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PubMedDate not supplied

Rhabdomyosarcoma of the uterus: report of two cases, including one of the spindle cell variant.

Most uterine sarcomas fall into the category of leiomyosarcoma, endometrial stromal sarcoma, or undifferentiated sarcoma. Pure rhabdomyosarcomas are extremely rare, although a rhabdomyosarcomatous element may be present as a component of an adenosarcoma or carcinosarcoma (malignant mixed müllerian tumor). This report describes two uterine rhabdomyosarcomas in 28- and 67-year-old women. These were of spindle cell and pleomorphic types, respectively. At presentation the pleomorphic rhabdomyosaroma was stage IV, exhibiting massive pelvic and abdominal dissemination that mimicked an ovarian neoplasm. The spindle cell rhabdomyosarcoma was stage I, being confined to the uterus. Grossly, both uterine tumors had a polypoid appearance. Immunohistochemically, tumor cells were positive with the skeletal muscle markers sarcomeric actin, myoglobin, and myoD1. The patient with stage IV disease died within a short time of diagnosis and the other patient is alive and well at 2 years' follow-up. This report adds to the published literature on uterine rhabdomyosarcomas. This is the first reported uterine case of the spindle cell variant of embryonal rhabdomyosarcoma. Based on these cases and the published literature, rhabdomyosarcoma, especially the pleomorphic variant, appears to be a very aggressive neoplasm with an extremely poor prognosis. Immunohistochemical demonstration of skeletal muscle differentiation is necessary for a definitive diagnosis.

Actins↗

PubMed1985

Skeletal muscle actin as tumor marker in the diagnosis of rhabdomyosarcoma in childhood.

Immunoaffinity-purified and tissue-specific antibodies against carp skeletal muscle actin have been assessed for their usefulness in diagnosing rhabdomyosarcoma. Routinely processed formaldehyde-fixed tissue and the avidin--biotinyl--peroxidase complex technique were used. Thirty-six tumors of patients varying in age from less than 1 year to 17 years were diagnosed as rhabdomyosarcoma on the basis of routine histological stains or electron microscopy, and on clinical grounds. Among them were 20 poorly differentiated tumors. All moderately and well-differentiated rhabdomyosarcomas and the majority of the poorly differentiated tumors (13 of 20) showed positive immunostaining for actin. Positive staining was observed in all three types of rhabdomyosarcoma, i.e., embryonal, alveolar, and pleomorphic. Besides rhabdomyosarcomas, the only other positive neoplasms were those that contained rhabdomyoblastic differentiation such as malignant "triton" tumors and malignant mixed müllerian tumors. Our results indicate that antibodies against skeletal muscle actin are a powerful tool for diagnosing rhabdomyosarcoma and that they can be used to distinguish the poorly differentiated forms from other types of small round cell tumors in childhood such as neuroblastoma, Ewing's sarcoma, and malignant lymphoma. The results are discussed in the light of the embryogenesis of cross-striated skeletal muscle.

Actins↗

PubMedDate not supplied

HER/erbB receptors as therapeutic targets of immunotoxins in human rhabdomyosarcoma cells.

Human rhabdomyosarcoma cells express HER/erbB growth factors receptors. Receptors belonging to this family are overexpressed and play a role in many types of epithelial and neural cancer and have been selected as targets for cancer therapy. In this paper EGF-R, HER-2 and HER-3 receptors were tested as therapeutic targets of immunotoxins in human rhabdomyosarcoma. Rhabdomyosarcoma cells were treated with indirect immunotoxins consisting in primary specific murine monoclonal antibodies recognizing EGF-R, HER-2 and HER-3 followed by secondary F(ab')2 antimouse immunoglobulin linked to saporin-S6, a type 1 ribosome-inactivating protein (RIP) from the seeds of Saponaria officinalis. The indirect immunotoxin targeting EGF-R caused a significant inhibition in cell growth and protein synthesis and a strong increase in apoptosis in rhabdomyosarcoma cells, whereas indirect immunotoxins against HER-2 and HER-3 were ineffective. The toxic activity of anti-EGF-R immunotoxin was also observed on rhabdomyosarcoma cells expressing low level of EGF-R. EGF-R could be a novel therapeutic target of immunotoxins in human rhabdomyosarcoma.

