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PubMed1985

Rhabdomyosarcoma in Nigerian children.

A review of eight cases of rhabdomyosarcoma in childhood seen at the Ahmadu Bello University Hospitals in Kaduna and Zaria (Nigeria) over five years is presented. The cases were equally divided between the head region and the urogenital system. Embryonal rhabdomyosarcoma and sarcoma botryoides were the only histologic types seen. Although the clinical presentations were not different from those reported in other series, the correct diagnosis was not suspected clinically in most of the patients, so the institution of rational management was delayed. Certain aspects of the disease and its therapy in this part of the world are discussed.

Antineoplastic Agents↗
PubMed1985

[An operative case of spermatic cord rhabdomyosarcoma--with a review of similar cases in Japan].

A 17-year-old Japanese boy was admitted with the complaint of painless, progressive enlargement of the left scrotal contents during the preceding two months. The tumor was firm and nontender without transillumination. Orchitectomy with high ligatation of the spermatic cord was performed. The tumor, weighing 13.1 g, was gray, solid, located at the distal part of the spermatic cord, and separated from the testis and epididymis. The pathological diagnosis showed left spermatic cord rhabdomyosarcoma of the combined embryonal and alveolar type. All of the retroperitoneal lymph nodes dissected were negative for metastasis. The patient was treated with actinomycin D and vincristin postoperatively, and is well three years after the operation. Ninety-two cases of intrascrotal rhabdomyosarcoma reported in Japan are reviewed.

Adolescent↗
PubMedDate not supplied

[Rhabdomyosarcoma of the spermatic cord: apropos of a case].

The rhabdomyosarcoma of the spermatic cord is a rare tumour of the genital apparatus, of which 80 cases have been published in world literature. In the present paper, we publish a case of pleomorphic rhabdomyosarcoma of the spermatic cord, pointing out the highly malignant nature of this lesion (a 10% survival rate after 5 years) as well as the delay in its diagnosis on some occasions due to confusion with hydroceles, hernias and cord cysts. As far as the treatment is concerned, all the authors agree on the extensive exeresis of the tumour, with a need for a para-aortic lymphadenectomy when there are greater divergences. In spite of these tumours being only slightly radio-sensitive, its usefulness when used locally to prevent relapses has been proved. The experience with chemiotherapy is very limited.

Aged↗
PubMedDate not supplied

Epibulbar rhabdomyosarcoma without proptosis.

Orbital rhabdomyosarcoma presenting as a localized, epibulbar mass is unusual, but common enough for the clinician to be aware of it. Although there has been a recent trend toward simple biopsy followed by irradiation and chemotherapy, cases presenting in this manner may sometimes be managed by total excision with preservation of the globe, follwed by irradiation and chemotherapy. A case of such an epibulbar rhabdomyosarcoma is presented as an illustrative example.

Child↗
PubMed1984

[Rhabdomyosarcoma of the bladder in a child: report of a case].

A case of rhabdomyosarcoma of the urinary bladder in an 11-month-old boy is presented. The chief complaint was complete urinary retention and histological examination showed embryonal rhabdomyosarcoma of the bladder. Initially, he was treated with vincristine, actinomycin-D and radiation therapy. This therapy was not effective, and he was next treated with vincristine, bleomycin, and cis-DDP. This therapy was significantly effective, and the tumor became non-palpable on physical examination. After six courses he was discharged and maintenance chemotherapy was continued until the tumor relapsed 1 year later. Finally he died of dyspnea due to diffuse pulmonary metastases approximately 2 years after the first diagnosis. We herein discuss several points which affect the prognosis and the usefulness of chemotherapy, especially combination therapy with vincristine, bleomycin, and cis-DDP for recurrent cases.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1984

Rhabdomyosarcoma causing rapid bilateral visual loss in children.

Rhabdomyosarcoma commonly involves the orbit resulting in unilateral proptosis, swelling, and restricted ocular motility. We saw two unusual cases of children with nasopharyngeal rhabdomyosarcoma which caused rapid bilateral visual loss due to simultaneous spread of the tumor to the orbital apices. Initial CT scans revealed the tumor, but were misinterpreted as "normal," causing a significant delay in diagnosis. We recommend high resolution CT scans of the nasopharynx, orbits, and suprasellar region in any child with rapid bilateral visual loss to rule out this common childhood tumor.

Blindness↗
PubMed1984

Cisplatin and doxorubicin for locally recurrent and metastatic childhood rhabdomyosarcoma.

Five pediatric subjects, each of whom had been treated with multiple agents including doxorubicin, received cisplatin and doxorubicin following the development of locally recurrent or metastatic rhabdomyosarcoma. Although there was no definite evidence that the tumor of these individuals was resistant to doxorubicin, tumor responses developed in 3 of 5 patients. Additional trials of this combination in similar patients with primary or recurrent metastatic rhabdomyosarcoma are indicated.

Adolescent↗
PubMed1983

[Rhabdomyosarcoma of the heart involving the left ventricle and left atrium].

