Primary renal inflammatory malignant fibrous histiocytoma: A diagnostic challenge

Department of Urology, Postgraduate Instiute of Medical Education and Research, Chandigarh, India.
International Journal of Urology (Impact Factor: 2.41). 08/2006; 13(7):1000-2. DOI: 10.1111/j.1442-2042.2006.01458.x
Source: PubMed


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.

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Available from: Shrawan K Singh, Sep 15, 2014
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    • "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 [12] [14] [17] [19] [22] [23]. "
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    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.
    Full-text · Article · Oct 2012
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    • "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 [11] [14] [15]. The case described by Singh and coworkers [11] was treated only with surgery and died within one month of diagnosis. Ishibiki and coworkers [14] 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. "
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    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.
    Full-text · Article · Sep 2010 · Case Reports in Medicine
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    ABSTRACT: To study paratesticular malignant fibrous histiocytomas (FHM) from the clinical, histological, immunohistochemical and histogenetic, point of view. Through Medline and not index-linked search of international scientific literature we have found a total of 77 cases of paratesticular FHM that globally create diagnostic, terminological and therapeutic problems. We include the case number 78 of FHM of spermatic cord, that presents a few special characteristics (not described before) of cutaneous fistula and infiltration of an inguinal leiomyoma, with histological and immunohistochemical confirmation. We performed a conceptual review of this type of tumours, including the evaluation of contradictions and reclassifications that has undergone from its first description, which causes that they are underestimated and their real prevalence in international series is very variable: from 7 to 37% of paratesticular sarcomas. Also, the fact of their low incidence has determined the lack of protocols for diagnosis and treatment. Paratesticular tumours, although infrequent, have a high rate of malignancy. The FHM concept has varied throughout the last 15 years. Today, this term is synonymous of pleomorphic undifferentiated sarcoma and has been an exclusion diagnosis (Vimentin [+]/ CD 68 [+]--occasionally--without ultrastructural differences), reserving the inflammatory subtype for which they have an inflammatory and histiocytic component. The accomplishment of an intraoperatory biopsy is essential in this location, since it is even difficult to know preoperatively, if a lesion is benign or malignant. The differential diagnosis is only solved after microscopic and inmunohistochemical study. Leiomyoma next to this location (inguinal) could be diagnosed and confused with a nodule of the main FHM. Their immunophenotype allowed us to diagnose it not only as leiomyoma (myogenic markers were positive -non skeletal muscle-), but also to observe the infiltration by the FHM.
    No preview · Article · Jun 2008 · Archivos españoles de urología
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