- Email: [email protected]
Giant intracranial Rosai–Dorfman disease
Rosai–Dorfman disease 563
mechanism of NPH in SLE might be more insidious, lacking overt inflammatory changes. In the report presented, deposition of immunoglobulins and complement components suggests the involvement of the dura in SLE complicated by NPH. Deposition of immune components may reflect pan-dural involvement, including the arachnoid villi. The involvement of the arachnoid villi may affect the absorption of CSF and could become a sufficient cause of NPH. REFERENCES 1. Uhl MD, Werner BE, Romano TJ, Zidar BL. Normal pressure hydrocephalus in a patient with systemic lupus erythematosus. J Rheumatol 1990; 17: 1689–1691. 2. Nakayama-Furukawa F, Takigawa M, Iwatsuki K, Sato N, Sato H. Hydrocephalus in two female siblings with neonatal lupus erythematosus. Arch Dermatol 1994; 130: 1210–1212. 3. You HY, Wang SR. Normal pressure hydrocephalus in a patient with systemic lupus erythematosus: a case report. Zhonghua Yi Xue Za Zhi (Taipei) 1998; 61: 551–555. 4. Kitching GB, Thompson JR, Hasso AN, Hirst AE. Angiographic demonstration of lupus cerebral phlebitis with communicating hydrocephalus. Neuroradiology 1977; 14: 59–63. 5. Mortifee PR, Bebb RA, Stein H. Communicating hydrocephalus in systemic lupus erythematosus with antiphospholipid antibody syndrome. J Rheumatol 1992; 19: 1299–1302.
Giant intracranial Rosai–Dorfman disease ur Tu € re1 MD, As¸kın S¸eker1 MD, Ug 3 € € heyla Uyar Bozkurt2 MD, Cu € neyt Uneri Su 2 1 Aydın Sav MD, M. Necmettin Pamir MD
MD,
1 Department of Neurosurgery, 2Department of Pathology, 3Department of ENT, Marmara University School of Medicine, Istanbul, Turkey
Summary Rosai–Dorfman disease is a rare, non-neoplastic disease characterized by an unusual proliferation of histiocytic cells. It rarely manifests intracranially, and only 50 cases of intracranial lesions have been reported. We describe an unusual case of a huge, solid mass in the paranasal sinuses, orbits, cavernous sinuses, and suprasellar cisterns. A 29-year-old patient was admitted to our hospital with nasal obstruction and proptosis and visual loss in the right eye. A biopsy was done at another institution and the diagnosis was “pseudotumor of orbit”. A right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy was used to excise the tumor with right orbital exenteration. The histopathological diagnosis was consistent with Rosai–Dorfman disease. The patient underwent postoperative chemotherapy. Involvement of the central nervous system in Rosai–Dorfman disease is rare, but the disease’s ability to mimic other pathologies underlines its importance. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(5), 563–566 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2003.11.012
Keywords: cavernous sinus, cranio-orbitozygomatic approach, lateral rhinotomy, Rosai–Dorfman disease, sinus histiocytosis Received 20 August 2002 Accepted 14 November 2003 ur Tu €re MD, Department of Neurosurgery, Ondokuz Correspondence to: Ug Mayis University School of Medicine, Kurupelit, 55139 Samsun, Turkey. E-mail: [email protected]
ª 2003 Elsevier Ltd. All rights reserved.
INTRODUCTION Sinus histiocytosis with massive lymphadenopathy (SHML) was first described in 1969 by Rosai and Dorfman.1 It is a rare, nonneoplastic, lymphoproliferative disorder clinically characterized by massive, painless, cervical lymphadenopathy, fever, leukocytosis, weight loss, an increased erythrocyte sedimentation rate, anemia, and hypergammaglobulinemia.1–3 The hypertrophied lymph node contains large histiocytes with phagocytosis of the lymphocytes. This pattern can be called emperipolesis. SHML can also involve extranodal sites, most commonly the orbit, skin, paranasal sinuses, bone, and salivary glands.2–7 Intracranial involvement is uncommon, however, with only 50 cases reported in the literature.4–34 We describe the case of a patient with a huge mass located in the anterior fossa and extending into the suprasellar cisterns, bilateral cavernous sinuses, and the maxillary, ethmoid, and sphenoid sinuses. The patient received an erroneous diagnosis and was followed for “pseudotumor” for 4 years.
