Intracranial Cystic Myofibroblastoma: a Case Report with Literature Review

Intracranial Cystic Myofibroblastoma: a Case Report with Literature Review

Accepted Manuscript Intracranial Cystic Myofibroblastoma: a Case Report with Literature Review Saksith Smithason, M.D., Richard A. Prayson, M.D., Jeff...

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Accepted Manuscript Intracranial Cystic Myofibroblastoma: a Case Report with Literature Review Saksith Smithason, M.D., Richard A. Prayson, M.D., Jeffry P. Mullin, M.D., M.B.A., J. Jordi Rowe, M.D., Alireza M. Mohammadi, M.D. PII:

S1878-8750(16)30870-1

DOI:

10.1016/j.wneu.2016.09.044

Reference:

WNEU 4584

To appear in:

World Neurosurgery

Received Date: 12 July 2016 Revised Date:

8 September 2016

Accepted Date: 9 September 2016

Please cite this article as: Smithason S, Prayson RA, Mullin JP, Rowe JJ, Mohammadi AM, Intracranial Cystic Myofibroblastoma: a Case Report with Literature Review, World Neurosurgery (2016), doi: 10.1016/j.wneu.2016.09.044. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Intracranial Cystic Myofibroblastoma:

Saksith Smithason, M.D.1 Richard A. Prayson, M.D.2

J. Jordi Rowe, M.D.2

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Jeffry P Mullin, M.D., M.B.A.1

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a Case Report with Literature Review

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Alireza M. Mohammadi, M.D.1

Department of Neurological Surgery, Neurological Institute, Cleveland Clinic, Cleveland, Ohio.

Department of Pathology and Laboratory Medicine, Cleveland Clinic, Cleveland, Ohio,

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Corresponding author: Alireza M. Mohammadi, M.D. Assistant Professor of Surgery (Neurosurgery) Cleveland Clinic Lerner College of Medicine at CWRU Rose Ella Burkhardt Brain Tumor & Neuro-Oncology Center Neurological Institute Cleveland Clinic 9500 Euclid Avenue, S7 Cleveland, Ohio 44195. Phone: 216 445 4290 Fax: 216 444 0924 Email: [email protected]

Key Words: Myofibroblastoma, cystic intracranial tumor, uncommon pathology

ACCEPTED MANUSCRIPT Page 2 Running Title: intracranial myofibroblastoma

Abstract

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Background: Myofibroblastoma is a rare benign soft tissue tumor comprised of

contractile myiod cells mostly in mammary gland. Only four prior cases arising in the central nervous system have been reported in the literature. We present a case of myofibroblastoma with a cystic component.

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Case description: The patient is a 76-year-old male with a history of Parkinson disease. The tumor was found incidentally after a minor fall. MRI revealed a 6.7 cm well

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circumscribed, partly cystic mass with a 2.4 cm calcified central nodule located at the left sylvian fissure. The frontal dural base showed avid enhancing after gadolinium injection. Gross total resection was achieved. The tumor was marked by dense collagenous tissue and bland spindled cells in pathology review. The spindled cells demonstrated positive staining with antibodies to CD34, estrogen receptor, and smooth muscle actin. A blush of immunoreactivity is observed in scattered cells with antibody to progesterone receptor.

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Patient recovered well post operatively.

Conclusion: This is a rare condition of a benign soft tissue tumor of mammary gland presented primarily in the brain. The literature on myofibroblastomas arising in the

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central nervous system is reviewed.

ACCEPTED MANUSCRIPT Page 3 Introduction Myofibroblastoma is a benign soft tissue tumor which can occur as solitary or multicentric lesion, approximately half of all solitary mass occur in the head and neck region,

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followed by trunk and extremities.(1) This tumor is an uncommon tumor of mammary gland.(2) Extra-mammary locations have been occasionally reported.(3-6) Only four

central nervous system cases have been reported.(7-10) This paper reports an additional case which had an unusual cystic component. The literature is reviewed with particular

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attention paid to presentation, pathologic differential diagnosis and treatment.

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Case report History, physical examination and imaging.

