The Open Urology & Nephrology Journal




ISSN: 1874-303X ― Volume 12, 2019

Long-Term Results of Radical Surgery and Adjuvant Radiotherapy in a Case of Primary Malignant Fibrous Histiocytoma of the Kidney



Luigi Cormio1, *, Francesca Sanguedolce2, Paolo Massenio1, Giuseppe Di Fino1, Giuseppe Lucarelli1, Vito Mancini1, Pantaleo Bufo2, Giuseppe Carrieri1
1 Department of Urology and Renal Transplantation, University of Foggia, Foggia, Italy
2 Department of Pathology, University of Foggia, Foggia, Italy

Abstract

Malignant fibrous histiocytoma of the kidney is an extremely rare tumor that isthought to arise from the renal capsule. Radical nephrectomy is the standard treatment for localized disease but 25% of patients die of metastatic disease despite radical surgical resection. In the absence of clear pathogenetic information, adjuvant treatment remains empiric. Herein, we describe the case of a previously healthy 58-year-old woman who underwent radical nephrectomy because of 8-cm solid lesion of the right kidney; pathology revealed a pleomorphic malignant fibrous histiocytoma invading the perinephric fat, stage pT3aN0. She received adjuvant radiotherapy. To date, five years after surgery, she is alive and there is no evidence of disease. To our knowledge, this is the first report of favorable long-term results of surgery and adjuvant radiotherapy in a locally-advanced malignant fibrous histiocytoma of the kidney.

Keywords: Adjuvant radiotherapy, histiocytoma, kidney, long-term, radical surgery, rare tumor.


Article Information


Identifiers and Pagination:

Year: 2014
Volume: 7
First Page: 1
Last Page: 3
Publisher Id: TOUNJ-7-1
DOI: 10.2174/1874303X01407010001

Article History:

Received Date: 11/12/2013
Revision Received Date: 14/2/2014
Acceptance Date: 14/2/2014
Electronic publication date: 21/2/2014

Article Metrics:

CrossRef Citations:
0

Total Statistics:

Full-Text HTML Views: 999
Abstract HTML Views: 1084
PDF Downloads: 318
ePub Downloads: 233
Total Views/Downloads: 2634

Unique Statistics:

Full-Text HTML Views: 619
Abstract HTML Views: 611
PDF Downloads: 181
ePub Downloads: 167
Total Views/Downloads: 1578
Geographical View

© Cormio et al.; Licensee Bentham Open

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.


* Address correspondence to this author at theDepartment of Urology and Renal Transplantation, University of Foggia, Viale Luigi Pinto 1, 71121 Foggia, Italy; Tel: +393208584073; E-mail: luigicormio@libero.it





INTRODUCTION

Malignant fibrous histiocytoma (MFH) is an extremely rare tumor that belongs to a heterogeneous group of soft tissue malignancies featuring high pleomorphic cells in a haphazard arrangement. MFH of the kidney is thought to arise from the renal capsule but it is not clear yet whether or not it is a non-epithelial renal tumor. As a matter of fact, genetic studies have shown that MFH cells, rather than being histiocytic, show strict analogies with bone-marrow derived mesenchymal stem cells [1Gazziola C, Cordani N, Wasserman B, Carta S, Colombatti A, Perris R. Malignant fibrous histiocytoma: a proposed cellular origin and identification of its characterizing gene transcripts Int J Oncol 2003; 23: 343-51.], which are currently regarded as the putative progenitor cells. Moreover, lack of a distinct gene cluster for this type of tumor suggests that most MFHs should be considered pleomorphic variants of different renal sarcomas, specifically lipo- and leiomyo- sarcomas [2Nakayama R, Nemoto T, Takahashi H , et al. Gene expression analysis of soft tissue sarcomas: characterization and reclassification of malignant fibrous histiocytoma Mod Pathol 2007; 20: 749-59.,3Tschoep K, Kohlmann A, Schlemmer M , et al. Gene expression profiling in sarcomas Crit Rev Oncol Hematol 2007; 63: 111-24.]. Radical surgery represents the mainstay of MFH treatment; nevertheless, 25% of patients with localized disease die within 1 year despite radical surgical resection. Lack of clear pathogenetic information makes empiric any adjuvant treatment, thus often unsuccessful. Herein, we report for the first time the long-term results of a pT3a MFH treated with radical surgery and adjuvant radiotherapy, and discuss challenges in diagnosis and treatment of this uncommon neoplasm.

