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Case Report pISSN 1738-2637 / eISSN 2288-2928 J Korean Soc Radiol 2015;73(1):62-65 http://dx.doi.org/10.3348/jksr.2015.73.1.62

Renal with Wide Extension into the Retroperitoneum: A Case Report 광범위한 후복막강 침범을 동반한 신 실질 연반증: 1예 보고

Soo Yeon Choi, MD1, Deuk Jae Sung, MD1, Na Yeon Han, MD1, Beom Jin Park, MD1, Min Ju Kim, MD1, Ki Choon Sim, MD1, Sung Bum Cho, MD1, Yoo Jin Lee, MD2 Departments of 1Radiology, 2Pathology, Anam Hospital, College of Medicine, Korea University, Seoul, Korea

Malakoplakia is a rare chronic inflammatory condition that results from defective phagolysosomal activity. Malakoplakia usually affects the urinary tract, and immuno- Received February 16, 2015 suppression is a predisposing factor in most patients. A 78-year-old woman under- Accepted April 21, 2015 Corresponding author: Deuk Jae Sung, MD going long-term steroid treatment presented with right flank pain. CT demonstrated Department of Radiology, Anam Hospital, a large, multilocular cystic mass with focal enhancing solid portion in the right kid- College of Medicine, Korea University, 73 Inchon-ro, Seongbuk-gu, Seoul 136-705, Korea. ney and retroperitoneum. The patient underwent ultrasonography-guided biopsy Tel. 82-2-920-5578 Fax. 82-2-929-3796 for enhancing the solid portion, and pathologic examination revealed malakoplakia. E-mail: [email protected]

This is an Open Access article distributed under the terms Index terms of the Creative Commons Attribution Non-Commercial Malakoplakia License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distri- bution, and reproduction in any medium, provided the Retroperitoneal Space original work is properly cited. Computed Tomography Anti-Bacterial Agents

INTRODUCTION should include renal abscess, xanthogran- ulomatous , renal malakoplakia and renal cell car- Malakoplakia is a rare chronic inflammatory condition that cinoma (6). Ninety-six cases of renal malakoplakia were found was first described by Michaelis and Gutmann in 1902. It is by searching the MEDLINE data base in a report published in characterized histologically by the presence of basophilic inclu- 2004 (4). In Korea, there have been 10 case reports of renal mal- sions within large eosinophilic macrophages (1). The urinary akoplakia, including 3 cases with extension to adjacent organ bladder is the most frequently involved site, but the kidney, ret- (5). Here, we reported a case of renal malakoplakia with wide roperitoneum, testis, and any other organs can also be retroperitoneal extension with emphasis on imaging findings. affected (1-3). It is usually associated with infec- tion and immunosuppression is a predisposing factor in most CASE REPORT patients (1, 3). Renal malakoplakia presents with a palpable mass or flank pain, which may be usually confused with renal malig- A 78-year-old woman was hospitalized in our institution be- nancy or other infectious disease (3-5). The imaging diagnosis cause of right flank pain aggravated by moving for 5 days before of renal malakoplakia is difficult, and it is only proved by patho- admission. She had a history of long-standing rheumatoid ar- logic findings of Michaelis-Gutmann bodies after biopsy or sur- thritis and had been taking prednisolone, 5 mg daily for 12 gery. In appropriate clinical settings of a renal mass, febrile uri- years. On physical examination, right side abdominal tender- nary tract and a history of immunosuppression, the ness was elicited. Laboratory tests revealed blood urea nitrogen

62 Copyrights © 2015 The Korean Society of Radiology Soo Yeon Choi, et al of 34.2 mg/dL, serum creatinine of 0.97 mg/dL, and septa, extending into the right retroperitoneum, bare area (WBC) count of 27400/μL, erythrocyte sedimentation rate of 31 and right psoas muscle. Focal enhancing solid portion was dem- mm/hr and C-reactive protein 157.16 mg/L, suggesting an onstrated at the cystic mass in the right kidney (Fig. 1A, B). acute infectious process. analysis revealed (30–60 There was no abnormality along the upper urinary collecting WBC/high power field) and urine culture was positive for Esch- system and the bladder. The complicated cystic lesion was ini- erichia coli. tially considered most likely as an abscess involving right kidney CT examination showed a large, multilocular cystic mass in and retroperitoneum. So, ultrasonography (US)-guided aspira- right kidney and perirenal space with irregular and thick wall tion was performed to confirm the presence of turbid gray-col-