Antibodies, Monoclonal↗

PubMed1983

Immunohistochemistry of myoglobin in rhabdomyosarcomas.

Immunoperoxidase staining for myoglobin was performed on formalin-fixed, paraffin-embedded tissues of normal human skeletal muscle and rhabdomyosarcomas in order to assess its potential diagnostic value. Myoglobin-positive cells were observed in normal adult and fetal muscles, and in 13 of 26 rhabdomyosarcomas. In normal skeletal muscle, the intensity of staining for myoglobin varied in individual cells. Of the 13 myoglobin-positive rhabdomyosarcomas tested, 6 showed cross striations in tumor cells, whereas the others did not. Myoglobin-positive tumor cells were not demonstrated in two rhabdomyosarcomas in which cross striations were observed. In other types of tumors examined that sometimes resembled rhabdomyosarcomas, no myoglobin was detected. Thus tests for myoglobin by the immunoperoxidase method seem helpful in identifying rhabdomyosarcomas and confirming their histological diagnosis.

Adolescent↗

PubMed2006

Rhabdomyosarcoma lysis by T cells expressing a human autoantibody-based chimeric receptor targeting the fetal acetylcholine receptor.

Rhabdomyosarcomas are the most frequent malignant soft tissue tumors of childhood; however, because current multimodality treatments fail to improve the poor survival rate of children with metastatic rhabdomyosarcoma, new treatments are required. We previously identified the gamma-subunit of the fetal acetylcholine receptor (fAChR) as a specific cell surface target in rhabdomyosarcoma. Here, we engineered human T lymphocytes to express chimeric receptors composed of the antigen-binding domain of a human anti-fAChR antibody joined to the signaling domain of the human T-cell receptor zeta-chain. The interaction of fAChRzeta-transduced T cells with fAChR-positive rhabdomyosarcoma cell lines, but not with fAChR-negative control cells, induced T-cell activation characterized by strong secretion of IFN-gamma and delayed lysis of tumor cells. Importantly, we found that in six of six rhabdomyosarcoma patients, chemotherapy increased fAChR expression on residual tumor cells in vivo. Our observations suggest that these fully human chimeric fAChRzeta-transduced T cells, which should be well tolerated by the patient, have potential use in vivo both as a primary treatment for rhabdomyosarcoma and as a complementary approach to eradicate residual tumor cells after chemotherapy.

Autoantibodies↗

PubMedDate not supplied

Rhabdomyosarcoma of the urachus. A case report.

BACKGROUND: Rhabdomyosarcoma of the urachus is extremely rare, and only two cases have been reported. Cytologic, histologic, immunochemical and electron microscopic findings in embryonal rhabdomyosarcoma of the urachus are presented. CASE: Embryonal rhabdomyosarcoma of the urachus developed in a 2-year-old boy. Imprint smears prepared prior to a histologic examination for a rapid report contained many malignant cells isolated or in clusters in a myxoid background. They were uniformly small, with round to oval nuclei and scant cytoplasm, and immunohistochemically positive for desmin and alpha-sarcomeric muscle actin but negative for myoglobin. This tumor was diagnosed cytologically as embryonal rhabdomyosarcoma because of its skeletal muscle origin and because of its similarity to one of the so-called small round cell tumors of childhood. Histologic examination confirmed the cytologic diagnosis. CONCLUSION: Cytologic, histologic and electron microscopic findings were identical to those described previously for embryonal rhabdomyosarcoma at other sites. In immunochemical examinations, cytologic samples fixed in ethanol are more useful than histologic ones fixed in formalin. Then, in order to differentiate poorly differentiated rhabdomyosarcoma from other small round cell tumors in children, desmin and alpha-sarcomeric actin, rather than myoglobin, are recommended.

Actins↗

PubMed2001

Which patients with microscopic disease and rhabdomyosarcoma experience relapse after therapy? A report from the soft tissue sarcoma committee of the children's oncology group.

