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PubMed1991

[Evolution of drug treatment of rhabdomyosarcoma in children].

Rhabdomyosarcoma is the most frequently occurring type of malignant solid tumors in children. The tumor site, aggressive growth, proneness to recurrence, metastatic spreading, and high neglect predetermine the negligible share of the surgical method in multimodality treatment of rhabdomyosarcomas. Of importance are the development and the use of different types and schemes of drug therapy which can be employed in combination with radiotherapy. Drug therapy of rhabdomyosarcomas has large practical potentialities which rise from year to year.

Antineoplastic Agents↗
PubMed1991

Carcinocythemia in a patient with rhabdomyosarcoma.

Solid tumor cells are rarely seen in peripheral blood smears. When this occurs the term carcinocythemia is used. This report describes an 18-year-old female who presented with a painless lump in the labia majora associated with pancytopenia. Tumor cells were identified in the peripheral blood smear and the bone marrow aspirate showed a predominant population of small round vacuolated primitive cells, many of which formed clumps of varying sizes. Biopsies of the vulvar mass and bone marrow were interpreted as alveolar rhabdomyosarcoma. Review of the literature revealed 12 previously reported cases in whom carcinocythemia had been documented; rhabdomyosarcoma was the specific cell type involved in only two of these. The median time between detection of the leukemic phase of the tumor and death was 8.5 weeks, reflecting the fact that carcinocythemia, when it occurs, represents the terminal event of the disease. To our knowledge, our case is the third well documented case of rhabdomyosarcoma in leukemic phase so far reported. The clinical evolution as well as the management of this patient will be described in detail along with a review of the pertinent available literature.

Adolescent↗
PubMedDate not supplied

Embryonal rhabdomyosarcoma of orbit--a case report.

Embryonal rhabdomyosarcoma of orbit presenting as a case of rapid proptosis of the right eye is reported in a 4 year old male child. There was no evidence of recurrence during a follow up of 6 months. Rhabdomyosarcoma is one of the most common primary malignant orbital neoplasms of child hood. It usually produces a precipitously progressing unilateral proptosis of sudden onset. It is a highly malignant neoplasm of pleuripotential embryonic mesoderm, which commonly differentiates to form cells similar to rhabdomyoblasts of the foetus. Because of the presence of elongated cells that contain abundance of eosinophilic glycogen rich cytoplasm, it is generally referred to as embryonal form of rhabdomyosarcoma.

Child, Preschool↗
PubMed1990

Orbital rhabdomyosarcoma. Immunohistochemical studies of seven cases.

An immunohistochemical analysis using a series of mono- and polyclonal antibodies against myoglobin, desmin, vimentin, actin, NSE, S-100 protein and keratin was done on alveolar rhabdomyosarcoma (3 cases), embryonal (3) and pleomorphic rhabdomyosarcoma of the orbit (1). All tumors were stained by monoclonal antibodies to desmin and vimentin, whereas 6 of the 7 cases were stained by myoglobin. The results indicate that desmin and vimentin are highly sensitive immunohistochemical markers for the diagnosis of rhabdomyosarcoma, especially in poorly differentiated ones.

Adult↗
PubMed1990

[Rhabdomyosarcoma in children].

Issues in diagnosis and complex treatment of pediatric rhabdomyosarcoma were studied in 198 patients with morphologically verified disease. Tumors most often developed in the genitourinary organs (36.6%), head and neck (37%) and--less frequently--on the trunk and extremities (26.4%). The diagnostic workup included instrumental methods, ultrasonography and computed tomography. All modern modalities of cancer treatment, viz. surgery, drug and radiotherapy were used in those patients. As a rule, treatment was either combined or complex. Two-year-survival rate ranged from 83% for rhabdomyosarcoma of the orbit to 50% for those of the trunk and extremities and 47% for head and neck neoplasms. Two-year survival for patients with rhabdomyosarcoma of non-genitourinary sites was 54%.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Amplification of N-myc in a rhabdomyosarcoma.

N-myc is a member of the myc oncogene family and has been thought to be specific to neurogenic cells, since it is amplified in some neuroblastomas, retinoblastomas and small-cell lung carcinomas with endocrine properties. Hybridization analyses of DNAs isolated from surgically removed tumors revealed that N-myc was amplified about 6-fold in one of the three embryonic rhabdomyosarcomas examined. The rhabdomyosarcoma containing the amplified N-myc had metastasized into bone marrow, which is preferentially involved in the metastasis of neuroblastomas but is usually not involved in the case of rhabdomyosarcomas. Since rhabdomyoblasts are derived from mesenchyme, this indicates that N-myc gene amplification is not restricted to neurogenic tumors.

