Vol. 2 No. 17. VOLUME 2 - No SEPTEMBER 15, 2012 Fortnightly ISSN

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1 VOLUME 2 - No SEPTEMBER 15, 2012 CENTAURO S.r.l., BOLOGNA Fortnightly ISSN Vol. 2 No. 17 Oficial Journal of: AINR - Associazione Italiana di Neuroradiologia and: The Neuroradiologists of Alpe-Adria ANRS - Albanian Neuroradiological Society PANRS - Pan Arab NeuroRadiology Society Radiological Society of Saudi Arabia, Division of Neuroradiology Egyptian Society of Neuroradiology ISNR - Indian Society of Neuroradiology Indonesian Society of Neuroradiology Neuroradiology Section of the Radiology Society of Iran Israeli Society of Neuroradiology College of Radiology Malaysia Neuroradiology Section - Pakistan Psychiatry Research Center Section of Neuroradiology - Polish Radiological Society The Neuroradiologists of Romania Section of Neuroradiology of Serbia and Montenegro SILAN - Sociedad Ibero Latino Americana de Neurorradiologia Neuroradiology Section of Singapore Radiological Society Slovenian Society of Neuroradiology The Neuroradiological Society of Taiwan TSNR - Turkish Society of Neuroradiology

2 Official Journal of: AINR - Associazione Italiana di Neuroradiologia and The Neuroradiologists of Alpe-Adria ANRS - Albanian Neuroradiological Society PANRS - Pan Arab NeuroRadiology Society Radiological Society of Saudi Arabia, Division of Neuroradiology Egyptian Society of Neuroradiology ISNR - Indian Society of Neuroradiology Indonesian Society of Neuroradiology Neuroradiology Section of the Radiology Society of Iran Israeli Society of Neuroradiology College of Radiology Malaysia Neuroradiology Section - Pakistan Psychiatry Research Center Section of Neuroradiology - Polish Radiological Society The Neuroradiologists of Romania Section of Neuroradiology of Serbia and Montenegro SILAN - Sociedad Ibero Latino Americana de Neurorradiologia Neuroradiology Section of Singapore Radiological Society Slovenian Society of Neuroradiology The Neuroradiological Society of Taiwan TSNR - Turkish Society of Neuroradiology Index Magnetic Resonance Spectroscopy 591 for Differentiating Pseudo-Progression from Tr ue Progression in GBM on Concurrent Chemoradiotherapy V. Sawlani, R. Taylor, K. Rowley, R. Redfern, J. Martin, H. Poptani A Rare Case of Intracranial 603 Rosai-Dorfman Disease Mimicking Multiple Meningiomas. A Case Report and Review of the Literature A. Catalucci, G. Lanni, L. Ventura, A. Ricci, R.J. Galzio, M. Gallucci Imaging Characteristics of Cavernous 609 Sinus Cavernous Hemangiomas. R.R. Yadav, D.K. Boruah, G. Yadav, R. Pandey, R.V. Phadke Traumatic Dissecting Posterior Cerebral 619 Artery Aneurysm. A Case Report and Review of the Literature M.G. Salehi, H. Ghanaati, M. Abedini, A.H. Jalali NRJ Digital - Corso ECM 625 FAD - Formazione a Distanza Information and Congresses 632 Instructions for Authors 629 Cover: Max Pechstein - Rotes Mädchen am Tisch (Red Girl at a Table), Oil on canvas, 75 75,5 cm Album di Cea,

