A 47-year-old man with lumbago had been found to have a perirenal hematoma at the lower pole of the left kidney on CT. MR imaging also demonstrated a hematoma，8 X8X9αn in size. After 1-month observation，the mass had very rapidly increased in size，suggesting it to be a neoplasm. Consequent surgery resulted in noncurative tumor extirpation，because of invasion to the descending colon，left kidney and surrounding tissues. Histlogically，the tumor consisted of uniform spindle-shaped cells with interlacing solid and bundle forms，showing a herring-bone pattern.
Immunohistochemical studies finally led to the diagnosis of fibrosarcoma with markedly
high malignant potential. Additional therapy was not performed. Rapid local spread of
the tumor was demonstrated when bowel obstruction developed 2 months later. The
patient died of the disease 3 months after surgery. No distant metastasis was observed
throughout the clinical course

Kagebayashi, Yoriaki

Konishi, Noboru

Nakai, Yasushi

Samma, Shoji

Shimada, Keiji

Torimoto, Kazumasa

English

Global Institutional repository of Nara Medical University

Case Reports RETROPERITONEAL FIBROSARCOMA WITH DISTINCTIVE CHARACTERISTICS ON  GROWTH P ATTERN AND FINDINGS IN DIAGNOSTIC IMAGING YASUSHI NAKAI， YORlAKI KAGEBA YASHI， KAZUMASA TORIMOTO and SHO]I SAMMA Detartment of Urology， Nara Prefectural Nara Hostital KEI]I SHIMADA and NOBORU KONISHI Dψartment of Pathology， Nara Med加1University Received July 30， 2010 (163) Abstract: A 47-year-old man with lumbago had been found to have a perirenal hematoma at the lower pole of the left kidney on CT. MR imaging also demonstrated a hematoma， 8X8X9αn in size. After 1-month observation， the mass had very rapidly increased in size， suggesting it to be a neoplasm. Consequent surgery resulted in non-curative tumor extirpation， because of invasion to the descending colon， left kidney and surrounding tissues. Hiおst旬ologi犯cally仏rん， the tumor consisted of uniform s叩pi出ndlewith 白nt伐erlacing solid and bundle forms， showing a herring-bone pattern. Immunohistochemical studies finally led to the diagnosis of fibrosarcoma with markedly high malignant potential. Additional therapy was not performed. Rapid local spread of the tumor was demonstrated when bowel obstruction developed 2 months later. The patient died of the disease 3 months after surgery. No distant metastasis was observed throughout the clinical course. Key words retroperitoneal fibrosarcoma， herring-bone pattern， immunohistochemical studies INTRODUCTION Fibrosarcoma in the retroperitoneal space is a very rare malignant tumor. The treatment of this tumor requires prompt surgical extirpation. We encountered a case of retroperitoneal fibrosarcoma， which showed a distinct growth pattern and very rapid local spread without distant metastasis. Similar findings to hematoma in diagnostic imaging modalities led to a complicated preoperative diagnosis. CASE REPORT A 47【year-oldman presented with a 2-week history of gradually increasing lumbago in the middle of March 2007. He had been found to have a perirenal hematoma at the lower pole of the left kidney on CT. At admission， he had tenderness in the left lower abdomen without a palpable tumor. Laboratory data showed anemia， hemoglobin of 10.4 g/dL， and C reactive (164) Y. N akai et al Fig. 1. MR imaging at the first admission. A : Plain T1-weighted image， showing a perirenal mass with very high intensity sports within the mass is d巴monstrated.B : Plain T2べλ7巴ight巴dimage， showing scattered low int巴nsityspots. There was no contrast巴nhancementinside the mass. These findings suggested that the mass was a hematoma with various points of bleeding in intermingled chronic and acute phases Fig町 2.MR imaging at the second admission The tumor had spread over the upper pole of the kidney， and recurrent intratumoral hemorrhag巴was suspected. protein of 6.3 mg/dL. Hematuria was not observed. Plain MR imaging also demonstrated a perirenal mass with very high intensity spots inside in Tl-weighted images， and scattered low intensity spots in T2-weighted imag巴s.No contrast enhancement was observed inside the mass. These findings suggested that the mass was a hematoma with various kinds of spots of bleeding in intermingled chronic and acute phases (Fig. 1). A neoplasm had to be ruled out. We explained the necessity of aingiography and subsequent surgical treatment. However， the patient left the hospital because of disappearance of the symptom and the patient's personal reasons. During the hospitalization， anemia did not progress， and growth A case of retroperitoneal fibrosarcoma (165) Fig.3. Microphotograph of th巴 resectedspecimen.