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At least 523 records · Page 29Linked to original sources
PubMed2000

TGF-beta autocrine loop regulates cell growth and myogenic differentiation in human rhabdomyosarcoma cells.

Transforming growth factor beta (TGF) is a well-known inhibitor of myogenic differentiation as well as an autocrine product of rhabdomyosarcoma cells. We studied the role of the TGF-beta autocrine loop in regulating growth and myogenic differentiation in the human rhabdomyosarcoma cell line, RD. We previously reported that the phorbol ester 12-O-tetradecanoylphorbol-13-acetate (TPA) induces growth arrest and myogenic differentiation in these cells, which constitutively express muscle regulatory factors. We show that TPA inhibits the activation of secreted latent TGF-beta, thus decreasing the concentration of active TGF-beta to which the cells are exposed. This event is mediated by the TPA-induced alteration of the uPA/PAI serine-protease system. Complete removal of TGF-beta, mediated by the ectopic expression of a soluble type II TGF-beta receptor dominant negative cDNA, induces growth arrest, but does not trigger differentiation. In contrast, a reduction in the TGF-beta concentration, to a range of 0.14-0.20 x 10(-2) ng/ml (which is similar to that measured in TPA-treated cells), mimics TPA-induced differentiation. Taken together, these data demonstrate that cell growth and suppression of differentiation in rhabdomyosarcoma cells require overproduction of active TGF-beta; furthermore, they show that a 'critical' concentration of TGF-beta is necessary for myogenic differentiation to occur, whereas myogenesis is abolished below and above this concentration. By impairing the TGF-beta autocrine loop, TPA stabilizes the factor concentration within the range compatible for differentiation to occur. In contrast, in human primary muscle cells a much higher concentration of exogenous TGF-beta is required for the differentiation inhibitory effect and TPA inhibits differentiation in these cells probably through a TGF-beta independent mechanism. These data thus clarify the mechanism underlying the multiple roles of TGF-beta in the regulation of both the transformed and differentiated phenotype.

Animals↗
PubMed2003

Embryonal rhabdomyosarcoma presenting in an adult: a case report and discussion of immunohistochemical staining.

Embryonal rhabdomyosarcoma is the most common soft tissue sarcoma of childhood but is rarely seen in adults. We report an embryonal rhabdomyosarcoma of the cheek in a 21-year-old Filipino man presenting as a rapidly enlarging mass. An incisional biopsy was consistent with embryonal rhabdomyosarcoma. The patient failed to respond to one cycle of chemotherapy after initial diagnosis and workup for metastatic disease. Wide local excision of the tumor was then completed with the use of the Mohs technique to clear the glabrous cutaneous margins during surgery. A Mohs surgeon and dermatopathologist were involved in interpretation of the Mohs sections. Subsequent immunohistochemical staining revealed a poorly differentiated spindled and epithelioid cell tumor, which stained diffusely positive for vimentin, S-100, and MyoD1. Rare rhabdomyoblasts were present and stained positively for desmin, muscle-specific actin, and phosphotungstic acid hematoxylin. The patient subsequently underwent radiation therapy for a total of 6,000 cGy, followed by two cycles of chemotherapy. The patient continues to be disease-free at 22 months after his wide local excision.

Adult↗
PubMed1996

Primary embryonal rhabdomyosarcoma of long bone. Case report and review of the literature.

We report a case of primary embryonal rhabdomyosarcoma of long bone, presenting as a lytic destructive bone tumor in the right femoral diaphysis of a 7-year-old girl. To our knowledge, this is only the third report of this entity. The neoplasm was a pure embryonal rhabdomyosarcoma with numerous rhabdomyoblasts. Immunohistochemistry confirmed the diagnosis: The cells were reactive with antibodies directed against desmin, muscle-specific actin, and myoglobin. No other neoplastic mesenchymal component was present within the tumor. Although rare, primary rhabdomyosarcoma, along with Ewing's tumor and osteosarcoma, should be considered in the differential diagnosis of malignant bone tumors in childhood.

