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PubMed1975

Thorotrast-induced extraskeletal osteosarcoma of the cervical region. Report of a case.

This is the seventh reported case of soft tissue sarcoma attributed to thorotrast extravasated during the course of an injection for arteriography. An extraskeletal osteosarcoma occurring the cervical region of a 51-year-old man 30 years after a cerebral arteriogram is presented. The other reported cases include a fibrosarcoma, two neurofibrosarcomas, two spindle cell sarcomas, and an extraskeletal chondrosarcoma. The latency period has varied from 5 to 30 years. The thorotrast granules are readily identified in macrophages near or adjacent to the tumor, and the demonstration of alpha tracks on autoradiographs is diagnostic.

Humans↗

PubMed1981

Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas.

From January 1963 through December 1977, 300 adults with soft-tissue sarcomas were treated by a conservative surgical excision and postoperative radiotherapy. The absolute two- and five-year disease-free survival rates are 74% (222/300) and 61.3% (103/168), respectively. The five-year survival rate varies with: (1) anatomic site, e.g., 69.4% (75/108) for extremity lesions vs. 33% (5/15) for abdominal lesions; (2) histopathologic diagnosis, e.g., 86.4% (19/22) for fibrosarcoma vs. 50% (15/30) for neurofibrosarcoma; and (3) stage of the lesion. The overall local recurrence rate was 22.3% (67/300) and 27% (81/300) of the patients developed distant metastases. The incidence of lymph node metastases as an initial site of spread was only 2.7% (8/300); therefore, elective treatment of the regional lymphatics is not indicated. The 6.5% incidence of significant complications in extremities is low and might be further diminished by careful treatment planning. The combination of conservative surgery and postoperative radiation therapy maintains a functional limb in 84.5% (169/200) of patients with extremity lesions. This rate is comparable to the five-year survival rate attained with radical surgery.

Abdomen↗

PubMed1984

Sarcoma of bone and soft tissue following treatment of Hodgkin's disease.

Intensive chemotherapy and radiotherapy of Hodgkin's Disease has resulted in improved survival rates. With long-term follow-up, however, a risk of secondary malignancy in these individuals is now appreciated. The authors of this report have encountered five patients who developed bone or soft tissue sarcomata more than 5 years after treatment of Hodgkin's Disease. The four males and one female ranged in age from 14 to 74 years at the time of diagnosis of Hodgkin's disease. Two had received radiotherapy alone for treatment of Hodgkin's disease, two were treated with radiation and chemotherapy, and one received only chemotherapy. The latent period prior to diagnosis of sarcoma ranged from 6 to 11 years. There was one case each of neurofibrosarcoma, fibrosarcoma, osteosarcoma, liposarcoma and leiomyosarcoma. Four patients died within 1 year of the diagnosis of sarcoma. One is alive with no evidence of disease 2 years following diagnosis and surgical excision of the sarcoma. On the basis of the Massachusetts General Hospital experience in the treatment of Hodgkin's Disease, the authors calculate a risk of 0.9% of sarcoma occurring in five year survivors of Hodgkin's disease. Previously reported cases of sarcoma following treatment of Hodgkin's disease are summarized. The pertinent literature is reviewed.

Adult↗

PubMed1987

Lymph nodes as sites of metastases from sarcomas of soft tissue.

Records of 323 patients with TNM Stage M0 sarcoma of soft tissue treated by the Radiation Medicine Service of the Massachusetts General Hospital over a 14-year period were reviewed to study the incidence and the implication of regional lymph node involvement. Nineteen patients (5.9%) had evidence of sarcoma metastatic to draining lymph nodes, zero of 63 (0%) were Grade 1 sarcomas, two of 118 (2%) were Grade 2, and 17 of 142 (12%) were Grade 3 sarcomas. Among patients with Grade 3 sarcomas, rhabdomyosarcoma (five of 14), vascular sarcoma (two of five), and epithelioid sarcoma (four of five) were associated with a higher incidence of lymph node involvement than synovial sarcomas (zero of four), fibrosarcomas (zero of 16), malignant fibrohistiocytomas (one of 29), neurofibrosarcomas (one of eight), liposarcomas (one of 14), and leiomyosarcomas (one of ten). From the 19 patients who had evidence of metastatic nodes, six (32%) were alive more than 58 months after the treatment of the nodes; four of six patients were without further tumor. The data of this study are compared with those cited in a review of the literature.

