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)

We present an unusual case of primary osseous pleomorphic rhabdomyosarcoma with focal matrix formation mimicking osteosarcoma. The patient was a 21-year-old man who had suffered from pain and slight enlargement of his left calf for 2 months. A plain radiograph demonstrated a large, predominantly osteolytic mass in the region of the proximal fibula with features typical of malignant primary bone tumor. On open surgical biopsy, the tumor consisted of atypical cells, some of them presenting spindle morphology. Between them, there were bands of densely hyalinized matrix with osteoid appearance, but without definite lacunae or calcifications, and an osteosarcoma was diagnosed. Consequently, the tumor was removed. The postoperative tissue presented more pleomorphic cells with some definite rhabdomyoblasts. Desmin, actin, Myf4, and MyoD1 were positive in tumor cells, and a diagnosis of rhabdomyosarcoma was eventually made. Only few cases of primary pure bone rhabdomyosarcoma have been reported. Other bone tumors with rhabdomyosarcomatous differentiation have been described: dedifferentiated chondrosarcoma, fibrosarcoma, and osteosarcoma. Our case does not meet the criteria for sclerosing rhabdomyosarcoma, as matrix formation is focal and cells are spindle-shaped and pleomorphic. However, it is a further example of a diagnostic error in connection with primary osseous tumor.
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PMID:Primary osseous rhabdomyosarcoma with focal matrix formation mimicking osteosarcoma. 1790 23

We describe herein a rare case of primary rhabdomyosarcoma (RMS) occurring in the sacrum. A 16-year-old woman presented with a 2-month history of pain in bilateral buttocks and posterior thighs. Computed tomography showed a primary tumor with bone destruction in the 2nd sacral vertebra and invasion to the 1st to 3rd vertebrae and retroperitoneal space. Histological examination of the tumor showed proliferation of spindle-shaped cells intermingled with rhabdomyoblasts in a fascicular and storiform growth pattern. Tumor cells showed immunoreactivity for vimentin, desmin, muscle-specific actin, sarcomeric actin, alpha-smooth muscle actin and CD99, and partial immunoreactivity for myoD1, myf-4, myogenin and myoglobin. Reverse transcription polymerase chain reaction demonstrated expression of myoD1. On the basis of the aforementioned findings, a poorly differentiated spindle cell variant of embryonal RMS was diagnosed. The patient underwent combined therapy with chemotherapy and radiotherapy, but died 17 months after incisional biopsy. The present case is instructive in differential diagnosis of primary bone tumors, and the possibility of skeletal RMS needs to be considered.
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PMID:Primary rhabdomyosarcoma of the sacrum: a case report and review of the literature. 1842 56

Rhabdomyosarcoma is exceedingly rare in adults. A 62-year-old woman was referred to our hospital because of general pain. Computed tomography revealed a solid tumor in the right nasal cavity. Histopathological examination showed solid proliferation of atypical small round cells, having cytologic features reminiscent of lymphomas, and lacking the fibrovascular stroma. The cells were CD56(+), desmin(+), vimentin(+), HHF35(+), myogenin(+) and MyoD1(+). The patient was positive for the PAX3-FKHR fusion gene. The patient was diagnosed as having alveolar rhabdomyosarcoma. We conclude that rhabdomyosarcoma should be included in the differential diagnoses of CD56(+) small round cell tumor, and immunohistochemical and cytogenetic studies should be performed.
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PMID:Alveolar rhabdomyosarcoma mimicking nasal lymphoma at the initial presentation. 1903 98

Sarcoma of the mammary glands is extremely rare. Liposarcoma, leiomyosarcoma, rhabdomyosarcoma, as well as hemangiopericytoma, are part of the soft tissue sarcoma group. Little is known about the progress, prognosis and dissemination of this infrequent tumor entity. We present the case of a woman, who received primary diagnosis of a malignant hemangiopericytoma of the left breast. She underwent a mastectomy with an axillary lymph node sampling (stage pT3 pN0 cM0), as adjuvant therapy was not mandatory. Eight months after diagnosis, the patient presented with lumbar back pain, gluteal pain and right accentuated adynamia in both legs because of a disseminated osseous metastasis. Diagnostic investigation presented a cerebral, pulmonary, cutaneous, hepatic and pleural metastatic disease. Two months after initiation of chemotherapy the patient died. Diagnostic criteria and treatment principles in the metastatic situation are presented in addition to the literature to give a review about this rare malignancy.
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PMID:Metastasized hemangiopericytoma of the breast: a rare case. 1916 99

