Intracranial Hodgkin's disease
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《美国医学杂志》
Department of Paediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi-221005, India
Primary central nervous system (CNS) lymphoma is an uncommon brain tumor. Approximately 2.2% of systemic diffuse large B-cell lymphomas have CNS involvement at presentation. In contrast, intracranial Hodgkin's disease occurs in only 0.2-0.5% of patients with systemic disease.[1] Primary Hodgkin's disease is even more uncommon with only 8 cases reported so far.[2] In the Indian literature there is a single case report of intracranial Hodgkin's disease in an adult[3] and none in a child. The report an interesting case of Hodgkin's disease in a child who presented with intracranial relapse.
Five-year-old male child presented in Jan. 2004 with off and on fever for 10 months and multiple neck swellings for 2 months. On examination, child had pallor, generalized lymphadenopathy and hepatosplenomegaly. X-ray chest showed bilateral hilar lymphadenopathy. USG abdomen revealed enlarged retroperitoneal and mesenteric nodes with multiple hypoechoic areas in spleen suggestive of deposits. Lymph node biopsy revealed Hodgkin's disease mixed cellularity type. Bone marrow examination was normal. The clinical staging of the disease was Stage III SB. Child received 6 cycles of chemotherapy (COPP) with complete response. He remained well for the next 6 months and again presented with proptosis and headache for two weeks. On examination, except for a right VI cranial nerve palsy there was no other neurological deficit. There was no lymphadenopathy or hepatosplenomegaly. Fundus examination showed bilateral papilloedema. CT cranium revealed a lymphomatous mass in left cerebellar hemisphere with thickening of the left occipital bone in the region of the mass lesion Figure1. USG abdomen showed multiple enlarged retroperitoneal lymph nodes. Chest X ray, bone marrow and cerebrospinal fluid examination was normal. In view of systemic relapse with intracranial involvement, it was planned to give him systemic chemotherapy using a different regimen(ABVD) with cranial irradiation. Parents of the patient refused radiotherapy and therefore he received only chemotherapy. After 4 cycles of ABVD, child showed marked improvement with disappearance of proptosis and papilloedema. Repeat CT scan of cranium showed complete disappearance of the intracranial mass lesion Figure2. USG abdomen done at this time was also normal. The child continues to be well in the follow up.
Intracranial Hodgkin's disease is a very rare manifestation of the disease. There is a paucity of such cases in the literature. From 1954 to 1999 only 25 reports involving 48 patients were published, to which the authors added another 3 cases of their own.[4] Intracranial Hodgkin's disease in the absence of disease elsewhere in the body is very rare. In a series of 12 patients[5] only two had intracranial disease without having disease elsewhere in the body at the time of relapse. In an earlier series,[6] 21 of the 23 patients had systemic disease at the time of CNS involvement.
It had previously been suggested that intracranial disease occurred by direct spread from the bones of the skull or in continuity with meningeal or dural deposits having gained access to the skull via the cranial nerves. But later it was suggested that this was not always the case and hematogenous spread was probably the commonest mechanism[5]. In the present case, as the lesion was deep seated, a hematogenous spread was more likely.
Regarding the histology of Hodgkin's disease with intracranial relapse, both nodular sclerosis and mixed cellularity variety have been described. In the present case it was a mixed cellularity variety. In a series of 12 patients, majority had nodular sclerosis variety.[5] In another report there was an equal distribution of mixed cellularity and nodular sclerosis.[2]
Treatment strategies for brain involvement include whole brain irradiation and systemic chemotherapy. Sapozink and Kaplan stress that intracranial Hodgkin's disease should be treated with an intent to cure especially if there is no disease elsewhere or is amenable to curative treatment. They have recommended a radiation dose of 30 Gy covering the whole brain and an additional 10 to 20 Gy to the involved area plus systemic chemotherapy. Another author[7] reported a patient with brain metastasis who survived more than two years after whole brain irradiation and systemic chemotherapy. He also recommended a combination of radiotherapy and chemotherapy. However, in the present case, the patient received only chemotherapy with good results.
References
1. Dujovny M, McBrick D, Segal R. Intracranial manifestations of Hodgkin's disease. Surg Neurol 1980; 13 : 258-265.
2. Hirmiz K, Foyle A, Wilke D, Burrell S, Brownstone R, Ago C. Intracranial presentation of systemic Hodgkin's disease. Leukemia & lymphoma 2004; 45 : 1667 -1671.
3. Maitreyan V, Sagar TG, Jayakumar KT, Shanta V. CNS involvement in Hodgkin's disease. J Assoc Physicians India 1987; 35 : 863-865.
4. Nakayama H, Tokuuye K, Kagami Y, Sumi M, Murayama S, Kawashima M et al. Brain involvement in Hodgkin's disease: case reports and review of literature. Radiation Medicine 2000; 18 : 205-208.
5. Sapozink MD, Kaplan HS. Intracranial Hodgkin's disease. Cancer 1983; 52 : 1301-1307.
6. Cuttner J, Meyer R, Huang YP. Intracerebral involvement in Hodgkin's disease: a report of 6 cases and review of the literature. Cancer 1979; 43 : 1497-1506.