Humans↗

PubMed1990

Somatostatin-producing duodenal carcinoids in patients with von Recklinghausen's neurofibromatosis. A predilection for black patients.

Eight patients with von Recklinghausen's disease (VRD) and duodenal carcinoids are presented. Seven patients were black, and one white. Six of the eight were women. The presenting symptom was either jaundice or abdominal pain. All tumors were located in the second portion of the duodenum, and three were multiple. Associated tumors other than neurofibromas included multiple leiomyomas, meningioma, neurofibrosarcoma, and prostatic sarcoma. Seven tumors had psammoma bodies, and in three they were numerous. Somatostatin-positive cells were demonstrated in all cases. Two tumors had spread to regional lymph nodes at the time of surgery. There appears to be a predilection for black patients among those with VRD and duodenal carcinoids.

Adult↗

PubMed1992

Primary sarcomas of the heart.

Seventy-five primary sarcomas of the heart were classified by histologic appearance as angiosarcoma (26 cases), undifferentiated sarcoma (18 cases), osteosarcoma (9 cases), fibrosarcoma (6 cases), malignant fibrous histiocytoma (6 cases), leiomyosarcoma (4 cases), myxosarcoma (3 cases), synovial sarcoma (2 cases), and neurofibrosarcoma (1 case). The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy. Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate. Immunostaining with commercially available antisera was useful in the diagnosis of sarcoma but not in subclassification of 19 tumors so tested. Although the prognosis for patients with cardiac sarcomas is dismal, histologic grading is useful in predicting outcome, as has been shown for soft tissue sarcomas of other sites.

Adolescent↗

PubMed1986

Glial growth factor-like activity in Schwann cell tumors.

To determine whether glial growth factor (GGF)-like activity is associated with human Schwann cell tumors, we tested extracts from these tumors for their ability to stimulate the proliferation of rat Schwann cells in culture. Extracts from 3 of 3 bilateral acoustic neuromas and from 5 of 7 unilateral acoustic neuromas demonstrated dose-dependent stimulation similar to that of partially purified bovine pituitary GGF. One neurofibroma also contained high levels of GGF-like activity, one demonstrated an intermediate level, and three showed low or no activity. Minimal activity was found in one neurofibrosarcoma and in one trigeminal schwannoma. Non-Schwann cell tumors studied included 3 meningiomas, 2 pituitary adenomas, 1 cerebellar astrocytoma, 1 glioblastoma, 1 hemangioblastoma, and 2 metastatic brain tumors. The cerebellar hemangioblastoma demonstrated high GGF-like activity; the others showed little or no activity. Normal tissues used as control specimens included brain, peripheral nerve, muscle, and fat. Some activity was noted in one nerve biopsy; all others showed minimal or no GGF-like activity. High-performance liquid chromatography demonstrated that the GGF-like activity from two acoustic neuromas eluted in a single peak close to that of bovine pituitary GGF. We conclude that acoustic neuromas contain a factor that is closely related to bovine pituitary GGF and that this factor may have a role in the abnormal proliferation of Schwann cells in these tumors.

Animals↗

PubMed1991

Molecular and cytogenetic analysis of tumors in von Recklinghausen neurofibromatosis.