CASE STUDY A 29-year-old man was admitted to another institution with nasal obstruction and decreased visual acuity. Physical examination revealed a white vascular lesion in the right middle meatus. After a biopsy, he was given a diagnosis of “pseudotumor” and administered dexamethasone. One year later, the patient was admitted to another center with the same complaint. Another biopsy was done and the diagnosis was “inflammatory lesion”. The patient was later admitted to our institution with proptosis and total loss of vision in the right eye, and nasal obstruction. Physical examination revealed a vascular lesion in the middle nasal meatus of the right nasal cavity. Neurological examination revealed a total loss of vision, no light reflex, total ophthalmoplegia, and proptosis in the right eye. Laboratory results were: Hgb 10.7 mg/ dL, ESR 127mm/h, polyclonal hypergammaglobulinemia, RF (–), ANA (–), LE cell (–), and an inverted CD4/CD8 ratio. Magnetic resonance (MR) imaging showed a solid mass occupying the right maxillary sinus and bilateral ethmoid and sphenoid sinuses. The tumor also invaded the posterior and medial walls of the left maxillary sinus and continued through the right cavernous sinus, reaching the rostrum along the interhemispheric fissure. The lesion enhanced markedly after gadolinium administration in T1weighted MR images (Fig. 1). A computed tomography scan showed that the roof of the right orbit was destroyed and cerebral angiography demonstrated highly vascular tumor blush in the anterior fossa, right orbit, and paranasal sinuses (Fig. 2). The chest X-ray was normal. We used a right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy for exenteration of the right eye and excision of the tumor. Histopathological findings were consistent with a diagnosis of extranodal Rosai–Dorfman disease (Fig. 3). At low power, the histological features included the presence of histiocytes, lymphoid aggregates, lymphocytes, and plasma cells within the parenchyma of the involved site. Each histiocytic cell was characterized by a large, round vesicular nucleus with a delicate nuclear membrane and prominent nucleoli. Emperipolesis was a conspicuous finding of the lesion. The immunohistochemical analysis showed diffuse S-100 (Biogenex, 15E2E2, 1:500), CD 68 (Neomarkers, KP1), and alpha-1 antitrypsin (Neomarkers) protein expression by the histiocytes. The results of immunostaining with CD 1 (Neomarkers, 1CA04) were negative (Table 1). The patient had an uneventful postoperative course and postoperative MR imaging studies confirmed near total excision of the tumor (Fig. 4). After surgery, the patient’s thorax, abdomen, Journal of Clinical Neuroscience (2004) 11(5)
€ et al. 564 Ture
Fig. 1 Preoperative T1-weighted axial (a), coronal (center), and sagittal (right) MR images studies after Gd-DTPA administration. An intensely enhanced and well-defined giant mass lesion is seen in the right orbit, extending intracranially to the anterior fossa and extracranially to the paranasal sinuses and nasopharynges.
Table 1
Immunohistochemical markers used in diagnosis
CD antigen or antibody EMA Synaptophysin CD68 S-100 a-1 Antitrypsin CD1 CD30 CD20 CD45 CD3 GFAP PRL GH Fig. 2 Lateral view of the preoperative right external carotid angiography demonstrating highly vascular tumor blush in the anterior fossa, orbit, and paranasal sinuses.
Fig. 3 Characteristic histiocytes of the lesion show lymphocyte and plasma cell emperipolesis (arrow) (H & E, original magnification 400).
and pelvis were screened, but no systemic findings of the disease were present. The patient was discharged for adjunctive chemotherapy with cytotoxic agent (chlorambucil) and prednisone. Journal of Clinical Neuroscience (2004) 11(5)
Reactivity in our patient ) ) + + + ) ) + ) + ) ) )
DISCUSSION Rosai–Dorfman (Destombes–Rosai–Dorfman) disease was first described in 1969 as a systemic non-neoplastic histioproliferative disorder.1;2;12;35 SHML was initially characterized as a node-based disease process. Since that time, it has been established that SHML is a systemic disease and may affect a variety of extranodal sites.2;3;7;8;21;23 More than 400 cases have been reported in the literature.3;8;15;16 The mean age of diagnosis is 20.6 years, with a female dominance.2 The typical clinical presentation is painless, bilateral cervical lymphadenopathy, and the head and neck are two of the most common extranodal areas affected.2–7 The disease also has a predilection for the nasal cavity and paranasal sinuses.7 Less frequently, the axillary, inguinal, and mediastinal nodes are affected. An unusual presentation is extranodal involvement without lymphadenopathy.24;29 Fever and weight loss are seen in patients with a cervical mass. Laboratory findings are generally non-specific; an increased erythrocyte sedimentation rate and mild anemia (normochromic, normocytic) are generally seen. Other serum immunological markers, such as rheumatoid factor and the antinuclear antibody, may be positive. Extranodal involvements of SHML occur in 43% of patients.2 The extranodal site is included in reports in the literature because ª 2003 Elsevier Ltd. All rights reserved.
Rosai–Dorfman disease 565
Fig. 4 tumor.
