Skip to main content
Journal cover image

Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients.

Publication ,  Journal Article
Kaliki, S; Jajapuram, SD; Maniar, A; Mishra, DK
Published in: Am J Ophthalmol
February 2019

PURPOSE: We studied the incidence, treatment, and outcome of ocular and periocular tumors in patients with xeroderma pigmentosum (XP). DESIGN: Retrospective case series. METHODS: This single-institution study included 120 patients with XP who underwent intervention with excisional biopsy, enucleation, or orbital exenteration. The primary outcome measures were the occurrence of eyelid or ocular surface tumor, globe salvage, locoregional and systemic metastasis, and death. RESULTS: The mean age at presentation was 19 years. A family history of XP was present in 32 (27%) patients. Over a mean follow-up of 61 months, 34 (28%) patients developed no ocular/adnexal tumor, 86 (72%) developed ocular surface malignancy, 15 (13%) developed eyelid malignancy, and 22 (18%) developed other head and neck malignancies. Of the 86 patients with ocular surface malignancy, 48 (56%) had unilateral tumor and 38 (44%) had bilateral tumors. Invasive squamous cell carcinoma (n = 51, 41%) was the most common ocular surface tumor. Of the 15 patients with eyelid tumors, 14 (93%) had unilateral tumor and 1 (7%) had bilateral involvement. Basal cell carcinoma (n = 8, 50%) was the most common eyelid tumor. There were events of ocular surface tumor recurrence (n = 55 eyes, 44%), eyelid tumor recurrence (n = 5 eyes, 31%), locoregional lymph node metastasis (n = 3, 2%), systemic metastasis (n = 1, 1%), and death (n = 1, 1%). Overall, globe salvage was achieved in 119 (99%) patients (both eyes were salvaged in 92 [76%] patients and at least 1 eye was salvaged in 27 [23%] patients). CONCLUSION: XP is frequently associated with ocular surface, eyelid, and other head and neck malignancies. Lifelong follow-up is mandatory in these patients.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Am J Ophthalmol

DOI

EISSN

1879-1891

Publication Date

February 2019

Volume

198

Start / End Page

146 / 153

Location

United States

Related Subject Headings

  • Young Adult
  • Xeroderma Pigmentosum
  • Visual Acuity
  • Retrospective Studies
  • Ophthalmology & Optometry
  • Middle Aged
  • Male
  • Lymphatic Metastasis
  • Infant
  • India
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Kaliki, S., Jajapuram, S. D., Maniar, A., & Mishra, D. K. (2019). Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients. Am J Ophthalmol, 198, 146–153. https://doi.org/10.1016/j.ajo.2018.10.011
Kaliki, Swathi, Sai Divya Jajapuram, Arpita Maniar, and Dilip K. Mishra. “Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients.Am J Ophthalmol 198 (February 2019): 146–53. https://doi.org/10.1016/j.ajo.2018.10.011.
Kaliki S, Jajapuram SD, Maniar A, Mishra DK. Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients. Am J Ophthalmol. 2019 Feb;198:146–53.
Kaliki, Swathi, et al. “Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients.Am J Ophthalmol, vol. 198, Feb. 2019, pp. 146–53. Pubmed, doi:10.1016/j.ajo.2018.10.011.
Kaliki S, Jajapuram SD, Maniar A, Mishra DK. Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients. Am J Ophthalmol. 2019 Feb;198:146–153.
Journal cover image

Published In

Am J Ophthalmol

DOI

EISSN

1879-1891

Publication Date

February 2019

Volume

198

Start / End Page

146 / 153

Location

United States

Related Subject Headings

  • Young Adult
  • Xeroderma Pigmentosum
  • Visual Acuity
  • Retrospective Studies
  • Ophthalmology & Optometry
  • Middle Aged
  • Male
  • Lymphatic Metastasis
  • Infant
  • India