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PubMed1997

bcl-2 expression in pleural and extrapleural solitary fibrous tumours.

This study evaluated the immunoreactivity for bcl-2, a molecule involved in the control of programmed cell death, in cases of pleural (14) and extrapleural (2) solitary fibrous tumour (SFT), malignant mesotheliomas of different histological types, and a variety of extrapleural CD34-positive and CD34-negative spindle-cell tumours. In all SFTs, strong and diffuse immunostaining was demonstrated with anti-bel-2 antibody, sharply contrasting with the complete lack of staining observed in all mesotheliomas. The specificity of immunodetection of bcl-2 in SFT was confirmed by immunoblot analysis, showing a band consistent with the bcl-2 protein. At extrapleural locations, strong bcl-2 immunoreactivity was observed in Schwannoma (2/3 cases), synovial sarcoma (4/4 cases), and all cases of CD34-positive gastrointestinal stromal tumour (GIST; 10/10 cases). Most sarcomas were bcl-2-negative. Lack of bcl-2 expression was demonstrated in tumours which can pose problems in the differential diagnosis of SFT and can exhibit haemangiopericytoma-like features, including haemangiopericytoma (3 cases), dermatofibrosarcoma protuberans (16 cases), and deep-seated fibrous histiocytoma (3 cases). The constitutive expression of bcl-2 in SFT widens the spectrum of available markers for these tumours, providing a useful adjunct to their differential diagnosis in difficult cases at pleural and extrapleural sites, and contributing to the understanding of their histogenesis and molecular pathogenesis.

Adult↗

PubMed1987

Soft tissue sarcomas of the adult thoracic wall.

Forty-nine adult patients with soft tissue sarcomas of the thoracic wall have been treated by the Division of Surgical Oncology at the University of Illinois. Fibrosarcoma was the most common histologic type (20%). Patients with dermatofibrosarcoma protuberans had the longest mean overall survival time (150.8 months). The mean overall survival time for all patients was 98.2 months, and the disease-free 2-, 5-, and 10-year survival rates were 68%, 51%, and 34%, respectively. Wide excision was the treatment of choice; selected patients also received adjuvant radiotherapy, chemotherapy, or both. In all 10-year survivors, the sarcomas were low grade and well differentiated, or were less than or equal to 5.0 cm in diameter. Aggressive treatment afforded these patients good long-term results.

Adult↗

PubMed1989

Mutagen sensitivity and cancer susceptibility. Report of a cancer-prone family.

A cancer-prone family was studied to determine if certain chromosomal abnormalities might have predisposed members to develop diverse types of malignancies. The types of neoplasia that occurred in this family included cancers of the breast and stomach, multiple myeloma, dermatofibrosarcoma, Wilm's tumor, and leukemia; the latter three occurred in children at an early age. Peripheral lymphocytes from 13 family members were examined for the presence of constitutional chromosomal abnormalities, fragile sites, and mutagen sensitivity. Our data shows that all living members of this family who had cancers were hypersensitive to chromosome breakage induced by bleomycin. In contrast, neither constitutional chromosomal abnormality nor heritable type of folate-sensitive fragile site was observed in any member. The above findings suggest that genetic defects affecting chromosomal breakage and repair may be contributing factors for cancer development in several members of this family.

Adult↗

PubMed2002

Fine-needle aspiration cytology of giant cell fibroblastoma: case report and review of the literature.