Postoperative T1-weighted axial (a), coronal (b), and sagittal (c) MR imaging studies after Gd-DTPA administration showing near total excision of the
of either the patient’s clinical presentation or an unusual location of the disease, such as the central nervous system (CNS). To our knowledge, only 50 cases with intracranial involvement have been reported.8–34 The majority of previously reported intracanial cases of SMHL lesions displayed dural attachment while some of them were associated with adjacent bone erosion. Imaging studies show an enhancing meningeal-based mass with a variable amount of edema surrounding the lesion and they tend to mimic meningiomas.8;11;16;17;32–34 These lesions are gadolonium enhancing on the T1-weighted MR images and suggestive of intracranial neoplasms such as meningioma. However, low signal intensity on T2-weighted MR imaging is a very unlikely characteristic of meningioma.8;28;32 Most commonly, the lesion is solitary, but it can be multiple.17;19 The aetiology of SHML is still uncertain.8;11;16 The possibility of SHML representing the expression of an immune dysfunction was advanced, but no specific immunological abnormalities were detected.36 Although, an infection was suggested as an underlying cause, a definitive agent has never been isolated.1;2;7;8;16 Levine et al.37 suggested that human herpes virus 6, and to a lesser extent Epstein-Barr virus may be involved in the aetiology of SHML. Serological evidence of Epstein-Barr virus is found in approximately 50% of patients with SHML. The increased serological titers may be the result of a non-specific host immune response and not the cause of SHML.11;16;31 Our patient’s case highlights some noteworthy characteristics. Extreme involvement of extranodal sites, such as the orbit, paranasal sinuses, and intracranial cavity, was observed but there was no nodal disease. The disease was progressive. The patient underwent the cranio-orbitozygomatic approach combined with rhinotomy, and tumor excision was done with exenteration of the right eye. The gross appearance of the lesion was nodular and exophytic with a white to yellow appearance. A characteristic finding in patients with SHML is histiocytes filled with intact lymphocytes, plasma cells, erythrocytes, and polymorphonuclear leukocytes – a phenomenon known as emperipolesis. In histiocytes of SHML that show emperipolesis, the presence of plasma cells helps differentiate between benign and malignant disorders, in which phagocytosis of cells is a prominent feature. The lesions included in the differential diagnosis are Langerhans cell histiocytosis and pseudotumor. In Langerhans ª 2003 Elsevier Ltd. All rights reserved.
cell histiocytosis, the nuclei are smaller and more irregular than those of SHML cells, and emperipolesis is not a feature.3 Langerhans cells also express CD 1 antibodies, but in our case, CD 1 reactivity was not seen. SHML may mimic the appearance of a fibro-inflammatory lesion.7 In that disease, however, the histiocytes do not show emperipolesis and S-100 protein reactivity is not seen. In our patient, a pituitary adenoma was considered in the differential diagnosis because of the location of the lesion. It was ruled out, however, because of synaptophysin immunonegativity. The natural history and treatment of these lesions are still controversial. Treatment varied and included surgical excision with or without adjuvant therapy (chemotherapy, radiotherapy) or steroids.2–4;6–8;13–17;20;28;32;38–40 Some authors advocated only radical surgery for intracranial SHML lesions.13;16 However, relapsing of intracranial SHML following surgical treatment becomes a serious problem. Intracranial tumor recurrence has been reported 14% of patients. In a further 25% of patients with CNS involvement no follow up information could be obtained from the literature.28 In patients where the complete surgical removal of the lesion was impossible adjuvant therapy such as radiotherapy and/or chemotherapy with cytotoxic agents can be applied.2;3;6–8;28;32;40 Recently, stereotactic radiosurgery is advocated for the treatment of the residual intracranial SHML lesion.14 The prognosis of SHML is variable.3;7;8;15–17;28 Most patients experience an indolent course that is characterized by exacerbations and remissions. However, SHML may be self-limiting or progressive. Mortality has been reported at approximately 7%.3;17 Unfavorable prognostic factors include disseminated nodal disease, involvement of the extranodal organ system, and deficiencies in the patient’s hematologic or immunologic status.2;3;17 After 2 years of follow-up, our patient has had no evidence of progression, and the disease seems to be in remission. Currently, SHML is studied as a histiocytic process. The extranodal involvement of SHML is well documented, but its diagnosis may be overlooked. The physician’s familiarity with its clinical manifestations in extranodal locations and the diagnostic histopathologic features should preclude any confusion with other disease entities. Although SHML has a benign histologic appearance, extranodal involvement may have an aggressive course and should be treated with radical surgery and adjuvant chemotherapy.2 Journal of Clinical Neuroscience (2004) 11(5)
