- Department of Neurosurgery, Hospital Dr. Luis E. Aybar, Calle 6, Santo Domingo, Republica Dominicana
- Section of Neuropathology, Hospital Dr. Luis E. Aybar, Calle 6, Santo Domingo, Republica Dominicana
- Department of Hematology, Hospital Dr. Luis E. Aybar, Calle 6, Santo Domingo, Republica Dominicana
- Department of Neurosurgery, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana
- Department of Radiology, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana
Department of Radiology, CEDIMAT, Plaza de la Salud, Santo Domingo, Republica Dominicana
DOI:10.4103/2152-7806.128003Copyright: © 2014 Rivera D. 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: Rivera D, Miguelina Pérez-Castillo, Belkis Fernández, Stoeter P. Long-term follow-up in two cases of intracranial Rosai–Dorfman Disease complicated by incomplete resection and recurrence. Surg Neurol Int 28-Feb-2014;5:30
How to cite this URL: Rivera D, Miguelina Pérez-Castillo, Belkis Fernández, Stoeter P. Long-term follow-up in two cases of intracranial Rosai–Dorfman Disease complicated by incomplete resection and recurrence. Surg Neurol Int 28-Feb-2014;5:30. Available from: http://sni.wpengine.com/surgicalint_articles/long-term-follow-up-in-two-cases-of-intracranial-rosai-dorfman-disease-complicated-by-incomplete-resection-and-recurrence/
Background:Although intracranial Rosai–Dorfman disease is a principally benign lymphohistiocytosis, some patients run a relapsing or progressive course. However, reports about long-term follow-up are extremely rare.
Case Description:In two patients, initial tumor resection was incomplete or followed by recurrences over 3 years, which finally subsided after application of chemotherapy, and patients remained tumor-free for more than 7 years thereafter.
Conclusion:Up to now there is no agreement on how to treat complicated cases of intracranial Rosai–Dorfman disease; our good experience with adjuvant chemotherapy and long-term follow-up will contribute to treatment planning in complicated cases.
Keywords: Intracranial Rosai–Dorfman disease, incomplete resection, recurrence, chemotherapy, long-term follow-up
Rosai–Dorfman disease (RDD) has been described as a benign lymphohistiocytosis presenting as a bilateral massive lymphoadenopathy with fever, neutrophilia, and an elevation of the sedimentation rate and gammaglobulins mainly affecting children and young adults. Histologically, the condition is characterized by large pale histiocytes containing engulfed erythro- and lymphocytes (“emperipolesis”). These so-called Rosai–Dorfman cells are CD68 and S-100 positive by immune-staining in the absence of Birbeck granules.[
The central nervous system (CNS) – mainly the meninges – are involved in less than 5% of cases. Because the clinical and radiologic differentiation from meningiomas is difficult, surgery has been applied as first therapy and is considered to be essential for the diagnosis as well. After total removal, the outcome is usually good, but postoperative corticosteroids have been recommended, and some cases may need additional chemotherapy or radiation. However, because there is no standard treatment and reports about long-term results in difficult cases are rare,[
A 22-year-old male presented with progressive headache, focal convulsions, and monoparesis of his right arm and loss of vision of the right eye due to papillary congestion. In addition, he had noted a left supraclavicular swelling. Imaging showed a large extracranial space-occupying dura-based mass [
A 22-year-old male patient (case 1). (a) CT after injection of contrast medium shows an extra-axial left parietal tumor with strong enhancement, which was removed completely on first operation. (b) CT of left temporal tumor recurrence 29 months after first operation, which again was removed completely on second operation. (c) CT of intraorbital retrobulbar tumor recurrence 12 months after second operation
A 39-year-old male presenting with progressive headache since one year and a focal convulsion of the right side of his face on the day of admission. Computed tomography (CT) showed four space-occupying hyperdense lesions with prominent surrounding edema and enhancement in a left fronto-temporal, parietal/paramedian, occipital, and right central/periventricular localization [
A 39-year-old male patient (case 2). CT after injection of contrast medium shows four enhancing lesions in a left fronto-temporal, parietal/paramedian, occipital, and right central/periventricular localization. On first operation, the fronto-temporal, and on second operation 2 years later, the parietal tumor was removed
In a first survey of 423 patients with RDD,[
Our two cases of a predominantly intracranial localization of RDD showed a complicated course after surgical removal as the only treatment, but have been free of any further recurrences after application of chemotherapy now for 7 years. To our knowledge, this is the longest follow-up time reported in intracranial recurrences of this condition. Long-term observation is needed for two reasons: First, because there is no standard therapeutic concept as shown earlier and second, because recurrences may occur after several years as we can see from reports of progressions or recurrences after a latency of several years. A stable intraorbital lesion treated by immune-suppressive therapy progressed after 5 years,[
In intracranial RDD, the usual first approach is a surgical one because preoperative differentiation from meningiomas on imaging criteria alone is extremely difficult, and debulking of a space-occupying tumor is indicated. In difficult localizations, however, or in systemic involvement, biopsy may be the first option. Histology will then show the correct diagnosis, and the further course can be followed. However, according to our experience with two complicated cases, we recommend additional chemotherapy if there are signs of tumor persistence or recurrence. Another secondary treatment option might be stereotactic radiation of local recurrences or partially resected tumors, although experiences in this field are yet limited. Thereafter, long-term follow-up for several years is strongly recommended.
1. Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S. Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res. 2010. 32: 572-8
2. Ben Turkia H, Ben Romdhane M, Azzouz H, Ben Chehida A, Abdelmoula MS, Ben Abdelaziz R. Rosai -Dorfman disease: A two cases report. Tunis Med. 2011. 89: 497-501
3. De Silva D, Joshi N. Rosai-Dorfman disease recurrence with bilateral orbital masses following immunosuppressant therapy. Orbit. 2005. 24: 51-3
4. Fisher RI, Gaynor ER, Dahlberg S, Oken MM, Grogan TM, Mize EM. Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin's lymphoma. N Engl J Med. 2005. 328: 1002-6
5. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Semin Diagn Pathol. 1990. 7: 19-73
6. Le Guenno G, Galicier L, Uro-Coste E, Petitcolin V, Rieu V, Ruivard M. Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system. J Neurosurg. 2012. 117: 486-9
7. Nalini A, Jitender S, Anantaram G, Santosh V. Rosai Dorfman disease: Case with extensive dural involvement and cerebrospinal fluid pleocytosis. J Neurol Sci. 2012. 314: 152-4
8. Seyednejad F, Tubbs RS, Shoja MM, Daghigi MH, Oakes WJ. Presumed recurrence of intracranial Rosai-Dorfman disease as a cervical spine tumor. Acta Neurochir (Wien). 2007. 149: 425-7
9. Walker RN, Nickles TP, Lountzis NI, Jacobs DL, Nawaz NK. Rosai-Dorfman disease with massive intracranial involvement: Asymmetric response to conservative therapy. J Neuroimaging. 2011. 21: 194-6
10. Zhu F, Zhang JT, Xing XW, Wang DJ, Zhu RY, Zhang Q. Rosai-Dorfman disease: A retrospective analysis of 13 cases. Am J Med Sci. 2013. 345: 200-10