Embryonal Rhabdomyosarcoma In Children Research Articles

Rare tumours of the bladder: A Saudi registry based descriptive study

ObjectiveThere is limited literature focusing on the characteristics and behaviours of bladder tumours outside of the common three morphologies, that is, urothelial carcinoma, squamous cell carcinoma, and adenocarcinoma. The presented study provides a descriptive analysis of rare bladder tumours in KSA. MethodsThis retrospective cohort study included all patients with a primary rare bladder tumour between 1 January 2008 and 31 December 2017. The data were acquired from the Saudi Tumour Registry. Frequencies and percentages were then generated for the categorical variables, while means and standard deviations were calculated for quantitative variables. ResultsThe study included 65 patients. The majority (n = 35, 53.8%) were aged 60 years and older. The patients were predominantly male (n = 53, 81.5%) and the majority lived in the Western region (n = 26, 40.?%). The most diagnosed tumour morphologies were small cell carcinoma in adults (n = 11, 16.9%) and embryonal rhabdomyosarcoma in children (n = 14, 21.5%), with the dominant diagnosis method being histology of primary tumour in 98.5% of the patients. Most tumours were localised (n = 30, 46.2%) and multifocal (n = 34, 52.3%). The overall mortality rate was 24.6%, with an overall diagnosis to death interval of 1.14 ± 0.75 years wherein small cell carcinoma was the shortest (0.84 ± 0.24) days. ConclusionThere remains a gap in the literature regarding uncommon urologic tumours. Shedding light on these factors will aid in further understanding the patterns of tumour behaviour in the region. This will facilitate enhanced risk-and response-based screening strategies and more favourable outcomes. Additionally, formulating a global registry for such patients is recommended.

Characteristics and prognosis of embryonal rhabdomyosarcoma in children and adolescents: An analysis of 464 cases from the SEER database.

ABSTRACTImportanceAs the most common subtype of pediatric rhabdomyosarcoma (RMS), the prognosis of embryonal RMS has rarely been investigated solely.ObjectiveTo perform a population‐based study to characterize the prognosis of embryonal RMS in children and adolescents.MethodsDemographic and clinical features were retrospectively evaluated in selected patients with embryonal RMS registered in the Surveillance, Epidemiology, and End Results (SEER) program from 1988 to 2016. Survival curves were compared using the log‐rank test. A multivariate Cox proportional hazards model was developed to assess the impact of each factor on the overall survival. A nomogram was constructed based on the results of Cox regression model.ResultsA total of 464 patients were included in the analysis, among which 64.6% were male and 70.2% were white patients. About 38.6% and 26.3% of the patients were at 1–4 years and 5–9 years, respectively. Cox analysis showed that patients at age group 5–9 years had the lowest risk of mortality (hazard ratio [HR], 0.277; 95% confidential interval [CI], 0.123–0.620), compared with patients diagnosed at less than 1‐year‐old, and age group 1–4 years had the second‐best prognosis. Patients having distant tumors had significantly higher mortality risk (HR, 4.842; 95% CI, 2.804–8.362) than the patients with localized tumor. Compared with receiving no surgery or radiotherapy, receiving any combination of surgery and radiotherapy would lower the risk of mortality significantly (for surgery without radiotherapy: HR, 0.418; for radiotherapy without surgery: HR, 0.405; and for surgery plus radiotherapy: HR, 0.410).InterpretationAge, stage at diagnosis, and treatment received were found to be the most important predictors of the overall survival of pediatric embryonal RMS.

Case Series on Four Treated Cases of Embryonal Rhabdomyosarcoma in Children.

We report four cases of embryonal rhabdomyosarcoma treated successfully with multimodality therapy which included surgery, chemotherapy and radiotherapy. Our first case was a 2year old who admitted with the chief complaint of swelling of the upper lip. Biopsy was taken, which showed embryonal rhabdomyosarcoma. It was followed by a CT scan (face + neck), MRI face, USG abdomen and bone scan of the patient. Pre operatively chemotherapy was given. After completion of chemotherapy regimen, wide local excision of the lesion along with a reverse fan flap and bilateral modified radical neck dissection type 3 was done. This was followed by post operative chemotherapy and radiotherapy. There was no recurrence at 6months post operatively. The second patient was 6years old with similar complaints, after undertaking all the investigations as in the previous case, patient was given neoadjuvant chemotherapy following which wide local excision of the lesion along with reconstruction with the help of bilateral nasolabial flap was done. This was followed by post operative chemotherapy. No recurrence was present 6months post operatively. The third patient was a 17year old male presenting with complaints of left sided nasal obstruction and left nasal swelling. Examination revealed left sided nasal mass which on biopsy turned out to be embryonal rhabdomyosarcoma. The patient underwent surgery followed by chemoradiation. At 6months the patient had no evidence of recurrence. The fourth patient was 16-year-old male who presented with complaints of right sided nasal swelling and nasal obstruction. Radiological investigation showed the tumor involving the right sinonasal cavity with extension to anterior cranial fossa and with neck nodes. Patient was given concurrent chemoradiation. Patient was followed up for 6months when the patient presented with recurrence of disease with distant metastasis. Patient is currently on palliative chemotherapy. To summarise, embryonal rhabdomyosarcoma in young children when treated aggressively with surgery and chemotherapy has better prognosis than with chemo-radiation alone.

