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 consultants agreed that the differential diagnosis should focus on congenital masses, including an encephalocele, glioma, dermoid, hamartoma, hemangioma, rhabdomyosarcoma, neurofibroma, and nasolacrimal duct cyst. There was some disagreement as to which is the best way to evaluate the mass, ranging from an MRI (Dr. Reilly), to CT scan (Dr. Cotton), to both MRI and CT (Dr. Koopman). Blood tests to evaluate pituitary function could be indicated if there was a sphenoid defect (Dr. Reilly). None of the experts would biopsy this lesion. All would proceed with a definitive resection. One surgeon would defer surgery for several months and then perform the resection via a biocoronal craniotomy (Dr. Reilly). A combined anterior craniotomy and external ethmoidectomy would be planned by another (Dr. Koopman). The third consultant would combine an anterior craniotomy with a mid-face degloving, external rhinoplasty, or lateral rhinotomy approach (Dr. Cotton). Routine perioperative antibiotics would only be used by two of the surgeons (Drs. Reilly and Koopman). If a CSF leak were encountered there are several options. A small lesion could be allowed to close on its own (Dr. Reilly). If the leak occurred while the bicoronal incision was still open or if the leak were large, it could be repaired from above (Drs. Reilly and Koopman). One surgeon would proceed with a repair from above even if the leak were encountered during the intranasal approach (Dr. Cotton). Only one surgeon would restrict postoperative activity with intubation and sedation or paralysis (Dr. Koopman). Regarding follow-up, no one was concerned about the final pathology report.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nasal mass in a pediatric patient. 139 78

A series of 1,682 cases in the service of the Department of Pathology, Ramathibodi Hospital from 1977 to 1986 was studied and classified according to WHO histologic typing of soft tissue tumors. The findings revealed that the three most common benign soft tissue tumors were lipoma (50.6%), vascular tumors (17.4%) and neurofibroma (7.3%), whereas, the three most common malignant soft tissue tumors were unclassified sarcoma (16.5%), rhabdomyosarcoma (15.5%) and neurofibrosarcoma (10.7%). Lipoma occurred at any age group with a mean age of 40 years and affected males and females equally and common sites of involvement were neck (19.7%), back (19.7%) and upper arm (18.4%). Unclassified sarcoma was the most common among the malignant group accounting for 16.5 per cent of the total malignant soft tissue tumors. The age ranged from 7 to 70 years with a mean age of 27.4 years. The most common sites of involvement were the extremities (50%) especially the thigh.
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PMID:Soft tissue tumors in Ramathibodi Hospital: study of 1,682 cases during 1977-1986. 140 58

Sixty-three pure mesenchymal tumors of the uterus were studied to explore the value of immunostaining in the diagnosis of unusual mesenchymal tumors encountered in the uterus, some not reported previously. Each tumor was evaluated using a panel of immunostains including actin, desmin, vimentin, S-100 protein, and cytokeratin. The final classification, which incorporated the immunohistochemical findings, resulted in the identification of 33 relatively common pure mesenchymal tumors (13 benign and malignant endometrial stromal tumors and 20 benign and malignant smooth muscle tumors) and 30 uncommon tumors (five leiomyosarcomas with osteoclastic giant cells, two xanthomatous leiomyosarcomas, one melanotic schwannoma, one pure rhabdomyosarcoma, one neurofibroma, five plexiform tumorlets, and 15 combined smooth muscle-stromal tumors). The normal endometrial stroma, present in 14 cases, invariably showed a negative reaction for all antibodies. With rare exceptions, the pure endometrial stromal tumors displayed a negative immunoreaction for all antibodies utilized, while the pure smooth muscle tumors consistently showed a positive reaction for actin. Only the two tumors of neural origin (a neurofibroma and a melanotic schwannoma) reacted with S-100 protein. Immunostaining influenced most the final classification of neoplasms initially interpreted as uterine tumors with a sex-cord stromal pattern, endometrial stromal tumors that diverged from the classic lesions by having a spindle cell component, and intravascular leiomyomas with areas of compact proliferation of small round cells with prominent vascularity. All tumors in these three groups were reclassified as combined smooth muscle-stromal tumors following immunohistochemical studies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Immunohistochemistry as a diagnostic aid in the interpretation of unusual mesenchymal tumors of the uterus. 171 Aug 7

