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Other Tumors (other + tumor)
Terms modified by Other Tumors Selected AbstractsPregnancy-associated anaplastic large-cell lymphoma of the breast: A rare mimic of ductal carcinomaDIAGNOSTIC CYTOPATHOLOGY, Issue 4 2006P. Iyengar M.D. Abstract Anaplastic large-cell lymphoma (ALCL) is a rare T-cell lymphoma typically seen in children and young adults. It has been described in numerous sites; however, the breast is one of the least common locations. We herein report a case of ALCL arising in the breast of a 36-yr-old pregnant woman. To our knowledge this is the second such case in the English literature. We would like to highlight the cytologic and histologic features of ALCL, as this case was initially misdiagnosed as a ductal carcinoma. Differential diagnosis with other tumors is also discussed. This case serves to emphasize the importance of the triple test, and the need for correlation of fine-needle aspiration findings with core biopsy findings in breast tumor management. Diagn. Cytopathol. 2006;34:298,302. © 2006 Wiley-Liss, Inc. [source] Clear-cell adenocarcinoma of the female genital tract: Presence of Hyaline stroma and tigroid background in various types of cytologic specimensDIAGNOSTIC CYTOPATHOLOGY, Issue 6 2005Surapan Khunamornpong M.D. Abstract Hyaline basement membrane-like stromal material and tigroid background are distinctive cytologic features observed in Diff-Quik (DQ)- or Giemsa-stained smears of clear-cell adenocarcinoma (CCA) of the female genital tract. However, it is uncertain how often these features are present in different types of cytologic specimens, and which type of preparation is optimal for this diagnosis. We therefore reviewed the cytologic features of CCA in three types of specimens, including 15 scrape cytology specimens, 7 fine-needle aspiration (FNA) specimens, and 15 peritoneal cytology specimens, with emphasis on the features observed in DQ-stained smears. The cell morphology in scrape cytology specimens and FNA specimens was comparable, whereas in peritoneal cytology specimens, the cytoplasm was better preserved. Most tumor cells had fragile cytoplasm containing variable amounts of fine vacuoles, and round nuclei with distinct or prominent nucleoli. Hyaline stroma was present in 93% of scrape cytology specimens, 71% of FNA specimens, and 80% of peritoneal cytology specimens. Tigroid background was observed in 47% of scrape cytology specimens, 43% of FNA specimens, but in none of the peritoneal cytology specimens. Formation of a tigroid background may be prevented by the abundant fluid content in peritoneal cytology specimens. Hyaline stroma and tigroid background were uncommonly seen in scrape smears from other types of primary ovarian tumors, mainly juvenile granulosa cell tumor and yolk sac tumor. However, the additional presence of papillary structures allows CCA to be readily distinguished from these other tumors. We propose that scrape cytology offers the best approach for the intraoperative cytologic diagnosis of CCA. Diagn. Cytopathol. 2005;32:336,340.© 2005 Wiley-Liss, Inc. [source] Identification of some human genes oppositely regulated during esophageal squamous cell carcinoma formation and human embryonic esophagus developmentDISEASES OF THE ESOPHAGUS, Issue 3 2010M. V. Zinovyeva SUMMARY Here we directly compared gene expression profiles in human esophageal squamous cell carcinomas and in human fetal esophagus development. We used the suppression subtractive hybridization technique to subtract cDNAs prepared from tumor and normal human esophageal samples. cDNA sequencing and reverse transcription polymerase chain reaction (RT-PCR) analysis of RNAs from human tumor and the normal esophagus revealed 10 differentially transcribed genes: CSTA, CRNN, CEACAM1, MAL, EMP1, ECRG2, and SPRR downregulated, and PLAUR, SFRP4, and secreted protein that is acidic and rich in cysteine upregulated in tumor tissue as compared with surrounding normal tissue. In turn, genes up- and downregulated in tumor tissue were down- and upregulated, respectively, during development from the fetal to adult esophagus. Thus, we demonstrated that, as reported for other tumors, gene transcriptional activation and/or suppression events in esophageal tumor progression were opposite to those observed during development from the fetal to adult esophagus. This tumor ,embryonization' supports the idea that stem or