A 76-year-old male with a past medical history of Parkinson disease and psoriasis, presented because of an unwitnessed fall. Physical examination revealed baseline hypomimia, upper extremities tremor and rigidity. He was awake and oriented but could not remember how he fell. He had a remote history of brief period of seizure when he

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was in 40s. Antiepileptic medication was administered at the time he arrived at the emergency department. A computed tomographic (CT) scan of the head showed a cystic mass with calcified mural nodule at the left sylvian fissure (Figure 1 A). A magnetic

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resonance imaging (MRI) study of the head with and without contrast disclosed a wellcircumscribed 6.7 cm partially cystic mass with a 2.4 cm central nodule adjacent to the left insula. There was an extensive surrounding edema on the T2/ Flair images. There was

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a susceptibility change in the central nodule suspected for calcification. The mass was slightly hypointense on T1 and isointense on T2. The cyst wall was non-enhancing after gadolinium injection. The central nodule showed heterogenous gadolinium enhancement as well as avid dural enhancing along the frontal base sphenoid ridge area (Figure 1 B-E). The preliminary differential diagnostic considerations of the mass included pleomorphic xanthoastrocytoma, ganglioglioma and metastasis. All blood tumor markers were negative.

ACCEPTED MANUSCRIPT Page 4 Operation and post-operative course

The resection of the mass was performed under frameless stereotactic navigation. The left sylvian fissure was microsurgically opened. Care was taken to identify the M2 and Broca

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area given proximity to the mass. The cyst entered at the middle third of sylvian fissure. Slightly yellowish tainted fluid was drained from the cyst. The central nodule was of

solid consistency with a calcify component and the tumor base was tightly adhered to the frontal base dura. The central nodule was removed in a single piece. The dural base was

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coagulated. The frozen section interpretation suggested either a fibrous meningioma or solitary fibrous tumor. The patient recovered well, returned back to his neurologically

perforator infarction (Figure 2).

Pathological findings

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baseline. MRI post-operative revealed gross total resection with a small mesial MCA

Histologically (Figure 3), the tumor is marked by large areas of dense collagenous tissue

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(Figure 3-A) and small foci of bland spindled cells (Figure 3-B). The spindled cells demonstrate positive staining with antibodies to CD34 (prediluted; Cell Marque, Rocklin, CA), estrogen receptor (predilyed; Ventana, Tucson, AZ) (Figure 3-C), and smooth muscle actin (1:50 dilution; DAKO, Carpinteria, CA) (Figure 3-D). A blush of

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immunoreactivity is observed in scattered cells with antibody to progesterone receptor (prediluted; Ventana). The tumor does not stain (Figure 4) with antibodies to desmin

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(Figure 4-A; 1:10 dilution; DAKO), EMA (Figure 4-B; 1:50 dilution; DAKO), STAT6 (Figure 4-C; 1:200 dilution; Abcam, Cambridge, MA), myogenin (Figure 4-D; prediluted; Ventana), and S-100 protein (1:500 dilution; DAKO). The morphology and immunohistochemical profile are consistent with that of a myofibroblastoma.

ACCEPTED MANUSCRIPT Page 5 Discussion Myofibroma and myofibromatosis are terms used to denote the solitary (myofibroma) or multicentric (myofibromatosis) occurrence of benign neoplasms composed of contractile

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myoid cells arranged around thin-walled blood vessels.(1) Myofibroblastoma is a rare benign mesenchymal mammary neoplasm, largely found in men as a solitary unilateral lesion.(2) These are uncommon tumors found in women.(11) This lesion has many morphological variant and represents less than 1% of breast cancer.(12) Androgen

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were positive for desmin, actin and vimentin.(14)

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receptor or its ligands may be pathologically related to the development.(13) Tumor cells

Series of myofibroblastoma cases were reported extramammary location such as tongue, soft tissue, orbit and superficial lamina propria of the cervix and vagina.(3-6) Electron microscope and immunohistochemistry demonstrated a myofibroblastic origin for a tumor cell.(6) Myofibroblastoma found in soft tissue showed well-defined myofibroblastic differentiation with less than three mitoses per 10 high power field.(4)

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Immunohistological stain positive for vimentin, estrogen, progesterone and desmin.(5) Orbital and intraocular myofibroblastoma stained positive for desmin and CD34.(3) By immunohistochemistry, there was a link between cellular angiofibroma, spindle cell

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lipoma and myofibroblastoma. (15)

Only four cases CNS myofibroblastoma have been reported (Table 1). Shinojima et al

