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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2017  |  Volume : 28  |  Issue : 4  |  Page : 921-924
Primary intracranial leiomyoma in renal transplant recipient


1 Department of Neurology, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Nephrology, Sir Ganga Ram Hospital, New Delhi, India

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Date of Web Publication21-Jul-2017
 

   Abstract 


Leiomyoma, the benign tumor of smooth muscle cell origin, is commonly seen in genitourinary and gastrointestinal tracts. Primary intracranial leiomyoma, however, is extremely rare occurrence. We hereby report a case of Epstein-Barr negative primary intracranial leiomyoma in a middle-aged renal transplant recipient, which mimicked left frontal parasagittal meningioma on neuroimaging. The tumor was completely excised and diagnosis of leiomyoma was clinched on pathological analysis with immunohistochemistry. The patient improved after tumor removal, and no evidence of tumor recurrence was noted on follow-up study after 10 months postsurgically.

How to cite this article:
Patel U, Patel N. Primary intracranial leiomyoma in renal transplant recipient. Saudi J Kidney Dis Transpl 2017;28:921-4

How to cite this URL:
Patel U, Patel N. Primary intracranial leiomyoma in renal transplant recipient. Saudi J Kidney Dis Transpl [serial online] 2017 [cited 2019 Aug 23];28:921-4. Available from: http://www.sjkdt.org/text.asp?2017/28/4/921/211329



   Introduction Top


Primary intracranial leiomyoma is an exceedingly rare benign mesenchymal neoplasm, and very few cases have been reported. Such cases have been reported in both immuno-compromised and immunocompetent patients, human immunodeficiency virus (HIV) being the most common association in the former category. Only one of Epstein-Barr virus (EBV)-associated primary intracranial leiomyoma in renal transplant recipient has been reported.[1] Ours is the first case of EBV-negative primary intracranial leiomyoma in renal transplant recipient after four years of transplant. Similarity with meningioma radiologically in such cases can mislead the diagnosis, underlining the importance of detailed immunohistochemical analysis of the biopsy tissue.


   Case Report Top


A 43-year-old Indian male presented to our hospital with a one-month history of intermittent left frontal throbbing headache with nausea, followed by recurrent generalized tonic-clonic seizures. He had undergone living donor renal allograft four years earlier due He received long-term immunosuppressive treatment comprising tacrolimus, mycophenolate mofetil and prednisolone. He developed biopsy-proven chronic allograft nephropathy two years after transplantation. Neurological examination showed mild right hemiparesis with the power of grade of 4/5. Magnetic resonance imaging of brain revealed a 2.73 cm × 1.20 cm dural-based extra-axial mass in the left high frontal region with significant perilesional edema. The mass was homogeneously hypointense on T1-, T2-, fluid attenuated inversion recovery, and diffusion-weighted sequences [Figure 1]. In view of chronic allograft nephropathy, we did not give gadolinium contrast so as to prevent nephrogenic systemic fibrosis. Preoperative noncontrast computerized tomography of chest and abdomen with pelvis in search of evidence of tumor anywhere else was nonrevealing. HIV, hepatitis B, and hepatitis C virus serology were negative.
Figure 1: Magnetic resonance imaging brain showing a left frontal primary intracranial leiomyoma in T2-weighted sagittal (a), coronal (b), axial (d), T1-weighted (c) and fluid attenuated inversion recovery (e) sequences.

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The tumor was completely excised after left frontal craniotomy. Histology of the specimen showed well-encapsulated spindle cell tumor, arranged in interlacing fascicles and bundles. No necrosis, mitotic figures, or cell pleomorphism were found. Immunohistochemistry (IHC) displayed tumor cell immunoreactivity to smooth muscle actin (SMA). They were negative for S-100, CD-34, and epithelial membrane antigen immunostaining [Figure 2] and [Figure 3]. The diagnosis of benign leiomyoma was made. EBV-specific IgM serology and EBV DNA polymerase chain reaction (PCR) were found to be negative.
Figure 2: Well-encapsulated spindle cell tumor arranged in interlacing fascicles and bundles (H and E, ×5) (arrow showing capsule and inset showing benign appearing spindle cells (H and E, ×10)].