PURPOSE: To identify which patients with rhabdomyosarcoma and microscopic residual disease (group II) are likely to not respond to therapy. PATIENTS AND METHODS: Six hundred ninety-five patients with group II tumors received chemotherapy and 90% received radiation therapy on Intergroup Rhabdomyosarcoma Study (IRS)-I to IRS-IV (1972 to 1997). Tumors were subgrouped depending on the presence of microscopic residual disease only (subgroup IIa), resected positive regional lymph nodes, (subgroup IIb), or microscopic residual disease and resected positive regional lymph nodes (subgroup IIc). RESULTS: Overall, the 5-year failure-free survival rate (FFSR) was 73%, and patients with embryonal rhabdomyosarcoma treated on IRS-IV fared especially well (5-year FFSR, 93%; n = 90). Five-year FFSRs differed significantly by subgroup (IIa, 75% and n = 506; IIb, 74% and n = 101; IIc, 58% and n = 88; P = .0037) and treatment (IRS-I, 68%; IRS-II, 67%; IRS-III, 75%; IRS-IV, 87%; P < .001). Multivariate analysis revealed positive associations between primary site (favorable), histology (embryonal), subgroup IIa or IIb, treatment (IRS-III/IV), and better FFSRs. Patterns of treatment failure revealed local failure to be 8%, regional failure, 4%, and distant failure, 14%. The relapse pattern noted over the course of IRS-I to IRS-IV shows a decrease in the systemic relapse rates, particularly for patients with embryonal histology, suggesting that improvement in FFSRs is primarily a result of improved chemotherapy. CONCLUSION: Group II rhabdomyosarcoma has an excellent prognosis with contemporary therapy as used in IRS-III/IV, and those less likely to respond can be identified using prognostic factors: histology, subgroup, and primary site. Patients with embryonal rhabdomyosarcoma are generally cured, although patients with alveolar rhabdomyosarcoma or undifferentiated sarcoma, particularly subgroup IIc at unfavorable sites, continue to need better therapy.

Adolescent↗

PubMed2005

Relationship between MYCN copy number and expression in rhabdomyosarcomas and correlation with adverse prognosis in the alveolar subtype.

PURPOSE: Amplification of the transcription factor MYCN is an important molecular diagnostic tool in stratifying treatment for neuroblastoma. Increased copy number and overexpression of MYCN in the pediatric cancer rhabdomyosarcoma has been described in a number of small studies with conflicting conclusions about its association with clinicopathologic characteristics. We aimed to study the phenomenon in the largest series to date. PATIENTS AND METHODS: Using quantitative polymerase chain reaction, we measured MYCN copy number and expression levels in rhabdomyosarcoma samples from 113 and 92 individuals with a confirmed diagnosis of rhabdomyosarcoma, respectively. RESULTS: Increased copy number of MYCN was found to be a feature of both the embryonal and alveolar subtypes. The copy number and expression levels were significantly greater in the alveolar subtype, although the range of expression in both subtypes spanned several orders of magnitude. MYCN copy number showed a significant correlation with expression in the alveolar subtype; this relationship between copy number and expression could be modeled as a logarithmic function. It is notable that relatively high expression frequently occurred in embryonal rhabdomyosarcoma without high copy number and that low expression was found in some cases with high copy number. In patients with alveolar rhabdomyosarcoma, overexpression (greater than median) or gain of genomic copies of MYCN were significantly associated with adverse outcome. CONCLUSION: MYCN deregulation is a feature of rhabdomyosarcoma tumorigenesis, defines groups of patients with a poor prognosis, and is a potential target for novel therapies.

Child↗

PubMed1982

Rhabdomyosarcoma of the head and neck.

Rhabdomyosarcoma is an uncommon neoplasm that is seen in the head and neck area. Since the symptoms of rhabdomyosarcoma, such as aural discharge and nasal obstruction, mimic common disease states, the clinical diagnosis is often delayed. Case reports and a literature review are presented to emphasize the clinical and roentgenological findings in rhabdomyosarcoma. Misinterpretation of microscopic findings can delay the histological diagnosis. Cross-striations, the "hallmark" of rhabdomyosarcoma, are not always found, while "racquet" and "spindle" cells may be seen. Special stains and electron microscopy can assist the pathologist. The pathological findings of the different variants of rhabdomyosarcoma and a review of cases accessioned by the A.F.I.P. Otolaryngic Registry are presented. Early diagnosis of rhabdomyosarcoma is important because the disease, once inevitably fatal, can now be controlled and apparently cured in most cases by a combination of surgery, radiation, and multidrug chemotherapy. This therapeutic approach, its results, complications, and support measures are considered.

