Stromal Tumors

  • Geeta K. VemugantiEmail author
  • Santosh G. Honavar


Eyelid stromal tumors could be considered under several distinct categories based on the tissue of origin: fibrous tissue tumors, fibrohistiocytic tumors, lipomatous tumors, smooth muscle tumors, skeletal muscle tumors, vascular tumors, perivascular tumors, neural tumors, lymphoid tumors, plasmacytic tumors, leukemic tumors, cartilage and bone tumors, secondary tumors, metastatic tumors, and hamartomas, choristomas, and other miscellaneous lesions. Some of the inflammatory and infective conditions such as chalazion, pyogenic granuloma, verruca vulgaris, and molluscum contagiosum may manifest with features clinically simulating tumors. This chapter describes the salient features and management of common eyelid stromal tumors. It would be worthwhile to mention here that further information on clinical classification of tumors of the eye could be obtained from the eighth edition of TNM which encompasses several important changes over the previous seventh edition.


Eyelid stromal tumors Fibrohistiocytic tumors Lymphomas Vascular tumors Choristomas Soft tissue tumors 


  1. 1.
    Witschel H. [Congenital solitary fibromatosis of the eyelid (author’s transl)]. Ophthalmologica. 1982;185(1):1–6.Google Scholar
  2. 2.
    Hidayat AA, Font RL. Juvenile fibromatosis of the periorbital region and eyelid. A clinicopathologic study of six cases. Arch Ophthalmol. 1980;98(2):280–5.PubMedCrossRefGoogle Scholar
  3. 3.
    John M, Chirayath S, Paulson S. Multiple soft fibromas of the lid. Indian J Ophthalmol. 2015;63(3):262–4.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 4.
    D’Hermies F, Dolle C, Verola O, et al. [Hypertrophic eyelid conjunctival scar. A tarsal keloid]. J Fr Ophtalmol. 2003;26(3):315–8.Google Scholar
  5. 5.
    Hymas DC, Mamalis N, Pratt DV, et al. Nodular fasciitis of the lower eyelid in a pediatric patient. Ophthalmic Plast Reconstr Surg. 1999;15(2):139–42.PubMedCrossRefGoogle Scholar
  6. 6.
    De Wan J, Evens F, De Mey A. Eyelid tumour and juvenile hyaline fibromatosis. Br J Plast Surg. 2005;58(1):106–11.PubMedCrossRefGoogle Scholar
  7. 7.
    Weiner JM, Hidayat AA. Juvenile fibrosarcoma of the orbit and eyelid. A study of five cases. Arch Ophthalmol. 1983;101(2):253–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Griffith DG, Salasche SJ, Clemons DE. Cutaneous abnormalities of the eyelid and face: an atlas with histopathology: New York, McGraw-Hill Companies; 1987.Google Scholar
  9. 9.
    Pandhi D, Gupta P, Singal A, et al. Xanthelasma palpebrarum: a marker of premature atherosclerosis (risk of atherosclerosis in xanthelasma). Postgrad Med J. 2012;88(1038):198–204.PubMedCrossRefGoogle Scholar
  10. 10.
    Park EJ, Youn SH, Cho EB, et al. Xanthelasma palpebrarum treatment with a 1,450-nm-diode laser. Dermatol Surg. 2011;37(6):791–6.PubMedGoogle Scholar
  11. 11.
    Dincer D, Koc E, Erbil AH, et al. Effectiveness of low-voltage radiofrequency in the treatment of xanthelasma palpebrarum: a pilot study of 15 cases. Dermatol Surg. 2010;36(12):1973–8.PubMedCrossRefGoogle Scholar
  12. 12.
    Jakobiec FA, Mills MD, Hidayat AA, et al. Periocular xanthogranulomas associated with severe adult-onset asthma. Trans Am Ophthalmol Soc. 1993;91:99–125; discussion -9PubMedPubMedCentralGoogle Scholar
  13. 13.
