Multiple plexiform schwannomas in the plantar aspect of the foot: case report and literature review
© Li et al.; licensee BioMed Central Ltd. 2014
Received: 5 June 2014
Accepted: 6 October 2014
Published: 11 October 2014
Plexiform schwannoma (PS) is a rare, peripheral nerve sheath tumor arranged in a plexiform pattern.
We report an unusual case of a 19-year-old woman, who complained of pain in the plantar aspect of the left foot. Magnetic resonance image (MRI) demonstrates three solitary nodules of varying sizes in the deep soft tissue of the plantar aspect of the foot that are homogeneously isointense to skeletal muscle on T1-weighted images and hyperintense on T2-weighted fat-suppressed images, especially the rim of the lesion. Subsequent pathological examination confirmed the diagnosis of PS.
MRI characteristic plays an important role in detecting this rare lesion. A review of the literature on PS is also presented.
KeywordsPlexiform schwannoma Neurofibromatosis Magnetic resonance imaging
Schwannoma is a benign, peripheral nerve sheath tumor, occurring commonly in the persons of 20–50 years old , and manifests about 5% of benign soft-tissue neoplasm . Schwannomas usually involve in head, neck, flexor surfaces of extremities and nerves [2, 3]. Plexiform schwannoma (PS) is a rare distinctive variant, accounting for 2-5% of all schwannomas [4–6]. It frequently affects patients aged 30–40 years with no sex predilection, and occurs commonly in the dermis and subcutis with a predilection for head and neck . Up to now only 11 cases located to the foot have been reported and published in English medical literature [8–11]. Multiple PSs in the plantar aspect of the foot are very rare. The diagnosis of PS mainly depends on histopathologic and immunohistochemical features. The MR imaging features of PS, however, were described only partially by some case reports . If the imaging features can be recognized, it would be the helpful for preoperative diagnosis. The current paper aims to demonstrate distinct imaging features of PS in the plantar aspect of the foot which may be vital to facilitate the diagnosis process in the future and to give a short literature review.
After operation, the patient reported hypoesthesia in the distant third and fourth toes. The patient was followed up for two and a half years postoperatively, and there was no evidence of local recurrence or new lesions at other parts of the body.
PS is a rare variant of schwannoma . It occurs mostly as a solitary lesion in the skin or subcutaneous tissue, or uncommonly located in the deep soft tissue . Most PSs are small with a maximal diameter less than 2 cm, originating from the superficial nerves. Trauma may play an etiological role in the formation of this lesion [14, 15]. Since up to 5% of cases are believed to be associated with NF-2 [3, 10, 16–19], careful intracranial and spinal MR examinations are required to exclude the potential NF-2 in young patients .
MRI is a useful tool in detecting soft tissue neoplasms. MRI is also used in the diagnosis of multiple PSs [10, 11]. Grossly, the rim of the PS nodule is smooth. The MRI features of this tumor are similar to peripheral nerve sheath tumors including heterogeneous hypo- or isointensity compared with surrounding muscles on T1-weighted MR images, and heterogeneous hyper- or isointensity to subcutaneous fat on T2-weighted MR images [21–23]. It is reported that multinodular growth pattern in a single lesion and evident cystic degeneration on T2 weighted image are the characteristics of PS, which is helpful in the differential diagnosis [24, 25]. However, confusing results were reported. Ikushima et al.  failed to delineate the multinodular architecture in PS of the foot based upon the preoperative CT and MRI. Multinodular pattern was not also observed in the current case. Therefore, we present this rare case as an effort to further characterize the lesion and to facilitate the diagnosis of similar cases.
In the current study, MRI showed three round-shape nodules located in the plantar aspect of the foot. These nodules were not scattered and they had a peculiar linear distribution originating from the branches of the common plantar digital nerves. The MR signal of these nodules was homogeneous, maybe partially due to the small size of the nodule. No evident cystic degeneration was noted in any of the three lesions, which were different from conventional schwannomas. The mass of the solitary nodule was surrounded by a hyperintense rim on T2-weighted images with fat suppression, which have not been reported and required further investigation to identify the reason. Particularly, the surrounding nerve of the tumor appeared like a mouse-tail, which implied the neurogenic tumor.
