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L G Kindblom

Publications and source records attributed to L G Kindblom.

At least 19 recordsLinked to original sources

Metastasizing gastric epithelioid leiomyosarcomas (leiomyoblastomas) in young individuals with long-term survival.

RESULTS. Four patients with metastasizing epithelioid leiomyosarcoma of the stomach, three females and one male (15, 20, 22, and 25 years of age, respectively), are reported. Two patients had recurrent tumors in the gastric remnant. Liver metastases occurred in all patients, three of whom had lymph node metastases; two had peritoneal metastases. The patients are alive at 17, 19, 27 and 48 years after the diagnosis was made and 17-27 years after the first demonstration of metastases. One woman had multiple chondromatous hamartomas of the lung. Operations were performed when the patients had symptoms and during periods of no symptoms, as directed by the second-look principle. One patient had 10 operations. None of the patients received adjuvant therapy. All three women have given birth to healthy children after metastases were diagnosed. Three of the primary tumors were large (10-20 cm) and multinodular, features that have been associated with unfavorable prognosis. The four primary tumors had a similar light microscopic appearance, characterized by moderate cell and nuclear pleomorphism and low mitotic activity, 0.03-0.1/mm2. Ultrastructurally, a network of intermediate filaments was found within the cytoplasm of the tumor cells corresponding to the immunohistochemical positivity for vimentin. The immunohistochemical findings (negative immunoreaction for desmin and alpha smooth-muscle actin) and the ultrastructural analysis produced no evidence of the production of smooth-muscle cell myofilaments. However, there were tumor cells with an abundance of mitochondria and a paucity of filaments, features that may be characteristic of epithelioid leiomyomatous tumors. In addition, immunohistochemical negativity for cytokeratins, epithelial membrane antigen, S-100 protein, neuron-specific enolase, and chromogranin militate against an epithelial or neuroectodermal cell differentiation. CONCLUSIONS. An awareness of this type of gastric leiomyosarcoma in children and young adults is of importance in making correct assessments of prognosis and choosing an active therapeutic approach. The biologic background to the clinical behavior of these metastasizing tumors remains an enigma. Additional studies are needed to elucidate the biology of these tumors.

Adolescent

Reciprocal translocation t(12;22)(q13;q13) in clear-cell sarcoma of tendons and aponeuroses.

Clear-cell sarcoma is a rare soft tissue sarcoma that displays certain similarities to malignant melanoma. In this paper we describe the karyotypic findings and in vitro growth characteristics of a short-term-cultured clear-cell sarcoma. The cultured tumor cells had preserved immunohistochemical characteristics and certain ultrastructural features of the primary tumour, including positivity for vimentin, S-100 protein, and a melanoma-associated antigen, supporting the authenticity of the cultured cells. Cytogenetic analysis revealed an abnormal stemline karyotype of 49,XY, -1, +8, +8, +12, +der(1)t(1;?)(p36.1-.3;?), t(12;22)(q13;q13). A similar or identical t(12;22) was recently reported in two of four clear-cell sarcomas. It is suggested that the t(12;22)(q13;q13) is a primary cytogenetic abnormality in clear-cell sarcoma and distinguishes this tumor type from malignant melanoma.

Adult

Characterization of four melanoma cell lines with electron microscopy, immunocytochemistry, cytogenetics, flow cytometry, and southern analysis.

Four cell lines established from human metastatic malignant melanoma, derived from four patients, were analyzed. Ultrastructurally and immunocytochemically, the cultured tumor cells had retained characteristic features of melanocytes and of the primary malignant melanomas. The genetic stability was investigated by repeated flow-cytometric and cytogenetic analyses over 24 months of continuous cultivation. The DNA indices ranged from 1.7 to 2.1 and were stable during the entire period. The same was true for the karyotypes, which had modal numbers ranging from 50 to 84. The most common types of abnormalities were: isochromosomes i(1q), i(9q), translocations (1;17) and (3;6), and other aberrations (1p+,4p+,5p+,11p+,11q-,11q+). Abnormalities involving chromosome 1 were present in all cell lines, but loss of genetic material from chromosome 1p was demonstrated in only one of four cell lines when tested by the Southern blotting technique using a lambda MS1 probe.

Adult

Plexiform fibrohistiocytic tumor. Report of a case involving preoperative aspiration cytology and immunohistochemical and ultrastructural analysis of surgical specimens.

