Malignant fibrous histiocytoma of bone

Malignant fibrous histiocytoma (MFH) of bone is a malignant primary bone tumour that is being increasingly recognised, as more details emerge in the literature regarding its natural history and precise (although sometimes diverse) histological appearance. When the whole bulk of a malignant bone tumour fits the criteria laid down, the designation of MFH seems appropriate. Seven cases of MFH of bone were encountered from a total of 220 primary malignant bone tumours in our files over a 4-year period. The metaphyses of long bones were the most common sites harbouring the tumour, and a wide age range was represented. Amputation was the treatment of choice in all cases. The relevant literature is reviewed.     

Chromosomal imbalances in familial chronic lymphocytic leukaemia: a comparative genomic hybridisation analysis.

A subset of B cell chronic lymphocytic leukaemia (CLL) is familial. Lack of large families makes it attractive to exploit methods in addition to genetic linkage analysis for the identification of a susceptibility locus. One strategy that can localise regions of the genome that may harbour tumour suppressor genes is to identify regions of chromosomal imbalance using comparative genomic hybridisation (CGH) analysis. We examined 24 familial CLL cases by CGH analysis. Losses that are documented as arising frequently in sporadic CLL were observed at a comparable frequency in familial CLL. However, gains and losses in two regions of the X chromosome - Xp11.2-p21 and Xq21-qter - appear more common in familial CLL than in sporadic CLL. This suggests these regions may harbour a susceptibility locus for CLL. There is also some evidence that chromosome regions 2p12-p14 and 4q11-q21 may harbour predisposition genes.     

Malignant fibrous histiocytoma (malignant fibrous xanthoma: xanthosarcoma) of bone.

Seven cases of malignant fibrous histiocytoma of bone are documented. In addition to typical histologic features such as the presence of a storiform pattern, histiocytic and fibrocytic cell types and severe cellular atypism, in five cases there were areas in which osteoclast-type giant cells were prominent and the lesion resembled a giant cell tumor. Three of the seven patients developed pulmonary metastases within two years of amputation, one developed extensive local recurrence including soft tissue and vascular invasion, and the other three are without evidence of disease but have been followed for only short periods.     

Malignant fibrous histiocytoma: an analysis of 200 cases.

The clinicopathologic findings in 200 cases of malignant fibrous histiocytoma (MFH) with follow-up information are presented. This tumor occurred principally as a mass on an extremity (lower extremity 49%, upper extremity 19%) or in the abdominal cavity or retroperitoneum (16%) of adults (peak incidence 61-70 years of age). It typically involved deep fascia (19%) or skeletal muscle (59%) and only rarely was confined to the subcutis without fascial involvement (7%). The MFH had variable morphologic features and frequently showed transitions from areas having a highly ordered storiform pattern to less differentiated areas having a pleomorphic appearance. The rate of local recurrence of the tumor was 44%, and of metastasis, 42%. Metastasis was most frequently to the lung (82%) and lymph nodes (32%). Factors that influenced the rate of metastasis included depth, size, and inflammatory component of the tumor. Tumors that were small, superficially located, or had a prominent inflammatory component metastasized less frequently than larger, more deeply located tumors. In our experience the MFH is the most common soft tissue sarcoma of late adult life, and many tumors previously diagnosed as pleomorphic variants of liposarcoma, fibrosarcoma, or rhabdomyosarcoma are probably examples of MFH. Although the histogenesis of this neoplasm remains controversial, we feel it is best regarded as a primitive and pleomorphic sarcoma showing partial fibroblastic and histiocytic differentiation, as reflected by collagen production and occasional phagocytosis.     

Malignant fibrous histiocytoma of bone - Review of thirteen cases

Malignant fibrous histiocytoma of bone (MFHB) constitutes 0.5-8% of all malignant primary bone tumours. It has a poor prognosis when treated with surgery alone. Chemosensitivity of this tumour entity with increased disease-free survival rates has been reported. We reviewed the treatment of patients with MFHB at Hannover University Medical School since 1982. Thirteen patients were diagnosed with MFHB, nine had localized and four metastatic disease. All patients have been treated with surgery, except two patients who received radiation treatment. Additionally, eleven patients underwent different chemotherapy regimens mainly including anthracyclines in neoadjuvant (n=5) or palliative (n=6) settings. At a median follow up of 35 months (range, 14-134+) six patients (46%) are alive and four of these patients have no evidence of disease (31%). This report confirms the chemosensivity of MFHB and neoadjuvant treatment approaches may be warranted in these patients.     