It is important to differentiate PS from plexiform neurofibroma, because there are some similarities in clinical and imaging characteristics of them. In contrast to PS, plexiform neurofibroma originating from peripheral nerves usually occurs in early childhood with a positive family history of neurofibromatosis [4, 26, 27], and has a significant risk of malignant transformation (4%), which has not been reported to occur in PS [26, 28].
Some authors reported [18, 29] that plexiform neurofibroma is similar to PS that presents on imaging with intermediate signal intensity to the skeletal muscle on T1-weighted images and hyperintensity on T2-weighted images. However, there are some imaging features which can distinguish PS from plexiform neurofibroma. Plexiform neurofibromas are essentially pathognomonic for NF1 with diffuse involvement along a nerve segment or its branches, giving a “bag of worms” appearance [30, 31]. Few flow voids within the lesion are displayed on T2-weighted images of plexiform neurofibromas . Although post-contrast T1-weighted image was helpful to make a differential diagnosis, Shinde et al.  reported that post-contrast images could not make a definite diagnosis for the various pattern of the nodule . No further contrast examination was done in the current study. Histopathologic findings are helpful in differentiating PS from plexiform neurofibroma definitely.
The histopathologic differential diagnosis of PS includes plexiform neurofibroma and plexiform fibrohistiocytic tumor , which requires wide excision to avoid potential recurrence and metastasis . PS consists of multiple intradermal or subcutaneous nodules composed primarily of cellular Antoni A regions with nuclear palisading and verocay bodies . But plexiform neurofibroma usually does not have these characteristics . In contrast to conventional schwannomas, PS grows in a plexifrom pattern. It consists of multiple interlacing and interconnecting fascicles and nodules usually with the Antoni A features of a solitary schwannoma  but without Antoni B zones. Nodules were full of slender spindle cells with nuclear palisading and plexiform and spiral arrangement, especially, consisting of verocay bodies. Immunohistochemical studies showed the tumor cells stained positive and exhibited strong immunoreactivity for S-100 protein.
Limitations of the study
Because the study was a retrospective study, no contrast MR examination was done and intraoperative photos were not acquired. Thus it must be assumed that the outcome of this case report would be more worth to study.
We present a case of multiple PSs in the plantar aspect of the foot. In contrast to conventional schwannomas, multiple PSs are rare, and the imaging features of this lesion include the homogeneously hyperintense signal without multinodular and cystic degeneration on T2 MRI. We report this case with an effort to describe its MRI characteristics for diagnosis and also hope to provide informative data for further comprehensive analysis.
We conform that the patient has given his written consent for the case report to be published. A copy of the signed consent is available for review upon request.
Ethical Board Review statement
This material has not been published and is not under consideration elsewhere. This study receives no financial support.
The authors wish to thank the patient of multiple plexiform schwannomas and her for supporting our work.
