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Rhabdomyosarcomas are malignant tumors, and especially occur in childhood. In adults they are extremely rare. A pleomorphe rhabdomyosarcoma of the skin, localized on the nose of an 85-year old female patient, is reported. After surgical removal the defect was reconstructed with a full skin graft. The four histologic types of rhabdomyosarcoma (pleomorphic, alveolair, embryonal, botryoid) are discussed.
Fast myosin and slow myosin are specific markers of skeletal muscle, in addition to myoglobin. This study of 15 specimens of rhabdomyosarcomas from 13 patients using specific antisera for the three markers as well as for desmin led to positive findings in all cases with at least one antiserum. Desmin was present in all cases; fast myosin and myoglobin were present in 10 cases each. Slow myosin was present in six cases. It appears that the combination of several markers is helpful in differentiating rhabdomyosarcomas from other tumors. The markers considered were generally more abundant in neoplastic elements with large amounts of cytoplasm. This finding suggests that the larger cells of rhabdomyosarcomas are more differentiated than smaller rhabdomyoblasts, which were often negative with some of the antisera used.
Vincristine (VCR) is an effective agent in the treatment of childhood rhabdomyosarcoma. Clinically, schedules differ in frequency of administration. To determine the influence of administration frequency, accumulation of VCR in xenografts of human rhabdomyosarcoma has been evaluated following administration of drug at 7- or 21-day intervals. Accumulation was estimated from initial uptake, retention of unchanged drug in tumor tissues, tumor sensitivity, and growth rate. Data suggested that scheduling VCR every 7 days would be more effective than every 21 days, due to more rapid accumulation to cytotoxic levels. The effect of scheduling was examined in two rhabdomyosarcoma xenografts, where in vivo responses were similar to those predicted based upon drug uptake, retention, and growth characteristics for the tumors. Scheduling VCR at 7-day intervals was clearly superior to administration at 21-day intervals in these models.
Rhabdomyosarcoma of the prostate in children is rare. This is a case report of a six-year-old boy with rhabdomyosarcoma of the prostate who was admitted to our hospital, complaining of pain on urination and dysuria. Further examination revealed metastasis to the lungs and lymph nodes. He was treated with vincristine, actinomycin-D, cyclophosphamide, but this therapy was not effective. He died of respiratory failure due to the diffuse pulmonary and pleural metastasis of tumor, 41 days after admission. This is only the 9th reported case in Japan of rhabdomyosarcoma of the prostate in a child.
One case of alveolar rhabdomyosarcoma appearing like an undifferentiated tumor by light microscopy is reported. Specific ultramicroscopic features of sarcomere containing Z line structures, thick and thin myofilaments with hexagonal arrangements in the cross section were noted in the cytoplasm of some individual malignant cells, thus establishing the diagnosis of rhabdomyosarcoma. Some of the tumor cells were positive to peroxidase-anti-peroxidase (PAP) immunohistochemical stain of anti-serum by anti-myosin. In the present paper, the ultrastructural features and differential diagnosis of poorly differentiated rhabdomyosarcoma are also discussed.
The accurate diagnosis of malignant tumor type is essential to enable the correct therapeutic regimen to be followed and to predict a patient's prognosis. However, the differential diagnosis of "small-round-cell" tumors, represented by neuroblastoma, rhabdomyosarcoma, lymphoma/leukemia and Ewing's sarcoma, can occasionally be difficult by conventional morphological and biochemical methods. If tumor membrane markers were available, these could provide rapid and accurate diagnostic aids. In the present work, a panel of 9 monoclonal antibodies raised against hematopoietic cells (BA-1, BA-2, J-5 and B7/21), brain cells (UJ-13A, UJ-127-11 and anti-Thy-1), and neuroblastoma cells (HSAN1.2 and PI153/3) was used to analyze the membrane phenotypes of 12 neuroblastoma, 4 rhabdomyosarcoma and 3 Ewing's sarcoma cell lines and cells of 3 fresh bone marrow tumors. BA-1, UJ-127-11 and PI153/3 antibodies may be useful for the differential diagnosis of neuroblastoma from rhabdomyosarcoma and Ewing's sarcoma.
A study has been made of 7 transplatable lines of mice rhabdomyosarcomas and one line of rat rhabdomyosarcoma during their transplantation into the eye anterior chamber subcutaneous tissue. In all, 10 subcutaneous transplants and 15 transplants into the eye anterior chamber (EAC) were examined. Etanol fixed print smears were subjected to the Feulgen reaction to measure the DNA content using a cytophotometer MCPhU-1; 100 cells being measured in each transplant. In the majority of the EAC transplants, a statistically significant decrease of the karyotypic variability was found in additionto the augmentation to the diploid cell ratio as compared to subcutaneously proliferating populations of the same tumour lines. In some cases EAC transplants displayed exclusively diploid (periploid) populations of tumour myoblasts. Shifts in the karyotypic structure of populations towards diploidy, revealed during the cultivation of transplantable rhabdomyosarcomas, may be regarded as a phenomenon of the "karyotypical normalization" of tumour cells. The disappearance or sharp decrease of tetraploid or hypertetraploid classes of cells in EAC transplants may be due to the increase of their selective value in condition of immunological privilege of diploid, karyotypically normal cells, and of reduction of the genome mutation frequency in a diploid fraction of tumor myoblast populations.
