Fibromatosis: a potential sequela of neuromuscular choristoma

Clinical article

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Object

Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion in which mature skeletal muscle fibers lie within the nerve and its fascicles. Given limited follow-up, its natural history is poorly understood. The occurrence of aggressive fibromatosis in one of the authors' patients and its occurrence in reported cases suggests an etiological relationship between the 2 lesions. This study attempts to explain the association and its frequency.

Methods

All cases of NMCs seen in consultation or treated at the Mayo Clinic were identified. Demographic and clinical data were reviewed in cases with coexistent aggressive fibromatosis. Pathology and neuroimaging studies were reexamined. In addition, an extensive literature review was performed to explore the association of NMC with aggressive fibromatosis, with special attention given to pathological and imaging characteristics and the development of aggressive fibromatosis.

Results

The authors identified 10 patients with a diagnosis of NMC who were treated at the Mayo Clinic between 1992 and 2010. Four of 5 with adequate follow-up had developed a definite or suspected aggressive fibromatosis. A review of the initial pathological specimens in these cases revealed no evidence of fibromatosis, but all of the lesions exhibited accompanying hypocellular collagenous tissue. On MR images, all cases showed areas of low signal intensity, which significantly differed from muscle, nerve, and NMC components. On available serial MR imaging studies, aggressive fibromatosis seemed to originate in such lower-intensity regions. In the 18 previously reported cases of NMC, 5 patients developed recurrent masses diagnosed as either definite (2 cases) or possible (3 cases) fibromatosis. Review of the published imaging studies in these cases suggests the presence of lower intensity areas similar to those observed in the 10 patients treated at the Mayo Clinic.

Conclusions

This study confirms that the development of aggressive fibromatosis in patients with NMC has been underreported. A direct relationship between the NMC and the development of aggressive fibromatosis is suggested by pathological and neuroimaging evidence.

Abbreviation used in this paper: NMC = neuromuscular choristoma.
Article Information

Contributor Notes

Address correspondence to: Robert J. Spinner, M.D., Mayo Clinic, 200 First Street SW, Gonda 8-214S, Rochester, Minnesota 55905. email: spinner.robert@mayo.edu.Please include this information when citing this paper: published online August 5, 2011; DOI: 10.3171/2011.6.JNS102171.

© AANS, except where prohibited by US copyright law.

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References
  • 1

    Awasthi DKline DGBeckman EN: Neuromuscular hamartoma (benign “triton” tumor) of the brachial plexus. Case report. J Neurosurg 75:7957971991

    • Search Google Scholar
    • Export Citation
  • 2

    Azzopardi JGEusebi VTison VBetts CM: Neurofibroma with rhabdomyomatous differentiation: benign ‘Triton' tumour of the vagina. Histopathology 7:5615721983

    • Search Google Scholar
    • Export Citation
  • 3

    Bassett GSMonforte-Munoz HMitchell WGRowland JM: Cavus deformity of the foot secondary to a neuromuscular choristoma (hamartoma) of the sciatic nerve. A case report. J Bone Joint Surg Am 79:139814011997

    • Search Google Scholar
    • Export Citation
  • 4

    Boman FPalau CFloquet AFloquet JLascombes P: [Neuromuscular hamartoma.]. Ann Pathol 11:36411991. (Fr)

  • 5

    Bonneau RBrochu P: Neuromuscular choristoma. A clinicopathologic study of two cases. Am J Surg Pathol 7:5215281983

  • 6

    Castro DERaghuram KPhillips CD: Benign triton tumor of the trigeminal nerve. AJNR Am J Neuroradiol 26:9679692005

  • 7

    Chapon FHubert PMandard JCRivrain YLechevalier B: [Spinal lipoma associated with a neuromuscular hamartoma. Report of one case.]. Ann Pathol 11:3453481991. (Fr)

    • Search Google Scholar
    • Export Citation
  • 8

    Chen KT: Neuromuscular hamartoma. J Surg Oncol 26:1581601984

  • 9

    Cunniffe GFernández JAlonso TBalaguer ODinares CHuguet P: Thyroid orbitopathy obscuring the diagnosis of a rare neuromuscular hamartoma of the superior rectus muscle in an adult. Orbit 29:1681702010

    • Search Google Scholar
    • Export Citation
  • 10

    Daley TDDarling MRWehrli B: Benign Triton tumor of the tongue. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 105:7637662008

  • 11

    Demir YUluoglu OOzmen SBoyacioglu ZMAtabay K: Neuromuscular hamartoma in the mental region. J Oral Maxillofac Surg 61:3974002003

    • Search Google Scholar
    • Export Citation
  • 12

    Galliani CAMatt BH: Laryngomalacia and intra-neural striated muscle in an infant with the Freeman-Sheldon syndrome. Int J Pediatr Otorhinolaryngol 25:2432481993

