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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 1  |  Page : 115-117

Primary intracranial multicentric angioleiomyomas

1 Department of Pathology, B.Y.L Nair Hospital, Mumbai, India
2 Department of Neurology, B.Y.L Nair Hospital, Mumbai, India

Date of Submission18-Oct-2011
Date of Decision14-Nov-2011
Date of Acceptance25-Dec-2011
Date of Web Publication7-Mar-2012

Correspondence Address:
Sweety V Shinde
Department of Pathology, B.Y.L Nair Hospital, Mumbai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.93613

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How to cite this article:
Shinde SV, Shah AB, Baviskar RB, Deshpande JR. Primary intracranial multicentric angioleiomyomas. Neurol India 2012;60:115-7

How to cite this URL:
Shinde SV, Shah AB, Baviskar RB, Deshpande JR. Primary intracranial multicentric angioleiomyomas. Neurol India [serial online] 2012 [cited 2022 Sep 27];60:115-7. Available from: https://www.neurologyindia.com/text.asp?2012/60/1/115/93613


Intracranial angioleiomyoma is an extremely rare tumor. [1],[2],[3],[4],[5] We report a case of multicentric intracranial angioleiomyomas associated with c-ANCA positivity which is not documented till date.

A 60-year-old male patient was admitted with two months' history of progressive headache, seizures and irritability. Examination showed no evidence of neurophakomatoses of tuberous sclerosis, neurofibromatosis Type I or Type II. Neurologic examination showed bilateral proptosis and sluggish reaction of pupil to light. Fundoscopy revealed central retinal vein occlusion. There was bilateral abducens and facial palsy. Motor strength was normal and deep tendon reflexes were preserved. Hemogram, liver and renal function tests were normal. Erythrocyte sedimentation rate (ESR) was 50 mm at the end of one hour. Urine analysis showed many pus cells, 25-30 red cells/high-power field, without casts or proteinuria. Enzyme Linked Immuno Sorbent Assay showed positive cytoplasmic pattern antineutrophil cytoplasmic antibodies (c- ANCA) in 1: 20 titer. Cerebrospinal fluid (CSF) analysis revealed elevated proteins (92 mg/dl), low sugar (10 mg dl), pleocytosis (28 lymphocytes and 02 neutrophils), and tests for syphilis and cryptococci were negative. CSF immunoglobulin was elevated 8.32 mg/100 ml. Magnetic resonance imaging (MRI) of brain showed well-defined lesions in the right putamen and left hippocampus, Both the lesions were iso to hypointense on T1-weighted (T1W) and hyperintense on T2-weighted (T2W) imaging and showed intense homogenous enhancement. Right corona radiate showed similar lesion with surrounding edema. Bilateral fronto-parietal dura was contrast-enhancing suggestive of hypertrophic pachymeningitis [Figure 1] and [Figure 2]. Computed tomography (CT) scan of the thorax showed bilateral ground glass opacities [Figure 3]. Clinical diagnoses included Wegener's granulomatosis, tuberculosis, multicentric glioma and carcinomatous meningitis. Stereotactic biopsy could not be carried out in view of short hospital stay. Patient showed partial clinical improvement with steroids and cyclophosphamide. He terminally developed recurrent seizures and septicemia and died. At autopsy, leptomeninges were thickened over the superolateral and peri-chiasmatic surfaces. The right putamen revealed a 2-cm circumscribed mass [Figure 4]a. Left hippocampus and bilateral optic nerves appeared bulky. Histopathology of the putamen mass showed concentric perivascular and intervascular arrangement of spindle cells with moderate eosinophilic cytoplasm and moderate nuclear pleomorphism. Mitosis, necrosis and microvascular proliferation were absent [Figure 4]b and c. Similar histopathology was seen involving the left hippocampus, bilateral optic nerves and thickened meninges [Figure 5]a-c. Superolateral dura had focal infiltrate of lymphocytes and plasma cells without necrotizing granulomas or vasculitis. Tumor cells were positive for vimentin and smooth muscle actin (SMA) [Figure 4]d, while desmin was variably positive. Glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), CD 31, CD 34 and MIB-1 were negative. A diagnosis of benign multicentric angioleiomyoma was made.
Figure 1: Magnetic resonance imaging showing well-defined lesion in the right putamen (dashed diamond arrow) with surrounding edema. The lesion is iso to hypointense on T1, hyperintense on T2 and homogenously intensely enhancing. Bilateral fronto-parietal leptomeninges show contrast enhancement (thin arrows)

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Figure 2: Magnetic resonance imaging showing well-defined lesion in the left hippocampus (block arrow). The lesion is iso to hypointense on T1, hyperintense on T2 and homogenously intensely enhancing. Right corona radiate shows similar lesion with surrounding edema (star). Bilateral fronto-parietal dura is contrast-enhancing, suggestive of hypertrophic pachymeningitis (arrows)

