Case Presentation: A 66-year-old-male presented with painless jaundice. Laboratory tests were consistent with biliary obstruction, Ca 19-9 was 304.3 IU/mL (N: 0.0-35), and CT scan revealed a pancreatic mass with omental metastasis. The patient underwent ERCP with biopsy. Pathology was consistent with pancreatic adenocarcinoma (Figure 1A-B). He underwent a Whipple procedure and completed 12 cycles of chemotherapy (mFOLFIRINOX). He had an excellent treatment response. Serial imaging over 1.5 years showed no evidence of disease and he maintained a high quality of life working as a welder. Two years after initial diagnosis, the patient re-presented with ten days of acute neck pain radiating into his right shoulder and arm. In the days preceding presentation, he experienced fatigue, loss of appetite, and hallucinations attributed to prednisone prescribed for cervical radiculopathy. In the emergency department, the patient was tachycardic and hypertensive. He had no focal neurologic deficits and was oriented. Labs were notable for a white blood cell count of 20 cells/μl and lactate of 2.9 mg/dL. He was given intravenous fluids and antibiotics. Within one week of hospitalization, he developed altered mental status, visual hallucinations, and vision changes that progressed to blindness. Ophthalmology exam was normal. A broad infectious workup was negative. LP was notable for an elevated opening pressure of 51 (N: 6-25 cmH20). CSF cytology demonstrated atypical cells consistent with malignant infiltrate (Figure 1C). MRI brain revealed diffuse leptomeningeal enhancement (Figure 1D). Full-body CT showed no other disease, a doppler abdominal ultrasound showed patent vasculature, and Ca 19-9 was 22 IU/mL. The patient’s acute neurologic symptoms and rapid decline were caused by isolated pancreatic leptomeningeal carcinomatosis (LC). This diagnosis was supported by CSF cytology and brain MRI findings. The patient was transitioned to hospice without treatment due to deteriorated functional status and his end-of-life goals.
Discussion: Most people diagnosed with pancreatic adenocarcinoma are expected to die of the disease, however current trends show increased earlier stage diagnoses, leading to earlier treatment [1]. LC is a devastating complication of malignancy, occurring in 5-8% of solid tumor cases [2]. Presentation is typically non-specific, including systemic symptoms and global neurologic dysfunction. Frequent symptoms are headache and pain in a spinal, radicular, or meningeal pattern. LC is diagnosed by neuropathological examination of CSF and leptomeningeal enhancement on contrast MRI brain. Treatment is palliative and median survival is <6 months. Pancreatic LC is even rarer, to date there are 17 reported cases, with a median survival of 10 weeks (Figure 2) [3-20]. The increasing occurrence of pancreatic LC in recent years may be due to better recognition of LC, improved disease control outside of the CNS, and use of systemic therapies with poor blood-brain-barrier penetration that leave the CNS vulnerable.
Conclusions: Hospitalists need to recognize the varied presentation of LC and include it in the differential for patients with cancer history. Lack of progressive systemic disease does not rule out the diagnosis. LC is a rare condition that can complicate almost all malignancies, including pancreatic. When suspected, the workup includes LP with cytology and contrast brain MRI. Early diagnosis allows for palliative treatment and timely goals of care discussions.
