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Castleman’s disease is a rare lymph node disease. Current
classifi cation differentiates unicentric disease with a sin-
gle lesion from multicentric disease with lymph node
involvement in multiple lymphatic stations. Both have a
signifi cantly different outcome. 1 The condition is caused
by human herpesvirus infection and is best understood as
a model of virus driven disease propagation in an expand-
ing B cell pool and diseased endovascular endothelium. 2
We demonstrate the rare case of unicentric disease with
locoregional lymph node involvement and surgical cure
achieved by oncological resection alone. Radical surgery
rather than excisional biopsy may be indicated in patients
with unicentric disease.
This generally well 78-year-old woman was initially
referred for primary hyperparathyroidism and a 4.5 cm
left adrenal lesion infi ltrating the left kidney reported by
an external MRI. Medical history includes arterial hyper-
tension. She had fatigue and joint aches. Family and
social history were unremarkable and clinical examina-
tion found no lymphadenopathy or any other suspicious
Full blood count and blood biochemistry were normal but
for eucalcaemic hyperparathyroidism with parathyroid
hormone elevated to 113 ng/l (n:15–70) and an estimated
glomerular fi ltration rate of 60–70 ml/min. Endocrine
testing revealed normal urine catecholamines, renin-
aldosterone ratio and morning cortisol. The iodine-131-
meta-iodobenzylguanidine adrenal scan did not reveal any
A preoperative CT scan did not reveal any focal lesions
beyond the known left retroperitoneal lesion and multiple
small liver cysts. Chest x-ray was clear.
The preoperative dedicated adrenal CT showed a 4.5 cm
low attenuation mass lesion at the inferior aspect of the
left adrenal with fairly ill-defi ned borders and extending
into the left renal hilum. There is infl ammatory stranding
in the retroperitoneal fat. The renal artery and vein course
through the mass lesion which extends into the hilum
abutting the left renal pelvis is shown in fi gure 1 .
Pathological examination of the surgical specimen
showed free resection margins. Fourteen retroperitoneal
lymph nodes were positive for Castleman’s disease, while
four interaortocaval nodes were free of disease. Left kid-
ney and adrenal tumour: the specimen weighs 363 g and
consists of left kidney and adrenal along with perinephric
fat and hilum. The surface of the specimen was painted
black. The specimen measures 190 mm superior to infe-
rior, 110 mm medial to lateral and 55 mm anterior to pos-
terior. The cut surface of the specimen shows a periadrenal
mass which appears ill-defi ned and brown in colour. There
is some identifi able residual normal looking adrenal tissue
within the lesion. The tumour extends very close to the
upper pole of the kidney and into the hilar fat. Due to the
poorly circumscribed nature of the tumour, it is diffi cult
to measure the size accurately; however, it is approxi-
mately 45×35×25 mm. In addition, there is a small simple
cyst noted at the lower pole measuring 14×15×5 mm.
The remaining renal parenchyma appears unremarkable.
Microscopy: sections of the adrenal appear normal. The
capsule is intact and there is normal cortical to medullary
ratio. The periadrenal tissue shows an extensive infi ltrate
of small lymphocytes intimately associated with dilated,
and occasionally thick-walled hyaline blood vessels. There
Other full case
Retroperitoneal unicentric Castleman’s disease with multiple
lymph node involvement
Klaus-Martin Schulte, 1 Prakash Sinha, 2 Nadia Talat, 1 Salvador Diaz-Cano 3
1 Department of Endocrine Surgery, King’s College Hospital, King’s Health Partners, London, UK ;
2 Department of Surgery, Princess Royal University Hospital, London, UK ;
3 Department of Pathology, King’s College Hospital, King’s Health Partners, London, UK
Correspondence to Dr Klaus-Martin Schulte, firstname.lastname@example.org
A 78-year-old woman with B-symptoms was referred for a left adrenal incidentaloma of 5 cm. Imaging revealed features compatible with
adrenal cancer. The authors excluded excess production of catecholamines or adrenal steroids. The tumour was removed by en bloc radical
left retroperitonectomy with adrenalectomy, nephrectomy, interaortocaval lymphadenectomy and splenectomy. Histology demonstrated
periadrenal hyaline vascular Castleman’s disease with local infi ltration and 14 positive lymph nodes. The lymphoid infi ltrate spilled into the
adjacent renal cortex. HHV8 was negative. The Ki67 proliferative index was 30–40% in germinal centres. There was no syn- or metachronous
disease on extended imaging including fl uorodeoxyglucose positron emission tomography-CT and narrow follow-up at 3 years. This is a rare
case of unicentric hyaline vascular Castleman’s disease with documented locoregional lymph node involvement. The case exemplifi es the
transition from unifocal unicentric disease into disseminated disease with involvement of multiple lymph node stations (multicentric disease).
The authors demonstrate surgical cure by oncological resection.
