Two Cases of Orbital Growth-(A) Mixed Tumor of the Lacrimal Gland. (B) Small Round-cell Sarcoma in an Infant.
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Journal Article↗
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Results for “Sarcoma, Small Cell”
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[On a rare case of reticulum cell sarcoma of the small intestine and ovary].
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Child↗
Tissue culture as a diagnostic technique for soft-tissue sarcoma of childhood.
A case of childhood small cell undifferentiated sarcoma was grown in vitro in a serum-free culture medium. In vivo the tumor showed no evidence of differentiation by light microscopy, electron microscopy, and immunoperoxidase staining for myoglobin or skeletal muscle myosin. After growth in vitro for two weeks in a defined culture medium, the tumor cells formed myotubes and demonstrated ultrastructural myogenic differentiation. This case demonstrates the use of the tissue culture as a diagnostic aid in the recognition of childhood small cell undifferentiated sarcomas. It also raises questions regarding the origin of these tumors and the ability of in vitro growth to induce differentiation.
Adolescent↗
A gastric carcinosarcoma with neuroendocrine cell differentiation and undifferentiated spindle-shaped sarcoma component possibly progressing from the conventional tubular adenocarcinoma; an immunohistochemical and ultrastructural study.
A 56-year-old Japanese man underwent total gastrectomy with lymph-node dissection for a gastric tumor. In the oral rim of the excised large ulcerated tumor, a small nodular mass demonstrated a unique histology of a carcinosarcoma composed of more than three distinctive components. A major part of the large ulcerated tumor tissue consisted of conventional tubular adenocarcinoma, with the coincidental focal distribution of solid cell nests of poorly differentiated or neuroendocrine cell (small cell) carcinoma. Sarcoma cells were another of the constituents of the small nodular tumor with atypical spindle-shaped cells. All the excised lymph-node metastasis demonstrated the histology of tubular adenocarcinoma. In the sarcoma cells, many of the lineage-specific immunohistochemical markers were negative. Only vimentin was positive. Electron microscopy revealed that the neuroendocrine cell carcinoma contained cells with abundant small aggregations of neurosecretory-type granules in their cellular cytoplasm. Primitive cellular junctions and incomplete basal lamina were seen in the sarcoma cells. No transitional or hybrid-type cells were seen between carcinoma and sarcoma cells. The interfaces between the cancer cell nests and the proliferation of sarcoma cells are clearly discerned. It was speculated that the unique histology of carcinosarcoma might have progressed from the conventional type of pre-existing advanced tubular adenocarcinoma.
Adenocarcinoma↗
Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor.
BACKGROUND: The EWS gene, a transcription factor of unknown function, is involved in chromosomal translocations associated with a wide variety of tumors, particularly small round blue cell tumors such as Ewing sarcoma. It has previously been reported that desmoplastic small round blue cell tumor (DSRBCT) frequently has an associated t(11;22) abnormality resulting from fusion of the EWS and WT-1 genes. PROCEDURE: We report a case of a small round blue cell tumor with characteristics of both Ewing sarcoma and DSRBCT with a t(11;22) translocation leading to fusion of the EWS and FLI1genes. RESULTS: The translocation point and fusion products were confirmed by polymerase chain reaction amplification and restriction fragment mapping of the products. CONCLUSIONS: The biphenotypic nature of this case and the apparent promiscuity of the EWS gene in tumor-associated translocations coupled with other reports of biphenotypic childhood sarcomas has potential implications for the relationship between small round blue cell tumors and the mechanism of EWS/FLI1 oncogenesis.
Carcinoma, Small Cell↗
Intensive combined modality therapy of small round cell and undifferentiated sarcomas in children and young adults: local control and patterns of failure.
Seventy-five patients (ages 4-35 years) with the following small round cell tumors and undifferentiated sarcoma were treated at the National Cancer Institute: Ewing's sarcoma (n = 32), peripheral neuroepithelioma (n = 14), rhabdomyosarcoma (n = 24), undifferentiated sarcoma (n = 5). Most patients had poor prognostic features including 36 (48%) with metastatic disease, and 42 (56%) with central (truncal) tumors (22 in the pelvis). Treatment included 5 cycles of intensive induction chemotherapy with vincristine, cyclophosphamide and adriamycin, plus aggressive local radiation therapy using simulation and computerized treatment planning for all patients. Thereafter, complete clinical responses were consolidated with intensive chemotherapy, total body irradiation and autologous bone marrow transplantation. There were three local only failures, 10 local plus distant failures, 36 distant only failures, 3 treatment-related deaths, and one intercurrent death. Overall actuarial survival and event-free survival at 4 years are 49 and 29%, respectively. Actuarial freedom from local progression was seen in 74% of patients at 4 years, quite remarkable considering the bulk and location of most of these tumors. Without aggressive surgery, many of these high risk patients had satisfactory outcomes, but better systemic treatments are still needed.
