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Aggressive Clinical Course (aggressive + clinical_course)
Selected AbstractsCytological features of signet-ring cell carcinoma of the lung: Comparison with the goblet-cell-type adenocarcinoma of the lungDIAGNOSTIC CYTOPATHOLOGY, Issue 3 2009Koji Tsuta M.D. Abstract Signet-ring cell carcinoma (SRCC) and goblet-cell-type adenocarcinoma (GCA) are mucin-producing lung adenocarcinomas. Primary SRCC shows an aggressive clinical course, whereas GCA shows infrequent distant metastasis, but more frequent intrapulmonary metastases resembling lobar pneumonia. To distinguish SRCC from GCA, this study investigated the respective cytological features of these lesions. We selected 10 cases each of SRCC and GCA from the archival imprint smears. We assessed them for the following 10 cytological features. Necrosis/debris was observed in 60% of the SRCC and 90% of the GCA. A mucinous background was observed in 10% of the SRCC and 90% of the GCA. Significant inflammation was observed in none of the SRCC and 80% of the GCA. Stromal cluster was observed in 30% of the SRCC and 70% of the GCA. Nuclear overlapping was observed in 50% of the SRCC and in all of the GCA. Single tumor cells were observed in 80% of the SRCC and 10% of the GCA. Honeycomb-like cluster was observed in none of the SRCC and 80% of the GCA. Prominent nucleolus was observed in 50% of the SRCC and 40% of the GCA. Nuclear membrane irregularity was observed in 70% of SRCC and 60% of the GCA. Nuclear pleomorphism was observed in all of the SRCC and none of the GCA. The cytological features of SRCC were the presence of single tumor cells and nuclear pleomorphism, whereas that of GCA were the presence of abundant mucin and significant inflammation in the background, and a honeycomb-like cluster. Diagn. Cytopathol. 2009. © 2009 Wiley-Liss, Inc. [source] Prognostic significance of soluble interleukin-2 receptor levels in patients with dilated cardiomyopathyEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 6 2003C. J. Limas Abstract Background Activation of T lymphocytes is thought to mediate myocardial dysfunction in dilated cardiomyopathy (CMP), probably through cytotoxic cytokines, but its value as a prognostic factor has not been evaluated. Methods For 2 years we prospectively followed 76 patients (65 males, 11 females, age 49 ± 7 years) with CMP and New York Heart Association(NYHA) Class II,III heart failure; left ventricular (LV) function was assessed echocardiographically. Thirty-three patients (28 males, five females, age 52 ± 6 years) with ischaemic heart disease (IHD) and similar NYHA and LV function characteristics were used as controls. Serum sIL-2R levels, peripheral blood lymphocyte proliferation (basal, + concanavalin A) and HLA-DQB1 genotyping was carried out in all patients. Results The CMP patients had increased sIL-2R levels (1259 ± 130 pg mL,1) compared with the IHD patients (703 ± 80 pg mL,1, P < 0·01, only 3 > 800 pg mL,1). In the CMP patients, there was a significant (r = +0·45, P= 0·04) correlation between sIL-2R and the LV end-diastolic diameter but not with the LV ejection fraction or NYHA Class. During the 24-month follow up, 17 of the CMP patients had an adverse clinical course (death, need for cardiac transplantation, or worsening heart failure). Of these, 14 (75%) had elevated (, 800 pg mL,1) sIL-2R levels (Group I) compared with only five (6%) with a stable clinical course (Group II). Neither [3H] thymidine incorporation into the peripheral blood lymphocytes nor the excess of HLA-DQB1-30 histidine homozygotes in the Group I patients (38% vs. 17%, P < 0·05) could predict the clinical outcome. Conclusion Increased sIL-2R levels in CMP patients are an independent predictor of a more aggressive clinical course. [source] Molecular cytogenetic characterization of proximal-type epithelioid sarcomaGENES, CHROMOSOMES AND CANCER, Issue 3 2004Elena Lualdi Proximal-type epithelioid sarcoma is a recently described soft-tissue tumor that is distinguished from conventional-type epithelioid sarcoma by a far more aggressive clinical course, frequent location in the proximal anatomic regions, and variable rhabdoid morphology. Because of their rarity and peculiar morphology, proximal-type epithelioid sarcomas frequently pose serious diagnostic dilemmas, being easily misdiagnosed as a variety of other malignant neoplasms. To date, the information available on the genetic alterations associated with this tumor entity has been confined to single conventional cytogenetic reports. In this article, we present the results of a conventional and molecular cytogenetic analysis of six proximal-type epithelioid sarcomas. Spectral karyotyping analysis of these cases deciphered the characteristics of several marker chromosomes and complex translocations, leading to the recognition of recurrent rearrangements. The most frequently involved chromosome arm was 22q, and the identification of two cases with a similar translocation, t(10;22), suggests a role for one or more genes on chromosome 22 in the pathogenesis of this tumor and provides an opportunity for finely mapping the translocation-associated breakpoints. Chromosome arm 8q gain was also a frequent event and correlated with gain of MYC gene copy number, as demonstrated by fluorescence in situ hybridization. A review of both cases reported in the literature and those presented in this study reinforced the involvement of chromosomes 8 and 22 and also indicated frequent rearrangements of chromosomes 7, 14, 18, and 20. © 2004 Wiley-Liss, Inc. [source] Low dose 2-CdA schedule activity in splenic marginal zone lymphomasHEMATOLOGICAL ONCOLOGY, Issue 4 2003R. Riccioni Abstract Splenic Marginal Zone Lymphoma (SMZL) is a rare clinicopathological entity among marginal zone lymphomas. SMZL is an indolent lymphoma usually treated by splenectomy. A subset of patients is characterized by a more aggressive clinical course and poor prognosis. Treatment of these cases and second-line therapy