Graefe's Archive for Clinical and Experimental Ophthalmology

https://doi.org/10.1007/s00417-021-05075-6

MEDICAL OPHTHALMOLOGY

Ring lymphoma: highly indicative ultrasound biomicroscopy

findings of ciliary body lymphoma
Ailing Bian 1 & Hanyi Min 1 & Rongping Dai 1 & Chan Zhao 1 & Yang Zhang 1 & Anyi Liang 1 & Meifen Zhang 1

Received: 14 October 2020 / Revised: 25 November 2020 / Accepted: 5 January 2021

# The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature 2021

Abstract
Background To describe the ultrasound biomicroscopy (UBM) findings of extremely rare ciliary body lymphoma.
Methods This was a retrospective noncomparative case series study. Five patients (8 affected eyes) diagnosed with ciliary body
lymphoma at Peking Union Medical College Hospital from 2008 to 2019 were included. The UBM findings, including the
location, height, and acoustic features, were documented to assess the tumour characteristics.
Results UBM in all 8 (100%) affected eyes revealed 360° ring-like, solid infiltration of the ciliary body with low and homoge-
neous internal reflectivity. The continuity of the tumours could be most vividly demonstrated by transverse sections.
Conclusions Ciliary body lymphoma tends to grow in a characteristic circumferential 360° pattern, and the name “ring lymphoma
of the ciliary body” is proposed.

Key messages

In this study, we found that cilary body lymphoma tends to grow in a characteristic circumferential 360° pattern.
On UBM, Ciliary body lymphoma presents with low and homogeneous internal reflectivity.
UBM is a very valuable noninvasive tool for the differential diagnosis of ciliary body tumours.

Keywords Lymphoma . Ciliary body . Ultrasound biomicroscopy . Tumour

Background biomicroscopy (UBM) is a valuable tool for detecting small

Uveal lymphoma can be subdivided into
choroidal, iridal, and ciliary body lymphoma. It can also be
defined as primary if the uvea is the initial or the only site and
secondary if the uvea is the secondary manifestation of
systemic non-Hodgkin lymphoma.
Primary ciliary body lymphoma is extremely rare [1–6] and
secondary ciliary body lymphoma is also seldom described.
With a high resolution of 15 ~ 35 μm, ultrasound
tumours of the ciliary body which could otherwise be unde-
tectable by other noninvasive methods. Previously, the UBM
findings of ciliary body lymphoma have only been described
in two separate cases, both showing a 360° infiltration of the
ciliary body [2, 3]. In this study, we aimed to describe the
characteristic UBM findings of the 8 eyes diagnosed with
ciliary body lymphoma.

Methods
* Meifen Zhang
meifen_zhang@hotmail.com
This was a retrospective case series study that adhered to the
1
Department of Ophthalmology, Peking Union Medical College
tenets of the Declaration of Helsinki. Five patients (8 affected
Hospital, Chinese Academy of Medical Sciences & Peking Union eyes) were diagnosed with ciliary body lymphoma at Peking
Medical College, Beijing, China Union Medical College Hospital from 2008 to 2019. Data
 Graefes Arch Clin Exp Ophthalmol

were collected regarding patients’ demographics, clinical fea-

tures, UBM findings, and histopathological findings.
UBM was performed using an SW-3200 L system
(Suowei, China) with a 35/50-MHz scan transducer capable
of toggling between 35 and 50 MHz. Under topical anaesthe-
sia, an eyecup was mounted over the eye and filled with sterile
distilled water. Both radial and transverse (parallel to the lim-
bus) sections through the tumour were obtained. The heights
and extent of the tumour were documented, as well as the
internal acoustic structures and reflectivity.

