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PubMed1996

Primary intratesticular sarcoma. Immunohistochemical ultrastructural and DNA flow cytometric study of three cases with a review of the literature.

BACKGROUND: Fourteen cases of testicular sarcoma have been reported in the contemporary era. These included 7 cases of rhabdomyosarcoma, 2 spindle cell sarcoma, 2 osteosarcoma, 1 leiomyosarcoma, 1 fibrosarcoma, and 1 chondrosarcoma coma. METHODS: In this report, immunohistochemical stains, electron microscopy, and DNA flow cytometric analysis were performed on primary testicular sarcomas from three patients. RESULTS: The patients were age 47, 40, and 33 years. Each presented initially with a testicular mass. The tumors measured 4.8, 4.0, and 4.0 cm in greatest dimension. There was no associated germ cell elements nor elevated alpha-fetoprotein or beta-human chorionic gonadotropin. Case 1 was positive for actin, vimentin, and alpha-1-chymotrypsin. Case 2 was positive for vimentin but not desmin. Case 3 was positive for desmin and S-100. Smooth muscle differentiation was identified by electron microscopy. Flow cytometric analysis revealed DNA aneuploidy in all cases: 1.27, 1.29, and 1.71. The 3 patients were alive and well without recurrent disease at 7, 6, and 4 years after diagnosis. Inguinal orchiectomy was the initial treatment in all 17 patients, there was 1 death from metastatic disease and 2 patients with distant metastases. CONCLUSION: Primary testicular sarcoma is a rare indolent tumor with potential for distant metastases. Two cases of primary testicular leiomyosarcoma and one of unclassified sarcoma of the testis are reported.

Adult↗
PubMed1990

Sarcomas metastatic to the ovary: a report of 21 cases.

Twenty-one cases in which sarcomas metastasized to the ovaries are reported. The patients ranged from 18 to 79 (average 42) years of age; only five of them were over 50 years old. Eleven tumors were primary in the uterus and 10 outside the genital tract. Three uterine tumors were leiomyosarcomas, and eight, endometrial stromal sarcomas. The extragenital primary tumors were leiomyosarcoma of the stomach (1) and small intestine (2), retrovesical leiomyosarcoma (1), fibrosarcoma of the anterior abdominal wall (1), sarcoma of the mesentery of smooth muscle or neural type (1), hemangiosarcoma probably primary in the heart (1), osteosarcoma of the maxilla (1), chondrosarcoma of the rib (1), and Ewing's sarcoma of the pubic bone (1). The ovarian tumors, most of which were large, were discovered at the same time as the primary tumors in 11 cases; in seven cases, the ovarian tumor was discovered 7 months to 9 years after diagnosis of the primary tumor. In three cases, the ovarian tumors were discovered 4, 7, and 10 months before detection of the primary neoplasm. Two of these tumors were endometrial stromal sarcomas, and one, an epithelioid leiomyosarcoma of the stomach. Eleven ovarian metastases were bilateral. On microscopic examination, the greatest difficulty in pathologic interpretation was posed by the metastatic endometrial stromal sarcomas because of their simulation of sex cord-stromal tumors. Features helpful in their distinction from these tumors included the frequent presence of extra-ovarian disease, bilaterality, and a characteristic content of small arteries resembling the spiral arteries of the late secretory endometrium. The other tumor that caused major diagnostic difficulty was the metastatic epithelioid leiomyosarcoma from the stomach, which had a pattern that initially suggested the solid-tubular pattern of a Sertoli cell tumor.

Adolescent↗
PubMed2004

Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group.

BACKGROUND: Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility. A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited. METHODS: The authors analyzed 25 patients who were registered for the Italian Cooperative Group protocols for pediatric STS from 1979 to 2000. The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients. SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients. Treatment generally was administered according to the guidelines for primary STS. RESULTS: Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy. Retreatment was feasible with acceptable toxicity. Fifteen patients were alive in complete remission of their SMN at the time of last follow-up. Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT. Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma. Two patients developed a third malignancy. CONCLUSIONS: Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas. Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.

