1/18. A new human pleomorphic rhabdomyosarcoma cell-line, HS-RMS-1, exhibiting MyoD1 and myogenin.A number of human cell lines derived from alveolar or embryonal rhabdomyosarcoma (RMS) have been described. To our knowledge, however, no cell line established from pleomorphic RMS has been reported. We describe here the establishment and characterization of a new human cell line, HS-RMS-1, which originated from a typical pleomorphic RMS arising in the gluteal muscle of a 26-year-old man. HS-RMS-1 cells had pseudotetraploid complex karyotypes with no specific abnormalities. Both in vitro and in vivo the cells on light microscopic examination exhibited pleomorphic features with immunopositive reaction for myogenic antigens including MyoD1 and myogenin, although no Z band-like structures were detected electron-microscopically. RT-PCR demonstrated the expression of MyoD1 and myogenin in HS-RMS-1 cells at the mRNA level, and direct sequencing analysis revealed cDNAs of MyoD1 and myogenin identical to those previously reported. This cell line, HS-RMS-1, established from pleomophic RMS will be useful for further studies including the molecular aspects of human RMS.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/18. Solid variant of alveolar rhabdomyosarcoma with unbalanced t(2;13) and hypotetraploidy, without MYCN amplification.The histological subtype of alveolar rhabdomyosarcoma (AR) is characterised by the cytogenetic translocation t(2;13)(q35;q14) in approximately 70% of cases, a rearrangement rarely present in the embryonal rhabdomyosarcoma (ER) subtype. The MYCN gene is amplified in some cases of AR. We present a young man with an unusual pattern, namely solid variant of AR with hypotetraploidy and the t(2;13) in an unbalanced form. The MYCN gene was not amplified on FISH, but showed increased copy number, consistent with ploidy.- - - - - - - - - - ranking = 5keywords = alveolar (Clic here for more details about this article) |
3/18. rhabdomyosarcoma in children: report of two cases.rhabdomyosarcoma of soft tissue is the commonest sarcoma in pediatric medicine and comprises 15% of the solid tumors treated in pediatric oncology. It has an ample variety of clinical presentations in diverse organs and also presents a gamut of histopathological patterns, which sometimes make diagnosis difficult. We report two cases of rhabdomyosarcoma: one of the embryonic histological variety and the second one of the alveolar variety, which presented themselves in the preauricular and masseteric areas respectively. Both patients were male, diagnosed and treated.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
4/18. Localized thigh swelling mimicking a neoplastic process: involvement of coxsackie virus type A21.A 4-y-old girl was admitted because of a left leg limp with an isolated swollen upper thigh and normal muscle enzymes. A radioisotope scan showed increased uptake especially in the bone and soft tissue of the left thigh, while magnetic resonance imaging of that region demonstrated widespread oedema in striated muscle. On muscle biopsy perivascular infiltrates were demonstrated but muscle fibres were not shown to be affected. sequence analysis of reverse transcription-polymerase chain reaction amplified fragments from the 5'-non-coding region of human enteroviruses identified a local strain of coxsackie virus A21 in the muscle. Clinical symptomatology subsided with oral steroids. CONCLUSION: Local swelling mimicking a neoplasm may be related to infestation of coxsackie virus in muscle tissue.- - - - - - - - - - ranking = 0.0036282327791184keywords = process (Clic here for more details about this article) |
5/18. Intramuscular dendritic fibromyxolipoma: myxoid variant of spindle cell lipoma?Dendritic fibromyxolipoma (DFML) is an uncommon, recently described, benign soft tissue lesion that shares many clinical and pathological features with myxoid variants of spindle cell lipoma (SCL). As described, DFML is distinguished from SCL by the presence of dendritic cytoplasmic processes, abundant keloidal collagen and a prominent, often plexiform vascular pattern. We describe the first known reported case of an intramuscular DFML that occurred in the right shoulder region of a 73-year-old man. The tumor displayed the typical histopathological features of DFML but also included foci of chondroid metaplasia, a previously unreported finding. This report also discusses the differential diagnosis, particularly distinguishing DFML from SCL and myxoid liposarcoma. In view of the similarities in many clinical and pathological features between SCL and DFML, we speculate that DFML probably represents an unusual variant of myxoid SCL.- - - - - - - - - - ranking = 0.00090705819477961keywords = process (Clic here for more details about this article) |
6/18. Primary perineal alveolar rhabdomyosarcoma metastatic to an extraocular muscle.A 17-year-old boy diagnosed with an alveolar rhabdomyosarcoma involving the perineum and with extensive lymphadenopathy was treated with chemotherapy yet developed metastases to the head and neck 6 months into therapy. Ten months after initial diagnosis, while receiving salvage chemotherapy and radiotherapy, he returned with pain on movement of his left eye, proptosis, and ptosis of the left upper eyelid. Computed tomography (CT) revealed a mass within the left lateral rectus muscle that biopsy confirmed to be metastatic alveolar rhabdomyosarcoma. Despite continued chemotherapy and radiotherapy, he ultimately died of the disease. Alveolar rhabdomyosarcoma from distant sites rarely metastasizes to the extraocular muscles. However, our case shows that alveolar rhabdomyosarcoma may metastasize to the orbit and involve a single muscle.- - - - - - - - - - ranking = 7keywords = alveolar (Clic here for more details about this article) |
