Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Ophthalmologic Manifestations of Kaposi Sarcoma Treatment & Management

  • Author: Jacqueline Freudenthal, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Jan 08, 2015
 

Medical Care

The goal of therapy in patients with Kaposi sarcoma is to relieve ocular irritation, mass effect, and disfigurement. Therapy is not curative. If the patient has no ophthalmic complaints, the tumor is usually observed. For AIDS-related Kaposi sarcoma, consider immune reconstitution with triple antiviral medication.[20]

Kaposi sarcoma tends to respond to chemotherapy. Ophthalmologists may want to delegate the use of chemotherapy to a chemotherapist or an oncologist familiar with the use of the agents. If the patient has systemic involvement that requires chemotherapy, the eye lesion often resolves or is reduced markedly after starting this therapy. However, recurrence commonly follows after discontinuation of chemotherapy. If local treatment is indicated, defer for at least 4 weeks after systemic chemotherapy to allow for regression of the lesions.

Conventional Adriamycin, bleomycin sulfate, and vinblastine sulfate (ABV) combination therapy

Doxorubicin hydrochloride (Adriamycin) is administered at 40 mg/m2 every 4 weeks or 20 mg/m2 every 2-3 weeks.

Bleomycin sulfate is administered at 10 U/m2 every 2 weeks.

Vinblastine sulfate 1.4 mg/m2 with 2 mg maximum dose every 2 weeks has been recommended with ABV combination therapy. See Medication.

Single-agent therapies

Liposomal daunorubicin (DaunoXome) is administered 40 mg/m2 intravenously (IV) every 2 weeks until complete response; the response is comparable to ABV but with less resultant neutropenia.

Pegylated liposomal doxorubicin (Doxil) is administered at 20 mg/m2 every 3 weeks; the response is comparable to ABV or BV but with either lower or similar neutropenia risk.

Liposomal anthracyclines have a prolonged half-life when compared with their unencapsulated counterparts.

Alternative therapies

Paclitaxel (Taxol) is administered at 135 mg/m2 IV over 3 hours every 3 weeks or a total dose of 100 mg/m2 given IV over 3 hours every 2 weeks. All patients should be premedicated with the following 3 medications to prevent hypersensitivity reactions:

  • Dexamethasone 10 mg orally (PO) 12 hours before treatment
  • Diphenhydramine 50 mg IV 30 minutes prior to treatment
  • Cimetidine or ranitidine IV 30 minutes before treatment

If the absolute neutrophil count is greater than 1,000 cells/mcL, then continue with treatments. Reduce the dose of subsequent treatments by 20% if the neutrophil count falls below 500 cells/mcL. See the Absolute Neutrophil Count calculator.

Other chemotherapeutic agents used in Kaposi sarcoma include etoposide (VePesid), dactinomycin (Cosmegen), cisplatin (Platinol), and interferon alfa-n3 (Alferon N).

Intralesional chemotherapy [21]

Interferon alfa-2a administered at 0.5 mL of 3 million IU in a subconjunctival injection adjacent to the tumor was reported to be efficacious in a 46-year-old man with AIDS- related conjunctival Kaposi sarcoma.[22]

A 31-year-old man on highly active antiretroviral therapy (HAART) and systemic doxorubicin who initially received topical 3 million IU/mL of interferon alpha-2b eyedrops with no improvement received 3 injections (3 million IU in 0.5mL, 1.5 million IU in 0.5mL, and 1.5 million IU in 0.5 mL) over a 4-week period, leading to a dramatic decrease in tumor mass.[23]

Investigational medications

Human chorionic gonadotropin–associated factors

Because males are at a higher risk for developing Kaposi sarcoma, some investigators believe that female hormones may play a protective role. Hermans et al experienced complete or partial regression of skin lesions in 38% of patients with AIDS-related Kaposi sarcoma after intralesional injections of human chorionic gonadotropin (hCG).[24]

Samaniego et al demonstrated that human chorionic gonadotropin–associated factors (HAF) can induce apoptosis of Kaposi sarcoma cells in vivo and in vitro.[25]

Iron chelators

Simonart et al is investigating the role of regulating iron concentration in tumor cells.[26] They demonstrated that the addition of iron salts strongly stimulates the growth of Kaposi sarcoma and that the use of iron chelators inhibits growth. Their findings are disclosing new therapeutics in tumor management with iron-chelating agents.

Hermans et al speculate that the lower risk for developing Kaposi sarcoma in females may be because women have lower iron stores.[27] The analysis concludes that patients with renal transplants are more susceptible to Kaposi sarcoma because they tend to have higher iron loads from repeated blood transfusions.

