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)

Fourteen cases of neoplasms metastatic to the ovaries in children 10 weeks to 15 years of age are reported. Eight tumors were neuroblastomas, 7 primary in the adrenal gland, and 1 primary in the posterior mediastinum. Three tumors were rhabdomyosarcomas primary in the ethmoid sinus, right occipital region, and left thigh, respectively. The final three tumors were a Ewing's sarcoma, a rhabdoid tumor, and a carcinoid tumor primary in the fibula, kidney, and lung, respectively. The ovarian involvement was an autopsy finding in nine of the patients. Three of the remaining five patients presented clinically with manifestations suggesting a primary ovarian tumor, and the final two patients, who had known extraovarian primary tumors, had symptomatic ovarian masses discovered during life. In one case of neuroblastoma and the case of rhabdoid tumor, the ovarian metastases were initially misinterpreted pathologically as primary ovarian cancers; the primary renal tumor was not discovered until autopsy in the latter case. The ovarian tumors were bilateral in 8 of the 14 cases. Ovarian enlargement was present in 10 cases. Our experience and that in the literature indicates that the childhood tumor that spreads to the ovary most frequently is the neuroblastoma and that rhabdomyosarcoma is the most common sarcoma of childhood that spreads to the ovary. The clinical features and the frequent bilaterality of ovarian metastatic tumors are helpful diagnostic features in many cases, but when the ovarian tumor is the presenting manifestation of the disease, is unilateral, or both, differentiation from various primary ovarian tumors may be difficult.
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PMID:Metastatic ovarian tumors in children: a report of 14 cases and review of the literature. 841 81

The objective of this study was to assess the diagnostic strategy in identifying occult primary carcinomas in metastatic bone disease. The records of 29 patients seen between 1983 and 1993 were reviewed retrospectively. The patients had been evaluated nonuniformly with a wide array of diagnostic procedures. The primary tumor was identified in 22 patients antemortem, and in 2 patients postmortem. In 5 patients a diagnosis of the primary tumor was never obtained. Eleven of the primary tumors were carcinomas of the lung, 3 were prostatic carcinomas, 2 were carcinomas of the breast and 2 were malignant lymphomas. In 4 patients respectively a carcinoma of the kidney, the pancreas, the ovary or the stomach was found. One patient had a carcinoid tumor of the small intestine, and one had a retroperitoneal rhabdomyosarcoma. The average survival time after clinical onset of skeletal metastases was 12 months. We propose a flow sheet to evaluate this category of patients including a medical history, thorough physical examination, routine laboratory tests, chest radiography, bone scintigram, ultrasonography or computed tomography of the abdomen and biopsy of the most accessible metastasis. These tests allowed premortem diagnosis of the primary tumor in 75% of the cases. Biopsy should be considered the last test to be performed.
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PMID:Skeletal metastases of unknown origin: a retrospective analysis of 29 cases. 915 54

Heart neoplasms are of increasing interest among clinicians and surgeons. A review of primary malignant cardiac tumors, secondary cardiac tumors and carcinoid heart tumors is presented, with special reference to their pathological and surgical aspects. Primary malignant heart tumors represent about 25% of all cardiac tumors, the great majority are sarcomas and the whole family of this group is described including angiosarcoma, rhabdomyosarcoma, fibrosarcoma, leiomyosarcoma, liposarcoma, neurogenic sarcoma, synovial sarcoma and osteosarcoma; mesothelioma, lymphoma, malignant teratoma and thymoma are also included. Metastatic heart tumors are 20-40 times more common than primary malignancies, their behavior and more relevant aspects in diagnostic and surgical therapy are mentioned. Carcinoid heart tumors represent a distinctive entity and are discussed individually.
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PMID:[Cardiac tumors (II). Malignant primary tumors. Metastatic tumors. Carcinoid tumor]. 954 34

The carcinoid tumor is an uncommon neuroendocrine neoplasm the hallmark of which is excessive serotonin production. In studying kinetics of tryptophan hydroxylase and aromatic-L-amino acid decarboxylase (AAAD) in human carcinoid hepatic metastases and adjacent normal liver (J. A. Gilbert et al, Biochem. Pharmacol., 50: 845-850, 1995), we identified one significant difference: the Vmax of carcinoid AAAD was 50-fold higher than that in normal liver. Here, we report Western and Northern analyses detecting large quantities of AAAD polypeptide and mRNA in human carcinoid primary as well as metastatic tumors compared with normal surrounding tissues. To assess the feasibility of targeting these high AAAD levels for chemotherapy, AAAD inhibitors carbidopa (alpha-methyl-dopahydrazine), alpha-monofluoromethyldopa (MFMD), and 3-hydroxybenzylhydrazine (NSD-1015) were incubated (72 h) with NCI-H727 human lung carcinoid cells. Carbidopa and MFMD were lethal (IC50 = 29 +/- 2 microM and 56 +/- 6 microM, respectively); NSD-1015 had no effect on proliferation. On exposure to other human tumor lines, carbidopa was lethal only to NCI-H146 and NCI-H209 small cell lung carcinoma (SCLC) lines (IC50 = 12 +/- 1 microM and 22 +/- 5 microM, respectively). Carbidopa (100 microM) decreased growth of (but did not kill) SK-N-SH neuroblastoma and A204 rhabdomyosarcoma cells and did not affect proliferation of DU 145 prostate, MCF7 breast, or NCI-H460 large cell lung carcinoma lines. The rank order of lines by AAAD activity was NCI-H146 > NCI-H209 > SK-N-SH > NCI-H727, whereas A204, DU 145, MCF7, and NCI-H460 had no measurable activity. For lung tumor lines (carcinoid, two SCLC, and one large cell lung carcinoma), AAAD activity was correlated with the potency of carbidopa-induced cytotoxicity. However, carcinoid cell death was not solely attributable to complete inhibition of either AAAD activity or the serotonin synthetic pathway. In further evaluating potential applications of these findings with carbidopa, we determined that sublethal doses of carbidopa produced additive cytotoxic effects in carcinoid cells in combination with etoposide and cytotoxic synergy in SCLC cells when coincubated with topotecan.
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PMID:The aromatic-L-amino acid decarboxylase inhibitor carbidopa is selectively cytotoxic to human pulmonary carcinoid and small cell lung carcinoma cells. 1110 55

