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We report a case of rhabdomyosarcoma of the bladder in an adult. A 73-year-old male was referred to our hospital due to macrohematuria. Ultrasonography, CT and MRI revealed a bladder tumor, and cystoscopic biopsy of the tumor pathologically revealed rhabdomyosarcoma. Total cystectomy with ileal conduit urinary diversion was performed.
BACKGROUND: Radiotherapy of head and neck tumors often leads to acute reactions of the adjacent normal tissues resulting e.g. in mucositis and xerostomia. Therefore, radioprotective drugs have been developed to reduce these effects. Studies on a tumor model using amifostine and sodium selenite adjuvant to fractionated irradiation should show whether the radioprotective effect on normal tissue leads to an increase of radioresistance in the tumor and its metastatic potential. METHODS: Rhabdomyosarcomas R1H of the rat growing subcutaneously in the right flank of male adult WAG/RijH rats were irradiated with 60Co-gamma rays (60 Gy/30 fractions/6 weeks). Amifostine (375 mg/m(2)), sodium selenite (15 microg/kg), and equivalent volumes of 0.9% saline were administered intraperitoneally 30 min before each irradiation. Tumor response was determined. Statistical analysis was performed using the post-hoc-test. RESULTS: Irradiation alone inhibited R1H tumor growth (AUC 86.8+/-18.3). The efficacy of irradiation during radiotherapy was significantly improved by amifostine (AUC 63.1+/-15.8) in comparison to the irradiated controls. The radiosensitizing effect of sodium selenite (AUC 73.6+/-21.3) as well as irradiation and amifostine plus sodium selenite (AUC 68.3+/-7.8) was less compared to the irradiated controls and not statistically significant. However, tumor growth delay and metastasis rate were not changed by the radioprotective drugs. Further, the administration of amifostine and amifostine plus sodium selenite induced an enhanced decrease of animal body weight except for sodium selenite in comparison to the controls. CONCLUSIONS: The application of amifostine and sodium selenite to conventionally fractionated irradiation have no influence on the radiosensitivity of the rhabdomyosarcoma R1H. The systemic toxicity of amifostine might be of importance for the radiation treatment of a patient.
Malignant tumors arising within dysrhaphic malformations are very rare and are mostly teratomas; so far, only one rhabdomyosarcoma has been reported in this context. We report another case of a girl with lipomyelomeningocele who developed a lumbar rhabdomyosarcoma 2 years after birth and primary closure of the neural tube defect. We present clinical, radiological and pathological findings, discuss possible mechanisms of malignant transformation and review the literature.
A specific chromosomal abnormality, t(2;13)(q35;q14), was discovered in five cases of advanced rhabdomyosarcoma. It was identified directly in cells that had metastasized from bone marrow in one patient and in xenografts derived from the tumors of four other patients. The translocation was not restricted by histologic subtype, but was found in cases classified as alveolar, undifferentiated, or embryonal. Cytogenetic hallmarks of gene amplification (double minute chromosomes and homogeneously staining regions) were apparent in three cases. Other frequent abnormalities included rearrangements of chromosomes lp and trisomy of chromosome 8. The absence of the t(2;13) in more than 100 cases of other pediatric solid tumors investigated in our laboratory indicates its specificity for rhabdomyosarcoma. These cytogenetic findings suggest directions for further investigation of the molecular events underlying the genesis of this tumor.
This report describes a case of rhabdomyosarcoma associated with a 2;13 translocation and multiple double minute chromosomes. The origin of the amplified DNA was identified using comparative genomic hybridization, which pinpointed a unique spot at 12q13-->q14. Band 12q13 has been shown to contain several genes that are occasionally amplified in other sarcomas. Fluorescene in situ hybridization to tumor metaphases with probes specific for this region indicated that the double minutes contained the MDM2 gene but not the CDK4 gene. MDM2 amplification was further quantified by Southern hybridization, which showed a mean value of 25 copies per haploid genome. This is the first example of MDM2 amplification in a rhabdomyosarcoma.
