Clinical Imaging 36 (2012) 239–242

Malignant fibrous histiocytoma arising from a hydronephrotic kidney:a case report and review of the literature

Ki Hwan Kim a, Seung Hwa Lee a,⁎, Sang Hoon Cha a, Young Sik Kimb, Deuk Jae Sung c

aDepartment of Radiology, Ansan Hospital, Korea University, College of Medicine, Korea University,Gojan1-Dong, Danwon-Gu, Ansan-City, Gyeonggi-Do, 425-707, Korea

bDepartment of Pathology, Ansan Hospital, Korea University, College of Medicine, Korea University, Gojan1-Dong, Danwon-Gu,Ansan-City, Gyeonggi-Do, 425-707, Korea

cDepartment of Radiology, Korea University, Anam Hospital, College of Medicine, 126-1, 5-ga Anamdong, Sungbuk-gu, Seoul 136-705, Korea

Received 5 June 2011; accepted 14 September 2011

Abstract

Primary malignant fibrous histiocytoma (MFH) is extremely rare, and MFH arising from a hydronephrotic kidney has not been reported.When MFH originates from a long-standing hydronephrotic kidney, the imaging findings can include nearly invisible renal parenchyma andatrophy of the ureter and renal artery, in addition to the findings attributable to the MFH, and the MFH with hydronephrosis may be confusedwith a cystic renal cell carcinoma.© 2012 Published by Elsevier Inc.

Keywords: Malignant fibrous histiocytoma; Kidney; Hydronephrosis; Cystic renal cell carcinoma

1. Introduction

Malignant fibrous histiocytoma (MFH) is the mostcommon soft tissue sarcoma in adults occurring in theextremities and retroperitoneum [1,2]. However, primaryMHF of the kidney is extremely rare [2]. A primary MFHarising from a hydronephrotic kidney has not beenpreviously reported. When MFH originates from a hydro-nephrotic kidney, the MFH superimposed on the hydrone-phrosis is indistinguishable from cystic renal cellcarcinoma. Here, we report a case of MFH in an adultpatient with severe hydronephrosis and discuss the clinicaland radiological findings.

⁎ Corresponding author. Department of Radiology, College of Medi-cine, Korea University, Gojan1-Dong, Danwon-Gu, Ansan-Si, Gyeonggi-Do 425-707, Republic of Korea. Tel.: +82 31 412 5228.

E-mail address: yaaong@hitel.net (S.H. Lee).

0899-7071/$ – see front matter © 2012 Published by Elsevier Inc.doi:10.1016/j.clinimag.2011.09.001

2. Case report

A 55-year-old man was admitted to our hospital with acomplaint of general weakness for a week. Physicalexamination was normal except for an elevated bloodpressure of 159/106 mmHg. Laboratory test revealed severeproteinuria, but the serum creatinine level was normal.Abdominal ultrasound demonstrated significant hydrone-phrosis of the left kidney (Fig. 1). A DTPA renal scandemonstrated a photon-deficient mass in the left renal fossa.An abdominal computed tomography (CT) with intravenouscontrast injection revealed an 8×9×13-cm region of hydro-nephrosis with a thin, atrophic cortex and calcifications;however, the left renal artery and ureter were not detected(Fig. 1). The contralateral kidney had a normal appearance.These radiologic findings were suggestive of severehydronephrosis caused by an ureteropelvic obstruction,with atrophic changes in the renal parenchyma, renal artery,and ureter.

Two years later, the patient presented with more severeproteinuria, hematuria, and a 7-kg weight loss over the

Fig. 1. Axial (A) and coronal (B) contrast-enhanced CT scans and (C)abdominal ultrasound demonstrate severe hydronephrosis of the left kidney.Note the thin, atrophic cortex (arrow) along the wall and calcifications. Theipsilateral renal vein (arrow head) is apparent but not the renal artery.

Fig. 2. Axial (A) and coronal (B) contrast-enhanced CT scans demonstrate aheterogeneously enhanced mass with eccentrically located lumpy calcifica-tions (arrow) replacing the left cystic kidney.

240 K.H. Kim et al. / Clinical Imaging 36 (2012) 239–242

previous 6-month period. Contrast-enhanced CT scansrevealed a 10×11×13-cm lobulated, complex cystic massoccupying the left hydronephrotic kidney (Fig. 2). The mass

had obvious enhancing soft tissue components, increasedmural calcification, and hemorrhagic components. Multiplemetastatic lung nodules and enlarged mediastinal lymphnodes were also observed on chest CT. A left radicalnephrectomy was then performed to remove the tumor.

