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Effects of hyperbaric oxygen and normobaric carbogen on the radiation response of the rat rhabdomyosarcoma R1H.

PURPOSE: Hypoxic tumor cells are an important factor of radioresistance. Hyperbaric oxygen (HBO) and normobaric carbogen (95% oxygen, 5% carbon dioxide) increase the oxygen delivery to tumors. This study was performed to explore changes of tumor oxygenation during a course of fractionated irradiation and to determine the effectiveness of normobaric carbogen and HBO during the final phase of the radiation treatment. METHODS AND MATERIALS: Experiments were performed on the rhabdomyosarcoma R1H growing on WAG/Rij rats. After 20 X-ray fractions of 2 Gy within 4 weeks, oxygen partial pressure (pO2) was measured using the Eppendorf oxygen electrode under ambient conditions, with normobaric carbogen or HBO at a pressure of 240 kPa. Following the 4-week radiation course, a top-up dose of 10-50 Gy was applied in 2-10 fractions of 5 Gy with or without hyperoxygenation. RESULTS: HBO but not carbogen significantly increased the median pO2 in irradiated tumors. The radiation doses to control 50% of tumors were 38.0 Gy, 29.5 Gy, and 25.0 Gy for air, carbogen, and HBO, respectively. Both high oxygen content gas inspirations led to significantly improved tumor responses with oxygen enhancement ratios (OERs) of 1.3 for normobaric carbogen and 1.5 for HBO (air vs. carbogen: p = 0.044; air vs. HBO: p = 0.02; carbogen vs. HBO: p = 0.048). CONCLUSION: Both normobaric carbogen and HBO significantly improved the radiation response of R1H tumors. HBO appeared to be more effective than normobaric carbogen, both with regard to tumor oxygenation and response to irradiation.

Animals↗

Congenital embryonal rhabdomyosarcoma of the hand and Apert's syndrome.

A unique case of congenital embryonal rhabdomyosarcoma of the hand in a patient with Apert's syndrome is presented. Immediately after birth a 1 cm soft, bluish mass of the right thenar eminence was clinically diagnosed as a hemangioma. Several months later when the lesion started rapidly enlarging the nature of the tumor was identified, and it was treated with a multidisciplinary approach that included surgical resection, chemotherapy, and radiation.

Acrocephalosyndactylia↗

Sinonasal radiation-associated osteosarcoma after combined therapy for rhabdomyosarcoma of the nose.

Radiation-associated (RA) osteosarcomas (OS) are exceptional in children, presenting more frequently in middle-aged and elderly patients. This is a case report of RA-OS of the sinonasal cavities in a 13-yr-old girl after combined therapy for rhabdomyosarcoma (RMS) of the nose diagnosed at the age of five. The treatment of choice is radical surgery with wide margins and postoperative adjuvant chemotherapy and radiotherapy. Despite an episode of recurrence, at the present time the patient is alive and free of disease 5 yr after the initial treatment of OS. The association between radiation, chemotherapy and sarcomas is reviewed and discussed.

Adolescent↗

Nasopharyngeal rhabdomyosarcoma in a patient with hypohidrotic ectodermal dysplasia syndrome.

Hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome) is characterized by partial or complete absence of sweat glands, hypotrichosis, hypodontia, prominent frontal ridges and chin, saddle nose, sunken cheeks, thick, everted lips, large ears and sparse hair. While association of other ectodermal dysplasia syndromes with tumors such a non-Hodgkin's lymphoma, hamartoma, keratoakanthoma, Merkel-cell cancer, squamous-cell carcinoma, syringofibroadenomatosis has been reported, association of hypohidrotic ectodermal dysplasia and a tumor has not been reported. In a five-year-old male patient admitted with nasal obstruction and nasal mass complaints, we have reported firstly an association of hypohidrotic ectodermal dysplasia and nasopharyngeal rhabdomyosarcoma.

Biopsy↗

Retropharyngeal rhabdomyosarcoma mimicking pseudotumor cerebri.

