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CASE REPORT

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ROSAI-DORFMAN DISEASE PRESENTING AS EXTRANODALRENAL MASS

ANAND KRISHNAN, AZIZA NASSAR, AND PETER T. NIEH

ABSTRACTe present a unique case of a man presenting with an abnormal prostate and large renal mass who was

ltimately found to have prostate cancer and renal Rosai-Dorfman disease. We discuss the method ofiagnosis and treatment in this patient, as well as review the presentation, diagnosis, and treatment of thisare disease. UROLOGY 66: 1319.e17–1319.e19, 2005. © 2005 Elsevier Inc.

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osai-Dorfman disease is a rare disease of un-known etiology with limited reports in uro-

ogic journals. We report a case of a man with anbnormal digital rectal examination and a renalass who proved to have prostate cancer and renalosai-Dorfman disease. We review the clinical pre-entation and essential histopathologic featuresecessary for the accurate diagnosis of this disease.

CASE REPORT

A 76-year-old white man was referred by his pri-ary care physician for an abnormal prostate ex-

mination with a serum prostate-specific antigenevel of 2.7 ng/mL. His past medical history re-ealed no pertinent medical conditions or familyistory of prostate cancer. The physical examina-ion was only significant for a 45-g diffusely nodu-ar prostate. He underwent transrectal ultrasound-uided sextant prostate needle biopsy, with thending of Gleason score 5 � 4 � 9 adenocarci-oma of the prostate in six of six cores. In antici-ation of external beam radiotherapy, he receivedndrogen deprivation therapy. He also underwentstaging bone scan, which was negative for meta-

tatic disease, and an abdominal and pelvic CTcan, which revealed an incidental 9-cm centrallyocated left renal mass (Fig. 1) , demonstrating het-rogeneous contrast enhancement. Positron emis-ion tomography with 18F-fluoroaminocyclopentane

rom the Departments of Urology and Pathology, Emory Univer-ity School of Medicine, Atlanta, Georgia

Address for correspondence: Peter T. Nieh, M.D., Department ofrology, Emory University School of Medicine, 1365 Clifton RoadE, Building B, Atlanta, GA 30322. E-mail: peter.nieh@

moryhealthcare.orgSubmitted: March 29, 2005, accepted (with revisions): June 22,

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2005 ELSEVIER INC.LL RIGHTS RESERVED

arboxylic acid, an investigational radiotracer withinimal renal and bladder excretion, showed mildptake in the renal mass, intense uptake in the para-ortic, paracaval, and retrocaval lymph nodes, andess intense uptake in a few pelvic nodes. With a pre-umptive diagnosis of synchronous adenocarcinomaf the prostate and either renal cell or transitional cellarcinoma of the left kidney with hilar nodal metas-ases, he underwent laparoscopic hand-assisted leftadical nephrectomy with regional lymph node dis-ection. The operation was performed without com-lications. The final pathologic examination of theenal mass showed Rosai-Dorfman disease (Fig. 2),nd the regional lymph node dissection revealedleason score 5 � 4 � 9 adenocarcinoma of therostate. The patient underwent complete andro-en deprivation for the prostate cancer. After with-rawal of the antiandrogen, he was asymptomaticith a prostate-specific antigen level of 2.6 ng/mLyear later.

COMMENT

The presence of prostate cancer in the renal hilarodes was most surprising, but it highlights theggressiveness of Gleason score 9 disease. Theoint of this discussion is the unusual finding inhe enlarged left kidney. Rosai-Dorfman disease orinus histiocytosis with massive lymphadenopathyas first described in 1969 by Rosai and Dorfman.1

t is classified as a non-neoplastic histiocytic disor-er and is characterized by massive lymphadenop-thy secondary to infiltration and dilation of theymph node sinuses by large histiocytes. It usuallyresents in younger individuals, in the first or sec-nd decade, with a slightly increased prevalence infrican Americans and males. It classically presents

ith chronic, bilateral, nontender cervical lymphad-

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nopathy of massive proportions. Extranodal diseaseas been reported in 33% to 40% of cases 2– 4 and is

ess common than the classic nodal variant. Approx-mately 4% of all cases of Rosai-Dorfman disease aressociated with kidney involvement, which, in someases, represents the sole manifestation of the dis-ase.5 Only one other case has been reported of renalosai-Dorfman disease in a patient with a prior his-

ory of the classic nodal variant later presentingith bilateral renal masses.2Histologically, the sine qua non of Rosai-Dorf-an disease is emperipolesis, in which histiocytesith eosinophilic cytoplasm can be seen engulfing

ymphocytes without digesting them. The less com-on extranodal variants of the disease tend to occurore often in older patients and histologically may

how less emperipolesis and more fibrosis. This may

IGURE 1. Computed tomography scan (A) without conB) with contrast.