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Figure 3: Computerized tomography of the lungs showing ground glass opacities in the left postero-basal and right antero-medial segments

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Figure 4: (a) Well-defined reddish granular mass (arrows) occupying the right internal capsule and putamen. (b) Histological section through the mass showing concentric perivascular arrangement of spindle cells with eosinophilic cytoplasm and occasional grooved nuclei (c). The spindle-shaped cells are labeled with antibody to smooth muscle actin on immunohistochemistry (d). b: H and E obj, × 10; c: H and E obj, × 40; d: Immunohistochemistry obj, × 40

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Figure 5: Histopathologic sections through leptomeninges, optic nerve and left hippocampus showing (a) diffuse leptomeningeal angioleiomyomatous proliferation (arrows). (b) Diffuse angioleiomyomatous proliferation in perivascular spaces (block arrows) of the optic nerve and overlying meninges (simple arrows). (c) Dentate granular layer of hippocampus (simple arrow) and angioleiomyomatous proliferation around Virchow-Robin spaces (block arrow). a: H and E obj, × 10; b: H and E obj, × 10; c: H and E obj, × 40

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Intracranial angioleiomyomas are extremely rare lesions. Only two cases [1],[2] were intraaxial while three cases were extraaxial. [1],[3],[4] Unicentric intracranial angioleiomyoma can mimic myopericytoma (desmin negative, CD34+), angiofibroma (SMA negative), angioglioma (GFAP positive), glioblastoma multiforme (GFAP positive, high MIB-1 index) and arteriovenous malformation. [5],[6],[7],[8] Multicentric leptomeningeal angioleiomyoma can mimic meningioangiomatosis (SMA negative, EMA positive), meningioma (EMA positive) and hemangiopericytoma (CD 34+ve). [5],[8] Optic neural and leptomeningeal chiasmatic angioleiomyoma can explain the proptosis, cranial nerve palsy and chiasmatic meningeal enhancement. [2],[3],[4] Positive c-ANCA, CT thorax and microscopic hematuria were suggestive of Wegener's granulomatosis. Partial brain autopsy prevented us from confirming Wegener's in lungs and kidneys. Thus Wegener's remains only a speculation in our patient. Angioleiomyoma may have induced hypergammaglobulinemia. This would explain CSF findings, raised c-ANCA, ESR and dural infiltrates. An autoimmune disorder like Wegener's granulomatosis can be associated with reactive myofibroblastic proliferation as in orbital pseudotumor and retroperitoneal fibrosis. [8]

Gross total resection is possible in solitary angioleiomyoma. None of the cases in the literature reported recurrences for up to a maximum of four years' follow-up. [1],[2],[3],[4] Our patient documents for the first time, angioleiomyoma involving the putamen, hippocampus, optic nerves and leptomeninges. Multicentricity would pose a therapeutic challenge to neurosurgeons. We postulate a probable underlying immune-mediated disease stimulating perivascular myoid proliferation, as well as angioleiomyoma-induced paraneoplastic hypergammaglobulinemia.

  Acknowledgment Top

The authors would like to acknowledge Dr. Shankar S. K., NIMHANS, Bangalore for his valuable assistance in immunohistochemistry and opinion on histopathological diagnosis.

  References Top

1.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. 1
2.Ravikumar C, Veerendrakumar M, Hegde T, Nagaraja D, Jayakumar PN, Shankar SK. Basal ganglionic angioleiomyoma. Clin Neurol Neurosurg 1996;98:253-57.  Back to cited text no. 2
3.Gasco J, Franklin B, Rangel-Castila LR, Campbell GA, Eltorky M, Salinas P. Infratentorial angioleiomyoma: A new location for a rare neoplastic entity. J Neurosurg 2009;110:670-74.  Back to cited text no. 3
4.Figueiredo EG, Gomes M, Vellutini E, Rosemberg S, Marino R. Angioleiomyoma of the cavernous sinus. Neurosurgery 2005;56:411.  Back to cited text no. 4
5.Brook J. Disorders of soft tissue. In: Mills S, Kluver W, editors. Diagnostic Surgical Pathology. 5 th ed. Philadelphia: Lippincott Williams and Williams; pp. 124-197, 2010.  Back to cited text no. 5
6.Rousseau A, Kujas M, van Effenterre R, Boch AL, Carpentier A, Leroy JP, et al. Primary intracranial myopericytoma: Report of three cases and review of the literature. Neuropathol Appl Neurobiol 2005;31:641-8.  Back to cited text no. 6
7.Jesus V, Zurita M, Coca S, Pedrosa M. Cerebral Angiofibroma: Case report. Neurosurgery 2000;46:748-50.  Back to cited text no. 7
8.McKeever P. The Brain, Spinal cord, and Meninges. In: Sternberg S, editor. Diagnostic Surgical Pathology. 3 rd ed. Philadelphia: Lippincott, Williams and Wilkins; 1999. p. 389-482.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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