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Bing-Neel syndrome: A cerebral richter syndrome? Sir, Bing-Neel syndrome (BNS) is a rare neurologic complication of Waldenström macroglobulinemia (WM) involving an association with WM and central nervous system infiltration by neoplastic cells.[1,2] Classification of BNS was historically defined into two subtypes: Focal or diffuse.[3] Here we present two cases of patients with focal forms of BNS and histology of diffuse large-B cells lymphoma (DLBCL). Figure 4: Post-op axial contrast CT brain confirming total excision
immediate sub-occipital retro-mastoid approach and en bloc resection of the 5 × 4.5 cm CPA hemangioblastoma was safe with a good outcome.
Bijesh Ravindran Nair, Vivek Joseph, Geeta Chacko1, Shyamkumar Nidugala Keshava2 Departments of Neurological Sciences, 1Pathology, Section of Neuropathology and 2Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India E-mail: [email protected]
References 1. 2. 3. 4.
5. 6. 7.
Hussein MR. Central nervous system capillary haemangioblastoma: The pathologist’s viewpoint. Int J Exp Pathol 2007;88:311-24. Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A. Hemangioblastoma of the cerebellopontine angle. Arch Otolaryngol Head Neck Surg 2010;136:734-8. Dow GR, Sim DW, O’Sullivan MG. Excision of large solid haemangioblastomas of the cerebellopontine angle by a skull base approach. Br J Neurosurg 2002;16:168-71. Kamitani H, Hirano N, Takigawa H, Yokota M, Miyata H, Ohama E, et al. Attenuation of vascularity by preoperative radiosurgery facilitates total removal of a hypervascular hemangioblastoma at the cerebello-pontine angle: Case report. Surg Neurol 2004;62:238-244. Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg (1979) 1980;88:555-9. Rachinger J, Buslei R, Prell J, Strauss C. Solid haemangioblastomas of the CNS: A review of 17 consecutive cases. Neurosurg Rev 2009;32:37-48. Asthagiri AR, Mehta GU, Zach L, Li X, Butman JA, Camphausen KA, et al. Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease. Neuro Oncol 2010;12:80-6.
Case 1: In 2003, a 72-year-old woman presented with hyperproteinemia and electrophoresis monoclonal immunoglobulin M (IgM) peak of 3.97 g/l. Bone marrow biopsy (BMB) demonstrated infiltration by lymphoplasmacytic cells. It was decided to follow-up her. In July 2006, she presented with progressive cognitive disorders. In July 2007, brain magnetic resonance imaging (MRI) showed a left occipital lesion [Figure 1]. Biopsy of the lesion confirmed the CD20+ DLBCL diagnosis. Cerebrospinal fluid (CSF) protein level was 0.33 g/dL without abnormal cells. IgM peak was at 1.80 g/L. Systemic computed tomography (CT) scan was normal. Patient was treated with four cycles of high-dose methotrexate (MTX), temozolomide and prednisone and achieved clinical and radiologic complete remission (CR). In August 2009, she presented with rapid cognitive status deterioration. MRI showed tumor progression. The patient died 2 years after DLBCL diagnosis due to recurrent lymphoma. Case 2: In January 1997, a 73-year-old woman presented with asthenia and electrophoresis monoclonal IgM peak of 28 g/L. BMB confirmed WM diagnosis. She received eight cycles of cyclophosphamide,
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.132450
Received: 19-01-2014 Review completed: 20-01-2014 Accepted: 06-04-2014 Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
Figure 1: Brain magnetic resonance imaging of the first case: Homogeneous lesion located in occipital horn of lateral right ventricle
229
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doxorubicin, vincristine, and prednisolone regimen. In November 1997, first relapse was treated with six cycles of chlorambucil. In May 2000, second relapse was treated with six cycles of fludarabine. In April 2003, she presented with confusion and memory disturbance, left hemiparesis and bi-temporal hemianopsia. IgM peak was at 4.9 g/L. Brain MRI showed a right frontal lesion. 1 month later, biopsy of the lesion showed DLBC. CSF analysis showed 2.35 g/L of protein with abnormal lymphoma cells. Systemic CT scan was unremarkable. The patient received high-dose MTX, lomustine, procarbazine and prednisone. 1 month later, a brain CT scan showed a progressive disease in context of neurological deterioration. The patient died 3 months after DLBCL diagnosis due to brain lymphoma. Waldenström macroglobulinemia is characterized by lymphoplasmacytic bone marrow infiltration with IgM monoclonal gammapathy. Neurological manifestations of WM are peripheral neuropathy, myelopathy, ischemic, or hemorrhagic cerebral stroke and cerebral tumoral infiltration, called BNS.