Article

Case report

Castelman's disease of the neck: a case report and

Amel El Korbi, Sondes Jellali, Mahbouba Jguiri, Ahlem Bellalah, Mehdi Ferjaoui, Rachida Bouatay, Naourez Kolsi,
Khaled Harrathi, Jamel Koubaa

Corresponding author: Amel El Korbi, ENT Department of Fattouma Bourguiba, University Hospital of Monastir,
Monastir, Tunisia. elkorbiamel@gmail.com

Received: 10 Nov 2020 - Accepted: 14 Nov 2020 - Published: 22 Dec 2020

Keywords: Castleman’s disease, lymphadenopathy, surgery

Copyright: Amel El Korbi et al. Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed
under the terms of the Creative Commons Attribution International 4.0 License
(https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any
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Cite this article: Amel El Korbi et al. Castelman's disease of the neck: a case report and literature review. Pan African
Medical Journal. 2020;37(369). 10.11604/pamj.2020.37.369.26909

Available online at: https://www.panafrican-med-journal.com//content/article/37/369/full

Castelman´s disease of the neck: a case report and Pathology Department, Fattouma Bourguiba
literature review University Hospital of Monastir, Monastir, Tunisia
&
Amel El Korbi1,2,&, Sondes Jellali1, Mahbouba Jguiri3, Corresponding author
Ahlem Bellalah4, Mehdi Ferjaoui1, Rachida Amel El Korbi, ENT Department of Fattouma
Bouatay1, Naourez Kolsi1,2, Khaled Harrathi1, Jamel Bourguiba, University Hospital of Monastir,
Koubaa1 Monastir, Tunisia

1
ENT Department of Fattouma Bourguiba,
University Hospital of Monastir, Monastir, Tunisia,
2
Research Unit “Quality and Security of care”
(UR12SP41), University of Monastir, Monastir,
Tunisia, 3Department of Rheumatology, Fattouma
Bourguiba University Hospital of Monastir,
Monastir, Tunisia, 4Anatomical and Cytological

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Abstract for 8 months. The physical examination found
multiple firm and non-fixed left lateral neck
Castleman’s disease is a rare pathology, poorly masses; the largest one measured five centimeters.
understood. It is considered as a Ultrasound echography showed a 4 centimeters
lymphoproliferative disorder, described for the first well-limited hypoechoic oblong mass with central
time in 1954, which may be confused with other and peripheral vascularization of the level IIb with
causes of lymphadenopathy. We report in this multiple centimeter lymph nodes at level V (Figure
paper the case of a young women presenting with 1). Computed tomography (CT) showed multiple
left latero-cervical lymphadenopathy. All the lymphadenopathies of the left levels II and V with
investigations were negative except a large high- an important enhancement after contrast sized
vascularized level II cervical lymphadenopathy. We between 20 and 41 millimeters in their largest
performed a cervicotomy. The extemporaneous dimension (Figure 2). Two fine needle aspirations
histological exam was non-contributive. We attempts were non-contributive. A surgical biopsy
decided to perform a complete level II and III left under general anesthesia was carried but the
cervical lymphadenectomy. The diagnosis of extemporaneous examination was non-conclusive.
unicentric Castleman's disease was confirmed We decided to perform a II, III and VA left levels
based on the final histological study of the lymphadenectomy removing the whole lymph
specimen, and the absence of other cervical and nodes. The extirpation was laborious because of
extra-cervical lymphadenopathy. The patient is free bleeding. The diagnosis of hyaline Castleman´s
of recurrence at the time of reporting this article. disease was made based on final histological
findings (Figure 3). The thoraco-abdominal CT scan
did not show any other lymph node considering
Introduction though the unicentric form of CD. We did not
observe recurrence or new lymphadenopathy
Castleman´s disease (CD) is a rare, poorly
appearance 12 months after surgery.
understood lymphoproliferative disorder that
share common lymph node histological features.
The disease was first described in a single case in Discussion
954 [1], followed by a small cases series in 1956 [2].
Two clinical entities have been described: CD is a rare lymphoproliferative disorder, described
Unicentric with a confined disease to a single for the first time by Castleman et al. [1,2] in 1954 in
anatomic lymph node and multicentric a single case then in 1956 in a group of 13 patients
characterized by generalized lymphadenopathy with localized benign lymphadenopathy. This entity
and more aggressive clinical course [3]. It has three is also termed localized nodal hyperplasia,
histological subtypes: hyaline vascular, plasma angiomatous lymphoid hamartoma or giant lymph
cellular and mixed type. Most of the previously node hyperplasia [1]. Williams and Kaude reported
reported cases of CD in the neck were of the hyaline that lymph nodes in the mediastinum were the
vascular type and the most common sign was an most common (70%) and that the neck and
asymptomatic neck mass [4]. The aim of this paper abdomen were the least common (15%) sites for
is to describe a case of cervical unicentric CD, its CD [5]. Although the exact pathogenesis of CD is
diagnostic tools and the perioperative unclear, studies have indicated that the occurrence
management. of CD may be related to the infection of human
herpes virus-8 (HHV-8) or human
immunodeficiency virus (HIV), immune dysfunction
Patient and observation and overproduction of interleukin-6 (IL-6). Asao et
al. have proved IL-6 transgenic mice showed similar
A 23-year-old woman who presented with an
disorders related to CD, which suggested the
isolated left side painless mass of the neck evolving

