Malignant Fibrous Histiocytoma Patients Research Articles

Oral Health and Molecular Aspects of Malignant Fibrous Histiocytoma Patients: A Systematic Review of the Literature.

Malignant fibrous histiocytoma is one of the most common soft tissue sarcomas in adults. It occurs only occasionally in oral soft tissues, and knowledge about its characteristics is based on a limited number of cases reported in the literature. Malignant fibrous histiocytoma belongs to the group of soft tissue sarcomas and makes up less than 10% of soft tissue sarcomas. For therapeutic purposes, complete exeresis of the lesion (macroscopic and microscopic) is performed because they have frequent recurrences. As for complementary therapy in addition to surgery, neither radiotherapy nor chemotherapy have been shown to reduce the risk of death related to the disease. Often patients complain of a swelling that grows in a short period of time. It is quite common for patients to report trauma in the area, which is not the cause, but rather the event that allows diagnosis. The mass usually does not cause pain unless it compresses an adjacent nerve structure. The aim of this study is to systematically review the scientific literature in order to identify the most recent studies concerning malignant fibrous histiocytomas localized in oral soft tissues and report their main data. The main outcomes of this study concern the immunohistochemical, molecular, and clinical aspects of this pathology. A systematic review of articles in the electronic databases pubmed, Scopus, and Web of Science was performed. After the selection process, 11 studies met the inclusion criteria and were included in the review. The mean age of the patients was 50.8 years old. The lesions affected various parts of the oral cavity, showing predominantly storiform–pleomorphic patterns. All cases except one were treated with surgical resection and radiation therapy. Although some data emerged from this review, they remain limited to a few case reports. Further studies are necessary in order to standardize the approach to patients affected by oral malignant fibrous histiocytoma (MFH).

Single nucleotide polymorphisms in the D-loop region predicts earlyage-at-onset of malignant fibrous histiocytoma

The accumulation of single nucleotide polymorphisms (SNPs) in the displacement loop (D-loop) of mitochondrial DNA (mtDNA) has been described in many cancers. In our previous studies, we have identified cancer risk and outcome associated SNPs in the D-loop of malignant fibrous histiocytoma (MFH). In the present study, we investigated the SNPs in the D-loop of mitochondria DNA (mtDNA) for their association with age-at-onset, metastasis, and relapse using a population-based series of MFH patients. Our data showed that the SNP site of nucleotide 16304 T/C was associated with age-at-onset using the Kaplan–Meier method and compared by the log-rank test. Meanwhile, allele 152C genotype was identified to be significantly associated with a high metastasis rate in MFH patients. In addition, patients with allele 16266C were proven to have a higher relapse rate compared with 16266T at a statistically significant level. Finally, we performed a multivariate analysis with the Cox proportional hazards model. The age-at-onset of patients with the minor allele C genotype was significantly earlier than that of patients with the T genotype at the 16304 site. In summary, the analysis of genetic polymorphisms in the D-loop may help to identify patient subgroups at a high risk of early onset, high metastasis and relapse rate in MFH, thereby helping to refine therapeutic decisions.

Clinical Features and Prognostic Factors of Primary Intracranial Malignant Fibrous Histiocytoma: A Report of 8 Cases and a Literature Review

Objective Primary intracranial malignant fibrous histiocytoma (MFH) is rare. We describe the detailed clinical features of 8 cases and fully review the literature to evaluate several prognostic factors. Methods Eight patients with pathologically confirmed primary intracranial MFH were retrospectively reviewed. We searched PubMed for relevant articles with the term “intracranial malignant fibrous histiocytoma”. Results Of the 8 patients, 4 were men and 4 were women. Three patients had received previous radiotherapy. The age of the patients ranged from 19 to 69 years, with a median age of 48 years. Most tumors could be totally resected; and only 1 tumor was subtotally resected. Six patients received postoperative radiotherapy and 3 patients received postoperative chemotherapy. Most patients died within the first year after surgery; and only 1 patient was still alive on the date of the last follow-up. We reviewed the literature and included a total of 46 patients in the Kaplan-Meier survival analysis. Young patients (less than 30 years old) seemed to have a better prognosis and survival rate than older patients (more than 30 years old) (log-rank test, P = 0.008). However, sex (P = 0.675), extent of resection (P = 0.934), postoperative radiotherapy (P = 0.592), and postoperative chemotherapy (P = 0.424) did not affect patient prognosis. Conclusions The prognosis of MFH is usually poor, and most patients die within the first year after surgery. Younger MFH patients (less than 30 years old) seem to have a better prognosis and improved survival compared to older patients.

