Carcinomatous Meningitis from Urachal Carcinoma: The First Reported Case

 

Shearwood McClelland III M.D.¹, Rebeca E. Garcia B.Ac.¹, Sara E. Monaco M.D.², James E. Goldman M.D., Ph.D.², Ty J. Olson M.D.¹, Grace H. Kim M.D.¹, Daniel P. Petrylak M.D.³, Robert R. Goodman M.D., Ph.D.¹

 

Departments of ¹Neurological Surgery, ²Pathology, and ³Medicine, Columbia University, College of Physicians and Surgeons, New York, NY

 

Introduction: Carcinomatous meningitis (CM) occurs when neoplastic cells gain access to the cerebrospinal fluid (CSF), and is recognized clinically in 4-7% of all cancer patients.  The most common causes of CM are breast, lung and skin cancer, with no previous reports of urachal carcinoma manifesting as CM.  We present the first reported case of a patient with metastatic cancer of the urachus who developed symptomatic leptomeningeal disease.

 

Methods: A 33-year-old woman with a three-year history of urachal adenocarcinoma presented with headache and skew diplopia.   Brain MRI revealed abnormal bilateral superior cerebellar enhancement with a nodule in the superior right cerebellum.  Cervical spine MRI revealed small superficial intradural enhancing foci at C5 and T1, suggestive for CM.  One week after suboccipital craniectomy confirmed metastatic adenocarcinoma, she experienced headache, nausea, vomiting and ataxia.  Lumbar puncture was performed to confirm the suspicion of CM.

 

Results:  Cytologic CSF examination revealed malignant cells, establishing the diagnosis of CM.  She subsequently underwent Ommaya reservoir placement and radiation therapy, but developed hydrocephalus, necessitating conversion to a right ventriculoperitoneal shunt.  Unfortunately, despite systemic chemotherapy with temozolomide, her disease progression was rapid, resulting in death two months after initial neurologic presentation. 

 

Conclusion: This case represents the first report of urachal carcinoma resulting in CM, and underscores the need to recognize CM as part of the differential diagnosis in patients with known primary tumor who present with unexplained neurological symptoms.  Although the prognosis for this patient population is disappointing, early diagnosis and treatment may improve quality of life and prolong survival.