[2] It is interesting to note that both our patients presented BNS independently of WM status. Two historical forms of BNS were described:[3] the most common characterized by diffuse cerebral infiltrations of WM cells;[4] the second “tumoral form” involves the occurrence of tumor mass as analyzed on neuro-imaging. Here we propose a new histological classification of BNS: A first infiltrative form by lymphoplasmocytoid cells and a second tumoral-DLBCL form. WM is a low-grade lymphoma, which is known to be at risk for developing high-grade lymphoma in rare cases (Richter syndrome).[5] Moreover, two reported BNS histologies[3,6] revealed the association of both tumoral populations’ cells, suggesting a cerebral transformation of WM. Therefore, BNS in its “tumoral form” could be assimilated to a Richter syndrome, a new BNS entity. Therapy of BNS appeared to be extremely variable, more related to WM treatment.[7,8] However, these therapies are not recommended for primary cerebral lymphoma for which current treatment is high-dose MTX.[5,9] Our two patients received high-dose MTX and one of them achieved a prolonged CR. Bing-Neel syndrome remains a rare complication of WM. It can be classified into two categories: infiltration by lymphoplasmacytic cells and cerebral DLBCL transformation. The latter form can be considered as a Richter transformation. There is no consensual treatment for BNS, but high-dose MTX-based regimen could represent an interesting option.
Emeline Tabouret1,2, Diane Coso2, Mona Matta1, Maryline Barrié1, Reda Bouabdallah2, Olivier Chinot1 230
1
Departement of Neuro-Oncology, APHM, Timone Hospital, 13005, Marseille, 2Departement of Hematology, Paoli Calmettes Institute, 13273, cedex9, Marseille, France E-mail: [email protected]
References 1. 2. 3.
4. 5.
6. 7.
8.
9.
Bing J, Neel AV. Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection basis. Acta Med Scan 1936;88:492-506. Monteiro PL, Wildi E. Waldenstrom’s macroglobulinemia with lymphorproliferative changes in the central nervous system. 4 cases. Schweiz Arch Neurol Neurochir Psychiatr 1975;116:59-82. Imai F, Fujisawa K, Kiya N, Ninomiya T, Ogura Y, Mizoguchi Y, et al. Intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstrom’s macroglobulinemia: Case report. Neurol Med Chir (Tokyo) 1995;35:575-9. Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH. Bing-Neel Syndrome revisited. Clin Lymphoma Myeloma 2009;9:104-6. Lin P, Mansoor A, Bueso-Ramos C, Hao S, Lai R, Medeiros LJ. Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Clinicopathologic features of 12 cases. Am J Clin Pathol 2003;120:246-53. Garderet L, Baudel JL, Cervera P, Azizi L, Maury E, Guidet B, et al. ‘Indolent’ Waldenstrom’s macroglobulinemia and a cerebrospinal fluid protein level of 16 g/L. Eur J Haematol 2006;77:80-2. Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG. Bing-Neel syndrome: A case report and systematic review of clinical manifestations, diagnosis, and treatment options. Clin Lymphoma Myeloma 2009;9:462-6. Dimopoulos MA, Gertz MA, Kastritis E, Garcia-Sanz R, Kimby EK, Leblond V, et al. Update on treatment recommendations from the Fourth International Workshop on Waldenstrom’s Macroglobulinemia. J Clin Oncol 2009;27:120-6. Ferreri AJ, Reni M, Foppoli M, Martelli M, Pangalis GA, Frezzato M, et al. High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet 2009;374:1512-20. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.132451
Received: 24-11-2013 Review completed: 01-01-2014 Accepted: 13-04-2014
Primary ventral foramen magnum meningeal melanocytoma Sir, Primary meningeal melanocytomas are intriguing because of their rarity and unusual radiological appearance. [1] They are usually located around Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Bing-Neel syndrome: A cerebral richter syndrome? Sir, Bing-Neel syndrome (BNS) is a rare neurologic complication of Waldenström macroglobulinemia (WM) involving an association with WM and central nervous system infiltration by neoplastic cells.[1,2] Classification of BNS was historically defined into two subtypes: Focal or diffuse.[3] Here we present two cases of patients with focal forms of BNS and histology of diffuse large-B cells lymphoma (DLBCL). Figure 4: Post-op axial contrast CT brain confirming total excision
immediate sub-occipital retro-mastoid approach and en bloc resection of the 5 × 4.5 cm CPA hemangioblastoma was safe with a good outcome.