A case of rhabdomyosarcoma of the left atrium and left ventricle demonstrated by echocardiography was reported. A 31-year-old man was admitted to our hospital for evaluation of recently developed exertional dyspnea. A holosystolic murmur and a protodiastolic sound were audible at the apex. A chest X-ray showed pulmonary congestion without cardiomegaly. The two-dimensional echocardiogram showed a dense stratified mass of echoes occupying the medial half of the left ventricular cavity, and a part of the abnormal mass of echoes was observed to move toward the left ventricular outflow tract during systole. Another small mass attached to the anterior mitral leaflet was also observed to prolapse partly into the left atrium during systole. The interatrial septum showed a thick and hard band of echo in the short-axis view. Right cardiac catheterization revealed pulmonary hypertension and the levogram of the pulmonary angiography showed left atrial and left ventricular filing defects. The repeated echocardiographic study showed the growth of the abnormal mass. The patient underwent operation, but he died of congestive heart failure thereafter. The necropsy diagnosis was rhabdomyosarcoma of the heart, involving the left atrium and left ventricle.

Adult↗
PubMed1983

Rhabdomyosarcoma and late malignant melanoma of the orbit.

Forty-five years following surgical excision and radiation for a childhood rhabdomyosarcoma of the left orbit, a patient with primary lymphedema developed an ipsilateral malignant melanoma of the anterior orbital tissue. This was excised, but a metastasis of the melanoma occurred in the contralateral upper lid. This is the first case report of treated rhabdomyosarcoma of the orbit followed by a second primary tumor occurring in the field of radiation.

Eyelid Neoplasms↗
PubMed1984

Determinants of intrinsic sensitivity to Vinca alkaloids in xenografts of pediatric rhabdomyosarcomas.

The determinants of intrinsic sensitivity to Vinca alkaloids in vivo were examined in 3 pediatric rhabdomyosarcoma xenografts maintained s.c. in immune-deprived mice. The three lines differed in their sensitivity to VCR and VLB: two lines (Rh12 and Rh28) were extremely sensitive to VCR, whereas Rh18 tumors were less sensitive. Rh28 tumors were also very responsive to VLB, which demonstrated only marginal activity in the other two lines. After administration of equimolar doses (3 mg/kg) of [3H]-VCR and [3H]VLB to tumor-bearing mice, [3H]VCR reached concentrations approaching 1.5 microM in cell water of each tumor line within 4 hr, at which time greater than 93% of the drug was cell-associated. The drug was subsequently retained at this level for at least 72 hr studied. [3H]VLB accumulated to lower maximal concentrations (approximately equal to 1 microM) within 8 hr, but was not retained and, by 72 hr, reached concentrations that were 3- to 4-fold lower than those of [3H]VCR. The extent of drug retention correlated with the antitumor activity except in Rh28 tumors, which were sensitive to VLB, but did not retain the drug. The threshold level for achieving cytotoxicity may, thus, be very low in this line. In normal tissues, maximal concentrations of both [3H]VCR and [3H]VLB were achieved within 1 hr of administration i.p. to tumor-bearing mice. In ileum, liver, and kidney, these were approximately 10-fold higher than the peak levels achieved within tumors or plasma, but declined rapidly to parallel the decrease in plasma reaching concentrations greater than 5-fold lower than the concentration of [3H]VCR in tumors at 72 hr after treatment. Drug concentrations in skeletal muscle also declined rapidly, whereas neither [3H] VCR nor [3H]VLB accumulated to any great extent in brain. The blood volumes of ileum, kidney, and liver were greater than for tumor tissues. Hence, the extent of drug delivery did not necessarily influence therapeutic selectivity. In the case of [3H]VLB, concentrations in tumors approached those of normal tissues at 72 hr after injection. At 24 hr after treatment, 86 to 99% of [3H] VCR and 78 to 90% of [3H]VLB were present in tumors as the parent compound, which also predominated in normal tissues. Metabolites or in vivo degradation products were also identified. Selective retention in tumors appears to be the mechanism by which therapeutic selectivity is achieved with VCR in rhabdomyosarcoma xenografts.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1984

Embryonal rhabdomyosarcoma of the hand. A case of mediastinal relapse 103 months after treatment.

A 9-year-old boy was seen to have embryonal rhabdomyosarcoma of his right hand. He was treated with surgery, radiotherapy and adjuvant combination chemotherapy and remained free of disease for 103 months. He then developed signs of upper respiratory tract obstruction and a mass in the superior mediastinum was found. Histopathological and immunohistochemical studies confirmed metastatic embryonal rhabdomyosarcoma. This very unusual, long, relapse-free interval is discussed.

Child↗
PubMed1982

Alveolar rhabdomyosarcoma. Demonstration of the muscle type of intermediate filament protein, desmin, as a diagnostic aid.

Three cases of soft-tissue sarcomas with the characteristic histologic features of alveolar rhabdomyosarcoma, but lacking cytoplasmic cross-striations, were studied ultrastructurally and immunohistochemically to confirm the diagnosis and evaluate the histogenesis. The results showed that it was not possible to judge the skeletal muscle derivation of the cells at the ultrastructural level. However, immunohistochemically, the results of every case were positive for desmin-the muscle type of the intermediate filament protein. The results suggest that demonstration of desmin may be a helpful adjunct tool in the diagnosis of poorly differentiated alveolar rhabdomyosarcomas.

