muramidase and Fibroma

Elastofibroma dorsi is an uncommon benign fibroblastic pseudotumor that typically occurs in the subscapular region of middle-aged or older individuals. The pathogenesis is still unclear and a matter of debate. Magnetic resonance imaging can be used as a first-line investigation of the lesion and reveals a lenticular soft-tissue mass with a signal intensity similar to that of skeletal muscle interlaced with strands of fat. Biopsy is not necessary if all pathognomonic criteria are present. A conservative "wait and see" attitude is reasonable and immediate surgery is no more the standard treatment of elastofibroma dorsi. This review provides an updated overview of the diagnosis, management and pathogenesis of elastofibroma dorsi. We also discuss recent advances in our understanding of genomic alterations in elastofibroma dorsi.

Topics: Antigens, CD34; Chromosome Aberrations; DNA Copy Number Variations; Fibroma; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Muramidase; Soft Tissue Neoplasms; Vimentin

Elastofibroma is a slow-growing soft tissue lesion characteristically found between the inferior scapula and chest wall. Because it behaves clinically in a benign manner, fine-needle aspiration (FNA) represents the simplest and quickest method of obtaining a definitive diagnosis, thus obviating more invasive means of obtaining a tissue diagnosis. However, due to the nature of this lesion a correct diagnosis can inadvertently be missed. Herein we describe the findings of a recent FNA that obtained abundant diagnostic material and elaborate upon the spectrum of cytologic features of the elastic fibers that can be identified. These features should be recognized, since aspiration biopsy in elastofibromas can lead to hypocellular smears. In addition, we discuss recent developments in the pathophysiology of elastic fibers and their application toward understanding the generation of an elastofibroma.

Topics: Aged; Biopsy, Needle; Elastic Tissue; Fibroma; Humans; Immunohistochemistry; Male; Muramidase; Scapula; Soft Tissue Neoplasms

Two unusual fibroxanthomas were studied by light microscopy. The first case contained numerous osteoclast-like cells and resembled malignant giant cell tumour of soft tissues, a variant of malignant fibrous histiocytoma. Osteoclast-like giant cells were negative for lysozyme and alpha-1-antitrypsin. The second case contained areas of chondroid differentiation which resembled chondrosarcoma. Tumour cells within the cartilaginous areas were positive for S100 protein.

Topics: Aged; Aged, 80 and over; alpha 1-Antitrypsin; Chondrosarcoma; Female; Fibroma; Genetic Variation; Histiocytoma, Benign Fibrous; Humans; Immunohistochemistry; Keratins; Muramidase; S100 Proteins; Skin Neoplasms

We have studied 12 cases of cutaneous atypical fibroxanthoma using immunohistochemistry to demonstrate lysozyme, alpha-1-antitrypsin, S-100-protein, receptors for peanut agglutinin, and intermediate filaments. Results were compared with immunostaining in 24 cases of other so-called fibrohistiocytic tumours. In addition 2 cases of atypical fibroxanthoma and 6 cases of fibrohistiocytic tumours were stained by monoclonal antibodies specific for the monocyte cell lineage (Ki-M1, Ki-M2, Ki-M6, Ki-M7, Ki-M8, OKM-1 and Leu-M1) and double-stained by monocyte-markers and Ki-67. The immunophenotype of atypical fibroxanthoma was rather similar to the marker profile found in malignant fibrous histiocytoma. All atypical fibroxanthomas were positive for vimentin and negative for epithelial markers. Monocyte lineage-specific determinants could be demonstrated in varying amounts in cells suggestive of being reactive. In contrast proliferating--Ki-67 positive--cells did not express monocyte/macrophage related antigens in atypical fibroxanthoma and malignant fibrous histiocytoma both. As to the histogenesis of these tumours our findings speak in favour of a derivation from primitive mesenchymal cells rather than from histiocytes.

Topics: Aged; alpha 1-Antitrypsin; Arachis; Female; Fibroma; Frozen Sections; Humans; Immunohistochemistry; Intermediate Filament Proteins; Male; Muramidase; Receptors, Mitogen; S100 Proteins; Skin Neoplasms

Metastases from an atypical fibroxanthoma of skin have been reported but are very few in the literature. We had a personal case which, originally localized on the nostril, gave metastases to the cervical regional lymph node with no local recurrence. We examined both the primary and metastatic tumor by conventional histological stains and by immunoperoxidase technique to localize lysozyme, alpha-1-antitrypsin, ferritin and factor VIII antigens. The results show a clear correspondence between the primary and metastatic tumor.