A typical case of plexiform fibrohistiocytic tumor (Enzinger and Zhang) occurring in the skin and subcutis of the abdominal wall in a 7-year-old girl is reported. Preoperative fine-needle aspiration cytology revealed a benign lesion with fibroblastic-histiocytic features which also contained bi- and multinucleated giant cells. The surgical specimen showed a tumor with multiple small nodules within fibrous septa; these nodules were composed of spindle cells and epithelioid cells and contained scattered multinucleated osteoclast-like cells. The tumor cells showed ultrastructural and immunohistochemical features of myofibroblasts and histiocyte-like cells. Thus, there was an abundance of lysosomes, prominent filopodia and bundles of thin cytofilaments along the cytoplasmic border, as well as immunoreactivity for alpha-smooth-muscle-specific actin, alpha-1-antitrypsin and alpha-1-antichymotrypsin. Ultrastructurally there were tumor cells exhibiting features of histiocytes which also contained bundles of actin of smooth muscle type. The presented case of plexiform fibrohistiocytic tumor appears to be composed of a rather peculiar cell form, somewhere between myofibroblasts and histiocytes.

Abdominal Neoplasms

Proliferative myositis and fasciitis. A light and electron microscopic, cytologic, DNA-cytometric and immunohistochemical study.

Two cases of proliferative myositis, four cases of proliferative fasciitis and one mixed form of proliferative myositis and fasciitis have been analyzed in terms of cell differentiation and DNA content. Light microscopically, the lesions were characterized by a mixture of proliferating spindle-shaped cells and uni-, bi- or occasionally multinucleated ganglion cell-like cells. The spindle cells showed ultrastructural features and immunohistochemical properties, including an immunoreactivity for smooth muscle-specific actin, indicative of a myofibroblastic differentiation. The ganglion cell-like cells displayed some resemblance to active osteoblasts ultrastructurally and differed immunohistochemically from the spindle cells by being non-immunoreactive for smooth muscle-specific actin. None of the two cell types showed immunoreactivity for desmin, myoglobin or factor VIII RAG. It is suggested that the two cell types represent different lines of cell differentiation. The cytologic features in smears, as seen in two cases of proliferative fasciitis and one case of proliferative myositis, are considered to be characteristic of these lesions and to permit the diagnosis to be made by fine-needle aspiration. In two of the cases, the lesion was diagnosed only cytologically and thereafter disappeared spontaneously within a month. Cytometric DNA measurements, using two different image analysis systems on Feulgen-stained sections and smears, revealed a "diploid" spindle-shaped cell population with a variable proportion of cells with scattered DNA values. The ganglion cell-like cells differed from the spindle cells by having a broad DNA peak in the diploid region and additional peaks in the tetraploid region, as well as a higher proportion of cells with scattered DNA values compared with those of the spindle-shaped cells. The results of the quantitative DNA analysis are well in keeping with the benign and proliferative nature of these lesions. However, with the technique used here, quantitative DNA analysis, does not distinguish these pseudosarcomatous fibrous lesions from diploid and tetraploid soft tissue sarcomas.

Actins

Expression of different cytokeratin subclasses in human chordoma.

A detailed immunohistochemical characterization of different cytokeratin subclasses was performed on frozen tumour tissue from three classical chordomas. Simple epithelium cytokeratins Nos 8, 18, and 19 were detected in all tumour cells while cytokeratin No. 7 was not found. Cytokeratins characteristic of squamous differentiation, including keratinization, were generally lacking, with the exception of the varying expression of cytokeratin No. 4. Vimentin was found in all the tumours, while they lacked desmin immunoreactivity. The present study indicates the co-expression of vimentin and cytokeratins, predominantly of the simple epithelium type. In addition, chordoma cells have the ability to express cytokeratins characteristic of squamous differentiation. This finding corresponds well to the electron microscopic findings of tonofilament bundles ending in well-developed desmosomes.

Antibodies, Monoclonal

Morphological and cytogenetic studies of angiosarcoma in Stewart-Treves syndrome.