Single nucleotide polymorphisms in the mitochondrial displacement loop region and outcome of malignant fibrous histiocytoma

Purpose: Single nucleotide polymorphisms (SNPs) in the mitochondrial DNA displacement-loop (D-loop) region have been reported to be associated with cancer risk and disease outcome in several types of cancer. In this study, we investigated whether the SNPs in mitochondrial D-loop were associated with the outcome of malignant fibrous histiocytoma (MFH). Experimental design: The D-loop region of mtDNA was sequenced for 80 MFH patients. The 3 years survival curve were calculated with the Kaplan–Meier method and compared by the log-rank test at each SNP site, a multivariate survival analysis was also performed with the Cox proportional hazards method. Results: The SNP sites of nucleotides 152T/C, 16,390G/A, 16,290C/T, 16,304T/C and the AC deletion at sites 523 and 524 were identified for prediction of post-operational survival by the log-rank test. In an overall multivariate analysis, the 16,290 and 16,390 alleles were identified as independent predictors of MFH outcome. The length of survival for patients with the rare allele 16,390A genotype was significantly shorter than that for patients with the frequent allele 16,390Gat the site 16,390. The same was seen for the rare allele 16,290T genotype when compared with matched allele 16,290C at the site 16,290 in MFH patients. Conclusions: These results suggested that SNPs in the D-loop are independent prognostic markers for patients with MFH. The analysis of genetic polymorphisms in the D-loop can help identify patient subgroups at higher risk of a poor disease outcome.

Identification of sequence polymorphisms in the D-loop region of mitochondrial DNA as risk biomarkers for malignant fibrous histiocytoma

Single nucleotide polymorphisms (SNPs) in the mitochondrial DNA Displacement-loop (D-loop) region particularly in a highly polymorphic homopolymeric C stretch named D310 have been reported to be associated with cancer risk in several types of cancer. In order to evaluate the frequency of D-loop SNPs in a large series of malignant fibrous histiocytoma (MFH) and establish correlations with cancer risk, we sequenced the D-loop of 92 MFH patients and analyzed their use as predictive biomarkers for MFH risk. The minor alleles of nucleotides 73G, 151T were associated with an increased risk for MFH patients, whereas the alleles of nucleotides 16,298C, 152C, and insertion of C at the site 315 (located within the D310) were associated with a decreased risk for MFH patients. These results suggest that SNPs in the mitochondrial D-loop should be considered as a biomarker which may be useful for the early detection of MFH in individuals at risk of this cancer.

Do Radiation-Associated Soft Tissue Sarcomas Have the Same Prognosis As Sporadic Soft Tissue Sarcomas?

PURPOSE To determine the prognostic significance of histologic type in radiation-associated soft tissue sarcomas (RASs) and determine whether RASs are associated with an inferior prognosis compared with sporadic soft tissue sarcomas (STSs). PATIENTS AND METHODS One hundred thirty primary RASs were identified from 7,649 STS patients from 1982 to 2007. Multivariate analysis of clinicopathologic factors for disease-specific survival (DSS) was performed for RASs, and a multivariate analysis of radiation exposure was also performed for RASs and sporadic sarcomas. A matched-cohort analysis was performed for radiation-associated and sporadic malignant fibrous histiocytoma (MFH). Results Most RASs were high grade (83%), deep (87%), and truncal (61.5%). The median interval between radiation therapy and RAS development was 10 years (range, 1.3 to 74 years), which varied significantly by histologic type (P = .003). The 5-year DSS was 58%, and independent predictors were size > 5 cm, margin positivity, and histologic type. Multivariate analysis of histologic types of primary, high-grade radiation-associated and sporadic STSs showed that RAS was associated with a worse DSS (hazard ratio, 1.7; range, 1.1 to 2.4; P = .007). For pleomorphic MFH-the most common RAS type-the 5-year DSS was 44% versus 66% in a matched cohort of sporadic MFH patients (P = .07). DSS was significantly worse in primary RAS malignant peripheral nerve sheath tumors (MPNSTs) compared with unmatched sporadic MPNSTs (P = .001). CONCLUSION Histologic type, margin status, and tumor size are the most important independent predictors of DSS in patients with RASs. DSS in patients with primary RAS is significantly worse compared with sporadic STS independent of sarcoma histologic type.

