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Year : 2017  |  Volume : 60  |  Issue : 3  |  Page : 402-404
Malignant fibrous histiocytoma arising from renal capsule: An extremely rare entity

1 Department of Pathology, SHKM, GMC, Mewat, Haryana, India
2 Department of Pathology, SMS, Jaipur, Rajasthan, India
3 Department of Pathology, BPS, Khanpur, Haryana, India

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Date of Web Publication22-Sep-2017


Malignant fibrous histiocytoma (MFH) usually presents in the extremities or retroperitoneum. MFH arising from renal parenchyma or renal capsule is extremely rare, only few cases have been reported in literature and portend a poor prognosis. Renal MFH is differentiated from renal cell carcinoma, renal sarcoma, and sarcomatoid renal tumor only by histological and immunohistochemical studies. Since the therapeutic options for MFH are different, its early diagnosis is imperative. Herein, we report a case of a primary renal MFH in a 35-year-old male.

Keywords: Kidney capsule, malignant fibrous histiocytoma, soft tissue sarcoma

How to cite this article:
Bairwa S, Sangwaiya A, Ansari M, Jindal A, Singla S, Yadav A. Malignant fibrous histiocytoma arising from renal capsule: An extremely rare entity. Indian J Pathol Microbiol 2017;60:402-4

How to cite this URL:
Bairwa S, Sangwaiya A, Ansari M, Jindal A, Singla S, Yadav A. Malignant fibrous histiocytoma arising from renal capsule: An extremely rare entity. Indian J Pathol Microbiol [serial online] 2017 [cited 2021 Jul 23];60:402-4. Available from: https://www.ijpmonline.org/text.asp?2017/60/3/402/215398

   Introduction Top

Malignant fibrous histiocytoma (MFH) was first described by O'Brien and Stout in 1964 and is believed to arise from primitive mesenchymal cells that demonstrate both fibroblastic and histiocytic differentiation.[1] It is the most common soft tissue sarcoma in adults and majority of cases occur in the trunk and extremities (70%–75%) followed by retroperitoneum comprising 16% of 200 reported cases.[2] However, MFH arising from renal parenchyma or renal capsule is extremely rare and approximately 55 cases have been reported in literature.[3] Herein, we report a new case of renal MFH as its renal localization is extremely rare and it is characterized by high risk of local and distant recurrences, poor prognosis, complicated clinical and histopathological diagnosis, and is very often a diagnosis of exclusion.

   Case Report Top

A 35-year-old male was admitted to our hospital with the chief complaint of right flank pain and abdominal discomfort for the past 8 months associated with fever, anorexia, weight loss, and gross hematuria. On physical examination, a nontender, fixed mass was noted in the right lumbar region. Laboratory analysis revealed anemia (Hb: 10 g/dL), raised erythrocyte sedimentation rate (98 mm in the 1st h), and leukocytosis (25,000/μL). All the other biochemical data including renal function tests were normal. Computed tomography scan revealed an exophytic mass measuring 8 cm × 8 cm arising from lower pole of the right kidney [Figure 1]. It showed heterogeneous enhancement on postcontrast study with few necrotic areas suggestive of renal cell carcinoma (RCC). Rest of the renal parenchyma appeared normal and showed normal enhancement and excretion on postcontrast images. There was no involvement of renal vein and inferior vena cava. The patient underwent right radical nephrectomy by thoracoabdominal approach and specimen was received for histopathological examination. Grossly, it was a well-demarcated, yellowish-white mass measuring 10 cm × 8 cm × 5 cm located at the lower pole and was adhered to the renal capsule. Cut surface showed focal areas of necrosis and hemorrhage [Figure 2]. The mass had neither invaded the renal parenchyma nor extended through Gerota's fascia. No lymph nodes were resected. Microscopically, renal parenchyma was spare [Figure 3]a. The tumor was closely abutting the kidney and infiltrating the renal capsule [Figure 3]b. The tumor was composed of pleomorphic spindle-to-multinucleated polygonal cells arranged in fascicles and storiform pattern admixed with scattered inflammatory cells along with few areas of necrosis [Figure 3]c. It was mitotically active, 8 mitotic figures per 10 high-power field were seen. On immunohistochemistry (IHC), the tumor cells showed positivity for vimentin and negative for cytokeratins, smooth muscle antigen, S-100, and epithelial membrane antigen (EMA) [Figure 4]. A final diagnosis of MFH was made, and the patient was referred for postoperative chemotherapy. Postoperative course proved uneventful, and the patient died within 6 months of surgery due to recurrence.
Figure 1: Photograph of computed tomography scan showing an exophytic growth arising from the lower pole of the right kidney

