Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The observation presented is based on one patient 18 years old who presents a paratesticular rhabdomyosarcoma stage I. A radical orchidectomy through on inguinal incision with high ligation of the spermatic cord was performed. We have omitted retroperitoneal lymph node dissection. The patient was treated by postoperative chemotherapy with V.A.C. during five weeks. Our patient remains disease free one year post-surgery, but after that, he presents suddenly a symptomatic bone metastasis of the thigh-bone and a massive metastatic pulmonary spreading, without retroperitoneal lymph nodes on the CT-scan. A multidisciplinary approach has considerably improved the prognosis of this tumor. Some reports suggest that routine retroperitoneal lymphadenectomy may be unnecessary for patients with no evidence of nodal involvement on CT-scan. The recent literature insists on problems cause by retroperitoneal lymph node involvement, but our clinical caused must induce us not to underestimate the potential of hematogenous spreading of this tumor.
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PMID:[Paratesticular rhabdomyosarcoma]. 797 54

Between 1985 and 1990, 10 cases of embryonal paratesticular rhabdomyosarcoma were treated at our institution. Patient age ranged from 5 months to 16 years at presentation (mean 8.7 years). Chest and abdominal computerized tomography (CT) was performed on all patients. Lymphangiography was performed on 2 patients, and 6 underwent retroperitoneal lymphadenectomy, due to suspicious CT (2) and a positive lymphangiogram (1). All 6 patients had pathologically negative nodes, and they received vincristine, actinomycin D and cyclophosphamide for 8 to 13 months (mean 10.6 months). Four patients also received doxorubicin. The 10 patients are alive and the disease is in complete remission for a mean 6.02-year disease-free survival. These data support the hypothesis that retroperitoneal lymphadenectomy can be avoided for paratesticular rhabdomyosarcoma after radical inguinal orchiectomy when CT is negative for nodal involvement.
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PMID:The role of retroperitoneal lymphadenectomy in localized paratesticular rhabdomyosarcoma. 802 14

Angiomatoid "malignant" fibrous histiocytoma (AMFH) has been considered to be a low-grade sarcoma of childhood, and, with its fibrous pseudocapsule, angiomatoid change, dense lymphoplasmacytic response, and proliferation of spindled or round cells, has been classified as a fibrohistiocytic neoplasm. We wanted to study the clinicopathologic and immunophenotypic features of a large number of these tumors and to especially further explore their myoid differentiation. Cases coded as AMFH from 1979 to 1995 were retrieved from the Soft Tissue Registry of the AFIP. Only cases that met the criteria for AMFH by light microscopy were included, a total of 158 cases. Immunohistochemistry was obtained on 98 cases. Clinical history on 92% of all cases revealed a gender ratio of 1.3 females: males, age range of 2 to 71 years, median size of 2.0 cm, and a distribution of extremities > trunk > head and neck, with 66% lesions occurring in areas of normal lymphoid tissue. All tumors with available margins were well-circumscribed. Eighty percent of cases had some degree of lymphoplasmacytic infiltration; 50% cases had pseudovascular spaces filled with blood. Fifty-two percent had predominantly round cell morphology; 48% had a predominantly spindle cell pattern. Desmin positivity was noted in 51% cases and occurred in both predominantly round cell and spindle cell tumors. Most of the desmin-positive cases with adjacent lymphoid infiltrate (67%) showed scattered similar, desmin-positive cells in the surrounding lymphoid infiltrate, adjacent to the tumor. Muscle-specific and smooth-muscle actins were seen in 14% cases. Heavy-caldesmon was strongly positive in 3%, and calponin was focally positive in 73% and extensively positive in 12% cases. MyoD1, myoglobin, and myogenin (myf4) were negative in all tumors studied. Forty-five percent of cases were positive for CD99; 52% of these had round cell morphology. Fifteen percent of cases were positive for KP-1. All tumors were positive for vimentin and negative for CD21, CD35, S100 protein, CD34, keratins 8/18, and lysozyme. Clinical follow-up on 86 patients indicated that only 1 patient was alive with a local nodal metastasis (1% frequency of metastasis) within 1 year, and 2 others had local recurrence, all over a mean follow-up period of 6 years. The myoid, primarily myofibroblastic, phenotype of these lesions is supported by desmin, calponin, and occasional actin positivity. The occasional heavy-caldesmon and smooth muscle actin additionally suggest rare smooth muscle phenotype; however, lack of skeletal muscle markers indicate no relationship of AMFH to skeletal muscle tumors. The resemblance of these lesions to lymph nodes, clinically and morphologically, the finding of similar desmin positive cells in the adjacent lymphoid infiltrate, and the fact that 66% cases were found in sites of normal lymphoid tissue raise the possibility that some of these lesions may arise from or be related to myoid cells of lymphoid tissue. AMFH has an almost invariably benign behavior, but the 1% metastatic rate warrants its classification as low-grade "malignant." The predominantly round cell, CD99-positive and desmin positive AMFH cases, respectively, should not be confused with Ewing's sarcoma/PNET or rhabdomyosarcoma, respectively.
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PMID:Angiomatoid "malignant" fibrous histiocytoma: a clinicopathologic study of 158 cases and further exploration of the myoid phenotype. 1057 14