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Figure 3: Immunohistochemistry tumor cells are positive for smooth muscle actin. They are negative for S-100, CD 34 and epithelial membrane antigen.

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The patient improved after tumor removal with no further seizures or headache. There was no evidence of tumor recurrence on follow-up of 10 months.


   Discussion Top


Primary intracranial leiomyoma was first reported by Kroe et al in a 68-year-old woman in intrasellar region.[2] Since then, quite a few cases have been reported in the literature in both immunocompromised and immunocompetent patient populations. Till date, a total of six cases have been reported with primary intracranial leiomyoma with underlying immunocompromised status, among which only one case was reported in solid organ transplant (SOT) recipient.[1],[3],[4],[5],[6],[7]

Out of these six patients, one SOT recipient and three HIV-positive patients were associated with EBV infec-tion.[1],[5],[6],[7] The case reported by Citow and Kranzler with HIV-positive status was considered nondiagnostic, as their conclusion was based on the absence of staining for EBV latent membrane protein-1 (LMP-1. In situ hybridization, the most sensitive and definitive method to demonstrate EBV in smooth-muscle cell tumors (SMT), was not performed. Because EBV-associated SMTs are generally LMP-1-negative, an association with the virus in this particular patient cannot be definitively excluded.[4] Bargiela et al did not test for EBV status in their HIV-positive patient.

In our patient, EBV-PCR and EBV-specific IgM were found to be negative. However, we could not do IHC and in situ hybridization for EBV in biopsy specimen. IgM and IgG antibodies directed against EBV-specific antigen have high sensitivity and specificity for the diagnosis (97 and 94%, respectively).[8] The sensitivity and specificity of EBV-DNA PCR has been 95.7 and 100%, respectively in available data.[9],[10],[11] Moreover, EBV-associated post-transplant lymphoproliferative disorder is well known to be associated with high plasma EBV viral load in the vast majority.[12] Considering these data, we believe that our case was not associated with EBV infection.

Primary intracranial leiomyomas affect both sexes equally, with the age of presentation usually in the second and third decade of life.[13] Leiomyomas appear homogenous iso-or hypo-intense on T1-weighted imaging and heterogeneous hyper- or hypo-intense lesion on T2-weighted imaging; hypointensity is reported most commonly.[3],[4],[13],[14],[15],[16],[17] Imaging, however, may be confused with that of meningioma as in our case and diagnosis requires IHC of biopsy specimen. On immunostaining, a leiomyoma will be positive for SMA, desmin, myosin, and vimentin, and negative for S100.[13],[18] In contrast to leiomyosarcoma, a more benign leiomyoma shows spindle-shaped cells with blunt ends and few mitotic figures in histology.[13]

Being a tumor with well-defined boundary, complete surgical excision generally is the treatment of choice, except in cases with invasion into important neurovascular structures such as cavernous sinus. Artificial dural graft is required in addition if it involves the dura matter. Given a slow-growing nature of this tumor and prolonged recurrence time frame, long-term follow-up of the patient after surgery is desired. Even in the case of partial excision, radiotherapy is not recommended for the remnant lesion as the tumor is radio-resistant and may even cause radiation-induced damage, as reported in one case.[19] However, no definitive recommendations regarding optimal management can be derived from the existing scarce data on primary intracranial leiomyoma in SOT recipients and need further research in this direction.

Conflict of interest: None declared.



 
   References Top

1.
Zevgaridis D, Tsonidis C, Kapranos N, Venizelos I, Tsitsopoulos P, Tsitsopoulos P. Epstein-Barr virus associated primary intracranial leiomyoma in organ transplant recipient: Case report and review of the literature. Acta Neurochir (Wien) 2009;151:1705-9.  Back to cited text no. 1
    
2.
Kroe DJ, Hudgins WR, Simmons JC, Blackwell CF. Primary intrasellar leiomyoma. Case report. J Neurosurg 1968;29:189-91.  Back to cited text no. 2
    
3.
Bargiela A, Rey JL, Díaz JL, Martínez A. Meningeal leiomyoma in an adult with AIDS: CT and MRI with pathological correlation. Neuroradiology 1999;41:696-8.  Back to cited text no. 3
    
