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Carcinomatous optic neuropathy: An unusual presentation of metastatic meningeal disease
Abstracts Carcinomatous Optic Neuropathy: An Unusual Presentation of Metastatic Meningeal Disease
E. J. Sawyer, St Thomas' Hospital, London, UK A 54-year-old lady with a past history of invasive lobular breast carinoma presented with a 2-month history of ataxia, headache and visual deterioration. As a child, she had suffered a penetrating injury to the left eye, leaving her blind in that eye. The vision in the right eye had fallen to the level of perception of light and fundoscopy revealed papilloedema. Whole-brain computed tomographic (CT) scanning showed no abnormality. At lumbar puncture, there was a high opening pressure (36 era), but no malignant cells were seen on cytology. A C T scan of the orbit showed dilatation of the optic nerve sheath and she underwent optic nerve sheath fenestration and biopsy. The histology confirmed metastatic breast cancer. Magnetic resonance imaging (MRI) with gadolinium did not reveal any evidence of meningeal disease and there was no evidence of metastases elsewhere. Despite optic nerve decompression, high dose dexamethasone, and whole brain irradiation to a dose of 30 Gy in 10 fractions given daily, her vision continued to deteriorate and she was registered blind. She remained well for the next 18 months, until she developed a mild pyramidal weakness of the legs. Once again there was a high opening pressure at lumbar puncture and high cerebrospinal fluid (CSF) protein (8.5 g/l), but normal CSF eytlogy and MRI of the brain and the spine. Although the high CSF pressure and increased CSF protein suggested metastatic meningeal disease, there was no imaging or cytological evidence to support this. Her condition remained stable for the next 5 months; when she developed further weakness in her legs and loss of sphincter control. MRI at this time revealed widespread meningeal disease. Unfortunately, she continued to deteriorate despite whole spine irradiation. Carcinomatous optic neuropathy is rare as a site of isolated metastasis in breast cancer. What was particularly unusual in this patient was the length of time (23 months) before the development of overt meningeal disease. Meningeal carcinomatosis occurs in 1%-5% of patients with breast cancer and generally has a poor prognosis, with a median survival of 3-7 months, this case history
407 illustrates that metastatic meningeal disease, as other forms of breast cancer metastases, can run an indolent course. It also demonstrates the difficulties in diagnosis.
A Case of Fatal Stevens-Johnson Syndrome on a Patient with NonHodgkin's Lymphoma Treated with Chlorambucil S. Sothi 1, J. J. Mould 1 and H. Lewis 2, 1Queen Elizabeth Hospital and 2Selly Oak Hospital, Birmingham, UK We report a case of fatal Stevens-Johnson syndrome in a patient with low grade non-Hodgkin's lymphoma treated with chlorambucil. A 63-year-old man presented with Stage II low grade nonHodgkin's lymphoma and was treated with radical radiotherapy. Two months later, Stage III disease recurred. A repeat biopsy was consistent with low grade non-Hodgkin's lymphoma. He was started on oral chlorambucil 10 mg daily. He tolerated the first 6week course of chlorambucil reasonably well. However, 3 days after restarting chlorambucil, he developed an urticarial rash, followed by severe oropharyngeal ulceration. The diagnosis of Stevens--Johnson syndrome was confirmed on skin biopsy and he was started on high dose steroids. He deteriorated, with bullous skin eruptions, severe mucositis and eye involvement. He became septicaemic and died. Post-mortem examination revealed persistent para-aortic lymphadenopathy. There are only two other reported cases of chlorambucil being associated with a bullous skin reaction. In both of these, the patients were rechallenged with chlorambucil after an initial reaction, and skin patch tests for chlorambucil were positive. Our patient was not rechallenged with chlorambucil. We cannot be certain that chlorambucil was the precipitating cause, especially as lymphoma itself is a well documented cause of the StevensJohnson syndrome. We feel, however, that the timing of events makes chlorambucil the likely cause. Stevens-Johnson syndrome is a rare complication of drug treatment, but it has a high mortality rate, which can be reduced with greater awareness and earlier diagnosis.