Four clone lines of transplantable cell polymorphic rhabdomyosarcoma A-7 were investigated during transplantation to the subcutaneous connective tissue (SCT) and into eye anterior chamber (EAC). Cell morphology of transplants was studied by light and electron microscopy, the activity of their LDH M- and H-subunits was examined cytochemically, and the quantity of their nuclear DNA--cytophotometrically. In the case of A-7/1, A-7/2 and A-7/3 cell lines of EAC transplants we noticed a decrease in cell element kataplasia levels, differences in LDH M- and H-form ratio, reduction in the karyotype variability. Transplants of A-7/4 clone line were similar in SCT and EAC for all the signs studied. The results obtained show that the transplantable cell polymorphic rhabdomyosarcoma A-7 is heterogeneous for its differentiation and normalizing capacities during EAC proliferation. The data reported elsewhere concerning capability of four lines of murine rhabdomyosarcomas to normalize in EAC are discussed, and some possible mechanisms of this effect are regarded.
A case of rhabdomyosarcoma of the urinary bladder in a child with urethral prolapse is reported. The patient was an 18-month-old girl and was admitted to our clinic with complaints of micturition pain, hematuria and tumor of the vulva. Histological examination revealed that this tumor was urethral prolapse. A grape-like tumor was demonstrated at bladder bottom by cystogram and cystoscopy. The histological pattern was embryonal type of rhabdomyosarcoma of the bladder. Combined chemotherapy with actinomycin D, adriamycin and cyclophosphamide has been started. A survey of Japanese literature on rhabdomyosarcoma of the bladder in children was done with reference to age, sex, site of tumor, symptom, histological findings, treatment and prognosis.
In spite of an excellent improvement of treatment strategies many questions about the best therapeutic approach in rhabdomyosarcoma remain open. The rate incidence (4,5 per million children and year), the biological heterogeneity, the different localisations and stages of disease still remain a challenge for the interdisciplinary cooperation in paediatric oncology. The optimum treatment consists of a qualified combination of chemo- and radiotherapy as well as surgical intervention. The importance of each modality is to be defined in every individual case. Only with such an approach severe mutilations can be avoided and a better chance of survival offered. A national study for rhabdomyosarcoma of the Austrian Paediatric Oncology Group is presented, which has been established in close collaboration with the paediatric oncologists of West-Germany. The study protocol since January 1982 is still in use. Therapeutic strategies of the Intergroup Rhabdomyosarcoma Study Group were taken into consideration. The chemotherapy essentially consists of a modified T9- and T11-protocol (Memorial Sloan Kettering Cancer Center, New York) including newer drugs like cisplatin and VP16-213. The surgical intervention is recommended as primary resection only when mutilations can be avoided (otherwise a biopsy is proposed). The secondary and definitive resection or biopsy is provided for the time period after completion of 16 weeks of chemotherapy. The addition of radiotherapy after the second look operation promises the maximum of curative effect. The following chemotherapy is planned for further 16 or 36 weeks according to the stage of disease.(ABSTRACT TRUNCATED AT 250 WORDS)
A 16-year-old boy was found to have a cavernous sinus syndrome secondary to rhabdomyosarcoma originating in the masseter muscle. Radiologic studies showed evidence of dissemination to the cavernous sinus without involvement of the skull base or its foramina. Despite aggressive therapy with transient improvement of his ocular palsy, the patient died within 16 months after the onset of his illness. Neuro-ophthalmic complications often develop as a result of direct extension from rhabdomyosarcoma originating in the head and neck region. However, to the best of our knowledge, this is the first report of a cavernous sinus syndrome caused by metastasis from rhabdomyosarcoma.
Polymorphic CC57W mice rhabdomyosarcoma MC-53, after selection for malignancy, was investigated during the transplantation to subcutaneous connective tissue (SCT) and the eye anterior chamber (EAC). SCT and EAC transplants appeared to be equal both morphologically and ultrastructurally. Relationship between M- and H-forms of LDH, and the karyotype structure of tumor cell populations were invariably similar in SCT and EAC. However, after EAC proliferation, in contrast to SCT proliferation, transplantability of tumor rhabdomyoblasts decreased, which may be associated with a decrease in proliferative activity of tumor cells in the EAC. Our data allow to suppose that mouse rhabdomyosarcoma MC-53 cells, during selection for malignancy, lost its capacity of differentiating and normalizing in EAC, unlike the previously investigated rhabdomyosarcomas MC-62, MC-III and A-7. These results may be explained by the fast progression of this tumor line. It is obvious that capacity of tumor cells of differentiating and normalizing during the EAC proliferation may depend on the tumor histological type, on the retention capacity of tumor cell elements of differentiating and on the stage of progression.