Protease inhibitory activity of eight cytostatic drugs on 5 proteases was tested by applying an immunoelectrophoretic system, a rhabdomyosarcoma's collagenolytic activity was investigated and inhibition studies were performed. We found 5-fluorouracil stopping collagenolytic activity of clostridial origin and the enzyme released by the tumor. No other cytostatic drug showed protease inhibition. The collagenolysis of the rhabdomyosarcoma was examined for its origin. Negative inhibition studies with ethylene-diamine-tetraacetate make the leukocyte origin highly improbable so that one can suggest that the hydrolytic enzyme derived from the tumor. Our findings could contribute to characterize the enzyme released by the tumor. We suggest its role as a mediator of invasion and metastasis and on the other hand we detected its relatively specific inhibitor, 5-fluorouracil, which could influence tumorous and normal growth, regeneration and the intermediary protein metabolism.
A vaginal rhabdomyosarcoma which occurred in an 11-month-old infant was treated with conservative surgical excision, radiation therapy and chemotherapy. The long-term sequelae of this therapy included rectal stenosis, which required surgical correction, and urinary incontinence. The patient may have had fewer long-term complications if a more aggressive surgical procedure had been performed at the time of diagnosis because a gross and microscopically complete excision would have obviated the need of postoperative pelvic irradiation. Strong consideration of total surgical excision should be given to infants with pelvic rhabdomyosarcoma.
Rhabdomyosarcoma is an uncommon tumour that may present at a wide variety of different sites. We report a 4-year-old girl who developed an embryonal rhabdomyosarcoma arising in the left pinna which was clinically indistinguishable from a lymphangioma. The case illustrates that this neoplasm can be easily misdiagnosed because of its variable morphology. Early recognition is important as successful treatment is now possible with a combination of chemotherapy, surgery and/or radiotherapy.
We report on 4 children suffering from orbital rhabdomyosarcoma. The ultrasonographical findings in both A and B mode are: irregular structure, poorly outlined borders, very low reflectivity, weak sound attenuation, hard consistency and little mobility of the tumor. There was no correlation between the ultrasonographical findings and the histological type of rhabdomyosarcoma.
BACKGROUND: Metastasis to the breast from extra-mammary malignancies is rare, but its recognition is important. A solitary metastasis must be distinguished from the primary breast cancer because the treatment and prognosis are quite different. CASE: A 30-year-old female presented with a 4.0-cm, solitary, nontender mass in the upper outer quadrant of the right breast 11 months after primary surgery for maxillary sinus embryonal rhabdomyosarcoma. The cytomorphology revealed features of small round cell tumor. Immunocytochemical staining disclosed a positive reaction to vimentin and desmin and negative reaction to cytokeratin, confirming the diagnosis of rhabdomyosarcoma. CONCLUSION: Fine needle aspiration with ancillary studies is essential in the diagnosis of metastatic malignancy of the breast in order to avoid unnecessary mastectomy and to implement appropriate systemic therapy.
18 children, 12 boys and 6 girls, were treated in our hospital for rhabdomyosarcoma of the urogenital tract between 1970 and 1980. This period saw a gradual change in the therapeutic approach to this disease as new therapeutic modalities became available. 7 of the patients (39%) died of the disease which, in 5 of them, arose from the prostate. The surviving children have no evidence of recurrent disease, although 1 is still undergoing chemotherapy. Surgical intervention was always required. To date, chemotherapy alone, or in combination with radiation, has not sufficed in our management of rhabdomyosarcoma of the urogenital tract.
Skeletal myogenesis is regulated by a group of transcription factors (MyoD, myogenin, myf5, and myf6) that are "basic helix-loop-helix" proteins that bind to the promoters of muscle-specific genes and promote their expression. We have previously shown that after a mutation of Leu122 to Arg the DNA binding basic domain of MyoD confers c-myc-like functional characteristics to the protein. In this study we used single-strand conformation polymorphism analysis to determine whether such mutations occur naturally in rhabdomyosarcomas. We have found that the basic domains of all the myogenic factors remain unaltered in rhabdomyosarcomas. Selection against such mutations may be the result of functional redundancy of these myogenic transcription factors.
The temporal bone is the primary site for approximately 10% of rhabdomyosarcomas of the head and neck in children. Until recently it has been a uniformly fatal tumor despite treatment with radical surgery and radiation therapy. Although experience with the Intergroup Rhabdomyosarcoma Study protocol-II is of relatively short duration, treatment of these children with radiation therapy and multiple-drug intravenous and intrathecal chemotherapy holds promise of significantly improved control rates.
