glycogen and Soft-Tissue-Neoplasms

Clear-Cell Sarcoma (CCS) is a rare malignant soft tissue tumor of young adults that often metastasizes to the lymph nodes. The fine needle aspirate cytology (FNAC) findings of CCS have been described previously and it is included in the cytological category of polygonal soft tissue tumors. We describe the cytology of a case of CCS, with emphasis on the so called "tigroid background." Though this tigroid background is a nonspecific feature and has been described in various other tumors, to the best of our knowledge, it has not been described in CCS. This feature may be useful in the recognition of CCS.

Topics: Adolescent; Biomarkers, Tumor; Biopsy, Fine-Needle; Cytoplasm; Glycogen; Humans; Lymph Nodes; Male; Periodic Acid-Schiff Reaction; Sarcoma, Clear Cell; Soft Tissue Neoplasms

Topics: Biomarkers, Tumor; Cytoplasm; Female; Glycogen; Histiocytoma, Benign Fibrous; Humans; Immunohistochemistry; Leg; Microscopy, Electron; Middle Aged; Soft Tissue Neoplasms; Terminology as Topic

Recent reports of Ewing's sarcoma (EW) and extraskeletal Ewing's sarcoma (EEW) support the hypothesis that these tumors are neuroectodermal in origin. Primitive neuroectodermal tumors (PNET) of bone (32 cases) and soft tissue (25 cases) including those previously categorized as EW in 27 cases and EEW in 15 cases were carefully studied histologically, immunocytochemically and morphometrically, focusing on tumor cell differentiation. This study attempts to subclassify these tumors on the basis of the size of tumor cells and nuclei, their variations (uniformity or diversity), arrangement of tumor cells (rosette or non-rosette), focal differentiation to larger ganglion-like cells, and staining intensity for neural markers. All tumors were histologically subclassified as small, medium or large cell types, three basic subtypes (rosette type, abortive rosette type, non-rosette type) and four complementary subtypes (fibrillary type, non-fibrillary type, angiomatoid type, ganglion cell type). Classic EW or EEW is consistent with small or medium, non-rosette, non-fibrillary type tumors, previously described large cell EW with large, non-rosette, fibrillary or non-fibrillary type tumors, and classic neuroectodermal tumor with small or medium, rosette, fibrillary type tumors, according to the present subclassification. Clinicopathologic correlations with the different subtypes are discussed. Long-term survival, more than 5 years, was seen in patients with small cell type, and those younger than 14 years of age.

Topics: Adolescent; Adult; Bone Neoplasms; Child; Child, Preschool; Female; Follow-Up Studies; Glycogen; Humans; Infant; Male; Neoplasms, Germ Cell and Embryonal; Neurofilament Proteins; Phosphopyruvate Hydratase; Sarcoma, Ewing; Soft Tissue Neoplasms; Survival Rate

Liposarcoma falls into the differential diagnosis of myxoid malignant mesenchymal tumors. On the other hand, its relation with white or brown fat is controversial. Two cases of liposarcoma have been studied by organ culture, a method which provides cell and tissue redifferentiation in vitro. Both cases developed successively cytoplasmic glycogen granules and lipid droplets as well as a single lipidic vacuole in the late phase of cultivation as a marker of fat differentiation. Our results support the possibility of identifying myxoid liposarcomas as well as their origin from white fat tissue.

Topics: Adipose Tissue; Adult; Glycogen; Glycosaminoglycans; Humans; Leg; Liposarcoma; Male; Middle Aged; Organ Culture Techniques; Soft Tissue Neoplasms

We report the cytogenetic findings in a case of malignant melanoma of soft parts. Overrepresentation of 1q together with a del(1)(q42), extra copies of chromosomes 7 and 8, and t(12;22)(q13;q13) were found. These findings allow further delineation of the chromosomal pattern found in this uncommon neoplasm.