Giant cell fibroblastoma is an uncommon soft tissue neoplasm occurring in childhood. It appears to be the juvenile form of dermatofibrosarcoma protuberans, with which it shares some histologic, cytogenetic, and immunohistochemical features. We report, to our knowledge, the second description of the cytologic features of giant cell fibroblastoma. The present case represents a recurrent lesion in the soft tissues of the scrotum of a 17-yr-old male. The aspirate produced moderately cellular smears containing mononuclear cells, usually lying singly, but occasionally forming clusters. The majority of the individual cells possessed scanty bipolar cytoplasm or were devoid of cytoplasm. The nuclei were bland, with small nucleoli. Nuclear membranes frequently contained notches, creases, or folds. Small fragments of metachromatic stroma were present in the background and were often associated with small aggregates of cells. Rare multinucleated giant cells containing bland oval or basillary-shaped nuclei were admixed with the spindle-cell component. Necrosis and mitotic figures were not a component of the smears. Surgical resection of the mass confirmed the diagnosis of giant cell fibroblastoma. We review the characteristic cytologic features of giant cell fibroblastoma and compare them with other soft tissue tumors in the differential diagnosis.

Adolescent↗

PubMed2004

Fine-needle aspiration of primary and recurrent benign fibrous histiocytoma: classic, aneurysmal, and myxoid variants.

There is a limited number of correlative cytopathological studies of fibrous histiocytoma (FHC). To better define cytopathological criteria of diagnosis, we have reviewed fine-needle aspirates (FNA) from 36 FHCs (32 classical, 1 myxoid, and 3 aneurysmal variants on corresponding histological sections). Original cytological diagnoses were benign in 33 (91.7%) cases (22 accurate) and false positive in 3 (8.3%) cases. All smears were surprisingly homogenous and composed of histiocytic cells with finely vacuolated cytoplasm in 27 (75%) cases, small regular spindle cells in 25 (69%) cases, and giant cells in 17 (47%) cases. Histiocytic cells were attached to vascular structures in 9 (25%) cases. Slight cytonuclear atypia was seen in five (14%) cases. Three (8.3%) cases showed numerous siderophages. In two (5.6%) cases, there were abundant inflammatory backgrounds and in one (3%) case there was a scant myxoid background. Storiform patterns, round cells, prominent atypia, necroses, or mitotic figures were not seen. FHC should be differentiated from other benign, low- and intermediate-grade spindle-cell neoplasms such as low-grade fibrosarcoma, dermatofibrosarcoma protuberans, nodular fasciitis, spindle-cell malignant melanoma, and monophasic synovial sarcoma. Some cases may be misinterpreted as malignant, especially in cases of recurrence or in patients with a cancer history.

Adolescent↗

PubMed1994

Fine-needle aspiration biopsy: pitfalls in the diagnosis of spindle-cell lesions.

Seven cases of spindle-cell proliferations in which fine-needle aspiration biopsy (FNAB) did not correlate with subsequent histology are presented. Three cases were considered low-grade sarcoma, one a dermatofibrosarcoma protuberans (DFSP), one a spindle-cell tumor with malignancy not excluded, and one a rhabdomyosarcoma vs. a fibrosarcoma. Two of the these three were histologically nodular fasciitis and one an inflammatory pseudotumor. Two cases were diagnosed cytologically as fibromatosis or nodular fasciitis (NF). One of these histologically was an intramuscular hemangioma, the other a DFSP. The last two cases were diagnosed by FNAB as spindle-cell lesion, undetermined if benign or malignant, and malignant fibrous histiocytoma (MFH). Histologically both of these case were leiomyosarcoma. The cytologic features of each case, differential diagnosis, and potential pitfalls are discussed. In the evaluation of FNAB smears dominated by spindle cells, cellullarity, individual cells and cell patterns, and background stromal features coupled with a precise clinical history may allow a narrow differential diagnosis with a focus on whether the lesion is benign or malignant. Caution is warranted in the exact classification of spindle-cell tumors from FNAB as this may have a major impact on patient management.

Adolescent↗

PubMedDate not supplied

Head and neck sarcoma: report of the Head and Neck Sarcoma Registry. Society of Head and Neck Surgeons Committee on Research.