Child↗
PubMed1988

Rhabdomyosarcoma presenting as a cutaneous nodule.

Two patients with rhabdomyosarcoma presented clinically with dermal nodules on the face. The first patient was a 12-month-old girl with a 2 X 3-cm-diameter red nodule on her left cheek that had begun as a small red papule when she was 3 months old and had progressively increased in size. The second patient was a 19-year-old girl who presented with an enlarging mass on her right cheek that had begun as a pea-sized nodule three months previously. In both cases, biopsies revealed a rhabdomyosarcoma in the underlying soft tissue with extension into the overlying dermis. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.

Adult↗
PubMed1989

Response of cultured human cell lines from rhabdomyosarcoma xenografts to treatment with chloroethylnitrosoureas.

DNA interstrand cross-links are thought to be the cytotoxic lesion resulting from treatment of cells with the chlorethylnitrosoureas (CENUs). We showed in an earlier study that the resistance of xenografts of pediatric rhabdomyosarcoma to therapy with CENUs correlates with levels of O6-alkylguanine-DNA alkyltransferase. We now demonstrate a relationship between levels of the alkyltransferase and CENU-induced cytotoxicity and DNA-interstrand cross-link formation in two cell lines recently established from such rhabdomyosarcoma xenografts. Rh18 cells were derived from the HxRh18 xenograft line, which contains the alkyl-transferase and is relatively resistant to CENUs, and Rh28 cells were derived from the HxRh28 xenograft line, which lacks detectable alkyltransferase activity and is sensitive to treatment with the CENUs. In vitro, Rh28 cells were 5- to 6-fold more sensitive to growth inhibition by 1,3-bis(2-chloroethyl)nitrosourea than Rh18 cells. Extracts of Rh18 cells contained 3.8 units of the alkyltransferase per mg of protein, whereas such activity was undetectable in Rh28 cells, a unit of the alkyltransferase being defined as 1 pmol of [3H] methyl transferred from [3H]methyl-labeled DNA to protein. DNA interstrand cross-links, measured by alkaline elution 6 h after a 1-h pulse treatment with CENU, could not be detected in Rh18 cells but were found in the Rh28 line. The phenotypes of the parental xenograft lines defined by their alkyltransferase levels and by responses to CENU therapy of the mice have clearly been retained in the cultured cell lines, and as predicted, cross-link formation was inhibited in the alkyltransferase-containing Rh18 cells. These two new cell lines thus provide a useful model for studying the role of DNA repair in rhabdomyosarcoma resistance to these alkylating agents.

Animals↗
PubMed1989

[A case of advanced paratesticular rhabdomyosarcoma].

A case of paratesticular rhabdomyosarcoma is presented. An 18-year-old male was admitted with the complaint of giant scrotal swelling and abdominal fullness on September 5, 1986. Left radical orchiectomy was performed with the pathologic diagnosis of alveolar rhabdomyosarcoma. The tumor was paratesticular in location and had invaded a spermatic cord. Radiological examination showed a gross metastatic mass in retroperitoneal lymph nodes, supraclavicular lymph nodes and Douglas pouch. The patient received induction chemotherapy containing vincristine, actinomycin-D, cyclophosphamide, bleomycin, CDDP and VP-16. After 3 courses, he had no mass in Douglas pouch and supraclavicular lesion. He received retroperitoneal lymph node dissection for residual retroperitoneal mass, and postoperative radiotherapy was given. However, recurrent disease was developed in the paraaortic region with malignant ascites. He was treated with salvage chemotherapy, had without any significant effect. He died of liver dysfunction due to progressive mass in hepatic hilum. A review of the current approach of paratesticular rhabdomyosarcoma with the usefulness of combination chemotherapy is given.

Adolescent↗
PubMed1989

[A case of embryonal rhabdomyosarcoma of the spermatic cord].

