Congenital diaphragmatic hernia: an unsolved problem

Primary and metastatic malignant fibrous histiocytoma of the alimentary tract is uncommon, even though it is the most frequently diagnosed malignant soft tissue tumor in adults. In this report, we describe a case of malignant fibrous histiocytoma of the colon.     

Different left ventricular relaxation parameters in isolated working rat and guinea pig hearts. Influence of preload, afterload, temperature, and isoprenaline

Six primary lung tumors with numerous multinucleated osteoclast-like giant cells (OLGCs) and no osteogenic component were evaluated histologically and immunohistochemically to examine pulmonary lesions inciting an OLGC response. The patients comprised four women and two men ranging in age from 61 to 80 years (average age, 69 years). The tumors consisted of one adenocarcinoma, two sarcomatoid carcinomas, and three giant cell variants of malignant fibrous histiocytoma. One tumor was endobronchial in location, while five were situated peripherally. Tumor diameter spanned from 1 to 6.5 cm (average, 2.7 cm). In addition to the giant cells, common characteristics included the malignant nature of the neoplasms and, in five of six cases, histologically malignant mesenchyme. This array of cases exemplifies the variability of lung lesions which may elicit an OLGC inflammatory response resulting in areas resembling the giant cell variant of malignant fibrous histiocytoma. The results of this study suggest that OLGCs occur preferentially in malignant rather than benign nonosteogenic lung tumors and that sarcomatoid regions of malignant tumors are more likely to be infiltrated by OLGCs than epithelial regions.     

Malignant fibrous histiocytoma of the gallbladder

We report two extremely rare cases of primary malignant fibrous histiocytoma (MFH) of the gallbladder. The first case occurred in a 70 year old woman who presented with a large tumor of the gallbladder and multiple liver metastases. The second case involved a 74 year old man with a small submucosal tumor of the gallbladder and a single large liver metastasis. Histologically, these tumors consisted of spindle cells in a storiform pattern intermingled with bizarre giant cells. Both of these patients died of liver failure 3 months post-operatively.     

Single somatic ras gene point mutation in soft tissue malignant fibrous histiocytomas

The frequency of ras gene mutations in human soft tissue malignant fibrous histiocytomas within and around the hot spot codons (12, 13, and 61) of all ras genes, (H-ras-1, K-ras-2, and N-ras) was studied by nested polymerase chain reaction and direct DNA sequencing from archival formalin-fixed, paraffin-embedded tissue. Light microscopy and immunohistochemistry served to define malignant fibrous histiocytoma. All of the four differentiation subtypes (storiform-pleomorphic, inflammatory, myxoid, and giant cell) were investigated. Nine of thirty-two malignant fibrous histiocytomas (28%) contained ras gene point mutations. The highest incidence was found in the myxoid subtype (four of nine). H-ras-1 gene codon 12.2 was the only codon affected and contained in all mutated cases a GGC-->GTC exchange. Seven of the nine mutations were homozygous and probably affected more than 80% of the tumor DNA. The flanking regions of all hotspot codons did not contain any point mutation. The presence of a single and often homozygous point mutation of the H-ras-1 gene, especially in myxoid malignant fibrous histiocytoma could serve as a basis for further genomic discrimination of myxoid sarcomas.     

Hepatic malignant fibrous histiocytoma: CT findings

Although malignant fibrous histiocytoma represents the most common soft tissue sarcoma in adults, its origination in visceral organs is very unusual and the liver is an exceptional site of involvement, with only 22 cases reported in the last 12 years. This controversial tumour, first described in 1964, probably originates from undifferentiated mesenchymal cells and has five different histological subtypes: storiform pleomorphic, myxoid, giant cells, inflammatory and angiomatoid. We describe herein the rather variable CT findings of three malignant fibrous histiocytomas of the liver and discuss their differential diagnosis.     

