International Journal of Surgery Case Reports 95 (2022) 107282

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International Journal of Surgery Case Reports

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Case report

Castleman's disease: A rare case report and review of literature

Mohamed Abdelraouf Hassan Saadallah *
Department of General Surgery, Kulhudhuffushi Regional Hospital, Maldives

A R T I C L E I N F O A B S T R A C T

Keywords: Castleman's disease – also known as giant lymph node hyperplasia – is a rare pathology, poorly understood.
Castleman's disease Described for the first time in 1954, it is considered a lymphoproliferative disorder that manifests without any
Lymph nodes other obvious symptoms and may be confused with other causes of lymph nodal enlargement. We are reporting
Neck surgery
in this paper the case of a 30-year-old Maldivian female who presented to our surgical OPD with a right cervical
large solitary soft tissue lesion. All the investigations were negative except for a large highly vascularized uni­
centric soft tissue mass measuring around 4 cm in the anterior triangle of the neck seen by the ultrasound
separable from all other surroundings. An excisional biopsy & complete resection was done under general
anesthesia in the operation theater of a Maldivian Regional hospital. Unicentric Castleman's disease as a diag­
nosis was confirmed based on the final histopathological study of the specimen, and the absence of other cervical
and extra-cervical lymphadenopathy. The patient doesn't have recurrence or newly developed lymph nodes at
the time of reporting this article.

1. Introduction evolving over the past 4 years. On physical examination a solitary firm
and non-fixed right lateral neck mass was found measuring around four
Castleman's disease (CD) is a rare, often poorly understood lym­ centimeters. Ultrasound echography showed a 4 centimeter well-
phoproliferative disorder that shares common lymph node histological defined hypoechoic oval mass with central and peripheral vasculariza­
features. The disease was first described by Dr. Benjamin Castleman in a tion of the right anterior aspect of the lower neck (Fig. 1). Excisional
single case in 1954 [1], followed by a small cases series in 1956 [2]. biopsy was done under general anesthesia by The Surgical Team of a
There were two clinical entities described: Unicentric with a confined General Surgery Specialist (the author) & OT nurses in the operation
disease to a single anatomic lymph node and multicentric which is theater of a Maldivian Regional hospital and the mass was completely
characterized by generalized lymphadenopathy and more aggressive & excised then sent for histopathological assessment (Fig. 2). The patient's
progressive clinical course [3]. It has three histological subtypes: hyaline postoperative stay went uneventful. The diagnosis of hyaline Castle­
vascular, plasma cellular and a mixed type. The clinical characteristics man's disease was made based on final histological findings (Fig. 3). The
and survival differ significantly between the three subtypes. The ma­ postoperative imaging did not show any other systemic lymph nodal
jority of the previously reported cases of CD in the neck were of the enlargement. We did not observe recurrence, newly developed neck
hyaline vascular type and the most common sign was an asymptomatic masses, or new systemic lymphadenopathy appearance 12 months after
neck mass same as our case report [4]. The aim of this case reporting is surgery during regular clinic monthly follow-ups and systemic workup.
to describe a spotted case of cervical unicentric CD, its diagnostic tools,
the perioperative management after 12 months of close monitoring. 3. Discussion
This case report has been reported in line with SCARE 2020 criteria
[5]. CD is a rare lymphoproliferative disorder, described for the first time
by Castleman et al. [1,2] in 1954 in a single case then in 1956 in a group
2. Presentation of case of 13 patients with localized benign lymphadenopathy. This rare disease
is also termed as localized nodal hyperplasia, angiomatous lymphoid
A 30-year-old Maldivian female patient medically & surgically free hamartoma, or giant lymph node hyperplasia [1]. Williams and Kaude
presented with an isolated right sided painless mass of the neck slowly both reported that lymph nodes in the mediastinum were the most

* Kulhudhuffushi, Maldives.
E-mail address: mohamedabdelraouf@[Link].

[Link]
Received 25 March 2022; Received in revised form 19 May 2022; Accepted 5 June 2022
Available online 7 June 2022
2210-2612/© 2022 The Author. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
([Link]
M.A.H. Saadallah International Journal of Surgery Case Reports 95 (2022) 107282

Fig. 1. Ultrasound images of the oval right sided neck mass.