A Head and Neck Sarcoma Registry was established by the Society of Head and Neck Surgeons to review treatment results of a rare tumor by surgeons with special interest in this anatomic site. Two hundred fourteen patients were analyzed. There were 194 adult tumors and 20 pediatric tumors. The major sites included parotid and neck, 20%; face and forehead, 18%; maxilla and palate, 13%; scalp, 12%; mandible, 11%; paranasal sinuses, 7%; larynx, 2%; and oral cavity, 5%. Eighty-four percent were resectable. The disease-free survival was 56%; overall survival was 70% at 5 years. Major determinants of survival were adequacy of resection (margins free of tumor) and tumor type. Survival differed according to tumor cell type (tumor grade was not available). Patients with chondrosarcoma and dermatofibrosarcoma had survival approaching 100%. Patients with malignant fibrous histiocytoma (MFH) and fibrosarcoma (FSA) had intermediate survival of 60% to 70%. The worst survival, less than 50% at 5 years, occurred in patients with osteosarcoma, angiosarcoma, and rhabdomyosarcoma in decreasing order. This suggests a rationale for identifying high-risk patients for prospective adjuvant protocols. This study emphasizes the value of recording uncommon tumors to provide relevant information for future study and possibly therapy.

Adolescent↗

PubMed2006

Genetic polymorphisms of the XPG and XPD nucleotide excision repair genes in sarcoma patients.

There are more than 50 subtypes of soft tissue sarcomas, among which 30% are associated with specific genetic alterations, including translocations. Several studies have reported associations between cancer risk and polymorphisms of DNA repair genes from the nucleotide excision repair (NER) pathway. NER involves more than 20 proteins whose inactivation leads to xeroderma pigmentosum (XP) or cockayne syndrome (CS), among which XPD, a helicase allowing DNA strand excision by the endonuclease XPG. DNA from 93 patients with synovial sarcomas, myxoid liposarcomas, dermatofibrosarcomas protuberans (DFSP), malignant fibrous histiocytomas and leiomyosarcomas were genotyped for both XPD Lys751Gln and XPG Asp1104His polymorphisms. Departure from Hardy-Weinberg was highly significant for the XPG polymorphism with an excess of heterozygotes in synovial sarcomas (p = 1.5 x 10(-5)), myxoid liposarcomas (p = 1.5 x 10(-4)) and to a lesser extent in DFSP (p = 0.028). In the case of XPD, a significant deviation was observed in synovial sarcomas (p = 3 x 10(-6)) and DFSP (p = 0.0014). When tumors were pooled according to their genetic alterations, the proportion of carriers of the variant XPG allele was significantly increased in sarcomas with specific translocations as compared to sarcomas with complex genetics (p < 10(-9)). No difference was found for XPD. Genotyping of the tumor samples in synovial sarcomas and myxoid liposarcomas revealed frequent loss of heterozygosity for XPG, mostly due to the loss of the frequent allele. For XPD, both alleles were lost with a similar frequency. Our results raise the potential implication of the XPG Asp1104His polymorphism in the occurrence of chromosomal translocations associated with specific subtypes of sarcomas.

Adolescent↗

PubMed1984

Enhanced chemotaxis of tumor-derived and virus-transformed cells to fibronectin and fibroblast-conditioned medium.

SV40 transformants of human embryo fibroblasts showed an enhanced chemotactic response to fibronectin and conditioned medium of fibroblasts in comparison to the non-transformed cells. An even higher chemotactic response was characteristic for a malignant human fibrosarcoma cell line, whereas cells derived from low-grade malignant dermatofibrosarcoma showed a normal response in the Boyden chamber assay. The high stimulation of chemotaxis is paralleled by a reduced synthesis and an altered deposition of fibronectin in the pericellular matrix of these cells. Both high chemotactic response and lack of deposition of fibronectin may play a role in tissue invasion and formation of metastasis by malignant tumors.

Cell Transformation, Viral↗

PubMed1985

Colonization of nonmelanocytic cutaneous lesions by dendritic melanocytic cells: a simulant of acral-lentiginous (palmar-plantar-subungual-mucosal) melanoma.

