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PubMed2005

Long-term follow-up and prognosis of orbital apex syndrome resulting from nasopharyngeal rhabdomyosarcoma.

PURPOSE: Nasopharyngeal rhabdomyosarcoma may present with a variety of ophthalmic symptoms. Direct extension of the tumor into the orbital apex can lead to ophthalmoplegia and loss of vision. The prognosis for recovery of vision and ocular motility in patients with an orbital apex syndrome due to nasopharyngeal rhabdomyosarcoma is examined. DESIGN: Retrospective case series. METHODS: Six eyes from four patients with nasopharyngeal rhabdomyosarcoma who presented to the Children';s Hospital of Philadelphia with a clinical orbital apex syndrome were identified. Complete ophthalmic examination, including visual acuity and extraocular motility at the time of presentation, was reviewed. Tumor extension into the orbital apex was confirmed radiographically. Follow-up ophthalmic evaluations were reviewed for all patients with an average follow-up of 5.5 +/- 3.1 years (range 1 to 8 years). RESULTS: Six eyes of four patients had limited ocular ductions along with marked loss of vision at presentation. All patients were treated with chemotherapy and radiation, with reduction of tumor mass. Ocular motility recovered in all patients, occurring by an average of 2.2 +/- 1.8 months after initiation of therapy. Four of six eyes had little or no recovery of visual acuity detected at long-term follow-up. CONCLUSIONS: Patients with ocular motor deficits in orbital apex syndromes caused by extension of nasopharyngeal rhabdomyosarcoma have an excellent prognosis for recovery after treatment of the tumor. The long-term prognosis for visual recovery, however, is poor.

Child, Preschool↗
PubMed2005

Rhabdomyosarcoma of the cervix in sisters with review of the literature.

OBJECTIVES: Pediatric embryonal heterologous rhabdomyosarcoma of the cervix is a rare tumor. METHODS: We present an interesting clinico-pathologic situation of two sisters presenting with pediatric embryonal heterologous rhabdomyosarcoma of the cervix. RESULTS: Pediatric embryonal heterologous rhabdomyosarcomas of the cervix are relatively uncommon. After a Pubmed search from 1952 to present, to our knowledge, this is the only report involving sisters presenting with this disease. Treatment has been extrapolated from collaborative groups such as Intergroup Rhabdomyosarcoma Study (IRS) Group so that optimal management may be achieved. CONCLUSIONS: We urge continued reporting of these rare tumors to enhance understanding if there may be a genetic component associated with them.

Adolescent↗
PubMed1997

Actinomycin D treatment leads to differentiation and inhibits proliferation in rhabdomyosarcoma cells.

Human embryonal cell line RD is derived from rhabdomyosarcoma, a tumor of childhood that arises from rhabdomyoblasts probably arrested somewhere along their pathway to maturation. Because actinomycin D is a drug of choice in the treatment of rhabdomyosarcomas, and because it has been used to induce differentiation as an alternative therapy for myeloproliferative syndromes, we treated RD cells with different concentrations of actinomycin D and evaluated the effects on growth and differentiation. Actinomycin D treatment in vitro caused time- and dose-dependent growth inhibition. Interestingly, RD cells treated with low doses (2.85 and 5.7 nmol/L) of actinomycin D for 6 days showed morphologic and phenotypic differentiation, with increased expression of desmin, alpha-actinin, and tropomyosin. However, treatment with 11.4 nmol/L actinomycin D strongly inhibited growth and had cytotoxic effects that prevented the cells from attaining myogenic differentiation. We conclude that exposure of this human embryonal rhabdomyosarcoma cell line to low concentrations of actinomycin D released the neoplastic cells from their blockade, allowing them to recover normal myogenic development. We suggest a potential role for differentiation therapy in the treatment of rhabdomyosarcomas.

Antibiotics, Antineoplastic↗
PubMed1997

Rhabdomyosarcoma treated with chemotherapy during the third trimester.