566 Ohkoshi et al.
REFERENCES 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognised benign clinicopathological entity. Arch Pathol 1969; 87: 63–70. 2. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymph adenopathy (Rosai–Dorfman disease): review of the entity. Semin Diagn Pathol 1990; 7: 19–73. 3. Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF. Tumors of the lymph nodes and spleen. In: Rosai J (ed). Atlas of Tumor Pathology, third series, Fascicle 14. Armed Forces Institute of Pathology, Washington, DC; 1995: 349–363. 4. Asai A, Matsutani M, Kohno T, Fujimaki T, Tanaka H, Kawaguchi K et al. Leptomeningeal and orbital benign lymphophagocytic histiocytosis. J Neurosurg 1988; 69: 610–612. 5. Katz DS, Poe LB, Corona Jr RJ. Sinus histiocytosis with massive lymphadenopathy: a case of simultaneous upper respiratory tract and CNS disease without lymphadenopathy. AJNR 1993; 14: 219–222. 6. Resnick DK, Johnson BL, Lovely TJ. Rosai–Dorfman disease presenting with multiple orbital and cranial masses. Acta Neuropathol (Berl) 1996; 91: 554–557. 7. Wenig BM, Abbondanzo SL, Childers EL, Kapadia SB, Heffner DR. Extra nodal sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) of the head and neck. Hum Pathol 1993; 24: 483–492. 8. Andriko JAW, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai–Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 2001; 14: 172–178. 9. Bhattacharjee MB, Wroe SJ, Harding BN, Powell M. Sinus histiocytosis with massive lymphadenopathy-isolated suprasellar involvement. J Neurol Neurosurg Psychiatr 1992; 55: 156–158. 10. Clark WC, Berry III AD. Extranodal sinus histiocytosis with massive lymphadenopathy: isolated central nervous system involvement mimicking meningioma. South Med J 1996; 89: 621–623. 11. Deodhare SS, Ang LC, Bibao JM. Isolated intracranial involvement in Rosai– Dorfman disease: a report of two cases and review of the literature. Arch Pathol Lab Med 1998; 122: 161–165. 12. Foucar E, Rosai J, Doerman RF et al. The neurological manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology 1982; 32: 365–372. 13. Gaetani P, Tancioni F, Di Rocco M et al. Isolated cerebellar involvement in Rosai–Dorfman disease: case report. Neurosurgery 2000; 46: 479–481. 14. Hadjipanayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D. Intracranial Rosai–Dorfman disease treated with microsurgical resection and stereotactic radiosurgery: case report. J Neurosurg 2003; 98: 165–168. 15. Huang HY, Huang CC, Lui CC, Chen HJ, Chen WJ. Isolated intracranial Rosai–Dorfman disease: case report and literature review. Pathol Int 1998; 48: 396–402. 16. Juric G, Jakic-Razumovic J, Rotim K, Zarkovic K. Extranodal sinus histiocytosis (Rosai–Dorfman disease) of brain parenchyma. Acta Neurochir 2003; 145: 145–149. 17. Kattner KA, Stroink AR, Roth TC, Lee JM. Rosai–Dorfman disease mimicking parasagittal meningioma: case presentation and review of literature. Surg Neurol 2000; 53: 452–457. 18. Kelly WF, Bradey N, Scoones D. Rosai–Dorfman disease presenting as a pituitary tumour. Clin Endocrinol (Oxf) 1999; 5050: 133–137. 19. Kim M, Provias J, Berstein M. Rosai–Dorfman disease mimicking multiple meningioma: case report. Neurosurgery 1995; 36: 1185–1187. 20. Kitai R, Lena J, Hirano A, Ido K, Sato K, Kubota T. Meningeal Rosai–Dorfman disease: report of three cases and literature review. Brain Tumor Pathol 2001; 18: 49–54. 21. Lopez P, Estes ML. Immunohistochemical characterization of the histiocytes in sinus histiocytosis with massive lymphadenopathy: analysis of an extranodal case. Hum Pathol 1989; 20: 711–715. 22. Losi L, Calbucci F, Mancini AM. Intracranial location of a case of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) mimicking meningioma. Pathologica 1998; 90: 403–407. 23. Mir R, Aftalion B, Kahn LB. Sinus histiocytosis with massive lymphadenopathy an unusual extranodal manifestations. Arch Pathol Lab Med 1985; 109: 867–870. 24. Mirra SS, Tindall SC, Check IJ et al. Inflammatory meningeal masses of unexplained origin. An ultrastructural and immunological study. J Neuropathol Exp Neurol 1983; 42: 453–468. 25. Morandi X, Godey B, Riffaud L, Heresbach N, Brassier G. Isolated Rosai– Dorfman disease of the fourth ventricle. Case illustration. J Neurosurg 2000; 92: 890. 26. Natarajan S, Post KD, Strauchen J et al. Primary intracerebral Rosai–Dorfman disease: a case report. J Neurooncol 2000; 47: 73–77. 27. Panicker NK, Sabhikhi AK, Rai R. Rosai–Dorfman disease presenting as a meningioma. Indian J Cancer 1996; 33: 192–194.