- Ozdemir O, Kurt C, Coskunol E, Calli I, Ozsoy MH: Schwannomas of the hand and wrist: long-term results and review of the literature. J Orthop Surg. 2005, 13: 267-272.Google Scholar
- Pilavaki M, Chourmouzi D, Kiziridou A, Skordalaki A, Zarampoukas T, Drevelengas A: Imaging of peripheral nerve sheath tumors with pathologic correlation: pictorial review. Eur J Radiol. 2004, 52: 229-239. 10.1016/j.ejrad.2003.12.001.View ArticlePubMedGoogle Scholar
- Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM: The pathobiologic spectrum of Schwannomas. Histol Histopathol. 2003, 18: 925-934.PubMedGoogle Scholar
- Woodruff JM, Scheithauer BW, Kurtkaya-Yapicier O, Raffel C, Amr SS, LaQuaglia MP, Antonescu CR: Congenital and childhood plexiform (multinodular) cellular schwannoma: a troublesome mimic of malignant peripheral nerve sheath tumor. Am J Surg Pathol. 2003, 27: 1321-1329. 10.1097/00000478-200310000-00004.View ArticlePubMedGoogle Scholar
- Ioannou M, Papanastassiou I, Iakowidou I, Kottakis S, Demertzis N: Plexiform schwannoma of the posterior tibial nerve: a case report. Cases J. 2009, 2: 8392-10.4076/1757-1626-2-8392.View ArticlePubMedPubMed CentralGoogle Scholar
- Chuang WY, Yeh CJ, Jung SM, Hsueh S: Plexiform schwannoma of the clitoris. APMIS. 2007, 115: 889-890. 10.1111/j.1600-0463.2007.apm_734.x.View ArticlePubMedGoogle Scholar
- Iwashita T, Enjoji M: Plexiform neurilemmoma: a clinicopathological and immunohistochemical analysis of 23 tumours from 20 patients. Virchows Arch A Pathol Anat Histopatho. 1987, 411: 305-309. 10.1007/BF00713373.View ArticleGoogle Scholar
- Ikushima K, Ueda T, Kudawara I, Nakanishi K, Yoshikawa H: Plexiform schwannoma of the foot. Eur Radiol. 1999, 9: 1653-1655. 10.1007/s003300050904.View ArticlePubMedGoogle Scholar
- Agaram NP, Prakash S, Antonescu CR: Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety. Am J Surg Pathol. 2005, 29: 1042-1048.PubMedGoogle Scholar
- Berg JC, Scheithauer BW, Spinner RJ, Allen CM, Koutlas IG: Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region. Hum Pathol. 2008, 39: 633-640. 10.1016/j.humpath.2007.10.029.View ArticlePubMedGoogle Scholar
- Jacobson JM, Felder JM, Pedroso F, Steinberg JS: Plexiform schwannoma of the foot: a review of the literature and case report. J Foot Ankle Surg. 2011, 50: 68-73. 10.1053/j.jfas.2010.07.002.View ArticlePubMedGoogle Scholar
- Mohammed SA, Pressman MM, Schmidt B, Babu N: Case presentations and review of plexiform schwannoma in the foot. J Foot Ankle Surg. 2014, 53: 179-185. 10.1053/j.jfas.2013.12.020.View ArticlePubMedGoogle Scholar
- Hirose T, Scheithauer BW, Sano T: Giant plexiform schwannoma: a report of two cases with soft tissue and visceral involvement. Mod Pathol. 1997, 10: 1075-1081.PubMedGoogle Scholar
- Fedakar R, Akan O, Eren B: Cervical schwannoma presenting with trauma-induced neurolofical symptoms. Acta Orthop Turc. 2008, 42: 208-210. 10.3944/AOTT.2008.208.View ArticleGoogle Scholar
- Sasaki M, Aoki M, Yoshimine T: Mobile schwannoma of the cauda equina incarcerated following caudal migration after trauma—case report. Neurol Med Chir. 2011, 51: 710-712. 10.2176/nmc.51.710.View ArticleGoogle Scholar
- Woodruff JM, Marshall ML, Godwin TA, Funkhouse JW, Thompson NJ, Erlandson RA: Plexiform (multinodular) schwannoma. A tumor simulating the plexiform neurofibroma. Am J Surg Pathol. 1983, 7: 691-697. 10.1097/00000478-198310000-00009.View ArticlePubMedGoogle Scholar
- Val-Bernal JF, Figols J, Vazquez-Barquero A: Cutaneous plexiform schwannoma associated with neurofibromatosis type 2. Cancer. 1995, 76: 1181-1186. 10.1002/1097-0142(19951001)76:7<1181::AID-CNCR2820760713>3.0.CO;2-P.View ArticlePubMedGoogle Scholar