Actins↗
PubMed1984

The role of surgical resection when combined with chemotherapy and radiation in the management of pelvic rhabdomyosarcoma.

With the development of combined chemotherapy and radiation therapy for embryonal rhabdomyosarcoma, the role and extent of surgical resection of these pelvic tumors need to be defined. Thirty-six children with pelvic genitourinary rhabdomyosarcoma seen at St. Jude Children's were managed on protocols combining surgical resection and radiation, and chemotherapy. Ten children presented with cervical-vaginal tumors, which were managed with combined therapy; the surgical resection was histovaginectomy in eight and pelvic exenteration in one. Eight of the ten are free of disease from 1 to 14 years. Twelve children presented with bladder and prostate tumors, which were resected with segmental cystectomy in four cases, biopsy in five, and pelvic exenteration in three. All received combination therapy and six of the twelve are surviving free of disease from 6 months to 16 years. Fourteen children presented with paratesticular rhabdomyosarcoma. Chemotherapy was combined with radical orchiectomy in all cases. Retroperitoneal node dissection was done in nine and five had inguinal node dissection. Nine of the 14 are surviving NED from 2 to 16 years. One patient died, free of disease, with complications of combination therapy. The results of this review supports the approach of combining chemotherapy, radiation, and complete surgical resection.

Adolescent↗
PubMed1996

Rhabdomyosarcoma and other soft tissue sarcomas in children.

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. During the 1960s, use of surgery and radiation therapy for local control led to survival in fewer than one third of children with this malignancy. Multimodal therapy (chemotherapy, radiation therapy, and surgery), refinements in tumor staging, and better supportive care have significantly improved outcome among children with rhabdomyosarcoma, with long-term survival rates approaching 70%. In contrast, little is known about a heterogeneous group of pediatric soft tissue sarcomas collectively termed nonrhabdomyosarcoma sort tissue sarcomas (NRSTSs). Response of NRSTSs to chemotherapeutic agents has been disappointing, and long-term remissions following chemotherapy are rare. Because the value of adjuvant therapy remains unproven, surgery is the mainstay of therapy for these tumors. This article presents clinical and biological findings of the past year that have significantly contributed to our understanding of rhabdomyosarcoma and NRSTSs.

Child↗
PubMed2000

Rhabdomyosarcoma and soft tissue sarcoma in childhood.

The past year has seen a greater understanding of the means by which the alveolar rhabdomyosarcoma gene fusions (PAX-FKHR) lead to the malignant phenotype. The treatment of the primary tumor in rhabdomyosarcoma has been reexamined, with the roles of surgery and radiation expanding in American studies and decreasing in European ones. "Megatherapy" approaches with stem cell or bone marrow autologous transplants still have not found a role in the treatment of metastatic rhabdomyosarcoma. Our understanding of the natural history of nonrhabdo soft tissue sarcomas in children has increased, and molecular diagnosis is becoming established. The role of chemotherapy in treatment remains controversial.

Child↗
PubMed1998

Primary ovarian rhabdomyosarcoma: a report of 13 cases.

Primary ovarian rhabdomyosarcomas were found in 13 patients aged 7 to 79 (mean 37) years who had reported abdominal pain and swelling. Six tumors involved the right ovary, 3 involved the left, 1 involved both, and the laterality was unknown in 3 cases. Four tumors were stage I, 2 were stage II, 4 were stage III, and 2 were stage IV; the stage of 1 tumor is not known. The tumors ranged from 10 to 19.5 (average 16) cm in diameter and had solid sectioned surfaces that varied from yellow to white to pink and from gelatinous to hemorrhagic. Microscopically, 11 tumors were embryonal and 2 were alveolar rhabdomyosarcomas. Follow-up information, available for 11 patients, revealed that 7 died of disease 10 days to 26 months postoperatively; 2 of these patients had stage II disease, 3 had stage III, and 2 had stage IV. Four patients were alive 2 to 9 months postoperatively; 3 had stage I and 1 had stage III disease. These 13 tumors and an additional 10 from the English-language literature are reviewed and the differential diagnosis of ovarian rhabdomyosarcoma is discussed.