Malignant fibrous histiocytoma: an institutional review

Background: A thorough understanding of malignant fibrous histiocytoma (MFH), the most common subtype of soft tissue sarcoma, will lead to improved histologic-specific protocols. Methods: 126 patients with histologically confirmed MFH were analyzed. The median follow-up was 42 months (range 1-233 months). Results: Overall survival was 58% at 5 years and 38% at 10 years. Grade significantly influenced prognosis, with 10-year survival of 90%, 60%, and 20% for low, intermediate, and high grade tumors, respectively (p = 0.0007). Distant metastases at initial presentation (p = 0.0002) and size of the primary tumor (p = 0.0007) influenced outcome. Neither anatomic site nor depth of the primary tumor were significant prognostic factors. Positive microscopic margins were associated with a decreased disease-free survival (p = 0.006). Conclusions: Tumor grade, size, and distant metastases at initial presentation remain the most important prognostic factors for MFH. Resection with negative microscopic margins decreased the incidence of local recurrence.     

Microarray comparative genomic hybridisation analysis of intraocular uveal melanomas identifies distinctive imbalances associated with loss of chromosome 3

Defining regions of genomic imbalance can identify genes involved in tumour development. Conventional cytogenetics has identified several nonrandom copy number alterations (CNA) in uveal melanomas (UVM), which include monosomy 3, chromosome 6 abnormalities and gain of 8q. To gain further insight into the CNAs and define the regions involved more precisely we analysed 18 primary UVMs using 1 Mb BAC microarray comparative genomic hybridisation (CGH). Our analysis showed that the most common genomic imbalances were 8q gain (78%), 6p gain (67%) and monosomy 3 (56%). Two distinct CGH profiles could be delineated on the basis of the chromosome 3 status. The most common genetic changes in monosomy 3 tumours, in our study, were gain of 8q11.21-q24.3, 6p25.1-p21.2, 21q21.2-q21.3 and 21q22.13-q22.3 and loss of 1p36.33-p34.3, 1p31.1-p21.2, 6q16.2-q25.3 and 8p23.3-p11.23. In contrast, disomy 3 tumours showed recurrent gains of only 6p25.3-p22.3 and 8q23.2-q24.3. Our approach allowed definition of the smallest overlapping regions of imbalance, which may be important in the development of UVM.     

Frequent deletion of the CDKN2A locus in chordoma: analysis of chromosomal imbalances using array comparative genomic hybridisation

The initiating somatic genetic events in chordoma development have not yet been identified. Most cytogenetically investigated chordomas have displayed near-diploid or moderately hypodiploid karyotypes, with several numerical and structural rearrangements. However, no consistent structural chromosome aberration has been reported. This is the first array-based study characterising DNA copy number changes in chordoma. Array comparative genomic hybridisation (aCGH) identified copy number alterations in all samples and imbalances affecting 5 or more out of the 21 investigated tumours were seen on all chromosomes. In general, deletions were more common than gains and no high-level amplification was found, supporting previous findings of primarily losses of large chromosomal regions as an important mechanism in chordoma development. Although small imbalances were commonly found, the vast majority of these were detected in single cases; no small deletion affecting all tumours could be discerned. However, the CDKN2A and CDKN2B loci in 9p21 were homo- or heterozygously lost in 70% of the tumours, a finding corroborated by fluorescence in situ hybridisation, suggesting that inactivation of these genes constitute an important step in chordoma development.     

Malignant fibrous histiocytoma of the lung

Primary malignant fibrous histiocytoma (MFH) of the lung is very rare. To date, only 32 reports of 63 cases of primary MFH of the lung have appeared in English, excluding tumors arising from the pulmonary arteries and pleura. We describe a patient with primary MFH of the lung who developed brain metastasis and involvement of pulmonary great vessels. In addition, we reviewed previously reported cases to establish the clinical characteristics and most appropriate management of primary pulmonary MFH. When disease is sufficiently limited, complete resection remains the mainstay of treatment.     

Malignant fibrous histiocytoma of the larynx

Malignant fibrous histiocytomas of the upper respiratory tract are rare, aggressive, mesenchymal neoplasms. There have been sporadic cases reported in the English language literature. This paper reports the case of a subglottic malignant fibrous histiocytoma in a 26-year-old female. A review of the important clinical-pathological features of this type of tumor is presented. The treatment of choice appears to be wide surgical resection of the tumor. Although the published experience is small, it appears that tumors of the subglottic space are characterized by a younger patient population and a better prognosis than those of the larynx.     

