Carcinomatous Myelitis and Meningitis after a Squamous Cell Carcinoma of the Lip
The patient was a 33-year-old Caucasian man without any specific personal or familial history. He was an active smoker without alcoholic intoxication. In 2009, he presented a lesion of the lower lip. He underwent resection of this lesion, and the first pathological analysis revealed a salivary ductal ecstasy.\nOne year later, he developed a peripheral facial paralysis associated with left trigeminal neuralgia. MRI showed perineural invasion of the trigeminal nerve within the temporal fossa (fig. ). Surgical biopsies performed in July 2010 identified secondary lesions from a well-differentiated SCC localized around the V and VII nerve sheaths. According to this diagnosis, a new pathological analysis of the previous resection of the lip was performed in a cancer reference center and diagnosis of SCC of the lip was made. The patient started radio-chemotherapy in September 2010. He received 70 Gy in 35 fractions, and five courses of an association of 5-fluorouracil and cisplatinum. First radiological assessments, 4 months after the end of radio-chemotherapy showed no residual disease.\nHowever, in November 2011, the patient presented with new neurological symptoms: paraparesis and back pain. MRI showed medullary myelitis without meningeal enhancement. Lumbar puncture revealed inflammatory cells without atypical cells within the cerebrospinal fluid (CSF). Cerebral MRI and positron emission tomography scan were normal with no evidence of visceral metastasis. Multiple sclerosis was suspected and the patient received several corticosteroid flashes who led to partial improvement of symptoms. Nevertheless, other explorations were negative, and it was not possible to certify the diagnosis of multiple sclerosis. In early 2012, the patient relapsed and developed paraparesis once again. Sequential lumbar punctures found no abnormal cells. He was given a symptomatic treatment combining corticosteroid and plasma depletion. In March 2012, CSF analysis showed intrathecal immunoglobulin syntheses with oligoclonal immunoglobulin bands. Medullary MRI revealed intramedullary hyperintensity without enhancement after gadolinium injection. The diagnosis of dysimmune myelitis was made and the patient received an immunosuppressive drug (mycophenolate mofetil). It is of note that his neurological symptoms partially improved under this treatment.\nIn August 2012, he subsequently developed progressive back pain, sphincter disorders, and lower limb weakness. A new MRI performed in November showed enhancement of the meningeal nodules and abnormal leptomeningeal contrast enhancement (fig. ). There was no evidence of brain lesions. New lumbar punctures revealed several atypical cells consistent with metastatic squamous cells (fig. ) associated with low glycorrhachia and high proteinorrhachia levels (1.25 g/l). These cells were well-differentiated with nuclear abnormalities consistent with meningeal infiltration by a well-differentiated SCC. Intrathecal immunoglobulin synthesis was no longer seen. Moreover, chest and abdominal CT-scans did not identify other secondary lesions, especially bone metastasis. Nevertheless, a PET scan showed an intramedullary spinal cord metabolism enhancement. All these data were consistent with the diagnosis of CM associated with a carcinomatous myelitis.\nThe patient underwent intrathecal weekly infusions of methotrexate (15 mg) and methylprednisolone combined with systemic treatment based on carboplatin every 3 weeks and weekly cetuximab (250 mg/m2). After two cycles, the disease was clinically stable with no extension of symptoms, the proteinorrhachia level was still high (1.5 g/l), and metastatic cells were still identified in the CSF. However, MRI showed a slight amelioration and cytological analysis revealed only isolated metastatic cells. A new chest and abdominal CT-scan showed no other secondary lesions. As the patient developed a febrile neutropenia, we decided to stop systemic chemotherapy. He received cetuximab 500 mg/m2 and intrathecal methotrexate 15 mg every 2 weeks. After three courses of treatment, clinical and MRI evaluation showed no neurological worsening and a stabilization of the medullar lesions. These observations were confirmed 6 months after treatment start.