CASE REPORT

In a previous paper we reported a case of a 68-year-old with a diagnosis of a renal malignant fibrous histiocytoma [4Marchese R, Bufo P, Carrieri G , et al. Malignant fibrous histiocytoma of the kidney treated with nephrectomy and adjuvant radiotherapy: a case report Medicine 2010.]. In this report we summarize the clinical and pathological findings with an updated immunoistochemical staining for different markers and provide data about the long-term outcome of this unusual neoplasia. A previously healthy woman presented with a one-month history of evening fever; there were no haematuria nor abdominal or flank pain. Laboratory analysis showed normal renal function, mild anemia (hemoglobin: 9.9 g/dl and hematocrit: 32%), leukocytosis with normal lymphocyte count (5.1 x103/µl), increased erythrocyte sedimentation rate (88mm/hr), along with increased alpha-1 (4,94%) and alpha-2 (16.82%) and decreased gamma (10.3%) globulins. There were no abnormalities on plain chest X-ray. Abdominal ultrasonography (US) showed a roundish hypo-echoic 8-cm solid lesion in the lower pole of the right kidney. Computed tomography (CT) confirmed the presence of an 8-cm solid mass with heterogeneous enhancement; no lymphadenopathy was noted. Right radical nephrectomy with hilar lymph node dissection was performed for a suspicious renal cell carcinoma (RCC) of the right kidney. Postoperative course was uneventful and the patient was discharged on 4th postoperative day. Macroscopic examination of the resected specimen revealed a solid grayish lesion, which microscopically consisted of haphazardly arranged highly atypical to pleomorphic, sometimes multinucleated cells, admixed with scattered inflammatory cells, with a brisk mitotic activity. Immunohistochemical analysis revealed intense vimentin staining, and negativity for cytokeratins, S-100 protein, desmin, CD34, smooth muscle actin and lymphoid lineage markers in neoplastic cells (Fig. 1). On the basis of morphologic and immunophenotypic findings, a diagnosis of pleomorphic malignant fibrous histiocytoma (MFH) invading the perinephric fat, stage pT3aN0, was

Fig. (1)

Pleomorphic sarcoma (top) with highly atypical cells admixed with inflammatory cells (haematoxylin-eosin, original magnification 200X); neoplastic cells (bottom) show intense staining for vimentin (original magnification 400X).



established. The patient received adjuvant radiotherapy (50 Gy in 25 sessions), which was well tolerated, and the patient was scheduled for a strict follow-up program including chest-abdomen-pelvis computed tomography (CT) scanning every six months and a bone scan every year. To date, 72 months after surgery, the patient is alive with no evidence of disease recurrence.