Von Recklinghausen neurofibromatosis (NF1) is a common autosomal dominant disorder mapped to 17q11.2 and typically characterized by the occurrence of neural crest-derived tumors. The gene has recently been cloned using reverse genetics or "positional cloning" approaches. Its function, however, remains unknown. We have performed cytogenetic and molecular analyses on 9 malignant tumors from NF1 patients to look for loss of alleles or chromosome rearrangements involving chromosome 17 to test the hypothesis that the NF1 gene acts as a recessive "tumor suppressor" gene. Loss of alleles on this chromosome was detected for 3 of 9 malignant tumors. Two peripheral nerve sheath tumors showed allele loss at informative loci on both the long and short arms of chromosome 17. In contrast, a glioblastoma with focal gliosarcoma showed loss of heterozygosity on the short arm of chromosome 17 only, and not at loci on the long arm. One nerve sheath tumor was previously shown by direct sequence analysis to have a point mutation at the TP53 locus at 17p13. These data support a role for the TP53 gene or other genes on the short arm of chromosome 17 in at least some malignancies in NF1. Six other neurofibrosarcomas showed no allele loss at informative loci on chromosome 17. Cytogenetic analysis was performed on 7 tumors, including 2 with allele loss. The two tumors with allele loss showed abnormal karyotypes while all others were normal. Southern blot and pulsed-field gel analysis using probes within or closely linked to the NF1 locus detected no gross deletions or rearrangements in the tumors studied.(ABSTRACT TRUNCATED AT 250 WORDS)

Alleles↗

PubMed1992

Loss of NF1 alleles in phaeochromocytomas from patients with type I neurofibromatosis.

Type I neurofibromatosis (NF1) is a common autosomal dominant disorder that affects tissues derived from the neural crest. The manifestations are varied, comprising generalised disorders of growth and development as well as an increased risk of benign and malignant tumours including phaeochromocytomas and neurofibrosarcomas. The NF1 locus has been mapped to chromosome bands 17q11-12, and recently the NF1 gene has been cloned. Deletions identified in the constitutional genotype of some patients have suggested that the NF1 phenotype may arise from loss of function mutations of the NF1 gene, consistent with the hypothesis that it is a tumour suppressor gene. To date, however, analysis of NF1 tumours has not revealed the frequent allele losses encompassing the NF1 locus, implying loss of the wild-type NF1 allele, which would support this hypothesis. We report allele losses with markers flanking the NF1 region in each of 7 NF1 phaeochromocytomas. In each of the 3 tumours for which this could be determined, the loss involved the wild-type chromosome. These results provide strong evidence that, in cells of the adrenal medulla at least, the NFI gene may act as a tumour suppressor.

Adrenal Gland Neoplasms↗

PubMed1994

Loss of neurofibromin in adrenal gland tumors from patients with neurofibromatosis type I.

The neurofibromatosis type I gene encodes a protein, neurofibromin, which may function as a tumor suppressor gene product. Recent studies have demonstrated loss of neurofibromin in tumors from NF1 and non-NF1 patients, including neurofibrosarcomas, neuroblastomas and malignant melanomas. Since neurofibromin is expressed in the adrenal gland, six pheochromocytomas and one adrenal cortical tumor were examined for neurofibromin expression. In all seven tumors, no neurofibromin could be detected. Furthermore, loss of heterozygosity (LOH) analysis demonstrated that in one of the pheochromocytomas, reduction to homozygosity was observed for both 17p and 17q markers while the adrenal cortical tumor demonstrated LOH for only 17q markers. The frequent LOH surrounding the NF1 locus and lack of neurofibromin expression in these tumors suggest that NF1 gene mutations may contribute to the development of adrenal gland neoplasms in patients with NF1.

Adrenal Gland Neoplasms↗

PubMed1995

Loss of neurofibromatosis type I (NF1) gene expression in pheochromocytomas from patients without NF1.

