Management of Recurrent B-Cell Acute Lymphoblastic Leukemia to the Skull Base: Chemotherapy, Radiotherapy, and the Role for Adjunctive Surgical Resection

Introduction: Although B-cell acute lymphoblastic leukemia (B-ALL) may be treated with systemic chemotherapy, the disease can recur, occasionally to the brain. This can cause acute neurological deficits requiring intervention, and while additional chemotherapy may be indicated, a conundrum exists when a large lesion is incompletely treated with this modality. We present a case of an invasive skull base B-ALL recurrence which was partially treated with targeted chemotherapy, and the decision-making utilized to continue treatment after this point.

Methods: A case illustration is presented demonstrating rapid regression of a massive skull base lesion following targeted chemotherapy, and subsequent care after this therapeutic approach ceased to be efficacious.

Results: An 8-year-old female with acute lymphoblastic leukemia treated with DFCI 11–001 (calaspargase pegol) chemotherapy at age 3 years presented acutely with a left facial droop. Imaging revealed a large enhancing skull base mass involving bilateral sphenoid sinuses, optic canals, and cavernous sinuses with a large portion in the left-middle cranial fossa with resultant mass effect on the left temporal lobe. A small sample was obtained surgically, and pathology revealed atypical lymphoid cells positive for CD9, 10, 20, 25, 38, 45. She was started on intrathecal triple therapy (MTX, cytarabine, and hydrocortisone) as per study protocol AALL1331, but not on blinatumomab because of CD19 negativity. Follow-up MRI at 1 month demonstrated significant reduction in the mass and her facial droop improved. However, the multidisciplinary team was concerned of limited efficacy of continued chemotherapy and a trial of focused radiotherapy was initiated.

Currently, the mass is centered in the left-middle cranial fossa with encasement of the left carotid artery and cranial nerve V. Following chemotherapy and radiotherapy, the surgical team anticipates that portions of the lesion will remain. This case elucidates the role of neurosurgical biopsies and possible further resection of recurrent B-ALL to the skull base after exhausting chemotherapy and radiotherapy treatment.

Conclusion: This case demonstrates that targeted treatment with chemotherapy can successfully and rapidly reduce mass effect and volume of aggressive recurrence of B-cell acute lymphoblastic leukemia invasive to the skull base. Subsequently, radiotherapy and possible surgical resection may be needed to completely resect the lesion. Because of the location of this tumor, surgical resection is especially hazardous and medical management has demonstrated good results. Surgeons must consider the risks and benefits of removing any remaining lesions in comparison to observation or further chemotherapy or radiotherapy.

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021