Hypertrophic osteoarthropathy (HOA) is characterized by digital clubbing, long bone periosteal reaction, and polyarthralgias. Primary familial HOA is very rare and is not associated with underlying disorders and has a good prognosis. Secondary pediatric nonneoplastic HOA is associated with cystic fibrosis, congenital heart disease, biliary atresia, and inflammatory bowel disease. Secondary neoplastic HOA may be associated with intra or extrathoracic tumors.A 5-year-old girl was admitted to our hospital for an abdominal mass, digital clubbing, and diffuse articular pain. The bone scan revealed symmetrical tracer uptake in the long bones. Upper and lower extremity x-rays were diagnostic for HOA. Paraneoplastic HOA in childhood accounts for not more than 12% of HOA paitents. HOA has been reported in 2 other cases of rhabdomyosarcoma.
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PMID:Rhabdomyosarcoma associated hypertrophic osteoarthropathy in a child: detection by bone scintigraphy. 1935 78

We report a case of alveolar rhabdomyosarcoma arising between the fourth and fifth metatarsal. A 13-year-old boy presented to outpatients with a history of pain and swelling in the lateral aspect of his left forefoot. Plain radiographs and MRI showed a soft tissue mass displacing the fourth metatarsal. Percutaneous biopsy revealed an alveolar rhabdomyosarcoma. Staging scans showed advanced metastatic disease. The patient was treated with chemotherapy. This highly malignant lesion remains challenging to diagnose, and difficult to treat successfully.
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PMID:Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review. 2065 99

Seven children with sciatic neuropathy associated with an underlying neoplasm are reported. Clinical presentation, electrophysiological data, imaging, pathology, and/or autopsy results are described. Pain and weakness, primarily foot drop, were the most common presenting symptoms. The mechanism of sciatic neuropathy was varied and included: nerve infiltration by the adjacent neoplasm (neuroblastoma, rhabdomyosarcoma, and leukemic or lymphomatous infiltration); an expanding, intrinsic neurogenic tumor (perineurioma); or intraoperative stretch injury (osteosarcoma resection). The prognosis for sciatic nerve recovery was good among children who survived their associated cancer. Three children died from the cancer or complications of treatment. One child with perineurioma remained clinically stable, and two children improved after treatment of their neoplasm.
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PMID:Pediatric sciatic neuropathy associated with neoplasms. 2125 82

Exophthalmus is the leading sign of space-occupying lesions of the orbit. Patients may further present with lid swelling, impaired ocular motility and optic neuropathy including a relative afferent pupillary defect, compressive optic disc edema or optic atrophy. Orbital tumors can be classified into various categories depending on the etiology, as lymphoproliferative lesions (in particular non-Hodgkin's lymphoma as the most common malignant orbital tumor of adulthood), optic nerve and meningeal lesions, lacrimal gland lesions, secondary orbital tumors which extend to the orbit from neighboring structures and metastases. Slightly less common are vasculogenic and cystic lesions including cavernous hemangioma as the most common benign orbital tumor of adulthood and dermoid cysts as the most common benign orbital tumor of childhood. Rhabdomyosarcoma is the most common malignant orbital tumor of childhood but has a low total incidence. Orbital tumors might not only cause symptoms like pain, diplopia and loss of visual acuity but may also lead to esthetically disfiguring changes. Particular attention should be paid to underlying systemic diseases and generalized tumor diseases. This article illustrates the approach to a detailed clinical and neuroradiological assessment which is mandatory for the care of orbital tumor patients.
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PMID:[Clinical and neuroradiological diagnostics of orbital tumors]. 2169 5

A 69-year-old woman presented with a 4-month history of dyspnoea and radiating upper-right quadrant pain and oedema in her lower extremities for more than 20 days. The ultrasonographic study of the heart revealed the adherence of a substantive hypoechoic mass (73 x 34 mm) to the antelateral wall of the pulmonary artery and resultant pulmonary stenosis. Computed tomographic imaging of the pulmonary artery revealed an irregularly shaped filling defect (approximately 41 x 39 x 59 mm) in the main pulmonary artery. The boundary of the defect was irregular, but demarcation with healthy tissue was clear. After surgical treatment, the histologic and immunohistochemical assays revealed a primary pulmonary artery rhabdomyosarcoma. Pulmonary artery rhabdomyosarcomas are usually misdiagnosed as other pulmonary artery obstructive diseases. There should be a greater focus of clinical attention and resection is the appropriate surgical treatment for such malignant tumours.
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PMID:Primary pulmonary artery rhabdomyosarcoma. 2174 14

The intrathecal infusion of drugs to provide analgesia for terminally ill children with refractory pain is a rarely utilized but very effective technique. A number of pharmacological agents, most commonly opioids and local anesthetics, have been administered intrathecally for this purpose. However, tachyphylaxis and neuraxial opioid-related side effects can limit their utility. The alpha-2 agonist clonidine is commonly used to augment local anesthetic techniques for postsurgical pain in children and for the management of refractory cancer pain in adults, but there is only a single report of the use of clonidine intrathecally in a terminally ill child. We present the case of the youngest reported child to have received intrathecal analgesia for terminal care: a 3-year-old boy with advanced pelvic rhabdomyosarcoma, whose refractory pain was managed effectively with an intrathecal infusion of bupivacaine and preservative-free clonidine.
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PMID:Intrathecal infusion of bupivacaine and clonidine provides effective analgesia in a terminally ill child. 2188 97


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