A B C Fig. 1. Renal malakoplakia with wide retroperitoneal extension in a 78-year-old woman. Axial (A) and coronal (B) contrast enhanced CT scan shows a large multilocular cystic mass with irregular wall and septa (arrowheads) in right kidney and perirenal space. The mass extends into the right retroperitoneum, bare area and right psoas muscle. Focal enhancing solid portion is demonstrated in the right kidney (arrow). On follow-up CT scan (C) obtained after 4 months with treatment, the complicated cystic le- sion is markedly reduced. And the enhancing solid portion is also decreased in size (curved arrow).

A B Fig. 2. Photomicrographs of malakoplakia. A. There are many scattered histiocytes (von Hansemann cells) containing intracytoplasmic lamellated basophilic inclusions, called Michaelis- Gutmann bodies (black arrows) (× 1000). B. A von Kossa stain highlights numerous Michaelis-Gutmann bodies (× 200). jksronline.org J Korean Soc Radiol 2015;73(1):62-65 63 Renal Malakoplakia with Wide Extension into the Retroperitoneum ored pus, and percutaneous abscess drainage was attempted im- an abdominal mass, , renal failure, bladder irritability mediately. Escherichia coli were identified from the aspirated or persistent urinary tract infection despite appropriate antibiot- fluid, and the patient was given IV antibiotic therapy for 3 weeks. ic therapy (1-5). Imaging features of malakoplakia are nonspe- On follow-up CT, the complicated cystic lesion decreased in cific and variable. Malakoplakia can present as a diffuse infiltra- size, but the enhancing solid portion was present without inter- tive disease, sharply demarcated solitary mass, or ill-defined solid val change. Then, we performed US-guided biopsy for the en- masses. The lesion demonstrates variable echogenicity at US, hancing solid portion to exclude the possibility of malignancy. and mildly enhanced, heterogeneous attenuation at CT. In the Diagnosis of malakoplakia was based on the numerous michae- case of renal parenchymal malakoplakia, imaging studies com- lis-Gutmann bodies within the histiocytes on pathologic exami- monly demonstrate the enlarged, irregularly contoured kidneys nation (Fig. 2). Subsequently, the patient’s general condition had and dedifferentiation of corticomedullary junction. Decreased improved, and she was discharged after more than 3 weeks of excretion of contrast is more pronounced in cases with extensive continuous antibiotic therapy. On the latest CT scan, 4 months af- parenchymal involvement. Parenchymal calcification is rare (1, ter treatment, the enhancing solid portion was decreased in size 3, 7). Perinephric extension and renal vein thrombosis have (Fig. 1C). been reported (1). Magnetic resonance imaging features include poorly defined multiple low signal intensity nodules on all se- DISCUSSION quences with intervening fibrous stroma (3, 8). Focal renal le- sions are often misdiagnosed as a malignancy such as necrotic Malakoplakia is a rare chronic granulomatous benign disease. renal cell carcinoma. In imaging studies for renal malakoplakia, It may affect any organ of the body but primarily affects the differential diagnosis includes xanthogranulomatous pyelone- genitourinary tract (1). Other locations include the retroperito- phritis, local abscess, granuloma, lymphoma and primary or met- neum, gastrointestinal tract, central nervous system, female geni- astatic tumors (3, 6). tal tract, lung, pleura, pancreas, spleen, lymph node, adrenal Malakoplakia rarely extends to other organs. However, the gland, and vertebra (1-3). Most patients have positive urine cul- presence of malakoplakia in retroperitoneal space is almost al- tures, usually with Gram-negative and a predisposition ways due to direct extension from an adjacent organ, mainly uri- to immunosuppression such as solid organ transplantation, au- nary tract malakoplakia (2). Hence, we could characterize our toimmune disease requiring long-term steroid use, chemothera- patient as having renal malakoplakia with wide retroperitoneal py, malignancy, alcohol abuse and mellitus (1, 3). extension. Malakoplakia results from defective phagolysosomal activity There is no established treatment for malakoplakia. However, with incomplete bacteria digestion, mostly Escherichia coli and there are treatment options including , surgical exci- , by macrophages and monocytes. Bacterial debris ac- sion or a combination of both (2). Successful medical manage- cumulates in the cytoplasm of these cells and usually becomes ment has been reported with antimicrobial agents (4, 5). Malako- mineralized. This leads to basophilic inclusion structures with plakia is a highly treatable disease when detected in its early surrounding clear halos i.e., Michaelis-Gutmann bodies, which stage. Immunosuppressive drugs should be discontinued as far are pathognomonic for diagnosis (1, 3, 5). as possible (3). Because renal malakoplakia may mimic renal There is a peak incidence in the fifth to seventh decades at pre- cell carcinoma on imaging studies, a percutaneous or an open bi- sentation and a female predominance with a female to male ra- opsy helps to diagnose a treatable disease and prevent unneces- tion of 4:1 (1, 2). Even though the symptoms may vary accord- sary surgery. ing to the affected organ, malakoplakia is commonly associated In conclusion, renal malakoplakia should be considered in with urinary tract , including acute renal failure in re- the differential diagnosis in patients with urinary tract infection, nal malakoplakia (3). , , and renal mass like lesion, especially in In most patients with malakoplakia, lesions are initially iden- patients that have a risk factor of immunosuppression. As shown tified at imaging studies in various clinical settings, including in this case, renal malakoplakia can present as extensive retro-