DISCUSSION

Renal MFH is an extremely rare tumor with heterogeneous clinical features. Based on the few reported cases, it may present with abdominal or flank pain, abdominal mass, fatigue, unexplained fever, and even acute urinary retention [5Ghosh A, Dwivedi US, Kumar A. Inflammatory malignant fibrous histiocytoma of kidney: A case report Pathol Res Pract 2008; 204: 857-61.-8Singh SK, Mandal AK, Agarwal MM, Das A. Primary renal inflammatory malignant fibrous histiocytoma: A diagnostic challenge Int J Urol 2006; 13: 1000-2.]. Routine investigations may reveal anemia and/or leukocytosis with neutrophylia [5Ghosh A, Dwivedi US, Kumar A. Inflammatory malignant fibrous histiocytoma of kidney: A case report Pathol Res Pract 2008; 204: 857-61.]. MFH has no specific US patterns; the diagnostic yield of CT scan and magnetic resonance imaging (MRI) for MFH seems to be related to the amount of fibrous tissue, the degree of vascularization, and the cellularity of the tumour [9Kwak HS, Kim CS, Lee JM. MR findings of renal malignant fibrous histiocytoma Eur Radiol 2003; 13: L245-6.]. The presence of a mass that appears hypointense on T2-weighted images and displays calcifications has been reported to be highly suggestive for MFH [7Kitajima K, Morita YK, Okuda Y, Sugimura K. Malignant fibrous histiocytoma arising from the renal capsule Magn Reson Med Sci 2003; 2: 199-202.,9Kwak HS, Kim CS, Lee JM. MR findings of renal malignant fibrous histiocytoma Eur Radiol 2003; 13: L245-6.]. Nevertheless, almost all cases mimic RCC and are treated accordingly. In the absence of distant metastases, treatment involves radical nephrectomy. The diagnosis of MFH relies heavily on pathologic evaluation. MFH features high pleomorphic cells in a haphazard arrangement, with several possible patterns and variants, including storiform-pleomorphic, myxoid, giant cell, inflammatory, angiomatoid [10Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma between the past and the present Arch Pathol Lab Med 2008; 132: 1030-5.]. Such cells usually show intense positivity for vimentin, but negativity for cytokeratin, S-100 protein, and lymphoid lineage markers; this panel of immunohistochemical markers plays a pivotal role in the differential diagnosis with sarcomatoid carcinoma. Radical surgical resection is of major importance as local recurrence is associated with poor prognosis even in case of early diagnosis and treatment [11May M, Marusch F, Helke C, Gunia S, Gastinger I, Hoschke B. Das lokal rezidivierende maligne fibröse Histiozytom der Niere oder des Samenstrangs Urologe [A] 2004; 43: 976-81.]. Adjuvant radiotherapy or chemotherapy, recommended to reduce the risk of cancer-related death, have yielded conflicting results [5Ghosh A, Dwivedi US, Kumar A. Inflammatory malignant fibrous histiocytoma of kidney: A case report Pathol Res Pract 2008; 204: 857-61.-7Kitajima K, Morita YK, Okuda Y, Sugimura K. Malignant fibrous histiocytoma arising from the renal capsule Magn Reson Med Sci 2003; 2: 199-202.,12Eroglu M, Bakirtas H, Cimentepe E, Unsal A, Ataoglu O, Balbay MD. Malignant fibrous histiocytoma arising from the renal capsule Urol Int 2005; 75: 368-70.,13Kim SJ, Ahn BC, Kim SR , et al. Primary malignant fibrous histiocytoma of the kidney Yonsei Med J 2002; 43: 399-402.]. As mentioned above, molecular studies suggest that most MFHs should be considered pleomorphic variants of different sarcomas. Accordingly, our patient underwent to adjuvant radiotherapy for the non-invasive nature, the low risk of side effects, and the responsiveness of sarcomas to this type of treatment. Moreover, we were dealing with a locally-advanced disease with an aggressive behavior. Patients presenting with metastatic disease are extremely challenging. Percutaneous tumor biopsy usually is of little diagnostic value, thus radical nephrectomy, if possible, is mandatory. Various sarcoma-directed radio- and/or chemo-therapy treatments have been used with variable results. Recently, tyrosine kinase inhibitors (TKIs) - which have been shown to be active in metastatic RCC - have been reported to provide beneficial effects also in treating metastatic renal MFH [14Mauri D, Panou C, Valachis A, Kamposioras K, Tsali L. Tyrosine kinase inhibitors in treatment of fibrous histiocytoma Exp Oncol 2009; 31: 60-1.,15Eichelberg C, Heuer R, Chun FK , et al. Sequential use of the tyrosine kinase inhibitors sorafenib and sunitinib in metastatic renal cell carcinoma: a retrospective outcome analysis Eur Urol 2008; 54: 1373-8.]. In conclusion, renal MFH is a rare and complex entity mimicking RCC. Treatment of localized and locally-advanced disease relies on radical surgical excision. Since renal MFH belongs to renal sarcomas, adjuvant radiotherapy should have the potential to reduce the risk of local recurrence. The reported case provides evidence for favorable long-term results of adjuvant radiotherapy; however, whether it definitely improves survival remains unknown. TKIs seem to represent new efficient opportunities to treat metastatic disease but molecular identification of targets amenable to specific therapy would be extremely welcome in the management of this rare but insidious disease.