The distribution of the beta-subunit of platelet-derived growth factor receptor (PDGFR-beta) was assessed by a sensitive immunoalkaline phosphatase technique using the monoclonal antibody PR7212. Frozen tissue sections of several nonneoplastic human tissues were stained along with 42 soft tissue sarcomas, 16 benign soft tissue proliferations, and 7 epithelial tumors. In all nonneoplastic tissue, there was intense labeling of cell processes of perivascular fibroblasts or pericytes in and about the walls of muscular blood vessels and of fibroblast cell processes around some glandular and ductal epithelia. No PDGFR-beta was found in the endothelial cells of muscular arteries and veins, but cells of uncertain identity within some capillaries were immunoreactive and the possibility that endothelial cells of some small capillaries express PDGFR-beta could not be excluded. In kidney there was strong labeling of glomerular mesangial cells and interstitial fibroblasts. Some histological types of soft tissue sarcomas were uniformly and strongly labeled with anti-PDGFR-beta, but other types were infrequently labeled or unreactive. The order of decreasing frequency and strength of labeling of the various types of benign and malignant soft tissue proliferations was as follows: benign fibromatosis and neurofibroma greater than malignant fibrous histiocytoma greater than liposarcoma greater than leiomyosarcoma greater than rhabdomyosarcoma. No tumor cell labeling was detected in epithelioid, synovial or clear cell sarcomas, leiomyomas, or carcinomas, but there was usually strong labeling of fibroblast and/or pericyte cell processes within tumor, especially around blood vessels. We conclude that PDGFR-beta is strongly expressed by vascular and stromal tissues of most tumors and normal organs and by tumor cells of several types of soft tissue tumors and proliferations, most notably those of fibroblastic origin.
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PMID:In situ distribution of the beta-subunit of platelet-derived growth factor receptor in nonneoplastic tissue and in soft tissue tumors. 216 45

The monoclonal antibody 5.1 H11 recognizes an antigen on human fetal muscle and on rhabdomyosarcoma cell lines and xenografts that has been shown to be homologous to the neural cell adhesion molecule. To evaluate its range of expression, we used immunoperoxidase staining of fresh frozen-tissue sections to determine monoclonal antibody 5.1 H11 reactivity in normal and neoplastic tissue. Among normal tissue specimens, intense antibody staining was seen in brain and peripheral nerve, and weaker staining in ganglionic elements of colon. In addition to 26 of 29 rhabdomyosarcoma specimens, 5.1 H11 antibody showed reactivity to 9 of 10 Wilms' tumors, 6 of 6 neural tumors, and 4 of 4 gliomas, and with single specimens of ectomesenchymoma, clear-cell sarcoma of kidney, undifferentiated sarcoma of liver, ovarian fibroma, and neurofibroma. We conclude that the monoclonal antibody 5.1 H11 recognizes an antigen present not only on fetal muscle but on normal brain and nerve as well. In addition to rhabdomyosarcoma, a variety of other tumors, most of which have been previously shown to express neural cell adhesion molecule, also appear to express the antigen recognized by 5.1 H11. Our results thus offer additional confirmatory evidence that an epitope of neural cell adhesion molecule is the antigen for 5.1 H11.
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PMID:Expression of the 5.1 H11 antigen, a fetal muscle surface antigen, in normal and neoplastic tissue. 218 73

The most common ocular and orbital tumors presenting in infancy, childhood and adolescence are presented and discussed in this review. It has been prepared specifically for the clinical pediatrician and focuses on the clinical recognition of ophthalmic neoplasms, their diagnostic evaluation employing the use of advanced imaging techniques, biopsy when indicated and extent of disease workup. In addition, current treatment modalities are discussed. Ocular tumors addressed include: retinoblastoma, capillary hemangioma, lymphangioma, dermoid and epidermoid cysts, teratoma, glioma, astrocytic hamartoma, neurofibroma, rhabdomyosarcoma and fibrous tumors. Two aggressive and potentially fatal tumors, rhabdomyosarcoma and retinoblastoma, are presented in detail. In addition, the ocular tumors associated with the phakomatoses (von Hippel-Lindau, tuberous sclerosis and neurofibromatosis) are reviewed.
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PMID:Ophthalmic neoplasms in infancy and childhood. 219 81

Identification of growth factors and receptors in mesenchymal tumors may be crucial to understanding of growth regulation in sarcomas. During an immunohistochemical study of the expression of growth factors and receptors in human soft tissue tumors (STT), only 1 antisera capable of working in paraffin-embedded tissue was noted. A detailed study of 141 STT was undertaken to determine the frequency of expression of nerve growth factor receptor (NGF-R), its specificity and sensitivity for neural tumors, and the effect of fixation on detection. In normal mesenchymal tissue, only nerve sheath and perivascular staining was seen. No immunoreactivity was seen in many tumors including rhabdomyosarcoma, angiosarcoma, liposarcoma, Ewing's sarcoma, and alveolar soft part sarcoma. Less than 15% of tumors of smooth muscle, fibrous, or fibrohistiocytic origin showed immunoreactivity, usually focal. In contrast, a high frequency of immunoreactivity was noted in tumors of neural origin (74%). This included granular cell tumors (100%), Schwannoma/neurofibroma (91%), malignant Schwannoma (78%), neuroblastoma/neuroepithelioma (60%), and paraganglioma (57%). A high rate of reactivity was also seen in synovial sarcomas (80%), undifferentiated sarcomas (60%), and hemangiopericytomas (43%), suggesting a potential relationship to the neural phenotype. Among the neural tumors, Bouin's fixation was superior to formalin, suggesting that immunoreactivity for NGF-R is affected by fixation. This antibody may be a useful adjunct marker diagnostically.
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PMID:Expression of nerve growth factor receptor in paraffin-embedded soft tissue tumors. 245 20