Adolescent↗
PubMed1994

Antitumor activity of nigericin and 5-(N-ethyl-N-isopropyl)amiloride: an approach to therapy based on cellular acidification and the inhibition of regulation of intracellular pH.

The extracellular pH (pHe) in solid tumors is frequently lower than the pHe in normal tissues, but the intracellular pH (pHi) is regulated to physiological levels. Cell killing can be achieved in an acidic environment in tissue culture by nigericin, which acidifies cells by transporting H+ from the extracellular space into the cytoplasm; this cell killing can be enhanced when used with 5-(N-ethyl-N-isopropyl)amiloride (EIPA), a potent inhibitor of membrane-based Na+/H+ exchange, which plays a major role in the regulation of pHi (R. P. Maidorn; E. J. Cragoe; I. F. Tannock, Br. J. Cancer 67:297-303; 1993). We have therefore assessed the ability of nigericin and EIPA to kill cells in two murine solid tumors (the KHT fibrosarcoma and the EMT-6 sarcoma). Hydralazine, which reduces tumor blood flow, or glucose, which stimulates glycolysis leading to accumulation of lactate, were also administered to mice to lower pHe in the tumors. We observed only a small decrease in the surviving fractions of cells in the tumors when tolerated doses of nigericin and EIPA were given IP to tumor-bearing mice. When nigericin and EIPA were combined with administration of hydralazine, the surviving fraction of cells in both tumors was reduced by a factor of 0.01, but there were minimal effects on growth delay. Administration of glucose with nigericin and EIPA led to a smaller reduction in surviving fraction of the KHT tumor (by approximately 0.1), although glucose was more effective than hydralazine in lowering the mean tumor pHe. When KHT tumors were treated with 15 Gy X-rays followed immediately by nigericin, EIPA, and hydralazine, a reduced surviving fraction as well as an increase in tumor growth delay was observed compared to radiation alone; however, there was little evidence to suggest that these agents were selectively toxic to the cells that survived radiation. Nigericin and EIPA, with or without hydralazine, had minimal effects on normal tissues, as assessed by changes in body weight, number of leukocytes, and serum creatinine levels. We conclude that pharmacological effects to acidify cells and to prevent regulation of pHi under the acidic conditions that exist in solid tumors can lead to moderate levels of cell killing, if additional strategies are used to lower tumor pHe.

Amiloride↗
PubMedDate not supplied

TUMORS OF BONE.

Explore the source record for details and available documents.

Bone Neoplasms↗
PubMed2002

Combined posterior pelvis and lumbar spine resection for sarcoma.

The oncologic outcome in the treatment of pelvic sarcomas is not comparable with that in the extremity. Particular problems arise when the tumor involves the posterior pelvis and the sacrum or even the lower lumbar spine. Because of the difficulty in achieving local control and the major functional deficits after extensive surgery, some authors suggest conservative, nonsurgical treatment for these patients. The purpose of the current study was to analyze the oncologic outcome of patients who were treated for a pelvic sarcoma necessitating resection of the ilium, part of the sacrum and part of the lower spine, which is defined as extended hemipelvectomy. Between 1979 and 1999, 11 males and seven females with a mean age of 34.5 years (range, 14-67 years) had an extended hemipelvectomy for a sarcoma of the posterior pelvis. The tumors included seven osteogenic sarcomas, six chondrosarcomas, and five fibrosarcomas: 13 were classified as high-grade lesions. The mean size of the tumor was 11 x 9 x 6 cm. In 11 patients, a hemivertebrectomy of L5 and in seven patients of L4 combined with L5 was done to achieve an adequate margin. In four patients, the resection was followed by reconstruction with a vascularized fibula or autograft. At a mean followup of 56 months (range, 3-220 months), 12 of 18 patients were alive and without disease. Six patients died; four of these patients had metastasis to the lung at presentation. The two remaining patients died of complications of adjuvant treatment. The authors conclude from this small series of patients that an aggressive surgical resection for localized, nonmetastatic, high-grade sarcomas of the posterior pelvis may be justified to provide local control and improve survival.