Topics: Aged; Female; Fibroma; Histocytochemistry; Humans; Immunoenzyme Techniques; Lymphatic Metastasis; Muramidase; Skin Neoplasms

The morphological and immunohistochemical characteristics of 37 atypical fibroxanthomas of the skin were examined. Twenty-four tumours were nodular ulcerative lesions on the head and face of patients with a median age of 75 years, whereas 13 tumours occurred on the trunk and limbs of patients with a median age of 48 years. Both pleomorphic polygonal and giant cells as well as the spindle cell component of the tumours stained for the histiocytic markers alpha 1-antichymotrypsin, alpha 1-antitrypsin, lysozyme and, less frequently, for ferritin. Leu M1 antigen and peanut agglutinin receptors were not demonstrable in tumour cells. This antigenic profile was contrasted with the findings in six cases of dermatofibroma which were largely not reactive with the antisera used. The immunohistochemical findings in atypical fibroxanthomas suggest that they represent a homogeneous group of tumours which are related to tissue histiocytes. These results are discussed in the context of the published findings in other so-called fibrohistiocytic tumours including dermatofibrosarcoma protuberans and malignant fibrous histiocytoma. The diagnoses in three cases coded as atypical fibroxanthomas were revised on the basis of their showing a different immunohistochemical profile.

Topics: Adult; Aged; Aged, 80 and over; alpha 1-Antichymotrypsin; alpha 1-Antitrypsin; Female; Ferritins; Fibroma; Histocytochemistry; Humans; Immunochemistry; Male; Middle Aged; Muramidase; Skin Neoplasms; Vimentin

183 cases of soft tissue tumors were studied utilizing the immunoperoxidase technique to demonstrate alpha-1-antitrypsin, ferritin and lysozyme. The series comprises 50 malignant lesions, 34 intermediate malignancy lesions, 99 benign lesions of fibrohistiocytic origin, and 23 malignant tumors of non fibrohistiocytic origin. The actual results of the study are as follows: alpha-1-antitrypsin, ferritin and lysozyme were always absent in 10 fibrosarcomas, 2 liposarcomas, 2 Ewing sarcomas, 3 synovial sarcomas, 4 neurofibrosarcomas, and 2 rhabdomyosarcomas, but in 24 malignant fibrous histiocytomas, 34 cases of dermatofibrosarcoma protuberans and 102 benign fibrohistiocytic lesions, these activities were present in a percentage that ranged between 12% and 38% (average 25%). Differences in the frequency of positive reactions did not occur between benign and malignant fibrohistiocytic lesions. The immunohistological examinations carried out have, therefore, only a very limited value for the practical diagnostic evaluation, but, when positive, are important to clarify the histogenesis of the tumor.

Topics: alpha 1-Antitrypsin; Ferritins; Fibroma; Fibrosarcoma; Histiocytoma, Benign Fibrous; Histocytochemistry; Humans; Immunochemistry; Muramidase; Sarcoma; Soft Tissue Neoplasms

Five cases of inflammatory pseudotumour of the liver are reported and compared with seven individual previously published case reports. Clinical presentation was variable but often comprised low grade intermittent fever, vague abdominal symptoms, and a history of weight loss. Leucocytosis, raised erythrocyte sedimentation rate, and polyclonal hyperglobulinaemia were also sometimes detected. All five cases in the present series were considered to be clinically malignant; and in two the histological diagnosis was also that of malignancy. The lesion is clearly inflammatory and reactive, but the aetiology remains unknown.

Topics: alpha 1-Antitrypsin; Antigens; Blood Sedimentation; Child; Child, Preschool; Factor VIII; Female; Fibroma; Humans; Immunoglobulins; Leukocyte Count; Liver Neoplasms; Male; Middle Aged; Muramidase; von Willebrand Factor

Topics: Adult; Aged; alpha 1-Antitrypsin; Breast; Breast Neoplasms; Female; Fibroma; Humans; Immunoenzyme Techniques; Immunoglobulins; Male; Middle Aged; Muramidase; Panniculitis, Nodular Nonsuppurative

Twelve cases of malignant spindle-cell and sarcomatoid tumours of the skin of debatable nature were studied by immunocytochemical methods, using four antisera which might help contribute to resolution of the problems. The initial diagnosis made on structural grounds was confirmed by immunocytochemistry in six of eight cases in which a specific diagnosis had been made (one melanoma, three squamous carcinomas and two atypical fibroxanthomas). One case, initially regarded as AFX was reclassified as a squamous carcinoma, while a further case of possible AFX could not be confirmed by immunocytochemical study. Of the four cases in which structural examination was inconclusive, two were identified as squamous carcinomas and one as a melanoma by virtue of tumour markers. The fourth case was an intriguing actin-rich tumour of uncertain nature. Immunocytochemistry, despite certain limitations, has a valuable role to play in the analysis of the problematic spindle-cell malignant and pseudomalignant tumours of the skin.