BACKGROUND: Rhabdomyosarcoma is an aggressive tumor that is rarely found in pregnancy, and is usually treated with multiagent chemotherapy. Chemotherapy given during pregnancy is associated with several maternal-fetal complications, including risks for mutagenicity, myelosuppression, and fetal death. CASE: An 18-year-old woman had stage III facial rhabdomyosarcoma diagnosed early in the third trimester. She achieved clinical remission with multiagent chemotherapy given during pregnancy, with no fetal complications. CONCLUSION: Invasive rhabdomyosarcoma is biologically predisposed to metastasize, and in the absence of effective chemotherapy, most patients will develop sites of distant recurrence. Chemotherapy plays an important role as frontline treatment in pregnant women with rhabdomyosarcoma.

Adolescent↗
PubMed1998

Conservative management of uterine rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcomas are rare, malignant tumors derived from primitive myogenic precursors and are the most common soft tissue neoplasms in children and adolescents. We used primary chemotherapy and subsequent removal of the residual polypoid mass to treat an adolescent female with uterine rhabdomyosarcoma. CASE: A 15-year-old white adolescent who presented with a polypoid uterine rhabdomyosarcoma was treated with vincristine, etopside, and ifosfamide, after which the residual polypoid mass was removed. CONCLUSION: Treating adolescent females with a polypoid uterine rhabdomyosarcoma with primary chemotherapy followed by removal of the residual mass preserves reproductive function and should be considered.

Adolescent↗
PubMed1987

Pleomorphic rhabdomyosarcoma in adults: immunohistochemistry as a tool for its diagnosis.

Pleomorphic rhabdomyosarcoma in adults over 30 years of age was a diagnosis frequently made in the 1960s and 1970s. Since the general acceptance of malignant fibrous histiocytoma (MFH) as a tumor entity at the end of the 1970s, however, it has become a very rare tumor in adults. Therefore, 21 cases originally diagnosed on the basis of histology and clinical data as pleomorphic rhabdomyosarcoma in the 1960s and 1970s were reexamined immunohistochemically. Other types of pleomorphic sarcomas involved in the differential diagnosis were also studied. Specific antibodies against vimentin, desmin, creatine kinase subunit M, skeletal muscle actin and myosin, and myoglobin, and the avidin-biotin-peroxidase complex technique were used. The immunohistochemical findings indicate that rhabdomyosarcoma occurs only rarely in adults over 30 years of age and that the majority of the tumors have to be reclassified as MFH or leiomyosarcoma. On the other hand, several pleomorphic sarcomas were found to be diagnosed incorrectly as MFH or liposarcoma by routine histologic stains and electron microscopy. The revised diagnosis was pleomorphic rhabdomyosarcoma for one case and pleomorphic leiomyosarcoma for the other cases. Thus, this study clearly shows the usefulness of immunohistochemistry as a technique in the diagnosis of pleomorphic sarcomas in adults.

Adult↗
PubMed2003

Osteogenic sarcoma after orbital radiation rhabdomyosarcoma.

PURPOSE: We describe the occurrence of maxillary and orbital osteogenic sarcoma in a child after treatment of contralateral orbital rhabdomyosarcoma with external beam radiation and chemotherapy. DESIGN: Interventional case report. INTERVENTION: Treatment of a maxillary and orbital rhabdomyosarcoma with surgical resection, systemic chemotherapy, and local radiation. MAIN OUTCOME MEASURES: Occurrence and histology of secondary malignancy after orbital radiation. RESULTS: An eleven year-old male presented for evaluation of left facial swelling, occurring ten years after successful treatment of a right orbital embryonal rhabdomyosarcoma with chemotherapy and 5040 cGY of external beam radiation. Computerized tomography demonstrated a mass involving the left maxillary sinus and orbital floor. Biopsy demonstrated osteogenic sarcoma. Despite attempted total excision with radical maxillectomy, resection margins were found to have microscopic extension of the tumor. Postoperatively he was treated with systemic chemotherapy and local radiation. Eight months postoperatively he remains alive despite local progression. CONCLUSIONS: Osteogenic sarcoma can occur as a secondary malignancy years after the successful treatment of orbital rhabdomyosarcoma with external beam radiation and chemotherapy. After orbital radiation, subjects should undergo routine lifelong examinations.

Child↗
PubMed1997

Multidisciplinary management of refractory orbital rhabdomyosarcoma.

