Management of Hodgkin’s lymphoma with midbrain involvement: A case report and review of literature Vinayak V. Maka1 , Rohit Chitrapur 1, Nalini Kilara1, Vinay M. D. Prabhu 2, Naveen Krishnamoorthy 3 Department of Medical Oncology, M S Ramaiah Medical College, Bangalore, Karnataka, India, 2Department of Radiology, M S Ramaiah Medical College, Bangalore, Karnataka, India, 3Triesta Reference Laboratory, Bangalore, Karnataka, India
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Introduction: Primary central nervous system (CNS) involvement of Hodgkin’s lymphoma is very uncommon. There are only a few previous reports of Hodgkin’s lymphoma of nodular lymphocyte predominant histology involving the CNS concurrently with systemic disease. Case presentation: A 12-year-old boy with a history of painless left inguinal swelling and acute diplopia. There was an intensely enhancing lesion in the right midbrain on magnetic resonance imaging. The patient was diagnosed with stage IV Hodgkin’s lymphoma of nodular lymphocyte predominance type by routine microscopy and immunohistochemistry of left inguinal lymph node biopsy with computed tomographyassisted staging. It was planned to treat him with six cycles of chemotherapy with intrathecal methotrexate, followed by radiotherapy to the CNS lesions. After two cycles of chemotherapy, the patient entered complete remission of all lesions including the CNS lesion documented by the positron emission tomography scan. Conclusion: We are describing the course of this rare presentation of Hodgkin’s lymphoma of nodular lymphocyte predominant histology involving the CNS and clinical challenge in its diagnosis and management of this case. Keywords: Hodgkin’s lymphoma, CNS involvement, Brain stem
Background
Un
Hodgkin’s lymphoma (HL) most commonly presents with progressive, painless enlargement of peripheral lymph nodes, especially around the cervical region or less frequently mediastinal or abdominal lymph nodes and progresses to other groups of lymph nodes and eventually to non-lymphoid organs. HL involves the central nervous system (CNS) in 0.2–0.5% of cases either by contiguous or hematogenous spread.1,2 CNS involvement is more common in patients with widespread relapsed disease or at initial diagnosis in immunocompromised patients, but can also be seen at initial diagnosis in immunocompetent patients.3 HL presenting as a lesion of the brain stem is less common than other intracranial sites.1,4–7 Mixed cellularity histology is the most frequent subtype of Hodgkin’s disease among these patients, but our patient has presented with HL with nodular lymphocyte predominance.8,9 Epstein–Barr virus (EBV)-associated Correspondence to: Vinayak V. Maka, Department of Medical Oncology, M S Ramaiah Medical College, M S R HCG Center of Oncology, MSRIT Post, Bangalore 560054, Karnataka, India. Email: [email protected]
© W. S. Maney & Son Ltd 2014 DOI 10.1179/1607845414Y.0000000190
polymorphic lymphoproliferative disorders (PLDs) can resemble more like HL. PLDs in which the atypical cells almost universally express B-cell markers and carry EBV genome.10 Although this is an uncommon clinical scenario, healthcare professionals should be aware of this possibility of HL with nodular lymphocyte predominance involving the midbrain.
