Primary renal inflammatory malignant fibrous histiocytoma: A diagnostic challenge
ABSTRACT Renal inflammatory malignant fibrous histiocytoma (MFH) is an extremely rare lesion and is a great masquerader of common benign renal inflammatory lesions, especially xanthogranulomatous pyelonephritis (XGPN). The clinical presentation of mass with fever and peripheral leucocytosis (marked at times), and marked inflammatory, predominantly neutrophilic, infiltrate obscuring the malignant cells on histopathology, can lead to delay in the diagnosis of this poor prognostic malignant tumor. We present the case of a patient who underwent radical nephrectomy with a clinical diagnosis of renal malignancy, but histopathology showing XGPN. The patient showed an initial clinical response, only to recur two times, ultimately leading to a histological and immunohistochemical diagnosis of inflammatory MFH. The diagnosis, histology, therapeutic options and prognosis of this rare lesion are discussed.
Full-textDOI: · Available from: Shrawan Singh, Sep 15, 2014
- SourceAvailable from: Marianne Frieri
[Show abstract] [Hide abstract]
- "The previous statement correlates with some of the cases described in which IMFH with/without LR has been detected in solid organs, intestinal tract, musculoskeletal tissue, and the integument      . "
ABSTRACT: Inflammatory malignant fibrous histiocytoma (IMFH) associated with leukemoid reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, leukemoid reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy.10/2012; 2012:946019. DOI:10.5402/2012/946019
[Show abstract] [Hide abstract]
- "As previously reported, local recurrence and distant metastases are frequent in patients affected by renal MFH, leading to a poor prognosis for this type of tumor   . The case described by Singh and coworkers  was treated only with surgery and died within one month of diagnosis. Ishibiki and coworkers  described a case of storiform-pleomorphic malignant fibrous histiocytoma arising from perirenal tissue not treated with adjuvant therapy that developed local recurrence in the left retroperitoneal space after a followup period of 11 months. "
ABSTRACT: Malignant fibrous histiocytoma (MFH) usually presents in the extremities or retroperitoneum. Cases involving the kidney are rare and portend a poor prognosis. Although radical nephrectomy is the most beneficial curative choice for this neoplasm, patients are often treated with adjuvant chemotherapy due to high risk of local recurrence and distant metastases. We describe a case of a 68-year-old woman affected by MFH, treated with both nephrectomy and radiotherapy without systemic therapy showing an unexpected twenty-four-month postsurgery survival outcome.Case Reports in Medicine 09/2010; 2010. DOI:10.1155/2010/802026
- [Show abstract] [Hide abstract]
ABSTRACT: Among the renal sarcomas, inflammatory malignant fibrous histiocytoma (MFH) is an extremely rare presentation. A 45-year-old woman presented with acute retention urine following an episode of gross hematuria. Computerized tomography showed a solid mass at the lower pole of the left kidney. The patient underwent left nephrectomy. Histologically and immunohistochemically, the tumor was diagnosed as an inflammatory subtype of MFH. Histological appearances of inflammatory MFH vary widely and frequently overlap with benign reactive conditions such as Xanthogranulomatous pyelonephritis (XGPN) and malignant lesions, e.g. malignant lymphoma and, less frequently, a sarcomatoid variant of renal cell carcinoma. It is important, though difficult, to differentiate inflammatory MFH from these lesions. Careful morphological examination and immunohistochemical findings of the lesion are of great value, in particular in excluding it from its mimics. We discuss the pathological features and challenges involved in differentiating inflammatory MFH from its masquerader.Pathology - Research and Practice 09/2008; 204(11):857-61. DOI:10.1016/j.prp.2008.03.012 · 1.56 Impact Factor