A morphological and cytogenetic analysis of a multifocal angiosarcoma in a typical case of Stewart-Treves syndrome is reported. The morphological analysis indicated differentiation along both blood and lymph vessel endothelium lines. By light and electron microscopy there were areas with well-developed erythrocyte-containing, capillary-like vessels and poorly differentiated areas with abortive vascular formations. In these the endothelium revealed immunoreactivity to factor VIII RAg, binding of Ulex europaeus I and Psophocarpus tetragonolobus agglutinin lectins, Weibel-Palade bodies ultrastructurally and presented a continuous enclosing external lamina and immunoreactivity for laminin and collagen IV, all features of blood-vessel differentiation. There were also lymphangioma-like areas as well as poorly differentiated areas where the immunohistochemical, lectin-binding and ultrastructural features were compatible with a lymph vessel differentiation. Cytogenetic analysis of cultured tumour cells revealed chromosome counts in the diploid region. About 40% of the cells analysed had a normal diploid karyotype. The remaining cells showed a multitude of mainly nonclonal structural alterations; 17 unique marker types resulting from different translocations and deletions were observed. There were also a few cells with clonal numerical deviations showing monosomy 22, monosomy X and trisomy 2 respectively. It is of interest that the losses of chromosome 22 and the X chromosome also have been observed in Kaposi's sarcoma and that the PD-ECGF gene, a novel angiogenetic factor, has been mapped to chromosome 22.

Adult

Fine-needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical, and immunocytochemical examination.

The cytologic findings in a series of 17 patients with chordoma (one clivus, three cervical, one lumbar, and 12 sacral tumors), all of whom underwent fine-needle aspiration biopsy (FNAB) in the preoperative investigation, were studied. Cytologically, three main cell types were recognized: large, mononucleated or binucleated physaliferous cells with a vacuolated "bubbly" cytoplasm; small, rounded, uniform cells; and short spindle-shaped cells. The May-Grünwald-Giemsa staining was found superior to Papanicolaou staining in demonstrating the mucoid matrix and the vacuolated cytoplasm of the physaliferous tumor cells. Ultra-structurally, the tumor cells contained prominent bundles of filament of the intermediate type, as well as large vacuoles and lumina often bordered by microvillous projections; the cells were connected to one another by well-developed desmosomes. The resin embedding technique for the light and electron-microscopic examination of FNAB material (eight cases), the histochemical demonstration of sulphated glucosaminoglycans in the matrix (four cases), and the immunocytochemical analysis (four cases) with positivity for cytokeratin, epithelial membrane antigen, vimentin, and S-100 protein and negativity for carcinoembryonic antigen were found to be of value for the cytologic diagnosis of chordoma, and helped in distinguishing it from other chondrogenic tumors and metastatic mucous-producing carcinoma. From this study we conclude that a preoperative diagnosis of chordoma can be reached by FNAB, provided the findings are carefully evaluated in relation to the clinical and roentgenographic findings. Adjunctive histochemical, immunocytochemical, and ultrastructural techniques applied on the FNA material may be helpful in reaching a conclusive diagnosis when differential diagnostic problems occur.

Adult

Clonal chromosome abnormalities and in vitro growth characteristics of classical and cellular schwannomas.

Cytogenetic analysis was performed on 12 schwannomas, 10 classical and two of the cellular type. Electron microscopic examination of cultured cells from two classical and two cellular schwannomas revealed features compatible with a Schwann cell differentiation. Immunohistochemical analysis of the cultures demonstrated immunoreactivity for S-100 protein in 6 of 6 cases and for vimentin in 2 of 2 cases of classical schwannoma, thus further supporting the authenticity of the cultured cells. Cultured cells from one cellular schwannoma also exhibited immunoreactivity for glial fibrillary acid protein (GFAP). Cytogenetic analysis of the 12 tumors revealed clonal abnormalities in 10 of the cases. In the majority of tumors, cells with a normal karyotype coexisted with different abnormal clones and sporadic deviations. Numerical changes predominated and were found in all tumors, while structural rearrangements were detected in eight tumors. The most common abnormality was clonal or sporadic loss of chromosome 22, which was found in all cases. Other clonal abnormalities included loss of one sex chromosome found in five cases, -15 found in three cases, and -12, +5, +7, +20 found in two cases each. These results provide further evidence in support of a role for loss of chromosome 22 in the pathogenesis of schwannomas. Our findings also indicate that there exist several different evolutionary pathways for schwannomas, and that some of these are shared by several other types of benign and malignant tumors of the nervous system.

Adult

Classical and chondroid chordoma. A light-microscopic, histochemical, ultrastructural and immunohistochemical analysis of the various cell types.