CONFLICT OF INTEREST

The authors confirm that this article content has no conflict of interest.

ACKNOWLEDGEMENT

Declared none.

REFERENCES

[1] Gazziola C, Cordani N, Wasserman B, Carta S, Colombatti A, Perris R. Malignant fibrous histiocytoma: a proposed cellular origin and identification of its characterizing gene transcripts Int J Oncol 2003; 23: 343-51.
[2] Nakayama R, Nemoto T, Takahashi H , et al. Gene expression analysis of soft tissue sarcomas: characterization and reclassification of malignant fibrous histiocytoma Mod Pathol 2007; 20: 749-59.
[3] Tschoep K, Kohlmann A, Schlemmer M , et al. Gene expression profiling in sarcomas Crit Rev Oncol Hematol 2007; 63: 111-24.
[4] Marchese R, Bufo P, Carrieri G , et al. Malignant fibrous histiocytoma of the kidney treated with nephrectomy and adjuvant radiotherapy: a case report Medicine 2010.
[5] Ghosh A, Dwivedi US, Kumar A. Inflammatory malignant fibrous histiocytoma of kidney: A case report Pathol Res Pract 2008; 204: 857-61.
[6] Gupta R, Gupta S, Aggarwal D, Singh S. Primary pleomorphic undifferentiated sarcoma of kidney: A rare renal tumor Indian J Pathol Microbiol 2008; 51: 573-6.
[7] Kitajima K, Morita YK, Okuda Y, Sugimura K. Malignant fibrous histiocytoma arising from the renal capsule Magn Reson Med Sci 2003; 2: 199-202.
[8] Singh SK, Mandal AK, Agarwal MM, Das A. Primary renal inflammatory malignant fibrous histiocytoma: A diagnostic challenge Int J Urol 2006; 13: 1000-2.
[9] Kwak HS, Kim CS, Lee JM. MR findings of renal malignant fibrous histiocytoma Eur Radiol 2003; 13: L245-6.
[10] Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma between the past and the present Arch Pathol Lab Med 2008; 132: 1030-5.
[11] May M, Marusch F, Helke C, Gunia S, Gastinger I, Hoschke B. Das lokal rezidivierende maligne fibröse Histiozytom der Niere oder des Samenstrangs Urologe [A] 2004; 43: 976-81.
[12] Eroglu M, Bakirtas H, Cimentepe E, Unsal A, Ataoglu O, Balbay MD. Malignant fibrous histiocytoma arising from the renal capsule Urol Int 2005; 75: 368-70.
[13] Kim SJ, Ahn BC, Kim SR , et al. Primary malignant fibrous histiocytoma of the kidney Yonsei Med J 2002; 43: 399-402.
[14] Mauri D, Panou C, Valachis A, Kamposioras K, Tsali L. Tyrosine kinase inhibitors in treatment of fibrous histiocytoma Exp Oncol 2009; 31: 60-1.
[15] Eichelberg C, Heuer R, Chun FK , et al. Sequential use of the tyrosine kinase inhibitors sorafenib and sunitinib in metastatic renal cell carcinoma: a retrospective outcome analysis Eur Urol 2008; 54: 1373-8.
Society Affiliation


Endorsements



"Open access will revolutionize 21st century knowledge work and accelerate the diffusion of ideas and evidence that support just in time learning and the evolution of thinking in a number of disciplines."


Daniel Pesut
(Indiana University School of Nursing, USA)

"It is important that students and researchers from all over the world can have easy access to relevant, high-standard and timely scientific information. This is exactly what Open Access Journals provide and this is the reason why I support this endeavor."


Jacques Descotes
(Centre Antipoison-Centre de Pharmacovigilance, France)

"Publishing research articles is the key for future scientific progress. Open Access publishing is therefore of utmost importance for wider dissemination of information, and will help serving the best interest of the scientific community."