    Vick VL, Wilson MW, Fleming JC, et al. Orbital and eyelid manifestations of xanthogranulomatous diseases. Orbit. 2006;25(3):221–5.PubMedCrossRefGoogle Scholar
  14. 14.
    Khong JJ, Chen CS, James CL, et al. Malignant fibrous histiocytoma of the eyelid: differential diagnosis and management. Ophthalmic Plast Reconstr Surg. 2005;21(2):103–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Gounder P, Lam M, Vinciullo C, et al. Malignant fibrous histiocytoma masquerading as pyogenic granuloma. Orbit. 2017;36(2):122–3.PubMedCrossRefGoogle Scholar
  16. 16.
    Font RL, Hidayat AA. Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol. 1982;13(3):199–209.PubMedCrossRefGoogle Scholar
  17. 17.
    Thyparampil P, Diwan AH, Diaz-Marchan P, et al. Eyelid lipomas: a case report and review of the literature. Orbit. 2012;31(5):319–20.PubMedCrossRefGoogle Scholar
  18. 18.
    Bock-Kunz AL, Lyon DB, Singhal VK, et al. Nasopalpebral lipoma-coloboma syndrome. Arch Ophthalmol. 2000;118(12):1699–701.PubMedGoogle Scholar
  19. 19.
    Enghardt MH, Warren RC. Congenital palpebral lipoblastoma. First report of a case. Am J Dermatopathol. 1990;12(4):408–11.PubMedCrossRefGoogle Scholar
  20. 20.
    Jolly SS, Brownstein S, Jordan DR. Leiomyoma of the anterior orbit and eyelid. Can J Ophthalmol. 1995;30(7):366–70.PubMedGoogle Scholar
  21. 21.
    Lin IC, Wu CT, Liao SL, et al. Primary orbital leiomyosarcoma. Ophthalmic Plast Reconstr Surg. 2005;21(6):451–3.PubMedCrossRefGoogle Scholar
  22. 22.
    Verdolini R, Goteri G, Brancorsini D, et al. Adult rhabdomyoma: report of two cases of rhabdomyoma of the lip and of the eyelid. Am J Dermatopathol. 2000;22(3):264–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Shields CL, Shields JA, Honavar SG, et al. Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology. 2001;108(12):2284–92.PubMedCrossRefGoogle Scholar
  24. 24.
    Ferry AP. Pyogenic granulomas of the eye and ocular adnexa: a study of 100 cases. Trans Am Ophthalmol Soc. 1989;87:327–43; discussion 43-7PubMedPubMedCentralGoogle Scholar
  25. 25.
    Werner MS, Hornblass A, Reifler DM, et al. Intravascular papillary endothelial hyperplasia: collection of four cases and a review of the literature. Ophthal Plast Reconstr Surg. 1997;13(1):48–56.PubMedCrossRefGoogle Scholar
  26. 26.
    Shields CL, Shields JA, Minzter R, et al. Cutaneous capillary hemangiomas of the eyelid, scalp, and digits in premature triplets. Am J Ophthalmol. 2000;129(4):528–31.PubMedCrossRefGoogle Scholar
  27. 27.
    Walter JW, Blei F, Anderson JL, et al. Genetic mapping of a novel familial form of infantile hemangioma. Am J Med Genet. 1999;82(1):77–83.PubMedCrossRefGoogle Scholar
  28. 28.
    Jackson R. The natural history of strawberry naevi. J Cutan Med Surg. 1998;2(3):187–9.PubMedCrossRefGoogle Scholar
  29. 29.
    Stagner AM, Jakobiec FA. A critical analysis of eleven periocular lobular capillary hemangiomas in adults. Am J Ophthalmol. 2016;165:164–73.PubMedCrossRefGoogle Scholar
  30. 30.
    Xue K, Hildebrand GD. Deep periocular infantile capillary hemangiomas responding to topical application of timolol maleate, 0.5%, drops. JAMA Ophthalmol. 2013;131(9):1246–8.PubMedCrossRefGoogle Scholar
  31. 31.