The aim of the present investigation was to characterize the various cell types of classical and chondroid chordomas. Eight cases of classical chordoma, 1 case of sacrococcygeal chordoma with chondroid areas and 2 cases of spheno-occipital chondroid chordoma were studied. Ultrastructurally and immunohistochemically (immunoreactivity for cytokeratins, epithelial membrane antigen [EMA], tissue polypeptide antigen [TPA] and human milk fat globule protein [HMFG]) the 3 cell types (physaliferous, epithelial-like, and spindle-shaped) recognized light-microscopically presented features of epithelial differentiation and rather formed a continuous spectrum than being distinct cell types. The chondroid areas of the chondroid chordomas had similar ultrastructural and immunohistochemical properties except for the lack of immunoreactivity for EMA and HMFG. The results of the critical electrolyte concentration technique according to Scott and Dorling indicated that there was no difference in the sulfated glycosaminoglycan content between classical and chondroid chordomas: all the tumors contained chondroitin sulfate. The presence of chondroitin sulfate, immunoreactivity for vimentin and S-100 protein and areas of cartilaginous differentiation in three cases indicate a relationship both to chondromatous tumors and to normal notochord, from which chordoma is believed to originate.

Adult

Intermediate and fine cytofilaments in cutaneous and subcutaneous leiomyosarcomas.

The expression of fine and intermediate cytofilaments in 10 cutaneous and seven subcutaneous leiomyosarcomas was studied immunohistochemically. All the tumors contained tumor cells which showed a positive immunoreactivity for desmin in formaldehyde-fixed and paraffin-embedded sections, but none of the seven anti-desmin antibodies used alone produced a distinct positive staining in all the tumors. A lack of correspondence in terms of immunoreactivity between tumor cells and the supposed muscle of origin was observed, especially in the subcutaneous leiomyosarcomas. In all cases, antibodies to muscle-specific and smooth muscle-specific actin were found to produce a positive staining in both the tumors and the supposed muscle of origin. Vimentin was detected in 8/10 cutaneous and 4/7 subcutaneous leiomyosarcomas, while the supposed muscle of origin was positive in 3/10 and 7/7 cases, respectively. Four of the cutaneous and three of the subcutaneous leiomyosarcomas contained tumor cells which stained positively for cytokeratins, while the supposed muscle of origin showed no positivity. It thus appears that a phenotypic shift in terms of vimentin and cytokeratin expression occurs in the tumor cells of cutaneous and subcutaneous leiomyosarcomas compared with the supposed muscle of origin. It is recommended that more than one monoclonal anti-desmin antibody is used to characterize these tumor entities. It is also concluded that the immunoreactivity for muscle-specific actins in superficial leiomyosarcomas is more constant, although less specific, than that of desmin and that the demonstration of the simultaneous expression of muscle-specific actins and desmin is helpful.

Actin Cytoskeleton

A cell culture, chromosomal and quantitative DNA analysis of a metastatic epithelioid sarcoma. Deletion 1p, a possible primary chromosomal abnormality in epithelioid sarcoma.

The chromosomal banding pattern and the in vitro growth characteristics of a metastatic epithelioid sarcoma are described. The cultured tumor cells retained growth characteristics as well as ultrastructural and immunohistochemical properties similar to the cells of the primary tumor. Cytogenetic analysis revealed a modal range in the diploid-hypodiploid region, a finding which was corroborated by quantitative DNA determinations of both the primary tumor and a lymph node metastasis. Fourteen different marker chromosomes were identified. The most frequent clonal rearrangement was a 1p-marker resulting from a short arm terminal deletion, i.e., del (1) (p21-22). A similar 1p- marker has previously been observed in an established epithelioid sarcoma cell line. The finding of an apparently identical 1p-marker in two of two analyzed epithelioid sarcomas suggests that this rearrangement may be a primary cytogenetic abnormality in epithelioid sarcoma. An elevated ras p21 expression was demonstrated using immunohistochemical methods. The possible involvement of the N-ras gene and/or a tumor suppressor in the 1p deletion is considered.

Chromosome Aberrations

Light and electron microscopic examination of fine-needle aspirates in the preoperative diagnosis of osteogenic tumors: a study of 21 osteosarcomas and two osteoblastomas.

