[Sarcomas of the small intestine in children. Presentation of cases].
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Ag-NOR staining in mouse sarcoma ascites cells.
In sarcoma cells, the nucleoli are small, ill-defined, and are present in large numbers making it difficult to stain them by classical staining procedures. A modified Ag-NOR technique has been applied to detect the nucleolus and the nucleolar-organizing region in the chromosome of a mouse sarcoma-180 ascites cell-line. The cells were found to have 8-14 nucleolar chromosomes. The nucleoli are present in varying numbers corresponding to the number of nucleolar chromosomes. Silver grains appear as dots at one end of the chromosome. This end is presumed to be the centromeric end as, in normal mice, the NORs are in association with the centromere. However, the centromeres are ATrich, whereas the NORs should be GCrich. This NORs in mouse chromosomes should be placed below the centromere as already shown by the nucleic acid hybridization technique.
Animals↗
Signal transduction pathways in sarcoma as targets for therapeutic intervention.
Investigations into the molecular alterations in sarcomas have made substantial progress during the past decade. Classical linkage analysis and the direct sequencing of chromosomal translocation fusions have identified candidate genes in many different sarcomas. A large group of these genes participate in signal transduction pathways and represent potential sites of disease intervention with targeted therapies. This review will discuss five types of sarcoma that display aberrant tyrosine kinase pathway signaling: gastrointestinal stromal tumor, inflammatory myofibroblastic tumor, congenital fibrosarcoma and mesoblastic nephroma, dermatofibrosarcoma protuberans, and desmoplastic small round cell tumor; one sarcoma predisposition syndrome with specific dysregulation of the ras pathway--neurofibromatosis--will also be discussed.
Antineoplastic Agents↗
Ganglioside vaccines with emphasis on GM2.
Gangliosides are neuraminic acid-containing glycosphingolipids that are anchored into the cell membrane lipid bilayer by lipophilic ceramide chains. They are overexpressed on tissues of neuroectodermal origin, and particularly in tumors such as melanomas, sarcomas, neuroblastomas, astrocytomas, and small cell lung cancers. Both active and passive immunotherapy trials have identified gangliosides as uniquely effective targets for antibody mediated melanoma immunotherapy. Induction of antibodies against GM2 by vaccination has correlated with an improved prognosis in American Joint Committee on Cancer (AJCC) stage III melanoma patients and vaccines containing GM2 chemically conjugated to keyhole limpet hemocyanin (KLH; GM2-KLH) plus the immunologic adjuvant QS-21 have proven to be consistently immunogenic. Phase III trials with this vaccine are ongoing in patients with melanoma in the United States, Canada, Europe, Australia, and New Zealand. GD2, fucosylated GMI, and GD3-KLH conjugates plus QS-21 are also consistently immunogenic, inducing IgM and IgG antibodies in the majority of patients. Polyvalent ganglioside-KLH conjugate plus QS-21 vaccines should be available in early 1999 for testing in phase II and III clinical trials.
Adjuvants, Immunologic↗
Integrity of the 1,25-dihydroxyvitamin D3 receptor in bone, lung, and other cancers.
Differentiation and proliferation can be regulated in diverse cell types by 1,25-dihydroxyvitamin D3. These effects derive from modulation of gene expression mediated by the interaction of 1,25-dihydroxyvitamin D3 with the vitamin D receptor (VDR). The VDR is one of the nuclear hormone receptors. Because these transcription factors play a key role in growth control, some nuclear hormone receptors, such as the retinoic acid receptor alpha, can be disrupted in cancer. With these alterations in mind, we looked for alterations of the VDR gene in a variety of cancers, including 68 osteosarcomas, 23 other sarcomas, 34 non-small cell lung cancers, and 44 cell lines representing many tumor types. Gross integrity of the VDR gene was examined on Southern blots probed with the coding region of the VDR cDNA. The presence of point mutations targeting VDR exons 2-7 was assessed by polymerase chain reaction-single-strand conformation polymorphism analysis and direct DNA sequencing. Two alterations were detected; direct DNA sequencing of these samples revealed one silent mutation in codon 79 and a base change in intron 3. These results suggest that mutations and rearrangement of the VDR do not play a role in the cancers studied.
Blotting, Southern↗
Small round-cell type of malignant peripheral nerve sheath tumor.