PURPOSE: Combined chemotherapy and radiation therapy have improved the survival of children with primary orbital rhabdomyosarcoma, but recurrence or persistence of the local orbital tumor still occurs. There are no established guidelines for dealing with these uncommon patients, and the authors present a review of the combined method treatment and outcome of children with refractory primary orbital rhabdomyosarcoma. METHODS: From clinical databases, 67 children with orbital rhabdomyosarcoma were identified. Seven (10%) of the 67 children had tumors refractory to combined chemotherapy and radiation therapy and underwent exenteration or eye-sparing tumor excision. Their clinical course and outcome were reviewed retrospectively. RESULTS: No patient was lost to follow-up, which ranged from 3.2 to 11 years. Five (71%) of the seven children with refractory tumor are still alive at more than 3 years after surgery (3.2-11 years; mean, 6.9). In one of the two children who died, tumor extended beyond the operative margins at exenteration, and the other child died with regional metastasis within a month of exenteration. CONCLUSIONS: Although more than 90% of children with orbital rhabdomyosarcoma respond to combined therapy by pediatric oncologists and radiotherapists, local orbital (salvage) surgery by ophthalmologists may be of value in the minority of children with refractory tumors. All of the five surviving children appear to be disease free.

Adolescent↗
PubMedDate not supplied

Pediatric sinonasal rhabdomyosarcoma: three cases and a review of the literature.

OBJECTIVE: To understand the clinical presentation, management, and natural history of paranasal rhabdomyosarcoma. DESIGN: Retrospective case series review. METHODS: Retrospective medical record review of patients less than 20 years of age who presented to our facility with rhabdomyosarcoma of the nasal cavity or paranasal sinuses. RESULTS: Medical records of all pediatric patients seen in our pediatric otolaryngology clinic were reviewed from January 1, 1995, through December 31, 2000. Three patients were identified with sinonasal rhabdomyosarcoma. Their presentation, evaluation, and treatment were evaluated. Relevant literature 1966 to the present was reviewed with the assistance of Medline. CONCLUSIONS: Rhabdomyosarcoma is an aggressive pediatric malignancy, requiring a high index of suspicion to detect it in its earliest stages. Patients with suggestive symptoms should undergo a full evaluation including nasal endoscopy and imaging. Because the current chemotherapy protocols are more effective on localized disease, early diagnosis is crucial to patient survival.

Child↗
PubMed1989

Cytokeratin expression in experimental murine rhabdomyosarcomas. Intermediate filament pattern in original tumors, allotransplants, cell culture and re-established tumors from cell culture.

Soft tissue sarcomas were induced by 20-methylcholanthrene in NMRI-mice. The tumors were characterized as rhabdomyosarcomas by light and electron microscopy as well as immunohistochemistry (vimentin, desmin and myoglobin expression). Cytokeratins could be demonstrated by a panel of different poly- and monoclonal antibodies in original rhabdomyosarcomas, their allotransplants and the re-established tumors from cell culture in nude mice. The cytokeratin positive tumor cells were arranged in small clusters and/or haphazardly single dispersed in the rhabdomyosarcomas. By means of monoclonal antibodies cytokeratins No. 8 and No. 19 could be evidenced and cytokeratin No. 18 could be made probably. Behind the background of cytokeratin expression in developing fetal cross striated muscle cells our findings are discussed as a reminiscence of embryonal muscle development in these tumors. The significance of cytokeratin expression in rhabdomyosarcomas for diagnostic histopathology is emphasized.

Animals↗
PubMed2000

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.

Biomarkers, Tumor↗
PubMed1994

Creatine kinase isoenzymes as markers of differentiation in rhabdomyosarcoma cells in culture.

Rhabdomyosarcomas have been classified on the basis of their degree of differentiation, a feature closely related with their response to chemotherapy. We recently reported the role of creatine kinase isoenzymes as tumoral markers in the diagnosis of rhabdomyosarcoma. The present study was designed to determine whether these isoenzymes are also good markers of the degree of differentiation of these neoplasm. Dimethyl sulfoxide, a well-known differentiating agent, was used to induce myogenic differentiation. This agent increased CK-MM and/or CK-MB fractions, and decreased CK-BB isoenzyme in rhabdomyosarcoma cell lines. The variable behavior of MCK-1 macromolecule was unrelated to the degree of differentiation. Our findings suggest that CK-MM, CK-MB and CK-BB isoenzymes are good markers of the degree of differentiation in rhabdomyosarcomas and could be used as specific markers of prognostic and diagnostic value.