64 J Korean Soc Radiol 2015;73(1):62-65 jksronline.org Soo Yeon Choi, et al peritoneal mass and often be confused with malignancy. 5. Yoon SY, Lee HJ, An JH, Kim SJ, Kim SW, Woo JH, et al. Re- nal parenchymal malakoplakia presenting with abscesses REFERENCES and hepatic extension misdiagnosed as a malignant tumor: a case report. Korean J Med 2012;82:764-768 1. Wielenberg AJ, Demos TC, Rangachari B, Turk T. Malacopla- 6. Cury J, Coelho RF, Franco M, Srougi M. Renal parenchymal kia presenting as a solitary renal mass. AJR Am J Roentgen- malacoplakia with pleural effusion. Clinics (Sao Paulo) 2007; ol 2004;183:1703-1705 62:87-88 2. Radin DR, Siskind B, Weiner S, Bernstein R, Ziprkowski M. 7. Dharmadhikari R, Crisp A. Sequential changes in sono- Retroperitoneal malacoplakia. Urol Radiol 1984;6:218-220 graphic appearances of childhood renal malakoplakia pro- 3. Zimina OG, Rezun S, Armao D, Braga L, Semelka RC. Renal gressing to end-stage renal failure. J Ultrasound Med 2006; malacoplakia: demonstration by MR imaging. Magn Reson 25:1219-1222 Imaging 2002;20:611-614 8. Kamishima T, Ito K, Awaya H, Mitchell DG. MR imaging of 4. Kajbafzadeh A, Baharnoori M. Renal malakoplakia simulat- bilateral renal malacoplakia after liver transplantation. AJR ing neoplasm in a child: successful medical management. Am J Roentgenol 2000;175:919-920 Urol J 2004;1:218-220

광범위한 후복막강 침범을 동반한 신 실질 연반증: 1예 보고

최수연1 · 성득제1 · 한나연1 · 박범진1 · 김민주1 · 심기춘1 · 조성범1 · 이유진2

연반증은 대식세포의 탐식작용 장애로 인해 발생하는 것으로 생각되는 드문 만성 염증성 질환이다. 연반증은 주로 요로계 를 침범하며, 대부분의 환자들은 면역 저하 요인을 가지고 있다. 장기간 스테로이드를 복용한 78세 여자 환자가 우측 옆구 리 통증을 호소하였다. CT에서 우측 신장과 후복막강에 걸쳐 큰 다방성의 낭성 종괴가 관찰되었으며, 일부 조영증강 되는 고형 성분이 보였다. 환자는 초음파 유도하에 고형 성분에 대해 조직검사를 시행 받았으며, 병리적으로 연반증으로 확인되 었다.

고려대학교 의과대학 안암병원 1영상의학과, 2병리과

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