Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From May, 1970 through December, 1972, Children's Cancer Study Group entered 112 patients on an amended treatment program for rhabdomyosarcoma and undifferentiated sarcoma in children. These patients had Group II disease with residual tumor remaining after surgery, or metastatic disease at onset. Another group consisted of patients who previously had treatment with surgery and radiotherapy and had recurrent disease. Cyclophosphamide was added to a previously used drug regimen which consisted of actinomycin D and vincristine. The drugs were given sequentially in repeated cycles for 18 months. Of 97 evaluable patients, there were 24 with microscopic residual disease, 37 with gross residual disease, 22 with metastatic disease at onset, and 14 patients who were treated with chemotherapy for the first time with recurrent or metastatic disease. All patients have been followed for 3 or more years. Survival in each group was 70.8%, 43.2%, 27.2%, and 28.2%, respectively. Although the number of complete remissions was greater than with two-drug therapy, survival with three-drug therapy was not significantly different than that seen in the earlier study.
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PMID:Treatment of rhabdomyosarcoma in children with surgery, radiotherapy and chemotherapy. 84 Jan 61

Prior to the use of chemotherapy, survival for rhabdomyosarcoma which had been completely resected was 50-60%. A controled study done by Children's Cancer Study Group showed the effectiveness of combined chemotherapy used as part of primary therapy in similar patients. Children who received actinomycin D (Act D) and vincristine (Vcr) for 1 year after surgery and radiotherapy had a lower metastatic rate than those who received none. This combination resulted in an 89% survival in patients with localized disease which was surgically resectable. Patients with microscopic residual disease had a 91% survival. With more aggressive use of combined chemotherapy, experience has accumulated demonstrating improved response and survival in both localized and gross residual disease. Combinations of Act D, Vcr, and cyclophosphamide can reduce initial tumor size, making large tumor masses more amenable to surgery and radiotherapy. Currently, an intergroup study is testing the response to four different drug combinations and the duration of therapy needed for various stages of the disease.
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PMID:The role of chemotherapy in the management of soft tissue sarcomas. 108 74

Thirty-six previously untreated patients younger than 21 years of age with sarcoma arising in the perineal region were entered on the Intergroup Rhabdomyosarcoma Studies (IRS) I and II from 1972 through 1984. The tumor histologic subtype was alveolar rhabdomyosarcoma (RMS) in 20 patients (56%), embryonal RMS in 11 patients (30%), and other types of sarcoma in 5 patients (14%). Fifteen children had grossly complete surgical excision (Clinical Groups I and II), and 15 had localized gross residual tumor (Group III) after initial operative management. Six patients had distant metastases (Group IV) at diagnosis. Twelve patients without distant metastases underwent regional lymph node biopsy; tumor involvement was found in six. Subsequent treatment consisted of chemotherapy with vincristine (VCR) and dactinomycin (AMD) for all patients; patients in Groups III and IV also received cyclophosphamide (CYC) with or without Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH). Radiation therapy (RT) was administered to patients in Clinical Groups II, III, and IV. Overall, 28 (78%) patients achieved a complete clinical response. The 3-year disease-free survival rate for these patients was 42%, compared with 52% for all other patients in the combined IRS I and IRS II series (P = 0.44). The overall 3-year survival rate was 59%, compared with 64% for all other patients in IRS I and IRS II (P = 0.48). Aggressive treatment is needed in children with perineal sarcoma to improve their prognosis.
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PMID:Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1972 through 1984. 218 89

Between 1962 and 1987, 112 consecutive patients were treated at St. Jude Children's Research Hospital for soft tissue sarcomas of the head and neck region; 18 of these children (16%) had histologic subtypes other than rhabdomyosarcoma. We evaluated the impact of surgery, postoperative chemotherapy, and irradiation on local control and survival in these cases. Three patients who had complete resection of tumors and received no further treatment are alive without disease at 36, 42, and 162 months. Local control was achieved in 1 of 2 patients with microscopic residual tumor and 4 of 9 patients with gross residual tumor who were treated with irradiation (2500-5040 cGy). Chemotherapy was the only postoperative treatment in three patients; only one achieved lasting local control. One patient was treated with irradiation only; his primary site showed no tumor cells at autopsy following an automobile accident. Overall, local control was achieved in 50% of patients; the disease-free survival rate at 3 years was 44%. The prognosis for patients with nonresectable tumors remains unsatisfactory because of the difficulty in securing local control. A revised therapeutic approach to these patients is presented.
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PMID:Local-regional non-rhabdomyosarcomatous soft tissue sarcomas of the head and neck. 221 Dec 68