Adolescent↗
Practical application of molecular genetic testing as an aid to the surgical pathologic diagnosis of sarcomas: a prospective study.
The strong correlation of specific reciprocal translocations with individual tumor types and the demonstration that polymerase chain reaction (PCR)-based methods can detect translocations in tissue samples have stimulated interest in the role of molecular genetic testing in diagnostic surgical pathology. To evaluate the clinical utility of PCR-based molecular analysis of soft tissue neoplasms in routine surgical pathology, 131 consecutive soft tissue tumors submitted for molecular genetic testing at a tertiary care teaching hospital were prospectively analyzed over a 36-month period. RT-PCR was used to test tumor RNA for fusion transcripts characteristic of malignant round cell tumors (including Ewing sarcoma/primitive neuroectodermal tumor, desmoplastic small round cell tumor, and alveolar rhabdomyosarcoma), spindle cell tumors (including synovial sarcoma and congenital fibrosarcoma), and fatty tumors (myxoid liposarcoma). DNA sequence analysis was used to confirm the identity of all PCR products, and the PCR results were compared with the histopathologic diagnosis. We found that sufficient RNA for RT-PCR-based testing was recovered from 96% of the 131 cases and the percentage of tumors that tested positive for the associated characteristic fusion transcript was in general agreement with those reported in the literature. DNA sequence analysis of PCR products identified both variant transcripts and spurious PCR products, underscoring the value of product confirmation steps when testing formalin-fixed, paraffin-embedded tissue. Only in rare cases did testing yield a genetic result that was discordant with the histopathologic diagnosis. We conclude that PCR-based testing is a useful adjunct for the diagnosis of malignant small round cell tumors, spindle cell tumors, and other miscellaneous neoplasms in routine surgical pathology practice.
Base Sequence↗
Aspiration cytology of pulmonary small cell variant of poorly differentiated synovial sarcoma metastatic from the tongue: a case report.
BACKGROUND: Small cell variant ofpoorly differentiated synovial sarcoma (PDSS) is a great mimic of Ewing's sarcoma/primitive neuroectodermal tumor, with cytologic and immunohistochemical overlap. CASE: A 21-year-old male was admitted to our hospital because of a solitary pulmonary nodule that developed 22 months after resection of a tongue mass, small cell variant of PDSS. The nodule was biopsied via fluoroscopy-guided fine needle aspiration (FNA). At low power, the cytologic smears were highly cellular, consisting of a mixture of dispersed cells and loose or tight tissue fragments. The cells were characteristically uniform and monotonous, showing round to ovoid nuclei and scant cytoplasm. However, at high power, mild nuclear pleomorphism and frequent mitotic figures were noted. Blood vessels were often seen within the tumor cell aggregates. A diagnosis of metastatic sarcoma was rendered. Metastatectomy of the lung nodule was performed, and cytogenetic study showed t(X;18). CONCLUSION: A diagnosis of small cell variant of PDSS is difficult on routinely stained smears, but in the appropriate clinical setting, especially when a prior history of a primary tumor is available, a confident diagnosis can be established by FNA cytology. Immunohistochemistry and identification of SYT/SSX fusion transcript are useful for confirmation.
Adult↗
Intrinsic and extrinsic characteristics of human tumors relevant to radiosurgery: comparative cellular radiosensitivity and hypoxic percentages.
UNLABELLED: We have collected the in vitro x-ray radiation survival characteristics of 181 lines from 12 different classes of exponentially growing human tumor cells (sarcomas, lung cancers, colo-rectal cancers, medulloblastomas, melanoma, breast cancers, prostate cancers, renal cell cancers, grades III and IV brain tumors, ovarian, and head and neck cancers). This information was used to intercompare survival after single high doses of 20-40 Gy for each tumor line. Radiosensitivities could roughly be divided into two groups. The more radiosensitive group included: sarcoma, small-cell lung cancer, non-small cell lung cancer, colorectal cancer, medulloblastoma and melanoma. The more radioresistant group included breast, prostate, renal cell, primary brain tumors, ovarian tumors, and head and neck cancers. Using a model of a 3 cm diameter brain lesion containing about 1.4 x 10(9) oxic cells, the single doses calculated to reduce survival to 1 cell were: sarcoma and small cell lung cancers-22-23 Gy; melanoma-25 Gy; non-small cell lung and colorectal cancer-26 Gy; medullo-blastoma-28 Gy; breast, prostate, renal cell, primary brain tumors, ovarian tumors, and head and neck cancers-30-36 Gy. If, however, tumors contained on average 20 percent hypoxic cells, the dose needed for equivalent cell killing increased by about a factor of 2.6-2.8. Also, there was no correlation between the ranking of relative radiosensitivities of the various classes of tumor cells at high doses (as in radiosurgery) to the sensitivity at low doses (as in conventional fractionated radiotherapy). CONCLUSION: available information on the intrinsic radiosensitivity of human tumor cells indicates that meaningful differences exist among different histological classes of neoplasm that are relevant to the single high doses used in radioneurosurgery, and which could constitute a basis for "tailoring" the administered dose to the particular neoplasm. However, if intracerebral lesions contain a large number of hypoxic cells (e.g., 20%), this may constitute a significant problem.