To extend flow cytometry (FC) to the diagnosis of nonhematopoietic neoplasms, we have developed new flow cytometric assays to identify expression of cytokeratin, epithelial cell adhesion molecule (EpCAM)/epithelial glycoprotein-2, myogenin, and CD99. To validate these assays, we correlated the flow cytometric results with the histologic and immunohistochemical results on paraffin-embedded tissue in a series of 21 cases, including 17 carcinomas, 1 atypical carcinoid, 2 rhabdomyosarcomas, and 1 Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET). Six of 7 assayed carcinomas and the carcinoid were positive for cytoplasmic cytokeratin by the flow cytometric assay. EpCAM was expressed by 11 of 12 carcinomas that were assayed by FC. Both rhabdomyosarcomas expressed myogenin by FC, and the ES/PNET case expressed CD99. Interestingly, the blast-associated antigen CD90 was expressed uniformly on the ES/PNET case and on subsets of cells in the rhabdomyosarcoma and carcinoma cases. Potential applications of the flow cytometric assay to nonhematopoietic neoplasms will include evaluating samples with limited material, monitoring disease persistence and recurrence in patients with previous diagnoses, and making rapid diagnoses in urgent cases.
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PMID:Lineage-specific identification of nonhematopoietic neoplasms by flow cytometry. 1276 Feb 82

Small blue cell tumors are a group of tumors that share a common histologic characteristic with H&E staining. This makes differentiation from one another difficult as they all appear small, blue and round. Even though they all appear the same, they are vastly different from each other. Several different techniques have been developed to help further delineate and classify these tumors which include: small cell lung cancer (SCLC); non-Hodgkin's lymphoma (NHL); Ewing's sarcoma; rhabdomyosarcoma; Merkel carcinoma; neuroblastoma; carcinoid tumors; and intra-abdominal desmpolastic small round cell tumor. Using immunoperoxidase staining, reverse transcriptase polymerase chain reaction and fluorescence in situ hybridization techniques, these tumors have been successfully differentiated from one another. This separation makes staging and treatment of these tumors more effective, as not all of these tumors respond to the same modality of treatment. The following review summarizes some of the recent findings in the various small blue cell tumors and with the potential of novel therapies.
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PMID:Recent advances in the molecular biology, diagnosis and novel therapies for various small blue cell tumors. 1292 79

Gonadal sex cord-stromal tumors contain some of the most morphologically interesting neoplasms of the gonads and these lead to many important issues in differential diagnosis. The pathology of these tumors is reviewed with emphasis on new information, similarities and differences in the two gonads, and diagnostic problems. Sertoli cell tumors occur in both gonads being more common in the testis where they usually exhibit a lobular pattern of hollow or solid tubules. In the ovary, tubular differentiation is usually the predominant feature but the lobulation typically seen in the testis is generally not as striking. One variant of Sertoli cell tumor, the large cell calcifying form, appears to be restricted to the male gonad and in contrast to other sex cord tumors is much more frequently bilateral and is associated in many cases with unusual clinical manifestations. In both sexes, patients with Peutz-Jeghers syndrome often have distinctive gonadal pathology. In females, it is in the form of the sex cord with annular tubules whereas in males, the lesion has features that are often intermediate between those of a sex cord tumor with annular tubules and a large cell calcifying Sertoli cell tumor. Sertoli-Leydig cell tumors are more morphologically diverse than pure Sertoli cell tumors and for practical purposes are an issue only in ovarian pathology being exceptionally rare in the testis. The classification proposed by Meyer into well, intermediate, and poor differentiation, remains important prognostically. More recently, heterologous and retiform differentiation has been described. Heterologous tumors most often contain mucinous epithelium, sometimes with small foci of carcinoid or less commonly, and generally in poorly differentiated neoplasms, rhabdomyosarcoma or fetal-type cartilage. Such tumors should be distinguished from pure sarcomas and teratomas. The retiform neoplasms, which tend to occur in young females, may mimic serous borderline tumors or even serous carcinomas. Granulosa cell tumors are much more common in females and in both gonads are divided into adult and juvenile forms. In females, granulosa cell tumors and other sex cord tumors may have markedly bizarre nuclei potentially leading to overdiagnosis as more malignant neoplasms. The juvenile granulosa cell tumor of the testis tends to occur in the first 6 months of life and should be carefully distinguished from the yolk sac tumor of the testis, which usually occurs in a slightly older age group. Occasional sex cord-stromal tumors cannot be readily categorized into the Sertoli or granulosa families and are diagnosed as sex cord-stromal tumors unclassified. In females, this is a relatively common placement for a neoplasm in a pregnant patient. Unclassified tumors are overall more common in males and may entrap residual normal germ cells potentially leading to the erroneous placement of the tumor in the category of a mixed germ cell sex cord-stromal tumor. From the practical viewpoint, the most helpful immunohistochemical findings are the negative staining of sex cord tumors for epithelial membrane antigen, and positive staining for inhibin and calretinin, findings that are converse to those seen in endometrioid carcinomas of the ovary, which commonly have formations that simulate sex cord tumors.
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PMID:Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems. 1550 9