    • Search Google Scholar
    • Export Citation
  • 13

    Gersdorff MCDecat MDuprez T: Neuromuscular hamartoma of the internal auditory canal. Eur Arch Otorhinolaryngol 253:4404421996

  • 14

    Giannini CReynolds CLeavitt JASchultz GAGarrity JAEbersold MJ: Choristoma of the optic nerve: case report. Neurosurgery 50:112511282002

    • Search Google Scholar
    • Export Citation
  • 15

    Hendrick SJSanchez RLBlackwell SJRaimer SS: Striated muscle hamartoma: description of two cases. Pediatr Dermatol 3:1531571986

    • Search Google Scholar
    • Export Citation
  • 16

    Karagama YGBridges LRvan Hille PT: Neuromuscular hamartoma of the cochlear nerve: a rare occurrence in the internal auditory meatus. Eur Arch Otorhinolaryngol 259:1191202002

    • Search Google Scholar
    • Export Citation
  • 17

    Kawamoto SMatsuda HUeki KOkada YKim P: Neuromuscular choristoma of the oculomotor nerve: case report. Neurosurgery 60:E777E7782007

    • Search Google Scholar
    • Export Citation
  • 18

    Kazim MKennerdell JSMaroon JRothfus WMarquardt M: Choristoma of the optic nerve and chiasm. Arch Ophthalmol 110:2362381992

  • 19

    Kim DHHong EKLee JD: [Subcutaneous neuromuscular hamartoma. A case report.]. Korean J Pathol 33:62641999. (Korean)

  • 20

    Kim SYKwon HPKwak KDAhn KB: Neuromuscular choristoma of the sciatic nerve: a case report. Korean J Pathol 39:1921962005

  • 21

    Kusuzaki KEmoto KMurata HKatsura KSugihara HTsuchihashi Y: Nerve contact with muscle component in neuromuscular hamartoma. Anticancer Res 20:5C380738112000

    • Search Google Scholar
    • Export Citation
  • 22

    Lai PHHo JTLin SLHsu SSChen CYeh LR: Neuromuscular hamartoma arising in the brachial plexus. Neuroradiology 46:2162182004

    • Search Google Scholar
    • Export Citation
  • 23

    Lassaletta LGranell JPatrón MGavilán J: Smooth muscle choristoma of the internal auditory meatus. Eur Arch Otorhinolaryngol 262:8348382005

    • Search Google Scholar
    • Export Citation
  • 24

    Le BHRivas SSimmons ZHarbaugh KTowfighi JPu CF: Painless lumbosacral plexopathy: a case of neuromuscular choristoma: 84. J Neuropathol Exp Neurol 64:4512005. (Abstract)

    • Search Google Scholar
    • Export Citation
  • 25

    Lee JINam DHKim JSHong SCShin HJPark K: Intracranial oculomotor nerve rhabdomyoma. Case illustration. J Neurosurg 93:7152000

    • Search Google Scholar
    • Export Citation
  • 26

    Lena GDufour TGambarelli DChabrol BMancini J: Choristoma of the intracranial maxillary nerve in a child. Case report. J Neurosurg 81:7887911994

    • Search Google Scholar
    • Export Citation
  • 27

    Louhimo IRapola J: Intraneural muscular hamartoma: report of two cases in small children. J Pediatr Surg 7:6966991972

  • 28

    Lynch JJParvizi JScheithauer BWKrauss WE: Development of postoperative fibromatosis after resection of an intraspinal meningioma. Case report. J Neurosurg 90:1 Suppl1211241999

    • Search Google Scholar
    • Export Citation
  • 29

    Maher COSpinner RJGiannini CScheithauer BWCrum BA: Neuromuscular choristoma of the sciatic nerve. Case report. J Neurosurg 96:112311262002

    • Search Google Scholar
    • Export Citation
  • 30

    Markel SFEnzinger FM: Neuromuscular hamartoma—a benign “triton tumor” composed of mature neural and striated muscle elements. Cancer 49:1401441982

    • Search Google Scholar
    • Export Citation
  • 31

    Masson P: Tumeurs Humaines: Histologie Diagnostics et Techniques ed 2ParisLibrairie Maloine1956. 973975

  • 32

    Menovsky TGrotenhuis JAde Vries JBartels RH: Endoscope-assisted supraorbital craniotomy for lesions of the interpeduncular fossa. Neurosurgery 44:1061121999

    • Search Google Scholar
    • Export Citation
  • 33

    Mitchell AScheithauer BWOstertag HSepehrnia ASav A: Neuromuscular choristoma. Am J Clin Pathol 103:4604651995

  • 34

    O'Connell JXRosenberg AE: Multiple cutaneous neuromuscular choristomas. Report of a case and a review of the literature. Am J Surg Pathol 14:93961990