progenitor cells are implicated in esophageal cancer emergence. [source] Inactivation of the cystatin E/M tumor suppressor gene in cervical cancerGENES, CHROMOSOMES AND CANCER, Issue 9 2008Mysore S. Veena We have previously localized a cervical cancer tumor suppressor gene to a 300 kb interval of 11q13. Analysis of candidate genes revealed loss of expression of cystatin E/M, a lysosomal cysteine protease inhibitor, in 6 cervical cancer cell lines and 9 of 11 primary cervical tumors. Examination of the three exons in four cervical cancer cell lines, 19 primary tumors, and 21 normal controls revealed homozygous deletion of exon 1 sequences in one tumor. Point mutations were observed in six other tumors. Two tumors contained mutations at the consensus binding sites for cathepsin L, a lysosomal protease overexpressed in cervical cancer. Introduction of these two point mutations using site directed mutagenesis resulted in reduced binding of mutated cystatin E/M to cathepsin L. Although mutations were not observed in any cell lines, four cell lines and 12 of 18 tumors contained promoter hypermethylation. Reexpression of cystatin E/M was observed after 5,aza 2-deoxycytidiene and/or Trichostatin A treatment of cervical cancer cell lines, HeLa and SiHa, confirming promoter hypermethylation. Ectopic expression of cystatin E/M in these two cell lines resulted in growth suppression. There was also suppression of soft agar colony formation by HeLa cells expressing the cystatin E/M gene. Reexpression of cystatin E/M resulted in decreased intracellular and extracellular expression of cathepsin L. Overexpression of cathepsin L resulted in increased cell growth which was inhibited by the reintroduction of cystatin E/M. We conclude, therefore, that cystatin E/M is a cervical cancer suppressor gene and that the gene is inactivated by somatic mutations and promoter hypermethylation. © 2008 Wiley-Liss, Inc. [source] Frequency and characterization of HMGA2 and HMGA1 rearrangements in mesenchymal tumors of the lower genital tractGENES, CHROMOSOMES AND CANCER, Issue 11 2007Fabiola Medeiros Mesenchymal tumors of the lower genital tract predominantly occur in women of reproductive age and are mainly represented by aggressive angiomyxoma (AAM) and angiomyofibroblastoma (AMF). Whether these tumors are different phenotypic expressions of the same biological entity is still debatable. Genetic rearrangements of HMGA2 have been reported in a few cases of AAM but its frequency and clinicobiological implications have not been studied systematically. We evaluated 90 cases of mesenchymal tumors of the lower genital tract that comprised 42 AAMs, 18 AMFs, 6 cellular angiofibromas, 5 fibroepithelial stromal polyps, 15 genital leiomyomas, 3 superficial angiomyxomas, and 1 spindle cell lipoma. Fluorescence in situ hybridization was used to identify rearrangements of HMGA2 and its homologue HMGA1. HMGA2 rearrangements were identified in 14 AAMs (33%) and in 1 vaginal leiomyoma. All other tumors were negative for HMGA2 rearrangements. HMGA1 rearrangement was not found in any of the cases. RT-PCR confirmed transcriptional upregulation of HMGA2 only in tumors with HMGA2 rearrangements. Standard cytogenetic analyses were performed in two AAMs and one AMF. One AAM had a t(1;12)(p32;q15); the other tumors had normal karyotypes. Mapping and sequence analysis of the breakpoint showed fusion to the 3, untranslated region of HMGA2 to genomic sequences derived from the contig NT 032977.8 on chromosome 1p32. Our findings support the hypothesis that AAM and AMF are distinct biological entities. The diagnostic usefulness of HMGA2 rearrangements to differentiate between AAM and other tumors of the lower genital tract may be limited due to the their low frequency. © 2007 Wiley-Liss, Inc. [source] Genetics of dermatofibrosarcoma protuberans family of tumors: From ring chromosomes to tyrosine kinase inhibitor treatmentGENES, CHROMOSOMES AND CANCER, Issue 1 2003Nicolas Sirvent Dermatofibrosarcoma protuberans (DP) is a rare, slow-growing, infiltrating dermal neoplasm of intermediate malignancy, made up of spindle-shaped tumor cells often positive for CD34. The preferred treatment is wide surgical excision with pathologically negative margins. At the cytogenetic level, DP cells are characterized by either supernumerary ring chromosomes, which have been shown by using fluorescence in situ hybridization techniques to be derived from chromosome 