Adolescent↗
PubMed1977

A clinical and pathological staging system for soft tissue sarcomas.

A system for staging the clinical status of patients with soft tissue sarcomas is presented, based on the clinical characteristics of the primary tumor (size, extension), the involvement of lymph nodes, the presence of metastases, and the grade of the tumor. This represents the TNM system with grade of tumor (G) added. The system evolved was based on examination of 1215 cases of 13 types of soft tissues sarcomas, primarily in the extremities (fibrosarcoma, liposarcoma, etc.). Nine stages are described, and they are correlated with survival in the cases reviewed. The staging system now can be used for case evaluation for therapy determination and for intercomparison of series of patients as to incidence of different kinds of tumors, effects of treatment, and survival.

Adult↗
PubMed2001

Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma.

Sclerosing epithelioid fibrosarcoma is a recently described sarcoma in which ultrastructural evidence of fibroblastic differentiation forms part of the diagnostic criteria. This report describes a further case of this tumour, which showed evidence of both fibroblastic and perineurial differentiation by immunohistochemistry and electron microscopy, and which had areas of high grade morphology. The tumour metastasised and the patient died of disease 12 months after presentation. The relevance of these findings to diagnosis and differentiation in these tumours is discussed.

Cell Differentiation↗
PubMedDate not supplied

Tumors of the heart: surgical considerations.

A series of 23 cardiac tumors is reported. Six were diagnosed at autopsy; 17 tumors were surgically explored. Eleven of the 17 were myxomas within the left atrium. Eight of the 17 patients presented with congestive heart failure; peripheral and cerebral emboli were also common. The diagnosis was made preoperatively in 10 patients. Two-dimensional echocardiography was the most reliable diagnostic tool. Follow-up averages 55 months; there have been no late deaths or recurrences. The other six surgically treated tumors were: a left ventricular rhabdomyoma, a septal lipoma, a right atrial calcified endocardial mass, a right ventricular fibrosarcoma, a rhabdomyosarcoma, and a sarcoma metastatic to the pericardium and right atrium. From this series and a review of the literature, we concluded that: benign cardiac tumors can usually be excised with a low morbidity and excellent long-term results; malignant cardiac tumors have a dismal prognosis, and operation is primarily diagnostic; tumors metastatic to the heart should be operated upon only if successful palliation seems possible.

Adult↗
PubMed1975

Synovial sarcoma of the neck: a followup study of 24 cases.

This article reviews the clinicopathologic features and the biological behavior of 24 cases of synovial sarcoma that took origin from the cervical prevertebral connective tissue space and manifested as a retropharyngeal tumor or as a palpable mass in the anterior or posterior cervical triangle. The age of the 24 patients ranged from 10 to 51 years, with a median of 19 years. Ten patients were women and 14 men. Hoarseness or difficulty in breathing or swallowing were the first symptoms in eight patients. The tumors were solitary and ranged from 2 to 10 cm in greatest dimension. Microscopically, all of the cases showed the characteristic biphasic cellular pattern of a synovial sarcoma, with epithelioid and fibrosarcoma-like areas in varying proportions. Synovioblastic origin of the neoplasm was confirmed by the results of histochemical staining procedures and, in 1 case, by the examination with the electron microscope. Of the 21 cases followup information, 12 had died (10 with pulmonary metastasis) and 9 were alive and free of symptoms. Prompt and complete surgical removal is required to prevent complications from recurrent tumor growth or metastasis.

Adolescent↗
PubMed2000

Induction of adenocarcinoma containing myoepithelial cells in rat submandibular gland by 7,12-dimethylbenz(a)anthracene.