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4 NRJ Digital - The Neuroradiology Journal 2: , 2012 A Rare Case of Intracranial Rosai-Dorfman Disease Mimicking Multiple Meningiomas A Case Report and Review of the Literature A. CATALUCCI, G. LANNI, L. VENTURA, A. RICCI, R.J. GALZIO, M. GALLUCCI Department of Neuroradiology, S. Salvatore Hospital, University of L Aquila, Italy Key words: Rosai-Dorfman disease, MRI, meningiomas SUMMARY Rosai-Dorfman disease (RDD) was firstly described in 1969 as a benign proliferative disorder of histiocytes with systemic symptoms and lymphadenopathy. This disease is of uncertain pathogenesis and mostly occurs in children and young adults. The typical clinical features of RDD include bilateral painless cervical lymphadenopathy, but extranodal involvement may also be present. The most common extranodal sites include organs such as the respiratory tract, skin, nasal cavity, orbit and bone. Isolated central nervous system (CNS) manifestations are extremely rare. In case of CNS involvement, the commonest imaging findings are dural-based extra-axial enhancing masses. We describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically in a 57-year-old man presenting with a six-year history of progressive right visual and hearing loss and tinnitus. In cases of multiple extra-axial lesions it is worth bearing in mind the possible differential diagnosis for intracranial RDD and eventually propose to the patient further investigations. Introduction The first report of sinus histiocytosis with massive lymphadenopathy (SHML) has been attributed to Destombes who described a lipid storage disorder after a biopsy performed in a young man in A decade later, Rosai and Dorfman recognized the clinical syndrome caused by this rare, benign, lymphoproliferative disorder for which they named SHML 1-7,9. The pathogenesis of this disease is uncertain but it has been attributed to an immunodepression caused by suppressor macrophages induced to differentiate from stimulation by M-CSF. Epstein-Barr virus or HIV could also be involved. Immunodepression derives from phagocytosis of autologous lymphocytes, a process called emperipolesis 2,8,10. This disease entity predominantly affects children and young adults with a mild male sex predilection. In 95% of cases Rosai-Dorfman disease/shml presents with painless and bilateral cervical lymphadenopathy associated with fever and systemic symptoms related to overproduction of monokines from histiocytes. In 25% of cases there is an extranodal disease with involvement of the orbits, superior airways, skeleton, skin, gastrointestinal tract, genitourinary tract, thyroid and central nervous system 1,2,5,12. The extranodal involvement has prompted the use of the terminology Rosai-Dorfman disease (RDD). Intracranial location of RDD is rare and described in about 5% of the cases, the majority of which presenting as an isolated lesion 2,5,13,19. We describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically. Case Report A 57-year-old man presented with a six-year history of progressive right visual and hearing loss and tinnitus. Head MRI examination revealed multiple, bilateral extra-axial enhancing lesions, seven extra-axial and one intraventricular (Figure 1). 603

5 A Rare Case of Intracranial Rosai-Dorfman Disease Mimicking Multiple Meningiomas A. Catalucci 604

6 NRJ Digital - The Neuroradiology Journal 2: , 2012 A B C D E F G H Figure 2 Right extra-axial sphenoidal lesion. The lesion appears isointense on T1- and T2-w images (A,B) (white arrows), strongly and omogeneously enhancing after gadolinium administration, with a dural tail (C,D) (black arrows). It was also isointense with brain tissue on DWI-w images (E) (thin white arrow), without significant diffusivity anomalies on ADC map (F) (thin black arrow). On CT scan, the lesion appears as a regular and slightly hyperdense mass (G) (large black arrow), without significant bony involvement (H) (large white arrow). All lesions had the same density on CT scan and the same signal intensity on MRI: they appeared slightly hyperdense in CT, and isointense to the brain in all MRI sequences, with dural attachment and homogeneous enhancement after contrast agent administration, as shown in Figure 2. Which kind of lesions could be consistent with these CT and MRI findings? As the patient had no history of systemic disease or can- Figure 1 Axial T1-weighted MRI images after contrast medium administration, showing multiple extra-axial strongly enhancing lesions, with different localizations: close to the cerebral falx within the interemisferic fissure (white arrows), in the right perirolandic region (black arrow), in the right temporo-polar/sphenoidal (thin white arrows) and insular region (large white arrow), close to the left tentorium (thin white dotted arrow) and, the biggest one, in the right petroclival region (large black arrow); another lesion is present within the atrium of right lateral ventricle (thin black dotted arrow). cer, imaging findings suggested the neuroradiological diagnosis of multiple meningiomas. He underwent surgical treatment of the largest infratentorial petroclival right mass, through a right transpetrosal approach. The surgical material, consisting of multiple grayish fragments, was fixed in 10% buffered formalin, routinely processed and embedded in paraffin to obtain 4μm-thick sections, stained with hematoxylin-eosin, periodic acid-schiff, Grocott and Ziehl-Neelsen methods. Further histological slides were immunostained with antibodies against AE1/AE3 cytokeratins, EMA, GFAP, vimentin, CD68, S100 protein and CD1a. Microscopic examination (Figure 3) revealed diffuse lymphocytic infiltrates with numerous plasma cells and prominent histiocytic cells showing abundant pale cytoplasm and central nuclei without atypia. Emperipolesis could not be demonstrated. Special stains for bacterial 605