A 54-year-old white man had a slowly growing painless epibulbar mass that clinically mimicked a lymphangioma. Morphologically, the paucicellular tumor contained stellate and spindly cells, mast cells, and dilated lymphatic channels embedded in a loose collagenous matrix. The clinical differential diagnosis included lymphangioma, amelanotic nevus, lymphoma, reactive lymphoid hyperplasia, dermoid, lipoma, and botryoid rhabdomyosarcoma. Pathologically, lymphangioma, myxoid neurofibroma, and spindle cell lipoma were all considered. The authors discuss the clinical and histopathologic features of the various tumors, and confirmation of the diagnosis of conjunctival myxoma by differential alcian blue staining properties dependent on critical electrolyte concentration.
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PMID:Conjunctival myxoma. A clinicopathologic study. 667 44

Primary retroperitoneal tumors are rare (0.05-0.2% of all tumors), often malignant and characterized by a poor and non-specific symptomatology and by a late diagnosis. Complete resection is possible in only a few patients, while recurrence is very common. The records of 29 adult patients who underwent operative treatment at Surgical Oncology Institute-University of Cagliari between November 1973 and June 1992 were reviewed; 9 were males, 20 females, median age 46.4 years (range 12-82). There were 4 benign tumors (13.8%) and 25 malignant (86.2%). Fibrosarcoma (9 cases, 31%) and liposarcoma (3 cases, 10.3%) were the most frequent histologic types. There were also two fibroleiomyomas, leiomyosarcomas, malignant fibrous histiocytomas and neuroblastomas, one case of fibroma, neurofibroma, rhabdomyosarcoma and schwannoma. Five sarcoma were not otherwise specified. Abdominal mass (25 cases, 86.2%), flank or abdominal pain (15 cases, 51.7%) and weight loss (8 cases, 27.6%) were most common symptoms; change in bowel habit and constipation (6 cases, 20.7%), fever (5 cases, 17.2%), urinary disorders (4 cases, 13.8%) nausea and vomiting (2 cases, 6.7%) were less common. Diagnosis was made by ultrasonography, computed tomography and traditional radiographic studies. median interval between first symptoms and diagnosis was 11 months. Complete surgical resection was possible in only 13 cases (46.4%): 10 of the 25 malignant tumors (40%) and 3 of the 4 benign tumors (75%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Primary retroperitoneal tumors. Our experience]. 751 58

Fibrous orbital tumors present clinically and radiographically in a broad spectrum ranging from a benign mass, to locally aggressive tumor, to invasive malignancy. Pathologic analysis and diagnosis are often challenging, usually based on a combination of light microscopy, immunohistochemistry, and electron microscopic findings. Some light microscopic and immunohistochemical findings, however, are relatively characteristic. A storiform or cartwheel pattern and vimentin staining are characteristic of fibrous histiocytoma. A herringbone pattern is usually found in fibrosarcoma. A "patternless pattern" and CD34 staining is found most commonly in solitary fibrous tumor. CT and MR imaging findings, as well as clinical presentation, in fibrous orbital lesions are often difficult to distinguish from those of other orbital masses, although there may be useful clues. Benign fibrous lesions are usually well-circumscribed and may chronically remodel bone, whereas more aggressive malignant fibrous tumors tend to have infiltrating margins and may destroy bone on CT or MR imaging. With malignant fibrous masses, enhancement patterns on CT or MR imaging may be more inhomogeneous, with avascular or necrotic nonenhancing regions. At MR imaging, benign lesions tend to be homogeneous on T1, T2, and postgadolinium T1-weighted images, whereas malignant soft tissue lesions may change their pattern from homogeneous on T1-weighted images to heterogeneous with low signal septations on T2-weighted images. Low T2 signal comprising part or all of a fibrous lesion correlates with dense collagen fibers, with a less cellular matrix. Areas of hyperintensity on T2-weighted images correspond with a more cellular matrix of fibroblasts and other cells. Calcification within a tumor, however, may give a similar appearance. Thus, if a lesion has predominantly low signal on T2-weighted images, or less specifically has low signal septations, then a fibrous orbital lesion with high collagen content may be ranked higher in the differential diagnosis (see Figs. 2E and 3B). When T2 signal is intermediate-to-high, then one has a difficult time narrowing the differential diagnosis. Radiographically, distinguishing these lesions from other fibrous orbital lesions, as well as from other varieties of orbital masses, is difficult. Differential diagnosis of fibrous orbital masses includes all the fibrous lesions described in this article, in addition to schwannoma (Fig. 7), neurofibroma (Figs. 4 and 8), hemangiopericytoma (Figs. 9 and 10), rhabdomyosarcoma, meningioma, lymphoma, and metastasis (Figs. 11 and 12). A history of prior orbital irradiation for retinoblastoma or other tumors may raise the possibility of radiation-induced secondary tumors, such as MFH, fibrosarcoma, and osteosarcoma. Determining the extent of orbital involvement remains the primary goal of the radiologist. The final diagnosis still rests with the pathologist.
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PMID:Fibrous histiocytoma and fibrous tissue tumors of the orbit. 1002 37


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