    • Search Google Scholar
    • Export Citation
  • 35

    Oeppen RSHarden SPArgent JD: Neuromuscular hamartoma: imaging features of a rare paediatric craniofacial tumour. Pediatr Radiol 33:50522003

    • Search Google Scholar
    • Export Citation
  • 36

    Orlandi E: Sopra un caso di rabdomioma del nervo ischiatico. Arch Sci Med (Torino) 19:1131371895

  • 37

    Owor GQian JPayner TMartin AShan Y: Intracranial neuromuscular choristoma: a case report and literature review. Ear Nose Throat J 83:5445465482004

    • Search Google Scholar
    • Export Citation
  • 38

    Shitabata PKRitter JHFitzgibbon JFDehner LPWick MR: Pleomorphic hamartoma of the subcutis: a lesion with possible myogenous and neural lineages. J Cutan Pathol 22:2692751995

    • Search Google Scholar
    • Export Citation
  • 39

    Smith MMThompson JEThomas DCastillo MCarrier DMukherji SK: Choristomas of the seventh and eighth cranial nerves. AJNR Am J Neuroradiol 18:3273291997

    • Search Google Scholar
    • Export Citation
  • 40

    Spencer ALee JPrayson RA: Optic nerve choristoma. Ann Diagn Pathol 9:93952005

  • 41

    Strome SEMcClatchey KKileny PRKoopmann CF Jr: Neonatal choristoma of the tongue containing glial tissue: diagnosis and surgical considerations. Int J Pediatr Otorhinolaryngol 33:2652731995

    • Search Google Scholar
    • Export Citation
  • 42

    Tiffee JCBarnes EL: Neuromuscular hamartomas of the head and neck. Arch Otolaryngol Head Neck Surg 124:2122161998

  • 43

    Tobias SKim CHSade BStaugaitis SMLee JH: Neuromuscular hamartoma of the trigeminal nerve in an adult. Acta Neurochir (Wien) 148:83872006

    • Search Google Scholar
    • Export Citation
  • 44

    Tolan SShanks JHLoh MYTaylor BWylie JP: Fibromatosis: benign by name but not necessarily by nature. Clin Oncol (R Coll Radiol) 19:3193262007

    • Search Google Scholar
    • Export Citation
  • 45

    Uysal ASungur NKoçer UCöloğlu HOruç MYalta T: Neuromuscular hamartoma of the occipital nerve: clinical report. J Craniofac Surg 16:7407422005

    • Search Google Scholar
    • Export Citation
  • 46

    Vajramani GDevi ISantosh VHegde TDas BSDas S: Benign triton tumor of the trigeminal nerve. Childs Nerv Syst 15:1401441999

    • Search Google Scholar
    • Export Citation
  • 47

    Vajtai IVarga ZHackel J: [Neuromuscular choristoma.]. Orv Hetil 140:174317461999. (Hungarian)

  • 48

    Van Dorpe JSciot RDe Vos RUyttebroeck AStas MVan Damme B: Neuromuscular choristoma (hamartoma) with smooth and striated muscle component: case report with immunohistochemical and ultrastructural analysis. Am J Surg Pathol 21:109010951997

    • Search Google Scholar
    • Export Citation
  • 49

    Vandewalle GBrucher JMMichotte A: Intracranial facial nerve rhabdomyoma. Case report. J Neurosurg 83:9199221995

  • 50

    Weston JAThe migration and differentiation of neural crest cells. Abercrombie MBrachet JKing TJ: Advances in Morphogenesis Vol 8 New YorkAcademic Press1970. 41114

    • Search Google Scholar
    • Export Citation
  • 51

    Wu SSLo WWMTschirhart DLSlattery WH IIICarberry JNBrackmann DE: Lipochoristomas (lipomatous tumors) of the acoustic nerve. Arch Pathol Lab Med 127:147514792003

    • Search Google Scholar
    • Export Citation
  • 52

    Zannolli RMostardini RPucci LSorrentino LBiagioli MPerotti R: Corpus callosum agenesis, multiple cysts, skin defects, and subtle ocular abnormalities with a de novo mutation [45,XX,der(5), t(5;14) (pter;q11.2)]. Am J Med Genet 102:29352001

    • Search Google Scholar
    • Export Citation
  • 53

    Zimmerman LEArkfeld DLSchenken JBArkfeld DFMaris PJG: A rare choristoma of the optic nerve and chiasm. Arch Ophthalmol 101:7667701983

    • Search Google Scholar
    • Export Citation
  • 54

    Zwick DLLivingston KClapp LKosnik EYates A: Intracranial trigeminal nerve rhabdomyoma/choristoma in a child: a case report and discussion of possible histogenesis. Hum Pathol 20:3903921989

    • Search Google Scholar
    • Export Citation
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