Abdominal Pain↗
PubMed2000

Primary ovarian rhabdomyosarcoma associated with clear cell carcinoma of the ovary: a case report and review of the literature.

The clinicopathologic and immunohistochemical findings of a case of coexistent primary ovarian rhabdomyosarcoma and clear cell carcinoma of the ovary are reported. The tumor was detected in a 41-year-old premenopausal woman who had a 1-year history of pelvic pain. On physical examination, a 14.0-cm left adnexal mass was found for which a hysterectomy with bilateral salpingo-oophorectomy and staging biopsies were performed. The tumor was composed of solid and cystic areas, and two distinct microscopic components were identified: clear cell carcinoma and rhabdomyosarcoma. Immunoperoxidase studies demonstrated that the neoplastic cells in the latter component were positive for desmin, muscle-specific actin, and myoglobin. A biopsy taken from the pelvic peritoneum revealed rhabdomyosarcoma (FIGO stage IIB). Despite two cycles of chemotherapy, the disease persisted in the pelvis 4 months after diagnosis.

Actins↗
PubMed1996

Surgical management of rhabdomyosarcoma in children.

This review of the past year's literature summarizes the most relevant advances in the surgical treatment of rhabdomyosarcomas. Improved responses to multimodality therapy and advances in surgical techniques have allowed for more aggressive surgical resection in some patients and less extensive resections in others. The Intergroup Rhabdomyosarcoma Study committee continues to examine prognostic factors in attempts to provide risk-adapted therapy. Development and evaluation of specific recommendations for surgical treatment by site of tumor continue to be a focus of the Intergroup Rhabdomyosarcoma Study. The overall trend is toward less radical surgical intervention with organ preservation.

Chemotherapy, Adjuvant↗
PubMed1996

Rhabdomyosarcoma presenting as acute leukemia.

PURPOSE: We describe a case of a very unusual presentation of rhabdomyosarcoma. PATIENT AND METHODS: An 18-year-old woman presented with symptoms and signs compatible with acute leukemia. The bone marrow picture showed diffuse involvement sustained by undifferentiated blasts that turned out to be of striated muscle origin by immunochemistry. While it is well known that rhabdomyosarcoma may metastasize to the bone marrow, extensive marrow involvement with leukemic spread as a unique clinical manifestation is extremely rare. CONCLUSION: Our observation further confirms the need to consider rhabdomyosarcoma among the possible differential diagnoses in patients who present with a leukemic picture and atypical blasts lacking all hematopoietic markers.

Acute Disease↗
PubMedDate not supplied

Cerebral metastases of alveolar rhabdomyosarcoma in an infant with multiple skin nodules.

PURPOSE: This report describes extremely rare cases of infantile rhabdomyosarcoma with multiple skin nodules. They are of interest not only for their anatomic sites, but also for subsequent cerebral metastases with sudden cranial hypertension. PATIENTS: Two infants had multiple skin nodules and excisional biopsy revealed alveolar type rhabdomyosarcomas. The patients were treated with tumor resection and combined chemotherapy without any clinical progression for 9 and 16 months, respectively. RESULTS: Evidence of cerebral metastases developed with sudden vomiting and convulsion as the first manifestation. In one patient, urgent radiographic examinations failed to reveal lesions except for dilated cerebral ventricles. Seven weeks after the onset of the neurologic symptoms, only Gd-DPTA-enhanced magnetic resonance imaging (MRI) revealed multiple punctate metastatic lesions hyperintense to the surrounding cerebral tissue. Despite appropriate chemotherapy, both patients had disease progression and died of central nervous system metastases. CONCLUSIONS: The authors emphasize the need to recognize the multiple cutaneous presentation of infantile rhabdomyosarcoma and the association of cerebral metastases as a potential and fatal complication. The diagnosis is facilitated by Gd-DPTA-enhanced MRI, particularly when cerebral computed tomography scans fail to disclose metastatic lesions.