Pancreatic tumors are rare in children. Over a 20-year period we have treated 13 children with pancreatic neoplasms. There were eight boys and five girls (age range, 4 months to 12 years). Seven tumors were benign, including five insulinomas, and two cystadenomas. Six lesions were malignant (rhabdomyosarcoma, 2; pancreatic carcinoma, 4). Children with insulinoma presented with hypoglycemia and irrational behavior. Three had abnormal insulin:glucose ratios ( greater than 1.0). The tumor was detected by computed tomography scan in three cases, at the time of surgery in one, and with intraoperative ultrasound in one. Surgical treatment included tumor enucleation in four cases and 80% pancreatectomy in one. Mucinous cystadenomas were observed in two patients, ages 4 months and 10 months. Tha latter infant underwent cyst excision alone, resulting in malignant recurrence at 18 months of age and death. The 4-month-old child had a distal pancreatectomy and is alive at 6 years. Two of the four children with pancreatic cancer had unresectable tumors at diagnosis, and were treated by biopsy (ductal adenocarcinoma), irradiation, and chemotherapy. Length of survival was 6 months and 9 months. Two others (ages 4 and 12 years) underwent 85% distal pancreatic resection for pancreatoblastoma and a pancreatoduodenectomy for papillary carcinoma, respectively. The latter is alive and tumor-free at 20 years of follow-up. The former underwent hepatic lobectomy for a 3.0 x 3.0 cm solitary liver metastases and is alive at 6 years with no evidence of disease. One child with rhabdomyosarcoma died of progressive disease, the other is alive with residual disease despite resection and chemotherapy. Most insulinomas can be treated by enucleation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pancreatic tumors in childhood: analysis of 13 cases. 226 58

A rare case of prostatic rhabdomyosarcoma in a 3-year-old child is presented. He received a multimodal treatment of chemotherapy including vincristine, cyclophosphamide, actinomycin-D plus adriamycin, and linac irradiation followed by total prostatectomy with segmental resection of bladder. Despite no viable sarcoma cells in the surgical specimen, he showed relapse 4 months later. By the combination of cisplatin, vinblastine and peplomycin (PVP therapy), the bulky mass in the pelvis rapidly reduced by 95%. Furthermore, cisplatin, etoposide and peplomycin (PEP therapy), instead of PVP therapy, were administered to the refractory disease. Although the residual tumor was salvaged after 7 courses of PEP therapy, abdominal dissemination and liver metastasis occurred 2 months postoperatively. The combined modality of vincristine, peplomycin, ifosfamide, methotrexate, adriamycin, melphalan plus nimustine, and irradiation of microtron were effective for the abdominal dissemination, but he died of cerebral hemorrhage 31 months after the start of treatment. We emphasize that more aggressive chemotherapy including cisplatin, etoposide and so on is required to manage the advanced, relapsed, or resistant cases compared to the usual modality of rhabdomyosarcoma.
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PMID:[New combination chemotherapy in refractory rhabdomyosarcoma of the prostate: a case report and review of the literature]. 245 11

After high inguinal semicastration in group-1 paratesticular rhabdomyosarcoma (RMS), the patient having undergone chemotherapy can be followed closely by CT scanning without retroperitoneal lymphadenectomy. In contrast, retroperitoneal RMS should be operated on as radically as possible after downstaging the tumor mass. In RMS of the female genitalia locally limited organ-preserving surgery is the method of choice. The prognosis is excellent with adjuvant chemotherapy. Only 20% of all bladder RMS arise from the bladder dome or the movable part of the bladder, where primary partial resection including a safety margin of 3 cm of healthy tissue is possible. The majority, however, arising from the submucous tissue of the bladder base, trigonal area and bladder neck, infiltrates the prostatic urethra and the surrounding pelvic fascia. Therefore differentiation between primary bladder or prostate RMS is often doubtful. The current strategy of downstaging by chemotherapy or chemo-radiotherapy followed either by limited organ-preserving surgery or by a wait-and-see policy includes the high risk of residual tumor or local tumor recurrence, which has proved to have the worst prognosis of all. As organ-preserving therapy was recommended as the method of choice, mainly to avoid anterior pelvic exenteration and urinary diversion, the question of advantages in comparison to the risk of tumor recurrence is still open. The routine use of chemoradiotherapy and increasing follow-up has called attention to a variety of chronic sequelae, such as contracted bladders with reduced capacity and upper urinary tract deterioration, which subsequently require secondary urinary diversion. Progress in continent urinary diversion may be an alternative procedure for better life quality.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Extent of surgery in rhabdomyosarcoma of urogenital structures. 271 28