Five cases of primitive, small, round-cell tumor that are a type of hitherto unclassified neurogenic sarcoma are described. The tumors were located deep within the soft tissue of the trunks and limbs without association with major nerves. The histologic features consisted mainly of uniform, small, round, tumor cells with scanty cytoplasm. Foci of uniform, short, spindle-shaped tumor cells arranged in whorl-like patterns were observed in some areas. Although the immunoreactivity for the neural markers, Leu-7 and MIC-2, was not marked, cell processes and fragmentous basal laminae, which are ultrastructural neural features, were found in both spindle-shaped and round tumor cells. In four cases, EWS chimeric transcripts were analyzed by reverse-transcription polymerase chain reaction. EWS chimeric mRNA (EWS-FLI-1, EWS-ERG, EWS-E1AF, EWS-ETV1, EWS-FEV) was not detected in any cases. The tumors were not consistent with peripheral primitive neuroectodermal tumor. We propose them as a small round-cell type of MPNST that might differentiate toward the immature neural cells.
Adolescent↗
Small-cell tumors of the liver: a cytological study of 91 cases and a review of the literature.
This study was designed to consider the cytomorphological spectrum, differential diagnosis, and the role of ancillary studies in small-cell tumors of the liver. Three independent pathologists reviewed cytological slides from 91 cases of small-cell tumors of the liver. The results were compared with the findings of three recently published studies (Cytopathology 11 (2000) 262-267; Diagn Cytopathol 19 (1998) 29-32; and Acta Cytol 40 (1996) 937-947). The role of immunohistochemistry in reaching timely and specific diagnoses was also examined. The diagnostic categories included 44 cases of metastatic small-cell undifferentiated carcinoma, 15 cases of metastatic neuroendocrine carcinoma, 10 cases of metastatic adenocarcinoma, 7 cases of malignant lymphoma, 4 cases of hepatocellular carcinoma with small-cell features, 2 cases of cholangiocarcinoma, 1 case of poorly differentiated carcinoma, and 8 cases of rare tumors including granulosa cell tumor (2 cases), sarcoma (4 cases), malignant melanoma with small-cell features (1 case), and meningioma with small-cell features (1 case). Metastatic granulosa cell-tumor, metastatic melanoma, and metastatic meningioma should be included in the differential diagnoses of small-cell malignancies found in the liver.
Adenocarcinoma↗
In vitro evaluation of new anticancer drugs, exemplified by vinorelbine, using the fluorometric microculture cytotoxicity assay on human tumor cell lines and patient biopsy cells.
The feasibility of combined studies on a cell-line panel and primary cultures of patient tumor cells in the preclinical evaluation of new anticancer drugs was evaluated in a study of the activity and cross-resistance pattern in vitro of the new semi-synthetic vinca alkaloid vinorelbine (Vrb). The activity of Vrb was investigated in ten cell lines representing different resistance mechanisms and in a total of 256 fresh human tumor samples, using the fluorometric microculture cytotoxicity assay (FMCA). Resistance to Vrb in the cell lines was associated with expression of the multidrug resistance-mediating P-glycoprotein and the multidrug resistance-associated protein (MRP) and by a recently described tubulin-associated mechanism, while the cell lines with topoisomerase II- and glutathion-associated resistance did not show decreased sensitivity to the drug. Cross-resistance to vincristine (Vcr) and other tubulin-active agents was high in cell lines as well as in patient cells. As with most commonly used anti-cancer drugs, Vrb was more active in hematological than in solid tumor samples. Among the solid tumors investigated, the highest in vitro response rates were observed in ovarian cancer (27%), sarcoma (25%), non-small cell lung cancer (21%) and bladder cancer (20%), while no response was observed in renal or colorectal cancer. Compared to Vcr, Vrb appeared to be slightly more active in solid tumors and slightly less active in hematological tumors. The results show that although Vrb displays a high degree of cross-resistance to Vcr and other tubulin-active drugs, some difference in the activity spectrum could be detected and that the drug is sensitive to multiple mechanisms of resistance. The results also suggest that leukemias, ovarian cancer, sarcoma and bladder cancer are possible further targets for Vrb. The combination of studies on a cell-line panel and patient tumor cells from a broad spectrum of diagnoses to evaluate a new drug seems feasible and may give information on the mechanism of action and target diagnoses for phase II trials.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
Extraosseous Ewing's sarcoma: report of a case and review of literature.
Extraosseous Ewing's sarcoma is a primitive small round cell neoplasm found in children and young adults. Extraosseous Ewing's sarcoma has been recognized as being histologically indistinguishable from Ewing's sarcoma of the bone and other round cell tumors. Our study reports the clinical course and histopathologic features of this rare variant, occurring in a 13 year-old boy, who presented with a subcutaneous tumor of the right thigh without osseous involvement. Wedge resection of the tumor was done followed by chemotherapy with Actinomycin-D, Oncovin and Endoxan.