Biopsy↗
PubMedDate not supplied

Chromosomal translocation (1:13) in a case of alveolar rhabdomyosarcoma.

PURPOSE: To describe a patient with a variant translocation (1;13)(p36;q14) in an alveolar rhabdomyosarcoma and compare the clinical course with four other cases. PATIENTS AND METHODS: A 10-year-old girl presented with multiple masses involving the thigh, abdomen, chest wall, and scalp with pleural effusion and edema of the lower extremities. RESULTS: A bone marrow biopsy, aspirate, and biopsy of the thigh mass all showed tumor invasion. Histopathology and cytogenetics of the thigh mass revealed an alveolar rhabdomyosarcoma with a t(1;13)(p36q14) variant. There was no response to aggressive therapy and the patient died within 3 weeks of admission. CONCLUSION: Variant t(1;13)(p36;q14) has now been described in 5 cases of rhabdomyosarcoma, and may define a subset of patients with extensive disease at diagnosis unresponsive to current therapeutic modalities.

Child↗
PubMed2005

Combination of trabectedin and irinotecan is highly effective in a human rhabdomyosarcoma xenograft.

Our objective was to evaluate in vitro and in vivo the effect of the combination of trabectedin (Yondelis, ET-743) and irinotecan (CPT-11) or its major metabolite SN-38 in a human rhabdomyosarcoma cell line. The schedule trabectedin (1 h) followed by irinotecan or SN-38 (24 h) and the opposite sequence (irinotecan or SN-38 24 h followed by trabectedin 1 h) were analyzed in a rhabdomyosarcoma cell line. In vivo studies were conducted with trabectedin and irinotecan at the doses of 0.2 and 20 mg/kg, respectively, simultaneously administered with a q4d x 3 schedule. In vitro studies indicated an overall additive effect [combination index (CI) relatively close to 1.0], with the former schedule slightly superior to the latter (at the IC50 effect levels: CI=0.89 versus 1.07). Neither transcription nor expression of DNA topoisomerase I was affected by trabectedin treatment. In vivo the therapeutic results of the combination were certainly more impressive: trabectedin and irinotecan combination caused a strong and long-lasting effect on tumor growth (tumor volume inhibition=89%, log10 cell kill=1.6), whereas each drug given as a single agent was only marginally active. The discrepancy between the in vitro and in vivo results suggests possible mechanisms involving host cells, other than tumor cells. The striking effects of the combination observed in vivo could be related to a combination of a direct cytotoxic and an anti-inflammatory indirect effect. The very marked and long-lasting effect of the trabectedin and irinotecan combination in vivo suggests a basis for a clinical evaluation in pediatric patients with rhabdomyosarcoma.

Animals↗
PubMed2005

Rhabdomyosarcoma masquerading as acute dacryocystitis.

An 11-year-old boy presented with right medial canthal swelling. He was initially diagnosed with dacryocystitis and treated with oral antibiotics, followed by incision and drainage of a presumed lacrimal sac abscess. Rapid recurrence of the swelling led to further clinical evaluation, including a maxillofacial CT, which revealed an extensive nasal and orbital mass that was consistent with embryonal rhabdomyosarcoma on histopathologic analysis. This represents an unusual case of rhabdomyosarcoma manifesting as acute dacryocystitis. Rhabdomyosarcoma should be considered in the differential diagnosis for acquired nasolacrimal obstruction and dacryocystitis in the pediatric population.

Biopsy↗
PubMed2006

Assessing the use of FDG-PET in the detection of regional and metastatic nodes in alveolar rhabdomyosarcoma of extremities.