Case presentation A 12-year-old boy presented with a history of painless left inguinal swelling of 1-month duration and abrupt onset of disabling diplopia without B symptoms. Physical examination revealed an enlarged left inguinal lymphadenopathy, bilateral tonsillar enlargement, paralysis of lateral gaze on the left eye, and nystagmus with right lateral gaze. Laboratory examinations revealed normal complete blood count and biochemical parameters. The patient underwent computed tomography (CT)-assisted staging, neuroaxis magnetic resonance imaging (MRI), lumbar puncture, and bone marrow biopsy. These studies demonstrated prevascular, pretracheal,
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Management of Hodgkin’s lymphoma with midbrain involvement
consistent with nodular lymphocyte predominance type of HL (Fig. 2). The lymphocytic and histiocytic cells express LCA, Pax-5, CD20, and OCT 1, and are immunonegative for CD30, CD15, and CD3. He underwent tonsillectomy as there was bilateral tonsillar enlargement and histopathological examination revealed reactive tonsillar lymphoid tissue. In view of the difficulty in obtaining tissue from the brain stem lesion without long-term neurological morbidity, the brain stem lesions were presumed to be involved and treated with intravenous steroids and intrathecal methotrexate with resolution of diplopia. The patient’s stage IV HL is planned to be treated with six cycles of chemotherapy with cyclophosphamide, vincristine, prednisolone, procarbazine, doxorubicin, bleomycin, and vinblastine (COPP/ABV), with 6 monthly treatments of intrathecal methotrexate on day one of each cycle, followed by radiotherapy to
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and supraclavicular lymphadenopathy; intensely enhancing lesion in the right midbrain (measuring 16 × 17 mm, Fig. 1A–C); normal cerebrospinal fluid (CSF) protein and negative CSF cytology; and negative bone marrow pathology. Serologic testing for HIV, EBV, and hepatitis B and C virus was negative. On positron emission tomography(PET) scan showed increased FDG uptake in the left external iliac and inguinal lymph node regions (largest measuring 2.0 × 1.5 cm, SUV-6), bilateral level II cervical lymph nodes (largest measuring 2.2 × 0.9 cm, SUV-2.7), adenoids and bilateral palatine tonsils (SUV-8.53), and three nodular enhancing lesions (SUV-8.5) in the right thalamus (1.1 × 0.7) and midbrain (1.3 × 1.0 and 0.6 × 0.4 cm) with perilesional edema and mass effect. Pathologic evaluation of left inguinal lymph node biopsy demonstrated a fibrous tumor composed of small lymphocytes and scattered plasma cells
Pro of
Figure 1 Pre-treatment magnetic resonance imaging with coronal (A) and sagittal T2 (B) with contrast (C) images showing intensely enhancing lesion in the right midbrain.
Figure 2 HPE (H&E) of right inguinal lymph node biopsy showing microscopic sections: a predominant nodular pattern (A) with morphologically typical Reed-Sternberg cells, the popcorn cells (B) showing positive immunostaining with CD45 (D), CD20 (C), and OCT (E) positivity with CD30 negativity.
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Hodgkin’s disease is cranial nerve palsy.12 Brain parenchymal involvement shows a predilection for supratentorial lesions, this being the most common intracranial site of involvement.1,4–7,12,13 Mixed cellularity histology is the most frequent subtype of Hodgkin’s disease among these patients,8,9,12,13 although the case reported by Vetter et al. 14 was of nodular lymphocyte predominance. The distinction between HL and HL-like PLD is often challenging and perhaps arbitrary. Atypical cells in such PLD cases usually are B cells expressing CD20, CD30, CD45, and EBV antigens, the latent membrane protein (LMP-1), and/or the Epstein–Barr virusencoded RNA 1 (EBER-1). In our patients, diagnosis of nodular lymphocyte predominant HL was confirmed by the absence of CD30 and EBV-associated antigens on immunohistochemistry.10 Median survival following local (10–20 Gy) and whole-brain (30–40 Gy) radiotherapy has been reported in the range of 8 months to 2 years.4 Systemic chemotherapy
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the CNS lesions. After two cycles of COPP/ABV, the patient entered complete remission of all lesions including the CNS lesion as documented by the MRI (Fig. 3A–C) and PET CT scan. PET CT scan after six cycles of chemotherapy has shown complete resolution of all previously seen CNS lesions without any metabolic activity (Fig. 4). The patient was treated with external radiation on 6 mv LINAC to a dose of 23.4 Gy to the whole brain followed by 6 Gy to the pre-chemotherapy tumor volume with 5 mm margins. Overall treatment was well tolerated by the patient without treatment breaks. The patient is currently on 2 monthly outpatient follow-up.
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Figure 3 Post-treatment magnetic resonance imaging with coronal (A) and sagittal T2 (B) with contrast (C) showing intensely enhancing lesion in the right midbrain.
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Etiology, treatment, and prognosis of intracranial HL have not been established.11 Common presentations include cranial nerve palsies, motor and/or sensory deficits, headaches, papilledema, coma, and seizures.8 The most common presenting feature of intracranial
Figure 4 Pre-treatment and post-treatment positron emission tomography computed tomographic scan images of midbrain lesion suggestive of remission of disease.
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Ethics approval Written informed consent was obtained from the subject for publishing this case report and accompanying images.
