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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 3  |  Page : 664-666

Dorsolumbar spine Epstein Barr virus associated leiomyosarcoma in a human immunodeficiency virus patient

1 Department of Neurosurgery, PD Hinduja National Hospital and Research Center, Mumbai, Maharashtra, India
2 Department of Pathology, PD Hinduja National Hospital and Research Center, Mumbai, Maharashtra, India

Date of Web Publication9-May-2017

Correspondence Address:
Satyashiva Munjal
Department of Neurosurgery, PD Hinduja National Hospital and Research Center, Mumbai - 400 016, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/neuroindia.NI_1_17

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How to cite this article:
Munjal S, Warade A, Samantray S, Madiwale C, Desai K. Dorsolumbar spine Epstein Barr virus associated leiomyosarcoma in a human immunodeficiency virus patient. Neurol India 2017;65:664-6

How to cite this URL:
Munjal S, Warade A, Samantray S, Madiwale C, Desai K. Dorsolumbar spine Epstein Barr virus associated leiomyosarcoma in a human immunodeficiency virus patient. Neurol India [serial online] 2017 [cited 2022 Oct 7];65:664-6. Available from: https://www.neurologyindia.com/text.asp?2017/65/3/664/205880


Leiomyosarcoma (LMS) is a malignant mesenchymal tumor with myogenic components.[1] It commonly involves the gastrointestinal tract and the genitourinary system.[2] Central nervous system (CNS) involvement by LMS is more common by metastatic spread.[1] We report a case of dorsolumbar spinal Epstein Barr virus (EBV) associated LMS in an immunocompromised patient, and the literature is briefly reviewed.

A 33-year old human immunodeficiency virus (HIV) infected female patient presented to us with complaints of low back pain for 2 months. The backache was insidious in onset, of dull-aching type, localized, and not radiating to the lower limbs. There was no associated history of trauma. Over the last 1 month, the motor weakness in the lower limbs had rapidly worsened. On examination, there was complete flaccid paraplegia. She had urinary retention for which an in-dwelling catheter had been inserted. The deep tendon reflexes were absent in the lower limbs, and the plantar reflex was not demonstrated bilaterally. There was no sensory deficit. The HIV-1 viral titer was 9880 copies/ml and CD4 count was 134/mm 3. The magnetic resonance imaging (MRI) scan of the thoracolumbar spine revealed a 6.5 × 1.4 × 3 cm extradural mass lesion extending from the D10 to L1 vertebral levels. A portion of the tumor also extended to the D12-L1 neural foramina on the left side. The tumor was isointense on T1-weighted and hypointense on T2-weighted images and showed a homogenous enhancement following contrast administration [Figure 1]. D10- L1 levels laminectomies were performed. The tumor was extradural, firm, well-encapsulated and avascular, and had caused severe compression of the thecal sac displacing it towards the right side. It was densely adherent to the dura and the L1 nerve root on the left side [Figure 2]. Near- total excision of the tumor was performed and a small sleeve of the tumor densely adherent to the L1 root on the left side was left behind.
Figure 1: MRI of the dorsolumbar spine reveals an extradural tumor at D10-L1 vertebral levels. (a) Axial T1-weighted image showing an isointense lesion. (b) Axial T2-weighted image showing the lesion to be hypointense. (c) Axial T1-weighted gadolinium enhanced image showing its homogenous enhancement. (d) Sagittal T1-weighted gadolinium enhanced image revealing its homogenous enhancement

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Figure 2: Intraoperative image showing an extradural tumor with compression and displacement of the dural sac towards the right side

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The histopathological examination of the tumor specimen revealed a fairly cellular, spindle cell tumor showing interlacing fascicles of tumor cells, punctuated by multiple scattered foci of coagulative necrosis and occasional foci of hydropic change. The tumor cells had blunt ended nuclei with no significant atypia [Figure 3]. The necrosis occupied approximately 30% of the total sectional area. Mitosis documented were <2/10 high power fields. Immunohistochemistry [Figure 4] demonstrated the tumor to be strongly positive for smooth muscle actin (SMA) and H caldesmon. It had focal areas which were positive for desmin. In-situ hybridization of the paraffin section of the tumor specimen demonstrated expression of EBV-encoded small RNA-1 (EBER-1). The histopathologic diagnosis was an EBV associated leiomyosarcoma in an HIV infected patient. Positron emission tomography (PET) computerized tomography (CT) scan showed uptake in the right lung and liver. Antiretroviral combination drug therapy was started. In consultation with the oncologist, the adjuvant radiotherapy and chemotherapy were deferred until recurrence in the dorsolumbar spine or increase in the lesions in liver and lung were documented. This decision was taken in view of the tumor relationship with the EBV infection. At 1 month of follow-up, she had motor power of grade 2 in the lower limbs proximally at the hips and knee joints. There was no associated sphincteric involvement.
Figure 3: (a and b) Histopathology image showing tumor composed of interlacing fascicles of spindle cells with areas of coagulative necrosis (H and E, x400)

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Figure 4: Immunohistochemistry (IHC) results: Tumor cells showed diffuse positivity for (a) H caldesmon. (b) Desmin. (c) Smooth muscle actin. (d) EBER-ISH

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Leiomyosacomas originate from smooth muscle cells and form approximately 7% of soft tissue sarcomas.[1] The commonly known LMS in immunocompetent individuals originate in the genito- urinary system and the gastrointestinal tract.[1],[2],[3],[4],[5]