22 and to contain low-level amplified sequences from 17q22-qter and 22q10,q13.1, or t(17;22), that are most often unbalanced. Both the rings and linear der(22) contain a specific fusion of COL1A1 with PDGFB. Similar to other tumors, the COL1A1-PDGFB fusion is occasionally cryptic, associated with complex chromosomal rearrangements. Although rings have been mainly observed in adults, translocations have been reported in all pediatric cases. DP is therefore a unique example of a tumor in which (i) the same molecular event occurs either on rings or linear translocation derivatives, (ii) the chromosomal abnormalities display an age-related pattern, and (iii) the presence of the specific fusion gene is associated with the gain of chromosomal segments, probably taking advantage of gene dosage effects. In all DP cases that underwent molecular investigations, the breakpoint localization in PDGFB was found to be remarkably constant, placing exon 2 under the control of the COL1A1 promoter. In contrast, the COL1A1 breakpoint was found to be variably located within the exons of the ,-helical coding region (exons 6,49). No preferential COL1A1 breakpoint and no correlation between the breakpoint location and the age of the patient or any clinical or histological particularity have been described. The COL1A1-PDGFB fusion is detectable by multiplex RT-PCR with a combination of forward primers designed from a variety of COL1A1 exons and one reverse primer from PDGFB exon 2. Recent studies have determined the molecular identity of "classical" DP, giant cell fibroblastoma, Bednar tumor, adult superficial fibrosarcoma, and the granular cell variant of DP. In approximately 8% of DP cases, the COL1A1-PDGFB fusion is not found, suggesting that genes other than COL1A1 or PDGFB might be involved in a subset of cases. It has been proposed that PDGFB acts as a mitogen in DP cells by autocrine stimulation of the PDGF receptor. It is encouraging that inhibitory effects of the PDGF receptor tyrosine kinase antagonist imatinib mesylate have been demonstrated in vivo; such targeted therapies might be warranted in the near future for treatment of the few DP cases not manageable by surgery. © 2003 Wiley-Liss, Inc. [source] Sequencing of intron 3 of HMGA2 uncovers the existence of a novel exonGENES, CHROMOSOMES AND CANCER, Issue 1 2002Sven Hauke Aberrations affecting the gene encoding the high mobility group protein HMGA2 (formerly HMGIC) have been found in a variety of human tumors, e.g., uterine leiomyomas, lipomas, and pulmonary chondroid hamartomas. These aberrations lead to fusion genes, transcriptional up-regulation, or aberrant transcripts of HMGA2. In the latter case, truncated transcripts consisting of exons 1 to 3 of HMGA2, encoding the three DNA-binding domains, and ectopic sequences derived from chromosome 12 are frequent. There are several lines of evidence indicating that the biological and tumorigenic features of truncated HMGA2 derivatives, i.e., those composed of the DNA-binding domains and a shortened acidic tail, clearly differ from those of the normal protein consisting of three DNA-binding domains and one large acidic tail. By sequencing the complete 112 kb third intron of HMGA2, we were able to detect several of the ectopic sequences, known as fused to HMGA2. Expression studies revealed co-expression of one of these transcripts with the normal transcript in tumors with 12q14-15 aberrations as well as in other tumors, and in normal tissues. Thus, this transcript (HMGA2b) is flanked by an alternative terminal exon of HMGA2. Due to the loss of the part encoding the acidic tail, the expression of the latter transcript may have more striking effects than the "wild type" HMGA2 (HMGA2a) in terms of tumorigenesis. This finding clearly indicates that functional studies also should address the role of the HMGA2b transcript. © 2002 Wiley-Liss, Inc. [source] Exploring the "two-hit hypothesis" in NF2: Tests of two-hit and three-hit models of vestibular schwannoma developmentGENETIC EPIDEMIOLOGY, Issue 4 2003Ryan Woods Abstract Neurofibromatosis 2 (NF2) is a genetic disease that occurs in approximately 1 in 40,000 live births. Almost all affected individuals develop bilateral tumors of Schwann cells that surround the vestibular nerves; these tumors are known as vestibular schwannomas (VS). Evidence from molecular genetic studies suggests that at least two mutations are involved in formation of VS