Sinus Histiocytosis with Massive Lymphadenopathy( Rosai-Dorfman Disease): A Rare Cause of Bilateral RenalMassesEric S. Bain �, Thomas B. Kinney, Justin M. Gooding, Giovanna Casola, Mariana Z. Ysrael
A benign entity named sinus histio-
cytosis with massive lymphaden-
opathy (Rosai-Dorfman disease)
was first described in 1969 [ I ]. Classically, it
is characterized by lymphadenopathy mainly
in the cervical area associated with fever, leu-
kocytosis, polyclonal gammopathy, and an
elevated erythrocyte sedimentation rate. His-
topathologically, an inflammatory prolifer-
ation of S 100 protein-positive histiocytes
characterized by lymphophagocytosis is seen.
Most cases involve patients in their first and
second decades, but all age groups can be af-
fected [1-3].
Extranodal sites are involved in 43% of
cases, and lymphadenopathy may be absent
in such patients [3]. Two reports have de-
scribed bilateral renal involvement [4, 5],
and renal infiltration was noted in 10 of 423
cases collected in a worldwide registry [3].
We present another case and discuss the im-
aging findings of this rare entity. To our
knowledge, the CT and sonographic charac-
teristics of renal Rosai-Dorfman disease
have not been described.
Case Report
A 75-year-old woman with a history of hy-
pertension and anemia was admitted for fever,
leukocytosis, and epigastric pain. Bilateral en-
larged cervical lymph nodes were detected on
physical examination, and urinalysis showed
microscopic hematuria. Abdominal sonogra-
phy with a curved multifrequency 2- to 4-
MHz transducer (HDI 3000; Advanced
Technology Laboratories, Bothell, WA) was
performed, and the patient was found to have
a large, nonmobile gallstone at the gallbladder
neck, gallbladder wall thickening, and peri-
cholecystic fluid, confirming the clinical sus-
picion of acute cholecystitis.
In addition, bilateral renal masses were
discovered (Fig. IA). Both masses measured
6-7 cm in diameter, were hypoechoic by
sonography, and showed areas of internal
calcifications. Flow within these masses was
documented on power Doppler imaging, but
no pulsed Doppler waveforms were detected.
The right renal artery and vein were identi-
fled and appeared normal, but the left renal
vessels could not be imaged adequately.
The patient was treated medically with IV
antibiotics and bowel rest and underwent heli-
cal CF (HiSpeed Advantage; General Electric
Medical Systems, Milwaukee, WI) 3 days after
admission. Helical technique was used to per-
mit multiplanar reconstructions if necessary.
Unenhanced and contrast-enhanced CT per-formed in the cortical and excretory phases was
performed (Figs. 1B-1D). A total of a 125 ml
of nonionic IV contrast material (Optiray 320;
Malhinckrodt Medical, St. Louis, MO) was
given at a rate of 4 mI/sec. Bilateral renal
masses were again identified, measuring 6 x 6
x 11cm on the right and 8 x5x 13 cm on the
left. Some calcifications were identified in the
right kidney, both within the mass and in the
collecting system. Both renal masses showed
homogeneous enhancement after IV contrast
administration. The medial aspect of the left-
sided mass was seen to constrict the left renal
vein; otherwise the renal vessels were unre-
markable. Lymphadenopathy was not detected.
A diagnosis of bilateral renal cell carcinoma,
lymphoma, or metastasis was entertained.
Six days after admission the patient un-
derwent sonographically guided percutane-ous biopsy of the renal masses. The bilateral
masses were biopsied multiple times with
20- to 25-gauge needles of various types.
Both fine-needle aspirates and core biopsies
were obtained. Cytology revealed histio-
cytes, plasma cells, neutrophils, and lympho-
cytes. No malignant cells were identified.
On the next hospital day, the patient un-
derwent open cholecystectomy and surgical
biopsy of the right renal mass. A lobulated
renal mass was found that was fleshy in char-
acter. Frozen sections performed at the time
of the procedure were indeterminate. The fi-
nal pathologic specimen showed histiocytes
that stained positive for 5100 protein, and a
histologic diagnosis of Rosai-Dorfman dis-
ease was made.
Discussion
Rosai-Dorfman disease is a rare condition
that can involve the kidneys without evi-
dence of regional lymphadenopathy [4, 5].