BMJ Case Reports 2011; doi:10.1136/bcr.03.2011.3938
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is also marked surrounding stromal oedema and fi brosis.
The lymphoid infi ltrate spills into the adjacent renal cortex
and there is associated focal global and segmental sclero-
sed glomeruli together with interstitial involvement in this
area. The renal medulla is unremarkable. There are also
three incidental simple renal cysts. Fourteen lymph nodes
show similar features. There is diffuse nodular expansion
with regressive germinal centres composed of large cells
with vesicular nuclei. Some of the follicles show vascu-
lar proliferation and hyalinisation of the germinal centre.
There is ‘onion-skinning’ appearance to the lymphocytes at
the periphery of the follicles. Immunohistochemical stains
show that the nodules are strongly CD20, BCL2 and CD10
positive, while CD3 and CD5 highlight the perifollicular T
cells. CD21 and CD23 are strongly positive in the follicular
dendritic cells in the germinal centres. HHV8 is negative.
Ki67 shows the germinal centres have a proliferative index
of 30–40%, the rest of the lymph node is very low. 2 The
diagnosis is localised retroperitoneal Castleman’s disease
(stromal rich, hyaline vascular type) ( fi gures 2 and 3 ).
The differential diagnosis of the CT-imaging fi ndings
An adrenal adenoma (unlikely due to malignant fea-
tures and washout criteria)
A malignant retroperitoneal process was considered
and felt to most likely be a primary adrenal lesion
Retroperitoneal sarcoma or retroperitoneal lymphoma
We performed open radical surgery as en bloc retroperi-
tonectomy with adrenalectomy and nephrectomy, inter-
aortocaval lymphadenectomy and splenectomy. A liver
biopsy, done to stage an eventual lymphoma, showed only
OUTCOME AND FOLLOW-UP
Follow-up after 3 years (36 months) did not reveal local
recurrence. The patient is free of symptoms. A postop-
erative whole-body fl uorine-18 fl uorodeoxyglucose posi-
tron emission tomography-CT did not reveal any specifi c
uptake beyond a left colon lesion. This was endoscopically
removed and demonstrated to be a high-risk colon polyp.
This case is a presentation of a rare disease. Its importance
relates to the fact that we demonstrate lymphadenopathy
in 14 surrounding lymph nodes localised in the retroperi-
toneal fat and renal hilum. The tumour is clearly shown
Figure 1 CT showing a 4.5 cm low attenuation mass lesion at the inferior aspect of the left adrenal.
BMJ Case Reports 2011; doi:10.1136/bcr.03.2011.3938
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to originate from the retroperitoneal fat, not the adrenal
gland itself, as likely erroneously claimed by some former
authors reporting on retroperitoneal Castleman’s. 3 – 6
There are only a few former reports describing locore-
gional lymph node seeding in CD. 7 – 10 The rarity of this
observation relates to the fact that less than 10% of all
Figure 2 The periadrenal soft tissue shows a multifocal nodular lymphocytic infi ltrate with perivascular and interstitial patterns embedded
in a hyalinised stroma-rich background (H&E 12.5×). The surrounding lymph nodes reveal classical picture of hyalin-vascular Castleman
disease (inset, H&E 100×).
Figure 3 The interstitial lymphocytic infi ltrate reveals perivascular aggregates, dilated hyalinised vessel walls (panels A and B, H&E 40×)
and extends to the sinusoidal spaces of the adrenal cortex (panel C, H&E 100×). A polymorphous infi ltrate contains mature lymphocytes
and scattered plasma cells without atypia, along with prominent endothelial network (panel D, H&E 200×).
BMJ Case Reports 2011; doi:10.1136/bcr.03.2011.3938 Download full-text
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patients operated for Castleman’s disease had systematic
We provide a strong argument for surgery aligned with
oncological principles. In our patient this is highly likely
to have enabled cure. Excision of the mass alone would
have left the patient at risk for early recurrence due to
expansion of the persistent lymph node disease. At the
same time removal of the adjacent regional lymph node
station (the interaortocaval nodes) has greatly contributed
to inform the prognosis. In the absence of disease, 11 we
have withheld highly toxic systemic chemotherapy and
local radiation. Long-term results for surgery in unicentric
Castleman’s disease show excellent but imperfect results. 1
We propose that local recurrence and eventually death are
consequences of incomplete removal of the locoregional
disease caused by confusion between unifocal and unicen-
▶ Retroperitoneal Castleman’s disease originates from
retroperitoneal lymph nodes rather than solid organs.
Surgical cure can be achieved by oncological
resection aiming at free margins and complete
Unicentric disease may present with locoregional
lymphatic spread. The prognosis is still sharply
different from that of multicentric disease.
Locoregional lymphatic spread may constitute a
transition stage between unifocal unicentric disease
and multicentric disease.
The present case provides clinical evidence for the
theoretically based proposal of lymphatic propagation
of Castleman’s disease.
Competing interests None.
Patient consent Obtained.
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