The incidence of smooth muscle tumors (SMTs) like a leiomyoma or a leiomyosarcoma in immunocompromised individuals has increased over the last few decades.[2] They are found in patients with HIV infection, as well as the organ transplant recipients who are on immuno- suppressive drugs.[6] They are now the second most common tumors in children who are suffering from acquired immunodeficiency syndrome (AIDS) following an HIV infection.[3] Their occurrence in adults with an immunocompromised state is still extremely rare.[3]

EBV, a member of the Herpes virus family, has been documented to be associated with Hodgkin's lymphoma, non-Hodgkin lymphoma, and nasopharyngeal carcinoma.[7],[8] In immunocompromised individuals, it has been documented to be associated with smooth muscle tumors such as a leiomyoma or a leiomyosarcoma.[5],[6] The association of EBV infection and SMT was first reported in a child in 1990. Since then, there has been an increase in the number of SMTs in children with an immunocompromised state. In contrast, a similar trend was not observed in adults with an impaired immunity secondary to the HIV infection or following administration of immunosuppressive drugs. The association of EBV in LMS can be demonstrated in plasma by real-time quantitative polymerase chain reaction, by assessing the anti-EBV nuclear antigen (EBNA) IgG levels and by in-situ hybridization of the paraffin section of the tumor with expression of EBV-encoded small RNA-1 (EBER-1).[6]

The LMSs occurring in immunocompetent individuals are focal in origin and metastases are common to the lungs, liver, and bones.[2],[6] The occurrence of metastasis in the CNS is rare.[3] In contrast, the EBV associated LMS has unique characteristics, such as a multicentric involvement, either concurrently or sequentially.[3],[6] They commonly involve the lung, liver, vocal cords, abdominal wall, and adrenal gland. CNS involvement, although extremely rare, has been found to have a predilection for the cranial and spinal epidura.[6],[9] A summary of the reported cases of spinal leiomyosarcoma is given in [Table 1].
Table 1: Summary of reported cases of spinal leiomyosarcoma

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The LMS in the CNS tends to arise from the mesenchymal elements within the blood vessels of bone, dura, and subarachnoid space. In the spine, the LMS can either arise from the intraosseous vascular smooth muscle cells that are capable of smooth muscle differentiation or can also arise from the paraspinal muscles that erode the spine or involve the nerve roots. The spinal LMSs generally mimic nerve sheath tumors, and myelopathy is the common presenting clinical finding.[3] In immunocompromised patients, the differential diagnosis of LMS should definitely be considered with other spinal extradural lesions.[3],[6]

The molecular study by Deyrup et al., documented that multifocal tumors located at different sites have different clones of EBV.[9] This confirms the multifocal nature of LMSs in immunocompromised patients.

The majority of EBV associated LMS in immunocompromised patients are well-differentiated tumors with a mild degree of polymorphism of the nuclei and a low level of mitotic activity.[2],[6] However, the aggressiveness of the LMS in immunocompromised individuals is determined by the immune status rather than by histological features.[2],[3],[6]

The EBV associated LMS in immunocompromised patients are relatively less aggressive when compared with LMS that occurs in immunocompetent patients.[6] The extent of excision and the role of adjuvant therapy such as radiotherapy and chemotherapy are debatable.[3],[6] Improvement in the immune status either by retroviral therapy in HIV infected cases, or by reduction in the dosage of the immunosuppressant drugs, definitely helps in achieving a better outcome.

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 » References Top

Ido K, Matsuoka H, Yoshida M, Urushidani H. Paraparesis due to spinal leiomyosarcoma lesion in the thoracic spine accompanied by two leiomyosarcoma lesions in the back and the thigh over an interval of 4 years. J Clin Neurosci 2002;9:325-8.  Back to cited text no. 1
Issarachaikul R, Shuangshoti S, Suankratay C. Epstein-Barr virus-associated smooth muscle tumors in AIDS patients: A largest case (series). Intern Med 2014;53:2391-6.  Back to cited text no. 2
Suankratay C, Shuangshoti S, Mutirangura A, Prasanthai V, Lerdlum S, Shuangshoti S, et al. Epstein-Barr virus infection-associated smooth-muscle tumors in patients with AIDS. Clin Infect Dis 2005;40:1521-8.  Back to cited text no. 3
Nishida J, Kato S, Shiraishi H, Ehara S, Sato T, Okada K, et al. Leiomyosarcoma of the lumbar spine: Case report. Spine 2002;27:E42-6.  Back to cited text no. 4
Gupta A, Moorthy RK, Prabhu AJ, Rajshekhar V. Lumbar paraspinal primary high-grade leiomyosarcoma mimicking an extraforaminal schwannoma. Neurol India 2016;64:1071-4.  Back to cited text no. 5
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Purgina B, Rao UNM, Miettinen M, Pantanowitz L. AIDS-related EBV-associated smooth muscle tumors: A review of 64 published cases. Patholog Res Int 2011;2011:561548  Back to cited text no. 6
Elhammady MSA, Manzano GR, Lebwohl N, Levi AD. Leiomyosarcoma metastases to the spine: Case series and review of the literature. J Neurosurg Spine 2007;6:178-83.  Back to cited text no. 7
Ritter AM, Amaker BH, Graham RS, Broaddus WC, Ward JD. Central nervous system leiomyosarcoma in patients with acquired immunodeficiency syndrome. Report of two cases. J Neurosurg 2000;92:688-92.  Back to cited text no. 8
Deyrup AT, Lee VK, Hill CE, Cheuk W, Toh HC, Kesavan S, et al. Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events. A clinicopathologic and molecular analysis of 29 tumors from 19 patients. Am J Surg Pathol 2006;30:75-82.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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