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Figure 2: Photograph of the gross picture of the right radical nephrectomy specimen showing a well-demarcated, gray-white area with focal areas of necrosis and mucoid degeneration

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Figure 3: Photomicrograph showing (a) normal renal parenchyma, (H and E, ×100) (b) the tumor was closely abutting the kidney and infiltrating the renal capsule, (H and E, ×100) (c) tumor composed of pleomorphic spindle-to-multinucleated polygonal cells along with brisk mitotic activity (H and E, ×400)

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Figure 4: Photomicrograph of the tumor cells showing (a) vimentin positivity (IHC, ×100), (b) epithelial membrane antigen negativity (IHC, ×100), (c) cytokeratin positivity (IHC, ×100), and (d) S100 negativity (IHC, ×100)

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   Discussion Top

MFH is considered synonymous with pleomorphic undifferentiated sarcoma according to recent World Health Organization classification of soft tissue tumors. The tumor may be classified as undifferentiated pleomorphic sarcoma after exclusion of other differentiation.[4] MFH is the most frequently reported histopathological diagnosis among soft tissue sarcomas.[2]

Primary sarcomas of the kidney are rare, constitutes only 1%–3% of malignant renal tumors during the seventh decade of life with equal sex distribution.[2] MFH arising from the renal parenchyma or renal capsule accounts <6% of renal sarcomas. MFH most commonly affects the left kidney.[2],[4] But in this case, the right kidney was involved. Flank pain, fever, weight loss, and a palpable renal mass are widely observed manifestations. Hematuria is rarely noted.[5] Here on physical examination, mass was palpated and gross hematuria was present as the patient was on anticoagulants for coronary artery disease. Before the clinical symptoms could appear, the mass grew rapidly and became bulky due to its retroperitoneal position and thus delayed the correct diagnosis.

There have been few cases reported in literature since Anderson et al. reported the first case in 1977. The infrequent occurrence, many pathological features, uncertain histogenesis, various subtypes, and many potential sites of presentation make these tumors a challenge for the diagnostician, surgeon, and oncologist. Establishing the diagnosis of renal MFH radiologically and clinically and differentiating it from other renal tumors such as RCC, sarcomatoid carcinoma, and other retroperitoneal tumors is a very tough and challenging task as it is relatively asymptomatic with nonspecific imaging finding.[5]

The histological differential diagnosis includes xanthogranulomatous pyelonephritis, sarcomatoid RCC, and a recently described entity anaplastic sarcoma of the kidney.[6] Xanthogranulomatous pyelonephritis is a reactive renal pseudotumor that shows histiocytes, mixed inflammatory infiltrates, and fibroblastic proliferation.[7] Sarcomatoid RCC is immunopositive for cytokeratin and vimentin, while MFH is positive for vimentin but negative for cytokeratin.[8] Anaplastic sarcoma of kidney is a polyphenotypic tumor composed of a spindle cell component, round-to-oval nuclei in a myxoid stroma with divergent differentiation such as chondroid and osteoid matrix.[6]

Few criteria have been suggested for diagnosing the primary renal MFH which are; (a) History of sarcoma should be ruled out to exclude metastasis, (b) The gross appearance must be compatible with the origin in kidney, (c) Sarcomatoid RCC must be excluded, and (d) Metastasis if present must be smaller than renal tumor.[6] A correct preoperative diagnosis was not made in any of the reports in literature.[9] In the case reported here, preoperative differential diagnosis from RCC was not feasible. Definitive diagnosis can be made on histopathology.