Biomarkers, Tumor↗
PubMed2000

Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma.

PURPOSE: To examine the long-term effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma. METHODS: From 1967 to 1994, a total of 30 children with head and neck rhabdomyosarcoma received megavoltage radiotherapy at one institution. Seventeen patients (57%) have survived and have at least a 5-year follow-up. There were 11 males and 6 females, with a median age of 5.7 years (range 2.2-11.6) at the time of radiotherapy. Tumor location was orbit in 6 patients, infratemporal fossa in 4, paranasal sinuses in 2, and supraglottic larynx in 2; the nasopharynx, pterygopalatine fossa, and parotid gland were sites for the remaining children. All but 2 patients had tumors of embryonal histology. The Intergroup Rhabdomyosarcoma Study (IRS) Group was I in 2, II in 3, and III in 11 children; 1 patient had a recurrent tumor after surgery alone. Radiotherapy volume was the primary tumor or tumor bed in 13, tumor and whole brain in 3, and tumor and craniospinal axis in 1. Median radiotherapy dose to the primary site was 5,040 cGy (range 4,140-6,500) and to the whole brain was 3,000 cGy. All but 1 were treated with 150-200-cGy fractions; 1 patient received 250-cGy fractions for a tumor in the larynx. Chemotherapy was vincristine (V), actinomycin-D (A), and cyclophosphamide (C) in 10 patients, VAC + adriamycin in 2, VA in 1, VA + ifosfamide in 1, VC + adriamycin in 1, and none in 2. One patient had salvage chemotherapy consisting of cisplatin and etoposide. Median follow-up time was 20 years (range 7.5-33). RESULTS: Late effects of treatment were seen in all patients and included facial growth retardation in 11, neuroendocrine dysfunction in 9, visual/orbital problems in 9, dental abnormalities in 7, hearing loss in 6, and hypothyroidism in 3. Intellectual and academic delays were documented in 3 patients who had received whole brain radiotherapy. While neuroendocrine, thyroid, dental, and cognitive sequelae were primarily attributed to radiotherapy, hearing loss was thought to be a direct result of tumor destruction and, in 1 case, cisplatin chemotherapy. Late effects at or beyond 10 years from radiotherapy were few, but severe, and included chondronecrosis, esophageal stenosis, second malignancy, and brain hemorrhage. CONCLUSION: Late effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma are frequent. Although radiotherapy is a significant contributor of neuroendocrine, dental, thyroid, and cognitive toxicity, it is not usually implicated with hearing loss. Late toxicity of treatment beyond 10 years is not as frequent as those occurring within 10 years of therapy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

Implant brachytherapy: a novel treatment for recurrent orbital rhabdomyosarcoma.

BACKGROUND: Orbital rhabdomyosarcoma is the most common primary malignancy of the orbit in childhood. Tumor resection and exenteration were the preferred treatment modalities for rhabdomyosarcoma. In the past 20 years, however, combined local radiation and systemic chemotherapy have shown excellent survival results. Tumor recurrence after any of the aforementioned therapies is almost always fatal. We have developed a novel treatment for recurrent disease that has resulted in long-term survival for three patients. METHODS: Three patients with recurrent orbital rhabdomyosarcoma were previously treated with primary radiation and chemotherapy. At the time of recurrence, exenteration and localized brachytherapy were performed. An individually molded poly(methylmethacrylate) (Lucite; E. I. du Pont de Nemours & Co., Wilmington, Del.) device loaded with radioactive iodine seeds delivered localized high-dose radiation, 6000 cGy over 6 days, to the orbit without irradiating the brain. RESULTS: All patients are alive and free of disease with follow-up ranging from 4 years and 4 months to 8 years and 4 months. CONCLUSION: A novel technique of delivering localized radiation to the orbit of three children with recurrent orbital rhabdomyosarcoma appears curative.

Brachytherapy↗
PubMed2003

Rhabdomyosarcoma development in mice lacking Trp53 and Fos: tumor suppression by the Fos protooncogene.

