Epstein-Barr Virus-associated Intracranial Leiomyosarcoma in an HIV-positive Adolescent

Abstract

A 17-year-old African American female with human immunodeficiency virus infection presented with an unresectable intracranial neoplasm with mass effect upon the brainstem. Stereotactic biopsy revealed an Epstein-Barr virus (EBV)-associated leiomyosarcoma. Radiation therapy and gemcitabine were used to shrink the mass with the aim to make it surgically resectable. Prolonged neutropenia and recurrent skin infections led to the discontinuation of gemcitabine. The mass stabilized after radiation therapy and has decreased in size in 15 months of follow-up. EBV has been demonstrated in most smooth muscle tumors associated with acquired immunodeficiency syndrome and other immunocompromised states. This is the first documented case of an EBV-positive intracranial leiomyosarcoma in a pediatric human immunodeficiency virus patient.

AIDS-Related EBV-Associated Smooth Muscle Tumors: A Review of 64 Published Cases

“…It may be that LMP expression is below that detected by immunohistochemistry in these tumors. Although there were some inconsistent results in cases tested for EBNA-2 and LMP-1 [7, 19, 20, 22, 27, 30, 45], it would appear that overall EBV-SMT demonstrate Type III latency. …”

“…Furthermore, each lesion is likely a separate infection event. Studies looking at EBV copy numbers in EBV-SMT have found variable results and it has been suggested that this variation is the result of EBV being present in the lytic or latent phase [2, 7, 8]. One could expect that patients with active replicating EBV will have EBV detectable in their serum [18, 22, 25, 32, 33].…”

Epstein-Barr virus-associated smooth muscle tumour presenting as a parasagittal brain tumour

“…3,8,10 The role of immunosuppression in the pathogenesis of EBV-SMT in patients infected with HIV/AIDS has been documented. 1,6,11 Long-standing low CD4 count and a high viral load may encourage tumor growth and enhance the possibility of tumor recurrence. 1 These tumours are commonly multifocal 3,8,10 and vary in description from ''leiomyoma'' to ''smooth muscle tumor of uncertain malignant potential'' to ''leiomyosarcoma'' reflecting the different criteria of malignancy utilized by various authors to histologically classify these tumours.…”

“…1,6,11 Long-standing low CD4 count and a high viral load may encourage tumor growth and enhance the possibility of tumor recurrence. 1 These tumours are commonly multifocal 3,8,10 and vary in description from ''leiomyoma'' to ''smooth muscle tumor of uncertain malignant potential'' to ''leiomyosarcoma'' reflecting the different criteria of malignancy utilized by various authors to histologically classify these tumours. 8,11 The presence of only moderate cytologic atypia and an average mitotic count of 0/10 HPF may categorise the tumour in our patient as a benign leiomyoma.…”

Primary Intracranial Leiomyosarcoma: Review of the Literature and Presentation of a Case

“…Of the 20/37 (54.1%) patients who received postoperative radiotherapy, the dosage, fractionation, administration methods, and tumor responses achieved were not well documented. For7/37(18.9%)patientswhoreceived chemotherapy, the chemotherapy regimens included the administration of vincristineandprocarbazine [13],doxorubicinalone [17,23],acombinationof ifosfamide, carboplatin, and etoposide [26], a combination of vincristine, ifosfamide,doxorubicin,andetoposide [27], orgemcitabinealone [28].…”