The three major categories of nonmammary malignancies of the breast include primary and secondary lymphoreticular malignancy, primary and secondary sarcoma, and hematogenous metastasis. This article describes the imaging features of 35 nonmammary malignancies of the breast and axilla with histopathologic confirmation. These include primary and secondary breast lymphoma, primary axillary nodal lymphoma, metastatic acute lymphatic leukemia, metastatic plasmacytoma, granulocytic sarcoma, primary angiosarcoma, metastatic rhabdomyosarcoma, hematogenous metastasis from primary lung, ovarian, cervical, thyroid, and colonic carcinoma, malignant melanoma, carcinoma of the nasal cavity, and adenocarcinoma of unknown primary. Wherever possible, correlation between mammography and ultrasound, computed tomography (CT), and/or magnetic resonance (MR) imaging is made.
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PMID:Nonmammary malignancies of the breast: ultrasound, CT, and MRI. 1107 18

The management of pediatric soft-tissue sarcomas has improved drastically through the use of multimodal therapy. These tumors include rhabdomyosarcomas and nonrhabdomyosarcomas. Both are staged using physical, radiographic, and histologic evaluation, and both have intricate staging and grouping systems that correlate closely with prognosis. However, approaches to therapy for the two tumor types remain somewhat different. Rhabdomyosarcomas are treated primarily with chemotherapy. Surgical intervention is limited to initial biopsy, wide local excision when clear margins are feasible, and resection of residual disease. Radiation therapy is reserved for patients with persistent or recurrent disease and may be delivered by external beam or brachytherapy. Nonrhabdomyosarcomas are best treated primarily by surgical resection, although radiation and chemotherapy are now being used with some success. Another major difference concerns evaluation of lymphatics. Nonrhabdomyosarcomas in children frequently behave similarly to adult sarcomas, and less commonly involve regional lymph nodes, whereas pediatric patients with rhabdomyosarcomas often have nodal involvement necessitating surgical evaluation of regional lymph nodes as part of the staging protocol. Multimodal therapy has led to improved survival as well as better functional and cosmetic results. With further clinical trials and improved techniques such as brachytherapy and lymphatic mapping with sentinel node biopsy, we expect to continue to optimize therapy for pediatric patients with soft-tissue sarcomas.
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PMID:Multidisciplinary management of pediatric soft-tissue sarcoma. 1109 12