Patrice Talaga
(UCB S.A., Belgium)

"Open access journals are a novel concept in the medical literature. They offer accessible information to a wide variety of individuals, including physicians, medical students, clinical investigators, and the general public. They are an outstanding source of medical and scientific information."


Jeffrey M. Weinberg
(St. Luke's-Roosevelt Hospital Center, USA)

"Open access journals are extremely useful for graduate students, investigators and all other interested persons to read important scientific articles and subscribe scientific journals. Indeed, the research articles span a wide range of area and of high quality. This is specially a must for researchers belonging to institutions with limited library facility and funding to subscribe scientific journals."


Debomoy K. Lahiri
(Indiana University School of Medicine, USA)

"Open access journals represent a major break-through in publishing. They provide easy access to the latest research on a wide variety of issues. Relevant and timely articles are made available in a fraction of the time taken by more conventional publishers. Articles are of uniformly high quality and written by the world's leading authorities."


Robert Looney
(Naval Postgraduate School, USA)

"Open access journals have transformed the way scientific data is published and disseminated: particularly, whilst ensuring a high quality standard and transparency in the editorial process, they have increased the access to the scientific literature by those researchers that have limited library support or that are working on small budgets."


Richard Reithinger
(Westat, USA)

"Not only do open access journals greatly improve the access to high quality information for scientists in the developing world, it also provides extra exposure for our papers."


J. Ferwerda
(University of Oxford, UK)

"Open Access 'Chemistry' Journals allow the dissemination of knowledge at your finger tips without paying for the scientific content."


Sean L. Kitson
(Almac Sciences, Northern Ireland)

"In principle, all scientific journals should have open access, as should be science itself. Open access journals are very helpful for students, researchers and the general public including people from institutions which do not have library or cannot afford to subscribe scientific journals. The articles are high standard and cover a wide area."


Hubert Wolterbeek
(Delft University of Technology, The Netherlands)

"The widest possible diffusion of information is critical for the advancement of science. In this perspective, open access journals are instrumental in fostering researches and achievements."


Alessandro Laviano
(Sapienza - University of Rome, Italy)

"Open access journals are very useful for all scientists as they can have quick information in the different fields of science."


Philippe Hernigou
(Paris University, France)

"There are many scientists who can not afford the rather expensive subscriptions to scientific journals. Open access journals offer a good alternative for free access to good quality scientific information."


Fidel Toldrá
(Instituto de Agroquimica y Tecnologia de Alimentos, Spain)

"Open access journals have become a fundamental tool for students, researchers, patients and the general public. Many people from institutions which do not have library or cannot afford to subscribe scientific journals benefit of them on a daily basis. The articles are among the best and cover most scientific areas."


M. Bendandi
(University Clinic of Navarre, Spain)

"These journals provide researchers with a platform for rapid, open access scientific communication. The articles are of high quality and broad scope."


Peter Chiba
(University of Vienna, Austria)

"Open access journals are probably one of the most important contributions to promote and diffuse science worldwide."


Jaime Sampaio
(University of Trás-os-Montes e Alto Douro, Portugal)

"Open access journals make up a new and rather revolutionary way to scientific publication. This option opens several quite interesting possibilities to disseminate openly and freely new knowledge and even to facilitate interpersonal communication among scientists."


Eduardo A. Castro
(INIFTA, Argentina)

"Open access journals are freely available online throughout the world, for you to read, download, copy, distribute, and use. The articles published in the open access journals are high quality and cover a wide range of fields."


Kenji Hashimoto
(Chiba University, Japan)

"Open Access journals offer an innovative and efficient way of publication for academics and professionals in a wide range of disciplines. The papers published are of high quality after rigorous peer review and they are Indexed in: major international databases. I read Open Access journals to keep abreast of the recent development in my field of study."


Daniel Shek
(Chinese University of Hong Kong, Hong Kong)

"It is a modern trend for publishers to establish open access journals. Researchers, faculty members, and students will be greatly benefited by the new journals of Bentham Science Publishers Ltd. in this category."


Jih Ru Hwu
(National Central University, Taiwan)


Browse Contents




Webmaster Contact: info@benthamopen.net
Copyright © 2019 Bentham Open