On gross examination, a 12×10×9-cm, conglomerated,brownish-white, solid tumor with extensive necrosis andhemorrhagic components was identified. The mass extendedinto the perirenal fat and was microscopically composed ofspindle cells and histiocytic cells with a storiform pattern ofgrowth and marked pleomorphism with numerous multi-nucleated giant cells (Fig. 3). Immunohistochemical analysisof the tumor cells was positive for CD10, vimentin, SMA,and CD68. The histopathological diagnosis was a storiform–pleomorphic variant of MFH. Normal transitional epitheliumoverlying the tumor was histologically identified, and thepelvicaliceal system was markedly dilated with invadingtumor cells. These findings confirmed that the MFH hadoriginated from the severely hydronephrotic kidney.

3. Discussion

Primary MHF of the kidney is extremely rare [1,2]. MFHarising from the kidney occurs most commonly in the fifth to

Fig. 3. Microscopic examination (A) demonstrate a mixture of spindle cellsand pleomorphic multinucleated giant cells, arranged in a storiform pattern.The tumor was associated with adjacent hemorrhage and necrosis. (B) Thenormal transitional cell epithelium (arrow) overlying the tumor was alsoidentified (hematoxylin and eosin staining, original magnification ×200).

241K.H. Kim et al. / Clinical Imaging 36 (2012) 239–242

seventh decades, with a slight left-sided predilection and nosex predominance [3]. Four subtypes of MFH exist, and thestoriform–pleomorphic subtype of MFH is the mostcommon histologic variant [4].

Previous literature has described the radiologicalfindings of MFH in detail [5–7]. On CT scans, MFHusually presents as a large, lobulated soft tissue mass witha central low attenuating region corresponding to necrosis,hemorrhage, or myxoid materials. Calcification may bedetected in approximately 20% of cases [6]. On magneticresonance (MR) imaging, MFH is seen as isointense onT1-weighted images and heterogeneously hyperintense onT2-weighted images [6,7]. The histologic characteristics,such as myxoid or fibrous components, are best reflectedon T2-weighted images. The angiographic findings ofMFH include marked hypervascularity without necrosis orhemorrhage [8]. Variable heterogeneous enhancement isalso seen on contrast-enhanced CT and MR images [5–7].However, there are no previous reports describing

imaging findings for MFH arising from a severelyhydronephrotic kidney.

In the current case, marked atrophic hydronephrosis withan atrophic ureter and atrophic renal artery was observed.The term “hydronephrosis” refers to different grades of renalpelvic dilatation. An atrophic ureter suggests that thehydronephrosis is caused by ureteropelvic junction (UPJ)obstruction, and UPJ obstruction is one of the predisposingfactors for hydronephrosis. Renal parenchymal thinning anda dilated renal pelvis may result from UPJ obstruction [9].This thinning occurs in adults less frequently than inneonates, but it is not a rarity [10].

Long-standing hydronephrosis also produces atrophicchanges in the renal artery, with atrophy beginning at theperipheral artery and progressing proximally to involvethe main renal artery [11,12]. The decrease in the caliber ofthe main renal artery is proportional to the duration of theobstruction [13]. Calcification is commonly found in benigncysts, congenital multiple cystic kidney. However, calcifi-cation in the wall of the hydronephrotic kidney, as in ourcase, is rare, and only a few cases have been reported in theliterature [14,15]. Progressive deposition of calcium in thechronic dilated renal pelvis may have caused calcification inthe hyalinized fibrous renal tissue.

Hydronephrosis with nearly invisible parenchyma and anatrophic ureter and renal artery may be difficult todistinguish from cystic renal disease, including the hydro-nephrotic variant of a multicystic dysplastic kidney (MCDK)[16,17]. In severe examples of both entities, identification ofa dilated renal pelvis and residual renal parenchyma ishelpful on radiographic examination, but histopathologicconfirmation, including observing cysts lined by immatureand primitive epithelium, is needed for the definite diagnosisof MCDK [18]. When congenital anomalies of the kidneyand urinary system are highly suspected, retrogradeureteropyelography may be useful to define the anatomicdefect [10].

When most of the renal parenchyma may be grosslyreplaced by hydronephrosis, detection of tumorous condi-tions becomes easier, but MFH arising from a hydrone-phrotic kidney may be indistinguishable from more commoncystic tumors, such as cystic renal cell carcinoma (RCC).Occasionally, MFH with extensive hemorrhage or necrosismay also stimulate a cystic tumor [19]. However, additionalimaging findings, including renal parenchymal thinning andan atrophic ureter and artery, are not common in cystic RCCand other cystic tumors. Therefore, when these findings aredemonstrated, one must consider the possibility of a solidtumor superimposed on long-standing hydronephrosis.Although renal tumors associated with significant hydrone-phrosis, containing more than 1 l of fluid, have beenreported, the association between significant hydronephrosisand renal tumors is still unclear [20–22].