Brachytherapy has been widely used at the Institut Gustave-Roussy since 1972 in pediatric oncology. In genitourinary rhabdomyosarcoma, because of its ballistic and physical characteristics, it represents the optimal treatment whenever irradiation is required and brachytherapy feasible. Between 1976 and 1998, 23 children with bladder or prostate rhabdomyosarcoma were treated with a protocol including brachytherapy, with five of them treated with a salvage brachytherapy. All but one brachytherapy was performed during the surgery. Among the 18 brachytherapies performed as a first-line treatment, eight presented a tumoral evolution: five presented a local evolution, one a local and nodal evolution and two a nodal evolution. Brachytherapy allowed a conservative treatment among ten out of 11 children alive with no evidence of disease. Among the five patients with salvage brachytherapy, two presented a second recurrence. Sequelae were minimal, consisting of one grade I rectitis and one asymptomatic vesical and ureteral reflux. These results are consistent with the published data using more radical treatment. Brachytherapy can represent an alternative to radical surgery, when indications are clearly defined in bladder or prostate rhabdomyosarcoma. This type of treatment can be performed only integrated with other treatments, more particularly with surgery. This approach requires a close cooperation between the different specialists: pediatricians, surgeons and brachytherapists.
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PMID:[Brachytherapy in the treatment of vesicoprostatic rhabdomyosarcomas in children]. 1119 54

Multimodal therapy, involving surgery, chemotherapy and radiation, now dominates the management of rhabdomyosarcoma (RMS) in childhood. Yet, despite improvements in these practices, extremity tumors continue to fare relatively poorly. Several investigators have identified prognostic factors that can be used to direct therapy and predict outcome. These factors include histology and metastatic disease, the latter requiring accurate staging to identify. The presence of lymph node metastases has been shown to be of prognostic significance and is incorporated into pre-treatment staging schemes. Up to 50% of all surgically evaluated nodes and 17% of clinically negative nodes in extremity RMS may harbor tumor, underscoring the increased risk of understaging the disease if accurate lymph node dissection is not undertaken. Despite its importance, there appears to be no standard format by which regional nodal status is evaluated in extremity RMS. Sentinel lymph node mapping and biopsy are a minimally invasive technique, currently used in the staging of adult breast cancer and melanoma. In adults, the technique is associated with optimum nodal yield and low morbidity. We describe a case in which sentinel node mapping and dissection were used to easily and accurately stage a distal upper extremity alveolar RMS in a child with clinically and radiologically negative regional lymph nodes. The procedure yielded no positive nodes, was associated with no morbidity and spared the child more extensive radiotherapy. We propose the further evaluation of this simple and innovative technique in the overall management of this childhood malignancy.
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PMID:Sentinel lymph node mapping and biopsy: a potentially valuable tool in the management of childhood extremity rhabdomyosarcoma. 1274 Jul 6

