Malignant Fibrous Histiocytoma Of Soft Tissue Research Articles

Ultrasound and MRI features of malignant fibrous histiocytoma of soft tissue

To investigate ultrasound and MRI features of malignant fibrous histiocytoma (MFH) of soft tissue. Ultrasound, MRI images and pathological data of 12 patients with malignant fibrous histiocytoma in soft tissue confirmed by operation and pathology were analyzed from January 2012 to August 2018, inlcuding 7 males and 5 females, aged from 36 to 69 years old with an average age of 53 years old; the courses of disease ranged from 4 to 49 months with an average of 28 months. Clinical manifestations were soft tissue masses and pain in the affected limbs. Ultrasound, MRI and contrast-enhanced examination were performed before operation. The lesions, morphology, echo/signal characteristics, color flow signals and enhancement features were observed and compared with pathology. In 12 patients with MFH, 9 patients were primary lesions and 3 patients were recurrent lesions after operation. There were 7 cases of bilateral thighs, 2 cases of calves, 1 case of upper arm, 1 case of buttocks and 1 case of posterior peritoneum. The size ranged from 5.1 to 17.1 cm with an average of 8.7 cm. Ultrasound feature showed lobulated or agglomerate, and focused on low echo; 5 cases had capsule and with clear border; 7 cases were unclear boundary with surrounding tissues; and 6 cases with irregular echo-free. The blood flow signals were around the CDFI, and the internal blood flow signals were different. MRI feature showed lobulated, agglomerate or irregular shape, T1WI showed slightly lower signal or equal signal, T2WI showed high signal and DWI signal increased. Six patients manifested mixed signal inside, 7 patients manifested low signal separation inside, 5 patients with false envelope, and 9 patients manifested infiltration and growth with peripheral edema. T1WI showed uneven strengthening after enhancement. Immunohistochemical expression of Vim, CD68 were positive. The age, location and imaging features of soft tissue MFH are characteristic. The diagnosis of MFH should be considered when irregular mass occurred in soft tissues of limbs at middle-aged and old people. Echo and signal are homogeneous or mixed. Separation, necrosis and cystic degeneration could be seen in the mass. When the blood flow signals are abundant and solid components are obviously enhanced, the diagnosis of MFH should be considered.

Surgical treatment and prognosis in patients with high-grade soft tissue malignant fibrous histiocytoma of the extremities

Malignant fibrous histiocytoma (MFH) of soft tissue is one of the most common sarcoma in adulthood. However, only a few series have separately studied the clinical behavior and prognosis of this malignancy. We retrospectively reviewed 61 patients treated for extremity soft tissue high-grade MFH. Four patients had a history of another malignancy and were excluded from analysis. In 12 referred patients with incomplete excision, re-excision of the tumor bed was offered. Clinical and treatment variables were analyzed for their impact on treatment complications, local recurrence (LR), metastatic disease (MD) and overall survival (OS). Four patients underwent primary amputation. Twenty-three patients necessitated a primary reconstructive procedure for wound closure. Wound healing complication (WHC) developed in 28.3% of the limb sparing group of patients. LR developed in 11 patients (19.3%), while 6 of them had second LR. Eighteen patients (31.5%) developed MD, involving lung at least. Patients who developed MD <12 vs >12months, died within 19.3 vs 8months mean time (p<0.05). Overall survivorship was 66.7% at 5years. No statistically significant variables were identified for LR, while multivariate analysis for MD revealed tumor size >5cm as the only statistically significant variable. For OS, development of MD and age >70years emerged as independent prognostic factors. The overall prognosis is poor. LR, although can be managed with tumor re-excision, has high second recurrence rate. Increased tumor size is associated with shorter metastasis-free interval which significantly decreases survival.

