Malignant fibrous histiocytoma (MFH) is an extremely rare tumor that belongs to a heterogeneous lipidized fibrous histiocytoma group of soft tissue malignancies featuring high pleomorphic cells in lipidized fibrous histiocytoma a haphazard arrangement. MFH of the kidney is thought to arise from the lipidized fibrous histiocytoma renal capsule but it is not clear yet whether or lipidized fibrous histiocytoma 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 [ 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 lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma tumor suggests that most mfhs should be considered pleomorphic variants lipidized fibrous histiocytoma of different renal sarcomas, specifically lipo- and leiomyo- sarcomas [ 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.]. Radical surgery represents the mainstay of MFH treatment; nevertheless, 25% of patients with localized disease die within 1 year despite lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma and adjuvant radiotherapy, and discuss challenges in diagnosis and treatment of this uncommon lipidized fibrous histiocytoma neoplasm.
In a previous paper we reported a case of a lipidized fibrous histiocytoma 68-year-old with a diagnosis of a renal malignant fibrous histiocytoma lipidized fibrous histiocytoma [ 4 marchese R, bufo P, carrieri G , et al. Malignant fibrous histiocytoma of the kidney treated with nephrectomy and lipidized fibrous histiocytoma adjuvant radiotherapy: a case report medicine 2010.]. In this report we summarize the clinical and pathological findings lipidized fibrous histiocytoma with an updated immunoistochemical staining for different markers and provide lipidized fibrous histiocytoma 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 x10 3/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 lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma for a suspicious renal cell carcinoma (RCC) of the right kidney. Postoperative course was uneventful and the patient was discharged on lipidized fibrous histiocytoma 4 th postoperative day. Macroscopic examination of the resected specimen revealed a solid grayish lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma (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
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.
Renal MFH is an extremely rare tumor with heterogeneous clinical lipidized fibrous histiocytoma 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 [ 5 ghosh A, dwivedi US, kumar A. Inflammatory malignant fibrous histiocytoma of kidney: A case report pathol res pract 2008; 204: 857-61.- 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.]. Routine investigations may reveal anemia and/or leukocytosis with neutrophylia [ 5 ghosh 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 lipidized fibrous histiocytoma (MRI) for MFH seems to be related to the amount of lipidized fibrous histiocytoma fibrous tissue, the degree of vascularization, and the cellularity of the tumour [ 9 kwak 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 lipidized fibrous histiocytoma highly suggestive for MFH [ 7 kitajima K, morita YK, okuda Y, sugimura K. Malignant fibrous histiocytoma arising from the renal capsule magn reson lipidized fibrous histiocytoma med sci 2003; 2: 199-202., 9 kwak 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 [ 10 al-agha OM, igbokwe AA. Malignant fibrous histiocytoma between the past and the present arch lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma the differential diagnosis with sarcomatoid carcinoma. Radical surgical resection is of major importance as local recurrence lipidized fibrous histiocytoma is associated with poor prognosis even in case of early lipidized fibrous histiocytoma diagnosis and treatment [ 11 may M, marusch F, helke C, gunia S, gastinger I, hoschke B. Das lokal rezidivierende maligne fibröse histiozytom der niere oder des lipidized fibrous histiocytoma samenstrangs urologe [A] 2004; 43: 976-81.]. Adjuvant radiotherapy or chemotherapy, recommended to reduce the risk of cancer-related death, have yielded conflicting results [ 5 ghosh A, dwivedi US, kumar A. Inflammatory malignant fibrous histiocytoma of kidney: A case report pathol res pract 2008; 204: 857-61.- 7 kitajima K, morita YK, okuda Y, sugimura K. Malignant fibrous histiocytoma arising from the renal capsule magn reson lipidized fibrous histiocytoma med sci 2003; 2: 199-202., 12 eroglu M, bakirtas H, cimentepe E, unsal A, ataoglu O, balbay MD. Malignant fibrous histiocytoma arising from the renal capsule urol int lipidized fibrous histiocytoma 2005; 75: 368-70., 13 kim SJ, ahn BC, kim SR , et al. Primary malignant fibrous histiocytoma of the kidney yonsei med J lipidized fibrous histiocytoma 2002; 43: 399-402.]. As mentioned above, molecular studies suggest that most mfhs should be considered pleomorphic lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma – have been reported to provide beneficial effects also in treating lipidized fibrous histiocytoma metastatic renal MFH [ 14 mauri D, panou C, valachis A, kamposioras K, tsali L. Tyrosine kinase inhibitors in treatment of fibrous histiocytoma exp oncol lipidized fibrous histiocytoma 2009; 31: 60-1., 15 eichelberg C, heuer R, chun FK , et al. Sequential use of the tyrosine kinase inhibitors sorafenib and sunitinib lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma 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 lipidized fibrous histiocytoma disease but molecular identification of targets amenable to specific therapy lipidized fibrous histiocytoma would be extremely welcome in the management of this rare lipidized fibrous histiocytoma but insidious disease.
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