Thallium-201 chloride scintigraphy in soft tissue tumors

We report the findings of Thallium-201 chloride ((201)TlCl) scintigraphy and consider how to use this technique to evaluate the character of soft tissue lesions. We enrolled 91 consecutive patients (45 males and 46 females, age range 8-91-years-old). Nineteen patients were malignant and 72 were benign. Patients were scanned 15 minutes (early phase) and 3 hours (delayed phase) after (201)TlCl injection. More intense uptake in the lesion compared to the normal side was considered as 'high', the same degree of uptake was considered 'iso', and decreased uptake was 'low'. The retention index (RI) was calculated in 9 patients in the malignant group and in 16 patients in the benign group. In malignant tumors, 15 of 19 patients showed high uptake in both the early and delayed phases. One malignant fibrous histiocytoma patient was high only in the delayed phase and 1 liposarcoma patient was high only in the early phase. Two liposarcoma patients showed an iso uptake in both phases. One of these patients was pathologically diagnosed as a myxoid type. In benign lesions, no lipoma showed increased uptake. All neurogenic tumors except for 2 demonstrated high uptake. All 3 ganglions of the lower extremities showed iso uptake. Most inflammatory diseases showed increased uptake. Clinically-considered benign patients consisted of tumorous lesions or inflammatory disease. Only 2 patients were considered 'low', and these were diagnosed as intramuscular hematoma and cyst. RI was variable in both malignant and benign lesions and no statistically significant difference was seen between malignant and benign lesions by t-test (p=0.72). A high (201)TlCl uptake lesion is more frequently seen in malignant tumors, but regardless of whether the tumor is benign or malignant, according to the histopathological variety, the (201)TlCl uptake pattern can not be the only indicator to differentiate malignant from benign tumors. We ultimately need to evaluate the nature of tumors by a combination of several imaging techniques.

Immunohistochemical expression of promyelocytic leukemia body in soft tissue sarcomas

BackgroundThe function of promyelocytic leukemia (PML) bodies is not well known but plays an important role in controlling cell proliferation, apoptosis and senescence. This study was undertaken to analyze the clinical significance of PML body expression in primary tumor samples from malignant fibrous histiocytoma (MFH) and liposarcoma patients.MethodsWe studied MFH and liposarcoma samples from 55 patients for PML bodies. Fluorescent immunostaining of PML bodies was performed in the paraffin-embedded tumor sections.ResultsPML body immunostaining was identified in 63.9% of MFH and 63.2% of liposarcoma samples. PML body expression rates of all sarcoma cells were 1.5 ± 1.8% (range: 0–7.0) in MFH and 1.3 ± 1.4% (0–5.2) in liposarcoma samples. PML body expression (p = 0.0053) and a high rate of PML body expression (p = 0.0012) were significantly greater prognostic risk factors for death than the other clinical factors in MFH patients. All liposarcoma patients without expression of PML were disease free at the end of the study.ConclusionOur study suggests that the presence of PML bodies may indicate a poor prognosis for MFH and liposarcoma patients.

Retroperitoneal malignant fibrous histiocytoma mimicking hepatocellular carcinoma

Authors review the case history and follow-up of a rare malignant fibrous histiocytoma patient, based on the relevant literary data. A palpatable abdominal mass was found in a 40-year-old man. Ultrasonography and magnetic resonance imaging revealed a 13 cm × 11 cm sized mass, suspect of being hepatocellular carcinoma in the right hepatic lobe. The tumor was resected through a transabdominal approach, but the origin of the mass was the retroperitoneam. The resected specimen measured 22 cm × 18 cm × 15 cm, and weighed 1800 g. The histologic examination showed that the lesion was malignant fibrous histiocytoma. Authors regard their case worthy of publication because of the retroperitoneal location and significant size of the tumor, and because of the unproven diagnosis prior to surgery. Even after 5 years the patient is symptom- and complaint-free, and CT has revealed no local recurrence.