Marked dissimilarities in the epidemiology of osteosarcoma, Ewing's tumor, and rhabdomyosarcoma indicate differences in their origins. A major clue to the genesis of Ewing's tumor comes not from defining persons at high risk but from the observation that blacks are at unusually low risk. The neoplasm does not aggregate in families and is not part of any known syndrome. No environmental causes have been identified. By contrast, osteosarcoma may be caused by external or internal ionizing radiation, and it aggregated in families with the same tumor or with dissimilar tumors and in certain genetic disorders of bone. In man and in dogs, the frequency of the neoplasm is related to bone mass and growth. Rhabdomyosarcoma of the upper versus the lower limbs seems related to muscle mass. Age peaks in the occurrence of the tumor elsewhere vary with the anatomic site; head and neck tumors develop in early childhood and urogenital tumors both in early years and in adolescence. The sex ratio (male to female) also varies with the site affected. Rhabdomyosarcoma aggregates with certain other tumors in families and overlaps with osteosarcoma in some of these relationships but is distinguished from that tumor by its excessive occurrence in neurofibromatosis.
At the age of three months an infant rapidly developed signs of cardiac failure as well as in- and exspiratory stridor, caused by an intrathoracic tumor. Thoracotomy and biopsy revealed an intrapericardial tumor, histologically myxosarcoma. In spite of chemotherapy and radiation the infant died at the age of seven months due to multiple intracerebral metastases now histologically rhabdomyosarcoma. This is one of the rare cases of primarily malignant intrapericardial tumors in infancy, and also shows the possible pleomorphism of childhood rhabdomyosarcoma. We know only one further case of pericardial rhabdomyosarcoma where similar histologic changes have been observed.
The morphology of 50 rhabdomyosarcomas was compared with that of developing skeletal muscles from 20 fetuses and neonates. Vimentin, desmin, HHF-35 and myoglobin were used in labeling these specimens. We found that the consistent sequence, and intensity of tumor (express) markers related to the degree of morphological differentiation and analogous to the normal sequence in fetal myogenesis. According to the degree of tumor differentiation, we believe that different types of rhabdomyosarcoma comprise cells of variable degree of differentiation and are derived from primitive mesenchymal cells differentiating towards skeletal muscle. Histological typing of rhabdomyosarcoma is proposed to retain the WHO typing protocol while the best also being able to express the degree of differentiation.
Rhabdomyosarcoma accounts for 5% of childhood malignant tumours; in 10% it occurs in the orbit where it is the most frequent malignant tumour. Rhabdomyosarcoma is a mesenchymatous tumour with striated muscular differentiation. However, this myogenous differentiation is sometimes undiscernable on standard histological examination and requires immunohistochemical and ultrastructural studies to be shown. Treatment depends on the assessment of local extension, principally based on tomodensitometry. When the tumour is limited to the orbit, survival rate may reach 100% with todays therapeutic protocols. When the orbital walls are invaded the tumour is parameningeal and recovery can only be achieved by increasing chemotherapy at the cost of long-term side effects. Hence, this tumour must be diagnosed and treated very early. The authors report two cases of rhabdomyosarcoma dealing with the two main histological forms of the tumour and with their evolutive risks.
A 56-year-old male suffered from primary rhabdomyosarcoma on the left internal thoracic wall, which was treated by chemotherapy, and local irradiation following biopsy. Four months after the diagnosis, he suddenly complained of headache and left paresthesia occurred followed by generalized convulsion and left hemiplegia. CT scan revealed a high density mass in the right parietal lobe. The patient was referred to our department and underwent emergency evacuation of the hematoma together with tumor removal. The pathological specimen showed spindle or oval-like cells with hypercellularity and some mitotic figures. Immunohistochemical study demonstrated that many cells were positive for desmin and myoglobin, which is specific to myogenic tumor. These characteristics were compatible with those of the primary thoracic lesion, and a diagnosis of metastatic rhabdomyosarcoma was made. Five weeks after the craniotomy, the metastatic brain tumor recurred in the same site and also in the bilateral occipital lobes. Although radiotherapy to the brain decreased the tumor size, the patient died of respiratory failure eleven and a half months after the initial diagnosis. Sarcomas metastasizing to the brain are rare and only 16 cases of rhabdomyosarcoma metastasizing to the brain have been reported so far. Recent advances in chemotherapy, however, have been able to show the increased incidence of sarcoma metastasis to the brain. Therefore, the necessity of follow-up CT scan for sarcoma patients of long survival is to be stressed, even if the patient shows no neurological symptoms.
Rhabdomyosarcoma, a common soft tissue sarcoma in children, is rarely reported. The head and neck are the most common sites of occurrence. A case of embryonal rhabdomyosarcoma of the nasopharynx is presented. The patient, a five-year-old boy, presented with a two-monthly history of epistaxis. Ear-Nose-Throat examination revealed a mass at the hard palate. Head and neck MRI showed a nasopharyngeal mass with invasion to the skull base, and intracranial extention. Histological examination of the mass revealed embryonal rhabdomyosarcoma. A combination chemotherapy and intrathecal medication was administered. This was followed by radiotherapy to the tumor site with 2 cm free margins. A good tumor response was found.