Journal of Clinical Neuroscience (2004) 11(5)
28. Petzold A, Thom M, Powell M, Plant GT. Relapsing intracranial Rosai–Dorfman disease. J Neurol Neurosurg Psychiatr 2001; 71: 538–541. 29. Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN. Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. J Neurosurg 1993; 79: 769–773. 30. Song SK, Schwartz IS, Strauchen JA et al. Meningeal nodules with features of extranodal sinus histiocytosis with massive lymphadenopathy. Am J Surg Pathol 1989; 13: 406–412. 31. Trudel M. Dural involvement in sinus histiocytosis with massive lympadenopathy. Case report. J Neurosurg 1984; 60: 850–852. 32. Udono H, Fukuyama K, Okamoto H, Tabuchi K. Rosai–Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg 1999; 91: 335–339. 33. Woodcock Jr RJ, Mandell JW, Lipper MH. Sinus histiocytosis (Rosai–Dorfman disease) of the suprasellar region: MR imaging findings – a case report. Radiology 1999; 213: 808–810. 34. Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai–Dorfman disease with literature review. Ann Diagn Pathol 2001; 5: 96–102. 35. Bernard F, Sarran N, Serre I, Baldet P, Callamand P, Margueritte G et al. Sinus histiocytosis (Destombes-Rosai–Dorfman disease) revealed by extranodal spinal involvement. Arch Pediatr 1999; 6: 173–177. 36. Maennle DL, Grierson HL, Gnarra DG, Weisenburger DD. Sinus histiocytosis with massive lymphadenopathy: a spectrum of disease associated with immune dysfunction. Pediatr Pathol 1991; 11: 399–412. 37. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). J Infect Dis 1992; 166: 399–412. 38. Komp DM. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). Semin Diagn Pathol 1990; 7: 83–86. 39. Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): report of a case and literature review. Am J Hematol 2002; 69: 67–71. 40. Suarez CR, Zeller WP, Silberman S, Rust G, Messmore H. Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy. Am J Pediatr Hematol Oncol 1983; 5: 235–241.
Acute flank pain, an unusual first symptom of a spinal arteriovenous malformation N. Ohkoshi
MD PHD,
A. Ishii
MD PHD,
S. Shoji MD PHD
Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Japan
Summary This case report describes a 46-year-old man whose first symptom was an attack of acute flank pain, followed by the gradual onset neurological symptoms. We demonstrated a small nidus and serpentine signal-void area in the spinal cord by MRI and diagnosed a glomus type of spinal arteriovenous malformation (AVM). Flank pain is a rare initial finding in an adult with spinal AVM. In cases with acute flank pain, neurologists should consider spinal AVM as a differential diagnosis. ª 2004 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(5), 566–567 0967-5868/$ - see front matter ª 2004 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2003.04.008
Keywords: spinal arteriovenous malformation, acute flank pain, intradural AVM, glomus type, subarachnoid hemorrhage Received 20 January 2003 Accepted 27 April 2003 Correspondence to: N. Ohkoshi, Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodar, Tsukuba, Ibaraki 305-8575, Japan. Tel.: +81-29-853-3224; Fax: +81-29-853-3224; E-mail: [email protected]
ª 2004 Elsevier Ltd. All rights reserved.
mechanism of NPH in SLE might be more insidious, lacking overt inflammatory changes. In the report presented, deposition of immunoglobulins and complement components suggests the involvement of the dura in SLE complicated by NPH. Deposition of immune components may reflect pan-dural involvement, including the arachnoid villi. The involvement of the arachnoid villi may affect the absorption of CSF and could become a sufficient cause of NPH. REFERENCES 1. Uhl MD, Werner BE, Romano TJ, Zidar BL. Normal pressure hydrocephalus in a patient with systemic lupus erythematosus. J Rheumatol 1990; 17: 1689–1691. 2. Nakayama-Furukawa F, Takigawa M, Iwatsuki K, Sato N, Sato H. Hydrocephalus in two female siblings with neonatal lupus erythematosus. Arch Dermatol 1994; 130: 1210–1212. 3. You HY, Wang SR. Normal pressure hydrocephalus in a patient with systemic lupus erythematosus: a case report. Zhonghua Yi Xue Za Zhi (Taipei) 1998; 61: 551–555. 4. Kitching GB, Thompson JR, Hasso AN, Hirst AE. Angiographic demonstration of lupus cerebral phlebitis with communicating hydrocephalus. Neuroradiology 1977; 14: 59–63. 5. Mortifee PR, Bebb RA, Stein H. Communicating hydrocephalus in systemic lupus erythematosus with antiphospholipid antibody syndrome. J Rheumatol 1992; 19: 1299–1302.