    Kushner B. Local steroid therapy in adnexal hemangioma. Ann Ophthalmol. 1979;11(7):1005–9.PubMedGoogle Scholar
  32. 32.
    Missoi TG, Lueder GT, Gilbertson K, et al. Oral propranolol for treatment of periocular infantile hemangiomas. Arch Ophthalmol. 2011;129(7):899–903.PubMedCrossRefGoogle Scholar
  33. 33.
    Snir M, Reich U, Siegel R, et al. Refractive and structural changes in infantile periocular capillary haemangioma treated with propranolol. Eye (Lond). 2011;25(12):1627–34.CrossRefGoogle Scholar
  34. 34.
    Ni N, Guo S, Langer P. Current concepts in the management of periocular infantile (capillary) hemangioma. Curr Opin Ophthalmol. 2011;22(5):419–25.PubMedCrossRefGoogle Scholar
  35. 35.
    Xue K, Hildebrand GD. Topical timolol maleate 0.5% for infantile capillary haemangioma of the eyelid. Br J Ophthalmol. 2012:bjophthalmol-2012-302396Google Scholar
  36. 36.
    Bajaj MS, Nainiwal SK, Pushker N. Multifocal cavernous hemangioma: a rare presentation. Orbit. 2003;22(3):155–9.PubMedCrossRefGoogle Scholar
  37. 37.
    McCannel CA, Hoenig J, Umlas J, et al. Orbital lesions in the blue rubber bleb nevus syndrome. Ophthalmology. 1996;103(6):933–6.PubMedCrossRefGoogle Scholar
  38. 38.
    Hidayat AA, Cameron JD, Font RL, et al. Angiolymphoid hyperplasia with eosinophilia (Kimura’s disease) of the orbit and ocular adnexa. Am J Ophthalmol. 1983;96(2):176–89.PubMedCrossRefGoogle Scholar
  39. 39.
    Hayes BH, Shore JW, Westfall CT, et al. Management of orbital and periorbital arteriovenous malformations. Ophthalmic Surg. 1995;26(2):145–52.PubMedGoogle Scholar
  40. 40.
    Rootman J, Hay E, Graeb D, et al. Orbital-adnexal lymphangiomas: a spectrum of hernodynarnically isolated vascular harnartornas. Ophthalmology. 1986;93(12):1558–70.PubMedCrossRefGoogle Scholar
  41. 41.
    Ferguson DC, Mawn LA, Al-Rohil RN. Cutaneous angiosarcoma of the eyelid mimicking morbihan disease. Am J Dermatopathol. 2018;40:617.PubMedCrossRefGoogle Scholar
  42. 42.
    Lemanski N, Farber M, Carruth BP, et al. Primary adnexal angiosarcoma masquerading as periorbital hematoma. Surv Ophthalmol. 2014;59(6):655–9.PubMedCrossRefGoogle Scholar
  43. 43.
    Guo QY. [Atypical lymphocytes in schizophrenic patients]. Zhonghua Shen Jing Jing Shen Ke Za Zhi. 1986;19(3):185–7.Google Scholar
  44. 44.
    Conway R, Hammer T, Viestenz A, et al. Cutaneous angiosarcoma of the eyelids. Br J Ophthalmol. 2003;87(4):514–5.PubMedPubMedCentralCrossRefGoogle Scholar
  45. 45.
    Papalas JA, Manavi CK, Woodward JA, et al. Angiosarcoma of the eyelid: a clinicopathologic comparison between isolated unilateral tumors and tumors demonstrating extrapalpebral involvement. Am J Dermatopathol. 2010;32(7):694–9.PubMedCrossRefGoogle Scholar
  46. 46.
    Brun SC, Jakobiec FA. Kaposi’s sarcoma of the ocular adnexa. Int Ophthalmol Clin. 1997;37(4):25–38.PubMedCrossRefGoogle Scholar
  47. 47.
    Dammacco R, Lapenna L, Giancipoli G, et al. Solitary eyelid Kaposi sarcoma in an HIV-negative patient. Cornea. 2006;25(4):490–2.PubMedCrossRefGoogle Scholar
  48. 48.
