Objective and importance: We report the first case of primary lymphoma of Meckel's cave. The ability of a lymphoma to mimic a trigeminal schwannoma, both clinically and radiographically, resulted in misdiagnosis and flawed surgical strategy. We discuss the characteristics of a Meckel's cave lymphoma on magnetic resonance images, the predisposing medical conditions that should cause the neurosurgeon to add lymphoma to the normal differential diagnosis, and appropriate management strategies.
Clinical presentation: A 40-year-old African-American woman presented with a 5-month history of progressive facial numbness and pain in all three divisions of the left trigeminal nerve. Magnetic resonance imaging revealed a mass in the left side of Meckel's cave, with extension into the lateral compartment of the cavernous sinus, without encasement of the internal carotid artery, through the foramen rotundum into the posterior aspect of the maxillary sinus, and through the foramen ovale into the pterygopalatine fossa. The diagnosis, based on clinical history and radiographic imaging, was schwannoma of Meckel's cave. The patient had a history of systemic lupus erythematosus that had been treated with intermittent steroid therapy.
Intervention: The surgical approach selected was a frontotemporal craniotomy with orbitozygomatic osteotomy and anterior petrosectomy. The lesion was totally excised, although the gross intraoperative appearance of the lesion was inconsistent with the preoperative diagnosis, and the pathological examination was unable to establish a histological diagnosis on the basis of frozen sections. Histological diagnosis was confirmed on permanent section after surgery as B-cell lymphoma. Evaluation for other primary sites produced negative results. The patient was then treated with cyclophosphamide (Cytotoxan; Bristol-Myers Oncology, Princeton, NJ), doxorubicin (Adriamycin; Pharmacia & Upjohn, Kalamazoo, MI), vincristine, and prednisone chemotherapy every 3 weeks for six cycles and then by radiation therapy to the affected area.
Conclusion: The diagnosis of lymphoma should be considered for lesions affecting Meckel's cave in high-risk immunocompromised patients. The presence of an apparent dural tail in an otherwise typical schwannoma is the distinguishing characteristic of a lymphoma. The absence of hyperostosis helps differentiate it from a meningioma. At this point, the preferred surgical strategy is biopsy for diagnosis and then radiotherapy and chemotherapy rather than major cranial base surgery for total resection.