Experiences with total femur replacement for malignant bone and soft tissue tumors

Background: Reasonable function can be restored after total femur replacement after massive resection of bone and soft tissue sarcomas of the thigh. The type of femoral prosthesis and surgical approach are tailored to the clinical characteristics of individual patients and to tumor anatomy. Though the complication rate is high, total femur replacement offers the patient limb salvage and a chance at functional ambulation. Objective: We described the function and complications of total femur replacement performed at the institution as well as relevant literature reviews. Methods: Seven patients underwent total femur replacement for the treatment of malignant bone and soft tissue tumors of the lower extremities between 1992 and 2010 at our institute. Ages ranged from 12 to 68 (mean=34) years. The tumor was pathologically diagnosed as osteosarcoma in two patients, Ewing’s sarcoma in two, chondrosarcoma (grade 3) in one, soft tissue malignant fibrous histiocytoma in one, and bone metastasis from renal cancer in one. Follow-up periods ranged from 1 to 17 years (mean = six years three months). All patients underwent wide resection, using the Howmedica Modular and Reconstruction System in five cases and the Kyocera Limb Salvage System in two cases. Function, complications and outcomes were evaluated in these patients, and the usefulness of the operative procedures is discussed herein. Results: The mean functional score was 60%. X-ray examination revealed migration in only one case. Complications were infection (n=2), bipolar head dislocation (n=1) and patellar fracture (n=2). The outcomes were DOD (died of disease) in three cases, NED (no evidence of disease) in two, AWD (alive with disease) in one, and CDF (continuous disease free) in one. Conclusion: The results suggest that total femur replacement is useful as a means of reconstructing affected limbs in patients with malignant bone and soft tissue tumors, but that latissimus dorsi muscle transplantation, as well as other procedures, must also be considered in cases requiring extensive soft tissue resection to prevent infection. Furthermore, early one-stage revision is advisable in cases showing signs of infection. Keywords: Femoral prosthesis, functional outcomes, malignant tumors, total femur replacement

Experiences with total femur replacement for malignant bone and soft tissue tumors

Background: Reasonable function can be restored after total femur replacement after massive resection of bone and soft tissue sarcomas of the thigh. The type of femoral prosthesis and surgical approach are tailored to the clinical characteristics of individual patients and to tumor anatomy. Though the complication rate is high, total femur replacement offers the patient limb salvage and a chance at functional ambulation. Objective: We described the function and complications of total femur replacement performed at the institution as well as relevant literature reviews. Methods: Seven patients underwent total femur replacement for the treatment of malignant bone and soft tissue tumors of the lower extremities between 1992 and 2010 at our institute. Ages ranged from 12 to 68 (mean=34) years. The tumor was pathologically diagnosed as osteosarcoma in two patients, Ewing’s sarcoma in two, chondrosarcoma (grade 3) in one, soft tissue malignant fibrous histiocytoma in one, and bone metastasis from renal cancer in one. Follow-up periods ranged from 1 to 17 years (mean = six years three months). All patients underwent wide resection, using the Howmedica Modular and Reconstruction System in five cases and the Kyocera Limb Salvage System in two cases. Function, complications and outcomes were evaluated in these patients, and the usefulness of the operative procedures is discussed herein. Results: The mean functional score was 60%. X-ray examination revealed migration in only one case. Complications were infection (n=2), bipolar head dislocation (n=1) and patellar fracture (n=2). The outcomes were DOD (died of disease) in three cases, NED (no evidence of disease) in two, AWD (alive with disease) in one, and CDF (continuous disease free) in one. Conclusion: The results suggest that total femur replacement is useful as a means of reconstructing affected limbs in patients with malignant bone and soft tissue tumors, but that latissimus dorsi muscle transplantation, as well as other procedures, must also be considered in cases requiring extensive soft tissue resection to prevent infection. Furthermore, early one-stage revision is advisable in cases showing signs of infection. Keywords: Femoral prosthesis, functional outcomes, malignant tumors, total femur replacement