IGURE 2. (A) High-power magnification demonstratinells, plasma cells, and lymphocytes—emperipolesis (lood cells also noted (asterisk). Hematoxylin-eosin staion with cytoplasmic immunostaining of histiocytic mCD68/KPI stain, original magnification �60).

ake the diagnosis more difficult, unless a high de- s

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ree of clinical suspicion is present. Immunohisto-hemistry will typically show positive staining ofistiocytes for S-100 in almost all cases, as well asositive staining for other antigens specific to theacrophage lineage or activated macrophages,

uch as CD68, CD14, Ki-M1P, MRP-8, and MRP-4. Histiocytes are characteristically negative forD1a staining.2,6

Clinically, most patients will have an indolentourse, with spontaneous resolution of symptoms,ut persistent stable disease. Fatalities, althoughncommon, have been reported in up to 7% ofases, although usually these have been due to under-ying comorbid conditions.7 Chemotherapy and ra-iotherapy tend to have a variable response, al-hough their use, as well as corticosteroid use, haseen advocated in case reports, with a good re-

showing large 9-cm solid left renal mass that enhanced

istiocytic proliferation with intracytoplasmic red bloodwhead). Hemosiderin deposition due to digested redriginal magnification �60). (B) High-power magnifica-r (CD68) highlighting proliferating cells (arrowheads)

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ponse. Surgery is reserved only for those with

UROLOGY 66 (6), 2005

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ompromise of adjacent vital organs by the disease.ositron emission tomography has been reportedo show positive uptake in proven Rosai-Dorfmanisease and may be a useful adjunct to the diagno-is, as was the case in our patient.8The etiology is unknown, although an infectious

r chronic inflammatory cause has been implicated.t is also known that clinically significant immuno-ogically mediated disease can occur in associationith sinus histiocytosis with massive lymphadenop-

thy and may adversely affect the prognosis. 5 To ournowledge, this represents the first reported case ofxtranodal variant Rosai-Dorfman disease presentings a renal mass in an elderly patient without a prioristory of the nodal variant disease.

REFERENCES1. Rosai J, and Dorfman RF: Sinus histiocytosis with mas-

ive lymphadenopathy: a newly recognized benign clinico-

athologic entity. Arch Pathol 87: 63–70, 1969. 2

ROLOGY 66 (6), 2005

2. Kugler A, Middel P, Gross AJ, et al: Unusual bilateralenal histiocytosis: extranodal variant of Rosai-Dorfman dis-ase. J Urol 157: 942–944, 1997.

3. Gilbert-Barness E: Pathological case of the month. Archediatr Adolesc Med 149: 57–58, 1995.4. Rodriguez-Galindo C, Helton KJ, Sanchez ND, et al: Ex-

ranodal Rosai-Dorfman disease in children. J Pediatr Hematolncol 26: 19–24, 2004.5. Foucar E, Rosai J, and Dorfman R: Sinus histiocytosis

ith massive lymphadenopathy (Rosai-Dorfman disease): re-iew of the entity. Semin Diagn Pathol 7: 19–73, 1990.

6. Lu D, Estalilla OC, Manning JT Jr, et al: Sinus histiocyto-is with massive lymphadenopathy and malignant lymphoma in-olving the same lymph node: a report of four cases and reviewf the literature. Mod Pathol 13: 414–419, 2000.7. Foucar E, Rosai J, and Dorfman RF: Sinus histiocytosisith massive lymphadenopathy: an analysis of 14 deathsccurring in a patient registry. Cancer 54: 1834 –1840,984.8. Yu JQ, Zhuang H, Xiu Y, et al: Demonstration of in-

reased FDG activity in Rosai-Dorfman disease on positronmission tomography. Clin Nuclear Med 29: 209 –210,

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