(H】Estain) A herring-bone pattem : the tumor consisted of uniform spindleωshaped cels with interlacing solid and bundle forms. of the tumor was not observed on CT. At re-admission one month later， he had a painful， palpable mass in the left lower abdomen. In MR imaging， the tumor had unexpectedly spread over the upper pole of the kidney， and a recurred intra同tumorhemorrhage was suspected (Fig 2). Distant metastasis was not detected. Subsequently performed left renal arteriography did not demonstrate active bleeding， vascular malformation， or tumor feeding vessels. Considering a possible neoplasm， tumor extirpation was att巴mptedwith a transperitoneal approach. The red-brown tumor accompanied with partial necrosis and hemorrhage was non-capsulated and fragile， involving the descending colon， left kidney and surrounding tissues. Although extended resection including these organs was attempted， curative surgery was not achieved. The external and communicating iliac lymph nodes were enlarged. Histologically， the tumor consisted of uniform spindle-shaped cells with interlacing solid and bundle forms， showing a herring-bone pattern (Fig. 3). The results of immunohistochemi-cal studies finally led to the diagnosis as fibrosarcoma: positive only for vimentin and negative for CK7， wide keratin， S-100， CD34， CD68， CD99， EMA， cytokeratin and calretinin. The additional findings indicated that th巴 tumorhad extremely high malignant potential: an increase in cell density and mitotic figures， scarcity of collagen， accompanying necrosis， and 70-80% of MIB-l index. Metastasis was not observed in the lymph node specimens. Postoperatively， we informed the patient and his family that: 1) curative surgery was not achieved， 2)the tumor seemed to have extr百 nelyhigh malignant potential， 3)the previously reported response rates of chemotherapy for the disease were not high， and 4) no effective therapy for the residual lesion had been established. As a result， no additional treatment was performed according to their decision. Two months postoperatively， the patient returned to the hospital due to bowel obstructive symptoms of emesis and abdominal pain. MR imaging demonstrated the extrem巴lyrapid spread of local tumor lesion. The patient died of the di町田e3 mon出spostoperatively. Metastasis of distant organs and lymph nodes were not observed until death. Y. Nakai et al (166) Table 1. Results of immunnhisto chemical staining for retroperitoneal fibrosarooma The Jaoan巴seliterature 日 sultsRepコ代ed~Jo year 80ur四1234567 Arata， et al. : Nishinih口n，1しIml581111 Takei，巳t司1..Acta U IJ1 Jpn 43: 487 I'，J日m臼t口， etal. Jロ1.')ClinしItu152:323 Asano，巳tal. Ob3tet Gvre P旧口ti回 501265Og.at司，日t81. .Jpl .J Urol 3Llrg 14: 1叩Takeuchi，日tョ1.:Act日υ回 1"Ip門 50:525 K昂kut日，日tal.: ActaリrolJpn 52: 271 N自国tive8100 810.間 E，Actin， rv1>，.usin. C8smin no円巴8100. C034. Actin. C恒smln3100， Cy，tロ|唱「ヨtin8100， Actin.， [回smin，HHF-35 8100. c8宕min.(1.-8MA 8100，CIくフ ¥Nide ぬratin，EMA. Ci1t口|氾ratil.ca4， C仁)68.ca8， ErvlA Calretini門Positive Vim巴ntinVim巴ntinVilT沼ntinnOle ¥/ilTl己ntinVim巴ntinVimentin 6即関門羽珂暗由回9α氏。内U4l41414ム円己ηノ』円J』。urcase ¥iimentin 2氏。8 DISCUSSION Ma1ignant tumors account for 70 to 80% of retroperitonea1 tumors.1) Fibrosarcoma is rare among ma1ignant soft tissue tumors， On1y 4.6% of fibrosarcomas deve10ped in the retroperitonea1 space.2) The disease-re1ated symptoms are an abdomina1 mass， weight 10ss， abdomina1 pain and fullness， which are caused by en1argement of the tumor.3) Our・ casepresented with 1umbago. Thus， the de1ayed deve10pment of subjective symptoms is one of the reasons for the poor prognosis of this disease. Among diagnostic imaging moda1ities， MR imaging wou1d be usefu1 for the diagnosis of fibrosarcoma.4) However， there were no reports in the ]apanese literature in which MR imaging 1ed to the accurate preoperative diagnosis of fibrosarcoma， because the findings vary according to the tumor differentiation， and the degree of degeneration， necrosis and hemorrhage.3) In our case， in fact， the findings of MR imaging suggested a hematoma at the initia1 presentation and even at re-admission when marked spread of the tumor was observed The fina1 diagnosis shou1d be reached by histo1ogica1 examinations. In hematoxy目eosinstaining， f白ibrosa但rc∞omaoften shows a her打ring-bonepattern: Un凶1廿if白orms叩pind1earranged in白nt白erla配cingsolid and bund1e