LN-2 (CD74). A marker to distinguish atypical fibroxanthoma from malignant fibrous histiocytoma

BACKGROUND: In this study, the authors examined the expression of LN-2, an antigen expressed by B cells, macrophages, and Reed-Sternberg cells, in a variety of spindle cell lesions of the skin to determine whether LN-2 immunoreactivity can be used to differentiate among these tumors. For comparison, they examined CD34 antigen expression in these lesions, which has been shown to be a useful marker in differentiating dermatofibrosarcoma protuberans from dermatofibroma. METHODS: Immunocytochemistry with anti-LN-2 and anti-CD34 monoclonal antibodies on formalin fixed, paraffin embedded material was performed on 102 spindle cell lesions, including dermatofibroma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, malignant fibrous histiocytoma, leiomyoma, and neurofibroma. RESULTS: LN-2 immunoreactivity did not distinguish between dermatofibroma and dermatofibrosarcoma protuberans, both of which showed weak immunoreactivity. In marked contrast, 90% of cases of malignant fibrous histiocytoma showed strong staining for LN-2, whereas the vast majority (90%) of cases of atypical fibroxanthoma were negative or stained only weakly with anti-LN-2 antibodies. Of the two cases of atypical fibroxanthoma that stained strongly for LN-2, both lesions were > 2 cm in size and extended deep into the subcutaneous fat. CONCLUSIONS: Differential expression of the LN-2 antigen by atypical fibroxanthoma and malignant fibrous histiocytoma distinguishes these two lesions and suggests that acquisition of LN-2 positivity may be a marker of tumor progression.     

Denatured venous homograft as an arterial substitute in civilian vascular injuries. Thirty months'' experience

Osteosarcoma, chondrosarcoma and tumors of the Ewing group are the most frequently observed primary malignant bone tumors. In an Internet homepage recently constructed for the Orthopedic Hospital Rizzoli Bologna, Italy, these tumors have represented the majority of 4423 malignant bone tumors in the archives of this institution since 1920 (http:/(/)www.tizeta.it/rizzoli). Malignant fibrous histiocytoma, fibrosarcoma, hemangioendothelioma, malignant hemangiopericytoma and giant-cell tumors are diagnosed less frequently. Since the introduction of modern molecular and cytogenetic techniques, knowledge of genetic aberrations in malignant bone tumors has steadily increased. However, so far only for the group of Ewing tumors has a recurrent chromosomal marker, the translocation t(11;22) (q24;q12), been identified.     

Angiosarcomas associated with bone infarcts

OBJECTIVE: A bone infarct may occasionally dedifferentiate to osteogenic sarcoma, fibrosarcoma or malignant fibrous histiocytoma. However, the association of an angiosarcoma with a bone infarct is extremely rare. Such an association is presented in three patients. Their clinical course is compared with that of patients with bone infarcts associated with other sarcomas. DESIGN AND PATIENTS: The three patients were men with a mean age of 43 years. Cases 1 and 3 presented with a pathological fracture at the site of the angiosarcoma. Plain radiography was done in the three patients, computed tomography (CT) was performed in cases 1 and 3 and magnetic resonance imaging (MRI) in case 3. The femur was the site of the three tumors: midshaft in cases 1 and 3 and distal shaft in case 2. On the basis of the radiographic findings, and clinical examination, an open biopsy was performed for the three men, which confirmed the diagnosis of a high-grade angiosarcoma associated with a bone infarct. RESULTS: Case 1 was treated with high-above knee amputation and is still alive after 18 months from the time of operation. Segmental resection of the distal femur with adjuvant chemotherapy and local irradiation was the treatment for case 2, who is still alive with no tumor recurrence on metastatic disease 3 years from the operation. Intramedullary rodding was done for case 3 who died 6 months later. CONCLUSION: The association of an angiosarcoma with a bone infarct has been established in only five cases. Although the number of such associations is small, it seems that such an association may be prognostically more or less the same as in those cases in which a bone infarct is associated with either osteosarcoma, fibrosarcoma or malignant fibrous histiocytoma, where the survival rate is unfavorable. A cause-and-effect relationship may exist between a bone infarct and subsequent development of a bone sarcoma.     

Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases

The light microscopic and immunohistochemical features of 30 primary mesenchymal neoplasms of the urinary bladder are reported. Half of the cases represented smooth and striated muscle tumors (five leiomyomas, seven leiomyosarcomas including epithelioid and myxoid subtypes, one rhabdomyoma, one embryonal rhabdomyosarcoma and one alveolar rhabdomyosarcoma). One third of the tumors were of fibrohistiocytic origin (one fibrous histiocytoma and eight malignant fibrous histiocytomas including fascicular and storiform, inflammatory and pleomorphic subtypes). In addition, a malignant epithelioid schwannoma, a round cell liposarcoma, two hemangiomas and two mixed mesodermal tumors were observed. The morphology of the vesical mesenchymal tumors was identical to that of their counterparts known to occur in other sites, particularly in the soft tissue. Muscle-specific actin, alpha-1-antichymotrypsin, S-100-protein and neuron-specific enolase proved to be useful and reliable immunomarkers for differential diagnosis of poorly differentiated leio- and rhabdomyosarcomas, malignant fibrous histiocytomas and malignant schwannomas. Since some tumors coexpressed several classes of intermediate filaments, diagnostic immunocytochemistry should only be used considering a larger panel of antibodies and in close correlation with the histological and cytological features of the neoplasms.     

Special diagnostic problems in primary malignant bone tumors

The diagnosis of primary malignant bone tumors, which only account for 1% of malignancies in humans, represents quite a challenge for the pathologist. Apart from the often overlapping morphology between different entities, heterogeneity within one and the same tumor has to be kept in mind. Furthermore, in most cases the diagnosis must be established based on small biopsy specimens and occasionally even on frozen sections. In this context detailed clinical information including the patient's age and exact localization of the tumor, as depicted by X-ray or MRI, are essential requirements for a correct diagnosis. In addition, multiple biopsies from different tumor sites may be of help. For example, 25% of osteogenic sarcomas (OSA) are chondroplastic. Others may contain areas similar to fibrosarcoma (FSA) or malignant fibrous histiocytoma (MFH). Since 90% of OSA occur in the second decade of life whereas chondrosarcomas, FSA and MFH usually manifest after the age of 40 years, a biopsy solely composed of malignant chondromatous tissue obtained from a tibial lesion of a 10-year-old child should most likely be considered as part of an OSA. In the differential diagnosis of the so-called small, round cell tumors, which include Ewing sarcoma, malignant lymphoma and small-cell OSA, immunohistochemical staining of the paraffin-embedded material and a molecular biological/genetic work-up of frozen material are most helpful. In this paper the histomorphological characteristics and criteria for differential diagnosis of the most common primary malignant bone tumors are discussed.     

The classification of pneumothorax]

We report a case of spontaneous rupture of malignant fibrous histiocytoma. A 50-year-old male with right flank pain was referred to our hospital. Computed tomography (CT) showed a heterogeneous space-occupying lesion on the upper pole of the right kidney. Selective right renal arteriography revealed a hypovascular mass. Preoperative clinical diagnosis was spontaneous rupture of renal cell carcinoma. Radical nephrectomy was performed. Histopathological diagnosis was malignant fibrous histiocytoma arising from the renal capsule.     

Viral hepatitis A, E and their mix (A+E) in children

Atypical ("pseudosarcomatous"), cutaneous, fibrous histiocytoma is a rare connective tissue tumor arising on the trunk and limbs in young adults. Its histological diagnosis is difficult. We report the case of a 25-year-old woman who presented a nodule on her left leg. Two years after an incomplete excision, she developed a large local recurrence. Additional radiotherapy, after total reexcision was performed. This treatment was successful and no further recurrence occurred. Clinicopathological features of atypical ("pseudosarcomatous"), cutaneous, fibrous histiocytoma are reviewed. Differential diagnoses, including atypical fibroxanthoma, angiomatoid fibrous malignant histiocytoma and aneurysmal fibrous histiocytoma are discussed.     

Clinical evaluation of Dyract in primary molars: 3-year results

Connective tissue growth factor (CTGF) is a member of a family of immediate early gene products that may play an important role during tissue regeneration, wound repair and skin fibrosis. In this study, CTGF gene expression in mesenchymal tumors was investigated by in situ hybridization and CD34 antigen expression was studied by means of immunohistochemical staining. CTGF mRNA was expressed in fibroblasts of all nine dermatofibromas examined, but five of seven dermatofibrosarcoma protuberans (DFSP) or two cases of malignant fibrous histiocytoma were negative for its expression. In contrast, CD34 antigen was expressed only in DFSP. In vascular tumors, CTGF mRNA was expressed in pyogenic granuloma but not in angiosarcoma. In addition, the endothelial cells in angiolipoma and angioleiomyoma, but not in venous lake, expressed CTGF mRNA. These vascular lesions were all positive for CD34 expression. Tumors of other origins were negative for CTGF mRNA. Our findings indicated that benign fibroblasts and/or vascular endothelial cells have the capability to express CTGF mRNA when activated, but these cells lose this ability when they achieve malignant potency. Thus, examination of CTGF gene expression may be useful for differentiating between benign and malignant mesenchymal tumors, or to determine the origin of the tumors in connective tissue.     

