|Year : 2017 | Volume
| Issue : 2 | Page : 67-69
Malignant fibrous histiocytoma of the kidney
Sunita Singh, Yashika Bhatia, Jyoti Sharma, Rajeev Sen
Department of Pathology, Pt. B. D. Sharma PGIMS, Rohtak, Haryana, India
|Date of Web Publication||20-Jun-2017|
Department of Pathology, Pt. B. D. Sharma PGIMS, Rohtak - 124 001, Haryana
Source of Support: None, Conflict of Interest: None
Primary renal malignant fibrous histiocytoma (MFH) is a rare tumor indistinguishable from renal cell carcinoma clinically and radiologically which can be differentiated only by histological and immunohistochemical studies. We report a case of primary renal MFH in a 40-year-old female who presented with left flank pain. Abdominal ultrasonography and computerized tomography revealed a well-defined renal mass. The contralateral kidney and renal functions were normal. Preoperative diagnosis was renal cell carcinoma and nephrectomy was performed. Histological examination and immunohistochemistry revealed MFH.
Keywords: Malignant fibrous histiocytoma, rare tumor, renal
|How to cite this article:|
Singh S, Bhatia Y, Sharma J, Sen R. Malignant fibrous histiocytoma of the kidney. J Integr Nephrol Androl 2017;4:67-9
| Introduction|| |
Malignant fibrous histiocytoma (MFH) or malignant fibroxanthoma is a primitive mesenchymal tumor and demonstrates both histiocytic and fibroblastic differentiation. It is regarded as the most common type of soft-tissue sarcoma in adults. Commonly involves the extremities (67%–75%) followed by retroperitoneum (6%–16%) and rarely genitourinary organs. Renal sarcoma constitutes only 1%–3% of all malignant renal tumors, in which MFH is an unusual type of renal sarcoma. Primary renal MFH is extremely rare, and only a few cases reported so far. We report this rare case in a 40-year-old female.
| Case Report|| |
A 40-year-old female presented with left flank pain, abdominal discomfort, fatigue, and weight loss for the last 4 months. On physical examination, a large mass in the left hypochondrium was observed. Hemogram showed anemia and leukocytosis with high erythrocyte sedimentation rate. Ultrasonographic imaging revealed a hypoechoic large mass measuring 15 cm × 12 cm × 10 cm in the left lumbar and pelvic region. Computer tomography (CT) confirmed the tumor with areas of necrosis in relation to the upper pole of the left kidney (no radiological image was available). However, no distant organ metastases were found. Clinically and radiologically, a diagnosis of the left renal cell carcinoma was made, and left nephrectomy was performed.
Nephrectomy specimen was sent to Department of Pathology for histopathological examination. Grossly, specimen weighing 1600 g and measuring 20 cm × 15 cm × 10 cm was received comprising kidney and perirenal fat. On serial sectioning, a gray-white to gray-yellow tumor area measuring 13 cm × 13 cm × 8 cm was identified in the upper pole of the kidney reaching up to the capsule [Figure 1].
|Figure 1: Gross appearance of renal mass in the upper pole with homogenous gray-white cut surface|
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Histopathological examination showed predominantly spindle-shaped cells arranged in groups, fascicles, and storiform pattern with the areas of myxoid change and necrosis. Tumor cells were large, pleomorphic with spindle-shaped hyperchromatic nucleus. Mitotic activity and tumor giant cells were also seen at some places [Figure 2]. On immunohistochemistry, tumor cells were vimentin and CD68 positive whereas cytokeratin, CD10, desmin, smooth muscle actin were negative excluding the diagnosis of other mesenchymal tumors and sarcomatoid renal cell carcinoma [Figure 3]. These morphological and immunohistochemical findings along with the lack of another tumor in retroperitoneal or soft-tissue location led to the diagnosis of primary renal MFH. Six months follow-up was done for this case, and the patient was doing well postoperatively.
|Figure 2: (a) Tumor mass with fibrous capsule (H and E, × 20), (b) Tumor cells with whorling pattern (H and E, × 20), (c) Tumor cells with whorling pattern (H and E, × 40), and (d) Tumor mass with giant cells. (H and E, × 40)|
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|Figure 3: Immunohistochemistry of tumor cells (a) Vimentin-positive, (b) Epithelial membrane antigen-positive, (c) CD10-positive, and (d) CD68-positive|
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| Discussion|| |
Malignant fibrous histiocytoma is a soft-tissue sarcoma first described in 1964 by O'brien and Stout. Most cases occur in the deep soft tissues of extremities, with a higher incidence in the lower extremities than upper extremities and the retroperitoneum. Common in adults with peak incidence in the seventh decade of life and equal sex predilection.
Primary renal MFH is a rare tumor comprising <6% of all renal sarcomas. Clinically, patients usually present with nonspecific symptoms such as palpable abdominal mass, fever, fatigue, weight loss, and gastrointestinal problems which delay the diagnosis of this tumor. Radiological diagnostic modalities usually include ultrasonography and CT. However, clinically and radiographically, renal MFH cannot be differentiated from other renal or retroperitoneal tumors and usually undergo simple or radical nephrectomy.
Definitive diagnosis is possible only by histological examination and immunohistochemistry. MFH is characterized by storiform arrangement of spindle tumor cells with an admixture of histiocyte-like cells and multinucleated giant cells admixed with variable numbers of inflammatory cells. Several morphological variants of MFH have been described, namely, storiform-pleomorphic type (most common), giant cell type, myxoid type, and inflammatory type depending on the predominant tumor component.
It may be difficult to differentiate these lesions from other sarcomas, sarcomatoid carcinoma, and renal cell carcinoma. Diagnosis of MFH is a diagnosis of exclusion. Tumor exhibits no specific cell lineage of differentiation except fibroblastic (vimentin positivity) and histiocytic (CD68 positivity). Primary renal MFH shows a high rate of local recurrence (more than 50%) and distant metastasis despite all treatment options with 5 year- survival rate of only 14%. The most common distant metastasis sites are the lungs, lymph nodes, bone, and liver. The best treatment is radical nephrectomy to which chemoradiotherapy can be added.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]