- Shinde SV, Tyagi DK, Sawant HV, Puranik GV: Plexiform schwannoma in schwannomatosis. J Postgrad Med. 2009, 55: 206-207. 10.4103/0022-3859.57406.View ArticlePubMedGoogle Scholar
- Ishida T, Kuroda M, Motoi T, Oka T, Imamura T, Machinami R: Phenotypic diversity of neurofibromatosis 2: Association with plexiform schwannoma. Histopathology. 1998, 32: 264-270. 10.1046/j.1365-2559.1998.00336.x.View ArticlePubMedGoogle Scholar
- Baser ME, Friedman JM, Evans DG: Increasing the specificity of diagnostic criteria for schwannomatosis. Neurology. 2006, 66: 730-732. 10.1212/01.wnl.0000201190.89751.41.View ArticlePubMedGoogle Scholar
- Ahmed AR, Watanabe H, Aoki J, Shinozaki T, Takagishi K: Schwannoma of the extremities: the role of PET in preoperative planning. Eur J Nucl Med. 2001, 28: 1541-1551. 10.1007/s002590100584.View ArticlePubMedGoogle Scholar
- Kobayashi H, Kotoura Y, Sakahara H, Hosono M, Hosono M, Tsaboyama T, Yamamuro T, Endo K, Konishi J: Schwannoma of the extremities: comparison of MRI and pentavalent technetium-99 m-dimercaptosuccinic acid and gallium-67-citrate scintigraphy. J Nucl Med. 1994, 35: 1174-1178.PubMedGoogle Scholar
- Söderlund V, Göranson H, Bauer HC: MR imaging of benign peripheral nerve sheath tumors. Acta Radiol. 1994, 35: 282-286. 10.3109/02841859409172383.View ArticlePubMedGoogle Scholar
- Yamada K, Harada M, Kunitoku N, Goto S, Kochi M, Ushio Y: MR imaging features of a scalp plexiform schwannoma. AJNR Am J Neuroradiol. 2004, 25: 291-294.PubMedGoogle Scholar
- Wollina U, Langner D, Gruner M, Schönlebe J, Haroske G: Isolated plexiform schwannoma of the hand. J Dermatol Case Rep. 2008, 2: 28-30. 10.1186/1752-1947-2-28.View ArticlePubMedPubMed CentralGoogle Scholar
- Nguyen KT, Pace R, Ludwin S, Ramsay DA: Plexiform neurofibroma: report of an unusual presentation. Can Assoc Radiol J. 1990, 41: 103-104.PubMedGoogle Scholar
- Reith JD, Goldblum JR: Multiple cutaneous plexiform schwannomas. Report of a case and review of the literature with particular reference to the association with types 1 and 2 neurofibromatosis and schwannomatosis. Arch Pathol Lab Med. 1996, 120: 399-401.PubMedGoogle Scholar
- Megahed M: Plexiform schwannoma. Am J Dermatopathol. 1994, 16: 288-293. 10.1097/00000372-199406000-00011.View ArticlePubMedGoogle Scholar
- Sehgal VN, Srivastava G, Aggarwal AK, Oberoi R, Sharma S: Solitary plexiform neurofibroma(s): role of magnetic resonance imaging. Skinmed. 2007, 6: 99-100. 10.1111/j.1540-9740.2007.05784.x.View ArticlePubMedGoogle Scholar
- Sheikh S, Gomes M, Montgomery E: Multiple plexiform schwannomas in a patient with neurofibromatosis. J Thorac Cardiovasc Surg. 1998, 115: 240-242. 10.1016/S0022-5223(98)70464-7.View ArticlePubMedGoogle Scholar
- Murphey MD, Smith WS, Smith SE, Kransdorf MJ, Temple HT: From the archives of the AFIP: imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 1999, 19: 1253-1280. 10.1148/radiographics.19.5.g99se101253.View ArticlePubMedGoogle Scholar
- Ko SF, Lee TY, Lin JW, Ng SH, Chen WJ, Hsieh MJ, Wan YL, Tsai CC: Thoracic neuilemomas: an analysis of computed tomography findings in 36 patients. J Thorac Imaging. 1998, 13: 21-26. 10.1097/00005382-199801000-00007.View ArticlePubMedGoogle Scholar
- Lim HS, Jung J, Chung KY: Neurofibromatosis type 2 with multiple plexiform schwannomas. Int J Dermatol. 2004, 43: 336-340. 10.1111/j.1365-4632.2004.01864.x.View ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2474/15/342/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.