Received August 10, 1998; accepted after revision October 19, 1998.
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Bain et al.
996 AJR:172, April 1999
Fig. 1-75-year-old woman with bi-lateral renal masses.A, Sonogram shows hypoechoic massof lower pole of right kidney. Noteshadowing calcification (arrow). Acrescent of normal renal parenchymais seen along margin oftumor oppositecalcification.B, Helical CT scan shows bilateral re-nal masses. Note soft-tissue attenu-ation. Also note small right renalcalcification (arrow). Inflammatorychanges (arrowhead) are seen ingallbladder.C, Helical CT scan obtained in corti-cal phase after IV injection of con-trast material shows enhancement ofrenal masses. Note that left renalvein is compressed by mass.D, Helical CT scan obtained in excre-tory phase after IV injection of con-trast material shows distortion ofintrarenal collecting systems.
Histologically it is characterized by massive
proliferation of inflammatory cells, including
histiocytes that can contain lymphocytes and
hematopoietic cells within their cytoplasm.
These histiocytes are strongly positive for
5100 protein on histochemical staining [2].
The disorder remains idiopathic and theclinical course varies widely from spontane-
ous remission to death from vital organ infil-
tration. Patients with renal involvement have
been associated with poorer outcomes, with
40% of patients dying of the disease and theremainder having persistent involvement [3,
6]. In a worldwide registry, eight of 10 patients
with renal disease also had nodal involvement
[3]. Although involvement of nearly every or-
gan has been described, the most frequent ex-
tranodal sites to be affected in patients with
renal disease are the eyelids, the orbits, and the
CNS [3]. A definitive treatment regimen has
not been established, but a combination of
chemotherapeutic agents and corticosteroids
appears to be the most effective [7].
Previous reports described detection of re-
nal involvement with sonography and CT but
did not delineate the imaging findings [4, 5].
In the patient described in this report, sonog-
raphy revealed large heterogeneous hypo-
echoic masses with foci of calcification. On
unenhanced CT, noncystic masses with soft-
tissue attenuation and focal calcifications
were identified. Administration of IV contrast
material revealed homogeneous enhancement
to a lesser degree than did the renal paren-
chyma. The collecting systems were distorted
by these masses but were not obstructed. No
vascular invasion or regional lymphadenopa-
thy was detected. Angiography was not per-
formed in this case but was reported to show
avascular renal tumors in another case [4].
On the basis of the sonographic and CT ap-
pearances, we could not differentiate this entity
from other renal tumors, and biopsy was recom-
mended to exclude lymphoma or metastasis.
Percutaneous sonographically guided biopsy
was performed, but it was thought to be nondi-
agnostic and the patient underwent open biopsy.
However, another report involving a patient with
known Rosai-Dorfnian disease described suc-
cessful percutaneous biopsy, which included the
use ofSlOO protein staining [5]. Our initial biop-
sies were likely nondiagnostic because of the
difficulty in diagnosing this rare disease without
the use of special stains. Greater awareness of
Rosai-Dorfman disease and the use of appropn-
ate histochemical stains should allow successful
percutaneous biopsy and nonsurgical treatment.Rosai-Dorfman disease of the kidneys, al-
though rare, should be considered in the dif-
ferential diagnosis of bilateral renal masses.
The appearance can resemble other entities,
particularly lymphoma. Awareness of this
disease will be especially useful in cases in
which percutaneous biopsy does not yield an
alternative diagnosis. The use of special
stains can then be suggested.
References
1. Rosai J. Dorfman RF. Sinus histiocytosis with
massive lymphadenopathy: a newly recognized
benign clinicopathologic entity. Arch Path 1969;
87:63-70
2. Rosai J, Dorfman RE Sinus histiocytosis with
massive lymphadenopathy: a pseudolymphoma-
tous benign disorder-analysis of 34 cases. (‘an-
cer 1972:30:1174-1188
3. Foucar E. Rosai J. Dorfman R. Sinus histiocytosis
with massive lymphadenopathy (Rosai-Dorfman
disease): review of the entity. Se,nin Diagn Pathol
l990;7: 19-734. Afzal M, Baez-Giangreco A. al Jaser AN, Onuora
