Comparison of Ocular Adnexal Mantle Cell Lymphoma and MALT Lymphoma

Jong Joo Lee; Min Su Kim; Hee Young Choi; Keun Sung Park; Sung Bok Lee

doi:10.3341/jkos.2015.56.6.815

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Lee, Kim, Choi, Park, and Lee: Comparison of Ocular Adnexal Mantle Cell Lymphoma and MALT Lymphoma

Original Article

J Korean Ophthalmol Soc 2015;56(6):815-822.

Published online: 7 September 2015

DOI: https://doi.org/10.3341/jkos.2015.56.6.815

Comparison of Ocular Adnexal Mantle Cell Lymphoma and MALT Lymphoma

Jong Joo Lee, MD¹, Min Su Kim, MD¹, Hee Young Choi, MD, PhD², Keun Sung Park, MD, PhD³, Sung Bok Lee, MD, PhD¹

¹Department of Ophthalmology, Chungnam National University, School of Medicine, Daejeon, Korea

²Department of Ophthalmology, Pusan National University School of Medicine, Busan, Korea

³Eyelove Eye Center, Daejeon, Korea

Address reprint requests to Sung Bok Lee, MD, PhD Department of Ophthalmology, Chungnam National University Hospital, #282 Munhwa-ro, Jung-gu, Daejeon 301-721, Korea, Tel: 82-42-280-7604, Fax: 82-42-255-3745 E-mail: sblee@cnu.ac.kr

Received 2 October 201416 January 2015 Accepted 12 May 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

Mantle cell lymphoma (MCL) is known to have systemic dissemination with poor prognosis, but very few cases have been reported in Korea. Therefore, we analyzed clinical features and prognosis of MCL by comparing 3 cases of ocular adnexal MCL and mucosa-associated lymphoid tissue (MALT) lymphoma.

Methods

The medical records of patients with ocular adnexal MALT lymphoma and MCL from Chungnam National University Hospital and Pusan National University Hospital from January 1999 to April 2014 were retrospectively reviewed. Immunohistochemical examination was selectively used to diagnose lymphoma subtypes. Systemic dissemination, treatment response, and recurrence were identified using radiological testing and bone marrow aspiration.

Results

This study included 39 patients with ocular and ocular adnexal MALT lymphoma and 1 patient with MCL from Chungnam National University Hospital and 53 patients with MALT lymphoma and 2 patients with MCL from Pusan National University Hospital. All 3 (100%) patients diagnosed with ocular adnexal MCL were over 60 years of age. However, 28 of 92 (30.4%) patients diagnosed with MALT lymphoma were over 60 years of age. In MALT lymphoma, 3 of 92 patients presented with systemic dissemination and most patients recovered with radiotherapy. Conversely, all 3 patients with MCL showed systemic dissemination and recurrence after radiotherapy or chemotherapy, therefore, additional chemotherapy was required.

Conclusions

Contrary to MALT lymphoma, ocular and ocular adnexal MCL usually presented with systemic dissemination and complete remission was difficult even though many regimens of chemotherapy were attempted. Because MCL can be misdiagnosed as MALT lymphoma, careful evaluation should be performed.

Keywords: MALT lymphoma, Mantle cell lymphoma, Ocular adnexal lymphoma

References

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Figure 1.

The photograph shows the both lower palpebral conjunctival mass.

Figure 2.

High-power photomicrograph shows a monotonous proliferation of small to medium sized lymphocyte with irreg-ular, hyperchromatic nuclei, and scant cytoplasm. There are hyalinized vessels in the background (H&E; ×400).

Figure 3.

Immunohistochemistry showing positive nuclear staining for CD20 (A), cyclin D1 (B), bcl-2 (C), and negative nuclear staining for CD5 (D), respectively.

Table 1.