Adolescent↗

PubMed1998

[Comparison of the transcription of the fetal type acetylcholine receptor and myogenin in rhabdomyosarcoma].

Myogenin and the fetal type acetylcholine receptor (AChR) subunits are specific immunohistochemical markers for the diagnosis of rhabdomyosarcomas. In spite of light microscopy and immunohistochemistry, the diagnosis of doubtful cases and small biopsies of rhabdomyosarcomas remains a challenge. Therefore, a PCR-based highly sensitive approach, would be a valuable diagnostic adjunct and should be free from the risk of contamination of the tumor sample with normal tissue. We studied the transcription of myogenin and fetal type AChR in rhabdomyosarcomas, other childhood and adult tumors and normal tissues. In all embryonal and alveolar rahbdomyosarcomas transcripts of both myogenin and fetal type AChR could be detected. The detection of myogenin mRNA however was not specific for rhabdomyosarcomas but occurred in normal muscle and the majority of other normal tissues and childhood tumors. In contrast the transcription of fetal type AChR, which is defined by an alpha subunit AChR/gamma subunit AChR ratio < 1 was encountered only in rhabdomyosarcomas and denervated muscle. Therefore we suggest, that mRNA of the fetal type AChR but not myogenin is a highly specific and sensitive target for the PCR-based diagnosis of rhabdomyosarcomas.

Adult↗

PubMed2000

The use of magnetic resonance imaging in the diagnosis and followup of pediatric pelvic rhabdomyosarcoma.

PURPOSE: Previous radiological descriptions of pelvic rhabdomyosarcoma emphasized ultrasonography and computerized tomography (CT). Few reports are available on the use of magnetic resonance imaging (MRI) for diagnosing and following pelvic rhabdomyosarcoma. We retrospectively compared MRI to CT for diagnosing and following children with pelvic rhabdomyosarcoma. MATERIALS AND METHODS: We treated 4 boys and 3 girls for pelvic rhabdomyosarcoma. Initial and followup evaluations included pelvic CT and MRI at intervals determined by treatment and disease status. We retrospectively reviewed the clinical charts and imaging studies of these patients. The initial radiological report was evaluated and then 1 radiologist reviewed all studies. Attention was directed toward identifying lesions revealed by CT or MRI but not by the other modality. RESULTS: MRI detected all lesions shown by CT. On the other hand, MRI detected residual disease in 1 case that was not demonstrated by CT. In 2 other patients MRI was superior to CT for delineating the local extent of disease, especially urethral involvement. CONCLUSIONS: Compared with CT, MRI improves the detection of residual pelvic rhabdomyosarcoma. Tissue planes are well delineated, allowing more accurate assessment of tumor invasion into adjacent structures. MRI is the imaging modality of choice for following pediatric patients with pelvic rhabdomyosarcoma.

Child↗

PubMed2000

Expression of HER/erbB family of receptor tyrosine kinases and induction of differentiation by glial growth factor 2 in human rhabdomyosarcoma cells.

Five human rhabdomyosarcoma cell lines were used to investigate the surface expression of HER/erbB receptors, particularly of HER-2, HER-3 and HER-4, by flow cytometry. HER-2 was expressed in 3/5 rhabdomyosarcoma cell lines. HER-3 was expressed in all rhabdomyosarcoma cell lines. None of the rhabdomyosarcoma cell lines investigated showed HER-4 surface expression. To study the biological activity of HER/erbB receptors in rhabdomyosarcomas, cells were cultured in the presence of glial growth factor 2 (GGF-2), a specific ligand of HER-3 that is able to stimulate myogenesis in normal myoblasts. In 3 of 3 human rhabdomyosarcoma cell lines positive for both HER-2 and HER-3 receptors, the treatment with GGF-2 induced a significant increase in myogenic differentiation.