Five malignant mixed müllerian tumors and one rhabdomyosarcoma were observed in LEWIS rats. Heterologous müllerian neoplasms (cases 1-4) consisted of epithelial and mesenchymal elements including squamous epithelium, glandular epithelium and sarcomatous, rhabdomyosarcomatous, cartilaginous as well as osseous parts. All the heterologous tumors revealed undifferentiated areas with an anaplastic appearance. The homologous müllerian tumor (case 5) was composed of glandular epithelium embedded in sarcomatous tissue. The rhabdomyosarcoma (case 6) consisted of bundles of elongated strap-like cells mixed with cells resembling rhabdomyoblasts.
A 27-year-old man was found to have a mediastinal tumour and the histological diagnosis was immature teratoma. Remission was achieved by chemotherapy and total resection. However, he developed anaemia and leukoerythroblastosis after 2 years of remission, and was referred to our hospital. Rhabdomyosarcoma cells were detected in the bone marrow and pleural effusion. Moreover, karyotype analysis of peripheral blood and bone marrow cells revealed mosaic-type Klinefelter syndrome. We diagnosed the case as transformation of teratoma into rhabdomyosarcoma in Klinefelter syndrome. Although intensive chemotherapy was performed, the patient died with meningeal infiltration.
The paper reports 5 cases of paratesticular rhabdomyosarcoma in patients of 10 to 18 years of age out of 438 cases of testicular tumours admitted to the Istituto Naxionale per lo Studio e la Cura dei Tumori in Milan (1.14%). Two cases were far advanced and died within a few months despite chemotherapy and radiotherapy. Only one of the three operated patients was cured and is still living 4 years after treatment. This patient had negative nodes and a full course of post-operative chemotherapy. The causes of failure in the other two cases are believed to be: not performing retroperitoneal lymphadenectomy in one and not giving post-operative chemotherapy in both. It is concluded that radical orchiectomy, retroperitoneal lymphadenectomy (followed by radiotherapy in N+ cases) and post-operative chemotherapy are the treatment of choice for operable paratesticular rhabdomyosarcoma.
The authors describe a case of undifferentiated rhabdomyosarcoma from unknown primary site, presenting as an acute hematologic malignancy with generalized lymphadenopathy, extensive bone marrow involvement and clinical and laboratory features of disseminated intravascular coagulation. Such a peculiar behaviour is known for rhabdomyosarcoma but is rare and can be a serious diagnostic problem for the clinician and the pathologist. The importance of a large spectrum immunohistochemistry as first diagnostic approach to any undifferentiated small-cell malignant tumor is stressed, together with the knowledge of the different immunoreactivity patterns. Desmin, MS-actin and myoglobin are the most reliable markers of this type of myogenic sarcoma.
At necropsy, a 7-year-old Holstein cow, clinically diagnosed via rectal palpation as having malignant abdominal neoplasia was found to have many metastatic tumors of various sizes in the abdominal and thoracic cavities and in the liver, lung, kidney, adrenal, uterus, and lymph nodes. These tumors were examined by histologic and immunohistochemical methods using anti-human myoglobin, anti-bovine myoglobin, and anti-desmin sera. Tumors were diagnosed as undifferentiated alveolar rhabdomyosarcomas. The tumors in the abdominal and thoracic cavities differed histologically from the metastases in organs. The former tumors consisted of only undifferentiated cells, most of which did not stain positively for desmin or myoglobin. The metastatic tumors contained a few rhabdomyoblastic cells that stained positively for desmin and myoglobin. Tumors tissues from the cow were transplanted and propagated through six passages in athymic nude mice. After one passage, the transplanted tumor histologically resembled those from the abdominal serosa and consisted of cells with scanty cytoplasm with an alveolar arrangement. Cells from this tumor stained positively for desmin and negatively for myoglobin. After two passages through nude mice, tumor cells were of two distinct histologic types: those cells with scanty cytoplasm and elongated cells with plentiful eosinophilic cytoplasm. Both of these cells stained positively for both desmin and myoglobin. After six passages, cross striations were detected in neoplastic cells by electron microscopy. These findings illustrate that a highly undifferentiated bovine rhabdomyosarcoma, in which most of the cells in the original tumor lacked desmin and myoglobin, became better differentiated and stained positively for desmin and myoglobin after serial transplantation in nude mice.