Characteristics of 95 patients with MCL and MALT lymphoma

Characteristics	MCL (n = 3)	MALT lymphoma (n = 92)	All patients (n = 95)
Characteristics	MCL (n = 3)	MALT lymphoma (n = 92)	All patients (n = 95)	Age
Mean age	67.7	50.4
≥60	3 (100.0)	28 (30.4)	31 (32.6)
<60	0 (0.0)	64 (69.6)	64 (67.4)
Gender (n, %)
Male	2 (66.7)	35 (38.0)	37 (38.9)
Female	1 (33.3)	57 (62.0)	58 (61.1)
Laterality (n, %)
Unilateral	1 (33.3)	74 (80.4)	75 (78.9)
Bilateral	2 (66.7)	18 (19.6)	20 (21.1)
Location (n, %)
Orbit	1 (33.3)	12 (13.0)	13 (13.7)
Conjunctiva	1 (33.3)	53 (57.6)	54 (56.8)
Lacrimal gland	0 (0.0)	8 (8.7)	8 (8.4)
Eyelid	1 (33.3)	17 (18.5)	18 (19.0)
Sclera	0 (0.0)	2 (2.2)	2 (2.1)
Ann Arbor stage (n, %)
Ⅰ	0 (0.0)	84 (91.3)	84 (88.4)
Ⅱ	0 (0.0)	2 (2.1)	2 (2.1)
Ⅲ	0 (0.0)	3 (3.3)	3 (3.2)
Ⅳ	3 (100.0)	3 (3.3)	6 (6.3)
Bone marrow involvement (n, %)
Involvement	3 (100.0)	2 (2.2)	5 (5.3)
No involvement	0 (0.0)	90 (97.8)	90 (34.7)
Metastasis (n, %)	3 (100.0)	3 (3.3)	6 (6.3)
Overall treatment (n, %)
Radiotherapy only	0 (0.0)	49 (53.3)	49 (51.6)
Chemotherapy only	2 (66.7)	12 (13.0)	14 (14.7)
Radiotherapy + chemotherapy	1 (33.3)	11 (12.0)	12 (12.6)
Observation	0 (0.0)	14 (15.2)	14 (14.7)
Recurrence (n, %)	3 (100.0)	5 (5.4)	8 (8.4)
Follow-up period (months)	76.0	37.6	38.8

Values are presented as n (%).

MCL = mantle cell lymphoma; MALT = mucosa-associated lymphoid tissue.

Table 2.

Clinical features of 3 patients with mantle cell lymphoma

Case no.	1	2	3
Sex/age (years)	M/79	M/60	F/64
Initial symptom	Both conjunctival mass	Inguinal mass	Both conjunctival mass
	Both foggy vision
Initial diagnosis	Low grade marginal zone lymphoma of MALT	Mantle cell lymphoma	Diffuse mixed small and large cell type B cell lymphoma
Initial treatment	CVP #8	R-CHOP #8	CHOP #6
Primary orbital site	Conjunctiva	Orbit	Eyelid
Immunohistochemistry	CD20, cyclin D1, bcl-2(+)	CD20, CD5, cyclin D1(+)	CD20, cyclin D1, Bcl-2(+)
	CD3, CD5(-)	CD3, bcl-6, CD10(-)	CD3, CD5(+)
Final diagnosis	Mantle cell lymphoma	Mantle cell lymphoma	Mantle cell lymphoma
Site of metastasis	Both parotid gland left thyroid gland cervical, thoracic, abdominal lymph node bone marrow	Cecum, bone marrow, orbit	marrow Nasopharynx, endometrium, bone
Additional treatment	FC #4	IEOX #6	HyperCVAD + MA #6
	Bortezomib #6	IVIG + rituximab #4	ESHAP #5
		ESHAP #4	MINE #1
Follow-up period (months)	60	132	36

MALT = mucosa-associated lymphoid tissue; CVP = cyclophosphamide, vincristine, prednisolone; R-CHOP = rituximab, cyclo-phosphamide, hydroxydaunorubicin, oncovin, prednisolone; CHOP = cyclophosphamide, hydroxydaunorubicin, oncovin, prednisolone; FC = fludarabine, cyclophosphamide; IEOX = ifosfamide, etoposide, oxaliplatin, mesna; IVIG = intravenous immunoglobulin; ESHAP = eto-poside, cisplatin, high dose cytarabine, methylprednisolone; HyperCVAD = hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone; MA = methotrexate/cytarabine; MINE = mesna, ifosfamide, mitoxantrone, etoposide.

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