Topics: Adult; Chromosome Aberrations; Glycogen; Hand; Humans; Immunohistochemistry; Male; Melanoma; Reticulin; Soft Tissue Neoplasms

Topics: Adult; Glycogen; Humans; Immunohistochemistry; Male; Sarcoma, Ewing; Soft Tissue Neoplasms; Thoracic Neoplasms; Vimentin

A clear cell sarcoma that developed on the left back of a 19-year-old Japanese female was studied ultrastructurally. The findings included basal laminae, desmosomelike junctions, cytoplasmic interdigitations, lamellar bodies, and immature melanosomes. These findings suggest schwannian differentiation of this tumor, since they are not usual features of malignant melanoma except for melanosomes. Therefore this tumor seems to be more akin to malignant schwannoma than to malignant melanoma.

Topics: Adult; Cell Differentiation; Female; Glycogen; Humans; Melanoma; Microscopy, Electron; Sarcoma; Schwann Cells; Soft Tissue Neoplasms

The ultrastructural features of five pleomorphic rhabdomyosarcomas, five high-grade malignant fibrous histiocytomas, and five pleomorphic liposarcomas were studied. Electron microscopy was found to be consistently useful in distinguishing between these tumors. The rhabdomyosarcomas showed thick and thin filaments in complexes and consistently contained glycogen. The malignant fibrous histiocytomas had numerous lysosomes, often in cells with ruffled borders, and contained cells showing "myofibroblastic" differentiation. The liposarcomas showed abundant and coalescing lipid droplets, sparse stroma with condensation of amorphous granular materials surrounding plasma membranes, and prominent vascularity. Fourteen of the 15 tumors could be identified on the basis of ultrastructure; thus, electron microscopic examination is an important diagnostic tool for pleomorphic tumors.

Topics: Cytoskeleton; Endoplasmic Reticulum; Glycogen; Histiocytes; Histiocytoma, Benign Fibrous; Humans; Liposarcoma; Microscopy, Electron; Rhabdomyosarcoma; Soft Tissue Neoplasms

Forty-two cases of Ewing's sarcoma (ES) have been studied with light microscopy during the 9-year period 1974 to 1982. Thirty-three patients had ES of bone, and in 9 patients the tumor was located in the extraskeletal soft tissues. Cases which fulfilled all the morphologic criteria were accepted as typical ES (31 cases), and those with some architectural or cytologic peculiarities were considered atypical forms of ES (11 cases). An immunohistochemical study (PAP method) to evaluate the presence in the tumor cells of the following markers: myoglobin, F-VIII-related antigen, lysozyme, alpha-1-antitrypsin, alpha-1-antichymotrypsin, and immunoglobulins (IgG, IgA, IgM, kappa and lambda light chains), was performed with negative results in all cases (paraffin blocks were available in 38 cases). The cytochemical study on fresh tissue imprints from five patients (PAS, Sudan Black, alpha-naphthyl acetate esterase, acid phosphatase, beta glucuronidase, myeloperoxidases, naphthol-AS-D chloroacetate esterase and alkaline phosphatase) gave no pattern of histogenetic significance, PAS being the best morphologic marker in tissue sections and touch preparations. A detailed ultrastructural study was performed on 34 cases; the main findings may be summarized as follows: medium sized cells, polygonal shape, oval nuclei, smooth nuclear envelope, abundant euchromatin, well-developed nucleolonema, scant membranous organelles, abundant hyaloplasmic glycogen, occasional lipid vacuoles, straight cell membranes, and primitive intercellular junctions. No differences were found between bone and extraskeletal ES; moreover, typical and atypical forms showed moderate quantitative differences with no qualitative change. The histogenesis is discussed; no functional or morphologic markers have been found to suggest the cell of origin; however, some cell lines may be excluded. It is the impression of the authors that they are dealing with a primitive noncommitted mesenchymal cell.