In an attempt to induce adenocarcinoma containing myoepithelial cells (MECs) in the rat submandibular gland, we injected 7,12-dimethylbenz(a)anthracene (DMBA) dissolved in acetone into the glands of rat pups at the age of 10 days. In both male and female pups, the glands, including their developing terminal secretory units, contained far greater numbers of cells positive for proliferating cell nuclear antigen (PCNA) than did adult glands. A single administration of 1% DMBA (0.05 ml/130 g b.w.) did not produce adenocarcinoma, but did induce occasional sarcomas, such as rhabdomyosarcoma and fibrosarcoma, in 2 months. Most glands regenerated with minimal scar formation. Microscopically, these glands were atypical in that they contained increased numbers of PCNA-positive cells, underdeveloped granular ducts, and striated ducts surrounded by MECs positive for alpha smooth muscle actin (alphaSMA). Though these features were also observed in the regenerated glands after acetone injection, the number of PCNA-positive cells was relatively high in the glands of DMBA-treated females, especially in the terminal secretory unit. The second DMBA injection at 10 weeks of age produced adenocarcinoma made up of alphaSMA-positive MECs and keratin 19-positive duct cells. Such MEC-associated adenocarcinoma was induced in the glands of more than half the female but not the male animals. Replacement of either of the double DMBA treatments with acetone, or DMBA treatment, single or double, of adult glands did not produce adenocarcinoma, but did produce sarcoma and squamous cell carcinoma. These results suggest that (1) at least two genetic mutations are necessary for induction of adenocarcinoma with MECs in the rat submandibular gland, (2) the mutation is efficiently introduced to pup glands whose terminal secretory units exhibit extreme proliferative activity, and (3) the second mutation is difficult to introduce in male glands, whose proliferative activity is relatively low, and/or transformed cells need some female hormone after the mutation to propagate.

9,10-Dimethyl-1,2-benzanthracene↗
PubMed1984

Sarcoma arising in a residual testicular teratoma after cytoreductive chemotherapy.

A case of testicular teratoma metastasized to the retroperitoneum and after cytoreductive chemotherapy was noted to contain areas of frank sarcoma. Sarcomatous areas included embryonal rhabdomyosarcoma with a pattern of sarcoma botyroides, alveolar rhabdomyosarcoma, and fibrosarcoma. These areas differed markedly from areas of immature teratoma, which composed the remainder of the retroperitoneal lesion and which also characterized the primary tumor. These sarcomatous areas were characterized by numerous mitoses, marked cellular pleomorphism and diagnostic histologic, ultrastructural, and immunocytochemical features. Residual germ cell tumors following cytoreductive chemotherapy are traditionally categorized as teratoma only or teratoma with embryonal carcinoma or choriocarcinoma for therapeutic and prognostic reasons. This case does not conform to this simple categorization and raises serious questions concerning subsequent therapeutic decisions.

Adult↗
PubMedDate not supplied

Histopathology of tumorous ribs: a review of 130 cases.

There were studied histopathologically 130 tumorous ribs out of 167 tumoral processes of the thoracal wall (77 per cent) and representing about 2 per cent of all the neoplasms studied between 1949 and 1984. There were distinguished 50 malignant primary tumors (Ewing's sarcomas, Parker-Jackson's reticulosarcomas, endotheliosarcomas, low differentiated myelosarcomas and myelomas, osteogenic sarcomas, chondro- and osteochondrosarcomas, and fibrosarcomas), 45 secondary (metastatic) malignant tumors of ribs out of which 32 (75 per cent) originated from lung cancers, and 35 benign lesions (osteoclastomas, chondromas and osteochondromas, fibromas and osteofibromas, eosinocytic histiocytomas, and various osteodystrophies). Morphological differentiating criteria were discussed for each group. There were analysed, when possible, the ways of rib involvement by neighbouring processes, especially the periosteum relationships with the soft tissues of the thoracal wall and with vessels and pleura. Some histogenetic problems were also emphasized.

Bone Neoplasms↗