Other experimental medications include fumagillin analogues (AGM 1470), bacterial cell-wall peptidoglycan (SP-PG derivatives), interleukin 4, and L-glutamine-tryptophan.

Intralesional cidofovir (an antiherpetic medication) injections have demonstrated poor results. Bailey et al have reported that all-trans -retinoic acid has not demonstrated optimistic results.[28]

Irradiation

Most patients respond immediately to radiation therapy, but recurrence is common. Consequently, irradiation is primarily used as a palliative measure. A typical treatment includes 2,000-3,000 cGy with the total dose given in fractions of 200-300 cGy per session over 3 weeks.

Ocular complications of radiation include lid atrophy, skin necrosis, ectropion, telangiectasia, tearing, eyelash loss, keratitis, cataract formation, conjunctival keratinization, skin erythema, keratoconjunctivitis sicca, alopecia, optic neuropathy, and lid notching.

Next

Surgical Care

Cryotherapy protocol

Subcutaneous injection of 2% lidocaine with epinephrine applied directly under the lesion is administered.

Cryoprobe with circulating liquid nitrogen is set at -30°C. Cryoprobe is applied to the tumor and extended out to 1-2 mm of clear margin until the lesion becomes bright white.

Antibiotic ointment is applied to the treated site.

Follow-up care is arranged for the following day.

Ocular complications of cryotherapy include depigmentation and erythema, lid notching, and epilation.

Surgical excision

Indications for local excision include cosmetically disturbing lesions, discomfort, and obstruction of vision from tumor bulk. Consider treating a lesion to prevent entropion formation with trichiasis and exposure keratopathy and corneal ulcer.

Protocol for local excision is as follows:

  • A cotton swab is soaked in 0.5% tetracaine hydrochloride, then applied directly over the tumor
  • Lidocaine 2% with epinephrine is injected subconjunctivally with a 25-gauge needle
  • Westcott scissors are used for blunt and sharp dissection to free the tumor. Clear margins of 1-2 mm are excised
  • The specimen is marked for orientation
  • Antibiotic ointment is applied, and the eye is patched
  • Patient should receive follow-up care the next day.

Protocol for fluorescein angiography demarcation of conjunctival lesions is as follows:

  • A 5 mL bolus of fluorescein dye is injected intravenously and photographed using a Zeiss SE-40 blue exciter filter.
  • The fluorescein angiography is used to mark tumor vessels with heat cautery.

Recurrence usually occurs within 4 weeks after surgical excision.

Conjunctival tumors

Dugel et al recommended an effective treatment regimen based on the clinical and histopathologic stage of the tumor.[29]

If the lesion is stage I or II and confined to the bulbar conjunctiva, excisional biopsy with 1-2 mm of clear margins is suggested.

For stage III tumors involving the bulbar conjunctiva, surgical excision guided by fluorescein angiography delineation of tumor margins resulted in no lesion recurrence during 4-8 months of follow-up care.

Alternative treatment can include intralesional mitomycin[30] or intralesional injections of interferon alfa-2b[23]

Recurrence usually occurs within 4 weeks after surgical excision.

Eyelid tumors [31]

Stage I and II lesions may be treated with cryotherapy. Stage II lesions may require radiation therapy.

Previous
Next

Consultations

If an eye lesion is identified, a thorough workup is necessary to rule out systemic involvement and HIV/AIDS infection.

After a thorough ophthalmologic examination, refer to an internist or oncologist/chemotherapist.

Disseminated Kaposi sarcoma may necessitate bronchoscopy, sigmoidoscopy, or lymph node biopsy for a definitive diagnosis and systemic chemotherapy.

Previous
 
 
Contributor Information and Disclosures
Author

Jacqueline Freudenthal, MD Co-Investigator, Ophthalmic Consultants Centre, Toronto

Jacqueline Freudenthal, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Canadian Ophthalmological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kevin Ryan Yuhan, MD Attending Physician, Cornea, Cataract, Refractive and External Diseases, Southern California Permanente Medical Group

Kevin Ryan Yuhan, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, Association for Research in Vision and Ophthalmology, Phi Beta Kappa, American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Timothy T You, MD Consulting Surgeon in Ophthalmology, Private Practice

Timothy T You, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Christopher J Rapuano, MD Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Hospital

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Ophthalmological Society, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, International Society of Refractive Surgery, Cornea Society, Eye Bank Association of America

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cornea Society, Allergan, Bausch & Lomb, Bio-Tissue, Shire, TearScience, TearLab<br/>Serve(d) as a speaker or a member of a speakers bureau for: Allergan, Bausch & Lomb, Bio-Tissue, TearScience.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

Anastasios J Kanellopoulos, MD Assistant Program Director, Clinical Associate Professor, Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, New York University

Anastasios J Kanellopoulos, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Eye Bank Association of America, and International Society of Refractive Surgery

Disclosure: Nothing to disclose.