Topics: Adolescent; Adult; Bone Neoplasms; Cell Membrane; Cell Nucleolus; Cell Nucleus; Child; Child, Preschool; Female; Glycogen; Histocytochemistry; Humans; Immunoenzyme Techniques; Male; Microscopy, Electron; Sarcoma, Ewing; Soft Tissue Neoplasms

A case of extraskeletal Ewing's sarcoma was studied ultrastructurally, cytologically, and cytochemically. This tumor was, as in that of osseous origin, composed of densely packed highly undifferentiated blastemic cells and so-called "darker cells." The principal tumor cells were undifferentiated cells, which were characterized by the scant cytoplasm containing a variable amount of glycogen granules, large round or oval nuclei with dispersed chromatin, and rudimentary cytoplasmic junctional complexes. Some of the darker cells seemed to show somewhat histiocytic differentiation from principal blastemic cells, but the others were degenerating. Cytochemically, no tumor cells revealed the presence of peroxidase, lysozymes, and Factor VIII-related antigen. It is likely that both Ewing's sarcomas of osseous and extraosseous origin are defined as undifferentiated sarcoma merely characterized by the presence of glycogen.

Topics: Adolescent; Antigens; Cytoplasmic Granules; Diagnosis, Differential; Factor VIII; Female; Glycogen; Humans; Immunoenzyme Techniques; Microscopy, Electron; Muscles; Sarcoma, Ewing; Soft Tissue Neoplasms; von Willebrand Factor

A clinico-pathological, light microscopic and immunohistochemical study of 15 clear-cell sarcomas, with an ultrastructural analysis of 6 of the tumors, is presented. The tumors showed a strong predilection for tendons and aponeuroses of the extremities in predominantly young and middle-aged people. The clinical setting, course and light microscopic appearance agree well with the original description by Enzinger (1965). Nine of the 15 patients developed metastases, most of them including lymph nodes, and 8 of the patients had died at the time of follow-up (median follow-up time 4.8 years). Reducing pigment was demonstrated within the cells of 2 tumors. Ultrastructurally the 6 tumors studied had a uniform appearance with characteristically rounded or oval tumor cells with a single nucleus containing one or two very prominent nucleoli, a light-staining cytoplasm with a moderate amount of organelles and a variable content of glycogen. Polymorphic melanosomes were seen in the cells of one of the tumors. External laminas enclosed groups of tumor cells and invested parts of individual tumor cells. With immunoperoxidase analysis for S-100 protein positive staining was observed in the vast majority of the tumor cells of all 15 clear-cell sarcomas. Metastases appearing in 9 of the 15 cases showed positive staining for S-100 protein. There was a strong staining of the cytoplasm and generally a weak and varying staining of nuclei. The immunohistochemical and electron microscopic findings indicate that clear-cell sarcoma is a homogenous entity among soft tissue sarcomas, of probable neural crest derivation.

Topics: Adolescent; Adult; Age Factors; Aged; Extremities; Female; Glycogen; Histocytochemistry; Humans; Immunoenzyme Techniques; Lymphatic Metastasis; Male; Melanocytes; Microscopy, Electron; Middle Aged; Nerve Tissue Proteins; S100 Proteins; Sarcoma; Soft Tissue Neoplasms; Tendons

The light-microscopic and ultrastructural findings in a case of so-called giant cell tumor of soft parts, localized at the dorsal side of the left foot of a 23-years-old male are described. An amputation of the lower extremity was performed and subsequently chemotherapy with adriamycin was given for 3 months. Despite the histology and cytologic malignant appearance and the evident vascular invasion, already present at the time of the first excision, the last known status 2 years later seems favorable. There are no pathologic lymph nodes in the groins and no signs of metastases on chest X-rays. From the electron-microscope study no definite conclusion can be drawn as regards the histogenesis of this tumor. we feel, as do others, that many of the principal mononuclear tumor cells are poorly differentiated mesenchymal cells. Some of the neoplastic cells, however, show ultrastructural features suggestive of chondro- or osteoblasts (a well-developed r.e.r. containing electron-dense material; multiple Golgi complexes; masses of glycogen; interdigitating cell membrane villi; cytoplasmic filaments; an extracellular amorphous matrix). Some of the larger tumor cells have the submicroscopic aspects of histiocytes as described in osseous, cutaneous, or pulmonary lesions of the histiocytosis X group. As yet undetermined cytoplasmic inclusion bodies constitute another rare observation in our material.

Topics: Adult; Amputation, Surgical; Cell Membrane; Doxorubicin; Endoplasmic Reticulum; Foot; Giant Cell Tumors; Glycogen; Golgi Apparatus; Humans; Inclusion Bodies; Male; Soft Tissue Neoplasms