Malignant fibrous histiocytoma of the lung

Malignant fibrous histiocytoma (MFH) is among the most common soft tissue sarcomas of adult life, but rarely occurs elsewhere. We report an example of primary MFH of the lung and review 15 previously reported acceptable cases with current follow-up information. Histologically, the tumor in our case was pleomorphic with storiform and fascicular areas. Tumor cells showed positive immunostaining for alpha 1-antitrypsin, alpha 1-antichymotrypsin, and vimentin. Stains for desmin, cytokeratin, myoglobin, epithelial membrane antigen, S-100 protein, and lysozyme were negative. Electron microscopic study showed histiocyte-like, fibroblast-like, intermediate, and undifferentiated tumor cells. A variety of methods were used to treat these patients. Two patients survived for 5 or more years, two were alive and well at 8 and 12 months, respectively, two were alive with metastatic tumor at 3 and 18 months, respectively, and ten patients died of tumor, with an average survival of 1 year.     

Malignant fibrous histiocytoma of the extremity

BACKGROUND: This study examines prognostic factors and outcomes for a group of patients with malignant fibrous histiocytoma of the extremity treated at a single referral institution. METHODS: A total of 239 patients underwent complete surgical excision between July 1982 and July 1996 and were followed prospectively. RESULTS: The overall 5-year and 10-year disease specific survival rates for this group were 65% and 59%, respectively. Age >50 years, tumor location, and presentation with local recurrence were all associated with increased risk of local recurrence. Tumor size, grade, depth, and presentation with local recurrence were all associated with adverse disease specific and metastasis free survival. Tumor size > or = 10 cm was associated with a particularly unfavorable outcome. CONCLUSIONS: The majority of malignant fibrous histiocytomas of the extremity are associated with a relatively favorable prognosis. Size > or = 5 cm, deep depth, and high grade are all unfavorable factors. Size > or = 10 cm is associated with an even more unfavorable prognosis.     

Malignant fibrous histiocytoma of the liver

Resumen El histiocitoma fibroso maligno (HFM) del hígado es un tumor infrecuente que ocurre predominantemente en hombres durante su vida adulta de media y tercera edad. No se asocia a enfermedad hepática crónica y estos pacientes habitualmente vienen a tratamiento con una enfermedad avanzada. Dolor abdominal es el síntoma principal. Las exploraciones radiológicas no podrían diferenciar HFM de otras lesiones hepáticas. La resección del hígado ha sido la forma más común de tratamiento. Se han usado quimioterapia y radioterapia sin éxito. Presentamos el caso de un histiocitoma fibroso maligno primario del hígado (HFMG) en una paciente de 62 a?os de edad que fue ingresada después de una biopsia laparoscópica complicada. Se hizo una segmentectomía lateral izquierda. El estudio immunocitoquímico fue positivo para alfa 1-antiquimiotripsina, vimentina, desmina y actina y confirmó el diagnóstico. La paciente presentó sarcomatosis peritoneal y murió 12 meses después de la operación.

     

Malignant fibrous histiocytoma of the lung

Twenty-two cases of primary malignant fibrous histiocytoma (MFH) of the lung are described, and a review of the literature is presented. As in the soft tissue, this sarcoma is found primarily in older individuals, usually as a solitary peripheral mass. Chest wall invasion at diagnosis was seen in four cases. The most common histologic subtype was the storiform-pleomorphic type of MFH. Vascular invasion was seen in 50% of cases. Histologic features did not correlate with prognosis, and subsequent aggressive behavior was related primarily to symptomatic presentation, advanced stage at diagnosis, incomplete excision, invasion of the chest wall or mediastinum, and subsequent recurrence or metastasis. Primary MFH of the lung should be distinguished from spindle cell carcinoma and inflammatory pseudotumors, and this histologic differential diagnosis is discussed.     

Malignant fibrous histiocytoma of ethmoid sinuses

A case of malignant fibrous histiocytoma of ethmoid sinuses is presented for its rarity. Its mode of presentation, histopathology and management is discussed.     

Malignant fibrous histiocytoma of ethmoid sinus

Malignant Fibrous Histiocytoma (MFH) is a rare tumour to involve ethmoid sinuses. So far only 7 cases have been reported in the literature to our best of knowledge. The disease predominantly affected younger people with no significant sex prediliction. The aggressive behaviour of the tumour clinically is an important parameter to clinch the malignant nature of the disease. Wide surgical resection is the accepted method of treatment. Local recurrences are frequent and distant metastasis are not known from ethmoid sinus.