Telomere-Maintenance Mechanisms in Soft-Tissue Malignant Fibrous Histiocytomas

Sarcomas are distinct from carcinomas in that a substantial portion of them use the alternative lengthening of telomeres (ALT) mechanism to maintain their telomeres. The present study clarifies the prevalence of the ALT mechanism and examines the prognostic importance of telomere factors in soft-tissue malignant fibrous histiocytomas. We investigated a series of forty-three soft-tissue malignant fibrous histiocytoma samples from forty-three patients with regard to telomere length, telomerase activity, and human telomerase reverse transcriptase (hTERT) mRNA expression. Tumor samples were obtained from surgical specimens and were stored at -80 degrees C until use. Univariate analysis of the tumor samples from patients for whom data were available on age, sex, histological grade, tumor size, surgical margin, recurrence, and telomere factors was performed with use of the log-rank test. Multivariate analysis with only significant variables was then performed. Telomerase activity was detectable in 79.1% of the tumor samples, hTERT expression was demonstrated in 90.7% of the tumor samples, and evidence of engagement of the ALT mechanism of telomere length maintenance was observed in 32.6% of the tumor samples. Among the variables tested, ALT-positive status emerged as the only independent prognostic factor for death of the patient (hazard ratio, 0.275; 95% confidence interval, 0.104 to 0.724; p=0.0089). There were no significant differences in survival rates between patients with ALT-positive, telomerase-positive tumors and those with ALT-positive, telomerase-negative tumors (p=0.301) or between patients with ALT-positive tumors that showed above-average telomerase activity and those with ALT-positive tumors that showed below-average telomerase activity (p=0.900). Therefore, telomerase activity does not affect the prognosis in patients with ALT-positive malignant fibrous histiocytoma. High telomerase expression is associated with a poor prognosis in patients with ALT-negative malignant fibrous histiocytoma (p=0.0027). More detailed analysis will be needed to identify the most valuable prognostic factor in patients with malignant fibrous histiocytoma, and a more thorough understanding of telomere biology may give an indication of telomere-targeting therapy in the future.

Malignant fibrous histiocytoma of bone: Analysis of genomic imbalances by comparative genomic hybridisation and C-MYC expression by immunohistochemistry

Malignant fibrous histiocytoma (MFH) of bone is a rare, highly malignant tumour. As very little is known about its genetic alterations, 26 bone MFHs were analysed by comparative genomic hybridisation (CGH). Twenty-three tumours (89%) had DNA sequence copy number changes (mean, 7.2 changes per sample). Gains were more frequent than losses (gains:losses = 2.5:1). Minimal common regions for the most frequent gains were 8q21.3-qter (35%), 9q32-qter (35%), 7q22-q31 (35%), 1q21-q23 (31%), 7p12-pter (31%), 7cen-q11.2 (31%) and 15q21 (31%). Minimal common regions for the most frequent losses were 13q21-q22 (42%) and 18q12-q22 (27%). High-level amplifications were detected in 8 out of the 26 tumours (31%). The only recurrent amplifications, 1q21-q23 and 8q21.2-q22, were present in two samples (8%). As copy number increase at 8q24 (the locus of C- MYC) was frequent, the expression of C- MYC was studied by immunohistochemistry. Increased levels of c-myc protein were detected in 7 out of 21 tumours studied (33%). 81% of the samples studied both by CGH and immunohistochemistry showed concordant results. Furthermore, the findings of the present study were compared to previous publications on osteosarcoma, soft tissue MFH and fibrosarcoma of bone. Clear differences were detected in CGH aberration patterns, further supporting the concept of bone MFH as an individual bone tumour entity. Finally, the findings of the present study reflect well the high malignancy and aggressive nature of bone MFH.