References
  1. Kaposi M. Idiopathisches multiple Pigment-sarkom der Haut. Arch Dermatol. Syphilol. 1872:4. [Medline].

  2. Jones JL, Hanson DL, Dworkin MS, et al. Surveillance for AIDS-defining opportunistic illnesses, 1992-1997. MMWR CDC Surveill Summ. 1999 Apr 16. 48(2):1-22. [Medline].

  3. Holland GN, Gottlieb MS, Yee RD, et al. Ocular disorders associated with a new severe acquired cellular immunodeficiency syndrome. Am J Ophthalmol. 1982 Apr. 93(4):393-402. [Medline].

  4. Mowatt L. Ophthalmic manifestations of HIV in the highly active anti-retroviral therapy era. West Indian Med J. 2013. 62(4):305-12. [Medline].

  5. Becker KN, Becker NM. Ocular manifestations seen in HIV. Dis Mon. 2014 Jun. 60(6):268-75. [Medline].

  6. Verma V, Shen D, Sieving PC, Chan CC. The role of infectious agents in the etiology of ocular adnexal neoplasia. Surv Ophthalmol. 2008 Jul-Aug. 53(4):312-31. [Medline]. [Full Text].

  7. Chang Y, Cesarman E, Pessin MS, et al. Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi's sarcoma. Science. 1994 Dec 16. 266(5192):1865-9. [Medline].

  8. Pantry SN, Medveczky PG. Epigenetic regulation of Kaposi's sarcoma-associated herpesvirus replication. Semin Cancer Biol. 2009 Jun. 19(3):153-7. [Medline]. [Full Text].

  9. Ye F, Zhou F, Bedolla RG, Jones T, Lei X, Kang T, et al. Reactive oxygen species hydrogen peroxide mediates Kaposi's sarcoma-associated herpesvirus reactivation from latency. PLoS Pathog. 2011 May. 7(5):e1002054. [Medline]. [Full Text].

  10. Sakakibara S, Tosato G. Viral interleukin-6: role in Kaposi's sarcoma-associated herpesvirus: associated malignancies. J Interferon Cytokine Res. 2011 Nov. 31(11):791-801. [Medline]. [Full Text].

  11. Ye F, Lei X, Gao SJ. Mechanisms of Kaposi's Sarcoma-Associated Herpesvirus Latency and Reactivation. Adv Virol. 2011. 2011:[Medline]. [Full Text].

  12. Traylen CM, Patel HR, Fondaw W, et al. Virus reactivation: a panoramic view in human infections. Future Virol. 2011 Apr. 6(4):451-463. [Medline]. [Full Text].

  13. Centers for Disease Control and Prevention. Update: AIDS--United States, 2000. JAMA. 2002 Aug 14. 288(6):691-2. [Medline].

  14. Mansour AM. Adnexal findings in AIDS. Ophthal Plast Reconstr Surg. 1993 Dec. 9(4):273-9. [Medline].

  15. Gigase PL, de Muynck A, de Feyter M. Kaposi's sarcoma in Zaire. IARC Sci Publ. 1984. 549-57. [Medline].

  16. Pantanowitz L, Dezube BJ. Kaposi sarcoma in unusual locations. BMC Cancer. 2008. 8:190. [Medline]. [Full Text].

  17. Dugel PU, Gill PS, Frangieh GT, Rao NA. Ocular adnexal Kaposi's sarcoma in acquired immunodeficiency syndrome. Am J Ophthalmol. 1990 Nov 15. 110(5):500-3. [Medline].

  18. Meyers D. Eye signs that alert the clinician to a diagnosis of AIDS. SADJ. 2005. 60:386-387. [Medline].

  19. Emina MO, Odjimogho SE. Ocular problems in HIV and AIDS patients in Nigeria. Optom Vis Sci. 2010 Dec. 87(12):979-84. [Medline].