A 6-year-old male presented with headache, vomiting, visual obscuration, and papilledema. Clinical presentation, initial laboratory data, and radiologic evaluation suggested a diagnosis of pseudotumor cerebri. The development of lower cranial nerve palsies after transient resolution of symptoms prompted reassessment of the diagnosis, which revealed retropharyngeal rhabdomyosarcoma involving the right jugular vein. Cerebral angiography demonstrated the complete venous occlusion at the jugular foramen. Careful neurologic follow-up is essential in a patient with pseudotumor cerebri or idiopathic intracranial hypertension.

Cerebral Angiography↗

Intracardiac rhabdomyosarcoma: transesophageal echocardiographic findings and diagnosis.

Transesophageal echocardiography (TEE) was performed on a 41-year-old woman who presented with a cerebrovascular accident. TEE confirmed the presence of a morphologically bizarre biatrial tumor with precarious, vigorous motion throughout the cardiac cycle. Surgical intervention was decided on, and the patient underwent cardiac surgery for tumor excision 16 hours after TEE. Intraoperative frozen section diagnosis was spindle cell sarcoma, and subsequent immunohistochemical analysis showed the tumor to be a rhabdomyosarcoma. The data are presented here, and the role of TEE to establish a preoperative diagnosis of intracardiac tumor is discussed.

Adult↗

Imaging of primary rhabdomyosarcoma of the diaphragm.

This is a report on a rare case of embryonal rhabdomyosarcoma involving the diaphragm in a 3-year-old boy. This case illustrates the importance of preoperative imaging in the management of this tumor.

Antineoplastic Combined Chemotherapy Protocols↗

Imaging and clinical spectrum of rhabdomyosarcoma in children.

We retrospectively analyzed the MRI findings of rhabdomyosarcoma (RMSA) in 23 patients to evaluate its role in staging and management. Heterogeneous signal abnormalities were noted in the sarcoma lesions with significant contrast enhancement. Seven head and neck cases showed direct bone invasion and destruction; only one had distant bony metastasis. Metastasis was noted in the lymph nodes, lung, bone, abdominoperitoneum, and head and neck soft tissue. MRI findings of RMSA are most helpful in staging and assessing therapeutic response.

Child↗

Comparison of rhabdomyosarcoma, buffalo green monkey kidney epithelial, A549 (human lung epithelial) cells and human embryonic lung fibroblasts for isolation of enteroviruses from clinical samples.

BACKGROUND: Traditionally, primary monkey kidney (PMK) epithelial cells have been one of the more widely used cell types for the isolation of enteroviruses from clinical samples. For the isolation of coxsackie A viruses, intraperitoneal inoculation of newborn mice has been used in some laboratories. OBJECTIVE: With the discontinued availability of PMK epithelial cells and the reported growth of coxsackie A viruses in rhabdomyosarcoma cells (RD), we compared the use of the latter cell line with our routinely used microwell cell cultures. STUDY DESIGN: Microwell cell cultures of buffalo green monkey epithelial cell line (BGM), human lung carcinoma epithelial cell line (A549) and human embryonic lung (HEL) fibroblasts were compared with RD cell line for the isolation of enteroviruses from clinical samples. RESULTS: A total of 39 enteroviruses was isolated from 3517 specimens. The HEL fibroblasts yielded 28 (72%) enteroviruses, followed by A549 (25 isolates, 64%), BGM (23 isolates, 59%) and RD cells (18 isolates, 46%). CONCLUSIONS: All isolates which grew in RD cells showed specific cytopathic effects in one or more of the other inoculated cell cultures. Quantitative determinations (TCID50) with five different enteroviruses showed that the HEL fibroblasts and RD cell line to be the most sensitive cell types, followed by BGM and A549 cell lines. However, integrity of the inoculated cell monolayers was best with HEL fibroblasts and A549 cells but morphology was not optimal with RD cells during the incubation period of 14 days.

Animals↗

Intravaginal iridium-192 in the management of embryonal rhabdomyosarcoma.