The neurofibromatosis type 1 (NF1) gene encodes a tumor-suppressor protein termed neurofibromin, which, in adults, is expressed predominantly in neurons, Schwann cells, and the adrenal medulla. Loss of NF1 gene expression has been reported in Schwann cell tumors (neurofibrosarcomas) from patients with NF1 as well as in malignant melanomas and neuroblastomas from patients without NF1. Previously, we demonstrated the lack of neurofibromin expression in six pheochromocytomas from patients with NF1, supporting the idea that neurofibromin might be an essential regulator of cell growth in these cells. To determine whether NF1 gene expression is similarly altered in pheochromocytomas from patients without NF1, we examined 20 pheochromocytomas for the presence of NF1 RNA and neurofibromin by reverse-transcribed polymerase chain reaction (RT-PCR) and immunohistochemistry, respectively. Reduced or absent NF1 gene expression was documented in 7 of these 20 tumors (35%) including 1 of 4 sporadic tumors, 3 of 10 tumors from patients with multiple endocrine neoplasia (MEN) 2A, 2 of 4 tumors from patients with MEN2B, and 1 of 2 tumors from patients with von Hippel-Lindau syndrome. In addition, most of these tumors expressed predominantly the type 1 NF1 isoform (75% type 1 NF1 isoform expression) as opposed to other neural crest-derived tissues such as adrenal gland and Schwann cells, which express predominantly type 2 NF1. This type 1 isoform predominance was also observed in the rat pheochromocytoma PC12 cell line, suggesting that this change in isoform expression may be associated with the genesis of these tumors.(ABSTRACT TRUNCATED AT 250 WORDS)

Base Sequence↗

PubMed1986

Incidence of soft-tissue cancers in blacks and whites in New York State.

Incidence rates for several histologic types of soft-tissue cancers were compared between black and white residents of New York State during 1976-81, using data from the population-based New York State Cancer Registry. Incidence rates for leiomyosarcoma, neurofibrosarcoma and malignant neurolemmoma were higher for blacks than for whites in almost all age groups. Black-white differences in malignant mesenchymoma, while not statistically significant, were similar to those from other population-based studies. There was no evidence for black-white differences in certain other histologic types (e.g., blood-vessel cancers, lymphangiosarcoma). Black-white differences in leiomyosarcoma included higher rates for the uterus in black than in white females, and higher rates for the stomach in blacks of both sexes. Possible explanations for these differences in soft-tissue cancer incidence were discussed, along with comparisons with limited data on black populations in Africa.

Adult↗

PubMed1979

Malignant fibrous histiocytoma arising in a patient with multiple neurofibromatosis: a case report and a literature review.

A case report of a 56-year-old man with the characteristic clinical and histologic features of multiple neurofibromatosis is presented. This patient was also diagnosed as having a malignant fibrous histiocytoma involving the right thigh with metastasis to the lung. A definite association between Von Recklinghausen's disease and various sarcomas (most commonly neurofibrosarcomas) of the peripheral nerves and the somatic soft tissues is well-known. This is apparently the first report in the literature of a malignant fibrous histiocytoma arising in a patient with multiple neurofibromatosis. The phenomenon of sarcomatous change in Von Recklinghausen's disease and the clinical and pathologic features of malignant fibrous histiocytoma are also discussed.

Histiocytoma, Benign Fibrous↗

PubMed1981

Radio frequency hyperthermia of advanced human sarcomas.

Hyperthermia greater than or equal to 42 degrees C is tumoricidal in vitro and in many animal models, although such temperatures have only recently been achieved experimentally in some human cancers. A recently developed radio frequency device that provides safe hyperthermia to any depth without surface tissue injury now permits evaluation of the effects of hyperthermia on advanced human sarcomas. Twelve patients with large sarcomas located intraabdominally [7], in the chest wall [2], proximal extremity [2], and the neck [1], were evaluated in this study. Tumor types include liposarcoma [3], rhabdomyosarcoma [2], leiomyosarcoma [2], neurofibrosarcoma [2], and one each malignant mesothelioma, undifferentiated sarcoma, and osteosarcoma. Intratumor temperatures greater than or equal to 42 degrees C were observed in all tumors, with virtually no normal tissue injury. Selective tumor heating greater than or equal to 45 degrees C occurred in 9/12 (75%) and greater than or equal to 50 degrees C in 6/12 (50%). One to five weekly treatments greater than or equal to 50 degrees C and ten daily treatments greater than or equal to 45 degrees C resulted in significant tumor necrosis and pain relief in some patients. Hyperthermia of advanced sarcomas is possible with little host toxicity and may be of potential therapeutic benefit.