A case of rhabdomyosarcoma of the spermatic cord in a 6-year-old boy is reported. On February 26, 1986, he visited Nakamura Hospital with the chief complaint of enlargement of the left scrotal content. The contralateral scrotum was normal. The left scrotal content was a hard thumb-head-sized tumor. The left testis and epididymis were not distinguishable from the tumor. On the same day, left high inguinal orchiectomy was performed. The tumor was 3.5 by 2.5 by 2.5 cm in size and was distinguishable from the testis, epididymis and tunica vaginalis. Histopathological findings were embryonal rhabdomyosarcoma and it appeared to have originated from the spermatic cord. Two years after operation, the boy is living without metastasis. Including our experience, 101 cases of the paratesticular rhabdomyosarcoma found in Japanese literature are reviewed and briefly discussed.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

Rhabdomyosarcoma of the alveolar ridge in an adult.

Rhabdomyosarcoma of the head and neck is rare in adults, and past reports indicate that it is a more aggressive disease than that which is found in children. We report the case of a 63-year-old woman with an alveolar type of rhabdomyosarcoma located on the mandibular alveolar ridge. Rhabdomyosarcoma of the oral cavity appears to carry a particularly poor prognosis, especially when bone is involved. Multimodal treatment with surgery, radiation therapy, and chemotherapy is indicated in all patients.

Alveolar Process↗
PubMed1989

Rhabdomyosarcoma and hypercalcemia.

Hypercalcemia has not been reported as a complication of rhabdomyosarcoma in adults. We present the case of a 56-year-old man with pain in his right leg and hypercalcemia secondary to a large pelvic tumor. He had a rapidly progressive downhill course resulting in death due to tonsillar herniation. Although the results of special stains were negative, light microscopy showed poorly differentiated tumor, with features consistent with rhabdomyosarcoma. In children, hypercalcemia secondary to rhabdomyosarcoma is not uncommon and has been associated with bony metastasis, increased parathyroid hormone, and increased levels of prostaglandin E2.

Humans↗
PubMed1987

Reciprocal cross-resistance in human rhabdomyosarcomas selected in vivo for primary resistance to vincristine and L-phenylalanine mustard.

Primary resistance to vincristine (VCR) has been selected in rhabdomyosarcoma xenograft HxRh12 by sequential administration of VCR at 1.5 and subsequently 3 mg/kg/passage. The resistant tumor (HxRh12/VCR-3) was approximately 4-fold resistant to VCR and resistance was stable in the absence of selecting pressure (greater than 2 yr). HxRh12/VCR-3 was 2- to 3-fold cross-resistant to L-phenylalanine mustard (L-PAM) but only slightly cross-resistant to ifosfamide. To determine whether selection for primary resistance to L-PAM conferred cross-resistance to VCR we selected an L-PAM-resistant subline of rhabdomyosarcoma xenograft HxRh28 (HxRh28/L-PAM-13). This tumor was 2- to 3-fold resistant to L-PAM and 3-(p-fluorophenyl)-L-alanyl-3-[m-bis-(2-chloroethyl)-aminophenyl]-L- alanyl-L-methionine ethoxyhydrochloride, cross-resistant to cyclophosphamide and ifosfamide, and completely resistant to VCR under in vivo conditions. Pharmacokinetic studies in HxRh12/VCR-3 showed decreased retention of [G-3H]VCR but not alteration in metabolism. Expression of mdr1, a gene that encodes P-glycoprotein, associated with the multiple drug resistance phenotype, was examined. Expression of mdr1 was detected in both HxRh12 and HxRh28 tumors, sensitive to VCR, but there was no increase in expression in tumors selected for primary resistance to VCR or L-PAM. Data suggest that mechanisms other than those associated with "classical" multiple drug resistance confer resistance in these tumors. In clinical evaluation against childhood rhabdomyosarcoma, L-PAM has demonstrated only slight activity in patients relapsing on conventional therapy (including VCR) but demonstrated marked activity in patients with advanced previously untreated disease. It appears likely, therefore, that cross-resistance between VCR and L-PAM as demonstrated in this model may have clinical significance.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1988

Primitive rhabdomyosarcoma presenting with diffuse bone marrow involvement: an immunohistochemical and ultrastructural study.

We recently identified three cases of primitive rhabdomyosarcoma (PRMS) presenting with diffuse bone marrow infiltration but inconspicuous soft tissue primaries, referred to The Johns Hopkins Hospital (JHH) as acute leukemia. In each case, the diagnosis of rhabdomyosarcoma was established using immunohistochemical staining and electron microscopy. Ultrastructural examination of tumor cells showed a feltwork of thin filaments, discontinuous basal lamina, glycogen, and primitive cell junctions without cell processes or neurosecretory granules. This presentation of PRMS may be more common than recognized, since it can be readily misdiagnosed as a hematopoietic tumor. While positive staining for muscle-specific actin, desmin, myoglobin, or other markers of skeletal muscle differentiation may be diagnostic, negative staining is inconclusive, requiring recognition of the "minimal" ultrastructural findings of primitive rhabdomyosarcomas.