Twenty-three patients with primary osteogenic tumors, two osteoblastomas and 21 osteosarcomas, underwent fine-needle aspiration biopsy in the preoperative investigation. The cytologic features in smears were compared with the light and electron microscopic findings of resin-embedded fine-needle aspirates (16 cases) and the histopathologic findings of the surgical specimens. Cytologic smears clearly indicated a primary bone malignancy in all 20 high-malignancy osteosarcomas. In the osteoblastic osteosarcomas, the diagnosis of osteosarcoma was strongly suggested by the osteoblastic appearance of the tumor cells and the presence of an osteoid-like fibrillar matrix in 6 of 11 cases in the May-Grünwald-Giemsa-stained smears. The distinction of chondroblastic osteosarcomas from high-grade chondrosarcomas and of polymorphic osteosarcomas from malignant fibrous histiocytomas is difficult in smears. Electron microscopic examination and immunocytochemistry indicating a positivity for vimentin but not for cytokeratin helped to exclude the possibility of a carcinoma in osteoblastic osteosarcomas where the tumor cells had epithelium-like features in the smears. The resin-embedding technique of fine-needle aspirates was useful for the typing since it gave additional light microscopic information about growth pattern, structure, and matrix production, as well as ultrastructural information about cell and tissue differentiation. The osteoid-like material seen in May-Grünwald-Giemsa-stained smears could be proven to represent osteoid with varying degrees of calcification at the ultrastructural examination. The fine-needle aspiration technique, especially when used for both smears and embedding, may play an important role in the preoperative investigation of bone tumors, provided that it is used with full knowledge of the clinical and radiographic findings.

Adolescent

Light and electron microscopic examination of fine-needle aspirates in the preoperative diagnosis of cartilaginous tumors.

Twenty-eight patients with chondrogenic tumours--2 chondroblastomas, 4 chondromas, 18 chondrosarcomas, 1 clear-cell chondrosarcoma, and 3 mesenchymal chondrosarcomas--underwent fine-needle aspiration biopsy (FNAB) in the preoperative investigation. The cytologic features in smears were compared with the histopathologic findings in the surgical specimens; in 14 cases they were also compared with the light and electron microscopic findings in resin-embedded fine-needle aspirates. The smears of the vast majority of the classical chondrosarcomas presented features that made possible the FNAB diagnosis of a chondrogenic tumor to be made. In the case of the low-grade chondrosarcomas in particular, which were poorly or moderately cellular in smears and showed chondroblastic cells often in lacunary structures of hyaline matrix, consideration of the clinical presentation, size, location, and roentgenographic appearance was essential for the diagnosis of chondrosarcoma. On the other hand, the high-grade chondrosarcomas presented cytologic features that clearly indicated their malignancy and they usually had a myxoid matrix. The possible differential diagnoses that may arise from the FNAB diagnosis of cartilaginous tumors are discussed. The resin-embedding technique for the light and electron microscopic examination of FNABs, along with the histochemical analysis for the demonstration of sulphated glucosaminoglycans and the immunocytochemistry applied to smears, was found to be of value in the definite diagnosis, especially in the distinction of chondrogenic tumors from chordoma and metastatic mucous-producing carcinoma.

Adolescent

Metastases of malignant melanoma simulating soft tissue sarcoma. A clinico-pathological, light- and electron microscopic and immunohistochemical study of 21 cases.

Metastases of cutaneous malignant melanoma (MM) of ordinary type can resemble various types of soft tissue sarcoma light microscopically to a degree which has not been previously recognized. Twenty-one cases are described, in which the tumours were originally diagnosed as a soft tissue sarcoma. Seven tumours were predominantly of blue and spindle-cell, fascicular type, resembling malignant peripheral nerve sheath tumour and at times monophasic synovial sarcoma. Ten tumours which were of fascicular and predominantly storiform type, and included uni- and multi-nuleated pleomorphic cells resembled malignant fibrous histiocytoma. Due to the presence of multivacuolated lipoblast-like tumour cells, 2 of these 10 tumours resembled pleomorphic liposarcoma. One had a predominantly myxoid and hypocellular appearance and 5 additional tumours included such areas. The diagnoses were revised after ultrastructural examination with the demonstration of melanosomes in 13 of 16 studied cases and the immunohistochemical demonstration of positivity using anti-S-100 protein antibodies and the anti-melanoma antibody NKI/C3 in all cases. The anti-melanoma antibody HMB 45 gave a positivity in 9 of 21 cases. Light microscopically, sparse amounts of melanin were noted in 7 tumours using the Whartin-Starry technique. Eleven tumours occurred at sites close to major lymph node groups and in 9 of these cases, lymphoid tissue was associated with the tumours, suggesting that they represented lymph node metastases. Following a review of the patients' clinical histories and renewed clinical examination, primary cutaneous MM was demonstrated in 10 of 21 patients and in 1 case an MM in regression was detected. The origin of the 10 tumours without a detected primary is discussed, including the possibility of an overlooked primary, spontaneous regression of a primary and a de novo origin from lymph nodes and soft tissues.

