interpreted as a hematologic disorder or an inflammatory pseudotumor. (b) Paraffin-embedded sections show a hypercellular pattern with features of polymorphous and mixed inflammatory infiltrate that was composed of mainly histiocytes in a background of collagen fibers. (c) The cytoplasm in some histiocytes was foamy and eosinophilic. Some histiocytes were seen to engulf viable lymphocytes, and this was thought to be indicative of emperipolesis (arrow)
These histiocytes were immunopositive for S-100 protein and CD68, but negative for CD1α [
The patient had incomplete left hemiparesis after surgery. A brain CT examination conducted without contrast revealed postoperative hemorrhage and a low-density lesion in the right frontal lobe, which seemed to be due to the sacrificed pial arteries from the brain-tumor interface. His seizures completely ceased.
In order to find evidence of extra-CNS RDD, we performed a whole-body CT examination postoperatively, and findings of lymphadenopathy or other extranodal involvements were not found. The patient has been free of seizures since the surgery, and he recovered from his hemiparesis 6 months after the surgery.
It is known that intracranial RDD may mimic a meningioma in clinical and radiographic findings as well as in surgical findings. Despite the significant increase in the number of reported CNS RDD cases, isolated intracranial RDD remains an underdiagnosed entity. This is, at least in part, due to the fact that neurosurgeons typically encounter meningiomas rather than RDD. Approximately 75% of all CNS RDD cases are intracranial, and 90% cases involve the leptomeninges.[
In the present case, the typical clinical and laboratory findings of systemic RDD, such as lymphadenopathy and an elevation of the erythrocyte sedimentation rate, were not present. Only normocytic anemia was indicative of systemic RDD. Whole-body CT showed that among patients with intracaranial RDD, lymphadenopathy and associated systemic disease were absent in 70% and 52% of the cases, respectively.[
Intracranial RDD mimics meningioma in many aspects of the radiographic appearances and it is still challenging to differentiate between these two diseases. RDD, which displays as an isodense and dura mater-seated mass, is often accompanied by marked peritumoral brain edema on the contiguous cerebral surface on CT and resembles meningioma. The presence of calcification has not been reported in RDD.
RDD lesions are seen as iso- to hyperintense with clear borders on MRI T1 sequences and iso- to hypointense on MRI T2 sequences, and they display obvious enhancement after contrast administration.[
However, a mass lesion of the intracranial RDD often tightly adheres to the adjacent cerebral cortex and its complete removal is sometimes difficult. Our case report is unique in that the mass was located along the convexity and was directly permeated by many pial arteries of the brain surface. It was impossible to preserve these penetrating arteries in the total removal of the tumor. Our patient had postoperative left hemiparesis that was thought to be due to secondary cortical ischemia caused by the sacrificed penetrating pial arteries of the cortex. In a study of meningioma, Inamura et al. and Michael et al. reported that the development of the vascular supply from intrinsic cerebral arteries on angiography significantly correlated with severe peritumoral brain edema.[
We observed both peritumoral brain edema on the CT examination and the penetrating vascular supply from intrinsic cerebral arteries in our intracranial RDD case. This relationship, which is reported for meningiomas, is consistent with our case. In addition, we consider that the findings of peritumoral brain edema on the CT examination in intracranial RDD is an effective predictor of penetrating blood vessels and the severe adherence between the mass surface and the adjacent cerebral cortex, which is just as likely as with meningioma. The finding of peritumoral brain edema in intracranial RDD is considered a risk factor for total removal. Considering the good prognosis after surgery of partially resected intracranial RDD, complete removal should be avoided in cases with high risk, as our case.
Postoperative intraparenchymal hemorrhage was observed in our case. We consider that venous outflow impairment due to sacrificed pial vein is one of the most considerable causes of the intraparenchymal hemorrhage. Parenchymal damage due to drag force and compression during peeling procedure of the adherent tumor, and ischemia due to sacrificed pial arteries are also considered as exacerbating factors. The adjuvant treatment modalities for recurrent intracranial lesions or residual lesions, i.e. radiosurgery and systemic corticosteroids, have been reported with success.[
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