forms. However， itis well known that it is very difficu1t to diagnose soft tissue tumors on1y by a routine histo1ogica1 examination. In the past two decades， immunohistochemica1 studies have been p1aying a ro1e to assist the differentia1 diagnosis， Takeuchi et a1.5) accumu1ated 37 cases of retroperitonea1 fibrosarcoma in the ]apanese literature. We collected 8 reports including our case， which were examin巴dimmunohistochemically for the fina1 diagnosis (Tab1e 1). The on1y positive finding was obtained with vimentin as well as that in our case. For the differentia1 diagnosis， solitary fibrous tumor， ma1ignant fibrous histiocytoma and synovica1 sarcoma were on the 1ist. In our case， CD34 was negative， suggesting solitary fibrous tumor was not the diagnosis because 90% of solitary fibrous tumor is positive for CD34.6) Being negative for both CK7 and wide keratin withdrew synovica1 sarcoma from the 1ist.7) A1though it was very difficu1t to differentiate ma1ignant fibrous histiocytoma from fibrosarcoma， the herring-bone pattern A case of retroperitoneal fibrosarcoma (167) observed in histology supported our diagnosis. The following findings indicated this case to have extremely high malignant potential: the increased cell density， frequent mitotic figures， scarcity of collagen， existence of necrosis， and high MIB-index of 70 to 80%.8) The first line treatment of fibrosarcoma would be surgical extirpation. In many cases as well as our case， however， the tumors have often become huge and have invaded to the surrounding tissues at the time of presentation. In addition， the tumor often shows a resinoid growth pattern without a capsule.3) Thus， ithas been reported that approximately 50% of fibrosarcoma resulted in non-curative surgery，4，9) and that the recurrence rate was 70% even after curative surgery.9) The present case showed extremely rapid growth and high invasion potential. However， no sign of metastasis was detected until death. Although metastasis has been recognized to be rare in fibrosarcomas， the prognosis of fibrosarcoma is poor: the 5-year survival rate is around 20%.心Thisfact indicates that therapies other than surgery are not effective. Surgery， radiation therapy， and chemotherapy are treatment options， but the most important factor in the treatment of primary tumors is complete surgical resection.lO) REFERENCES 1) Resnik， M.1. and Kursh， E.D.: Extrinsic obstruction of the ureter. In: Campbell's Urology， Walsh PC， Retic SB， Stamey T A， etal. (巴ds)，6th ed， WB Saunders Company， Philadelphia， pp 558-559， 1992. 2) Krandorf， M.J.: Malignant soft tissue tumors in a larg巴 referralpopulation: distribution of diagnosis by age， sex and location. A]R， 164: 129-134， 1995. 3) Felix， E.L.， Wood， D.K. and Gupta， T.K.: Tumors of retroperitoneum. In: Current problem in cancer， Hickey RC (ed)， Year Book Medical Publishers， Chicago， pp 33-35， 1981. 4) Nishikawa， K.， Fukui， J.， Kiyota， A.， Nishimoto， K.， Nishio， S.and Maekawa， T.: A case of retroperito-neal fibrosarcoma: usefulness of magnetic resonance imaging (in ]apanese). Acta Urol. ]pn. 44 : 17-20， 1998. 5) Takeuchi， Y.， Sawada， Y.， Yabuki， D.， Masuda， E.， Sato， D.， Iwasawa， T.， Kuroda， K.， Tajima， M.， Matsushima， M.， Oharaseki， T. and Hiruta， N.: Retroperitoneal fibrosarcoma with myxoidmatrix: a case report (in ]apanese)目 ActaUrol. ]pn. 50 : 525-529， 2004. 6) Znati， K.， Chbani， L.， El Fatemi， H.， Harmouch， T.， Kamaoui， 1.， Tazi， F.， Bennis， S.and Amarti， A.: Soli-tary fibrous tumor of the kidney: a case report and review of th巴 literature.Rev. Uro. 9 : 36-40， 2007. 7) Miettinen， M.， Limon， J.， Niezabitowski， A. and Lasota， J.・Calretininand other mesothelioma markers in synovial sarcoma: analysis of antigenic similarities and differences with malignant mesothelioma. Am. ]. Surg. Pathol目 25: 610-617， 2001. 8) Hasegawa， T.: Histological grading and MIB.1 labeling index of soft-tissu巴 sarcomas.Pathol. Int. 57 121-125， 2007 9) Cody， H.S. m， Tumbull， A.D.， Fortner， M.D. and Hajdu， S.I.: The continuing chall巴ngeof retrop巴ritonealsarcomas. Cancer 47 : 2147-2152， 1981. 10) Windham， T.C. and Pisters， P.羽T.:Retroperitoneal sarcomas. Cancer Control 12 : 36-43， 2005. 

RETROPERITONEAL FIBROSARCOMA WITH DISTINCTIVE CHARACTERISTICS ON GROWTH P ATTERN AND FINDINGS IN DIAGNOSTIC IMAGING

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RETROPERITONEAL FIBROSARCOMA WITH DISTINCTIVE CHARACTERISTICS ON GROWTH P ATTERN AND FINDINGS IN DIAGNOSTIC IMAGING

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