Alveolar rhabdomyosarcoma (ARS) accounts for 20% to 30% of childhood rhabdomyosarcoma and is known to have a worse prognosis than embryonal rhabdomyosarcoma. Metastatic disease is more frequent in patients with alveolar tumors and these children with metastatic disease fare poorly, with a 5-year survival between 20% and 30%. Therefore, ARS represents a significant diagnostic and therapeutic challenge that requires techniques to provide better assessment of the disease than provided by traditional means. F18 fluorodeoxyglucose-positron emission tomography (FDG-PET) depicts the increased metabolism in abnormal tissues, enabling accurate evaluation of suspicious regional and metastatic disease. The new combined PET/CT systems can further improve PET interpretation and affect patient management. The value of FDG in patients with soft tissue sarcomas has been demonstrated in several series, but none specifically in ARS. This report assesses the use of FDG-PET/CT in the detection of regional and metastatic nodes in 3 children diagnosed with ARS of the extremities. All the 3 patients we present had focally increased tracer uptake in nodal stations on a pretherapy PET performed at diagnosis. Tissue confirmation available in 2 patients was negative in 1 patient and positive for metastatic nodal spread in the other. Metastatic axillary disease was possibly also present in the third patient according to his later course of disease.

Child, Preschool↗
PubMed1985

An evaluation of enzyme histochemistry in the diagnosis of childhood rhabdomyosarcoma.

Six rhabdomyosarcomas were assessed by means of a battery of enzyme histochemical methods. The reactions were compared with those of a small number of other tumours belonging to the small-cell tumour category. Four of the rhabdomyosarcomas were positive for myophosphorylase and acetylcholinesterase. Myoblasts were strongly reactive for adenosine triphosphatase at alkaline pH and after acid pre-incubation, whereas the small undifferentiated neoplastic cell of the four alveolar rhabdomyosarcomas showed also discernible cytoplasmic reaction, but only after acid pre-incubation. Other tumour categories revealed positive staining for adenosine triphosphatase with acid pre-incubation but the degree of reaction was minimal by comparison. Other enzyme reactions were variable and, generally, did not distinguish between different tumour categories. It is concluded that enzyme histochemistry has a potential role in the diagnostic evaluation of the small cell tumour and should be included in the growing list of special techniques that may assist the pathologist confronted with this problem.

Acetylcholinesterase↗
PubMed1989

Expression of desmin and myoglobin in rhabdomyosarcomas and in developing skeletal muscle.

Immunohistochemical staining for desmin and myoglobin was investigated in 35 rhabdomyosarcomas from young people and in skeletal muscle from 16 human fetuses of known gestational age. Twenty-nine of the rhabdomyosarcomas expressed desmin but six undifferentiated or poorly-differentiated tumours were desmin negative. Of the desmin positive cases, most undifferentiated or poorly-differentiated sarcomas expressed desmin alone (12/35). Tumours with increasing rhabdomyoblastomic differentiation co-expressed myoglobin (9/35) and well-differentiated examples also contained cross-striations (7/35). Skeletal muscle from fetuses aged 8 weeks or less consisted mainly of primitive desmin negative round cells. As the cells began to differentiate they quickly expressed desmin and, at approximately 10 weeks, myoglobin was expressed and cross-striations were seen. The combined results strengthen the view that desmin (within a strictly defined context of round cell tumours in young people) is a reliable marker for rhabdomyoblastic differentiation. Support is also given to the notion that very primitive rhabdomyosarcomas may be desmin-negative, although the difficulties of establishing firm diagnoses for some of these tumours is emphasized.

Adolescent↗
PubMed1989

Clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses.

This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. The closely packed tumour cells were polygonal and arranged in sheets and packets. They had an appreciable amount of clear cytoplasm due to accumulation of glycogen. The diagnosis of rhabdomyosarcoma was confirmed by positive staining for desmin and myoglobin. Rhabdomyosarcoma should be included in the differential diagnosis of nasal clear cell tumours, particularly in young adults. A correct diagnosis is important, because chemotherapy is indicated even for apparently localized disease.

Adult↗