Cells, Cultured↗
Overexpression of the c-erbB-2 oncogene in sarcomas and small round-cell tumors of childhood. An immunohistochemical investigation.
Amplification and overexpression of the c-erbB-2 oncogene have been associated with a poor prognosis in breast cancer. The present study evaluates c-erbB-2 protein over-production in sarcomas and in small round-cell tumors of childhood by applying an immunohistochemical technique to formalin-fixed, paraffin-embedded tissues. This technique had previously demonstrated high sensitivity for the detection of c-erbB-2 activation. One peripheral neuroepithelioma demonstrated equivocal immunoreactivity for c-erbB-2 protein. There was no evidence of c-erbB-2 protein overproduction in 11 angiosarcomas, 21 chondrosarcomas, 10 epithelioid sarcomas, 14 Ewing's sarcomas, 20 hepatoblastomas, nine leiomyosarcomas, 12 liposarcomas, 13 malignant fibrous histiocytomas, nine malignant lymphomas, 15 neuroblastomas/ganglioneuroblastomas, 16 neurofibrosarcomas, 10 osteosarcomas, seven peripheral neuroepitheliomas, 10 rhabdomyosarcomas, six synovial sarcomas, and 20 Wilm's tumors. We conclude that overproduction of the c-erbB-2 protein is rare or absent in these neoplasms and that its detection is therefore not likely to be of practical utility in their pathologic evaluation and prognostication.
Bone Neoplasms↗
Ovarian small cell tumors: an electron microscopic review.
Small cell tumors of the ovary are uncommon but represent an important group to recognize in the differential diagnosis of primary and metastatic ovarian neoplasms. In some cases the correct diagnosis cannot be confidently made on the basis of clinical setting, routine light microscopy, and immunohistochemistry, and electron microscopy may be supportive or definitive in establishing cell type. The cell type is often important in choosing optimal therapy and in predicting prognosis. The authors performed electron microscopy on a moderate number of ovarian small cell tumors and here describe and illustrate the diagnostic features of representative examples of various types. The ultrastructural features of the metastatic tumors, such as embryonal rhabdomyosarcoma, neuroblastoma, and melanoma, are identical to those of their respective primary tumors, are well known, and usually pose no problem in diagnosis. On the other hand, the ultrastructural features of some primary ovarian small cell tumors may present a more difficult differential diagnosis, because they have features that are subtle and/or in common. Exemplary of tumors in this category are diffuse adult granulosa cell tumor, endometrial stromal sarcoma, and small cell carcinomas of the hypercalcemic and pulmonary (oat cell) types. Distinguishing among them may be difficult but is possible, and electron microscopy may be a valuable supplement to the diagnostic information obtained from the clinical presentation, light microscopy, immunohistochemistry and, in some tumors, cytometric analysis of these neoplasms.
Carcinoma, Merkel Cell↗
Ewing's sarcoma and related small round cell neoplasms in children.
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Bone Neoplasms↗
Extraskeletal Ewing's sarcoma. Diagnosis of a case by fine needle aspiration cytology.
A retroperitoneal mass was subjected to fine needle aspiration biopsy for cytologic evaluation. The aspirate consisted of a monotonous population of undifferentiated tumor cells whose cytologic appearance was consistent with a small-cell (Ewing's) sarcoma. The differential diagnosis of extraskeletal Ewing's sarcoma from other small-cell neoplasms, such as lymphoma, neuroblastoma and other soft-tissue sarcoma, cannot be made solely on the basis of morphologic studies. As in this case, histochemical studies and consideration of the clinical features are needed to make the final diagnosis.
Adult↗
Laboratory investigation, genetics, and experimental models in sarcomas.