7. Komatsu N, Kitagawa S, Ohata M, Sakamoto S, Miura Y. A long term survival case with intracranial involvement of Hodgkin's disease. J Jpn Soc Intern Med 1989; 77 : 589-590.(Gupta V, Ghosh J, Pant P,)
Primary central nervous system (CNS) lymphoma is an uncommon brain tumor. Approximately 2.2% of systemic diffuse large B-cell lymphomas have CNS involvement at presentation. In contrast, intracranial Hodgkin's disease occurs in only 0.2-0.5% of patients with systemic disease.[1] Primary Hodgkin's disease is even more uncommon with only 8 cases reported so far.[2] In the Indian literature there is a single case report of intracranial Hodgkin's disease in an adult[3] and none in a child. The report an interesting case of Hodgkin's disease in a child who presented with intracranial relapse.
Five-year-old male child presented in Jan. 2004 with off and on fever for 10 months and multiple neck swellings for 2 months. On examination, child had pallor, generalized lymphadenopathy and hepatosplenomegaly. X-ray chest showed bilateral hilar lymphadenopathy. USG abdomen revealed enlarged retroperitoneal and mesenteric nodes with multiple hypoechoic areas in spleen suggestive of deposits. Lymph node biopsy revealed Hodgkin's disease mixed cellularity type. Bone marrow examination was normal. The clinical staging of the disease was Stage III SB. Child received 6 cycles of chemotherapy (COPP) with complete response. He remained well for the next 6 months and again presented with proptosis and headache for two weeks. On examination, except for a right VI cranial nerve palsy there was no other neurological deficit. There was no lymphadenopathy or hepatosplenomegaly. Fundus examination showed bilateral papilloedema. CT cranium revealed a lymphomatous mass in left cerebellar hemisphere with thickening of the left occipital bone in the region of the mass lesion Figure1. USG abdomen showed multiple enlarged retroperitoneal lymph nodes. Chest X ray, bone marrow and cerebrospinal fluid examination was normal. In view of systemic relapse with intracranial involvement, it was planned to give him systemic chemotherapy using a different regimen(ABVD) with cranial irradiation. Parents of the patient refused radiotherapy and therefore he received only chemotherapy. After 4 cycles of ABVD, child showed marked improvement with disappearance of proptosis and papilloedema. Repeat CT scan of cranium showed complete disappearance of the intracranial mass lesion Figure2. USG abdomen done at this time was also normal. The child continues to be well in the follow up.
Intracranial Hodgkin's disease is a very rare manifestation of the disease. There is a paucity of such cases in the literature. From 1954 to 1999 only 25 reports involving 48 patients were published, to which the authors added another 3 cases of their own.[4] Intracranial Hodgkin's disease in the absence of disease elsewhere in the body is very rare. In a series of 12 patients[5] only two had intracranial disease without having disease elsewhere in the body at the time of relapse. In an earlier series,[6] 21 of the 23 patients had systemic disease at the time of CNS involvement.
It had previously been suggested that intracranial disease occurred by direct spread from the bones of the skull or in continuity with meningeal or dural deposits having gained access to the skull via the cranial nerves. But later it was suggested that this was not always the case and hematogenous spread was probably the commonest mechanism[5]. In the present case, as the lesion was deep seated, a hematogenous spread was more likely.
Regarding the histology of Hodgkin's disease with intracranial relapse, both nodular sclerosis and mixed cellularity variety have been described. In the present case it was a mixed cellularity variety. In a series of 12 patients, majority had nodular sclerosis variety.[5] In another report there was an equal distribution of mixed cellularity and nodular sclerosis.[2]
Treatment strategies for brain involvement include whole brain irradiation and systemic chemotherapy. Sapozink and Kaplan stress that intracranial Hodgkin's disease should be treated with an intent to cure especially if there is no disease elsewhere or is amenable to curative treatment. They have recommended a radiation dose of 30 Gy covering the whole brain and an additional 10 to 20 Gy to the involved area plus systemic chemotherapy. Another author[7] reported a patient with brain metastasis who survived more than two years after whole brain irradiation and systemic chemotherapy. He also recommended a combination of radiotherapy and chemotherapy. However, in the present case, the patient received only chemotherapy with good results.
References
1. Dujovny M, McBrick D, Segal R. Intracranial manifestations of Hodgkin's disease. Surg Neurol 1980; 13 : 258-265.
2. Hirmiz K, Foyle A, Wilke D, Burrell S, Brownstone R, Ago C. Intracranial presentation of systemic Hodgkin's disease. Leukemia & lymphoma 2004; 45 : 1667 -1671.
3. Maitreyan V, Sagar TG, Jayakumar KT, Shanta V. CNS involvement in Hodgkin's disease. J Assoc Physicians India 1987; 35 : 863-865.
4. Nakayama H, Tokuuye K, Kagami Y, Sumi M, Murayama S, Kawashima M et al. Brain involvement in Hodgkin's disease: case reports and review of literature. Radiation Medicine 2000; 18 : 205-208.
5. Sapozink MD, Kaplan HS. Intracranial Hodgkin's disease. Cancer 1983; 52 : 1301-1307.
6. Cuttner J, Meyer R, Huang YP. Intracerebral involvement in Hodgkin's disease: a report of 6 cases and review of the literature. Cancer 1979; 43 : 1497-1506.
7. Komatsu N, Kitagawa S, Ohata M, Sakamoto S, Miura Y. A long term survival case with intracranial involvement of Hodgkin's disease. J Jpn Soc Intern Med 1989; 77 : 589-590.(Gupta V, Ghosh J, Pant P,)