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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors retrospectively analyzed the therapy results in a large group of children affected by rhabdomyosarcoma and treated from 1978 to 1988 with chemotherapy, radiotherapy and surgery. 39/58 patients (67%) achieved a complete response and 25/39 maintained the NED status. An individualized treatment was given to 13 patients who relapsed, but only 3 of them obtained a second response. Those with primary tumor in the orbit or in the genito-urinary tract had lower recurrence rate than patients with head and neck, extremities, pelvis and trunk diseases. Patients in clinical group I and II (according with I.R.S. stadiation) had a significantly better survival than those with more advanced disease (clinical groups III and IV). Relatively to the primary site of the disease, the late effects of combined treatment are mentioned.
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PMID:[The multidisciplinary treatment of rhabdomyosarcoma in childhood. A 10-year multicenter experience (1978-1988) in Liguria]. 769 28

An experimental model for the induction of hepatic metastasis by means of the subcutaneous injection of rhabdomyosarcoma cultured cells (S4MH) is described. The growth of the primary tumor and the dissemination process (local, ganglionar and hematogenous) are studied. The particular ability of the tumor to produce liver metastasis is assessed. The microscopic foci may be found in all of the specimens on day 25th post-injection. The maximum metastatic load is achieved by day 35 th. In contrast, the ganglionar and pulmonary metastasis were only found on the last step of the process.
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PMID:[Development of an experimental model for the study of hepatic metastasis]. 791 65

Rarely, rhabdomyosarcoma can present with bone pain and bone lesions on radiographs without evidence of a primary tumor. Of 428 children with biopsy-proven rhabdomyosarcoma, four presented with radiographic evidence of bone metastases, but no primary tumor was found on subsequent evaluation. On radiographs, these metastases, located most commonly in the metaphyses of the extremities and in the spine, displayed a destructive or diffusely permeative pattern without sclerotic margins and mimicked the more common neuroblastoma. One patient also had diaphyseal cortical lytic metastases of the tibia. Radiographs defined metastases of the extremities better than the correlative bone scans. In the spine, on T2-weighted magnetic resonance (MR) images, metastases displayed high signal intensity which contrasted with the low-signal-intensity marrow in these pediatric patients. On histopathologic examination, metastatic rhabdomyosarcoma was composed of small cells of variable size, shape, and growth pattern similar to other round cell tumors. A positive desmin immunohistochemical test helped to establish the diagnosis. The radiologist, pathologist, and clinician should be aware of this unusual presentation of rhabdomyosarcoma so that suitable immunohistochemical tests are performed and appropriate chemotherapy given.
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PMID:Bone metastases as the presenting manifestation of rhabdomyosarcoma in childhood. 824 17

The pleomorphic subtype of rhabdomyosarcoma (RMS) is now rarely diagnosed in both children and adults. Most cases previously called pleomorphic RMS are probably diagnosed as something else, most often embryonal RMS in children and malignant fibrous histiocytoma in adults. To analyze the concept of pleomorphic RMS in children, we reviewed the tumors of patients entered on the Inter-group Rhabdomyosarcoma Study (IRS I, II, and III). The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the common tumor cell population (anaplastic cells) was selected as the main criterion. Of about 3,000 cases, 110 showed these types of cells, had sufficient histologic material, and had available follow-up data. These tumors were divided into two subgroups: Subgroup I tumors contained only scattered anaplastic cells, and tumors with foci or large sheets of anaplastic cells were classified as subgroup II. Besides the anaplastic-pleomorphic areas, most of these tumors had distinctive features of embryonal RMS (105 cases) and rarely had characteristics of alveolar RMS (five cases). The age distribution of these patients did not differ significantly from those whose tumors did not show the anaplastic features, the average being 6 years and the median 4 years. Lower extremity, retroperitoneum, and the head and neck region were the most common primary tumor sites. The 5-year survival rate was 60% for subgroup I tumors and 45% for subgroup II tumors compared with the survival rate of 68% for 482 IRS II embryonal RMS cases with no anaplastic-pleomorphic features. The lower survival rate for patients in subgroup II was statistically significant (p = 0.004) and similar to the unfavorable survival of patients with alveolar RMS and undifferentiated sarcoma. Because anaplastic cells are seen in many soft tissue sarcomas and in both embryonal and alveolar RMS in children, this feature is not sufficiently unusual to separate a pleomorphic subtype of RMS. The presence of anaplastic cells in aggregates or diffuse sheets throughout the tumor, however, portends a poor survival for these patients.
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PMID:Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study. 847 Jul 59

Even though rhabdomyosarcoma is the most common soft tissue sarcoma in children, accounting for 5-10% of all malignant disease in children under 15 years of age, so few cases are seen in a single institution that only the combined efforts of multicentre prospective trials made it possible fro adequate treatment schedules to be devised. Thank to this cooperation, survival rates have increased dramatically in recent decades; risk factors have been identified and treatment can now be adapted accordingly. This is especially true for the paratesticular rhabdomyosarcoma (PTRM), which now has a good prognosis in all stages. The striking similarity of tumor behavior and metastatic pathways to those in germ-cell tumors in young male adults can provide us with more extensive data derived from a much larger group of patients. Recent data are gathered and evaluated in this review. Only in this way will it be possible to eliminate all treatment modalities known to be followed by severe sequelae, thus avoiding exposure of the patients to a therapy that carries more risks than the primary tumor itself.
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PMID:Paratesticular rhabdomyosarcoma. 852 95