Topics: Actins; Aged; Carcinoma, Squamous Cell; Female; Fibroma; Humans; Immunoenzyme Techniques; Keratins; Male; Melanoma; Middle Aged; Muramidase; S100 Proteins; Skin; Skin Neoplasms

The aim of this study was to localize alpha 1-antitrypsin, ferritin, and lysozyme by means of the indirect immunoperoxidase technique and to evaluate the significance of these antigens as markers of histiocytic differentiation in tumors of a supposed dual fibroblastic-histiocytic origin. The series comprised 31 malignant fibrous histiocytomas (MFH) of the pleomorphic, spindle cell, and myxoid types, four cutaneous fibrous histiocytomas, and four atypical fibroxanthomas, four dermatofibrosarcoma protuberans, and two osteoclastomas of bone. For comparison, 15 soft tissue sarcomas of various other types were examined. Of the MFHs of the pleomorphic type, 18 of 22 (82 per cent) were positively stained for alpha 1-antitrypsin and 12 of 22 (54 per cent) were positively stained for ferritin. Of the five MFHs of the spindle cell type, none was positively stained for alpha 1-antitrypsin, three were positive for ferritin, and one was positive for lysozyme. None of the myxoid variants (corresponding to grade I-II myxofibrosarcoma) was positively stained for either of the antigens. These results and the observations made on the cutaneous fibrous histiocytomas, atypical fibroxanthomas, dermatofibrosarcoma protuberans, and the various soft tissue sarcomas indicated that 1) alpha 1-antitrypsin is a valuable marker of histiocytic differentiation in both benign and malignant fibrous histiocytomas, 2) ferritin can be visualized in more than half of these fibroblastic-histiocytic tumors, and the presence of ferritin distinguishes the spindle cells of these tumors from fibroblasts of connective tissue and most fibrosarcomas, and 3) lysozyme, although a good marker of histiocytic differentiation in ordinary histiocytes and benign fibrous histiocytomas, is a poor marker of neoplastic histiocytes of malignant tumors. The results further support the concept that MFH is a tumor of a dual fibroblastic-histiocytic origin.

Topics: alpha 1-Antitrypsin; Ferritins; Fibroma; Histiocytoma, Benign Fibrous; Humans; Immunochemistry; Muramidase; Sarcoma; Soft Tissue Neoplasms

Distribution of intracytoplasmic lysozyme in proliferative and neoplastic fibrohistiocytic lesions and non fibrohistiocytic tumors was studied by immunoperoxidase technique on formalin fixed, paraffin-embedded sections. The cases examined were 161 fibrohistiocytic lesions and 86 non-fibrohistiocytic tumors. Contrary to our expectation, the lysozyme positive cells were found only in the minority of cases with fibrohistiocytic lesions. Cells positive for lysozyme were found only in 13 out of 100 cases of dermatofibroma, one out of 4 cases of xanthogranuloma and 8 out of 33 cases of malignant fibrous histiocytoma. Dermatofibrosarcoma protuberans and non-fibrohistiocytic tumors were negative for lysozyme. It is suggested that in proliferative fibrohistiocytic lesions, induction of lysozyme synthesis is weak or absent. Some malignant fibrous histiocytomas showed scattered lysozyme positive neoplastic cells, indicating their probable histiocytic origin or differentiation. On the other hand, evidence of histiocytic differentiation of dermatofibrosarcoma protuberans was not obtained using lysozyme immunohistiochemistry.

Topics: Aged; Cytoplasmic Granules; Fibroma; Granuloma; Histiocytes; Histiocytoma, Benign Fibrous; Histocytochemistry; Humans; Immunoenzyme Techniques; Liposarcoma; Male; Middle Aged; Muramidase; Skin Diseases; Skin Neoplasms; Tuberculosis, Lymph Node

Histiocytosis X, multicentric reticulohistiocytosis, juvenile xanthogranuloma, the "fibrous" type of dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma are all characterized by dermal and/or subcutaneous infiltrates composed at least partially of cells having morphologic features suggestive of histiocytes. Paraffin-embedded tissues representing these conditions were stained for lysozyme (muramidase) with a peroxidase-antiperoxidase technic. The cells of juvenile xanthogranuloma were rich in lysozyme. Some of the cells of histiocytosis X showed a positive pattern, and the cells of the other three conditions were essentially negative. This study confirmed the histiocytic nature of juvenile xanthogranuloma and multicentric reticulohistiocytosis, supported the interpretation that there is a histiocytic component in the lesions of histiocytosis X, and cast some doubt on the alleged histiocytic nature of "fibrous" dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma.

Topics: Fibroma; Granuloma; Histiocytes; Histiocytoma, Benign Fibrous; Histiocytosis, Langerhans-Cell; Humans; Immunoenzyme Techniques; Lipoma; Lymphatic Diseases; Muramidase; Skin Diseases; Skin Neoplasms