Giant intracranial Rosai–Dorfman disease ur Tu € re1 MD, As¸kın S¸eker1 MD, Ug 3 € € heyla Uyar Bozkurt2 MD, Cu € neyt Uneri Su 2 1 Aydın Sav MD, M. Necmettin Pamir MD
MD,
1 Department of Neurosurgery, 2Department of Pathology, 3Department of ENT, Marmara University School of Medicine, Istanbul, Turkey
Summary Rosai–Dorfman disease is a rare, non-neoplastic disease characterized by an unusual proliferation of histiocytic cells. It rarely manifests intracranially, and only 50 cases of intracranial lesions have been reported. We describe an unusual case of a huge, solid mass in the paranasal sinuses, orbits, cavernous sinuses, and suprasellar cisterns. A 29-year-old patient was admitted to our hospital with nasal obstruction and proptosis and visual loss in the right eye. A biopsy was done at another institution and the diagnosis was “pseudotumor of orbit”. A right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy was used to excise the tumor with right orbital exenteration. The histopathological diagnosis was consistent with Rosai–Dorfman disease. The patient underwent postoperative chemotherapy. Involvement of the central nervous system in Rosai–Dorfman disease is rare, but the disease’s ability to mimic other pathologies underlines its importance. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(5), 563–566 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2003.11.012
Keywords: cavernous sinus, cranio-orbitozygomatic approach, lateral rhinotomy, Rosai–Dorfman disease, sinus histiocytosis Received 20 August 2002 Accepted 14 November 2003 ur Tu €re MD, Department of Neurosurgery, Ondokuz Correspondence to: Ug Mayis University School of Medicine, Kurupelit, 55139 Samsun, Turkey. E-mail: [email protected]
ª 2003 Elsevier Ltd. All rights reserved.
INTRODUCTION Sinus histiocytosis with massive lymphadenopathy (SHML) was first described in 1969 by Rosai and Dorfman.1 It is a rare, nonneoplastic, lymphoproliferative disorder clinically characterized by massive, painless, cervical lymphadenopathy, fever, leukocytosis, weight loss, an increased erythrocyte sedimentation rate, anemia, and hypergammaglobulinemia.1–3 The hypertrophied lymph node contains large histiocytes with phagocytosis of the lymphocytes. This pattern can be called emperipolesis. SHML can also involve extranodal sites, most commonly the orbit, skin, paranasal sinuses, bone, and salivary glands.2–7 Intracranial involvement is uncommon, however, with only 50 cases reported in the literature.4–34 We describe the case of a patient with a huge mass located in the anterior fossa and extending into the suprasellar cisterns, bilateral cavernous sinuses, and the maxillary, ethmoid, and sphenoid sinuses. The patient received an erroneous diagnosis and was followed for “pseudotumor” for 4 years.
CASE STUDY A 29-year-old man was admitted to another institution with nasal obstruction and decreased visual acuity. Physical examination revealed a white vascular lesion in the right middle meatus. After a biopsy, he was given a diagnosis of “pseudotumor” and administered dexamethasone. One year later, the patient was admitted to another center with the same complaint. Another biopsy was done and the diagnosis was “inflammatory lesion”. The patient was later admitted to our institution with proptosis and total loss of vision in the right eye, and nasal obstruction. Physical examination revealed a vascular lesion in the middle nasal meatus of the right nasal cavity. Neurological examination revealed a total loss of vision, no light reflex, total ophthalmoplegia, and proptosis in the right eye. Laboratory results were: Hgb 10.7 mg/ dL, ESR 127mm/h, polyclonal hypergammaglobulinemia, RF (–), ANA (–), LE cell (–), and an inverted CD4/CD8 ratio. Magnetic resonance (MR) imaging showed a solid mass occupying the right maxillary sinus and bilateral ethmoid and sphenoid sinuses. The tumor also invaded the posterior and medial walls of the left maxillary sinus and continued through the right cavernous sinus, reaching the rostrum along the interhemispheric fissure. The lesion enhanced markedly after gadolinium administration in T1weighted MR images (Fig. 1). A computed tomography scan showed that the roof of the right orbit was destroyed and cerebral angiography demonstrated highly vascular tumor blush in the anterior fossa, right orbit, and paranasal sinuses (Fig. 2). The chest X-ray was normal. We used a right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy for exenteration of the right eye and excision of the tumor. Histopathological findings were consistent with a diagnosis of extranodal Rosai–Dorfman disease (Fig. 3). At low power, the histological features included the presence of histiocytes, lymphoid aggregates, lymphocytes, and plasma cells within the parenchyma of the involved site. Each histiocytic cell was characterized by a large, round vesicular nucleus with a delicate nuclear membrane and prominent nucleoli. Emperipolesis was a conspicuous finding of the lesion. The immunohistochemical analysis showed diffuse S-100 (Biogenex, 15E2E2, 1:500), CD 68 (Neomarkers, KP1), and alpha-1 antitrypsin (Neomarkers) protein expression by the histiocytes. The results of immunostaining with CD 1 (Neomarkers, 1CA04) were negative (Table 1). The patient had an uneventful postoperative course and postoperative MR imaging studies confirmed near total excision of the tumor (Fig. 4). After surgery, the patient’s thorax, abdomen, Journal of Clinical Neuroscience (2004) 11(5)
€ et al. 564 Ture
Fig. 1 Preoperative T1-weighted axial (a), coronal (center), and sagittal (right) MR images studies after Gd-DTPA administration. An intensely enhanced and well-defined giant mass lesion is seen in the right orbit, extending intracranially to the anterior fossa and extracranially to the paranasal sinuses and nasopharynges.