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contribution of IL-6 to CD [6]. The definitive often shows well defined, mildly hypodense or
diagnosis of CD is based on histopathological isodense, homogeneous nodules or masses on non-
examination. Three types of CD have been enhanced computed tomography/magnetic
identified: hyaline-vascular type, plasma cell type resonance (CT/MR) images, and intermediate and
and mixed variant type [4,7]. Hyaline-vascular type marked enhancement on contrast-enhanced
is the most common clinical variant (90%) CT/MR images. The hypertrophied vessels are
characterized by follicular hyperplasia with valuable features [11]. The calcification in affected
regressed germinal centers and important vascular lesions is not rare and is more commonly observed
proliferation. Plasma cell type is characterized by in hyaline vascular variant unicentric CD [12].
Russell bodies and exhibits larger lymphoreticular
nodules and fewer hyalinized blood vessels In our case, ultrasound and CT scan evoked the
compared with hyaline-vascular type. Mixed tuberculosis origin as a first diagnosis. Since
variant type is a rare variant and exhibits features cytological appearances vary depending on the
of hyaline-vascular and plasma cell type [4]. type and extent of hyperplasia, fine needle
aspiration cytology (FNAC) findings may not always
The incidence of CD is unknown and can occur at be conclusive in all cases [13]. Even in our case, the
any age, though it is mostly reported in adults in the two FNAC performed were non-contributive.
literature with a slight feminine predominance Surgical resection is considered the cornerstone of
(60%). There are two different clinical entities: the curative treatment for unicentric CD and is the
unicentric and the multicentric type. The localized most widely accepted therapy in the literature. A
form of the disease is mostly asymptomatic with a systematic review by Talat et al. [14] of 278
single site lymph node enlargement. Although unicentric patients found that surgical resection
localized CD most often occurs in the mediastinum, resulted in 95% disease-free survival at 3 years. If
it may occur in any area of the body where lymph surgical removal is not possible, in cases that the
nodes are found such as the lung, neck, axilla, lymph node is difficult to get to, radiation therapy
mesentery, pelvis, and retroperitoneum [6]. It is may be an effective way to destroy the affected
often discovered incidentally during routine tissue. Radiation doses of 30-45 Gy appear to be
examination, chest X rays or due to discomfort effective, although tumor responses have been
secondary to local compression. Diagnosis is made documented at lower doses [7]. Multicentric CD
by histological analysis of the lymph node however tends to have a variable prognosis with no
biopsy [7,8]. Multicentric form, however, presents documented treatment consensus. A variety of
with systemic symptoms along with multiple lymph combination treatments have been tried with
node hyperplasia. The systemic symptoms are surgical excision, chemotherapy and steroids [11].
thought to be primarily a consequence of elevated In patients with associated Kaposi´s sarcoma
Interleukin-6 (IL-6) production. They present as monthly polychemotherapy (e.g.
asthenia, weight loss, fever, polyadenopathy with a cyclophosphamide, vincristine, doxorubicin and
mean of four-site involvement and is often prednisone) has been tried with limited success [8].
associated with hepatosplenomegaly [8]. Some Anti-IL 6 antibodies have shown success with
forms of multicentric CD are associated with systemic symptoms, as have steroids. Most
Kaposi´s sarcoma, which develops in the clinical treatment modalities involve immunosuppression,
course of most HIV positive multicentric CD cases. increasing the chances of opportunistic
An association with HIV negative Kaposi´s sarcoma infections [11]. Recent suggestions are that
has also been seen to a much lesser extent [9]. HIV treatment with the anti-herpes virus drug
positive multicentric CD shows an increased gangciclovir or the antiCD20 B cell monoclonal
prevalence of pulmonary symptoms and can be antibody, rituximab, may markedly improve
differentiated from other types of HIV-associated outcome [8].
systemic lymphoproliferative disorders [9,10]. CD

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Castleman's disease: A case report of the
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Figure 1: ultrasound images of cervical lymph nodes located in left IIB and V levels

Figure 2: cervical CT scan with contrast enhancement showing cervical lymph node in left IIB and V
levels with a strong enhancement of the IIB level lymph node (white arrow)

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Figure 3: A) (HE x 100) the follicle is surrounded by a broad mantle zone consisting of a concentric layering
of lymphocytes resulting in an "onion-skin" appearance; B) (HE x 400). The follicle is penetrated radially by
a sclerotic blood vessel

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