References 1 Ashby MA, Barber PC, Holmes AE, et al. Primary intracranial Hodgkin’s disease. Am J Surg Pathol. 1988;12:294–9. 2 Figueroa BE, Brown JR, Nascimento A, et al. Unusual sites of Hodgkin’s lymphoma: CASE 2. Hodgkin’s lymphoma of the CNS masquerading as meningioma. J Clin Oncol. 2004;22: 4228–30. 3 Anselmo AP, Proia A, Cartoni C, et al. Meningeal localization in a patient with Hodgkin’s disease: description of a case and review of the literature. Ann Oncol. 1996;7:1071–5 4 Biagi J, MacKenzie RG, Lim MS, et al. Primary Hodgkin’s disease of the CNS in an immunocompetent patient: a case study and review of the literature. Neuro-oncology 2000;2: 239–43. 5 Johnson MD, Kinney MC, Scheithauer BW, et al. Primary intracerebral Hodgkin’s disease mimicking meningioma: case report. Neurosurgery 2000;47:454–6. 6 Klein R, Mullges W, Bendszus M, et al. Primary intracerebral Hodgkin’s disease. Am J Surg Pathol. 1999;23:477–81. 7 Nakayama H, Tokuuye K, Kagami Y, et al. Brain involvement in Hodgkin’s disease: case reports and review of the literature. Radiat Med. 2000;18:205–8. 8 Akyuz C, Yalcin B, Atahan IL, et al. Intracranial involvement in Hodgkin’s disease. Pediatr Hematol Oncol. 2005;22:589–96. 9 Antonio G, Dahlstrom J, Chandran KN, et al. Cerebellopontine angle Hodgkin’s disease. Australas Radiol. 2000;44:115–7. 10 Tao J, Wasik MA. Epstein–Barr virus associated polymorphic lymphoproliferative disorders occurring in non-transplant settings. Lab Invest. 2001;81:429–37. 11 Sapozink MD, Kaplan HS: Intracranial Hodgkin’s disease: a report of 12 cases and review of the literature. Cancer 1983;52: 1301–7. 12 Hirmiz K, Foyle A, Wilke D, et al. Intracranial presentation of systemic Hodgkin’s disease. Leuk Lymphoma 2004;45:1667–71. 13 Gerstner ER, Abrey LE, Schiff D, Ferreri AJ, Lister A, Montoto S, et al. CNS Hodgkin lymphoma. Blood 2008;112:1658–61. 14 Vetter M, Tzankov A, Engert A, Mehling M, Herrmann R, Rochlitz C. Hodgkin’s lymphoma and paraneoplastic phenomena in the central nervous system: a case report and review of the literature. Case Rep Oncol. 2011;4:106–14. 15 Canellos GP, Mauch P. What is the appropriate systemic chemotherapy for lymphocyte-predominant Hodgkin’s lymphoma? J Clin Oncol. 2010;28(1):e8. 16 Fanale MA, Lai C-M, McLaughlin P, et al. Outcomes of nodular lymphocyte predominant Hodgkin’s Lymphoma (NLPHL) patients treated with R-CHOP. ASH Annual Meeting Abstracts 2010;116(21):2812. 17 Kresak JL, Nguyen J, Wong K, Davis R. Primary Hodgkin lymphoma of the central nervous system: two case reports and review of the literature. Neuropathology 2013;33:658–62. . 18 Henkenberens C, Franzke A, et al. Primary EBV-positive Hodgkin’s lymphoma of the CNS under azathioprine treatment. Strahlenther Onkol. 1–6 19 Burnelli R, Fabbri E, et al. Neurological presentation of Hodgkin lymphoma in the Italian Association of Pediatric Hematology and Oncology LH-2004 protocol. LeukLymphoma 52(6):1049–54
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in combination with radiotherapy has been advocated, and treatment protocols have included ABVD, COPP (cyclophosphamide, vincristine, procarbazine, prednisone), MOPP (nitrogen mustard, vincristine, procarbazine, prednisone), and/or intrathecal methotrexate.2,3,6,11 Long-term disease-free survival has been reported following combined-modality therapy.2,3,6,8 In this case report, the patient was diagnosed with stage IV nodular predominance Hodgkin’s lymphoma (NPLHL) on histopathology and immunohistochemistry of the left inguinal lymph node. We had faced clinical dilemma in conducting a diagnostic biopsy from the brain stem (view of proximity to vital centers) and decision regarding appropriate combination chemotherapy with adequate CNS activity. Despite need for histopathological evidence, we proceed to systemic combination chemotherapy COPP/ABV protocol without biopsy. Use of alkylator-based therapy may have an advantage over non-alkylator-based regimens in NLPHL. This is supported by various retrospective studies and so we initiated COPP/ABV protocol with intrathecal methotrexate.15–19 Thus, we present the case report of an unusual intracranial site of NPLHL in the brain stem and clinical challenge in its diagnosis and management of this case.
Pro of
Maka et al.
Written informed consent was obtained from the subject for publishing this case report and accompanying images.