Chemotherapy as monotherapy for treatment of non-metastatic bladder/prostate embryonal rhabdomyosarcoma in children: Preliminary report

Objective This work aims at evaluation of chemotherapy as mono-therapy for treatment of non-metastatic bladder/prostate embryonal rhabdomyosarcoma in children. Patients and methods Between 1996 and 2002, 10 children presented with RMS of the bladder/prostate at a mean age of 6.6 years (range 1.5–18). Four patients had stage 2 disease and 6 had stage 3. The histology was embryonal in 8 and botryoid in 2. All patients received VAC chemotherapy for a mean duration of 14 months (range 12–18). CT or MRI was performed at 8 weeks, every 3 months in the first year and every 6 months thereafter. Results The mean follow-up duration was 48 months (range 12–96). At 8 weeks, 7 children showed more than 50% reduction in tumour size and the remaining 3 showed complete disappearance on CT or MRI. At final follow-up 8 patients are living, disease-free, with functioning bladder. The remaining 2 patients died of metastatic disease at 12 and 14 months from start of treatment. Out of the 8 survivors, 5 had complete disappearance of the tumour at last follow-up while 3 had residual tumour on CT/MRI. Those children underwent transurethral endoscopic resection and the pathology showed no evidence of malignancy in all. The 4-year disease-free survival was 100% for stage 2 and 66% for stage 3. Conclusions Chemotherapy as mono-therapy is effective treatment in children with stage 2 embryonal RMS of the bladder/prostate with the advantage of bladder preservation. Within stage 3, non-survivors had statistically significant larger tumours. Adjuvant therapy or more intensive chemotherapy might be required for those with large stage 3 tumours.

Spindle cell rhabdomyosarcoma in adults. A case report and literature review

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.

Childhood Rhabdomyosarocoma with Anaplastic (Pleomorphic) Features

The pleomorphic subtype of rhabdomyosarcoma (RMS) is now rarely diagnosed in both children and adults. Most cases previously called pleomorphic RMS are probably diagnosed as something else, most often embryonal RMS in children and malignant fibrous histiocytoma in adults. To analyze the concept of pleomorphic RMS in children, we reviewed the tumors of patients entered on the Inter-group Rhabdomyosarcoma Study (IRS I, II, and III). The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the common tumor cell population (anaplastic cells) was selected as the main criterion. Of about 3,000 cases, 110 showed these types of cells, had sufficient histologic material, and had available follow-up data. These tumors were divided into two subgroups: Subgroup I tumors contained only scattered anaplastic cells, and tumors with foci or large sheets of anaplastic cells were classified as subgroup II. Besides the anaplastic-pleomorphic areas, most of these tumors had distinctive features of embryonal RMS (105 cases) and rarely had characteristics of alveolar RMS (five cases). The age distribution of these patients did not differ significantly from those whose tumors did not show the anaplastic features, the average being 6 years and the median 4 years. Lower extremity, retroperitoneum, and the head and neck region were the most common primary tumor sites. The 5-year survival rate was 60% for subgroup I tumors and 45% for subgroup II tumors compared with the survival rate of 68% for 482 IRS II embryonal RMS cases with no anaplastic-pleomorphic features. The lower survival rate for patients in subgroup II was statistically significant (p = 0.004) and similar to the unfavorable survival of patients with alveolar RMS and undifferentiated sarcoma. Because anaplastic cells are seen in many soft tissue sarcomas and in both embryonal and alveolar RMS in children, this feature is not sufficiently unusual to separate a pleomorphic subtype of RMS. The presence of anaplastic cells in aggregates or diffuse sheets throughout the tumor, however, portends a poor survival for these patients.

