Rhabdomyosarcoma study

Raney RB, Asmar L, Newton WA, Bagwell Ch, Bren-eman JC, Crist W et al. Ewing s sarcoma of soft tissues in childhood A report from the Intergroup rhabdomyosarcoma study, 1972-1991. J Cfin Oncol 1997 15 574-82. 

Ortega, J.A., Donaldson, S.S., Ivy, S.P., Pappo, A., Maurer, HJM. Venoocclusive disease of the liver after chemotherapy with vincristine acti-nomycin D, and cyclophosphamide for the treatment of rhabdomyosarcoma a report of the intergroup rhabdomyosarcoma study group. Cancer 1997 79 2435-2439... 

Parham DM, Webber B, Holt H, et al. Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasms Results of an Intergroup Rhabdomyosarcoma Study project. Cancer. 1991 67 3072-3080. 

Kodet R, Newton Jr WA, Sachs N, et al. Rhabdoid tumors of soft tissues A clinicopathologic study of 26 cases enrolled on the Intergroup Rhabdomyosarcoma Study. Hum Pathol. 1991 22 674-684. 

Mauer HM, Beltangady M, Gehan EA, et al. The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer. 1988 61(2) 209-220. 

Raney RB, Anderson JR, Barr FG, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol. 2001 23 215. 

Newton Jr WA, Gehan EA, Webber BL, et al. Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification—an Intergroup Rhabdomyosarcoma Study. Cancer. 1995 76 1073-1085. 

Qualman SJ, Coffin CM, Newton WA, et al. Intergroup Rhabdomyosarcoma Study update for pathologists. Pediatr Dev Pathol. 1998 1 550-561. 

Kodet R, Newton Jr WA, Hamoudi AB, et al. Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study. Am J Surg Pathol. 1993 17 443-453. 

Studies of the metabolism of vincristine and vinblastine have been complicated by chemical alterations of the drugs that occur during processing of samples for analysis. Extraction of these compounds under acidic conditions has been reported to minimize chemical degradation, and HPLC studies of the metabolism of vincristine indicate that microsomal preparations from mouse liver, but not those from human rhabdomyosarcoma tissue, convert vinblastine to 4-deacetyl vinblastine in vitro (38). 

Rhabdomyosarcomas developed in rats injected intramuscularly with the powder of either pure cobalt metal or cobalt oxide. In other studies implantation of cobalt caused local fibrosarcomas in rabbits, but inhalation studies in hamsters did not reveal any increase in tumors from cobalt oxide. Lifetime exposure to cobalt sulfate by inhalation resulted in increased incidence of alveolar/bronchiolar neoplasms and a spectrum of inflammatory, flbrotic, and proliferative lesions in the respiratory tract of male and female rats and mice. ... 

Chronic animal studies have also yielded varying results. Intratracheal implantation of lead chromates in rats failed to significantly increase the carcinogenic response after 2 years. Intrapleural administration caused a 9% incidence of lung tumors in rats within 19-21 months. Intramuscular injection resulted in lymphomas, renal tumors, fibrosarcomas, and rhabdomyosarcomas at the site of injection in rats. ... 

Chronic 2-year studies showed a significant increase in the incidences of adenomas and carcinomas of the nasal cavity in high-dose rats fed diets containing 3000ppm of 2,6-xylidine. The carcinomas were highly invasive and frequently destroyed the nasal turbinates and nasal septum. Rhabdomyosarcomas, a rare tumor of the nasal cavity were also observed in the high-dose male and females. The nonneo-plastic lesions observed in the nasal cavity included acute inflammation, epithelial hyperplasia, and squamous metaplasia. In addition, subcutaneous fibromas and fibrosarcomas occurred in both males and females and there was an increased incidence of neoplastic nodules in the livers of female rats. 