Metastasis to the breast is uncommon, with an incidence of 0.5-3%. Alveolar soft part sarcoma is rare, accounting for < 1% of malignant soft tissue tumors, which are themselves unusual. Excluding contralateral breast and hematologic malignant disease, the primary lesion in most cases of metastasis to the breast is melanoma, small cell carcinoma of the lung, or ovarian carcinoma, although rhabdomyosarcoma is the most common primary tumor in children. We describe a 26-year-old woman with no history of malignant disease who presented with two masses in the right breast that clinical evaluations and ultrasonography indicated were fibroadenomas. Pathological studies after excisional biopsy, however, indicated alveolar soft part sarcoma. Subsequent computed tomography showed the primary tumor in the anterior left thigh and multiple bilateral lung metastases. Because of the presence of distant metastases, the patient was treated with chemotherapy.
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PMID:Alveolar soft part sarcoma metastatic to the breast. 860 50

Staging systems are used in staging most pediatric solid tumors outside the central nervous system. Common solid, nonneurologic pediatric tumors include liver tumors, Hodgkin disease, non-Hodgkin lymphoma, Wilms tumor, rhabdomyosarcoma, neuroblastoma, Ewing sarcoma, and osteosarcoma. Traditional staging of pediatric tumors depends on the anatomic distribution of the malignant disease. Almost all staging systems are based on the spread of the local primary tumor, metastasis to regional lymph nodes, and distant blood-borne metastatic spread. There is some variability as to how tumor spread is assessed. Such assessment may be performed before or after surgery. There are many potential problems with tumor staging systems. The systems vary in complexity and clinical usefulness, and there is some variation in the criteria used in the different systems. It is important for radiologists to have a sound working knowledge of staging systems to facilitate accurate staging. Imaging is an important aspect of every staging system.
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PMID:Visual presentation of the staging of pediatric solid tumors. 889 21

The risk of second malignancy after retinoblastoma is reported to be as high as 20% at 10 years after initial diagnosis. This incidence may be an overestimate because of difficulties in distinguishing a second malignancy from recurrent tumor. We encountered a patient with bilateral retinoblastoma who developed a temporal mass 3.5 years after initial treatment for what had first been diagnosed as rhabdomyosarcoma; further study suggested that it was recurrent retinoblastoma manifesting as primitive neuroectodermal tumor (PNET) with multilineage differentiation. Chromosome 13 abnormalities were compatible with either rhabdomyosarcoma or recurrent retinoblastoma. To determine how often second malignancies in retinoblastoma patients may be confused with recurrent primary tumor, we reviewed our experience at Children's Hospital of Pittsburgh. Of 43 retinoblastoma patients seen between 1951 and 1992, presumed second malignancies were documented in four, including the current case. Of the three other second tumors, one had both neural and skeletal muscle differentiation; another was diagnosed as rhabdomyosarcoma unclassifiable as embryonal or alveolar; the last was an osteosarcoma. Only the osteosarcoma was clearly a second neoplasm; two and perhaps three of the other cases may be recurrent retinoblastoma. The distinction between second malignancy and recurrent retinoblastoma may be difficult but is worth determining, because treatment may differ, depending on the correct designation.
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PMID:Malignancy after retinoblastoma: secondary cancer or recurrence? 902 3

Three germline mutations in the TP53 tumor-suppressor gene are reported, two of which are not reported previously. A missense mutation at codon 265 of TP53 was found in three patients of a family that complied with the definition of the Li-Fraumeni syndrome. A nonsense mutation in codon 306 was found in a woman who had had a rhabdomyosarcoma at age 4 and a subsequent breast cancer at age 22. She was part of a Li-Fraumeni-like family, but the parental origin of the mutation could not be traced. Finally, while screening for somatic alterations in TP53 in a series of 141 sporadic breast tumors, we detected a constitutional missense mutation in codon 235 in a woman diagnosed with breast cancer at age 26 and a recurrence 4 years later. The recurrence, but not the primary tumor, showed an additional missense mutation at codon 245 as well as loss of the wild-type allele. This suggests that the 245 mutation was particularly important for tumor progression and that there might exist heterogeneity in terms of cancer predisposition potential among the various germline TP53 mutations.
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PMID:Three germline mutations in the TP53 gene. 906 56

The treatment of six patients with advanced-stage or high-risk rhabdomyosarcoma (RMS) is described. These patients were treated with a delayed-accelerated hyperfractionated radiation therapy (DAHFRT) regimen which delivers 5200 cGy over 20 treatment days. Acceptable early toxicity was noted when radiation therapy was given after a full course of chemotherapy and major attempts at resection of the primary tumor. The DAHFRT regimen has inherent biological and time-intensity advantages compared to other fractionation schemes which may be exploited to improve local control. The DAHFRT regimen should be considered as an alternate fractionation scheme for RMS patients and a possible foundation from which dose-escalation of radiation therapy may be attempted using advanced treatment planning technology. Late effects of high-dose radiation therapy, although a major concern, should assume less priority given the high local failure rates of advanced-stage patients and the advent of conformed radiation therapy treatment planning and delivery which can be used to reduce treatment-related toxicity.
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PMID:Delayed-accelerated hyperfractionated radiation therapy for advanced-stage or high-risk rhabdomyosarcoma. 914 6


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