Adolescent↗
PubMed1980

Rhabdomyosarcoma of the brain.

The authors present a case of primary intracranial rhabdomyosarcoma. This is only the 10th reported case and is the only one in which the patient has survived longer than 2 years. A 9-month-old boy was found to have a large mass in the right posterior fossa. Posterior fossa craniotomy revealed an unencapsulated tumour involving almost the entire right cerebellar hemisphere and extending to the right cerebellar pontine angle. Subtotal removal was done for internal decompression. On examination of the specimen by light microscopy there were definite sarcomatous features with occasional rhabdopoietic elements and many malignant giant cells. The ultrastructural appearance confirmed the diagnosis of malignant rhabdomyosarcoma. The child was treated with combination chemotherapy and cobalt-60 teletherapy. He is alive and well 2 years after operation and has no clinical evidence of recurrent disease. His physical growth and mental development are satisfactory. The response of our patient suggests that such tumours may be controlled by subtotal removal followed by radiotherapy and chemotherapy.

Adolescent↗
PubMed1981

Surgical lessons from the Intergroup Rhabdomyosarcoma Study.

The Intergroup Rhabdomyosarcoma Study, initiated in 1972, has admitted more than 700 patients with childhood rhabdomyosarcoma in a 6-year period. Although it was designed primarily to answer specific questions about various aspects of radiotherapy and chemotherapy, some lessons have been learned concerning the surgical approach to this disease. Operative resection, which is not always feasible, is determined chiefly by the clinical stage and anatomic site. Thus far, data for some anatomic sites indicated that total gross resection at some point in the treatment schedule achieved better results than did incomplete resection. A possible exception to this occurred when the orbit is the primary site. The incidence of lymphatic metastases from lesions in some anatomic sites (e.g., paratesticular, pelvic genito-urinary, and extremity) was higher than was previously appreciated. This finding will influence decisions concerning surgical biopsy or dissection of regional lymph nodes for many of these lesions. Differences in histology, particularly those relating to the alveolar type, indicated the need for a more aggressive approach to selected lesions, but we need more data to confirm this determination. Lastly, patients who initially received radiotherapy or chemotherapy to pelvic sites have had striking benefits, and this finding should encourage the development of schedules that specify operative resection of these lesions later in the program rather than as the initial treatment.

Arm↗
PubMed1982

[Treatment of orbital rhabdomyosarcoma (author's transl)].

From March 1973 to December 1981 embryonal rhabdomyosarcoma of the orbit was diagnosed in 5 children. In two children exenteration was done after a previous local radiotherapy. In one child the exenteration of the orbit was done after a primary chemo- and radiotherapy because of local progression of the tumor. In two children the exenteration was avoidable by this therapy. In four of these five children this was followed by vincristine, actinomycin D and cyclophosphamide for 16 to 24 months. After the diagnosis these four children survived until now from 3 1/2 to more than 8 years. They are free of treatment and free of disease. Seven months after diagnosis one child died with an acute respiratory distress syndrome (at post mortem: hyaline membranes). In patients with rhabdomyosarcoma of the orbit it is justified to avoid the mutilating exenteration by primary chemotherapy followed by irradiation of the reduced manner. If exenteration becomes subsequently necessary the chance for survival is not diminished in our opinion.

Child↗
PubMed1982

Effect of microwave heating on the radiation response of the rhabdomyosarcoma.

The response of the rhabdomyosarcoma R1H of the rat was investigated after single doses of X-rays applied alone or in combination with hyperthermia. Tumours of 2.3 cm3 growing in the flank of the animals were locally irradiated with 15 and 30 Gy of 200-kV X-rays and/or heated by microwaves of 2450 MHz with a non-contact applicator. Temperature of the tumours as well as the body of the animals was measured using thermocouples. Heating the tumours at 43 degrees C for various time intervals caused a regrowth delay that increased linearly with treatment time. In combined treatments, hyperthermia was applied 20 minutes after irradiation. The rhabdomyosarcoma R1H showed an enhanced volume regression and tumour growth delay after combined treatment as compared to irradiation alone. After a single dose of 15 Gy, postirradiation treatments at 43 degrees C for 30 and 60 minutes lead to TER values of 1.3 and 1.8, respectively. After combining a dose of 30 Gy with hyperthermia at 43 degrees C (30 or 60 min), the TER values obtained ranged from 1.5 to 1.7.

Animals↗
PubMedDate not supplied

Medical therapy of orbit rhabdomyosarcoma in children.

Rhabdomyosarcoma is the most frequent malignant tumour of the orbit in the pediatric age. The chances of survival of patients affected by such a highly malignant neoplasia have significantly improved in recent years. The results obtained up until now with various therapeutic approaches are reviewed briefly and the results obtained in four children affected by rhabdomyosarcoma of the orbit are reported who were treated at the Ophthalmology and Pediatric Clinics of Siena University. The possibility is advanced that the less aggressive and a cautiously conservative treatment can produce the equivalent excellent results which are presently obtained by very destructive therapeutic approaches.

Antineoplastic Agents↗