Disclaimer statements
Un
Contributors All authors have revised the manuscript critically and gave final approval of the version to be published. Funding None.
Conflicts of interest None.
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Introduction: Primary central nervous system (CNS) involvement of Hodgkin’s lymphoma is very uncommon. There are only a few previous reports of Hodgkin’s lymphoma of nodular lymphocyte predominant histology involving the CNS concurrently with systemic disease. Case presentation: A 12-year-old boy with a history of painless left inguinal swelling and acute diplopia. There was an intensely enhancing lesion in the right midbrain on magnetic resonance imaging. The patient was diagnosed with stage IV Hodgkin’s lymphoma of nodular lymphocyte predominance type by routine microscopy and immunohistochemistry of left inguinal lymph node biopsy with computed tomographyassisted staging. It was planned to treat him with six cycles of chemotherapy with intrathecal methotrexate, followed by radiotherapy to the CNS lesions. After two cycles of chemotherapy, the patient entered complete remission of all lesions including the CNS lesion documented by the positron emission tomography scan. Conclusion: We are describing the course of this rare presentation of Hodgkin’s lymphoma of nodular lymphocyte predominant histology involving the CNS and clinical challenge in its diagnosis and management of this case. Keywords: Hodgkin’s lymphoma, CNS involvement, Brain stem
Background
Un
Hodgkin’s lymphoma (HL) most commonly presents with progressive, painless enlargement of peripheral lymph nodes, especially around the cervical region or less frequently mediastinal or abdominal lymph nodes and progresses to other groups of lymph nodes and eventually to non-lymphoid organs. HL involves the central nervous system (CNS) in 0.2–0.5% of cases either by contiguous or hematogenous spread.1,2 CNS involvement is more common in patients with widespread relapsed disease or at initial diagnosis in immunocompromised patients, but can also be seen at initial diagnosis in immunocompetent patients.3 HL presenting as a lesion of the brain stem is less common than other intracranial sites.1,4–7 Mixed cellularity histology is the most frequent subtype of Hodgkin’s disease among these patients, but our patient has presented with HL with nodular lymphocyte predominance.8,9 Epstein–Barr virus (EBV)-associated Correspondence to: Vinayak V. Maka, Department of Medical Oncology, M S Ramaiah Medical College, M S R HCG Center of Oncology, MSRIT Post, Bangalore 560054, Karnataka, India. Email: [email protected]
© W. S. Maney & Son Ltd 2014 DOI 10.1179/1607845414Y.0000000190
polymorphic lymphoproliferative disorders (PLDs) can resemble more like HL. PLDs in which the atypical cells almost universally express B-cell markers and carry EBV genome.10 Although this is an uncommon clinical scenario, healthcare professionals should be aware of this possibility of HL with nodular lymphocyte predominance involving the midbrain.
Case presentation A 12-year-old boy presented with a history of painless left inguinal swelling of 1-month duration and abrupt onset of disabling diplopia without B symptoms. Physical examination revealed an enlarged left inguinal lymphadenopathy, bilateral tonsillar enlargement, paralysis of lateral gaze on the left eye, and nystagmus with right lateral gaze. Laboratory examinations revealed normal complete blood count and biochemical parameters. The patient underwent computed tomography (CT)-assisted staging, neuroaxis magnetic resonance imaging (MRI), lumbar puncture, and bone marrow biopsy. These studies demonstrated prevascular, pretracheal,
Hematology
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Maka et al.