Four cases of colonization of nonmelanocytic lesions by dendritic melanocytic cells are reported, one in a verruca vulgaris of the lip, one in a squamous cell carcinoma in situ of mucous membrane overlying a tonsil, one in a lesion of lichen simplex chronicus with a nevocellular nevus of the external ear, and one in a dermatofibrosarcoma protuberans (Bednar tumor). This is an important biological process of melanocytes that must not be confused with the acral-lentiginous (palmar-plantar-subungual-mucosal (P-S-M) melanoma. It is probably much more common than the paucity of published reports would indicate.

Adult↗

PubMed1987

Nonosseous sarcomas in a military hospital.

This is a retrospective review of 67 patients with soft tissue or visceral sarcomas treated during 1973-1982. There were 40 males and 27 females. The mean age was 36 with a range of 1 to 77 years of age. The most common histologic subtypes were rhabdomyosarcoma (19%), malignant fibrohistiocytoma (13%), fibrosarcoma, leiomyosarcoma, dermatofibrosarcoma protuberans (12% each), and liposarcoma (10%). Among the 42 soft tissue sarcomas 33% occurred in the trunk, 35% and 21% in the lower and upper extremities, and 10% in the head and neck region. Among the 25 visceral sarcomas, 16% were in the thorax, 56% involved abdominal or retroperitoneal organs, and 28% were in the head and neck region. Overall, 33 of the 54 patients with early sarcomas were treated by surgical resection alone while 21 others had postoperative adjuvant radiotherapy and/or chemotherapy. Factors influencing survival are presented and patterns of metastasis discussed. Overall, 45% of the patients showed metastatic disease either at diagnosis (15%) or during the follow-up period (30%). The lung was involved in two-thirds of the cases and was the most common site of spread. In patients with rhabdomyosarcoma and malignant fibrohistiocytoma, regional lymph node metastasis was noted in 36% and 17%, respectively.

Adolescent↗

PubMed2002

Connective tissue tumors.

Connective tissue consists of collagen, elastic fibers and ground substances produced by fibrocytes. These cells are usually spindle-shaped with slender nuclei and bipolar cytoplasmic extensions. Apart from labeling for vimentin and variable reactivity for factor XIIIa and CD34, fibrocytes are immunonegative. Electron microscopy reveals prominent endoplasmic reticulum, but is otherwise indistinct. Lesions with fibrocytic differentiation can be divided into five categories: scars, keloids, dermatofibromas, nodular fasciitis, and superficial fibromatoses are inflammatory lesions. Thereby, dermatofibromas and their subcutaneous/deep soft tissue counterpart nodular fasciitis can present with a wide variety of clinicopathologic variants which may be misinterpreted as malignancies. Prurigo nodularis, chondrodermatitis nodularis helicis, acanthoma fissuratum, and knuckle pads are hyperplasias; fibroma molle, fibrous papules, connective tissue nevi, and elastofibroma are hamartomas; and fibroma of tendon sheath, pleomorphic fibroma, and giant cell tumor of tendon sheath are benign neoplasms. Deep fibromatoses, dermatofibrosarcoma protuberans, giant cell fibroblastoma, giant cell angiofibroma, hyalinizing spindle cell tumor with giant rosettes, solitary fibrous tumor, myxofibrosarcoma, low-grade fibromyxoid sarcoma, acral myxoinflammatory fibroblastic sarcoma, and classical fibrosarcoma, are malignant neoplasms, that is fibrosarcomas of variable malignant potential. Lesions dominated by myocytes/ myofibroblasts, e.g. cutaneous myofibroma/infantile myofibromatosis, or by macrophages, e.g. xanthogranulomas, are not part of this chapter.

Humans↗

PubMed1984

Delayed cerebral pseudo-tumoral radionecrosis following scalp-tumour irradiation. Case report and review of literature.

