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Leptomeningeal metastasis (LM) or carcinomatous meningitis is infiltration of cancer cells to the leptomeninges and cerebrospinal fluid and is extremely rare in the setting of pancreatic adenocarcinoma with only 19 English cases reported (Supplementary Table 1, available online). There are no previous reports of an optic neuropathy from metastatic pancreatic cancer in the context of LM. Here, we report a case of pancreatic adenocarcinoma leading to LM and a severe optic neuropathy.
A 69-year-old woman with a history of pancreatic cancer presented with progressive, painless vision loss in her left eye. She was diagnosed with T2N1 well-differentiated pancreatic adenocarcinoma 9 years ago, for which she underwent a Whipple procedure followed by adjuvant gemcitabine for 5 months. Her first recurrence at 3.5 years from surgery consisted of low-volume lung nodules that were observed and, when proven indolent, resected and confirmed to be the same adenocarcinoma as the pancreas. Two years later she had radiological evidence of peritoneal metastatic disease and more lung metastasis. She commenced on standard-of-care first-line FOLFIRINOX chemotherapy. Over the next 4 years, she was treated with multiple FOLFIRINOX cycles, demonstrating radiological and biochemical partial responses followed by surveillance periods off chemotherapy and then rechallenge of FOLFIRINOX with progression. She had a repeat lung resection for dominant disease. More recently, she developed radiological signs of bone metastasis, enlarged mediastinal lymph nodes, and bilateral lung nodules consistent with progressive disease.
She developed blurry vision in her left eye 10 days before presentation, and the blurry vision gradually worsened. She had a visual acuity of 20/20 in the right eye and no light perception in the left eye. There was a left relative afferent pupillary defect, and dilated fundus examination was normal in both eyes. Humphrey 24-2 SITA-Fast visual field testing was normal in the right eye. Due to the concern for metastatic disease to the left retrobulbar optic nerve, she underwent urgent magnetic resonance imaging of the brain and orbits with contrast, which showed enhancement of both optic nerves and leptomeningeal enhancement of the corpus callosum, cingulate gyrus, and medial aspect of the temporal lobe (Fig. 1). This was consistent with metastatic disease. A lumbar puncture was performed with cytology, which confirmed the presence of adenocarcinoma.
Fig. 1Magnetic resonance imaging of the brain and orbits with contrast after vision loss demonstrating areas of leptomeningeal enhancement (red arrows) including both optic nerves.
She underwent whole-brain radiotherapy (20 Gy in 5 fractions) and concomitant treatment with dexamethasone. She subsequently developed hyponatremia, loss of rectal tone, and bladder retention owing to widespread leptomeningeal deposits in the spine. She underwent urgent radiotherapy to this area and was assessed by the palliative care team. The patient deteriorated further and died 3 months after developing vision loss. She had no light perception vision in her left eye at her final 1-month follow-up and maintained good visual function in her right eye until death.
Overall, LM develops in 5%–8% of metastatic cancer cases.
However, the rate of LM from pancreatic cancer is very low, with only 19 English cases reported (Supplementary Table 1). Here we present the first case of optic nerve dysfunction from LM in a woman with a 9-year history of pancreatic cancer. We confirmed a diagnosis of LM infiltrating the left optic nerve by both magnetic resonance imaging and cerebrospinal fluid cytology.
Grira et al. reported the only case of LM from pancreatic cancer with vision manifestations.
The report mainly focused on the neurological symptoms of the patient and the rare occurrence of LM carcinomatosis as the presenting sign of pancreatic cancer in general. There was only a brief mention of decreased visual acuity and papilledema, but no formal description of visual symptoms or ophthalmological assessment. Like most other reports, this patient had a poor outcome and died just 7 weeks after presentation. The remaining 18 cases of LM originating from pancreatic tumours did not have ophthalmological manifestations (Supplementary Table 1).
In contrast to the infrequency of reported cases of metastasis to the optic nerve, there have been more reports of vision loss owing to pancreatic cancer metastasis to the choroid and other structures of the eye.
Overall, when approaching a patient with a history of pancreatic cancer presenting with vision loss, it is important to consider LM in the differential diagnosis and conduct the necessary tests if suspected. This will ensure that treatment with radiation, chemotherapy, or corticosteroids can be considered to help maintain quality of life in these patients and allow for palliative care services to be offered given the poor prognosis.
We believe that the unique presentation of this case is attributed to the unusually long survival of the patient after the diagnosis of metastatic pancreatic cancer. Her indolent disease course and metastatic distribution was atypical, despite typical pathology. She was certainly an exceptional responder to standard chemotherapy, where the typical control period is less than 1 year. On tumour sequencing, her genomic profile described a classical pancreas adenocarcinoma with no exceptional features to explain responsiveness or atypical progression patterns. Living for 9 years with pancreatic cancer certainly increased the chances of metastasis to spread to atypical parts of the body, including the central nervous system and, in this case, the optic nerve. With more effective systemic therapies that prolong the natural history, these rare cases may become a more common presentation.
Overall, we report the case of a 69-year-old woman with a history of pancreatic adenocarcinoma metastasising to the leptomeninges, presenting with complete vision loss of the left eye. This is the first case of an optic neuropathy in this setting.
Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
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