Adolescent↗
Fine-needle aspiration (FNA) cytology diagnosis of small round cell tumors: value and limitations.
Small round cell tumors (SRCTs) are a group of malignancies (non-Hodgkin lymphoma, neuroblastoma, retinoblastoma, hepatoblastoma, nephroblastoma, rhabdomyosarcoma, small cell anaplastic carcinoma, Ewing sarcomal peripheral neuroectodermal tumor, and desmoplastic small round cell tumor), characterized both cytologically and histologically by a predominantly small round to oval, and relatively undifferentiated cells. Together they form a formidable group and an overwhelming majority of childhood malignancies. The patients may present in later (inoperable) stage with huge intrathoracic and intraabdominal mass, when chemotherapy and/or radiation therapy may be the first or only line of treatment. As a less invasive procedure fine needle aspiration (FNA) cytology has definite advantage over surgical excision biopsy to arrive at a tissue diagnosis before initiation of therapy. Because of the morphologic similarities, the SRCTs may pose a differential diagnostic problem in the practice of clinical cytology, especially when they are poorly differentiated. Important cytomorphological features, which help in the identification of various SRCTs include completely dissociated cell population and lymphoglandular bodies (cytoplasmic fragments) in non-Hodgkin lymphoma (NHL), eosinophilicfibrillar material and Homer-Wright rosettes along with cellular processes in neuroblastoma, acinar formation in hepatoblastoma, blastema cells with tubular differentiation in nephroblastoma, tadpole shaped cells in embryonal rhabdomyosarcoma, extreme nuclear molding and perinuclear blue inclusion in small cell anaplastic carcinoma (SCAC), irregular, punched out and large cytoplasmic vacuolations due to glycogen in Ewing sarcoma, and sheets of undifferentiated small round cells surrounded by collageneous stroma in desmoplastic small round cell tumor (DSRCT). Some of these features such as nuclear molding, rosette, and acinar formation are noticed in more than one type of SRCTs. Moreover, the characteristic cytomorphological features may be present in 70-80% cases and for categorization of the remaining cases, contribution from ancillary studies is essential. It is suggested that cytomorphological features along with one or more of the parameters such as special stains (cytochemistry), immunocytochemistry (ICC), electron microscopy (EM), tissue culture, DNA ploidy, karyotype and molecular analysis can increase the diagnostic accuracy of SRCTs. However, these facilities may not be available in all the laboratories, especially in the developing countries, and even if available in a limited form, a tissue diagnosis has to be offered often by FNA cytology based on morphological features, as a life saving measure in seriously ill patients before the results of ancillary studies are finalized.
Biopsy, Fine-Needle↗
Ewing's sarcoma. Pathology, tissue culture, and cytogenetics.
The pathology, in vitro growth pattern and karyotype of an Ewing's sarcoma are reported. Light and electron microscopic findings of a talus biopsy showed pathologic characteristics typical of Ewing's sarcoma. In vitro tumor cells grew on a monolayer as small, plump, refractile cells. Tumor cells were also capable of proliferating in soft agar. Chromosome studies of cultured cells showed two consistent abnormalities: trisomy 8 and a translocation, t(11;22)(q24;q13), which has been reported previously in Ewing's sarcoma. Since this tumor is frequently difficult to distinguish from other small cell tumors, the use of several techniques (electron microscopy, tissue culture, chromosome studies) may aid the diagnosis.
Adolescent↗
Primitive neuroectodermal tumour of the chest wall.
The concept of primitive neuroectodermal tumour (PNET) has been evolving for many years, as has its nomenclature. It was first described as a tumour arising in peripheral nerve, when it was called neuroepithelioma. These tumours are part of the differential diagnosis of malignant small round cell tumours, which include Ewing's sarcoma, rhabdomyosarcoma, neuroblastoma and lymphoma, and which appear as sheets of monotonous small round cells on light microscopy, staining dark blue with haematoxylin and eosin. In the 1970's, reports of tumours displaying neural features introduced new terms such as primitive neuroectodermal tumour and peripheral neuroectodermal tumour. A relationship to the undifferentiated primitive neuroectodermal tumours of the central nervous system in children was assumed by the title of these tumours, although it was recognised that they were not necessarily related to peripheral nerves.
Adult↗
Scanning electron microscopic evidence for neural differentiation in Ewing's sarcoma cell lines.