Adult↗

PubMed1984

Primary retroperitoneal soft-tissue sarcomas.

Of the 98 soft-tissue sarcomas treated between 1973-1978, 12 were retroperitoneal in origin. Fifty percent underwent one to three previous surgical procedures. All patients were operated on. In 75% (nine out of 12), the tumor was completely resected. The operative mortality for total tumor excision was 11%. There were five liposarcomas, three leiomyosarcomas, two neurofibrosarcomas, and one synovial sarcoma and one unclassified sarcoma. Six of the 12 patients survived five years and three (25%) were alive without evidence of disease at 5 years. Three of the completely resected group received no further treatment; 2/3 survived 5 years; three had postoperative chemotherapy; 3/3 survived; and three others had radiation and chemotherapy subsequent to surgery; all three have died. The two most important prognostic factors were tumor grade and surgical excision. Only one patient with high grade tumor survived. Total surgical excision of the tumor offers the only hope for cure. Aggressive surgery in treating retroperitoneal soft part sarcomas is strongly recommended.

Adult↗

PubMed1988

Surgical management of soft tissue sarcomas, with an analysis of 313 cases.

From 1964 to 1978, 313 patients, 192 males and 121 females, with soft tissue sarcoma underwent surgery in our hospital. All patients have been followed up for over 5 years. One hundred ninety patients (60.7%) had recurrence of the tumor after previous surgery. The tumor was located in the head in 55 cases, in the trunk in 136, and in the extremities in 122 cases. Regional lymph node metastases occurred in 23 patients (7.4%). Fibrosarcoma, neurofibrosarcoma, and rhabdomyosarcoma were the types most commonly encountered in this series. The 5-year survival rate, according to the extent of surgical resection for 117 patients with local resection, was 47.9%; for 75 patients with wide local resection, 62.7%; for 43 patients with amputation, 18.6%; and for 78 patients with local resection combined with irradiation, 48.7%. For the whole series, the 5- and 10-year survival rates were 47.6% and 35.8%, respectively. The 5-year survival rates of stages I, II, III, and IV (TNM classification) were 60%, 46.9%, 40%, and 22.2%, respectively.

Adult↗

PubMed1997

Risk assessment on the carcinogenic potential of hybridoma cell DNA: implications for residual contaminating cellular DNA in biological products.

