1/638. Mixed-type liposarcoma of the oral cavity: a case with unusual features and a long survival.A case of mixed-type liposarcoma, which showed unusual dedifferentiation in the recurrence, is reported. The rapidly growing mass in the palate of a 60-year-old Japanese woman first revealed a combination of myxoid liposarcoma with features resembling storiform malignant fibrous histiocytoma. The recurrent neoplasm, showing an abrupt transition between myxoid and non-lipogenic parts, partially reverted to sclerosing well-differentiated liposarcoma. The patient died 10.1 years after the first operation.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/638. Sialoblastoma: clinicopathological/immunohistochemical study.Sialoblastoma is an extremely rare salivary tumor diagnosed at birth or shortly thereafter with significant variability in histologic range and clinical course, so that for an individual case it may be difficult to predict the most appropriate therapy. We detail the case of a toddler noted to have a firm 1-2-cm mass in the left cheek at 21 months. Parotidectomy was performed at 26 months, revealing a sialoblastoma; the resection margins were positive. During the next 10 months, the mass recurred or persisted, necessitating numerous procedures. The tumor was composed of basaloid cells with fine chromatin and other more mature cuboidal epithelial cells. Ductules and solid organoid nests with some tendency toward peripheral pallisading were also noted. There was no perineural invasion; necrosis initially was sparse but increased over time. The mitotic rate also increased from 6 to 7/10 high-power fields in the first resection to 20/10 high-power fields in the last resection. Nuclear pleomorphism increased with time. The MiB1 proliferative index revealed a dramatic increase in the number of labeled nuclei: from 3 cells/10 high-power fields in the first specimen to 94 cells/10 high-power fields for the last specimen. Cytokeratin accentuated the ductal structures. S-100 showed a diffuse staining pattern, with darker staining of the spindled myoepithelial cells. The Her-2-neu protein showed moderate cytoplasmic staining, whereas the p53 showed only occasional labeling of nuclei. This is the first case of sialoblastoma with evidence of increasing anaplasia based on increasing proliferative capacity. Therefore, the distinction between benign and malignant sialoblastomas may not be as well defined as previously thought. The patient's prognosis is likely to be determined by the tumor grade as well as the stage at presentation and the extent of resection.- - - - - - - - - - ranking = 0.058549471295611keywords = ductal (Clic here for more details about this article) |
3/638. A pathologic and clinical study of adenosquamous carcinoma of the larynx. Report of four cases and review of the literature.Four cases of adenosquamous carcinoma are described. Emphasis is put on the rarity of this histological type of tumour which was first described in the larynx by Gerughty et al. (1968) who reported three cases. An exhaustive histological documentation of the neoplasm accompanies the discussion of its pathomorphological characteristics, histogenesis, differential diagnosis, prognosis and treatment. Stress is laid on the extreme aggressiveness of the neoplasm and on its high biologic malignancy. Elective treatment is radical surgery. The tumour in question is a distinct anatomo-clinical entity with its own connotations and therefore must be distinguished from the most common squamous cell carcinoma of the larynx.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
4/638. Ductal carcinoma in situ recurrent on the chest wall after mastectomy.Two patients with local recurrence on the chest wall subsequent to mastectomy for ductal carcinoma in situ (DCIS) are presented. One recurrence was invasive carcinoma and the second was DCIS. Excision and chest wall irradiation, together with chemotherapy in the first patient, have provided subsequent disease-free survivals of 6 and 12 years respectively. Although mastectomy for DCIS is almost always curative, the possibility of local recurrence requires careful surveillance.- - - - - - - - - - ranking = 0.058549471295611keywords = ductal (Clic here for more details about this article) |