7 A Rare Case of Intracranial Rosai-Dorfman Disease Mimicking Multiple Meningiomas A. Catalucci A B C Figure 3 Histological findings. A) Inflammatory infiltrate of lymphocytes, plasma cells, and histiocytes in a fibrous background (hematoxylin eosin, original magnification 100 ). B) Cytoplasmatic positivity (brown) of histiocytes for CD68. C) Cytoplasmatic positivity of histiocytes for S-100 protein, on the left. and fungal organisms were negative. The histiocytes were strongly positive for S100 protein and CD 68, without immunostaining for CD1a and other antibodies. Such findings indicated the histopathological diagnosis of intracranial RDD. Subsequently, a post-operative total-body CT examination showed other localizations of the disease: a paravertebral dorsal mass, mediastinal swollen lymph nodes and splenomegaly (Figure 4). Discussion Rosai-Dorfman disease (RDD) is considered a benign self-limiting histiocytic disorder of presumed non-neoplastic nature. Although nonneoplastic, RDD may be associated with significant morbidity, and can even cause death, namely in patients with immune dysfunction. The pathogenesis is unknown, but immunological studies suggest that dysfunction of the immune system or an autoimmune process might be the causative factor 1,2,4,7,9. The disease may involve virtually any organ or system. Extensive disease and involvement of visceral organs and/or vital structures are associated with a worse prognosis 7,14. CNS RDD is rare (5% of the cases) and most commonly involves patients between 22 and 63 years of age, with a male predominance; the mean age at presentation is 41 years. Blood values include anemia, neutrophil leukocytosis, increased polyclonal gammaglobulines levels and elevation of inflammation indexes. Histological examination of affected lymph nodes shows widening of lymphatic sinuses with consequent disruption of internal architecture of lymph nodes. Sinuses are full of lymphocytes, plasmocytes and histiocytes without malignant characteristics. Typically, histiocytes stain positively for S-100 protein and CD68, but negatively for CD1a. Emperipolesis, signifying the phagocytosis of autologous lymphocytes, is characteristic of RDD but is present in only 70% of cases 15. Isolated central nervous system (CNS) manifestations are extremely rare, in particular if 606

8 NRJ Digital - The Neuroradiology Journal 2: , 2012 purely intraparenchymal lesions, the commonest imaging findings are dural-based extra-axial enhancing masses 2,4,5,11. The common sites of RDD in the CNS are the cerebral convexity, cranial base, parasagittal, suprasellar region, cavernous sinus, and petroclival region; intraparenchymal and intraventricular lesions could be also found. Clinically, like meningiomas, intracranial RDD causes a variety of symptoms depending on lesion location. Thus headache, epilepsy, visual and hearing loss, cranial nerve deficits, weakness of limbs, confusion, short-term memory problems, difficulty with speech, visual hallucinations and various other symptoms may be seen or become evident with disease progression 7. The commonest findings on imaging suggest dural-based, extra-axial, well-circumscribed enhancing masses with possible perilesional cerebral edema On CT-scans RDD lesions are well-defined hyperdense masses. On T1- weighted MRI, RDD lesions are hyperintense or isointense with clear borders, while on T2-weighted images they have an isointense signal with possible intralesional foci of lowsignal intensity. DWI-weighted images and relative ADC maps, useful as other functional techniques in characterization of extra-axial lesions 21,22, do not show diffusivity anomalies in case of RDD lesions. After administration of contrast agent, the enhancement is intense and homogeneous. Thus, the disease closely mimics meningiomas clinically and radiologically, in agreement with other cases reported in literature 2,4,5,11,16. The differential diagnosis may include other pathologies characterized by dural involvement and strong enhancement after gadolinium injection, as such as meningioangiomatosis 23, dural metastases 22, Wegener s granulomatosis, Erdheim-Chester disease, sarcoidosis 20, Hodgkin lymphoma, plasma cell granuloma, inflammatory pseudotumor and Langerhans histiocytosis. The natural history of the disease is quite variable, usually alternating periods of exacerbations and resolutions or, rarely, pursuing a progressive course. Thus, most patients do not require any treatment, the disease being self-limiting in most cases. Some patients may present more severe clinical features that require treatment to slow disease progression. There is no codified treatment. Corticosteroid therapy has been proposed and chemotherapy is another modality used to manage RDD, A B C Figure 4 CT scans after contrast agent administration show splenomegaly (A) (white arrow), periaortic swollen lymph nodes (B) (white arrow) and paravertebral solid tissue (C) (white arrow). 607