Management of Hodgkin’s lymphoma with midbrain involvement
consistent with nodular lymphocyte predominance type of HL (Fig. 2). The lymphocytic and histiocytic cells express LCA, Pax-5, CD20, and OCT 1, and are immunonegative for CD30, CD15, and CD3. He underwent tonsillectomy as there was bilateral tonsillar enlargement and histopathological examination revealed reactive tonsillar lymphoid tissue. In view of the difficulty in obtaining tissue from the brain stem lesion without long-term neurological morbidity, the brain stem lesions were presumed to be involved and treated with intravenous steroids and intrathecal methotrexate with resolution of diplopia. The patient’s stage IV HL is planned to be treated with six cycles of chemotherapy with cyclophosphamide, vincristine, prednisolone, procarbazine, doxorubicin, bleomycin, and vinblastine (COPP/ABV), with 6 monthly treatments of intrathecal methotrexate on day one of each cycle, followed by radiotherapy to
Un
cor rec t
ed
and supraclavicular lymphadenopathy; intensely enhancing lesion in the right midbrain (measuring 16 × 17 mm, Fig. 1A–C); normal cerebrospinal fluid (CSF) protein and negative CSF cytology; and negative bone marrow pathology. Serologic testing for HIV, EBV, and hepatitis B and C virus was negative. On positron emission tomography(PET) scan showed increased FDG uptake in the left external iliac and inguinal lymph node regions (largest measuring 2.0 × 1.5 cm, SUV-6), bilateral level II cervical lymph nodes (largest measuring 2.2 × 0.9 cm, SUV-2.7), adenoids and bilateral palatine tonsils (SUV-8.53), and three nodular enhancing lesions (SUV-8.5) in the right thalamus (1.1 × 0.7) and midbrain (1.3 × 1.0 and 0.6 × 0.4 cm) with perilesional edema and mass effect. Pathologic evaluation of left inguinal lymph node biopsy demonstrated a fibrous tumor composed of small lymphocytes and scattered plasma cells
Pro of
Figure 1 Pre-treatment magnetic resonance imaging with coronal (A) and sagittal T2 (B) with contrast (C) images showing intensely enhancing lesion in the right midbrain.
Figure 2 HPE (H&E) of right inguinal lymph node biopsy showing microscopic sections: a predominant nodular pattern (A) with morphologically typical Reed-Sternberg cells, the popcorn cells (B) showing positive immunostaining with CD45 (D), CD20 (C), and OCT (E) positivity with CD30 negativity.
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Hodgkin’s disease is cranial nerve palsy.12 Brain parenchymal involvement shows a predilection for supratentorial lesions, this being the most common intracranial site of involvement.1,4–7,12,13 Mixed cellularity histology is the most frequent subtype of Hodgkin’s disease among these patients,8,9,12,13 although the case reported by Vetter et al. 14 was of nodular lymphocyte predominance. The distinction between HL and HL-like PLD is often challenging and perhaps arbitrary. Atypical cells in such PLD cases usually are B cells expressing CD20, CD30, CD45, and EBV antigens, the latent membrane protein (LMP-1), and/or the Epstein–Barr virusencoded RNA 1 (EBER-1). In our patients, diagnosis of nodular lymphocyte predominant HL was confirmed by the absence of CD30 and EBV-associated antigens on immunohistochemistry.10 Median survival following local (10–20 Gy) and whole-brain (30–40 Gy) radiotherapy has been reported in the range of 8 months to 2 years.4 Systemic chemotherapy
Conclusion
cor rec t
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the CNS lesions. After two cycles of COPP/ABV, the patient entered complete remission of all lesions including the CNS lesion as documented by the MRI (Fig. 3A–C) and PET CT scan. PET CT scan after six cycles of chemotherapy has shown complete resolution of all previously seen CNS lesions without any metabolic activity (Fig. 4). The patient was treated with external radiation on 6 mv LINAC to a dose of 23.4 Gy to the whole brain followed by 6 Gy to the pre-chemotherapy tumor volume with 5 mm margins. Overall treatment was well tolerated by the patient without treatment breaks. The patient is currently on 2 monthly outpatient follow-up.
Pro of
Figure 3 Post-treatment magnetic resonance imaging with coronal (A) and sagittal T2 (B) with contrast (C) showing intensely enhancing lesion in the right midbrain.
Un
Etiology, treatment, and prognosis of intracranial HL have not been established.11 Common presentations include cranial nerve palsies, motor and/or sensory deficits, headaches, papilledema, coma, and seizures.8 The most common presenting feature of intracranial
Figure 4 Pre-treatment and post-treatment positron emission tomography computed tomographic scan images of midbrain lesion suggestive of remission of disease.
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Management of Hodgkin’s lymphoma with midbrain involvement
Ethics approval Written informed consent was obtained from the subject for publishing this case report and accompanying images.