Primary malignant fibrous histiocytoma of the descending colon

We herein present the case of a 50-year-old woman with malignant fibrous histiocytoma arising from the descending colon and localizing in the colonic wall. Malignant fibrous histiocytoma of the large bowel is a very rare tumor. A total of 18 cases, including our case, have been reported in the world medical literature so far and we also reviewed these cases. In our case, combined adjuvant chemotherapy was administered after a complete resection had been performed. No clinical signs of local recurrence or distant metastasis were found at 7 years after the operation.     

Enhanced expression of catalytic subunit isoform PP1 gamma 1 of protein phosphatase type 1 in malignant fibrous histiocytoma

The expression of the three catalytic subunits of protein phosphatase (PP) type 1 and 2A, PP1 alpha, PP1 gamma 1, and PP2AC, was examined in malignant fibrous histiocytoma using immunohistochemical analysis. The percentage of cells stained positively with antiserum against PP1 catalytic subunit isoform PP1 gamma 1 was significantly higher in tumorous region than in non-tumorous region of malignant fibrous histiocytoma. Furthermore, tumorous region showed markedly high S-phase fraction in the cell cycle, as compared to non-tumorous region. These results suggest that PP1 gamma 1 is involved in the accelerated growth of tumor cells in malignant fibrous histiocytoma.     

Resection of malignant fibrous histiocytoma invading the thoracic aorta

We report a case of surgically resected malignant fibrous histiocytoma which arose in the posterior mediastinum. Tumor removal with the required sufficient-margin and the resection of the affected thoracic aorta, led to flaccid paraplegia below the tenth thoracic level. This patient is now surviving with no evidence of recurrence at 42 months after the operation. Although malignant fibrous histiocytoma in the thorax generally shows a poor prognosis, this patient with complete resection could have a relatively long survival.     

Serial sonographic changes in a case of isthmic pregnancy treated with methotrexate

A case report of a primary malignant fibrous histiocytoma (MFH) in the mandible of a young adult male is presented and relevant literature reviewed. The clinical presentation and course of the case is typical of MFH of the jaw as reported in the literature.     

Anti-macrophage monoclonal antibody D-11 in the diagnosis of histiocytic tumors

Anti-macrophage monoclonal antibody (Mab) D-11 was tested in surgical material and biopsies of non-epithelial tumors and tumor-like lesions from 181 patients in order to assess possibility of using this Mab for diagnosis of histiocytic tumors, malignant fibrous histiocytoma in particular. The study was performed in parallel on cryostat sections and smears by immuno-peroxidase method. It is established that D-11 reacts positively with both histiocytic tumors and tumors of other genesis this being a limiting factor in differential diagnosis of histiocytic tumors. However, taking into consideration 100% of positive results with histiocytic tumors only, this antibody can be used for exclusion of tumors studied from the group of histiocytomas in cases of negative reaction.     

Malignant histiocytofibroma. Apropos of 3 cases of cervicofacial localization

We report 3 cases of head and neck malignant fibrous histiocytoma (MFH). The first case is a xanthomatous subtype of the inferior lip with lymph node metastasis. The two others cases are storiform-pleomorphic MFM of the hypopharynx and Killian area. A summary of actual knowledges about this entity is reviewed.     

Positron emission tomography (PET) methodology for small animals and its application in radiopharmaceutical preclinical investigation

Among the more uncommon tumors that may sometimes be encountered in the laryngeal region is the recently described giant cell tumor of the larynx. This lesion is a true neoplasm, presumably of the fibrohistiocytic series. Histologically, it closely resembles the more familiar true giant cell tumor of long bone. The laryngeal giant cell tumors appear, to date, to be nonmetastasizing lesions; it is possible that they may recur locally if incompletely excised (although this remains to be demonstrated). In view of the rarity of these tumors, a tentative diagnosis of such a neoplasm should always prompt consideration of other (more frequently encountered) differential diagnostic possibilities, including cytologically malignant giant cell-rich tumors such as malignant fibrous histiocytoma and sarcomatoid carcinoma.