The Fos protein, a major component of the AP-1 transcription factor, is essential for osteoclast differentiation, acts as an oncogene, potentiates transforming signals, and controls invasive growth and angiogenesis during tumor progression. To investigate a potential genetic interaction between the Trp53 and Fos pathways, Trp53/Fos double knockout mice were generated. These mice develop highly proliferative and invasive rhabdomyosarcomas of the facial and orbital regions, with more than 90% penetrance at 6 months of age. Rhabdomyosarcoma cell lines established from the primary tumors express characteristic muscle-specific markers, and reexpression of Fos is associated with enhanced apoptosis in vitro. Moreover, Fos is able to repress Pax7 expression in rhabdomyosarcoma cell lines and primary myoblasts, suggesting a molecular link to genetic alterations involved in human rhabdomyosarcomas.

Animals↗
PubMed1998

Laryngeal rhabdomyosarcoma in adults.

Laryngeal rhabdomyosarcoma is a rare disease. Only nine of these tumours have been adequately described in world literature in the adult population. Adult patients with laryngeal rhabdomyosarcoma often present at a later stage than other laryngeal tumours, including squamous cell carcinoma. Diagnosis is made by identification of cross-striations histochemically or cytoplasmic myoglobin by immunohistological methods. We present a 66-year-old woman with pleomorphic rhabdomyosarcoma of her larynx. This is the first female in the adult age group to be presented. Surgical treatment with adjuvant radiotherapy is currently the treatment of choice for this disease. We provide a review of the literature on laryngeal rhabdomyosarcoma, including presentation, pathology and management of this rare disease.

Aged↗
PubMed1986

Comparative studies between a new human rhabdomyosarcoma cell line, JR-1 and its tumour of origin.

A new human embryonal rhabdomyosarcoma cell line, designated JR-1, is described that closely resembles the tumour from which it was derived. Comparative studies, by light and electron microscopy reveal morphological features such as myofibre formation, that are concordant with embryonal rhabdomyosarcoma. Immunohistological investigations using a panel of monoclonal antibodies indicate that the cell surface antigen profile of the JR-1 cell line is similar to other embryonal rhabdomyosarcomas. In addition the cell line expresses the cytoplasmic intermediate filament protein desmin, only found in cells of rhabdoid origin. Karyotyping JR-1 shows the cells to contain variable numbers of chromosomes (range 44-100). DNA flow cytometry indicates that cells have an DNA content which is approximately twice normal. The JR-1 cell line has a doubling time of 29 h in culture and, in common with several other human cell lines, produces xenografts in nude mice within 6 weeks of inoculation. With detailed studies on the original tumour and the JR-1 cell line, the latter should prove an excellent model system for investigating the biology of rhabdomyosarcoma.

Animals↗
PubMed1991

In vitro differentiation of rhabdomyosarcomas induced by nickel or by Moloney murine sarcoma virus.

In vitro cultures and clonal derivatives have been established from rat rhabdomyosarcomas induced by Moloney-Murine Sarcoma Virus (MSV) or by nickel sulfide; differentiation ability has been studied as expression of desmin, embryonic and adult myosin isoforms, alpha-actin isoforms and cellular fusion. The two rhabdomyosarcoma models showed different levels of myogenic differentiation. Multinucleated myotube-like structures were frequently observed in cultures derived from nickel-induced tumours. Desmin was present in 50-80% of cells and embryonic myosin in up to 10%. In MSV-tumour-derived cultures and in their metastases or clonal derivatives two cell types are present in different ratios: spindle-shaped cells, adherent to plastic surfaces, and rounded cells, loosely attached or floating free in the medium. These cultures showed features of myogenic differentiation (10-80% desmin-positive cells), but embryonic myosin expression and production of multinucleated myotube-like structures were very rare events. Cultures from autochthonous lymph node and lung metastatic cells showed similar patterns of differentiation. Retinoic acid increased differentiated features (myotube formation and embryonic myosin expression) only in nickel-induced rhabdomyosarcoma cells. The two models described here mimic the heterogeneity in differentiation pattern found among human rhabdomyosarcomas. Myogenic differentiation ability was retained at a good level by nickel-induced tumours, whereas it was strongly impaired in MSV-induced tumours.

Animals↗