in patients with NF2. Several authors proposed probabilistic models for this process in other tumors, and showed that such models are consistent with incidence data. We evaluated two different probabilistic models for a "2-hit" hypothesis for VS development in NF2 patients, and we present results from fitting these models to incidence data. Molecular evidence does not exclude the possibility that additional hits are necessary for the development of VS, and we also assessed a "3-hit" model for tumor formation. The "3-hit" model fits the data marginally better than one of the "2-hit" models and much better than the other "2-hit" model. Our findings suggest that more than two mutations may be necessary for VS development in NF2 patients. Genet Epidemiol 24:265,272, 2003. © 2003 Wiley-Liss, Inc. [source] Cancer patterns in nasopharyngeal carcinoma multiplex families in TaiwanINTERNATIONAL JOURNAL OF CANCER, Issue 7 2009Kelly J. Yu Abstract Genetic and environmental factors have been implicated in the etiology of nasopharyngeal carcinoma (NPC), a tumor known to be closely associated with Epstein-Barr virus (EBV) infection. Studies have reported familial aggregation of NPC and have suggested the possible aggregation of NPC and other cancers. We evaluated familial aggregation of cancer in 358 high-risk families with two or more NPC cases enrolled in a NPC genetics study in Taiwan. Participants were linked to the Taiwan National Cancer Registry to identify incident cancers diagnosed after study enrollment (started in 1996) and before December 31, 2005, or death. In total, 2,870 individuals from the NPC Multiplex Family Study contributed 15,151 person-years over an average of 5.3 years of follow-up. One hundred ten incident cancers were identified. Multiple-primary standardized incidence ratios (MP-SIRs) were computed to evaluate overall cancer risk associated with infectious agents and with other tumors. The overall MP-SIR was 1.3 (95% CI: 1.1,1.6), which was largely explained by an excess in NPC (MP-SIR = 15; 95% CI: 10,23). Exclusion of incident NPC diagnoses led to an overall MP-SIR of 1.0 (95% CI: 0.83,1.3). Similarly, the observed excess risk of cancers associated with infectious agents (MP-SIR = 2.0; 95% CI: 1.5,2.6) was driven by the excess in NPC; exclusion of NPC cases led to a reduced MP-SIR that did not differ from 1.0. Analysis of the largest NPC multiplex family study to date confirms the presence of coaggregation of NPC within families in Taiwan but does not provide evidence for a broader familial syndrome involving NPC and other tumors. © 2008 Wiley-Liss, Inc. [source] Correlations between the Sonic Hedgehog Pathway and basal cell carcinomaINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 11 2007Omar Lupi MD The Hedgehog (HH) family of intercellular signaling proteins has some essential functions in patterning both invertebrate and vertebrate embryos. Identified as an important regulator of segment polarity and tissue organization in flies, the HH pathway can also play a significant role in human development and in cutaneous carcinogenesis. The family received their name because when the D. melanogaster HH protein malfunctions the mutant fly ends up looking like a small prickly ball, similar to a curled up hedgehog. The Sonic hedgehog (SHH) pathway is implicated in the etiology of the most common human cancer, the basal cell carcinoma (BCC). Mutations in the receptor of SHH, the patched gene (PTCH), have been characterized in sporadic BCCs as well as those from patients with the rare genetic syndrome nevoid BCC. Human PTCH is mutated in sporadic as well as hereditary BCCs, and inactivation of this gene is probably a necessary if not sufficient step for tumorigenesis. Delineation of the biochemical pathway in which PTCH functions may lead to rational medical therapy for skin cancer and possibly other tumors. [source] Immunohistochemical characteristics of melanomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2008Steven J. Ohsie Melanoma has a wide spectrum of histologic features which mimic epithelial, hematologic, mesenchymal, and neural tumors. Immunohistochemistry has been the primary tool to distinguish melanomas from these other tumors; it has also been studied for use as an adjunct to distinguish benign and malignant melanocytic tumors and to elucidate prognosis. Furthermore, there has been extensive effort to find a suitable marker to differentiate