Cell Differentiation↗

PubMed1990

In vitro purging of human rhabdomyosarcoma cells using 4-hydroperoxycyclophosphamide.

The outcome of patients with advanced stage rhabdomyosarcoma is extremely poor, with a disease-free survival of less than 20% at 3 years. Autologous bone marrow transplantation for patients with Clinical Group IV rhabdomyosarcoma may be an effective therapy. The bone marrow involvement diagnosed by light microscopy is 29% for patients with advanced disease. The present study was performed to test the ability of 4-hydroperoxycyclophosphamide (4-HC) to eliminate clonogenic rhabdomyosarcoma cells. Four different human rhabdomyosarcoma cell lines were treated in vitro with 4-HC at a concentration of 100 micrograms/ml. Limiting dilution analysis was performed to detect surviving clonogenic tumor cells. Treatment with 4-HC resulted in 1.7-5.7 log of elimination of clonogenic tumor cells in all four cell lines. Exactly the same log tumor cell kill was obtained after mixing normal human bone marrow mononuclear cells with rhabdomyosarcoma cells. Treatment with 4-HC may be an effective method of eliminating clonogenic rhabdomyosarcoma cells ex vivo.

Bone Marrow↗

PubMed1988

Alveolar and poorly differentiated rhabdomyosarcoma. A clinicopathologic, light-microscopic, ultrastructural and immunohistochemical analysis.

Eighteen poorly differentiated, small and dark cell malignancies afflicting young individuals without light-microscopic evidence of a rhabdomyoblastic differentiation or a growth pattern characteristic of rhabdomyosarcoma were analyzed and compared with a series of 30 alveolar rhabdomyosarcomas of varying differentiation, where the diagnosis could be established light-microscopically. The study comprised clinical data, light and electron microscopy and immunohistochemistry, using a battery of mono- and polyclonal antibodies against intermediate filaments, myoglobin, epithelial membrane antigen, neuron-specific enolase, S-100 and leucocyte common antigen. All 30 alveolar rhabdomyosarcomas were positive for desmin, while a minority were positive for myoglobin, using monoclonal antibodies. In 8 of the 18 small and dark cell malignancies, support for a rhabdomyoblastic differentiation was obtained by a positive staining for desmin. In only 3 of these 8 cases was there ultrastructural evidence of rhabdomyosarcoma. The results of the investigation indicate that immunohistochemistry is a more useful tool than electron microscopy in the diagnosis of poorly differentiated rhabdomyosarcoma and that the criteria for the diagnosis of poorly differentiated rhabdomyosarcoma may need to be reformulated.

Antibodies, Monoclonal↗

PubMed1980

Antimyosin and antirhabdomyoblast sera: their use for the diagnosis of childhood rhabdomyosarcoma.

Antisera against myosin of human normal skeletal muscle and against rhabdomyoblasts of autopsy-proved rhabdomyosarcoma were raised in white rabbits, purified, and assessed for their usefulness in the diagnosis of childhood rhabdomyosarcoma. The specificity of the antisera was tested by both immunofluorescence and immunoperoxidase methods in seven cases of autopsy-proved rhabdomyosarcoma, five of malignant lymphoma, three of neuroblastoma, and two of Ewing's sarcoma. Antimyosin serum tested positive for all cases of rhabdomyosarcoma and negative for other types of tumor. Antirhabdomyoblast serum was positive in all cases of rhabdomyosarcoma and cross-reacted with cases of neuroblastoma and Ewing's tumor, although the intensity of staining was much decreased. Our results indicate that antimyosin serum is specific for childhood rhabdomyosarcoma and can be used to differentiate this from other childhood "round cell" tumors.

Adolescent↗

PubMed1981

[Immunomorphological method in the diagnosis of rhabdomyosarcomas].