7/18. Case report: myxoma of the temporalis muscle.Intramuscular myxomas of the head and neck are rare entities. Primarily found in the myocardium, these lesions also affect the bones and soft tissues. We present a case of an intramuscular myxoma of the temporalis muscle. After reviewing the literature, this case constitutes, to our knowledge, the second reported occurrence of a myxoma in this specific location. The patient was treated for this condition with an excisional biopsy after fine needle aspiration revealed a benign process. No recurrence was seen 18 months after the excision.- - - - - - - - - - ranking = 0.00090705819477961keywords = process (Clic here for more details about this article) |
8/18. computer-aided design and fabrication of an electron bolus for treatment of the paraspinal muscles.PURPOSE: Demonstrate the technology for the design, fabrication, and verification of an electron bolus used in the preoperative irradiation of a mesenchymal chondrosarcoma in the paraspinal muscle region (T8-T12), in which the target volume overlay a portion of the spinal cord, both lungs, and the right kidney. methods AND MATERIALS: An electron-bolus design algorithm implemented on a three dimensional (3D) radiotherapy treatment planning system designed the bolus to yield a dose distribution that met physician-specified clinical criteria. Electron doses were calculated using a 3D electron pencil-beam dose algorithm. A computer-driven milling machine fabricated the bolus from modeling wax, machining both the patient surface and the beam surface of the bolus. Verification of the bolus fabrication was achieved by repeating the patient's computed tomography (CT) scan with the fabricated bolus in place (directly on the posterior surface of the prone patient) and then recalculating the patient's dose distribution using the 3D radiotherapy treatment planning system. RESULTS: A treatment plan using a 17-MeV posterior electron field with a bolus delivered a superior dose distribution to the patient than did the same plan without a bolus. The bolus plan delivered a slightly increased dose to the target volume as a result of a slightly broader range of doses. There were significant reductions in dose to critical structures (cord, lungs, and kidney) in the bolus plan, as evidenced by dose-volume histograms (DVHs). The patient dose distribution, calculated using CT scan data with the fabricated bolus, showed no significant differences from the planned dose distribution. CONCLUSIONS: A bolus can provide considerable sparing of normal tissues when using a posterior electron beam to irradiate the paraspinal muscles. Bolus design and fabrication using the tools described in this paper are adequate for patient treatment. CT imaging of the patient with the bolus in place followed by calculation of the patient's dose distribution demonstrated a useful method for verification of the bolus design and fabrication process.- - - - - - - - - - ranking = 0.00090705819477961keywords = process (Clic here for more details about this article) |
9/18. Successful treatment for solitary brain metastasis from alveolar soft part sarcoma.Following pulmonary metastases, alveolar soft part sarcoma has an unexplained predisposition to metastasize to the brain. Herein, a case of alveolar soft part sarcoma solitarily metastatic to the brain is described. A 23-year old female underwent the resection of alveolar soft part sarcoma from the right thigh. Three years after the surgery, a brain tumor was resected and pathology proved it was a metastatic alveolar soft part sarcoma. She is currently alive and well 6 years after brain surgery. Our report suggests that surgical resection for solitary brain metastasis from alveolar soft part sarcoma is an effective treatment modality.- - - - - - - - - - ranking = 9keywords = alveolar (Clic here for more details about this article) |
10/18. Myxoid liposarcoma with cartilaginous differentiation.Common histologic variants of liposarcoma are readily recognized. Histologic classification might be difficult, however, when liposarcoma demonstrates cartilaginous differentiation. Although this phenomenon has previously been noted, it has not, to our knowledge, been reported as a specific pathologic entity. All three patients in the present study were men, ages 37, 42, and 63 years. Each presented with a solitary, enlarging mass of the thigh that was surgically excised. The tumors ranged in size from 8 to 13 cm. Microscopically, each lesion displayed characteristic features of myxoid liposarcoma; in addition, all possessed discrete, rounded foci of mature-appearing hyaline cartilage. One case displayed small foci showing chondrocyte atypia. No other patterns of mesenchymal differentiation were present. All patients received postoperative radiation therapy. No recurrences or metastases have been identified (mean duration, 39 mo). Myxoid liposarcomas with cartilaginous differentiation are of importance because they might be misdiagnosed as malignant mesenchymoma. The latter, if not qualified as to histologic grade, might be presumed to be a biologically more aggressive lesion. In addition, these lesions must be distinguished from two benign processes: chondroid lipoma and extraskeletal chondroma with lipoblast-like cells. Additional studies of this uncommon variant of liposarcoma will be necessary to document further its status as a low-grade sarcoma.- - - - - - - - - - ranking = 0.00090705819477961keywords = process (Clic here for more details about this article) |
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