spindle cell and desmoplastic melanoma from other tumors. We have reviewed the literature investigating melanocytic differentiation markers, proliferation markers, immunomodulatory markers, signaling molecules, and nerve growth factors and receptors. Despite the proliferation of immunohistochemical markers, S-100 remains the most sensitive marker for melanocytic lesions, while markers such as HMB-45, MART-1/Melan-A, tyrosinase, and MITF demonstrate relatively good specificity but not as good sensitivity as S-100. No marker has proven useful in distinguishing spindle cell and desmoplastic melanomas from other tumors. Ki67 remains the most useful adjunct in distinguishing benign from malignant melanocytic tumors. None of the markers reviewed has been shown conclusively to have prognostic value for melanocytic neoplasms. [source] Immunoreactivity of CD99 in invasive malignant melanomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 10 2006Anne E. Wilkerson Background:, CD99, also known as p30/32, is a glycoprotein product of the MIC2 gene. It was originally utilized in immunohistochemistry as a unique marker for Ewing sarcoma, other primitive neuroectodermal tumors, and subsequently in other tumors. Its expression in malignant melanoma (MM) has not been well documented, with just two isolated cases of MM recently reported. Recent studies have documented CD99 expression in a significant percentage of atypical fibroxanthomas (AFX), posing potential diagnostic problems in differentiating these two entities. As mistaking MM for AFX based on immunohistochemical staining pattern has significant consequences, we sought to determine the percentage of invasive MM in our archives that have this staining pattern. Methods:, Seventy-eight cases of invasive melanoma were retrieved from our files. Each case was stained with mouse anti-human CD99 and evaluated for membranous expression. Results:, Our evaluation revealed that 47 of 78 MM cases (60%) stain positive for CD99. Conclusion:, This study is the first to demonstrate, in a large series, the prevalence of CD99 expression in primary cutaneous melanoma. Additionally, this introduces in the histologic differential diagnosis of CD99 expressing dermal spindle cell lesions. [source] Cutaneous mesenchymal hamartoma with mixed myogenous differentiationJOURNAL OF CUTANEOUS PATHOLOGY, Issue 4 2006John Edwin Schrecengost A 3-month-old infant girl presented with a polypoid lesion in the perianal region. No changes in this lesion had been noted since birth. Microscopic examination of the excised specimen showed a mixture of mesenchymal elements, dominated by haphazard thin fascicles of skeletal muscle. Collagen and vascular changes were also apparent. Immunohistochemistry showed positive staining for muscle-specific actin and desmin in the fascicular components of the lesion, and smooth muscle actin, desmin, and h-caldesmon positivity in a haphazard collection of muscle fibers in the deep dermis and anal submucosa. Numerous Verhoeff-van Gieson positive elastic fibers were also noted. Hamartomas containing skeletal muscle have rarely been reported outside of the head and neck region. They must be distinguished from a variety of other tumors, including juvenile rhabdomyoma, benign Triton tumor, and rhabdomyosarcoma. [source] Molecular diagnosis of basal cell carcinoma and other basaloid cell neoplasms of the skin by the quantification of Gli1 transcript levelsJOURNAL OF CUTANEOUS PATHOLOGY, Issue 2 2005Naohito Hatta Background:, Distinguishing basal cell carcinoma (BCC) from other benign and malignant skin tumors is sometimes a difficult task for the pathologists. Because the activation of hedgehog signals and the up-regulation of its critical transcriptional factor Gli1 are well documented in BCC, a molecular technique measuring Gli1 transcripts may aide the diagnosis. Methods:,Gli1 transcript levels were measured by real-time quantitative reverse transcriptase polymerase chain reaction (RT-PCR) using RNA extracted from formalin-fixed, paraffin-embedded tissues of 68 cases of various skin tumors. Hematoxylin and eosin-stained pathology slides were independently reviewed by three expert dermatopathologists. Results:, The histological diagnoses were unambiguous in 53 tumors. The tumors included BCC (21), squamous cell carcinoma (13), seborrheic keratoses (8), trichoepithelioma (5), eccrine poroma/porocarcinoma (4), and sebaceous epithelioma/carcinoma (2). In these unambiguous cases, all BCC and trichoepithelioma tumors showed high expression of Gli1mRNA, while the expression was virtually absent in other tumors. The diagnosis was discordant among three pathologists in the remaining 15 tumors. Histological diagnoses included BCC, BCC with sebaceous differentiation, sebaceoma/sebaceous epithelioma, trichoblastoma, trichoepithelioma, basaloid follicular harmartoma, basosquamous carcinoma, etc. Six of them showed high Gli1 transcript levels. Conclusions:, Quantification of Gli1 transcripts by RT-PCR is helpful in discriminating BCC and trichoepithelioma from other skin tumors. [source] Radiotherapy for hepatocellular carcinoma: Systematic review of radiobiology and modeling projections indicate reconsideration of its useJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 4 2010Alan J Wigg Abstract Background and Aims:, External beam radiotherapy currently has a limited role in the treatment of hepatocellular carcinoma (HCC). The purpose of this article was to review available radiobiological data on HCC and normal liver and incorporate these data into radiobiological models that may be used to explain and improve treatment. Methods:, Volume doubling times of HCC were described and used to demonstrate growth of HCC with time, assuming both exponential and logistic growth. Radiosensitivity of HCC was described and used to demonstrate the probability of uncomplicated tumor control as tumor size increases. The relationship between tolerance of liver to irradiation and volume irradiated was examined. Results:, The median volume doubling time for untreated HCC was 130 days. HCC have a long period of subclinical growth. Radiosensitivity of HCC lies within the range of other tumors commonly treated with radiotherapy. When treating small volumes of normal liver, relatively high doses may be used with low risk of late radiation damage. There is a high probability of sterilizing subclinical disease and small HCC with tolerable radiation doses. Conclusion:, New radiobiological data, modeling, emerging clinical data and the advantages offered by standard external beam radiotherapy techniques suggest the need for reconsidering the use of radiotherapy and for new trials. [source] Chemoradiotherapy in gallbladder cancerJOURNAL OF SURGICAL ONCOLOGY, Issue 8 2006FACS, Xabier de Aretxabala MD Abstract Gallbladder cancer (GC) is considered a rare disease associated with a poor prognosis. Unfortunately, the low number of cases makes the performance of trials addressing the role of adjuvant, neoadjuvant, and/or palliative therapy difficult. For a long time, the majority of trials were 5-fluorouracil (5 FU)-based, and results were uniformly poor. Since the introduction of Gemcitabine, response rates of approximately 30% have been observed through the use of this drug and new approaches have been tested. In this sense, drugs such as Cisplatin and Capecitabine have been employed concurrently with gemcitabine and/or radiation. Since a recurrence pattern is both distant and local, chernoradiation seems a logical option to deal with the disease. However, at the present time, the lack of valid and scientific evidence means that most of the recommendations originate from trials dealing with other tumors, such as pancreas cancer and biliary tract cancer (BTC). The aforementioned treatment alternatives warrant further evaluation focusing on GC. J. Surg. Oncol. 2006;93:699,704. © 2006 Wiley-Liss, Inc. [source] Elevation of XPA protein level in testis tumor cells without increasing resistance to cisplatin or UV radiationMOLECULAR CARCINOGENESIS, Issue 8 2008Beate Köberle Abstract Most testicular germ cell tumors are curable using cisplatin-based chemotherapy, and cell lines from these tumors are unusually sensitive to cisplatin and other DNA-damaging agents. It has been suggested that this might be caused by a lower-than normal nucleotide excision repair (NER) activity. Previous studies found that cell lines from testicular germ cell tumors have on average about one-third the level of the NER protein XPA in comparison to cell lines from other tumors. We asked whether over-expression of XPA protein would alleviate the cellular sensitivity and increase the DNA repair capacity of a testis tumor cell line. Increasing XPA levels in 833K cells by 10-fold did not increase resistance to UV irradiation. XPA was localized to the cell nucleus in all cell lines, before and after exposure to UV-radiation. 