The sarcomas, particularly those of soft-tissue origin, pose substantial diagnostic challenges for the clinician and pathologist. Several small round cell sarcomas, including Ewing's sarcoma, peripheral primitive neuroectodermal tumor, and alveolar rhabdomyosarcoma, can be difficult to distinguish from one another. These same sarcomas can be difficult to distinguish from other small round cell tumors, including non-Hodgkin's lymphoma and neuroblastoma. Spindle cell sarcomas, including malignant peripheral nerve sheath tumor, synovial sarcoma, and leiomyosarcoma, present similar diagnostic challenges. This review discusses 1) recent advances in immunohistochemistry, electron microscopy, and cytogenetics that enable a specific diagnosis in virtually all sarcoma cases; 2) cell biology and oncogenetic implications of novel morphologic and genetic findings in sarcomas; and 3) clinical implications of the recent characterization of several family cancer syndrome genes.
Animals↗
Calretinin expression in tumors of adipose tissue.
Although well established as a marker of mesothelial cells, calretinin is also expressed in several other tissue types, including adipose tissue. Accordingly, immunohistochemical staining for calretinin has been described in an increasing number of neoplasms other than mesothelioma. A detailed analysis of calretinin expression in lipogenic tumors has not yet been reported, however. Given the known expression patterns of calretinin in normal tissues, we predicted that calretinin immunoreactivity would be detected in lipoma and the various histologic subtypes of liposarcoma, and that this marker might be of use in the differential diagnosis of selected fatty tumors. A variety of pleomorphic and small round cell sarcomas were studied for comparison. Calretinin immunoreactivity was detected, at least focally, in all 10 samples of normal adipose tissue and in 22 of 23 lipomas or lipoma variants. Staining for calretinin was also positive within the lipogenic component of 28 of 29 liposarcoma variants. Of the 7 dedifferentiated liposarcomas, 3 were focally positive for calretinin. Pleomorphic variants of other sarcomas, including undifferentiated high-grade pleomorphic sarcoma, rhabdomyosarcoma, leiomyosarcoma, and malignant peripheral nerve sheath tumor also exhibited focal calretinin immunoreactivity in a minority of cases, as did some small round cell sarcomas. These results suggest that calretinin immunoreactivity in normal and neoplastic adipose tissue is more ubiquitous than previously reported and may be a useful, albeit nonspecific marker of lipogenic differentiation. However, its utility in the differential diagnosis of fatty tumors appears limited.
Adipose Tissue↗
Abdominal Burkitt's lymphoma diagnosed by fine needle aspiration cytology--a case report.
A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
Biopsy, Needle↗
Surgical treatment of lung metastases: The European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group study of 255 patients.
BACKGROUND: Several reports have shown a prolonged survival after surgical treatment of pulmonary metastases from soft tissue sarcomas. However, it is still unclear which prognostic factors predict a favorable outcome. Series are not comparable and the data are conflicting. Therefore, a multi-institutional study was undertaken to analyze prognostic factors in selecting patients for resection of pulmonary metastases from soft tissue sarcomas. METHODS: This report is a retrospective study of the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group. Two hundred fifty-five patients underwent complete resection of lung metastases from soft tissue sarcomas. Cases with chondrosarcoma and small round cell sarcomas like Ewing sarcoma were excluded. RESULTS: The 3 year and 5 year overall postmetastasectomy survival rates were 54% and 38%, respectively. The disease free postmetastasectomy survival rates were 42% and 35%, respectively. Analysis of prognostic factors for a more favorable outcome revealed disease free intervals of 2.5 years or more, following a resection with microscopically free margins, age less than 40 years, and Grade I and II tumors. These prognostic factors have an independent influence on overall survival, using a multivariate Cox regression model. CONCLUSIONS: Surgical excision of lung metastases from soft tissue sarcomas is well accepted and should be considered as a first line of treatment if preoperative evaluation indicates that complete clearance of the metastases is possible. Further investigation is needed before chemotherapy can be recommended as additional therapy.
Adolescent↗
Dissociation of body weight and lean body mass during cancer chemotherapy.
Body weight and lean body mass are different reflections of the nutritional status of patients receiving cancer chemotherapy. In the present study, the relation between lean body mass and body weight during cytostatic treatment was investigated in 3 groups of newly-diagnosed children and young adults with acute lymphocytic leukemia, osteosarcoma, or a small round cell sarcoma. Body weight and lean body mass were determined before and after an initial period of cytostatic treatment. Lean body mass was derived from total body water volume, which was assessed by deuterium oxide dilution. A significant dissociation between body weight and lean body mass was observed in leukemia patients (n = 8, P = 0.008, paired t-test), and in osteosarcoma patients (n = 13, P = 0.001). No dissociation was found in patients with a small round cell sarcoma (n = 8, P = 0.839). We conclude that during cancer chemotherapy periodic assessment of body weight may give a false picture of the preservation of lean body mass. Considering the course of body weight alone may prevent the establishment of a timely diagnosis of malnutrition, which is mandatory for optimal supportive care.
Adolescent↗