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroy’s disease

Malignant fibrous histiocytoma, now termed high-grade undifferentiated pleomorphic sarcoma, is a term used to refer to a group of malignant soft-tissue tumors characterized by a storiform or cartwheel-like growth pattern [1–4]. This lesion is the most common malignant soft-tissue tumor of adult life, representing approximately 25% of all soft tissue sarcomas [5]. The undifferentiated pleomorphic sarcoma will demonstrate a wide range of histological appearances, and is often further subclassified, although in general large and deep lesions have a poorer prognosis [1]. Desmoid tumors are benign, locally aggressive, softtissue tumors that typically arise in association with fascia, aponeurosis and associated muscle. Often termed musculoaponeurotic fibromatosis, these non-metastasizing lesions show an infiltrative pattern of growth and a significant predilection for local recurrence [6, 7]. We report the extremely unusual association of an undifferentiated pleomorphic sarcoma arising within a desmoid tumor in a patient with Milroy’s disease (chronic hereditary lymphedema of unknown origin). To our knowledge, there have been no similar previous reports. Case report

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroys disease

Pleomorphic Malignant Fibrous Histiocytoma at the Site of an Arthroscopic Reconstruction of the Anterior Cruciate Ligament

P reviously regarded as a distinct tumor type representing the most common adult soft-tissue sarcoma1,2, the term malignant fibrous histiocytoma is now reserved for a small group of undifferentiated pleomorphic sarcomas3. The tumor is most often primary, but rare cases of secondary malignant fibrous histiocytoma in burn scars4-8 or infected or noninfected surgical scars9-12 have been reported (see Appendix). Secondary malignant fibrous histiocytoma has been described in contact with prostheses13-28, osteosynthesis material29-35, or implanted Dacron grafts36. Malignant degeneration of benign tumors35, Paget disease37, osseous infarcts38, or fibrous dysplasia39 also has been reported (see Appendix). We report a unique case of a patient with soft-tissue malignant fibrous histiocytoma that developed on the medial side of the knee six years after arthroscopic reconstruction of the anterior cruciate ligament with use of the patellar tendon. D uring a soccer game in June 1993, a nineteen-year-old man experienced an indirect injury to the right knee that caused a rupture of the anterior cruciate ligament. A magnetic resonance imaging scan confirmed the diagnosis. An arthroscopic reconstruction of the anterior cruciate ligament was carried out in October 1993 with use of the patellar tendon. Stainless-steel (alloy-316L) screws (with a diameter of 6.5 mm) were used for the femoral and tibial fixation. The rest of the intra-articular examination was normal, and the postoperative course was uneventful. In 1995, a hemarthrosis developed in the right knee subsequent to a new injury. The knee was stable, and the hemarthrosis resolved with symptomatic treatment. In 1997, the patient experienced a locking episode of the right knee, which was found to be caused by a bucket-handle tear of the medial meniscus at …

Frequent expression of smooth muscle markers in malignant fibrous histiocytoma of bone

Background/Aims: Malignant fibrous histiocytoma (MFH) of bone, a relatively rare primary malignant bone tumour, is a distinct clinicopathological entity as opposed to MFH derived from soft tissue. Although the true...

Clinical implications of serum C-reactive protein levels in malignant fibrous histiocytoma.

Paraneoplastic syndromes (PNSs) associated with mesenchymal tumors are uncommon. Previous reports sporadically described inflammatory PNSs with elevated serum C-reactive protein (CRP) levels and leukocytosis in patients with inflammatory malignant fibrous histiocytoma (MFH) of soft tissue; however, the relationship between other subtypes of MFH and PNS has not been extensively investigated. Forty-six patients with primary MFH of soft tissues who underwent radical surgery were retrospectively analyzed. These patients were divided into 2 groups according to preoperative serum CRP level: normal (<1.0 mg/dl) and elevated (> or = 1.0 mg/dl). The correlation between serum CRP level and several clinicopathologic factors was analyzed. Correlation between preoperative serum CRP level and metastasis-free and overall survival was also investigated by univariate and multivariate analyses. Elevated preoperative serum CRP levels were found in 65% of patients with a mean of 3.7 mg/dl. There were statistically significant relationships regarding tumor size, depth, histologic subtypes, grade, stage and metastatic rate among normal and elevated CRP groups (p < 0.001, p < 0.02, p < 0.005, p < 0.001, p < 0.001 and p < 0.05, respectively). When the tumor was removed, the elevated CRP level subsided into the normal range and other abnormal laboratory findings diminished in all cases. In 11/14 relapsed cases that showed elevated CRP preoperatively, the serum CRP level re-elevated with tumor relapse. The normal CRP group showed significantly more favorable prognosis than the elevated CRP group in metastasis-free and overall survival on univariate analysis (p < 0.02, p < 0.05, respectively). Patients with MFH frequently present with an inflammatory PNS, such as elevated serum CRP level, which can be a useful marker of disease activity and a valuable prognostic indicator.