Results

A total of 5 patients (8 affected eyes) were diagnosed with

ciliary body lymphoma at Peking Union Medical College
Hospital from 2008 to 2019. The patients’ demographics
and clinical features are presented in Table 1. One patient
was diagnosed with unilateral primary ciliary body lympho-
ma, and the other 4 were diagnosed with secondary ciliary
body lymphoma, which was bilateral in 3 cases and unilateral
in one case. All 5 patients presented with persistent, steroid-
resistant anterior uveitis. The intraocular pressure (IOP) was
elevated in 2 patients, with iris neovascularization. The best-
corrected visual acuity of the affected eyes at referral was as
follows: light perception in the left eye of patient 1; 20/100 in
the right eye and hand motion in the left eye of Patient 2; 20/
100 in both eyes of patient 3; hand motion in both eyes of
patient 4; and hand motion in the right eye of patient 5. Four
eyes demonstrated evident iris involvement, while the other 4
eyes showed suspicious involvement (Fig. 1a). None of the 8
eyes showed evident involvement of the choroid or retina.
UBM in all 8 (100%) affected eyes revealed 360° ring-like,
solid infiltration of the ciliary body (Fig. 1b). All the tumours
of the ciliary body grew in a circumferential pattern and the
continuity of the mass could be most vividly demonstrated by
transverse sections (Fig. 1c). The maximum height of the tu-
Fig. 1 Ultrasound biomicroscopy (UBM) images of ciliary body lym-
mours in the 8 affected eyes was 2.90 mm, 1.73 mm, phoma. (a) UBM showing prominent iris involvement in patient 1. (b)
3.26 mm, 1.15 mm, 1.01 mm, 2.20 mm, 1.81 mm, and UBM showing low homogeneous infiltration of the ciliary body. (c) The
3.70 mm, respectively. In all 8 (100%) affected eyes, the in- continuity of the 360° ring-like ciliary body lymphoma is evident on
ternal acoustic reflectivity was low and homogeneous. transverse sections with several ciliary body processes shown (white
arrow)

Table 1 Demographics and clinical features of patients with ciliary body lymphoma

Pt. no. Age Sex Involved eyes Uveitis IOP elevation Rubeosis Iris involvement Type Pathologic diagnosis

1 77 F Left Yes Yes Yes Yes Primary B cell lymphoma

2 60 M Both Yes No No Suspicious Secondary Mantle cell lymphoma
3 41 F Both Yes No No Suspicious Secondary T cell lymphoma
4 56 F Both Yes Yes Yes Yes Secondary NK/T cell lymphoma
5 49 M Right Yes No No Yes Secondary NK/T cell lymphoma

Abbreviations: Pt. no., patient number; F female; M male

Graefes Arch Clin Exp Ophthalmol

ƒFig. 2 Histopathology was performed on the enucleated eyeball of

patient 1. (a) Haematoxylin and eosin staining showing solid infiltration
of the ciliary body, with the optic nerve demonstrated. (b)
Immunohistochemical staining for CD20 showing a predominant
infiltrate of B cells in the ciliary body, with several ciliary body
processes clearly demonstrated. (c) Haematoxylin and eosin staining
showing the sclera and the ciliary body (magnification ×150). (d) A
higher magnification view of the diffusely distributed, large dysplastic
neoplastic cells. (magnification × 600)

Enucleation of the left eye was performed in patient 1 with

primary ciliary body lymphoma and the pathological examina-
tion demonstrated a completely circumferential B cell lymphoma
of the ciliary body, which was consistent with the UBM findings
in terms of the location of the tumour (Fig. 2). The pathological
diagnosis of the other 4 patients with secondary ciliary body
lymphoma was mantle cell lymphoma, T cell lymphoma, NK/
T cell lymphoma, and NK/T cell lymphoma, respectively
(Table 1). All the 4 patients diagnosed with secondary ciliary
body lymphoma had multiple extranodal involvements.