9 A Rare Case of Intracranial Rosai-Dorfman Disease Mimicking Multiple Meningiomas A. Catalucci in cases of extensive disease or progressive. Jabali et al. reported that the combination of prednisone, 6-mercaptopurine, methotrexate, and vinblastine was effective and quite safe for the treatment of the disease. Surgery is used to relieve compression of vital structures, alleviate local damage, or preserve function. For intracranial localization there s a poor disease progression after surgery while the operative mortality after surgery is nearly 4%. Petzold et al. found intracranial tumor regrowth or recurrence of symptoms in 14% of 29 patients with a mean follow-up of 10.1 years 19. Radiation therapy has been employed for local control of the disease in such areas as the spinal cord or the airways 3,8,11,16,18. Conclusions The majority of patients with RDD do not require treatment, especially surgical treatment, demonstrating spontaneous resolution or stable disease. Intracranial lesions may not be distinguished from meningiomas. On the bases of our experience, in cases of multiple extra-axial lesions it is good to bear in mind the possible differential diagnosis for intracranial RDD and eventually suggest the patient undergo further investigations. Although neuroradiology might be of aid in the diagnosis of isolated intracranial RDD, only histopathologic and immunocytochemical examinations permit a firm diagnosis. References 1 Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990; 7: Konishi E, Ibayashi N, Yamamoto S, et al. Isolated intracranial Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). Am J Neuroradiol. 2003; 24: Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969; 87: Sharma MS, Padua MD, Jha AN. Rosai-Dorfman disease mimicking a sphenoid wing meningioma. Neurol India. 2005; 53: Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol. 2001; 5: Nespoli L, Broggini M., Binda S., et al. Malattia di Rosai-Dorfman e connettivite nella stessa paziente: continuum patogenetico? RIAIP. 2001; XV: Yetiser S, Cekin E, Tosun F, et al. Rosai-Dorfman disease associated with neurosensorial hearing loss in two siblings. Int J Pediatr Otorhinolaryngol. 2004; 68: Jabali Y, Smrcka V, Pradna J. Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol. 2005; 13: Alatassi H, Ray MB, Galandiuk S, et al. Rosai-Dorfman disease of the gastrointestinal tract: report of a case and review of the literature. Int J Surg Pathol. 2006; 14: Levine PH, Jahan N, Murari P, et al. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis. 1992; 166: Kidd DP, Revesz T, Miller NR. Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement. Neurology. 2006; 67: Wang Y, Gao X, Tang W, et al. Rosai-Dorfman disease isolated to the central nervous system: A report of six cases. Neuropathology. 2010; 30: Andriko JA, Morrison A, Colegial CH. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol. 2001; 14: Franco-Paredes C, Martin K. Extranodal Rosai-Dorfman disease involving the meninges. South Med J. 2002; 95: Favara BE, Feller AC, Pauli M, et al. Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol. 1997; 29: Antonius JI, Farid SM, Baez-Giangreco A. Steroid-responsive Rosai-Dorfman disease. Pediatr Hematol Oncol. 1996; 13: Simos M, Dimitrios P, Philip T. A new clinical entity mimicking meningioma diagnosed pathologically as Rosai-Dorfman disease. Skull Base Surg. 1998; 8: McPherson CM, Brown J, Kim AW, et al. Regression of intracranial Rosai-Dorfman disease following corticosteroid therapy. Case report. J Neurosurg. 2006; 104: Petzold A, Thom M, Powell M, et al. Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatry. 2001; 71: Sandhu FA, Schellinger D, Martuza RL. A vascular sarcoid mass mimicking a convexity meningioma. Neuroradiology. 2000; 42: Harting I, Hartmann M, Bonsanto MM, et al. Characterization of necrotic meningioma using diffusion MRI, perfusion MRI, and MR spectroscopy: case report and review of the literature. Neuroradiology. 2004; 46: Kremer S, Grand S, Rémy TC, et al. Contribution of dynamic contrast MR imaging to the differentiation between dural metastasis and meningioma. Neuroradiology. 2004; 46: Chen Y, Tiang X, Li Z, et al. Sporadic meningioangiomatosis-associated atypical meningioma mimicking parenchymal invasion of brain: a case report and review of the literature. Diagn Pathol. 2010; 5: 39. Alessia Catalucci, MD Neuroradiology Department San Salvatore Hospital Via Vetoio L Aquila Italy alessiacat@tiscali.it 608