Table 1
Immunohistochemical markers used in diagnosis
CD antigen or antibody EMA Synaptophysin CD68 S-100 a-1 Antitrypsin CD1 CD30 CD20 CD45 CD3 GFAP PRL GH Fig. 2 Lateral view of the preoperative right external carotid angiography demonstrating highly vascular tumor blush in the anterior fossa, orbit, and paranasal sinuses.
Fig. 3 Characteristic histiocytes of the lesion show lymphocyte and plasma cell emperipolesis (arrow) (H & E, original magnification 400).
and pelvis were screened, but no systemic findings of the disease were present. The patient was discharged for adjunctive chemotherapy with cytotoxic agent (chlorambucil) and prednisone. Journal of Clinical Neuroscience (2004) 11(5)
Reactivity in our patient ) ) + + + ) ) + ) + ) ) )
DISCUSSION Rosai–Dorfman (Destombes–Rosai–Dorfman) disease was first described in 1969 as a systemic non-neoplastic histioproliferative disorder.1;2;12;35 SHML was initially characterized as a node-based disease process. Since that time, it has been established that SHML is a systemic disease and may affect a variety of extranodal sites.2;3;7;8;21;23 More than 400 cases have been reported in the literature.3;8;15;16 The mean age of diagnosis is 20.6 years, with a female dominance.2 The typical clinical presentation is painless, bilateral cervical lymphadenopathy, and the head and neck are two of the most common extranodal areas affected.2–7 The disease also has a predilection for the nasal cavity and paranasal sinuses.7 Less frequently, the axillary, inguinal, and mediastinal nodes are affected. An unusual presentation is extranodal involvement without lymphadenopathy.24;29 Fever and weight loss are seen in patients with a cervical mass. Laboratory findings are generally non-specific; an increased erythrocyte sedimentation rate and mild anemia (normochromic, normocytic) are generally seen. Other serum immunological markers, such as rheumatoid factor and the antinuclear antibody, may be positive. Extranodal involvements of SHML occur in 43% of patients.2 The extranodal site is included in reports in the literature because ª 2003 Elsevier Ltd. All rights reserved.
Rosai–Dorfman disease 565
Fig. 4 tumor.