    Jhaj G, Ilyas G, Shinder R. Eyelid Kaposi’s sarcoma in an immunocompetent woman. Ophthalmology. 2017;124(3):392.PubMedCrossRefGoogle Scholar
  49. 49.
    Teixeira AI, Neno M, Badura R, et al. Kaposi Sarcoma of the eyelid as an initial manifestation of AIDS. Dermatol Online J. 2016;22(7)Google Scholar
  50. 50.
    Pica F, Volpi A. Transmission of human herpesvirus 8: an update. Curr Opin Infect Dis. 2007;20(2):152–6.PubMedCrossRefGoogle Scholar
  51. 51.
    Brunetti AE, Guarini A, Lorusso V, et al. Complete response to second line Paclitaxel every 2 weeks of eyelid kaposi sarcoma: a case report. Ophthalmic Plast Reconstr Surg. 2013;29(5):e114–5.PubMedCrossRefGoogle Scholar
  52. 52.
    Zhang ML, Suarez MJ, Bosley TM, et al. Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa. Hum Pathol. 2017;63:70–8.PubMedPubMedCentralCrossRefGoogle Scholar
  53. 53.
    Butt Z, Ironside JW. Superficial epithelioid schwannoma presenting as a subcutaneous upper eyelid mass. Br J Ophthalmol. 1994;78(7):586–8.PubMedPubMedCentralCrossRefGoogle Scholar
  54. 54.
    Singh AD, Eagle RC Jr, Shields CL, et al. Merkel cell carcinoma of the eyelids. Int Ophthalmol Clin. 1993;33(3):11–7.PubMedCrossRefGoogle Scholar
  55. 55.
    Kivela T, Tarkkanen A. The Merkel cell and associated neoplasms in the eyelids and periocular region. Surv Ophthalmol. 1990;35(3):171–87.PubMedCrossRefGoogle Scholar
  56. 56.
    Amber K, McLeod MP, Nouri K. The Merkel cell polyomavirus and its involvement in Merkel cell carcinoma. Dermatol Surg. 2013;39(2):232–8.PubMedCrossRefGoogle Scholar
  57. 57.
    Feng H, Shuda M, Chang Y, et al. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008;319(5866):1096–100.PubMedPubMedCentralCrossRefGoogle Scholar
  58. 58.
    Hanafi H, Verdijk RM, Paridaens D. Eyelid Merkel cell carcinoma in a patient treated with golimumab. Orbit. 2018;37(1):21–5.PubMedCrossRefGoogle Scholar
  59. 59.
    Sniegowski MC, Warneke CL, Morrison WH, et al. Correlation of American Joint Committee on Cancer T category for eyelid carcinoma with outcomes in patients with periocular Merkel cell carcinoma. Ophthalmic Plast Reconstr Surg. 2014;30(6):480–5.PubMedCrossRefGoogle Scholar
  60. 60.
    Moll I, Kuhn C, Moll R. Cytokeratin 20 is a general marker of cutaneous Merkel cells while certain neuronal proteins are absent. J Invest Dermatol. 1995;104(6):910–5.PubMedCrossRefGoogle Scholar
  61. 61.
    Peters GB III, Meyer DR, Shields JA, et al. Management and prognosis of Merkel cell carcinoma of the eyelid. Ophthalmology. 2001;108(9):1575–9.PubMedCrossRefGoogle Scholar
  62. 62.
    Tuskada A, Fujimura T, Hashimoto A, et al. Successful local control of cutaneous Merkel cell carcinoma on the eyelid with CyberKnife radiosurgery. Eur J Dermatol. 2013;23(5):725–6.PubMedGoogle Scholar
  63. 63.
    Pfeiffer ML, Savar A, Esmaeli B. Sentinel lymph node biopsy for eyelid and conjunctival tumors: what have we learned in the past decade? Ophthalmic Plast Reconstr Surg. 2013;29(1):57–62.PubMedCrossRefGoogle Scholar
  64. 64.
