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| Journal of the New Zealand Medical Association,
04-July-2008, Vol 121 No 1277
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Castleman’s disease is a rare lymphoproliferative
disorder characterised by masses of lymphoid tissue. The most common site of the
disease is the mediastinum.
We report the rare case of a 36-year-old Chinese man with a
Castleman’s tumour in the retroperitoneal cavity located below the level
of the right kidney—and mimicking a sarcoma, neural tumour, metastasis, or
lymphoma.
Case reportA 36-year-old man presented with a self-detected mass in the
right lower abdominal quadrant. He had no medical history or past surgical
history. Physical examinations were generally normal, except for the palpable
mass over the right lower quadrant of the abdomen. The results of a routine
complete blood count and his serum biological profile were unremarkable.
A heterogeneously enhanced lesion with multiple
low-attenuation areas was present on post-contrast computed tomography (CT;
Figure 1). The mass was in the right retroperitoneal cavity, adjacent to the
right psoas muscle. The fat plane between the retroperitoneal mass, right psoas
muscle, common iliac vessels, and right ureter was clear. The encapsulated mass
was completely removed.
Figure 1. Axial post-contrast CT scan shows a
heterogeneously enhanced right retroperitoneal mass with multiple
low-attenuation areas (black arrow)
 Its gross pathology revealed an encapsulated tumour
measuring about 8 cm, located in the right retroperitoneal cavity, grey in
colour, with an ovoid-shaped contour and with haemorrhage. Microscopically, the
specimen showed the proliferation of numerous lymphoid follicles with
collagenous germinal centres and hyaline vascular proliferation, the appearance
typical of hyaline vascular-type Castleman’s disease. There were no
surgical complications and the patient recovered well.
A CT scan after 3 months was normal, with no evidence of
recurrence.
DiscussionCastleman’s disease is a poorly understood
proliferative disease of the lymphoid tissue that occurs mainly in young,
healthy patients in the age range of 8–66 years, with no sex
predominance.1 Most (70%) Castleman’s
disease occurs in the mediastinum, with 20% in the neck, shoulder, and inguinal
regions, and 7% in the retroperitoneum.2
Castleman’s disease is classified into two
pathological subtypes: a localised and a multicentric
subtype.3 Localised Castleman’s disease
presents as a solitary mass, which may be well circumscribed and usually has a
benign course. In contrast, multicentric Castleman’s disease usually has a
worse clinical course, which can include malignancy, infection, and
death.4
Abdominal Castleman’s disease has been described in
the radiology literature as an enhanced mass
over the mesentery, retroperitoneum, porta hepatis, and
pancreas.5 Meador and McLarney reported that an
enhanced pattern on abdominal CT and a Castleman’s disease tumour of less
than 5 cm usually presents with homogeneous enhancement, whereas tumours greater
than 5 cm usually present with heterogeneous enhancement with areas of low
attenuation.5 These radiological features are
not sufficiently specific to differentiate Castleman’s disease from
retroperitoneal masses such as sarcoma, metastasis, lymphoma, or neural
tumour.
To the best of our knowledge, retroperitoneal sarcoma is
usually a large unilateral mass, displacing the retroperitoneal organs or
viscera. It is usually heterogeneously enhanced on contrast-enhanced CT.
Retroperitoneal lymphoma tends to surround the adjacent
vessels, presenting with a “floating”
aorta5 and low attenuation,
without significant enhancement on
contrast-enhanced CT.6
Although radiological images can help us differentiate the
possible diagnoses of a retroperitoneal mass, surgical resection and
histological evaluation allow an absolute diagnosis of Castleman’s
disease. The use of haematoxylin-eosin (H&E) staining for Castleman’s
disease was important in this case and led to the correct diagnosis.
In conclusion, we have reported a large retroperitoneal mass
(>5 cm) with a heterogeneously enhanced pattern and areas of low attenuation.
Radiological imaging studies are not sufficiently specific to identify
Castleman’s disease and a histopathological examination is necessary for a
final diagnosis.
Author information: Chien-Ming Liao;
Ying-Chun Chiu; Wei-Chou Chang; Chang-Hsien Liu; Ching-Jiunn Wu; Guo-Shu Huang;
Radiologists; Department of Radiology, Tri-Service General Hospital, National
Defense Medical Center, Taipei, Taiwan, Republic of China
Correspondence: Ching-Jiunn Wu, MD,
Department of Radiology, Tri-service General Hospital, 325, Sec 2, Cheng-Kung
Rd, Neihu 114, Taipei, Taiwan, Republic of China. Fax:+886 (0)2 87927245; email:
shadow45@ms88.url.com.tw
References:
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