The study was a result of joint studies conducted by the Academician N. N. Burdenko Research Institute of Neurosurgery, Russian Academy of Medical Sciences, and the P. A. Herzen Moscow Cancer Research Institute, Ministry of Health of the Russian Federation, in 1996 to 2001. During this period, the joint team operated on 32 patients with malignant craniofacial tumors. Of them there were 18 males and 14 females whose age was 8 to 74 years. Most patients were aged 36 to 60 years. Epithelial tumors were predominant in the histological pattern of 18 (56%) patients. Squamous-cell (8 patients) and adenoid cystic (4 patients) carcinomas were prevalent among the epithelial tumors and rhabdomyosarcoma (4 patients) was prevalent among the mesenchymal tumors. Craniofacial block-resection was one of the stages of complex treatment in patients with malignant tumors of the base of the skull. Malignant craniofacial tumors are prone to local spread and rarely give rise to both regional and distal metastases. This is the basis for the concept that the patient may be cured via block removal of a tumor with a portion of its adjacent intact tissues in a combination with pre- and/or postoperative multidrug and radiation therapies. There were intraoperative and postoperative fatal cases. The signs of tumor remnants were absent, as evidenced by computed tomography and magnetic resonance imaging (MRI) performed in all cases. Further control MRIs were performed every 3 months during 2 years, then every 6 months, Complications as graft rejection were observed in 2 cases: on day 2 after surgery in one case and on day 6 in the other. They were not a cause of liquorrhea and meningitis due to the effectiveness of a pedicle periosteal graft that restricts the dura matter. Repeated microsurgical operations using other types of grafts. The patients were followed up for 3 to 5 years. During this period, 12 (37.5%) patients died of dissemination in the period of 1 month to 2 years; 1 (3.1%) patient died of the causes unassociated with the underlying pathological process. In 7 (21.8%) patients, the relapse-free was 5 years or more; of them there were 5 patients with epithelial tumors, 1 with a mesenchymal tumor, and 1 with a malignant chondroid tumor. In these patients, cosmetic corrective operations were made by plastic surgeons. Twelve patients, including 5 with epithelial tumors, 4 with mesenchymal tumors, and 3 with chondroid ones, were operated on for recurrences. Seven patients underwent lymphadenectomy due to the cervical lymph nodal metastases. Nineteen (59.4%) and 17 (53.1%) patients survived > or = 3 and > or = 5 years, respectively.
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PMID:[Craniofacial block-resections for malignant tumors of the skull base]. 1532 60

For patients with soft tissue sarcoma in an extremity, the outcome is thought to be poor if lymph node metastasis develops. The purpose of this study was to examine the impact of lymphatic involvement from soft tissue sarcoma on patient survival. Thirty-nine (3.7%) of 1066 patients who had surgery for soft tissue sarcoma in an extremity had lymph node metastases develop. Three (20%) of 15 patients with epithelioid sarcoma, four (19%) of 21 patients with rhabdomyosarcoma, two (11.1%) of 18 patients with clear cell sarcoma, and two (11.1%) of 18 patients with angiosarcoma had lymphatic involvement. Thirty patients who had resection of involved lymph nodes had an estimated 5-year survival of 57%, whereas nine patients treated without surgery all died within 30 months. An estimated 4-year survival of 71% for patients with isolated lymph node metastases was significantly better than 21% for patients with synchronous systemic and lymph node involvement. There was no difference in outcome for patients with isolated lymphatic involvement compared with patients with American Joint Committee on Cancer Stage III extremity sarcomas. These results suggest that long-term survival is possible after surgical resection of lymphatic metastases from soft tissue sarcoma. The American Joint Committee on Cancer should consider separating isolated nodal metastases from systemic involvement in patients with Stage IV sarcoma.
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PMID:Lymph node metastasis in soft tissue sarcoma in an extremity. 1534 63

A 40-yr-old woman with an asymptomatic sinonasal rhabdomyosarcoma (RMS) initially presented with submental nodal metastasis. The fine-needle aspiration (FNA) and the subsequent biopsy of the nodal metastasis were misinterpreted as metastatic carcinoma because the primary tumor was occult, the tumor cells were exclusively round cells with a nested arrangement, and rhabdomyoblasts were absent. The correct diagnosis of metastatic RMS became apparent when the primary sinonasal tumor, detected in a CT, was biopsy proven to be an alveolar RMS. Retrospectively, there were helpful clues to the correct diagnosis in the nodal FNA and biopsy. When FNA cytology or biopsy histology of a lymph node suggests metastatic carcinoma but the tumor cells are nonimmunoreactive to carcinoma markers, the differential diagnosis should be expanded to include not only metastatic melanoma but also metastatic sarcoma and lymphoma. Cytologically, the presence of multinucleated giant tumor cells, including the rosette forms, in the FNA smears should alert the cytopathologist to the possibilities of sarcoma and anaplastic large cell lymphoma.
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PMID:Rhabdomyosarcoma in an adult presenting with nodal metastasis: a pitfall in fine-needle aspiration cytology of lymph nodes. 1583 Mar 59


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