Fig. 2. Intraoperative view of the solitary lymph node from the anterior triangle of the neck.

common presentation (70 %) and that the neck and abdomen were the
least common (15 %) sites for CD [6]. Even though the exact patho­
genesis of CD is vague, studies have indicated that the occurrence of CD
may be related to the infection of human herpes virus-8 (HHV-8) or
human immunodeficiency virus (HIV), immune dysfunction, and
excessive production of interleukin-6 (IL-6). Asao et al. was the first one
to prove that IL-6 transgenic mice showed similar disorders related to
CD, which supported the contribution of IL-6 to CD [7]. The final
conclusive diagnosis of CD is based on histopathological assessment.
There are three different types of CD: hyaline-vascular type, plasma cell
type and mixed variant type [4,8]. Hyaline-vascular type is the most
common clinical finding (90 %) characterized by follicular hyperplasia
with regressed germinal centers and important vascular proliferation.
Plasma cell type is characterized by Russell bodies and exhibits larger
lymphoreticular nodules and fewer hyalinized blood vessels compared
with the hyaline-vascular type. Mixed variant type is a rare variant and
exhibits features of hyaline-vascular and plasma cell type [4].
The incidence of CD is unknown and can occur at any age, however it
Fig. 3. Histology slides: the follicle is surrounded by a broad mantle zone is mainly reported in adults in the literature with a slight feminine
consisting of a concentric layering of lymphocytes resulting in an “onion- predominance (60 %). There are two different clinical types: the uni­
skin” appearance. centric and the multicentric type. The localized form of the disease like
the case that we are reporting is mostly asymptomatic with a single site
lymph node enlargement. Although localized CD most often occurs in
the mediastinum, it may occur in any other areas of the body where

2
M.A.H. Saadallah International Journal of Surgery Case Reports 95 (2022) 107282

lymph nodes are found such as the lung, neck, axilla, mesentery, pelvis, Consent
or retroperitoneum [6]. It is often discovered incidentally during routine
examination, chest X rays, or due to discomfort secondary to local Written informed consent was obtained from the patient for publi­
compression. Diagnosis is confirmed by histopathological assessment of cation of this case report and accompanying images. A copy of the
the lymph node biopsy sent postoperative [8,9]. The multicentric form, written consent is available for review by the editor of this journal.
however, presents with systemic symptoms along with multiple lymph
nodes hyperplasia. The systemic symptoms are thought to be initially a Ethical approval
consequence of elevated Interleukin-6 (IL-6) production. They present as
asthenia, weight loss, fever, polyadenopathy with a mean of four-site Not applicable.
involvement and are often associated with hepatosplenomegaly [9].
Some other forms of multicentric CD are associated with Kaposi's sar­ Funding
coma, which develops in the clinical course of most HIV-positive mul­
ticentric CD cases. Such cases show an increased prevalence of No funding was obtained for the development of this paper.
pulmonary symptoms and can be differentiated from other types of HIV-
associated systemic lymphoproliferative disorders [10,11]. It was Guarantor
noticed that HIV-negative Kaposi's sarcoma is associated with CD to a
much lesser extent [10]. CD often shows well-defined, mildly hypodense N/A.
or isodense, homogeneous nodules or masses on different imaging mo­
dalities (CT/MR), and intermediate and marked enhancement on Research registration number
contrast-enhanced CT/MR images. The hypertrophy of blood vessels is
also considered as valuable features [12]. The calcification in affected N/A.
lesions is common and is more commonly observed in hyaline vascular
variant unicentric CD [13]. Credit authorship contribution statement
In our case, ultrasound was enough as an indicator for the solitary
well-defined lymph node that was clear and separable from other sur­ Mohamed Saadallah was responsible for collecting information of
roundings. Fine needle aspiration cytology (FNAC) findings may not the current case, performing a literature review, and writing the
always be conclusive. CT and MRI are usually of great value in the case manuscript.
prognosis. Surgical resection is considered the cornerstone of radical
treatment for unicentric CD and is the most widely accepted therapy in
the literature therefore the upfront excision decision was taken in our Declaration of competing interest
case for diagnostic and radical concerns. A systematic review by Talat
et al. [14] of 278 unicentric patients found that surgical resection None declared.
resulted in 95 % disease-free survival at 3 years. In case the surgical
resection was not applicable, or the lymph node is not accessible, radi­ References
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