    Margo CE, Mulla ZD. Malignant tumors of the eyelid: a population-based study of non-basal cell and non-squamous cell malignant neoplasms. Arch Ophthalmol. 1998;116(2):195–8.PubMedCrossRefGoogle Scholar
  65. 65.
    Graue GF, Finger PT, Maher E, et al. Ocular adnexal lymphoma staging and treatment: American Joint Committee on Cancer versus Ann Arbor. Eur J Ophthalmol. 2013;23(3):344–55.PubMedCrossRefGoogle Scholar
  66. 66.
    Svendsen FH, Rasmussen PK, Coupland SE, et al. Lymphoma of the eyelid - an international multicenter retrospective study. Am J Ophthalmol. 2017;177:58–68.PubMedCrossRefGoogle Scholar
  67. 67.
    Ahamed E, Samuel L, Tighe J. Extramedullary plasmacytoma of the eyelid. Br J Ophthalmol. 2003;87(2):244–5.PubMedPubMedCentralCrossRefGoogle Scholar
  68. 68.
    Yaghouti F, Nouri M, Mannor GE. Ocular adnexal granulocytic sarcoma as the first sign of acute myelogenous leukemia relapse. Am J Ophthalmol. 1999;127(3):361–3.PubMedCrossRefGoogle Scholar
  69. 69.
    Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63–70.PubMedGoogle Scholar
  70. 70.
    Hsu HY, Chen ZT, Tsai CY, et al. Atypical Rosai-Dorfman disease with lacrimal gland involvement. Semin Ophthalmol. 2013;28(4):253–5.PubMedCrossRefGoogle Scholar
  71. 71.
    Jat KR, Panigrahi I, Srinivasan R, et al. Cutaneous Rosai-Dorfman disease: presenting as massive bilateral eyelid swelling. Pediatr Dermatol. 2009;26(5):633–5.PubMedCrossRefGoogle Scholar
  72. 72.
    Levinger S, Pe’er J, Aker M, et al. Rosai-Dorfman disease involving four eyelids. Am J Ophthalmol. 1993;116(3):382–4.PubMedCrossRefGoogle Scholar
  73. 73.
    Foucar E, Rosai J, Dorfman RF. The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol. 1979;87(3):354–67.PubMedCrossRefGoogle Scholar
  74. 74.
    Vemuganti GK, Naik MN, Honavar SG. Rosai dorfman disease of the orbit. J Hematol Oncol. 2008;1(1):7.PubMedPubMedCentralCrossRefGoogle Scholar
  75. 75.
    McCulley TJ, Yip C-C, Kersten RC, et al. An ectopic site of lacrimal gland secretion mimicking epiphora. Arch Ophthalmol. 2002;120(11):1586–7.PubMedGoogle Scholar
  76. 76.
    Gordon AJ, Patrinely JR, Knupp JA, et al. Complex choristoma of the eyelid containing ectopic cilia and lacrimal gland. Ophthalmology. 1991;98(10):1547–50.PubMedCrossRefGoogle Scholar
  77. 77.
    Holbach LM, Naumann GO, Font RL. Neuroglial choristoma presenting as congenital lid tumor. Graefes Arch Clin Exp Ophthalmol. 1989;227(6):584–8.PubMedCrossRefGoogle Scholar
  78. 78.
    Dithmar S, Schmack I, Volcker HE, et al. Phakomatous choristoma of the eyelid. Graefes Arch Clin Exp Ophthalmol. 2004;242(1):40–3.PubMedCrossRefGoogle Scholar
  79. 79.
    Ellis FJ, Eagle RC Jr, Shields JA, et al. Phakomatous choristoma (Zimmerman’s tumor). Immunohistochemical confirmation of lens-specific proteins. Ophthalmology. 1993;100(6):955–60.PubMedCrossRefGoogle Scholar

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Authors and Affiliations

  1. 1.School of Medical Sciences, University of HyderabadHyderabadIndia
  2. 2.Department of Ophthalmic Plastic Surgery and Ocular OncologyCentre for SightHyderabadIndia

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