Bijesh Ravindran Nair, Vivek Joseph, Geeta Chacko1, Shyamkumar Nidugala Keshava2 Departments of Neurological Sciences, 1Pathology, Section of Neuropathology and 2Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India E-mail: [email protected]
References 1. 2. 3. 4.
5. 6. 7.
Hussein MR. Central nervous system capillary haemangioblastoma: The pathologist’s viewpoint. Int J Exp Pathol 2007;88:311-24. Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A. Hemangioblastoma of the cerebellopontine angle. Arch Otolaryngol Head Neck Surg 2010;136:734-8. Dow GR, Sim DW, O’Sullivan MG. Excision of large solid haemangioblastomas of the cerebellopontine angle by a skull base approach. Br J Neurosurg 2002;16:168-71. Kamitani H, Hirano N, Takigawa H, Yokota M, Miyata H, Ohama E, et al. Attenuation of vascularity by preoperative radiosurgery facilitates total removal of a hypervascular hemangioblastoma at the cerebello-pontine angle: Case report. Surg Neurol 2004;62:238-244. Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg (1979) 1980;88:555-9. Rachinger J, Buslei R, Prell J, Strauss C. Solid haemangioblastomas of the CNS: A review of 17 consecutive cases. Neurosurg Rev 2009;32:37-48. Asthagiri AR, Mehta GU, Zach L, Li X, Butman JA, Camphausen KA, et al. Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease. Neuro Oncol 2010;12:80-6.
Case 1: In 2003, a 72-year-old woman presented with hyperproteinemia and electrophoresis monoclonal immunoglobulin M (IgM) peak of 3.97 g/l. Bone marrow biopsy (BMB) demonstrated infiltration by lymphoplasmacytic cells. It was decided to follow-up her. In July 2006, she presented with progressive cognitive disorders. In July 2007, brain magnetic resonance imaging (MRI) showed a left occipital lesion [Figure 1]. Biopsy of the lesion confirmed the CD20+ DLBCL diagnosis. Cerebrospinal fluid (CSF) protein level was 0.33 g/dL without abnormal cells. IgM peak was at 1.80 g/L. Systemic computed tomography (CT) scan was normal. Patient was treated with four cycles of high-dose methotrexate (MTX), temozolomide and prednisone and achieved clinical and radiologic complete remission (CR). In August 2009, she presented with rapid cognitive status deterioration. MRI showed tumor progression. The patient died 2 years after DLBCL diagnosis due to recurrent lymphoma. Case 2: In January 1997, a 73-year-old woman presented with asthenia and electrophoresis monoclonal IgM peak of 28 g/L. BMB confirmed WM diagnosis. She received eight cycles of cyclophosphamide,
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.132450
Received: 19-01-2014 Review completed: 20-01-2014 Accepted: 06-04-2014 Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
Figure 1: Brain magnetic resonance imaging of the first case: Homogeneous lesion located in occipital horn of lateral right ventricle
229
[Downloaded free from http://www.neurologyindia.com on Thursday, May 15, 2014, IP: 202.177.173.189] || Click here to download free Android application for th journal Letters to Editor
doxorubicin, vincristine, and prednisolone regimen. In November 1997, first relapse was treated with six cycles of chlorambucil. In May 2000, second relapse was treated with six cycles of fludarabine. In April 2003, she presented with confusion and memory disturbance, left hemiparesis and bi-temporal hemianopsia. IgM peak was at 4.9 g/L. Brain MRI showed a right frontal lesion. 1 month later, biopsy of the lesion showed DLBC. CSF analysis showed 2.35 g/L of protein with abnormal lymphoma cells. Systemic CT scan was unremarkable. The patient received high-dose MTX, lomustine, procarbazine and prednisone. 1 month later, a brain CT scan showed a progressive disease in context of neurological deterioration. The patient died 3 months after DLBCL diagnosis due to brain lymphoma. Waldenström macroglobulinemia is characterized by lymphoplasmacytic bone marrow infiltration with IgM monoclonal gammapathy. Neurological manifestations of WM are peripheral neuropathy, myelopathy, ischemic, or hemorrhagic cerebral stroke and cerebral tumoral infiltration, called BNS.