INOS‐dependent DNA damage in patients with malignant fibrous histiocytoma in relation to prognosis

Malignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas. MFH has been proposed to be a lesion accompanied with inflammatory responses. During chronic inflammation, reactive nitrogen and oxygen species generated from inflammatory cells are considered to participate in carcinogenesis by causing DNA damage. 8-nitroguanine is a mutagenic nitrative DNA lesion formed during chronic inflammation. We examined whether nitrative DNA damage is related to the prognosis of MFH patients. We performed immunohistochemical analyses to examine the distribution of DNA damage and the expression of inflammation-related molecules including inducible nitric oxide synthase (iNOS), nuclear factor-kappaB (NF-kappaB), and cyclooxygenase-2 (COX-2) in clinical specimens from 25 patients with MFH. We also analyzed the correlation of DNA damage or the expression of these genes with the prognosis of MFH patients. Immunohistochemical staining revealed that the formation of 8-nitroguanine and 8-oxo-7,8-dihydro-2'-deoxyguanosine (8-oxodG), an oxidative DNA lesion, occurred to a much greater extent in MFH tissue specimens from deceased patients than in live patients. iNOS, NF-kappaB and COX-2 were colocalized with 8-nitroguanine in MFH tissues. It is noteworthy that the statistical analysis using the Kaplan-Meier method demonstrated strong 8-nitroguanine staining to be associated with a poor prognosis. In conclusion, 8-nitroguanine appears to participate in not only the initiation and promotion of MFH, but also in the progression of MFH, and could therefore be used as a promising biomarker to evaluate the prognosis of cancer patients.

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.

Postradiation sarcomas of bone.

Fifty-nine patients with osteogenic sarcomas arising in bones following exposure to x rays and 20 patients with postradiation malignant fibrous histiocytomas of bone arising as a direct consequence of irradiation were studied. These represent 5.5% of all osteogenic sarcomas and 4.9% of all malignant fibrous histiocytomas of bones. The sarcomas may affect any skeletal site, but most commonly they arose in bones of the pelvic and shoulder girdles or the distal end of the femur. Grounds for irradiation were either nonosseous conditions or preexistent skeletal lesions. Reasons for incidental osseous irradiation included Hodgkin's disease, carcinoma of cervix, breast or lung; bilateral retinoblastoma and others, and giant cell tumor predominated among the irradiated skeletal lesions. The mean and the median radiation doses were 6,040 cGy (rad) and 5,700 cGy (rad), respectively. The period of latency between irradiation and the appearance of the bone sarcoma ranged from 3.5 to 47 y with a mean of 16.5 and median of 14.5, respectively. The cumulative disease-free survival rate for malignant fibrous histiocytoma patients at 3 y was 58%. The cumulative disease-free survival rate at 5 y for patients with osteogenic sarcoma was 17%, with a median survival estimate of 1 y. Although all patients with malignant fibrous histiocytoma who received their radiation therapy for a preexistent bone lesion survived, only 27% of the patients whose bone was normal at the time of irradiation are alive and well at the 3-y mark.

Lectin Histochemistry of Malignant Fibrohistiocytic Tumors

The staining pattern by Ricinus communis agglutinin (RCA), a lectin used as a good marker for histiocytes, in 24 cases with malignant fibrous histiocytoma (MFH) was studied and compared with that of 12 cases of fibrosarcoma (FS). In 20 (83%) of 24 cases of MFH, varying degrees of RCA binding were observed, whereas only four (33%) of 12 cases of FS were positive. RCA-positive FS included three cases with infantile FS and one adult case with post-radiation FS. Eight adult patients with FS were entirely negative. This positivity rate of RCA binding in MFH was much higher than those of antisera against lysozyme, alpha-1-antitrypsin, and alpha-1-antichymotrypsin previously reported. Seven MFH patients with focal aggregation of RCA-positive benign-appearing (reactive) histiocytes died earlier than the other patients with only scattered RCA-positive histiocytes; 5-year survival rates were 32% and 69%, respectively (p less than 0.05). These findings suggest that RCA reactivity can be used as a potential diagnostic and prognostic marker for MFH.

Osteosarcoma: intra-arterial treatment of the primary tumor with cis-diammine-dichloroplatinum II (CDP). Angiographic, pathologic, and pharmacologic studies.

Intra-arterial CDP was utilized to treat the primary tumor in 11 pediatric patients with osteosarcoma and in one with malignant fibrous histiocytoma. The investigation commenced with a phase I-II pilot study in four osteosarcoma patients. A dose of 150 mg/m2 was found to be safe and effective in producing a clinical response. This was followed by a definitive study in the remaining seven osteosarcoma patients and in the one malignant fibrous histiocytoma patient. The results were assessed by specific clinical, pharmacologic, radiographic and pathologic criteria. The overall response in the definitive study was 50% with two patients exhibiting total tumor destruction. The success of intra-arterial CDP was attributed to its ability to achieve high local drug concentration and tumor penetration. This was demonstrated by pharmacologic studies.