NK/T-cell Lymphoma With Orbital Involvement: A Case Report and Systematic Review of the Literature

Abstract

Purpose: To present the clinical course of a patient with recurrent NK/T-cell lymphoma (NKTL) involving the orbit and to review the literature on patients with NKTL involving the orbit. Methods: The PubMed database was searched for all cases of NKTL involving orbital, intraocular, or adnexal ocular structures. Results: Ninety-six patients were included in the final analysis. The mean age of diagnosis was 48.1 ± 16.8 years. The patients were 53/96 (55.2%) male and 43/96 (44.8%) female. Tumor location varied and included the orbit in 80/96 (83.3%), nasosinus in 56/96 (58.3%), uvea in 11/96 (11.5%), lacrimal gland in 9/96 (9.4%), lacrimal drainage system in 11/96 (11.5%), and conjunctiva in 7/96 (7.3%) cases. Management included surgical debulking in 29/96 (30.2%) cases, radiotherapy in 52/96 (54.2%) cases, and chemotherapy in 82/96 (85.4%) cases. Median survival was 6 months (95% CI: 5–9). Chemotherapy (hazard ratio = 0.80, 95% CI: 0.67–0.95, p = 0.013), radiotherapy (hazard ratio = 0.75, 95% CI: 0.64–0.87, p < 0.001), and orbital involvement being a recurrence of disease (hazard ratio = 0.79, 95% CI: 0.67–0.95, p = 0.009) were associated with improved survival. Advanced Ann Arbor stage (III–IV) at diagnosis (hazard ratio = 1.22, 95% CI: 1.08–1.38, p = 0.001), vision loss (hazard ratio = 1.18, 95% CI: 1.04–1.34, p = 0.009), proptosis (hazard ratio = 1.15, 95% CI: 1.01–1.30, p = 0.035) and periorbital swelling (hazard ratio = 1.15, 95% CI: 1.00–1.33, p = 0.048) were associated with poor survival. Conclusions: NK/T-cell lymphoma involving the orbit, globe, or ocular adnexa heralds a poor prognosis where early diagnosis and therapy are critical. The use of radiotherapy and chemotherapy is associated with improved survival.

Anaplastic Large Cell Lymphoma With Intraocular or Ocular Adnexal Involvement: A Case Report and Systematic Review

“…An aggressive course is more common in T-cell OALs. 21 In our review, most ALCLs exhibited aggressive behavior, with notable inflammatory signs and symptoms. Furthermore, in patients with a history of misdiagnoses and prior glucocorticoid use, the results of pathological studies may be misleading.…”

“…Both OAL and IOL are considered “masquerade syndromes,” as they can present a wide variety of signs that mimic other conditions, including orbital cellulitis, pseudotumor, and uveitis. 21,38 This diversity of presentations can delay proper evaluation and appropriate treatment. 21 A study of 350 patients with NK/T-cell OAL found that 50% of primary OALs presented with symptoms that resembled orbital cellulitis, and 16.7% presented with pseudotumor-like symptoms.…”

“…Delayed diagnosis/treatment usually contributes to the poor prognosis of T-cell lymphomas, while poor response to initial treatment also indicates a dire outcome. 21 Prognosis is highly dependent on the stage at initial presentation. 30 Our study was significantly limited by the small number of cases and the lack of complete information.…”

“…39 In a systematic review of 96 patients of T-cell IOL and OAL revealed that 59.4% of patients (57 individuals) were initially misdiagnosed, causing a delay of at least 3 months in achieving the correct diagnosis for 26 patients, and 20 patients required multiple biopsies to confirm the diagnosis. 21 It should be noted that the most frequent form of OAL is the B-cell form, which typically molds rather than invades adnexal tissues with an indolent course. An aggressive course is more common in T-cell OALs.…”

“…20 Diagnosing IOL and OAL is challenging for both ophthalmologists and pathologists, as they can mimic steroid-resistant ocular inflammation, orbital cellulitis, and orbital pseudotumor. 21 The time between a confirmed diagnosis and the onset of ocular symptoms can range from 4 to 40 months, 22,23 although swift progression may also occur, which can contribute to tumor dissemination, clinical stage progression, and potentially poorer outcomes in some cases.…”