  20. [Guideline] Mofenson LM, Brady MT, Danner SP, et al. Guidelines for the Prevention and Treatment of Opportunistic Infections among HIV-exposed and HIV-infected children: recommendations from CDC, the National Institutes of Health, the HIV Medicine Association of the Infectious Diseases Society of America, the Pediatric Infectious Diseases Society, and the American Academy of Pediatrics. MMWR Recomm Rep. 2009 Sep 4. 58:1-166. [Medline]. [Full Text].

  21. Brasnu E, Wechsler B, Bron A, et al. Efficacy of interferon-alpha for the treatment of Kaposi's sarcoma herpesvirus-associated uveitis. Am J Ophthalmol. 2005 Oct. 140(4):746-8. [Medline].

  22. Hummer J, Gass JD, Huang AJ. Conjunctival Kaposi's sarcoma treated with interferon alpha-2a. Am J Ophthalmol. 1993 Oct 15. 116(4):502-3. [Medline].

  23. Qureshi YA, Karp CL, Dubovy SR. Intralesional interferon alpha-2b therapy for adnexal Kaposi sarcoma. Cornea. 2009 Sep. 28(8):941-3. [Medline].

  24. Hermans P. Epidemiology, etiology and pathogenesis, clinical presentations and therapeutic approaches in Kaposi's sarcoma: 15-year lessons from AIDS. Biomed Pharmacother. 1998. 52(10):440-6. [Medline].

  25. Samaniego F, Bryant JL, Liu N, et al. Induction of programmed cell death in Kaposi's sarcoma cells by preparations of human chorionic gonadotropin. J Natl Cancer Inst. 1999 Jan 20. 91(2):135-43. [Medline].

  26. Simonart T, Noel JC, Andrei G, et al. Iron as a potential co-factor in the pathogenesis of Kaposi's sarcoma?. Int J Cancer. 1998 Dec 9. 78(6):720-6. [Medline].

  27. Hermans P, Clumeck N, Picard O, et al. AIDS-related Kaposi's sarcoma patients with visceral manifestations. Response to human chorionic gonadotropin preparations. J Hum Virol. 1998 Jan-Feb. 1(2):82-9. [Medline].

  28. Bailey J, Pluda JM, Foli A, et al. Phase I/II study of intermittent all-trans-retinoic acid, alone and in combination with interferon alfa-2a, in patients with epidemic Kaposi's sarcoma. J Clin Oncol. 1995 Aug. 13(8):1966-74. [Medline].

  29. Dugel PU, Gill PS, Frangieh GT, Rao NA. Treatment of ocular adnexal Kaposi's sarcoma in acquired immune deficiency syndrome. Ophthalmology. 1992 Jul. 99(7):1127-32. [Medline].

  30. Korn BS, Park DJ, Kikkawa DO. Intralesional mitomycin-C for the treatment of conjunctival Kaposi sarcoma. Ophthal Plast Reconstr Surg. 2011 Jul-Aug. 27(4):e88-90. [Medline].

  31. Shuler JD, Holland GN, Miles SA, et al. Kaposi sarcoma of the conjunctiva and eyelids associated with the acquired immunodeficiency syndrome. Arch Ophthalmol. 1989 Jun. 107(6):858-62. [Medline].

  32. Gill PS, Wernz J, Scadden DT, et al. Randomized phase III trial of liposomal daunorubicin versus doxorubicin, bleomycin, and vincristine in AIDS-related Kaposi's sarcoma. J Clin Oncol. 1996 Aug. 14(8):2353-64. [Medline].

  33. Northfelt DW, Dezube BJ, Thommes JA, et al. Efficacy of pegylated-liposomal doxorubicin in the treatment of AIDS-related Kaposi's sarcoma after failure of standard chemotherapy. J Clin Oncol. 1997 Feb. 15(2):653-9. [Medline].

  34. Welles L, Saville MW, Lietzau J, et al. Phase II trial with dose titration of paclitaxel for the therapy of human immunodeficiency virus-associated Kaposi's sarcoma. J Clin Oncol. 1998 Mar. 16(3):1112-21. [Medline].

 
Previous
Next
 
Section of eyelid with Kaposi sarcoma lesion under high magnification. This tissue section demonstrates increased angiogenesis and spindle-shaped cells. Courtesy of Ben Glasgow, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
Section of eyelid with Kaposi sarcoma lesion under high magnification. This tissue section demonstrates endothelium-lined vascular channels and proliferation of spindle-shaped cells. Increased angiogenesis with erythrocyte extravasation is observed. Courtesy of Ben Glasgow, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
The inferior conjunctiva is involved more commonly than the superior conjunctiva in Kaposi sarcoma. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
Kaposi sarcoma involvement of the eyelid. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.