Adolescent

Cellular schwannoma. A clinicopathologic study of 29 cases.

A series of 29 cellular schwannomas is described in terms of their clinical presentation and course, light and electron-microscopic appearance, immunohistochemical properties and cytogenetics. The study indicates that cellular schwannoma can be defined as a subtype of classical schwannoma, characterized by spindle cells forming a compact fascicular, sometimes fibrosarcoma-like growth pattern, a low mitotic activity, a generally moderate nuclear and cellular polymorphism and a high degree of Schwann cell differentiation as seen by electron microscopy and immunohistochemistry. The tumour is characteristically located close to the vertebral column, in the mediastinum or retroperitoneum and has a benign course. Occasionally bone destruction and neurological symptoms develop. The clinical appearance together with the high cellularity, fascicular pattern and mitotic activity had led to the erroneous diagnosis of a soft tissue sarcoma in a few cases, and cellular schwannoma may thus be considered to be a pseudosarcoma. Immunohistochemically, cellular schwannomas appear to deviate from classical schwannomas and malignant peripheral nerve sheath tumours by their expression of glial fibrillary acidic protein. The chromosome analysis revealed a normal diploid stemline karyotype, with a variety of abnormal clones, including one with monosomy 22.

Adolescent

Expression of muscle-specific actins and myosin in light microscopically undifferentiated small and dark cell malignancies of soft tissues.

Eighteen light microscopically undifferentiated small and dark cell malignancies, previously studied ultrastructurally and immunohistochemically in terms of desmin, vimentin and myoglobin expression, were analyzed using mono- and polyclonal antibodies to muscle-specific isoforms of myosin and actin. For comparison, 10 characteristic rhabdomyosarcomas, 5 alveolar and 5 embryonal, were included in the study. A polyclonal antibody to skeletal myosin produced an indistinct staining in all the alveolar and embryonal rhabdomyosarcomas. The staining was most prominent in well-differentiated rhabdomyoblastic tumor cells. In the analysis of the small and dark cell malignancies, this antibody produced a weak and indistinct positivity which can not be interpreted with certainty as an expression of muscle-specific properties. An antibody directed at the alpha and gamma isoforms of actin, which are present in smooth and striated muscle, produced a distinct positive staining in all the alveolar and embryonal rhabdomyosarcomas and in 8/18 small and dark cell malignancies, 7 of which were also shown to express desmin. An antibody directed at the alfa-smooth muscle isoform of actin did not produce any positive staining in any of the tumors. The present study indicates that both muscle-specific actin and desmin can be expressed in tumors lacking ultrastructural evidence of a rhabdomyoblastic differentiation and that the combined use of monoclonal antibodies to desmin and muscle-specific actin is of value when it comes to recognizing rhabdomyosarcomas within the group of undifferentiated small and dark cell malignancies of soft tissue tumors.

Actins

Surgery for soft tissue sarcoma in the extremities. A multivariate analysis of the 6-26-year prognosis in 137 patients.

In 137 patients with soft tissue sarcomas in the extremities, the influence of patient and tumor characteristics and surgical procedures on prognosis was studied using a multivariate statistical analysis. The minimum follow-up time was 6 years, and no patient was lost to follow-up. Eighty-nine patients were referred with the tumor intact (primary series), while 48 were referred after biopsy or with local recurrence after previous surgery (secondary series). The frequency of amputation was 15 percent in the primary series and 48 percent in the secondary series. A local, function-preserving operation with a wide margin was performed in 37 patients without biopsy (clinical diagnosis alone). Local control of the disease was obtained in all but one of these patients without any adjunctive treatment. Independent, unfavorable factors concerning local recurrence were advancing age, open biopsy, and marginal surgery. Independent, unfavorable factors concerning survival were advancing age, increasing histologic malignancy grade, and ablative surgery. A tumor-related death was observed in 0, 29, 47, and 67 percent of the patients with tumors of malignancy grades I, II, III, and IV, respectively.

Adolescent