Actins↗
PubMed1986

[A resected case of collision tumor of hepatocellular carcinoma and primary liver rhabdomyosarcoma].

Primary liver sarcoma is rare and especially its coexistence with hepatocellular carcinoma is extremely rare. We have collected eight cases of their coexistence in the literatures. The patient was a 62-year-old male complaining of right hypochondralgia and general malaise for about one month. CT scanning revealed the liver tumor and right hepatic lobectomy was performed. The tumor was sized in 14 by 9 by 9 cm and composed of hepatocellular carcinoma and rhabdomyosarcoma, which were were collided. Apparent striations in the rhabdomyosarcoma cells was observed under light-microscope. Alphafetoprotein, of which serum level was extraordinarily high, was detected only in hepatocellular carcinoma by special stains. He discharged one month after operation. He was readmitted due to recurrence and received trans-arterial embolisation therapy and systemic chemotherapy. Though transient effect was obtained. He died forty-five days after operation. Autopsy revealed that recurrent liver tumors consisted of only hepatocellular carcinoma and that no other tumorous lesion was found in the other parts of the body. Rhabdomyosarcoma was proved to be primary tumor the liver.

Carcinoma, Hepatocellular↗
PubMed1987

[Rhabdomyosarcoma of the kidney: report of a case].

A case of rhabdomyosarcoma of the right kidney is presented. A 78-year-old man was admitted with the complaint of abdominal pain and abdominal fullness on March 15, 1985. Radiological examination showed a giant tumor of the right kidney. Radical nephrectomy and right hemicolectomy were performed. Histological findings were embryonal rhabdomyosarcoma of the kidney. Residual tumor in the duodenal area recurred and he died of peri-duodenal abscess 2 months after admission. This case is the 17th case of rhabdomyosarcoma of the kidney in Japan. The literature is reviewed and discussed.

Aged↗
PubMed1987

[Rhabdomyosarcoma of the prostate: report of a case and review of the literature].

Rhabdomyosarcoma of the prostate is a rare tumor and its prognosis is extremely poor. However, the survival rate has been gradually improved by using combined chemotherapy. A 17-year-old man with the chief complaint of dysuria was referred to our hospital on April 7, 1986. Prostatic needle biopsy was performed and pathological diagnosis was rhabdomyosarcoma of the prostate. The patient was given preoperative combined chemotherapy consisting of actinomycin D and cyclophosphamide. Total cystoprostaurethrectomy and ileal conduit were performed on April 30, 1986. He underwent postoperative combined chemotherapy (vincristine, actinomycin D, cyclophosphamide). He is well 13 months after his initial symptoms and is clinically free of tumor 12 months postoperatively. Forty-two cases of rhabdomyosarcoma of the prostate including our case were collected from the Japanese literature and reviewed with respect to the multimodal treatment and prognosis.

Adolescent↗
PubMed1988

Diagnosis of rhabdomyosarcomas with HHF35, a monoclonal antibody directed against muscle actins.

The authors have recently developed a monoclonal antibody, HHF35, that recognizes the muscle-specific isoforms of actin. To determine its potential usefulness in the differential diagnosis of "small, round, blue cell" tumors of childhood, they immunolabeled formalinor B-5-fixed tissue sections from known cases of rhabdomyosarcoma or rhabdomyoma (30), neuroblastoma (9), retinoblastoma (2), and Ewing's sarcoma (9) with HHF35 and with antibodies to creatine kinase M, myoglobin, vimentin, and neuron-specific enolase. HHF35 reacted with 29 of 30 cases of rhabdomyosarcoma, whereas antibodies to creatine kinase M and myoglobin were positive on only 12 and 7 tumors, respectively. HHF35 did not react with any case of neuroblastoma, retinoblastoma, or Ewing's sarcoma when the antibody diluent contained 50 mM EDTA. These results indicate that HHF35 is a highly sensitive and specific marker for myogenic differentiation and that it will be useful in the differential diagnosis of rhabdomyosarcomas.

Actins↗