The aim of this study was to evaluate the possible carcinogenic potential of residual DNA derived from immortalized and possibly tumorigenic cell lines due to activated oncogenic sequences (oncogenes). These cell lines have been used for the production of biologicals, i.e. monoclonal antibodies, lymphokines and vaccines. The authors used hybridoma DNA as a first model. For this reason experiments in two species were performed, namely in 3-4 week-old female Balb/c mice and newborn Riv:TOX rats. Doses of 250 micrograms DNA, derived from Balb/c hybridoma cells, were injected subcutaneously (s.c.) in 200 mice. These mice also received a s.c. injection of the solvent only (TE buffer) at another site of the back skin (negative control for local tumour development). An additional group of 50 mice was treated intraperitoneally (i.p.) with the solvent only to serve as a negative control group for possible systemic tumorigenic effects. Doses of 5 micrograms plasmid pPy1 DNA, containing the entire Polyoma virus genome, served as positive control and were injected s.c. and i.p. in 20 and 50 mice, respectively. Doses of 50 micrograms hybridoma DNA or 5 micrograms pPy1 DNA were injected s.c. in rats too, using nine animals per group. During the experiment, animals were observed weekly, especially for the occurrence of subcutaneous tumours at the injection sites. The mouse study was terminated after more than 2 years, the rat study after 1 year. Gross necropsy was performed on all animals and histopathological examination of grossly suspected neoplastic lesions was performed. In the mouse experiment, tumour development at the s.c. injection site of the DNA was observed in one out of 20 animals in the pPy1-treated positive control group (neurofibrosarcoma) and one out of 200 animals in the hybridoma DNA-treated group (haemangioma-like lesion). Tumour development at or near the s.c. injection site of the solvent only was observed in two out of 200 animals. In the rat study none out of nine hybridoma DNA-treated rats developed tumours at the injection site, while three out of nine rats of the positive control group, injected with the pPy1 DNA, showed local tumour development (benign and malignant soft tissue tumours.) It is concluded that, at the high dose and numbers of animals tested, parenteral administration of hybridoma DNA does not induce local tumour development. Furthermore, no indications were found for systemic carcinogenic potential of the hybridoma DNA used. Based on a worst case approach of our data, the oncogenic risk of 100 pg residual DNA was estimated to be 2 x 10(-9), a value intermediate of the estimations of the WHO (1987) and the Dutch Health Council (1988) 5 x 10(-11) and 2 x 10(-7), respectively. Therefore, it is unlikely that the risk of 100 pg of DNA derived from other immortalized cell lines will exceed the level of generally accepted cancer risk of 10(-6).

Animals↗

PubMed1991

Mediastinal masses in childhood: a review from a paediatric pathologist's point of view.

From 1970 to 1989, 121 children with mediastinal masses of various sorts were seen in the Department of Pathology, Royal Children's Hospital, Melbourne. The series is considered representative of the true incidence of these conditions in the state of Victoria, which had an average paediatric population during the time of this series of 900,000 children. The commonest cause of a mediastinal mass was NHL (36 cases). This was followed by HD (24 cases), then neuroblastoma and ganglioneuroma (16 and 9 cases respectively), duplication cysts (10 cases), teratomas (7 cases), neurofibroma (4 cases) and lymphangioma (3 cases). A great variety of rare conditions made up the remainder of the series and included mediastinal abscess, thymic cyst, pericardial cyst, accessory lobe of lung, plasma cell granuloma, fibromatosis, paravertebral Ewing's tumour, carcinoid tumour and neurofibrosarcoma. Presentation of the children with NHL was often acute with respiratory distress, while the child with HD was usually older and symptoms were more often systemic than local. The surgeon's role in diagnosis of these most frequently encountered mediastinal masses can be crucial and biopsy when indicated must be carried out with great care to produce material that is adequate for diagnosis and for the performance of cell marker studies and chromosome analysis. Neuroblastoma (NBL) and ganglioneuroma (GN) together were the third largest group. Children with neuroblastoma were usually young; 15 of the 18 cases were less than 2 years old. One-third of the infants with neuroblastoma presented with paraplegia and one-third with respiratory symptoms including wheeze, stridor and respiratory difficulty. Three children had Horner's syndrome. Prognosis of children with thoracic neuroblastoma is very good and contrasts with the poor outlook for those with abdominal neuroblastoma. Stage at presentation is probably the most important single prognostic variable. Ganglioneuroma presents at a later age than neuroblastoma and symptoms may be present for a long time or may be completely absent. Catecholamines, usually raised in neuroblastoma, are mostly normal in ganglioneuroma. Duplication cysts were the next most frequent group. Symptoms can often be acute and life threatening, although in three of our ten cases the cyst was an incidental finding on chest X-ray. However, only three of our patients had a normal respiratory examination. Teratomas were usually large and more often benign than malignant. Excision is the mandatory treatment and is usually curative. Although teratomas in young infants are often cellular and composed of many immature tissue types, their behaviour is benign.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