4.
Citow JS, Kranzler L. Multicentric intracranial smooth-muscle tumor in a woman with human immunodeficiency virus. Case report. J Neurosurg 2000;93:701-3.  Back to cited text no. 4
    
5.
Karpinski NC, Yaghmai R, Barba D, Hansen LA. Case of the month: March 1999 – A 26 year old HIV positive male with dura based masses. Brain Pathol 1999;9:609-10.  Back to cited text no. 5
    
6.
Kleinschmidt-DeMasters BK, Mierau GW, Sze CI, et al. Unusual dural and skull-based mesenchymal neoplasms: A report of four cases. Hum Pathol 1998;29:240-5.  Back to cited text no. 6
    
7.
Kumar S, Santi M, Vezina G, Rosser T, Chandra RS, Keating R. Epstein-Barr virus-associated smooth muscle tumor of the basal ganglia in an HIV+ child: Case report and review of the literature. Pediatr Dev Pathol 2004;7:198-203.  Back to cited text no. 7
    
8.
Bruu AL, Hjetland R, Holter E, et al. Evaluation of 12 commercial tests for detection of Epstein-Barr virus-specific and heterophile antibodies. Clin Diagn Lab Immunol 2000; 7:451-6.  Back to cited text no. 8
    
9.
Bauer CC, Aberle SW, Popow-Kraupp T, Kapitan M, Hofmann H, Puchhammer-Stöckl E. Serum Epstein-Barr virus DNA load in primary Epstein-Barr virus infection. J Med Virol 2005;75:54-8.  Back to cited text no. 9
    
10.
Berth M, Vanheule G, Depuydt C, Benoy I. Serum Epstein-Barr virus (EBV) viral load can be a complementary sensitive test in primary Epstein-Barr virus infection. J Clin Virol 2011; 50:184-5.  Back to cited text no. 10
    
11.
Berger C, Day P, Meier G, Zingg W, Bossart W, Nadal D. Dynamics of Epstein-Barr virus DNA levels in serum during EBV-associated disease. J Med Virol 2001;64:505-12.  Back to cited text no. 11
    
12.
Wagner HJ, Wessel M, Jabs W, et al. Patients at risk for development of posttransplant lymphoproliferative disorder: Plasma versus peripheral blood mononuclear cells as material for quantification of Epstein-Barr viral load by using real-time quantitative polymerase chain reaction. Transplantation 2001;72:1012-9.  Back to cited text no. 12
    
13.
Hua W, Xu F, Mao Y, et al. Primary intracranial leiomyomas: Report of two cases and review of the literature. Clin Neurol Neurosurg 2009;111:907-12.  Back to cited text no. 13
    
14.
Ali AE, Fazl M, Bilbao JM. Primary intracranial leiomyoma: A case report and literature review. Virchows Arch 2006;449:382-4.  Back to cited text no. 14
    
15.
Dorwal P, Kaul S, Arora D, Prasad R. Intracranial leiomyoma in a male patient. Indian J Pathol Microbiol 2010;53:837-9.  Back to cited text no. 15
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16.
Wang KC, Kim CJ, Cho BK, Kim IO, Lee HJ, Chi JG. Cerebral leiomyoma in a child. J Korean Med Sci 1997;12:378-82.  Back to cited text no. 16
    
17.
Lai PH, Yang CF, Huang CH, Yeh LR, Lin SL, Pan HB. Primary intracranial leiomyoma: Case report. Neuroradiology 1998;40:238-41.  Back to cited text no. 17
    
18.
Lach B, Duncan E, Rippstein P, Benoit BG. Primary intracranial pleomorphic angioleiomyoma – A new morphologic variant. An immunohistochemical and electron microscopic study. Cancer 1994;74:1915-20.  Back to cited text no. 18
    
19.
Jakobiec FA, Howard GM, Rosen M, Wolff M. Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol 1975;80:1028-42.  Back to cited text no. 19
    

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Correspondence Address:
Upasana Patel
Department of Neurology, Sir Ganga Ram Hospital, New Delhi - 110 060
India
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PMID: 28748899

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