Proliferating cell nuclear antigen staining as a prognostic indicator in soft-tissue malignant fibrous histiocytoma.

To determine the value of proliferating cell nuclear antigen (PCNA) staining as a prognostic indicator in soft-tissue malignant fibrous histiocytoma (MFH), we studied 27 patients who underwent resection including an adequately wide margin but without chemotherapy. The survival rate of patients with a lesion in which less than 70% of the cells was positively stained was significantly higher (P < 0.01) than of those with more than 70% positive staining. Twenty-two patients (81.5%) evidenced disease-free survival (mean follow-up period 4.6 years), 1 patient was alive with disease, and 4 (14.8%) died of the disease with lung or lymph node metastasis. In all 4 patients who died, PCNA staining was over 70% positive. In 2 of them, an increase in the number of PCNA-positive cells was observed after repeated recurrence. We conclude that PCNA is a useful prognostic indicator which provides a quantitative measure of the grade of malignancy in MFH patients who receive operative treatment without chemotherapy.

Malignant Fibrous Histiocytoma of Bone and Soft Tissue – Two Different Tumor Entities? A Retrospective Study of 45 Cases

<i>Background</i>: Malignant fibrous histiocytoma (MFH) can arise as primary bone or soft-tissue sarcoma. Primary MFH of bone and soft tissue are histopathologically indistinguishable. The aim of the study was to assess the clinical course and oncological outcome of MFH of bone and soft tissue. <i>Methods</i>: 45 patients with a confirmed diagnosis of MFH (25 primary bone MFH, 20 primary soft-tissue MFH) were seen between 1974 and 1993 in our institution. Patient records, pathology, and operation reports were assessed for staging and oncological outcome with respect to differences in clinical behavior and response to chemo- and radiotherapy. Follow-up was more than 36 months in 33 patients. <i>Results</i>: MFH of bone metastasizes more frequently and earlier and responds better to chemotherapy than MFH of soft tissue. Soft-tissue MFH has a higher local recurrence rate, and local control can only be achieved by adequate resection margins. 3-year tumor-free survival in our series is 21 % for bone and 57% for soft-tissue MFH. <i>Conclusion</i>: With respect to clinical behavior MFH of bone and MFH of soft tissue can be considered as 2 different tumorentities. The reason forthis remains unclear. In the authors opinion MFH of bone should be treated with chemotherapy and surgery.

From the archives of the AFIP. Musculoskeletal malignant fibrous histiocytoma: radiologic-pathologic correlation.

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma, occurring most frequently in the deep soft tissues of the extremities. Primary osseous MFH is less common. MFH is the most common soft-tissue sarcoma of late adult life. Although its imaging appearance is often nonspecific, any deep-seated invasive intramuscular mass in a patient over 50 years of age is most likely an MFH. Cortical involvement by soft-tissue MFH is common, and identification of this finding increases the likelihood of MFH. Prominent fluid components with peripheral nodular enhancement after contrast material administration and lack of adipose elements are suggestive of a specific histologic subtype, myxoid MFH. Osseous MFH shows aggressive bone destruction with cortical involvement and a soft-tissue mass and is located in the diaphysis or metaepiphysis. Lesions in the metaepiphysis may have a less aggressive appearance and do not extend to subchondral bone. Computed tomography and magnetic resonance imaging are vital for preoperative staging and surgical planning and in detecting early recurrence postoperatively.

