Non-Hodgkin B-cell MALT lymphoma of marginal zone of the upper eyelid
DOI:
https://doi.org/10.31288/oftalmolzh202256570Keywords:
orbit, non-Hodgkin’s lymphoma, oncologist, brain, biopsyAbstract
Background: Non-Hodgkin lymphoma (NHL) represents a heterogenous group of malignant lymphoproliferative neoplasms originating from lymphoid tissue cells and having distinct clinical, cytomorphological, immunological and molecular and genetic features. Primary orbital lymphomas are rare and account for approximately 1% of all NHL. Lymphomas are, however, the most common primary orbital tumor in adults 60 years of age and older.
Purpose: To present a rare case of primary B-cell MALT lymphoma of marginal zone of the upper eyelid, to highlight its clinical features, and to demonstrate the importance of a multidisciplinary approach to its treatment.
Results: A male patient presented to the eye clinic complaining of bilateral pterygium and periodic nasal congestion. Within the course of examination and prolonged observation, the ophthalmologist suspected the symptoms of massive orbital disease, and the patient was referred for magnetic resonance imaging (MRI) of the brain. On the basis of imaging assessment and the oncologist’s opinion, the patient was hospitalized at the Podillia Regional Oncology Center with a preliminary diagnosis of orbital lymphoma. Thereat, a final diagnosis of “extranodal, non-Hodgkin’s B-cell lymphoma of the marginal zone (MALT lymphoma) of Stage 3a, with metastases in the lungs, pleura and lymph nodes of the mediastinum” was established. At the moment, he is under observation by an oncologist and on the way to stabilization of his major disease.
Conclusion: Management of orbital non-Hodgkin’s lymphomas remains a challenge requiring a multidisciplinary approach involving ophthalmologists and oncologists.
References
1.Chissov VI, Dar'yalova SL, editors. [Manual of Oncology]. Moscow: GEOTAR-Media; 2009. Russian.
2.Ahmed S, Shahid RK, Sison CP, Fuchs A, Mehrotra B. Orbital lymphomas: a clinicopathologic study of a rare disease. Am J Med Sci. 2006;331:79-83. https://doi.org/10.1097/00000441-200602000-00013
3.Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology. 2002;109:243-248. https://doi.org/10.1016/S0161-6420(01)00932-0
4.Isaacson PG, Wright DH. Malignant lymphoma of mucosal associated lymphoid tissue: a distinctive type of B cell lymphoma. Cancer. 1983;52:1410-1416. https://doi.org/10.1002/1097-0142(19831015)52:8<1410::AID-CNCR2820520813>3.0.CO;2-3
5.Ishii Y, Tomita N, Takasi H, Ogusa E, Hattori Y, Matsuura S, Matsumoto C, Takemura S, Kuwabara H, Ishigatsubo Y. Clinical features of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. Hematol Oncol. 2012;30:186-189. https://doi.org/10.1002/hon.2006
6.Poddubnaya IV, Moskalenko OA, Balakireva YuN. [Non-Hodgkin's lymphomas of the marginal zone]. Sovremennaia onkologiia. 2006;1(8):8-13. Russian.
7.Kozarezova TI, Klimkovich NN. [Pediatric blood disorders: a tutorial]. Minsk: Belorusskaia nauka;2001. Russian.
8.Grishina EE. [Malignant ocular lymphomas. Challenges and Perspectives from the Ophthalmologist's Viewpoint]. Sovremennaia onkologiia. 2006;8(4):27-29. Russian.
9.Coupland S, Krause L, Delecluse H, et al. Lymphoproliferative disorders of the ocular adnexa. Analysis of 112 cases. Ophthalmology. 1998 Aug;105(8):1430-41. https://doi.org/10.1016/S0161-6420(98)98024-1
10.Cahill M, Barnes C, Moriarty P, et al. Ocular adnexal lymphoma- comparison of I MALT lymphoma with other histological types. Br J Ophthalmol. 1999 Jun;83(6):742-7. https://doi.org/10.1136/bjo.83.6.742
11.Nakata M, Matsuno Y, Katsumata N, et al. Histology according to the Revised European-American Lymphoma classification significantly predicts the prognosis of ocular adnexal lymphoma. Leuk Lymphoma. 1999 Feb;32(5-6):533-43. https://doi.org/10.3109/10428199909058411
12.Knowles DM, Jacobiec FA, McNally L, Burke J. Lymphoid hyperplasia and malignant lymphoma occurring in the ocular adnexa (orbit, conjunctiva and eyelids): a prospective multiparametric analysis of 108 cases during 1977 to 1987. Hum Pathol. 1990 Sep;21(9):959-73. https://doi.org/10.1016/0046-8177(90)90181-4
13.Meunier J, Lumbroso-Le Rouich, Dendale R, et al. Conjunctival low-grade non-Hodgkin's lymphoma. Leuc Lymphoma. 2006 Jul;47(7):1295-305. https://doi.org/10.1080/10428190500518966
14.Khalil HA, de Keiser RJ, Kluin PM, et al. Clinical course and pathologic features of conjunctival non-Hodgkin's lymphoma. A report of six cases. Graefes Arch Clin Exp Ophthalmol. 1990;228(3):246-5. https://doi.org/10.1007/BF00920029
15.Cher L. Primary CNS Lymphoma. Cancer Forum. 1998;22(2): 138-40.
16.Connor JM. Problem in Lymphoma Management: Special Sites of Presentation. Oncology (Williston Park). 1998 Feb;12(2):185-91; discussion 192-5.
17.Jensen O.A., Johansen S., Kiss K. Intraocular T-cell lymphoma mimicking a ring melanoma. First manifestation of systemic disease. Report of a case and survey of the literature. Graefes Arch Clin Exp Ophthalmol. 1994 Mar;232(3):148-52. https://doi.org/10.1007/BF00176784
18.Roos DE, O'Brich PC, Crompton JL. Ocular involvement in primary central nervous system lymphoma: an increasing clinical problem? Australas Radiol. 1993 Nov;37(4):372-4. https://doi.org/10.1111/j.1440-1673.1993.tb00099.x
19.Medeiros LJ, Harmon DC, Linggood R.M., Harris N.L. Immunohistologic features predict clinical behavior of orbital and conjunctival lymphoid infiltrates. Blood. 1989. 1989 Nov 1;74(6):2121-9. https://doi.org/10.1182/blood.V74.6.2121.bloodjournal7462121
20.[Non- Hodgkin's lymphomas (NHL)]. In: Martynov AI, Kokorin VA, Gaewski P, editors. [Evidence-based medicine]. Medycyna Praktyczna: Krakow; 2018. Russian.
21.Armitage JO. Peripheral T-cell lymphoma. In: Canellos GP, Lister TA, Sklar JL, eds. The Lymphomas. Philadelphia: WB Saunders; 1998: 439-48.
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