Local control of embryonal rhabdomyosarcoma in children by radiation therapy when combined with chemotherapy

Between August 1970 and March 1978, 58 patients with embryonal rhabdomyosarcoma (ERMS) were treated at the Radiation Therapy and Pediatric Departments of MSKCC. Chemotherapy was given according to T2 protocol (sequential administration of dactinomycin, vincristine, adriamycin and cyclophosphamide) or the T6 protocol (simultaneous administration of the previous drugs plus bleomycin, methotrexate and BCNU), which was introduced in 1975. The primary tumor or regional metastases were completely or partially removed in 43 patients, while biopsy was the only surgical procedure in 15. There were 41 boys and 17 girls, between 4 months and 19 years old. Eight had stage I-B disease, (microscopic residual), 16 stage II, (gross residual), 24 stage III, (node metastases), and 10 patients stage IV (disseminated tumors). Thirty-five patients were treated with T2 protocol, twenty-three with T6 protocol. Sixteen patients received more than 5000 rad, 21 had between 4000 and 5000 rad and 21 had less than 4000 rad. Forty-four patients are alive, 38 of them disease free. Local tumor control was not achieved in 14 patients, 10 of them were treated with T2 and 4 with T6. There were no local failures in patients treated for microscopic disease with doses between 3000 and 4000 rad. In patients treated for bulky tumors with 4000–5000 rad there were 3 failures out of 11 tumors and 3 out of 17 in those treated with higher doses. Radiation doses 3000–4000 rad were sufficient for local control of microscopic disease and 4000–5000 rad were as effective for control of bulky tumors as higher doses.

Pharmacokinetics of gentamicin during exchange transfusions in neonates

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Local control of embryonal rhabdomyosarcoma in children by radiation therapy when combined with chemotherapy

Local control of embryonal rhabdomyosarcoma in children by radiation therapy when combined with chemotherapy

Local control of embryonal rhabdomyosarcoma in children by radiation therapy when combined with concomitant chemotherapy

Twenty-one patients are described in whom mufti-drug chemotherapy has been administered in addition to surgery and irradiation for control of local disease. Doses of irradiation and time administered have been related to extent of local residual tumor. Of 14 patients treated for bulky tumor, 7 received less than 5000 rad and 2 are alive without recurrence; 5 died with local recurrence. Seven patients received doses in excess of 5000 rad and 6 are alive without recurrence. Of 7 patients treated for microscopic residual disease, 4 received less than 5000 rad and are alive without recurrence; 2 received more than 5000 rad and are alive without recurrence. One patient who received less than 5000 rad, for microscopic residual disease, and who died with local recurrence, is thought to have suffered a “geographical miss”.

Multidisciplinary treatment of embryonal rhabdomyosarcoma in children.

Twenty-nine children under 15 years of age with embryonal rhabdomyosarcoma were treated according to a multidisciplinary protocol (T-2). The protocol consisted of surgical removal of the tumor if possible, followed by chemotherapy, and also with radiation therapy in patients with gross or microscopic residual disease. Radiation therapy was given in the 4500-7000 rads range. The chemotherapy consisted of cycles of sequential administration of dactinomycin, Adriamycin, vincristine, and cyclophosphamide, with obligatory periods of rest. The drug therapy was continued for 2 years. Following surgery, clinicopathologic staging of the disease revealed 10 patients with no residual disease (I-A), 5 with microscopic residual disease (I-B), 5 with unresectable tumors (II), 6 with unresectable tumors plus regional lymph node involvement (III), and 3 with disseminated tumors (IV). Twenty-four (82%) of the patients (20 Stages I-II, 4 Stage III) are alive with no evidence of disease for 4 plus to 42 plus months. These results are superior to those achieved between 1960-1970 among 108 children treated at Memorial Sloan-Kettering Cancer Center.

Combination therapy of urogenital embryonal rhabdomyosarcoma in children: F. Ghavimi, P.R. Exelby, G.J. D'Angio, W.F. Whitmore, Jr., P.H. Lieberman, J.L. Lewis, Jr., V. Mike, and M.L. Murphy. Cancer 32, 1178–1185 (November), 1973

Combination therapy of urogenital embryonal rhabdomyosarcoma in children: F. Ghavimi, P.R. Exelby, G.J. D'Angio, W.F. Whitmore, Jr., P.H. Lieberman, J.L. Lewis, Jr., V. Mike, and M.L. Murphy. Cancer 32, 1178–1185 (November), 1973

Management of Embryonal Rhabdomyosarcoma in Children

Embryonal rhabdomyosarcoma, a highly malignant tumor with a previously poor prognosis, is the commonest soft tissue sarcoma seen in children. Due largely to improved chemotherapy and advances in radiation therapy, it is now possible to produce higher cure rates with less extensive surgical procedures.

Proceedings: Combination therapy of urogenital embryonal rhabdomyosarcoma in children.

Twenty-seven children with embryonal rhabdomyosarcoma of the urogenital tract were treated between 1960-1971. A review of their courses reveals that early stage of the disease at the time of diagnosis and multidisciplinary therapy —surgery, radiotherapy, and multiple drug therapy—offer the best chance for prolonged survival and cure. Seventeen (63%) of the 27 children are living and have been free of the tumor for 18 months-10 years.

Chemotherapy of urologic embryonal rhabdomyosarcoma in children

Chemotherapy of urologic embryonal rhabdomyosarcoma in children