5/638. Well-differentiated adenocarcinoma, gastric foveolar type, of the extrahepatic bile ducts: A previously unrecognized and distinctive morphologic variant of bile duct carcinoma.Two examples of a rare but distinctive morphologic variant of extremely well-differentiated adenocarcinoma of the extrahepatic bile ducts are reported. One tumor arose in the common bile duct of a 51-year-old man; the other arose in the common hepatic duct of a 27-year-old man. Both tumors were composed predominantly (>95%) of gastric foveolar-type epithelium. Because of their bland nuclear features, low mitotic index, and focal polypoid and lobular architecture, they were initially confused with adenomas. Foci of less-differentiated adenocarcinoma and perineural invasion present in the deep portions of the tumors facilitated recognition. The neoplastic cells and extracellular mucin were periodic acid-Schiff- and alcian blue-positive. By immunohistochemistry, the tumor cells expressed cytokeratins 8 and 20 as well as cathepsin d, as reported in normal foveolar cells. Likewise, p53 overexpression was documented immunohistochemically in both adenocarcinomas, which also stained with the Ki-67 antibody. Despite the well-differentiated nature of the neoplasms and their deceptively benign microscopic appearance, one patient developed recurrence and liver metastasis 5 years after surgery. The other patient is disease-free 2 years following a segmental resection of the common hepatic duct, cystic duct, and gallbladder. The cell phenotype of these tumors can be explained by the ability of the bile duct epithelium to differentiate along gastric cell lines.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/638. Coexisting carcinoid tumors in familial adenomatous polyposis-associated upper intestinal adenomas.Upper gastrointestinal polyps and extraintestinal tumors are well recognized in association with familial adenomatous polyposis (FAP). Although carcinoid tumors have been reported in association with sporadic colonic neoplasms and ulcerative colitis, to date, carcinoids have not been reported in association with FAP. We report a patient with FAP who has recurrent carcinoid tumors located at the bases of duodenal adenomas. The genetic basis of carcinoid neoplasms is still uncertain. This report may represent the clinical effect of the APC gene mutation on the enterochromaffin cell line manifesting as recurrent carcinoid tumors in physical association with intestinal adenomas. Future genetic analysis and epidemiological studies may be of value in determining whether a true association exists.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
7/638. Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma.Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/638. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/638. A rare chimeric TLS/FUS-CHOP transcript in a patient with multiple liposarcomas: a case report.Myxoid liposarcomas harbor a unique and specific t(12;16)(q13,p11) chromosomal translocation. The breakpoint has recently been identified, and involvement of the TLS/FUS gene on chromosome 16 and the CHOP gene on chromosome 12 was demonstrated. We report a case of a 45-year-old woman who developed multiple malignant lipomatous tumors of unknown origin and myxoid/round cell histology at different locations. To examine the diagnostic potential of this translocation and to develop a hypothesis on the origin of the tumors, we used cytogenetic and molecular cytogenetic methods (reverse transcription polymerase chain reaction, RT-PCR). We identified a chimeric rna transcript in the second recurrence in the thigh/groin, as well as in another tumor in the mediastinum, which has an additional sequence of 33 bp, known as fusion transcript type III. cytogenetic analysis of another tumor in retroperitoneal space revealed a rare type of unbalanced translocation der(16)t(12;16). We hypothesize that these tumors are metastases rather than multicentric tumors. The detection of the chimeric message in the present case is not only useful for differential diagnosis, but also for analyzing the origin of multiple neoplasms.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/638. Sarcoma botryoides of the cervix. Report of a case with cytopathologic findings.BACKGROUND: Cytologic findings of sarcoma botryoides were still equivocal because sarcoma botryoides of the uterine cervix is an extremely rare neoplasm, and few cases have been reported to date. CASE: A 17-year-old female was diagnosed with sarcoma botryoides of the uterine cervix. The entire vaginal canal was occupied with polypoid masses, which arose from the anterior lip of the uterine cervix, and the tumor was classified as group I (Intergroup rhabdomyosarcoma Study). After wedge resection and six courses of combination chemotherapy, the tumor recurred in the same location of the cervix as the primary lesion. touch smear of the polypoid mass formed loose clusters and also showed short spindle cells in a necrotic background. The nucleus of the tumor cells had a thin nuclear membrane, fine chromatin pattern and partly clear nucleolus, showing mild nuclear atypia. Immunohistochemically, some of the tumor cells showed positive staining for myoglobin and desmin. CONCLUSION: The cytologic findings of sarcoma botryoides of the female genital tract are typical features of nonepithelial malignant tumor. Immunohistochemical study is useful for the diagnosis of rhabdomyosarcoma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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