Discussion

In this study, we found that all the 8 eyes diagnosed with ciliary
body lymphoma bore the same characteristic UBM findings:
360° of infiltration with low and homogeneous internal reflec-
tivity. Previously, UBM findings of ciliary body lymphoma
have only been described in 2 separate cases, both of which
also showed 360° involvement of the ciliary body [2, 3]. In one
case in this study, the mass of the lymphoma was not as prom-
inent as that in other cases, with a maximum height of the
tumour as low as 1.0 mm, but it still presents a 360° appearance.
The continuity of the lymphoma was clearly demonstrated in
transverse sections on UBM, and we may infer that ciliary body
lymphoma tends to grow circumferentially; hence, we would
like to propose the term “ring lymphoma of the ciliary body”.
Because of the low incidence of ciliary body tumours, dif-
ferential diagnosis poses a big challenge. Malignant melanoma
is the most common ciliary body malignancy, and ring mela-
noma is a very unusual variant [7–9]. Ring melanoma of the
ciliary body is defined as a melanoma involving six or more
clock hours of the ciliary body in a circumferential growth
pattern, not necessarily meaning a 360° involvement. Hakan
Demerci et al. [7] reported that of the 8800 patients with uveal
melanoma, only 23 patients (0.3%) were found to have ring
melanoma of the ciliary body, and only 7 patients (0.08%)
had complete 360° involvement. How can we differentiate a
ciliary body lymphoma from a ring melanoma? The majority of
ring melanomas are melanotic, and in up to 100% of patients,
blockage of the light in the ciliary body at the site of the tumour
can be observed on transillumination [7]. Although both mela-
noma and lymphoma have a tendency towards extrascleral ex-
tension [8, 9], the clinical appearance, such as the colour and

blood vessels, may allow for differentiation. The acoustic fea-
tures of UBM may also have some diagnostic value. Most
ciliary body melanomas are described to have low to medium
reflectivity, so the differentiation may not be so straightforward,
but ciliary body melanoma often shows some irregular acoustic
sites, cystic spaces, lobulation, or hollowness [10–12], while all
the 8 eyes with ciliary body lymphoma in this study demon-
strated acoustic uniformity. Other lesions, such as
melanocytoma, metastatic tumours, leiomyoma, and hemangi-
oma, should also be considered as in the differential diagnosis,
but none of these lesions has been reported to have a 360°
growth pattern [10, 13] and would usually present a high or
heterogeneous acoustic reflectivity [10, 14]. In addition, we
would like to point out that uveal reactive lymphoid hyperplasia
(RLH), which has only been reported in dozens of cases, may
also present diffuse infiltration of the choroid and perhaps to a
lesser extent, the ciliary body. However, the term “uveal reac-
tive lymphoid hyperplasia” itself is controversial [15–17], and
with the development of newer, more modern diagnostic
methods, most of the cases previously considered to be RLH
have been found to actually be well differentiated B cell lym-
phoma [15]; additionally, uveal RLH may develop into lym-
phoma [17]. To date, no single case of RLH mainly involving
the ciliary body has ever been reported.
The iris was definitely involved in three cases in this study,
and for these cases, the term “iridociliary lymphoma” would be
more appropriate. Iris involvement had the appearance of nod-
ules or lumps, which may converge, although not in a circum-
ferential pattern; thus, in this manuscript, we only described the
characteristic 360° ring-like pattern of ciliary body involvement.
In summary, UBM is a highly valuable tool to detect small
ciliary body tumours that may be undetectable by other nonin-
vasive means. The 360° ring-like, solid infiltration of the ciliary
body on UBM with low and homogeneous internal reflectivity
is highly indicative, if not pathognomonic, of the diagnosis of
lymphoma. Due to the extremely low incidence of ciliary body
lymphoma, this study included only the 8 affected eyes, and
more cases are needed to corroborate our conclusion.
Authors’ contributions ALB wrote the main manuscript text and pre-
pared all figures. RPD, HYM, and MFZ oversaw the project and assisted
with the writing of the manuscript. CZ and YZ performed ophthalmic
examinations. AYL helped collect the data. All authors reviewed the
manuscript.
Compliance with ethical standards
Conflict of interest The authors declare that they have no conflicts of
interest.
Ethics approval This study was approved by the Institutional Review
Board of Peking Union Medical College Hospital and conformed to the
tenets of the Declaration of Helsinki.
Graefes Arch Clin Exp Ophthalmol
Consent to participate Written informed consent was obtained from all
subjects after an explanation of the nature of the study before entry into
this study.
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