References 1 Ashby MA, Barber PC, Holmes AE, et al. Primary intracranial Hodgkin’s disease. Am J Surg Pathol. 1988;12:294–9. 2 Figueroa BE, Brown JR, Nascimento A, et al. Unusual sites of Hodgkin’s lymphoma: CASE 2. Hodgkin’s lymphoma of the CNS masquerading as meningioma. J Clin Oncol. 2004;22: 4228–30. 3 Anselmo AP, Proia A, Cartoni C, et al. Meningeal localization in a patient with Hodgkin’s disease: description of a case and review of the literature. Ann Oncol. 1996;7:1071–5 4 Biagi J, MacKenzie RG, Lim MS, et al. Primary Hodgkin’s disease of the CNS in an immunocompetent patient: a case study and review of the literature. Neuro-oncology 2000;2: 239–43. 5 Johnson MD, Kinney MC, Scheithauer BW, et al. Primary intracerebral Hodgkin’s disease mimicking meningioma: case report. Neurosurgery 2000;47:454–6. 6 Klein R, Mullges W, Bendszus M, et al. Primary intracerebral Hodgkin’s disease. Am J Surg Pathol. 1999;23:477–81. 7 Nakayama H, Tokuuye K, Kagami Y, et al. Brain involvement in Hodgkin’s disease: case reports and review of the literature. Radiat Med. 2000;18:205–8. 8 Akyuz C, Yalcin B, Atahan IL, et al. Intracranial involvement in Hodgkin’s disease. Pediatr Hematol Oncol. 2005;22:589–96. 9 Antonio G, Dahlstrom J, Chandran KN, et al. Cerebellopontine angle Hodgkin’s disease. Australas Radiol. 2000;44:115–7. 10 Tao J, Wasik MA. Epstein–Barr virus associated polymorphic lymphoproliferative disorders occurring in non-transplant settings. Lab Invest. 2001;81:429–37. 11 Sapozink MD, Kaplan HS: Intracranial Hodgkin’s disease: a report of 12 cases and review of the literature. Cancer 1983;52: 1301–7. 12 Hirmiz K, Foyle A, Wilke D, et al. Intracranial presentation of systemic Hodgkin’s disease. Leuk Lymphoma 2004;45:1667–71. 13 Gerstner ER, Abrey LE, Schiff D, Ferreri AJ, Lister A, Montoto S, et al. CNS Hodgkin lymphoma. Blood 2008;112:1658–61. 14 Vetter M, Tzankov A, Engert A, Mehling M, Herrmann R, Rochlitz C. Hodgkin’s lymphoma and paraneoplastic phenomena in the central nervous system: a case report and review of the literature. Case Rep Oncol. 2011;4:106–14. 15 Canellos GP, Mauch P. What is the appropriate systemic chemotherapy for lymphocyte-predominant Hodgkin’s lymphoma? J Clin Oncol. 2010;28(1):e8. 16 Fanale MA, Lai C-M, McLaughlin P, et al. Outcomes of nodular lymphocyte predominant Hodgkin’s Lymphoma (NLPHL) patients treated with R-CHOP. ASH Annual Meeting Abstracts 2010;116(21):2812. 17 Kresak JL, Nguyen J, Wong K, Davis R. Primary Hodgkin lymphoma of the central nervous system: two case reports and review of the literature. Neuropathology 2013;33:658–62. . 18 Henkenberens C, Franzke A, et al. Primary EBV-positive Hodgkin’s lymphoma of the CNS under azathioprine treatment. Strahlenther Onkol. 1–6 19 Burnelli R, Fabbri E, et al. Neurological presentation of Hodgkin lymphoma in the Italian Association of Pediatric Hematology and Oncology LH-2004 protocol. LeukLymphoma 52(6):1049–54
Consent
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in combination with radiotherapy has been advocated, and treatment protocols have included ABVD, COPP (cyclophosphamide, vincristine, procarbazine, prednisone), MOPP (nitrogen mustard, vincristine, procarbazine, prednisone), and/or intrathecal methotrexate.2,3,6,11 Long-term disease-free survival has been reported following combined-modality therapy.2,3,6,8 In this case report, the patient was diagnosed with stage IV nodular predominance Hodgkin’s lymphoma (NPLHL) on histopathology and immunohistochemistry of the left inguinal lymph node. We had faced clinical dilemma in conducting a diagnostic biopsy from the brain stem (view of proximity to vital centers) and decision regarding appropriate combination chemotherapy with adequate CNS activity. Despite need for histopathological evidence, we proceed to systemic combination chemotherapy COPP/ABV protocol without biopsy. Use of alkylator-based therapy may have an advantage over non-alkylator-based regimens in NLPHL. This is supported by various retrospective studies and so we initiated COPP/ABV protocol with intrathecal methotrexate.15–19 Thus, we present the case report of an unusual intracranial site of NPLHL in the brain stem and clinical challenge in its diagnosis and management of this case.
Pro of
Maka et al.
Written informed consent was obtained from the subject for publishing this case report and accompanying images.
Disclaimer statements
Un
Contributors All authors have revised the manuscript critically and gave final approval of the version to be published. Funding None.
Conflicts of interest None.
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