Since 1978, six boys with prostatic rhabdomyosarcoma have been treated at our institution. Three had localized disease and were managed by initial biopsy, vincristine, actinomycin-D, and cyclophosphamide (VAC) chemotherapy, and bladder-sparing surgery with or without irradiation. Further combination chemotherapy ("pulse" VAC, Adriamycin, VP-16, cisplatin, and ifosfamide) was continued for 20 to 22 months following the induction course. Two boys had microscopic residual disease undetected by frozen section and unresponsive to radiotherapy. Subsequent total cystectomy 4 and 7 months later resulted in eradication of disease. In one patient, preservation of the bladder was achieved at the age of 3 months for 8 years. Artificial sphincter inserted to cure his urinary incontinence failed because of ischemia secondary to cuff compression and scar tissue. He is alive today with a modified Koch pouch urinary diversion. Of the 50% who had metastatic disease at presentation, two were dead within 12 months despite aggressive chemotherapy and irradiation. The third is currently on treatment. Although chemotherapy has markedly improved the prognosis, surgery is still necessary in most cases for cure. Bladder salvage is a desirable goal; however, residual microscopic disease, difficulty with frozen-section disease detection, and poor tissue vascularization for subsequent sphincter replacement remain significant obstacles.
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PMID:Rhabdomyosarcoma of the prostate in childhood: current challenges. 280 70

Modern multidisciplinary treatment of childhood cancer has made extent of disease evaluation important for proper treatment planning. Accurate staging is essential to cooperative group studies and for comparing treatment modalities at different centers. Operative staging plays an important role where clinical or imaging methods are limited, as in abdominal Hodgkin's disease or regional nodal metastasis. Operative staging is carried out either as a special diagnostic procedure, as in lymphoma, or as part of a planned surgical resection of a solid tumor. For lymphomas: Operative staging of abdominal Hodgkin's disease is required where protocols include involved field irradiation and sparing of normal growing tissue in the child. In non-Hodgkin's lymphoma, bulky abdominal tumor may be surgically evaluated after intensive chemotherapy either in delayed primary surgery or in second look procedures. Residual tumor may be excised or tagged with clips for localized irradiation to the tumor sparing normal abdominal organs. For solid tumors: During surgical resection of neuroblastoma, Wilms' tumor and rhabdomyosarcoma, the correct procedure involves regional staging either by formal node dissections or by multiple biopsies to determine extent of spread. Regional node dissections are often part of a correct cancer operation for cure, but also give staging information unobtainable by other methods. The surgeon must plan every procedure carefully with the aim of curing the patient and also deriving maximum information from the operation to enable correct planning of further treatment.
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PMID:The objectives and importance of operative staging of children with cancer. 301 92

The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study-I (IRS-I) were analyzed after a minimum potential follow-up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80% of patients given either treatment were still disease-free and there was no significant difference between treatments in the overall percentages of patients surviving of 93% and 81%, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72% and 65% of the patients, respectively, were disease-free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72% for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either "pulse" VAC + radiation or "pulse" VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69% in Clinical Group III and 50% in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60% chance of staying in remission for 5 years in Clinical Group III compared with approximately 30% in Clinical Group IV. The overall survival percentage at 5 years was 52% in Clinical Group III compared to 20% in Clinical Group IV (P less than 0.0001). The 5-year survival percentage for the entire cohort of 686 patients was 55%. Survival after relapse was poor, being 32% at 1 year and 17% at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low-dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.
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PMID:The Intergroup Rhabdomyosarcoma Study-I. A final report. 327 86


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