International Ophthalmology

, Volume 34, Issue 5, pp 1097–1106 | Cite as

Clinicopathological study of three cases of infantile fibromatosis of the orbit

  • Kengo Hayashi
  • Nobutada Katori
  • Yoshiro Otsuki
  • Kyoko Ohno-Matsui
Original Paper


We report the clinical and pathological findings in three infants with infantile fibromatosis that involved several extraocular muscles which led to disorders of ocular motility. We also describe their clinical features before and after surgery. This was a retrospective interventional case study with clinicopathological correlations. We present three cases of infantile fibromatosis that were diagnosed by clinical features and histopathlogical examination of biopsy specimens. The three patients were all female children aged 1, 3, and 3 years at their initial visit. The orbital tumor was unilateral in all patients. All three patients had disorders of ocular motility because the tumors involved ≥2 extraocular muscles. The margins between the tumor and the involved extraocular muscles were not distinct. We performed partial resection of the tumors to preserve the extraocular muscles. In all cases, the tumors partially remained, but periodic postoperative magnetic resonance imaging showed no enlargement of the tumors during the follow-up period. All three patients had residual limitations of eye movements. We should consider the postoperative binocular function when we treat infantile benign fibrous tumors involving the extraocular muscles.


Orbit Infantile fibromatosis Infantile myofibromatosis Infantile myofibroma Juvenile fibromatosis 



The authors thank Dr. Duco I. Hamasaki Professor Emeritus, Bascom Palmer Eye Institute, Department of Ophthalmology University of Miami, School of Medicine Miami, Florida, for his critical discussions and final manuscript revisions.


  1. 1.
    Hidayat AA, Font RL (1980) Juvenile fibromatosis of the periorbital region and eyelid. A clinicopathologic study of six cases. Arch Ophthalmol 98:280–285PubMedCrossRefGoogle Scholar
  2. 2.
    Campbell RJ, Garrity JA (1991) Juvenile fibromatosis of the orbit: a case report with review of the literature. Br J Ophthalmol 75:313–316PubMedCentralPubMedCrossRefGoogle Scholar
  3. 3.
    Waeltermann JM, Huntrakoon M, Beatty EC Jr, Cibis GW (1988) Congenital fibromatosis (myofibromatosis) of the orbit: a rare cause of proptosis at birth. Ann Ophthalmol 20:394–396PubMedGoogle Scholar
  4. 4.
    Fornelli A, Salvi F, Mascalchi M, Marchetti C, Foschini MP (1999) Orbital (desmoid type) fibromatosis. Orbit 18:203–210PubMedCrossRefGoogle Scholar
  5. 5.
    Amerasinghe N, Rogers S, Rowlandson P, Kohler J, Hodgkins P (2006) Fibromatosis (desmoid tumor) involving the orbit and neck. J AAPOS 10:479–481PubMedCrossRefGoogle Scholar
  6. 6.
    Weiss SW, Goldblum JR (2008) Fibrous tumors of infancy and childhood. Enzinger and Weiss’s Soft Tissue Tumors, 5th edn. Mosby, St. Louis, pp 257–302Google Scholar
  7. 7.
    Shields JA, Shields CL (2007) Orbital Fibromatosis, Myofibromatosis, and Myofibroma. Eyelid, Conjunctival, and Orbital Tumors: An Atlas and Text. 2nd edn. Lippincott. 622-623Google Scholar
  8. 8.
    Schutz JS, Rabkin MD, Schutz S (1979) Fibromatous tumor (desmoid type) of the orbit. Arch Ophthalmol 97:703–704PubMedCrossRefGoogle Scholar
  9. 9.
    Mynatt CJ, Feldman KA, Thompson LD (2011) Orbital infantile myofibroma: a case report and clinicopathologic review of 24 cases from the literature. Head Neck Pathol 5:205–215PubMedCentralPubMedCrossRefGoogle Scholar
  10. 10.
    Larsen AC, Prause JU, Petersen BL, Heegaard S (2011) Solitary infantile myofibroma of the orbit. Acta Ophthalmol 89:e600–e601PubMedCrossRefGoogle Scholar
  11. 11.
    Harstein ME, Thomas SM, Ellis LS (2006) Orbital desmoid tumor in a pediatric patient. Ophthal Plast Reconstr Surg 22:139–141CrossRefGoogle Scholar
  12. 12.
    Westfall AC, Mansoor A, Sulliyan SA, Wilson DJ, Dailey RA (2003) Orbital and periorbital myofibromas in childhood: two case reports. Ophthalmology 110:2000–2005PubMedCrossRefGoogle Scholar
  13. 13.
    Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB (1990) Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol 14:335–341PubMedCrossRefGoogle Scholar
  14. 14.
    Coffin CM, Dehner LP, O’Shea PA (1997) Fibroblastic-myofibroblastic tumors. Pediatric Soft Tissue Tumors. Williams & Wilkins, Baltimore, pp 155–161Google Scholar
  15. 15.
    Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G (2004) High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer 100:612–620PubMedCrossRefGoogle Scholar
  16. 16.
    Lackner H, Urban C, Kerbl R, Schwinger W, Beham A (1997) Noncytotoxic drug therapy in children with unresectable desmoids tumors. Cancer 80:334–340PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2014

Authors and Affiliations

  • Kengo Hayashi
    • 1
  • Nobutada Katori
    • 2
  • Yoshiro Otsuki
    • 3
  • Kyoko Ohno-Matsui
    • 1
  1. 1.Department of Ophthalmology and Visual ScienceTokyo Medical and Dental UniversityTokyoJapan
  2. 2.Department of Ocular Plastic & Orbital SurgerySeirei Hamamatsu General HospitalHamamatsuJapan
  3. 3.Department of PathologySeirei Hamamatsu General HospitalHamamatsuJapan

Personalised recommendations