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reported a tumor at suprasellar region, gross tumor was very similar to fibrous meningioma.(9) Surgery was performed via a right frontobasal translamina-terminalis approach with subtotal removal of the tumor and patient’s vision improved immediately. (9) Histological examination showed mixture of spindle shaped and round cells in collagen matrix. Immunohistostaning demonstrated strong vimentin and actin.(9) Prayson et al reported posterior falcine tumor with visual symproms removed through occipital craniotomy with some improvement of vision. Patient died a month later because of pulmonary embolism. Tumor stained negative for EMA (epithelial membrane antigen), cytokeratin, S-100, desmin, myosin, GFAP and factor VII related antigen.(7) Xu et al

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tumor most likely arises from myofibroblast that reside in the meninges.(7)

The differential diagnosis of meningeal myofibroblastoma are solitary fibrous tumor of meninges, fibrous meningioma and hemagiopericytoma.(10) Differentiation can be achieved through viewing their ultrastructure and using immunohistochemical

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technique.(16) Meningeal hemangiopericytoma exhibits a distinct antigenic profile with a rare expression of desmin or cytokeration.(17) Another spectrum of

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hemogiopericytoma is a solitary fibrous tumor of the meninges which presents as dural base masses, resembling fibrous meningioma.(18) Solitary fibrous tumor showed diffuse CD-34 immunoreactivity, STAT 6 and reticulin fibers. Neither EMA nor S-100 protein were positive.(19)

Perhaps the greatest challenge in making a pathologic diagnosis of myofibroblastoma is

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distinguishing it from other similar appearing spindled lesions. Fibrous meningiomas typically are more cellular than the current tumor with nuclei that are more cigarshaped. Most meningiomas demonstrate focal immunoreactivity with antibodies to EMA and PR (progesterone receptor); myofibroblastomas typically do not stain with EMA but

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may also demonstrate PR immunoreactivity as was noted in the current case. Lesions along the solitary fibrous tumor-hemangiopericytoma spectrum may be marked by

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spindled cells with increased collagen, typically deposited between individual cells. By immunostaining, these tumors typically demonstrate CD34 immunoreactivity in the tumor cells (not just in blood vessel walls) and STAT 6; myofibroblastomas generally do not stain with these antibodies. Schwannomas usually demonstrate diffuse S-100 protein staining, a pattern not observed in myofibroblastomas. Myofibroblastomas may variably stain with markers of muscle differentiation such as smooth muscle actin. Gliosarcomas may have a spindled cell mesenchymal component to them; often these tumors have focal areas of glioblastoma (which would be glial fibrillary acidic protein positive) and typically demonstrate features of a high grade neoplasm including necrosis and readily

ACCEPTED MANUSCRIPT Page 7 identifiable mitotic figures. Sarcomas would also demonstrate features of higher grade neoplasm (necrosis, pleomorphism and prominent mitotic activity).

This study adds valuable data for this rare CNS tumor. More importantly, this is the first

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cystic myofibroblastoma reported in the literature. Tumor histologically revealed dense collagen and spindle cells. Immunohistochemistry staining positive for CD34, estrogen receptor and smooth muscle actin. Tumor does not stain with EMA, S-100, desmin,

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myogenin or STAT6. These profiles concordant with myofibroblastoma.

ACCEPTED MANUSCRIPT Page 8 References 1.

Fletcher CDM, Unni KK: World Health Organization Classification of Tumours Pathology and Genetics of Tumours of Soft Tissue and Bon Edited by. World

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Powari M, Srinivasan R, Radotra BD: Myofibroblastoma of the male breast: a diagnostic problem on fine-needle aspiration cytology. Diagn Cytopathol

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2002;26:290–293

Costin BR, Plesec TP, Rubinstein TJ, Medina CA, Singh AD, Goldblum JR,

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Herrera GA, Johnson WW, Lockard VG, Walker BL: Soft tissue myofibroblastomas. Mod Pathol 1991;4:571–577

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Laskin WB, Fetsch JF, Tavassoli FA: Superficial cervicovaginal

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myofibroblastoma: fourteen cases of a distinctive mesenchymal tumor arising from the specialized subepithelial stroma of the lower female genital tract. Hum Pathol 2001;32:715–725

Sahin AA, Ro JY, Ordoñez NG, Luna MA, el-Naggar AK, Goepfert H, Ayala

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and flow cytometric study. Am J Clin Pathol 1990;94:773–777

Prayson RA, Estes ML, McMahon JT, Kalfas I, Sebek BA: Meningeal myofibroblastoma. Am J Surg Pathol 1993;17:931–936

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Prayson RA, McMahon JT, Barnett GH: Solitary fibrous tumor of the meninges. Case report and review of the literature. J Neurosurg 1997;86:1049–1052

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Shinojima N, Ohta K, Yano S, Nakamura H, Kochi M, Ishimaru Y, Nakazato Y,

ACCEPTED MANUSCRIPT Page 9 Ushio Y: Myofibroblastoma in the suprasellar region. Case report. J Neurosurg

2002;97:1203–1207 10.