A number of recent studies have suggested a relationship between Ewing's sarcoma (ES) and other small round cell tumours of childhood such as peripheral neuroepithelioma (PN). We report scanning electron microscopic studies on the character of induced neural differentiation in ES, neuroblastoma, PN, osteosarcoma and colon carcinoma. We found evidence of neural differentiation in both neural lines and in one of two Ewing's lines before treatment. After differentiation, both Ewing's and neural lines developed neuritic processes with varicosities and little arborization, except for the initially undifferentiated Ewing's line (A4573) which displayed extensive lateral sprouting from neuritic processes after differentiation. Neither treated nor untreated osteosarcoma or colon carcinoma displayed any evidence of neural differentiation. Further, neuroblastoma cells are easily distinguished from ES and PN by virtue of their single, unbranched neurites and lack of lateral sprouting or filopodia. These results provide further evidence for the neural character and close relationship between ES and PN.
Adenocarcinoma↗
Fine-needle aspiration cytology of malignant fibrothecoma of the ovary.
Fibrothecomas are common, but their malignant counterpart is extraordinarily rare. To the best of our knowledge, this is the first report on the cytologic features of malignant fibrothecoma. We had an opportunity to study it because the 70-yr-old woman refused initial surgery until the tumor reached 22 cm in size and weighed 1, 500 gm. A CT-guided fine-needle aspiration biopsy was obtained from a 5 cm left pelvic mass, which was the second recurrence within 5 yr. The smears showed large fragments of tightly packed, small, oval cells with scanty, eccentric blue cytoplasm (Diff-Quik stain), and finely granular chromatin with small central nucleoli (Ultrafast Papanicolaou stain), transected by delicate blood vessels. The tumor resembled well-differentiated carcinoma, low-grade endometrial stromal sarcoma, and other small oval cell gynecologic neoplasms. Cytodiagnosis of nonepithelial ovarian neoplasms can be difficult. However, it is not impossible, especially for recurrent tumors with previously established histodiagnosis. Diagn. Cytopath. 21:284-286, 1999.
Aged↗
Drug evaluation: STA-4783--enhancing taxane efficacy by induction of Hsp70.
The apoptosis stimulator STA-4783 acts by inducing expression of heat shock protein (Hsp)70 on tumor cell surfaces and disrupting the cytoskeletal network. Currently in development by Synta Pharmaceuticals for the potential treatment of solid tumors, phase II clinical trials in non-small-cell lung cancer, melanoma and sarcoma have been initiated.
Animals↗
A brief-duration combination chemotherapy for elderly patients with poor-prognosis non-Hodgkin's lymphoma.
Curative combination chemotherapy is available for many patients with aggressive non-Hodgkin's lymphoma (NHL); however, treatment of elderly patients with these regimens is difficult due to excessive toxicity. From 1983 to 1988 the authors treated 26 patients 65 years and older with aggressive NHL with a novel 8-week chemotherapy regimen containing bleomycin, etoposide, cyclophosphamide, doxorubicin, methotrexate with leucovorin, and prednisone (BECALM), designed to preserve dose intensity and minimize toxicity. Median age was 75 years. Histologic types included the following: 20 intermediate grade (16 large noncleaved cell; two large cleaved cell; one intermediate grade, unspecified); six high grade (four small noncleaved cell; one immunoblastic sarcoma B-cell; one high grade, unspecified). Twenty-one patients were Stage III or IV. Twenty-two of 26 patients had one or more of the following: tumor greater than 10 cm; multiple extranodal sites; lactate dehydrogenase (LDH) 400 IU/l or greater; small noncleaved cell histologic type. Chemotherapy consisted of bleomycin 20 U intravenously (IV) weeks 1 and 7; etoposide 75 mg/m2 IV every day x 3 days on week 4; cyclophosphamide 600 mg/m2 IV weeks 1, 4, 7; doxorubicin 40 mg/m2 IV weeks 1, 7; methotrexate 50 mg/m2 IV weeks 1, 2, 4, 5, 7, 8 with oral leucovorin rescue; prednisone 60 mg orally for 10 days on weeks 1, 4, 7. Eighteen patients completed the 8-week treatment course. There were 13 complete responses (CR); seven patients remain in continuous CR at a median follow-up of 37.5 months. There have been five relapses, including one late relapse; and one patient died of an intercurrent illness in CR. Overall and actual event-free survivals are 38% and 27%, respectively. The major toxicities were neutropenic fever and mucositis. There were four treatment-related deaths. The authors conclude that BECALM chemotherapy can be administered to elderly patients with aggressive NHL. Although neurotoxicity and cumulative toxicity from bleomycin and anthracycline are avoided, the regimen remains moderately toxic, particularly with respect to myelosuppression. Treatment results compare favorably with other reported regimens in this group of patients with multiple poor prognostic features.