A case of delayed radionecrosis of the brain is reported, following irradiation of a scalp dermatofibrosarcoma. Signs and symptoms were those of an intracranial neoplasm and appeared 5 years after an irradiation dosage of 1890 rets. Twenty-seven similar cases were gathered from a survey of the literature. Surgical excision was the treatment of choice and led to cure or improvement in 20 cases. Analysis of doses and fractionation revealed excessive irradiation in all but one of the cases.

Adult↗

PubMed1991

Immunohistochemical characterization of Ki-M6 monoclonal antibody in Bouin-fixed, paraffin-embedded sections of normal and neoplastic human tissues.

A monoclonal antibody (Ki-M6) against the CD 68 antigen, which labels cells of the monocyte/macrophage system, was tested on Bouin-fixed, paraffin-embedded samples of normal, reactive and neoplastic tissues by an avidin-biotin-peroxidase complex method, with the aim of establishing its value in diagnostic pathology. In normal human tissues, Ki-M6 reactivity was confined to the so-called resident macrophages populating normal organs under physiological conditions. Moreover, restricted reactivity against cells of macrophage lineage was observed in reactive and inflammatory lesions. Granulocytes, monocyte/macrophage-related immune accessory cells, and other analysed normal tissue structures did not reveal any reactivity. Ki-M6 was strongly reactive with the cases of benign (4/4) and malignant (15/15) fibrous histiocytomas, in addition to the true histiocytic lymphomas (3/3). Cases of granular cell tumour (2/3) showed strong reactivity with Ki-M6, whereas only few immunoreactive cells, with weak staining, were seen in the other Ki-M6-positive neoplasms [neurofibroma (3/3), benign schwannoma (1/2), ganglioneuroma (1/1), malignant schwannoma (5/9), melanoma (9/28), dermatofibrosarcoma protuberans (1/1), myelomonocytic leukaemia (3/3)]. Among the epithelial malignancies tested (47 cases), Ki-M6 was positive only in renal cell carcinoma (11/14). Malignant lymphomas of the Hodgkin (56 cases) and non-Hodgkin type (67 cases) were uniformly non-reactive. From these data, Ki-M6 appears to be an excellent marker of monocyte/macrophage-related cells and appears to be a reliable indicator for fibrous histiocytomas and true histiocytic malignancies. The availability of this additional antibody capable of staining routinely processed tissue is of practical interest.

Antibodies, Monoclonal↗

PubMed2001

A case of spindle cell carcinoma of the breast--long survival achieved by multiple surgical treatment.

Spindle cell carcinoma of the breast was formerly called carcinosarcoma, and is relatively rare. We report a case of spindle cell carcinoma of the breast. The patient was treated with multiple surgeries and achieved long-term survival. The patient was a 52-year-old woman, in whom small induration developed at the areola of the nipple of the right breast. The lesion was resected, and benign tumor was diagnosed pathologically. Four years later, she had recurrence at the scar, and a typical mastectomy was performed. A tumor developed again 5 years later; the lesional focus was at the scar of the right chest wall and invasion of the ribs and the sternum was noted. The sternum and the right costal cartilage of ribs 3-9 were dissected together. The right chest wall was reconstructed and adjuvant radiation therapy performed. Four years after this operation, tumor recurred near the scar and chest wall resection including part of the pericardial cavity and the left lung was performed. However, 6 months later, invasion of the mediastinum, heart and lung were noted. The patient died 16 years after the first surgery. Dermatofibrosarcoma protuberance of the breast was diagnosed at the second operation. However, the diagnosis was changed to spindle cell carcinoma of the breast following immunohistochemical studies. Spindle cell carcinoma of the breast is rare, and definitive histopathological diagnosis is often difficult. When spindle cell carcinoma is suspected, comprehensive diagnostic studies including immunohistochemical examinations should be performed. Even in case with multiple recurrences correctly performed operations may contribute to prolongation of survival.