A variety of benign and malignant masses can be found in the inguinal canal (IC). Benign causes of masses in the IC include spermatic cord lipoma, hematoma, abscess, neurofibroma, varicocele, desmoid tumor, air, bowel contrast material, hydrocele, and prostheses. Primary neoplasms of the IC include liposarcoma, Burkitt lymphoma, testicular carcinoma, and sarcoma. Metastases to the IC can occur from alveolar rhabdomyosarcoma, monophasic sarcoma, prostate cancer, Wilms tumor, carcinoid tumor, melanoma, or pancreatic cancer. In patients with a known malignancy and peritoneal carcinomatosis, the diagnosis of metastases can be suggested when a mass is detected in the IC. When peritoneal disease is not evident, a mass in the IC is indicative of stage IV disease and may significantly alter clinical and surgical treatment of the patient. A combination of the clinical history, symptoms, laboratory values, and radiologic features aids the radiologist in accurately diagnosing mass lesions of the IC. Supplemental material available at radiographics.rsnajnls.org/cgi/content/full/28/3/819/DC1.
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PMID:The inguinal canal: anatomy and imaging features of common and uncommon masses. 1848 Apr 86

In recent years, the synchronous occurrence of tumors of different histotypes arising in the same organ has been reported more frequently in the literature. In the stomach, adenocarcinoma has been described with coexisting primary rhabdomyosarcoma, carcinoid, and low-grade B-cell lymphoma of mucosa-associated lymphoid tissue. The simultaneous development of adenocarcinoma and gastric mesenchymal tumor has been documented rarely. We report one such case. A 65-year-old male was diagnosed with a proximal gastric adenocarcinoma and underwent subtotal gastrectomy. Subsequent histopathological examination revealed the presence of another tumor at the gastric antrum. This was a gastrointestinal stromal tumor of low risk category (GIST). The literature has only a few previous reports of this very rare association. It is not known whether this synchronicity is incidental or there is a causative factor inducing the development of tumors of different histotypes in the same organ. Pathologists, oncologists and surgeons should be aware of this interesting condition.
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PMID:Synchronous adenocarcinoma and gastrointestinal stromal tumor in the stomach. 2061 20

Teratoma with a malignant somatic component (MSC) is a rare phenomenon recognized when a somatic type malignancy occurs in the context of a germ cell tumor (GCT). The authors present their 29-year experience with 40 patients treated from 1981 to 2009 in their institution. The average age was 31 years. All patients underwent radical orchiectomy, which demonstrated a GCT in 19 with an MSC. The MSC was observed in the other 21 cases in metastatic sites, including lung (13), liver (3), pleura (1), mediastinum (7), supraclavicular lymph nodes (1), and retroperitoneal lymph nodes (9). The most common histologic types were rhabdomyosarcoma (n = 11) and primitive neuroectodermal tumors (n = 10), followed by adenocarcinoma (n = 9), sarcoma, not otherwise specified (n = 5), well-differentiated liposarcoma (n = 2), leiomyosarcoma (n = 2), chondrosarcoma (n = 2), nephroblastoma (Wilms's tumor), carcinoid, malignant peripheral nerve sheath tumor, and a gemistiocytic astrocytoma with choroid plexus tumor (each n = 1). Clinical data were collected through a dedicated database and are available for all 40 cases. After a median follow-up of 37 months, 29/40 (72.5%) were alive and disease-free. Stage-specific survival was 100% for stage I, 87.5% for stage II, and 53% for stage III patients suggesting that patients with MSC confined to the testis have a good prognosis. A lower sensitivity to chemotherapy for a MSC when compared with typical GCT is reported. Surgical excision of the MSC is therefore an essential part of the management.
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PMID:Teratoma with somatic-type malignant components in germ cell tumors of the testis: a clinicopathologic analysis of 40 cases with outcome correlation. 2113 86


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