Eight rhabdomyosarcomas were examined by indirect Coons method using rabbit antisera to myosin of human striated and smooth muscles. Positive immunofluorescence with the antiserum to striated muscle myosin was observed in 6 out of 8 tumors. In the preparations treated with the antiserum to smooth muscle myosin, fluorescence of smooth muscles of the blood vessels was observed. Immunofluorescence was absent in 1 embryonal and 1 pleomorphic rhabdomyosarcomas. The tumors with positive fluorescence included 2 embryonal, 1 alveolar rhabdomyosarcomas, 1 metastasis of testicular rhabdomyosarcoma into the lymph nodes, 1 rhabdomyosarcoma of undifferentiated type, and 1 rhabdomyosarcoma with signs of therapeutic pathomorphosis. The high resolving capacity of the method allows its use for specification ppf histological diagnosis of tumors with different levels of cytodifferentiation.

Animals↗

PubMed1997

GOK: a gene at 11p15 involved in rhabdomyosarcoma and rhabdoid tumor development.

The expression of GOK, a gene recently identified at 11p15.5, was studied in breast cancer, rhabdomyosarcoma, and rhabdoid tumor cell lines. In these neoplasms, deletions at 11p15 and suppression of tumorigenicity induced by a normal human chromosome 11 were previously demonstrated. Whereas breast cancer cell lines express readily detectable levels of GOK mRNA, expression is absent in rhabdomyosarcoma and rhabdoid tumor cell lines. This is in contrast with the high expression of GOK in skeletal muscle, the normal tissue of origin of rhabdomyosarcomas, suggesting that down-regulation of GOK expression could be involved in tumor development. In agreement with this hypothesis, transfection of GOK cDNA into G401 derived from a rhabdoid tumor and RD cells derived from a rhabdomyosarcoma that do not express detectable levels of GOK mRNA, induced cell death. Because GOK expression is not compatible with growth of these tumor cells, these results support the hypothesis that loss of GOK expression plays a role in tumor establishment or progression and suggest that GOK may act as a recessive tumor suppressor gene in rhabdomyosarcomas and rhabdoid tumors. On the contrary, transfection of GOK cDNA into the breast cancer cell line HBL100 produced no detectable effects, indicating that the growth-suppressive effect of GOK in RD and G401 cells was specific. Because rhabdomyosarcomas have been observed in cases of Beckwith-Wiedemann syndrome, a genetic disorder linked to 11p15, a role of GOK in this disease cannot be excluded.

Chromosome Mapping↗

PubMed1998

A Southwest Oncology Group and Cancer and Leukemia Group B phase II study of doxorubicin, dacarbazine, ifosfamide, and mesna in adults with advanced osteosarcoma, Ewing's sarcoma, and rhabdomyosarcoma.

BACKGROUND: Ewing's sarcomas, osteosarcomas, and rhabdomyosarcomas are significantly more responsive to chemotherapy than other sarcomas. Adjuvant chemotherapy is used routinely based on data from randomized trials. Although a percentage of children with locally advanced or metastatic tumors remain curable, few data exist regarding the tumor's natural history or response and survival in adults. METHODS: This Phase II study evaluated doxorubicin, dacarbazine, ifosfamide, and mesna (MAID) in adults with inoperable or metastatic Ewing's sarcoma, rhabdomyosarcoma, or osteosarcoma. RESULTS: Between 1987-1991, 81 patients were entered; 69 patients were eligible. One patient died of neutropenic infection. Ten patients (14%) responded completely and 34 patients (49%) had a complete or partial response. Response rates were significantly higher for patients with Ewing's sarcoma and rhabdomyosarcoma than for those with osteosarcoma (77%, 64%, and 26%, respectively; P < 0.005). Although there were no significant differences in progression free survival by histology, survival for patients with Ewing's sarcoma was significantly longer than for patients with osteosarcoma (P = 0.004.) At the time of last follow-up, 7 patients (10%) were alive without progression: 3 with Ewing's sarcoma, 1 with osteosarcoma, and 3 with rhabdomyosarcoma. CONCLUSIONS: MAID chemotherapy is an active regimen in adults with advanced or metastatic Ewing's sarcoma and rhabdomyosarcoma. Although there was no direct comparison with a doxorubicin and cisplatin-based regimen, the response rate and survival in patients with osteosarcoma suggest that doxorubicin and cisplatin-based chemotherapy would remain the accepted initial chemotherapy regimen. For patients with rhabdomyosarcoma and Ewing's sarcoma, 10-20% of patients remained disease free at 5 years.

Adolescent↗