Three patients with vaginal rhabdomyosarcoma and residual vaginal disease following surgery and chemotherapy have been treated using high dose irradiation with vaginal moulds loaded with iridium-192. These patients remain well and disease-free seven years, 30 months and 18 months after their initial presentations. This paper describes a technique for local vaginal irradiation with individualized vaginal moulds made using a rapid-setting silastic foam impression and loaded with a single plane of iridium wires. Details of the dosimetry are also included.

Antineoplastic Combined Chemotherapy Protocols↗

A novel local treatment strategy for advanced stage head and neck rhabdomyosarcomas in children: results of the AMORE protocol.

The AMORE protocol is a local treatment regimen for head and neck rhabdomyosarcomas (HNRMS), consisting of Ablative surgery, Moulage technique brachytherapy and surgical Reconstruction. The aim of AMORE is to intensify local treatment for children with HNRMS and to avoid external beam radiation therapy (EBRT) and its long-term sequelae. All children with primary irresectable, non-orbital HNRMS in whom EBRT was indicated, were evaluated for the feasibility of AMORE. In 20 children, AMORE was performed (15 with parameningeal disease and five with non-parameningeal disease). Complete remission was achieved in all 20 patients. Local complications were limited. 5 patients experienced a local relapse and 1 patient developed distant metastases. Estimated 5-year OS and EFS were 67.5 and 64.1% for the entire group, and 64.2 and 60.0% for the parameningeal subgroup. We conclude that the AMORE protocol is a feasible strategy, with a good local control rate. Long-term sequelae of EBRT might be avoided although, to date, the follow-up is too short for definitive conclusions regarding these sequelae.

Antineoplastic Combined Chemotherapy Protocols↗

Rhabdomyosarcoma of the oral cavity. Report of four cases.

Clinical and pathologic findings of four cases of rhabdomyosarcoma of the oral soft tissues are described that include findings from immunohistochemistry and electron microscopy. Three cases occurred in children under 16 years of age and one in a 22-year-old. Included is a brief discussion on reported gene abnormalities that may contribute to neoplastic development.

Adolescent↗

Dento-maxillofacial sequelae in a child treated for a rhabdomyosarcoma in the head and neck. A case report.

This case report describes severe dento-maxillofacial defects after chemoradiation therapy in a child aged 9 years 6 months with a parameningeal rhabdomyosarcoma. A clinical and radiologic (apical dental radiographs, orthopantograph, lateral skull roentgenogram) dental follow-up over 4 years showed such dental abnormalities as root blunting, mild to severe root shortening, premature closure of the root apices, and severe radiation caries. Craniofacial morphology evaluated by cephalometric analysis and dental models showed deficiency with mandibular and maxillary hypoplasia.

Child↗

Protruding giant cervical polyp in a young adolescent with a previous rhabdomyosarcoma.

Giant cervical polyps are rare entities, with only 8 reported cases in the international literature (MEDLINE 1966-2002). Of these cases, only one has been described in the pediatric population. There has been no report of a giant cervical polyp occurring in the older, although early, adolescent period. The discovery of a giant cervical polyp in a young adolescent is a gynecological oddity. The immense size of giant cervical polyps, together with their menacing appearance and unusual presentation, can simulate a malignant neoplasm. Care must be taken in their diagnosis and management so that the patient's fertility is not compromised. We report on a young adolescent girl with a previous history of a rhabdomyosarcoma (RMS), who presented with a giant cervical polyp protruding from the vaginal introitus. The literature on this entity is reviewed.

Child↗

Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases.

Rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992-2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5-8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.

Child↗

Re-irradiation for recurrent rhabdomyosarcoma of the nasopharynx in a child.

There are few clinical situations where high dose re-irradiation with curative intent is indicated in children. Because of the potential for serious late sequelae, re-irradiation is considered only in the unusual situation where there is just local recurrence and surgery and/or chemotherapy are unlikely to provide long-term control. The authors present a case of nasopharynx rhabdomyosarcoma in a child which recurred after high dose irradiation and chemotherapy and was then re-irradiated to a high dose. The child is well without any serious sequelae more than 13 years following completion of the second course of radiotherapy.

Antineoplastic Agents↗