Bone Neoplasms↗

PubMed2004

Oncogenic derivatives of platelet-derived growth factor receptors.

Platelet-derived growth factor receptors (PDGFRs) and their ligands, platelet-derived growth factors (PDGFs) play critical roles in mesenchymal cell migration and proliferation. In embryogenesis the PDGFR/PDGF system is essential for the correct development of the kidney, cardiovascular system, brain, lung and connective tissue. In adults, PDGFR/PDGF is important in wound healing, inflammation and angiogenesis. Abnormalities of PDGFR/PDGF are thought to contribute to a number of human diseases, and especially malignancy. Constitutive activation of the PDGFRalpha or PDGFRbeta receptor tyrosine kinases is seen in myeloid malignancies as a consequence of fusion to diverse partner genes, and activating mutations of PDGFRalpha are seen in gastrointestinal tumours (GISTs). Autocrine signalling as a consequence of PDGF-B overexpression is clearly implicated in the pathogenesis of dermatofibrosarcoma protruberans (DFSP) and overexpression of PDGFRs and/or their ligands has been described in many solid tumours. PDGFR signalling is inhibited by imatinib mesylate, and this compound has clear clinical activity in patients with myeloid malignancies, GIST and DFSP.

Benzamides↗

PubMed2003

[Benign cellular fibrous histiocytoma with erosion of the phalanx].

Fibrohistiocytic tumors are characterized by the presence of fibroblast like spindle cells and histiocytes. The benign fibrous histiocytoma (dermatofibroma, BFH) as well as the malignant dermatofibrosarcoma protuberans (DFSP) and the malignant fibrous histiocytoma (MFH) belong to this group. A recurrent painful, hard 2 cm tumor on the left hallux of a 54-year-old woman led to an erosion of the underlying phalanx. The patient had suffered from ingrown toenails for more than 10 years. Histologically there was a deep penetrating fibrohistiocytic tumor that grew in a storiform pattern with interspersed foam cells. The tumor was CD34 negative and mitoses were scarce. The diagnosis was benign cellular fibrous histiocytoma (BZFH). BZFH belong to the group of BFH with a high recurrence rate especially after incomplete removal. Damage to the underlying bone has not been reported so far.

Female↗

PubMed1998

[Myofibroblastic tumors. Brief review of clinical aspects, diagnosis and differential diagnosis].

This review summarizes myofibroblastic tumours that have been characterized in the last years. These lesions include: fibromatoses in adults and infants (infantile digital fibromatosis and infantile myofibromatosis); myofibroma of adults, an almost exclusively solitary lesion in the skin which is characterized morphologically as a biphasic lesion composed of spindle-shaped eosinophilic tumour cells and more primitive mesenchymal tumour cells associated with a haemangiopericytoma-like vasculature; dermatomyofibroma (plaque-like dermal fibromatosis), a band-like myofibroblastic proliferation in young female patients, which is mainly located in the periaxillar region and in which distinction from more aggressive, plaque-like variant of dermatofibrosarcoma protuberans is mandatory; myofibroblastoma of the breast, a well-circumscribed lesion composed of spindle shaped, desmin-positive tumour cells, which is seen mainly in elderly male patients and has to be distinguished from other spindle cell lesions of the breast; angiomyofibroblastoma, a well-circumscribed myofibroblastic neoplasm of the vulva and vagina composed of avoid to round myoid tumour cells with scattered multinucleated cells, which forms a continuous morphological spectrum with the clinically more aggressive angiomyxoma in this location; intranodal myofibroblastoma, a distinctive proliferation of myofibroblastic cells associated with so-called amianthoid fibres, which is seen most commonly in inguinal lymph nodes; myofibroblastoma/myofibroblastic tumour of soft tissues, a variably well-circumscribed myofibroblastic lesion which lacks atypia and is composed of actin and/or desmin positive tumour cells, and poorly delineated sarcomas with myofibroblastic differentiation (myofibrosarcoma).

Adult↗