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A case report. Oncol Lett 2014;8:1291–1294

Hamele-Bena D, Cranor ML, Sciotto C, Erlandson R, Rosen PP: Uncommon

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D’Ambrosio G, De Laurentis F, Scoglio D, Balla A, Quaresima S, Mattei F,

Chir 2013;84:5–7

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Lezoche E: Breast myofibroblastoma in a young woman: a case report. Ann Ital

Morgan MB, Pitha J V: Myofibroblastoma of the breast revisited: An etiologic association with androgens? Hum Pathol 1998;29:347–351

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Salomão DR, Crotty TB, Nascimento AG: Myofibroblastoma and solitary fibrous

2001;10:49–54

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tumour of the breast: histopathologic and immunohistochemical studies. Breast

Flucke U, van Krieken JHJM, Mentzel T: Cellular angiofibroma: analysis of 25

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cases emphasizing its relationship to spindle cell lipoma and mammary-type

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myofibroblastoma. Mod Pathol 2011;24:82–89

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Yilmaz C, Kabatas S, Ozen OI, Gulsen S, Caner H, Altinors N: Solitary fibrous tumor. J Clin Neurosci 2009;16:1578–1581

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Perry A, Scheithauer BW, Nascimento AG: The immunophenotypic spectrum of meningeal hemangiopericytoma: a comparison with fibrous meningioma and solitary fibrous tumor of meninges. Am J Surg Pathol 1997;21:1354–1360

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Tihan T, Viglione M, Rosenblum MK, Olivi A, Burger PC: Solitary fibrous tumors in the central nervous system. A clinicopathologic review of 18 cases and comparison to meningeal hemangiopericytomas. Arch Pathol Lab Med

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2003;127:432–439

Suzuki SO, Fukui M, Nishio S, Iwaki T: Clinicopathological features of solitary fibrous tumor of the meninges: An immunohistochemical reappraisal of cases

previously diagnosed to be fibrous meningioma or hemangiopericytoma. Pathol

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Figure 1. Non contrast CT shows calcify cystic mass at left sylvian fissure(A). Coronal

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T1 shows enhancing mural nodule(B). Avid enhancing dural tail without cyst wall enhancement(C). Surrounding edema on T2 and Flair images (D). Susceptibility changes

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on SWI (E).

Figure 2. Post operative coronal MRI shows gross total resection with residual blood

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product in the cyst (A). Diffusion weight images shows mesial infarction from perforators injury (B left). Pre operative MRI shows proximity of the tumor to MCA (B right).

Figure 3. Dense area of collagen tissue(A). Small foci of spindle cell(B). Spindle cell

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shows positive staining with antibodies CD34 and estrogen(C). Positive staining with antibody smooth muscle actin(D).

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Figure 4. Negative staining results for Desmin (A), EMA (B), STAT6 (C), and Myogenin (D) in tumor tissue

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Table reference(1)

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Table 1. Literature review of prior cases report. Age 9

Symptoms Diplopia

Location Meninges

Prayson RA, Estes ML, McMahon JT, Kalfas I, Sebek BA: 19937

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Headache

Bilateral Posterior falx

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Shinojima N, Ohta K, Yano S, Nakamura H, Kochi M, Ishimaru Y, et al: 20029

34

Headache

Suprasellar

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Xu Q, Feng Y, Wu P, Zhou Y: 201410

47

Present study

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Frontal lobe

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Incidental

Insular,sylvian

6.7

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Size (cm) 1.2

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Author Carneiro F, Goncalves V, Simoes MS: 198320

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Myofibroblastoma is a rare benign soft tissue tumor comprised of contractile myiod cells mostly in mammary gland. Only four prior cases arising in the central nervous system have been reported in the literature. We present a case of myofibroblastoma with a cystic component. This is a rare condition of a benign soft tissue tumor of mammary gland presented primarily in the brain. The literature on myofibroblastomas arising in the central nervous system is reviewed.

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Abbreviations CT – computed tomography MRI – magnetic resonance imaging

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EMA – epithelial membrane antigen

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PR – progesterone receptor