833K cells were proficient in removing UV radiation-induced photoproducts from the genome and increased XPA did not enhance the rate of removal. Further, over-expressing functional XPA protein did not correlate with increased resistance of 833K testis tumor cells to cisplatin. Thus, although the amount of XPA in this testis tumor cell line is lower than normal, it is sufficient for NER in vivo. The relative sensitivity of testis tumor cells to cisplatin, UV radiation, and other DNA damaging agents is likely related not to NER capacity, but to other factors such as the integrity of the p53 pathway in these cells. © 2008 Wiley-Liss, Inc. [source] Neurofibromatosis type 2 in an infant with multiple plexiform schwannomas as first symptomTHE JOURNAL OF DERMATOLOGY, Issue 1 2007Takehiko MIYAKAWA ABSTRACT Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder that is caused by inactivating mutations or a loss of both alleles in the NF2 tumor-suppressor gene. Bilateral vestibular schwannomas are considered to be the hallmark of this disease, with hearing loss and tinnitus which are caused by these tumors, usually presenting as the initial symptoms. In addition to other tumors and ocular findings, skin abnormalities also occur in NF2, however, they are not so characteristic as neurofibromatosis type 1 (NF1). We herein report a case of NF2 which occurred in a 5-year-old boy. He had multiple cutaneous tumors but did not have any symptoms related to vestibular schwannomas. He also had multiple depigmented spots. A histopathological examination revealed these tumors to be plexiform schwannomas; we therefore suspected NF2. As a result of magnetic resonance imaging with gadolinium enhancement, bilateral vestibular schwannomas were detected and a final diagnosis of NF2 was thus made. The association between NF2 and multiple depigmented spots is unknown, we therefore consider that multiple cutaneous plexiform schwannomas may strongly suggest an association with NF2. [source] Malignancy After Heart Transplantation: Incidence, Prognosis and Risk FactorsAMERICAN JOURNAL OF TRANSPLANTATION, Issue 5 2008M. G. Crespo-Leiro The Spanish Post-Heart-Transplant Tumour Registry comprises data on neoplasia following heart transplantation (HT) for all Spanish HT patients (1984,2003). This retrospective analysis of 3393 patients investigated the incidence and prognosis of neoplasia, and the influence of antiviral prophylaxis. About 50% of post-HT neoplasias were cutaneous, and 10% lymphomas. The cumulative incidence of skin cancers and other nonlymphoma cancers increased with age at HT and with time post-HT (from respectively 5.2 and 8.9 per 1000 person-years in the first year to 14.8 and 12.6 after 10 years), and was greater among men than women. None of these trends held for lymphomas. Induction therapy other than with IL2R-blockers generally increased the risk of neoplasia except when acyclovir was administered prophylactically during the first 3 months post-HT; prophylactic acyclovir halved the risk of lymphoma, regardless of other therapies. Institution of MMF during the first 3 months post-HT reduced the incidence of skin cancer independently of the effects of sex, age group, pre-HT smoking, use of tacrolimus in the first 3 months, induction treatment and antiviral treatment. Five-year survival rates after first tumor diagnosis were 74% for skin cancer, 20% for lymphoma and 32% for other tumors. [source] Markers aiding the diagnosis of chondroid tumors: an immunohistochemical study including osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit), and YKL-40APMIS, Issue 7 2009SŘREN DAUGAARD Chondroid tumors comprise a heterogenous group of benign to overt malignant neoplasms, which may be difficult to differentiate from one another by histological examination. A group of 43 such tumors was stained with nine relevant antibodies in an attempt to find consistent marker profile(s) for the different subgroups. Archival material from three extraskeletal myxoid chondrosarcomas, five chordomas, five chondromyxoid fibromas, five chondroblastomas and 25 chondrosarcomas was stained with antibodies against osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit) and YKL-40. All 25 chondrosarcomas showed a positive staining reaction for D2-40, none for actin and CD117, and a partial reactivity for bcl-2 (36%). Chondroblastomas (5/5) and chondromyxoid fibromas (2/5) were the only tumors with a positive reaction for actin, and all chondroblastomas (n=5) and extraskeletal myxoid chondrosarcomas (n=3) were positive for bcl-2. In contrast to all other tumors, two of three extraskeletal myxoid chondrosarcomas were also positive for CD17 and negative for osteonectin, cox-2, mdm-2 and actin. All five chordomas were negative for D2-40 and positive for mdm-2 and YKL-40. The diagnosis of chondrosarcoma may be aided by its positivity for D2-40 and YKL-40 and its lack of reactivity for actin and CD117. This should be seen in the light of no reaction for D2-40 in chordomas and a corresponding lack of reaction for osteonectin, cox-2, mdm-2 and actin in extraskeletal myxoid chondrosarcomas. A convincing immunoreactivity for calponin and/or actin in chondromyxoid fibromas and chondroblastomas may also be helpful in differentiating these tumors from chondrosarcomas. [source] Intracranial tumor surgery in patients >70 years of age: is clinical practice worthwhile or futile?ACTA NEUROLOGICA SCANDINAVICA, Issue 5 2009S. G. Rogne Objectives,,, To study survival and functional outcome after intracranial tumor surgery in elderly patients. Materials and methods,,, This is a retrospective study of 289 consecutive patients of age ,70 years, who underwent primary surgery (resection or biopsy) in the time period 2003,2007 for an intracranial tumor (87 astrocytomas, 79 meningiomas, 62 brain metastases, 33 pituitary adenomas and 28 other tumors). Results,,, The surgical mortality was 2.8%. Overall survival at 6 months, 1, 2 and 5 years was 73%, 57%, 46% and 38% respectively. Histology, pre-operative Eastern Cooperative Oncology Group (ECOG) performance score and resection, as opposed to biopsy, were significantly associated with survival. Gender, age and American Association of Anaesthetists (ASA) score were not significantly related to survival. One-year survival after surgery for astrocytoma, meningioma, brain metastases and pituitary adenoma were 24%, 94%, 31% and 96% respectively. More than 85% of the patients who were alive 6 months after surgery had a stable or improved ECOG score compared with their pre-operative score. Conclusions,,, Surgery for intracranial tumors in selected elderly patients is worthwhile, not futile. Age alone should not be used as a selection criterion for treatment. [source] Adoptive immunotherapy with allogeneic Epstein,Barr virus (EBV)-specific cytotoxic T-lymphocytes for recurrent, EBV-positive Hodgkin diseaseCANCER, Issue 9 2004Kenneth G. Lucas M.D. Abstract BACKGROUND It has been shown that adoptive immunotherapy with Epstein,Barr virus (EBV)-specific cytotoxic T-lymphocytes (CTL) is effective for the treatment of EBV-induced lymphoproliferative disease in stem cell transplantation recipients and organ transplantation recipients. The role of EBV CTL in other tumors for which this virus has been implicated in pathogenesis, such as EBV-positive Hodgkin disease (HD), has not been demonstrated clearly. METHODS To investigate the antitumor effects and toxicity of allogeneic EBV CTL in EBV-positive HD, the authors initiated a pilot trial in which EBV CTL were cultured from allogeneic, partially human leukocyte antigen-matched donors and were infused into patients who had therapy-refractory disease. The first cohort of 3 patients (Cohort I) received 3 separate infusions of EBV CTL (5.0 × 106 EBV CTL/kg per dose), and the second cohort (Cohort II) received 30 mg/m2 per day of fludarabine for 3 days followed by a single CTL infusion (1.5 × 107 EBV CTL/kg). RESULTS All three patients in Cohort I had decreases in measurable disease after EBV CTL infusions, and one of those patients was without evidence of disease 22 months after infusion. Two of 3 patients in Cohort II had decreases in measurable disease, although it was not determined whether those decreases were related to fludarabine or to CTL, and 1 patient in Cohort II had 7 months without disease progression. Unlike the patients in Cohort I, fludarabine recipients did not have increases in antidonor CTL responses. Donor cells could not be detected in any of the CTL recipients. CONCLUSIONS Adoptive immunotherapy with allogeneic EBV CTL was safe for patients with recurrent, refractory, EBV-positive HD; and clinical responses may be observed without the establishment of detectable donor lymphoid chimerism. Cancer 2004. © 2004 American Cancer Society. [source] | |||||||||||||||||||||||||