[2] It is interesting to note that both our patients presented BNS independently of WM status. Two historical forms of BNS were described:[3] the most common characterized by diffuse cerebral infiltrations of WM cells;[4] the second “tumoral form” involves the occurrence of tumor mass as analyzed on neuro-imaging. Here we propose a new histological classification of BNS: A first infiltrative form by lymphoplasmocytoid cells and a second tumoral-DLBCL form. WM is a low-grade lymphoma, which is known to be at risk for developing high-grade lymphoma in rare cases (Richter syndrome).[5] Moreover, two reported BNS histologies[3,6] revealed the association of both tumoral populations’ cells, suggesting a cerebral transformation of WM. Therefore, BNS in its “tumoral form” could be assimilated to a Richter syndrome, a new BNS entity. Therapy of BNS appeared to be extremely variable, more related to WM treatment.[7,8] However, these therapies are not recommended for primary cerebral lymphoma for which current treatment is high-dose MTX.[5,9] Our two patients received high-dose MTX and one of them achieved a prolonged CR. Bing-Neel syndrome remains a rare complication of WM. It can be classified into two categories: infiltration by lymphoplasmacytic cells and cerebral DLBCL transformation. The latter form can be considered as a Richter transformation. There is no consensual treatment for BNS, but high-dose MTX-based regimen could represent an interesting option.
Emeline Tabouret1,2, Diane Coso2, Mona Matta1, Maryline Barrié1, Reda Bouabdallah2, Olivier Chinot1 230
1
Departement of Neuro-Oncology, APHM, Timone Hospital, 13005, Marseille, 2Departement of Hematology, Paoli Calmettes Institute, 13273, cedex9, Marseille, France E-mail: [email protected]
References 1. 2. 3.
4. 5.
6. 7.
8.
9.
Bing J, Neel AV. Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection basis. Acta Med Scan 1936;88:492-506. Monteiro PL, Wildi E. Waldenstrom’s macroglobulinemia with lymphorproliferative changes in the central nervous system. 4 cases. Schweiz Arch Neurol Neurochir Psychiatr 1975;116:59-82. Imai F, Fujisawa K, Kiya N, Ninomiya T, Ogura Y, Mizoguchi Y, et al. Intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstrom’s macroglobulinemia: Case report. Neurol Med Chir (Tokyo) 1995;35:575-9. Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH. Bing-Neel Syndrome revisited. Clin Lymphoma Myeloma 2009;9:104-6. Lin P, Mansoor A, Bueso-Ramos C, Hao S, Lai R, Medeiros LJ. Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Clinicopathologic features of 12 cases. Am J Clin Pathol 2003;120:246-53. Garderet L, Baudel JL, Cervera P, Azizi L, Maury E, Guidet B, et al. ‘Indolent’ Waldenstrom’s macroglobulinemia and a cerebrospinal fluid protein level of 16 g/L. Eur J Haematol 2006;77:80-2. Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG. Bing-Neel syndrome: A case report and systematic review of clinical manifestations, diagnosis, and treatment options. Clin Lymphoma Myeloma 2009;9:462-6. Dimopoulos MA, Gertz MA, Kastritis E, Garcia-Sanz R, Kimby EK, Leblond V, et al. Update on treatment recommendations from the Fourth International Workshop on Waldenstrom’s Macroglobulinemia. J Clin Oncol 2009;27:120-6. Ferreri AJ, Reni M, Foppoli M, Martelli M, Pangalis GA, Frezzato M, et al. High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet 2009;374:1512-20. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.132451
Received: 24-11-2013 Review completed: 01-01-2014 Accepted: 13-04-2014
Primary ventral foramen magnum meningeal melanocytoma Sir, Primary meningeal melanocytomas are intriguing because of their rarity and unusual radiological appearance. [1] They are usually located around Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