Implanted solid human tumours grow under the renal capsule of cyclosporin-immunosuppressed rats.

Cyclosporin (CsA) is a potent immunosuppressive drug widely used in organ transplantation. We transplanted fresh surgical samples from human solid malignant tumours into 45 CsA-immunosuppressed rats. Eight out of nine tumour types grew and remained viable for 5 weeks or more in at least two of the transplanted rats. In 29 rats (64%) a distinct growth of primary human tumours was recorded. Five malignancies (intestinal-type gastric carcinoma, adenocarcinoma of the lung, lymph node metastasis of a testicular teratocarcinoma, soft tissue malignant fibrous histiocytoma (MFH), and small-cell sarcoma) showed invasive and progressive growth. In all five cases the largest tumours were 0.9 cm or over in diameter when the rats were killed 5-9 weeks after transplantation. In three cases (adenocarcinoma of the colon, hypernephroma, and a second MFH) the growth of the implants under the kidney capsule was slow, but small living tumour transplants were still found 3-6 weeks later. In every case the microscopic morphology of the xenograft tumour was identical with the original tumour. In two cases the primary xenografts (teratocarcinoma and small-cell sarcoma) were retransplanted into 11 CsA-immunosuppressed rats. In both types the second passage tumours grew, and the take-off and growth rates were comparable to the primary xenografts. Cyclosporin-treated laboratory rats are an alternative to immunodeficient nude and SCID mice for growing fresh human tumour transplants in vivo. Although a few infections were encountered, most of the rats survived the CsA treatment well for up to 2 months.

Soft tissue malignant fibrous histiocytoma in a finger

W e treated a patient with a malignant fibrous histiocytoma (MFH) of the index finger by radical ray resection followed by external-beam radiotherapy and chemotherapy. This is a rare lesion. Two cases have been reported in the finger,‘, ’ six in the hand,3-8 and two in the wrist.6 Multimodality protocols for treatment of soft tissue sarcoma (STS) were introduced in the 1970~~ and confirmed in the 198Os,” with improvement in survival rates. One case of MFH in the palm of the hand has been treated with multimodality treatment with a 4-month survival,3 but results of multimodality treatment in the digits have not been described.

Malignant fibrous histiocytoma. A tumor of facultative histiocytes showing mesenchymal differentiation in cultured cell lines

The histogenesis of malignant fibrous histiocytoma (MFH) is controversial. To elucidate the cellular origin and characteristics of this neoplasm, the authors analyzed cell lines grown from 17 patients (15 soft tissue MFH and 2 bone MFH) by using light and electron microscopy, immunocytochemistry, enzyme cytochemistry, and functional tests for receptors for the Fc portion of immunoglobulin (Fc receptors) and immunophagocytosis. Each culture exhibited a storiform/pleomorphic pattern with mixed cellular populations consisting of spindle cells, polygonal cells, and bizarre giant cells; these morphologic features corresponded to the histologic characteristics of the primary tumors. The cells in each MFH line displayed histiocytic functional markers such as lysosomal enzymes, Fc receptors and immunophagocytosis. However, these cells differed from monocyte-derived macrophages (histiocytes) in immunoreactivity; the MFH cells expressed a mesenchymal antigen (FU3) distributed among perivascular cells and fibroblasts but demonstrated no positive reactions with Leu-M1 (CD15) and Leu-M3 (CD14), which recognize the cells of the monocyte-macrophage lineage. In conclusion, these findings suggest that MFH is not a tumor of true histiocytes but of facultative histiocytes showing mesenchymal differentiation in vitro. Chromosomal analysis performed in one MFH line demonstrated abnormal karyotypes; the modal chromosome number was 58, with 5 marker chromosomes.