In conclusion, we report a rare case of primary MFHarising from a hydronephrotic kidney. Certain imagingcharacteristics, including a lobulated soft tissue mass with

242 K.H. Kim et al. / Clinical Imaging 36 (2012) 239–242

isoattenuation similar to that of muscle, heterogeneousenhancement, and calcifications, may be indicative ofMFH. But MFH arising from long-standing hydronephrosismay be misdiagnosed as a cystic RCC. Although primaryMFH of the kidney is rare, we suggest that MFH should beconsidered in the differential diagnosis of kidney tumors,even when associated with hydronephrosis.

References

[1] Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analysisof 200 cases. Cancer 1978;41:2250–66.

[2] Shirkhoda A, Lewis E. Renal sarcoma and sarcomatoid renal cellcarcinoma: CT and angiographic features. Radiology 1987;162:353–7.

[3] Tarjan M, Cserni G, Szabo Z. Malignant fibrous histiocytoma of thekidney. Scand J Urol Nephrol 2001;35:518–20.

[4] Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma: betweenthe past and the present. Arch Pathol Lab Med 2008;132:1030–5.

[5] Singh SK, Mandal AK, Agarwal MM, Das A. Primary renalinflammatory malignant fibrous histiocytoma: a diagnostic challenge.Int J Urol 2006;13:1000–2.

[6] Kitajima K, Kaji Y, Morita M, Okuda Y, Sugimura K. Malignantfibrous histiocytoma arising from the renal capsule. Magn Reson MedSci 2003;2:199–202.

[7] Park SW, Kim HJ, Lee JH, Ko YH. Malignant fibrous histiocytoma ofthe head and neck: CT and MR imaging findings. AJNR Am JNeuroradiol 2009;30:71–6.

[8] Yaghmai I. Malignant giant cell tumor of the soft tissue: angiographicmanifestations. Radiology 1976;120:329–31.

[9] Rosen S, Peters CA, Chevalier RL, Huang WY. The kidney incongenital ureteropelvic junction obstruction: a spectrum from normalto nephrectomy. J Urol 2008;179:1257–63.

[10] Grasso M, Caruso RP, Phillips CK. UPJ Obstruction in the adultpopulation: are crossing vessels significant? Rev Urol 2001;3:42–51.

[11] Siegelman SS, Bosniak MA. Renal arteriography in hydronephrosis.Its value in diagnosis and management. Radiology 1965;85:609–16.

[12] Aron B, Tessler A, Morales P. Angiography in hydronephrosis.Urology 1973;2:231–6.

[13] Idbohrn H. Renal angiography in experimental hydronephrosis. ActaRadiol Suppl 1956:1–85.

[14] Tsujimura A, Imazu T, Nishimura K, Sugao H, Oka T, TakahaM, Takeda M, Kurata A. Ureteropelvic junction obstruction withrenal pelvic calcification: a case report. J Urol 1993;150:1889–90.

[15] Gold RP, Saitas V, Pellman C. Congenital ureteropelvic junctionobstruction with calcified renal pelvis and superimposed spindle cellurothelial carcinoma. Urol Radiol 1990;12:15–7.

[16] Felson B, Cussen LJ. The hydronephrotic type of unilateralcongenital multicystic disease of the kidney. Semin Roentgenol1975;10:113–23.

[17] Singh I, Sharma D, Singh N, Jain BK, Minocha VR. Hydrone-phrotic obstructed kidney mimicking a congenital multicystickidney: case report with review of literature. Int Urol Nephrol2002;34:179–82.

[18] Truong LD, Choi YJ, Shen SS, Ayala G, Amato R, Krishnan B. Renalcystic neoplasms and renal neoplasms associated with cystic renaldiseases: pathogenetic and molecular links. Adv Anat Pathol 2003;10:135–59.

[19] Huang CC, Ko SF, Ng SH, Lee TY, Wan YL, Lin JW, Chen W. Cysticmalignant fibrous histiocytoma of the gastrocolic ligament. Br J Radiol2001;74:651–3.

[20] Ochsner MG, Fuselier HA, Brannan W, Simo JB. Congenital gianthydronephrosis in adults. Urology 1977;10:422–4.

[21] Chiang PH, Chen MT, Chou YH, Chiang CP, Huang CH, Chien CH.Giant hydronephrosis: report of 4 cases with review of the literature. JFormos Med Assoc 1990;89:811–7.

[22] Funahashi Y, Kamihira O, Hagikura S, Kasugai S, Kimura K, FukatsuA, Matsuura O. Renal cell carcinoma presenting high serum level ofCA19-9 due to hydronephrosis: a case report. Nippon HinyokikaGakkai Zasshi 2008;99:39–42.