Histologically, the tumor contains both fibroblast-like and histiocyte-like cells in varying proportion, with spindled and rounded cells exhibiting a storiform arrangement. Five histological subtypes have been described; (a) storiform/pleomorphic, (b) myxoid, (c) giant cell, (d) inflammatory, and (e) angiomatoid.[3] Pleomorphic is the most commonly seen subtype.

The factors denoting poor prognosis are old age, tumor size, location, either superficial or deep, tumor necrosis, high mitotic count, degree of infiltration, and distant metastasis.[10] On performing IHC, the tumor cells shows positivity for vimentin. The tumor cells were negative for cytokeratins, EMA, and S-100.

Radical nephrectomy is the mainstay of treatment. Adjuvant chemotherapy and radiotherapy is required to minimize the risk of local recurrences and distant metastasis. Twenty-five percent of the reported cases died within 1 year of surgery.[9] The common distant metastatic sites are the lungs, liver, lymph nodes, and bone.[11]

   Conclusion Top

To conclude, primary renal MFH is an extremely rare entity with no distinctive clinical and radiological features and portend an unfavorable prognosis. The pathologist must be aware of this entity and must include in the differential diagnosis of all kidney tumors.

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Conflicts of interest

There are no conflicts of interest.

   References Top

O'Brien JE, Stout AP. Malignant fibrous xanthomas. Cancer 1964;17:1445-55.  Back to cited text no. 1
Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: An analysis of 200 cases. Cancer 1978;41:2250-66.  Back to cited text no. 2
Gögüs Ç, Gökce MI, Süer E, Tulunay Ö, Safak M. Primary malignant fibrous histiocytoma of the kidney: Report of a case and literature review. Turk J Urol 2013;39:194-7.  Back to cited text no. 3
Shirkhoda A, Lewis E. Renal sarcoma and sarcomatoid renal cell carcinoma: CT and angiographic features. Radiology 1987;162:353-7.  Back to cited text no. 4
Fletcher CD. Pleomorphic malignant fibrous histiocytoma: Fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma. Am J Surg Pathol 1992;16:213-28.  Back to cited text no. 5
Gupta R, Gupta S, Aggarwal D, Singh S. Primary pleomorphic undifferentiated sarcoma of the kidney: A rare renal tumor. Indian J Pathol Microbiol 2008;51:573-6.  Back to cited text no. 6
[PUBMED]  [Full text]  
Singh SK, Mandal AK, Agarwal MM, Das A. Primary renal inflammatory malignant fibrous histiocytoma: A diagnostic challenge. Int J Urol 2006;13:1000-2.  Back to cited text no. 7
Ptochos A, Karydas G, Iosifidis N, Tyrothoulakis E, Papazafiriou G, Kehagia-Koutoufari T. Primary renal malignant fibrous histiocytoma. A case report and review of the literature. Urol Int 1999;63:261-4.  Back to cited text no. 8
Kitajima K, Kaji Y, Morita M, Okuda Y, Sugimura K. Malignant fibrous histiocytoma arising from the renal capsule. Magn Reson Med Sci 2003;2:199-202.  Back to cited text no. 9
Kumar S, Bansal P, Tiwari P, Kundu AK. Renal pleomorphic undifferentiated sarcoma: A rarity. Saudi J Kidney Dis Transpl 2012;23:1241-2.  Back to cited text no. 10
[PUBMED]  [Full text]  
Senturk N, Kelten C, Ozdemir NO, Duzcan E. Primary Malignant Fibrous histiocytoma of the kidney: Report of a case.. Turk Patoloji Derg 2010;26:165-7.  Back to cited text no. 11

Correspondence Address:
Shilpa Bairwa
H. No. 1444, Lane No. 10, Laxman Vihar, Phase II, Gurgaon - 122 501, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_810_15

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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