In another study, Heston (1953) fonnd that snbcntaneous injections of HD (0.05 cc of a 0.05% solution at weekly intervals for 6 weeks, or 0.1 cc of a 0.1% solntion in olive oil at 2-day intervals for a total of 6 doses) into the mid-dorsal region of mice (strains A, C3H, and C3Hf) resulted in injection-site tumors, whereas injections of vehicle alone did not indnce tnmor formation. Tumors occurring at the injection site included sarcomas, sarcomas neurogenic in origin, a rhabdomyosarcoma, papillomas, a squamous cell carcinoma, a hemangioendothelioma, and a mammary carcinoma. 

In an ongoing phase I evaluation of datelliptium (384), there have been observed no significant toxic effects (dry mouth, hemolysis, and hypotension) (289). The dose-limiting toxicity is local phlebitis (220 mg/m ). However, no antitumor response has been noticed thus far in the study. In another phase I trial of this new drug, similar low toxicity was observed (290). Hepatic toxicity was the only acute dose-limiting toxicity. Thus far, of 12 patients evaluated, there has been 1 minor response in a patient with metastatic cervical carcinoma and the stabilization of a patient with metastatic resistant rhabdomyosarcoma. 

Folpe and associates were the first to study FEI-1 immu-noreactivity in human endothelial neoplasms.They observed a 94% marker sensitivity in that specific context and found no labeling of nonvascular tumors. However, more recently, Mhawech-Fauceglia and colleagues have documented FLI-1 positivity in some carcinomas, lymphomas, and rhabdomyosarcomas as well. In aggregate, these data support the use of FLI-1 as an adjuvant to the diagnoses of PNET or vascular neoplasms, but only in combination with other immunoreactants. 

FIGURE 4.10 The solid form of primitive embryonal or alveolar rhabdomyosarcoma represents a prototypical small round cell malignancy for which several adjunctive studies are necessary. 

Dystrophin, the protein product of the Duchenne muscular dystrophy locus, is a major cytoskeleton protein in skeletal muscle cells. There have been only limited studies on the use of this marker in the diagnosis of rhabdomyosarcoma. Dystrophin was found in most cases of rhabdomyosarcoma in frozen sections, and was lacking in other small cell neoplasms including lymphoma, PNET, and Wilms tumor. 

FIGURE 4.30 Pleomorphic rhabdomyosarcoma, shown here, is often indistinguishable from MFH on conventional morphologic studies. 

Dodd S, Malone M, McCulloch W. Rhabdomyosarcoma in children A histological and immunohistochemical study of 59 cases. J Pathol. 1989 158 13-18. 

Gruchala A, Niezabitowski A, Wasilewska A, et al. Rhabdomyosarcoma Morphologic, immunohistochemical, and DNA study. Gen Diagn Pathol. 1997 142 175-184. 

Coindre JM, deMascarel A, Trojani M, et al. Immrmohisto-chemical study of rhabdomyosarcoma Unexpected staining with SlOO protein and cytokeratin. J Pathol. 1988 155 127-132. 

Seidal T, Kindblom LG, Angervall L. Myoglobin, desmin, and vimentin in ultrastructurally proven rhabdomyomas and rhabdomyosarcomas An immunohistochemical study utilizing a series of monoclonal and polyclonal antibodies. Appl Pathol. 1987 5 201-219. 

Tsokos M, Howard R, Costa J. Immunohistochemical study of alveolar and embryonal rhabdomyosarcoma. Lab Invest. 1983 48 148-155. 

Cessna MH, Zhou, H, Perkins, SL, et al. Are myogenin and myoDl expression specific for rhabdomyosarcoma A study of 150 cases, with emphasis on spindle cell mimics. Am Surg Pathol 1150 25(9) 1150-1157. 

Kumar S, Perlman E, Harris CA, et al. Myogenin is a specific marker for rhabdomyosarcoma an immunohistochemical study in paraffin-embedded tissues. Mod Pathol. 2000 13 988-993. 

Parham DM. Pathologic classification of rhabdomyosarcomas and correlations with molecular studies. Mod Pathol. 2001 14 506-514. 

Scupham R, Gilbert EF, Wilde J, Wiedrich TA. Immrmohisto-chemical studies of rhabdomyosarcoma. Arch Pathol Lab Med. 1986 110 818-821. 

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