E. J. Sawyer, St Thomas' Hospital, London, UK A 54-year-old lady with a past history of invasive lobular breast carinoma presented with a 2-month history of ataxia, headache and visual deterioration. As a child, she had suffered a penetrating injury to the left eye, leaving her blind in that eye. The vision in the right eye had fallen to the level of perception of light and fundoscopy revealed papilloedema. Whole-brain computed tomographic (CT) scanning showed no abnormality. At lumbar puncture, there was a high opening pressure (36 era), but no malignant cells were seen on cytology. A C T scan of the orbit showed dilatation of the optic nerve sheath and she underwent optic nerve sheath fenestration and biopsy. The histology confirmed metastatic breast cancer. Magnetic resonance imaging (MRI) with gadolinium did not reveal any evidence of meningeal disease and there was no evidence of metastases elsewhere. Despite optic nerve decompression, high dose dexamethasone, and whole brain irradiation to a dose of 30 Gy in 10 fractions given daily, her vision continued to deteriorate and she was registered blind. She remained well for the next 18 months, until she developed a mild pyramidal weakness of the legs. Once again there was a high opening pressure at lumbar puncture and high cerebrospinal fluid (CSF) protein (8.5 g/l), but normal CSF eytlogy and MRI of the brain and the spine. Although the high CSF pressure and increased CSF protein suggested metastatic meningeal disease, there was no imaging or cytological evidence to support this. Her condition remained stable for the next 5 months; when she developed further weakness in her legs and loss of sphincter control. MRI at this time revealed widespread meningeal disease. Unfortunately, she continued to deteriorate despite whole spine irradiation. Carcinomatous optic neuropathy is rare as a site of isolated metastasis in breast cancer. What was particularly unusual in this patient was the length of time (23 months) before the development of overt meningeal disease. Meningeal carcinomatosis occurs in 1%-5% of patients with breast cancer and generally has a poor prognosis, with a median survival of 3-7 months, this case history
407 illustrates that metastatic meningeal disease, as other forms of breast cancer metastases, can run an indolent course. It also demonstrates the difficulties in diagnosis.
A Case of Fatal Stevens-Johnson Syndrome on a Patient with NonHodgkin's Lymphoma Treated with Chlorambucil S. Sothi 1, J. J. Mould 1 and H. Lewis 2, 1Queen Elizabeth Hospital and 2Selly Oak Hospital, Birmingham, UK We report a case of fatal Stevens-Johnson syndrome in a patient with low grade non-Hodgkin's lymphoma treated with chlorambucil. A 63-year-old man presented with Stage II low grade nonHodgkin's lymphoma and was treated with radical radiotherapy. Two months later, Stage III disease recurred. A repeat biopsy was consistent with low grade non-Hodgkin's lymphoma. He was started on oral chlorambucil 10 mg daily. He tolerated the first 6week course of chlorambucil reasonably well. However, 3 days after restarting chlorambucil, he developed an urticarial rash, followed by severe oropharyngeal ulceration. The diagnosis of Stevens--Johnson syndrome was confirmed on skin biopsy and he was started on high dose steroids. He deteriorated, with bullous skin eruptions, severe mucositis and eye involvement. He became septicaemic and died. Post-mortem examination revealed persistent para-aortic lymphadenopathy. There are only two other reported cases of chlorambucil being associated with a bullous skin reaction. In both of these, the patients were rechallenged with chlorambucil after an initial reaction, and skin patch tests for chlorambucil were positive. Our patient was not rechallenged with chlorambucil. We cannot be certain that chlorambucil was the precipitating cause, especially as lymphoma itself is a well documented cause of the StevensJohnson syndrome. We feel, however, that the timing of events makes chlorambucil the likely cause. Stevens-Johnson syndrome is a rare complication of drug treatment, but it has a high mortality rate, which can be reduced with greater awareness and earlier diagnosis.