Aged↗
Prognostic factors and survival in late adolescent and adult patients with small round cell tumors.
The primary objective of this study is to review the clinical characteristics of 25 patients in the adult and late adolescent age group, diagnosed and treated with small round cell tumors involving soft tissues (extraosseous Ewing sarcoma, rhabdo-myosarcoma, primitive neuroectodermal tumor, and undiffer-entiated small round cell tumors). Additionally, survival and prognostic factors influencing the outcome with multimodality treatment are evaluated. There were 19 males (76%) and 6 females (24%). The median age was 26 years (range: 15-56 years). In 9 patients (36%), the tumor was located at an extremity, whereas 16 patients (64%) had central localizations. Tumor size was larger than 10 cm in 7 patients (29.2%). Six patients (24%) had metastatic disease. Twelve patients (48%) received radiation and 16 patients (64%) underwent surgery. Among the resected tumors, 2 were resected with contaminated margins (12.5%), whereas 2 were radically resected and 12 (75%) were resected with wide margins. All patients were given a median of 4 cycles of multiagent chemotherapy (1-14 cycles). With preoperative chemotherapy, complete regression (CR) of the tumor was achieved in 6 patients (24%). In 4 patients (16%), a partial response was obtained. After the completion of multimodality treatment, 12 patients (48%) had a CR. Progression-free (PFS) and overall survival (OS) for the entire group was 25.0 +/- 10.8% at 1 year and 30.5 +/- 15.5% at 3 years, respectively. Nonmetastatic disease, wide and radical resection, and presence of CR to multimodality treatment were associated with a significantly longer PFS and OS by univariate analysis. By multivariate analysis, CR to multimodality treat-ment was the only independent predictive factor for a longer OS (p: 0.0036, relative risk [RR]: 23.6, 95% CI: 2.8; 198.7) and metastatic presentation was the only independent factor predic-tive for a shorter PFS (p: 0.017, RR. 15, 95% CI: 1.6; 141.2). Large-scale, multicenter studies are required for a better eval-uation of the nonpediatric age group with small round cell tumors.
Adolescent↗
Diagnosis and behavior of juvenile rhabdomyosarcoma.
The main problems in the diagnosis of rhabdomyosarcoma are 1) distinction of undifferentiated examples from other small cell malignancies, especially soft-tissue Ewing's tumor and lymphoma; 2) distinction of spindling examples from fibrosarcoma, leiomyosarcoma, malignant fibrous histiocytoma, polyhistioma, and other sarcomas; 3) recognition of minimal criteria on small samples such as needle biopsy specimens or frozen sections; and 4) recognition of rhabdomyosarcoma in uncommon sites such as bone (mandible), perineum, retroperitoneum, and chest. In 95 pediatric cases diagnosed and treated at Royal Alexandria Hospital for Children--45 after the introduction of combined therapy--minimal diagnostic criteria were assessed. Cross-striations were found in only one third of cases; longitudinal myofibrils were more common and more helpful. There was much overlap between histologic types, and the microscopic patterns had little bearing on prognoses in preadolescent children. Fourteen cases could not be further differentiated ("embryonal sarcoma, probably rhabdomyosarcoma")--nine small-cell tumors; four tumors from genitourinary tract or head for which very small biopsy specimens were available, and one spindling retroperitoneal neoplasm. In all, slight evidence suggested embryonal rhabdomyosarcoma; this evidence included oat-shaped nuclei and, in a few cells, deeply eosinophilic cytoplasm, small elongated processes, or myxoid or alveolar foci--features that exclude lymphoma and Ewing's tumor. In six cases that were originally classified as poorly differentiated or undifferentiated, later material confirmed the presence of rhabdomyosarcoma by showing a predominantly well-differentiated (pleomorphic) or alveolar pattern after therapy. In 14 remaining undifferentiated cases, immunoperoxidase staining with antihuman-myoglobin serum was positive in five. With combined therapy there was 100 per cent survival among patients with paratesticular, limb, and stage I and stage II tumors; considerably improved survival among patients with head and neck, pelvic, and stage III tumors; and 100 per cent mortality among patients with intra-abdominal and stage IV tumors.
Biopsy↗