Sarcoma rhabdomyosarcoma

Dactinomycin is used in curative combined treatment of Wilms tumor, Ewing s sarcoma, rhabdomyosarcoma, and gestational choriocarcinoma. It is active in testicular tumors, lymphomas, melanomas, and sarcomas, although its use in most of these malignancies has been supplanted by other agents. 

Wilms tumor, Ewing s sarcoma, rhabdomyosarcoma, gestational choriocarcinoma, testicular tumors, lymphomas, melanomas Breast, ovarian, endometrial, bladder, thyroid cancers oat cell cancer of the lung Testicular, ovarian germ cell cancers, small-cell lung cancer, acute myelogenous and lymphoblastic leukemia... 

Dactinomycin Cosmegen Carcinoma of testicle and endometrium carcinosarcoma of kidney [Wilms tumor] Ewing sarcoma rhabdomyosarcoma Blood disorders [leukopenia, thrombocytopenia, others] Gl distress [nausea, vomiting, loss of appetite] mucocutaneous lesions skin disorders [rash, hair loss] local irritation at injection site... 

Clear cell sarcoma, angiosarcoma, adrenal oncocytoma, Kaposi s sarcoma, rhabdomyosarcoma... 

Figure 70 The Sarcoma (rhabdomyosarcoma) Cells Use the Sirtuin Longevity Proteins of the Yeast for the Prolongation of Their Life Span. Reference Appendix 2, Explanations to the Figures...

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. 

Childhood Acute lymphocytic leukemia Burkitt s sarcoma Ewing s sarcoma Retinoblastoma Rhabdomyosarcoma Wilms tumor... 

The treatment of Hodgkin s disease also illustrates the use of combined modalities, that is, radiation plus chemotherapy. The combined modality approach to several childhood tumors (e.g., Ewing s sarcoma, Wilms tumor, and rhabdomyosarcoma) has dramatically increased the cure rates for these diseases. 

Cyclophosphamide Same as above Breast cancer, ovarian cancer, non-Flodgkin s lymphoma, CLL, soft tissue sarcoma, neuroblastoma, Wilms tumor, rhabdomyosarcoma Nausea and vomiting ... 

Vincristine has been effectively combined with prednisone for remission induction in acute lymphoblastic leukemia in children. It is also active in various hematologicmalignancies such as Hodgkin s and non-Hodgkin s lymphomas, and multiple myeloma, and in several pediatric tumors including rhabdomyosarcoma, neuroblastoma, Ewing s sarcoma, and Wilms tumor. 

Dactinomycin is mainly used to treat pediatric tumors such as Wilms tumor, rhabdomyosarcoma, and Ewing s sarcoma, but it has activity also against germ cell tumors and gestational trophoblastic disease. Dactinomycin can also induce a "radiation recall reaction." See Table 55 1 for other toxi cities. 

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. 

Ifosfamide is an alkylating agent belonging to the group of oxazaphosphorines. It is used to treat a variety of solid tumors in children, including rhabdomyosarcoma, soft tissue sarcomas, Wilms tumor, bone sarcomas, and neuroblastoma, and leukemias and lymphomas in adults. It is also sometimes used in combination with other drugs, such as doxorubicin or cisplatin and etoposide. 

KER, keratin, detected by a mixture of GAMS.2, MAK-6, and AEl /AE3 EMA, epithelial membrane antigen VIM, vimentin DES, desmin MSA, muscle-specific actin SMA, smooth muscle (alpha isoform) actin GALD, h-caldesmon S-IOOP, S-100 protein OCN, osteocalcin LM, laminin UL, Ulex europaeus I lectin binding FS, fibrosarcoma SGRMS, spindle cell rhabdomyosarcoma LMS, leiomyosarcoma MPNST, malignant peripheral nerve sheath tumor MSS, monophasic spindle cell synovial sarcoma SCAS, spindle cell angiosarcoma KS, Kaposi s sarcoma FOS, fibroblastic osteosarcoma. 

Leiomyosarcomas are most commonly found in the retroperitoneum in adults. They uncommonly occur in the deep soft tissues of the extremities but may be seen in more superficial sites, particularly in the dermis and subcutis. The differential diagnosis of LMS traditionally includes other sarcomas composed of intersecting spindle-cell fascicles, including fibrosarcoma, MPNST, synovial sarcoma, and spindle cell rhabdomyosarcoma. Additional conditions such as IMT (inflammatory pseudotumor), neurofibroma, and hemangiopericytoma (see Fig. 4.5) are also considerations. 

Soft tissue sarcomas with a potentially pleomorphic histologic appearance (Fig. 4.28 see Table 4.7) include so-called MFH (undifferentiated pleomorphic sarcoma), pleomorphic rhabdomyosarcoma, pleomorphic or dedifferentiated liposarcoma, dedifferentiated LMS, and pleomorphic MPNST. 

Foschini MP, Eusein V. Alveolar soft part sarcoma A new type of rhabdomyosarcoma Semin Diagn Pathol. 1994 4 58-68. 

Many of the tumors of the nasal cavity and paranasal sinuses fall under the category of round cell neoplasms. Among these are olfactory neuroblastoma, sinonasal undifferentiated carcinoma, malignant melanoma, neuroendocrine carcinoma-small cell neuroendocrine carcinoma, malignant lymphoma, extramedullary plasmacytoma, invasive-ectopic pituitary adenoma, rhabdomyosarcoma, and Ewing s sarcoma (ES)-peripheral neuroectodermal tumor (ES/ PNET). But there is also a host of other epithelial lesions that are unique to the sinonasal tract. 

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of children and, in this age group, approximately one third of all RMSs occur in the head and neck, particularly involving the orbit, middle ear-mastoid, nasopharynx, and sinonasal tract. Ti jjj contrast, RMS is uncommon in adults, especially in the head and necb. TT tumor does involve... 

Neuroblastomas are small, round, blue cell tumors that may arise in the adrenal gland and a variety of extra-adrenal sites. The differential diagnosis is wide and includes rhabdomyosarcoma, Ewing s sarcoma-primitive neuroectodermal tumor (ES-PNET), medulloblastoma, small cell osteosarcoma, lymphoblastic lymphoma, blastematous Wilms tumor, and small cell desmoplastic tumor. Numerous markers have been used for the diagnosis of neuroblastomas including NE markers, cytoskeletal proteins, catecholamine-synthesizing enzymes, and neuroblastoma-"specific antibodies (Eig. 

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. 

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