Postoperative T1-weighted axial (a), coronal (b), and sagittal (c) MR imaging studies after Gd-DTPA administration showing near total excision of the
of either the patient’s clinical presentation or an unusual location of the disease, such as the central nervous system (CNS). To our knowledge, only 50 cases with intracranial involvement have been reported.8–34 The majority of previously reported intracanial cases of SMHL lesions displayed dural attachment while some of them were associated with adjacent bone erosion. Imaging studies show an enhancing meningeal-based mass with a variable amount of edema surrounding the lesion and they tend to mimic meningiomas.8;11;16;17;32–34 These lesions are gadolonium enhancing on the T1-weighted MR images and suggestive of intracranial neoplasms such as meningioma. However, low signal intensity on T2-weighted MR imaging is a very unlikely characteristic of meningioma.8;28;32 Most commonly, the lesion is solitary, but it can be multiple.17;19 The aetiology of SHML is still uncertain.8;11;16 The possibility of SHML representing the expression of an immune dysfunction was advanced, but no specific immunological abnormalities were detected.36 Although, an infection was suggested as an underlying cause, a definitive agent has never been isolated.1;2;7;8;16 Levine et al.37 suggested that human herpes virus 6, and to a lesser extent Epstein-Barr virus may be involved in the aetiology of SHML. Serological evidence of Epstein-Barr virus is found in approximately 50% of patients with SHML. The increased serological titers may be the result of a non-specific host immune response and not the cause of SHML.11;16;31 Our patient’s case highlights some noteworthy characteristics. Extreme involvement of extranodal sites, such as the orbit, paranasal sinuses, and intracranial cavity, was observed but there was no nodal disease. The disease was progressive. The patient underwent the cranio-orbitozygomatic approach combined with rhinotomy, and tumor excision was done with exenteration of the right eye. The gross appearance of the lesion was nodular and exophytic with a white to yellow appearance. A characteristic finding in patients with SHML is histiocytes filled with intact lymphocytes, plasma cells, erythrocytes, and polymorphonuclear leukocytes – a phenomenon known as emperipolesis. In histiocytes of SHML that show emperipolesis, the presence of plasma cells helps differentiate between benign and malignant disorders, in which phagocytosis of cells is a prominent feature. The lesions included in the differential diagnosis are Langerhans cell histiocytosis and pseudotumor. In Langerhans ª 2003 Elsevier Ltd. All rights reserved.
cell histiocytosis, the nuclei are smaller and more irregular than those of SHML cells, and emperipolesis is not a feature.3 Langerhans cells also express CD 1 antibodies, but in our case, CD 1 reactivity was not seen. SHML may mimic the appearance of a fibro-inflammatory lesion.7 In that disease, however, the histiocytes do not show emperipolesis and S-100 protein reactivity is not seen. In our patient, a pituitary adenoma was considered in the differential diagnosis because of the location of the lesion. It was ruled out, however, because of synaptophysin immunonegativity. The natural history and treatment of these lesions are still controversial. Treatment varied and included surgical excision with or without adjuvant therapy (chemotherapy, radiotherapy) or steroids.2–4;6–8;13–17;20;28;32;38–40 Some authors advocated only radical surgery for intracranial SHML lesions.13;16 However, relapsing of intracranial SHML following surgical treatment becomes a serious problem. Intracranial tumor recurrence has been reported 14% of patients. In a further 25% of patients with CNS involvement no follow up information could be obtained from the literature.28 In patients where the complete surgical removal of the lesion was impossible adjuvant therapy such as radiotherapy and/or chemotherapy with cytotoxic agents can be applied.2;3;6–8;28;32;40 Recently, stereotactic radiosurgery is advocated for the treatment of the residual intracranial SHML lesion.14 The prognosis of SHML is variable.3;7;8;15–17;28 Most patients experience an indolent course that is characterized by exacerbations and remissions. However, SHML may be self-limiting or progressive. Mortality has been reported at approximately 7%.3;17 Unfavorable prognostic factors include disseminated nodal disease, involvement of the extranodal organ system, and deficiencies in the patient’s hematologic or immunologic status.2;3;17 After 2 years of follow-up, our patient has had no evidence of progression, and the disease seems to be in remission. Currently, SHML is studied as a histiocytic process. The extranodal involvement of SHML is well documented, but its diagnosis may be overlooked. The physician’s familiarity with its clinical manifestations in extranodal locations and the diagnostic histopathologic features should preclude any confusion with other disease entities. Although SHML has a benign histologic appearance, extranodal involvement may have an aggressive course and should be treated with radical surgery and adjuvant chemotherapy.2 Journal of Clinical Neuroscience (2004) 11(5)
566 Ohkoshi et al.
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Acute flank pain, an unusual first symptom of a spinal arteriovenous malformation N. Ohkoshi
MD PHD,
A. Ishii
MD PHD,
S. Shoji MD PHD
Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Japan
Summary This case report describes a 46-year-old man whose first symptom was an attack of acute flank pain, followed by the gradual onset neurological symptoms. We demonstrated a small nidus and serpentine signal-void area in the spinal cord by MRI and diagnosed a glomus type of spinal arteriovenous malformation (AVM). Flank pain is a rare initial finding in an adult with spinal AVM. In cases with acute flank pain, neurologists should consider spinal AVM as a differential diagnosis. ª 2004 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(5), 566–567 0967-5868/$ - see front matter ª 2004 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2003.04.008
Keywords: spinal arteriovenous malformation, acute flank pain, intradural AVM, glomus type, subarachnoid hemorrhage Received 20 January 2003 Accepted 27 April 2003 Correspondence to: N. Ohkoshi, Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodar, Tsukuba, Ibaraki 305-8575, Japan. Tel.: +81-29-853-3224; Fax: +81-29-853-3224; E-mail: [email protected]
ª 2004 Elsevier Ltd. All rights reserved.