for relapsed patients have not been yet identified. We report 10 cases treated with cladribrine (5,mg/m2/week) for six courses. Six patients (60%) achieved partial response, two patients (20%) achieved a complete response and the two remaining patients did not respond and died as a result of progression of the disease. The treatment was well tolerated. A total of 60% of the patients had an overall survival rate of 48 months and 24 months progression-free-survival was achieved by 37% with a median time of progression-free-survival of 17 months. Interestingly, in addition to a relevant percentage of hematological remission, some patients also experienced a molecular remission. We conclude that this treatment is safe and well tolerated and is able to induce a substantial number of responses. Our results suggest that this schedule is well tolerated and could be an useful alternative to splenectomy. Copyright © 2003 John Wiley & Sons, Ltd. [source] Crohn's Disease runs a more aggressive course in young asian patientsINFLAMMATORY BOWEL DISEASES, Issue 1 2006Kelvin Teck Joo Thia MRCP Abstract Background: Crohn's disease is a heterogeneous inflammatory bowel disease. The impact of age at diagnosis on the clinical course of patients varies widely as reported in the Western literature. Using the Vienna Classification, we seek to determine whether young Crohn's disease patients in an Asian population followed a different clinical course than old patients. Methods: The case records of 100 Crohn's disease patients who were treated at the Inflammatory Bowel Disease Center, Singapore General Hospital, were studied retrospectively. The age group and location of disease and behavior according to the Vienna classification were determined at diagnosis. Results: A1 group (age <40 years) defined as "young" and A2 group (age ,40) defined as "old" contained 65 and 35 patients, respectively. Median age for the young group was 27.4 years and that for the old group was 52.6 years. Of the young patients, 66.7% flared at least once compared with 28.6% of the old patients, odds ratio of 5.0 (P < 0.001). Young patients were more likely to be steroid dependent (20.0% of A1 versus 8.6% of A2, P = 0.14), received azathioprine (38.5% of A1 versus 5.7% of A2, P < 0.001) and experienced complications (31% of A1 versus 20% of A2, P = 0.25)-numerically higher rates that did not reach statistical significance. There was no significant difference between the age groups for the location and behavior of disease as well as requirement for surgery. Conclusion: In this first Asian study looking specifically at the impact of age at diagnosis of Crohn's disease, we found that young patients underwent a more aggressive clinical course. [source] Extracutaneous transformation into a high-grade lymphoma: a potential pitfall in the management of patients with Sézary syndromeINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 3 2006Sonja Michaelis MD Background, Transformation into a high-grade lymphoma in cutaneous T-cell lymphoma (CTCL) occurs in approximately 25% of cases and is associated with an aggressive clinical course. Methods, We identified four cases of transformation of Sézary syndrome (SS) into pleomorphic T-cell lymphoma. Results, In all patients, transformation occurred first in the lymph nodes, an average of 43 months after the diagnosis of SS. These high-grade lymphomas were composed of CD30-positive (two patients) and CD30-negative (two patients) pleomorphic large cells. All patients died of lymphoma an average of 29 months after nodal transformation. Conclusion, Because of an apparently poorer prognosis, the early recognition of transformation, especially by lymph node biopsy, is important for adequate therapy. [source] Subcutaneous Panniculitis-Like T Cell Lymphoma Developing in a Patient with Chronic B-Cell Lympocytic LeukemiaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L Shahabi Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an unusual peripheral lymphoma most typically presenting with a cytotoxic (CD8-positive, TIA-1-positive) immunophenotype. SPTCLs may have an indolent or highly aggressive clinical course. Histologically, SPTCL may be notoriously difficult to diagnosis. Cases of SPTCL with a deceptively benign appearance similar to that of subcutaneous lupus erythematosus have been described. SPTCL associated with a concomitant systemic leukemia/lymphoma has not been documented in the literature. We report a case of SPTCL arising in a 65-year-old female with a well-established history of B-cell lymphocytic leukemia (BCLL). She presented with two months of recurrent fever and painless erythematous nodules on bilateral lower extremities that were clinically felt to be erythema nodosum. Initial biopsies demonstrated a polymorphous lobular infiltrate with neutrophils, karyorrhexis and lipomembranous change. An excisional biopsy demonstrated an atypical lymphoid population that expressed CD8 and TIA1. PCR analysis confirmed T-cell receptor gene arrangement. The patient was treated with systemic chemotherapy with resolution of her symptoms and complete remission. This is the first well documented case of SPTCL occurring in a patient with long standing B-CLL, and highlights the difficulty of establishing an unequivocal diagnosis of SPTCL. [source] Extracranially extended meningothelial meningiomas with a high MIB-1 index: A report of two casesNEUROPATHOLOGY, Issue 1 2004Shoko M. Yamada Meningiomas that extend from the meninges to the extracranial tissue and result in skull osteolysis have been known to take an aggressive clinical course. Two such cases in elderly patients are reported. Case 1 is an 82-year-old woman who had undergone removal of the parasagittal meningioma (meningothelial meningioma with 5% of MIB-1 index) 4 years and 6 months previously, developed recurrence of the tumor that extended to extracranial soft tissue. Biopsy obtained from the subcutaneous tissue showed an atypical meningothelial meningioma with 20% of MIB-1 index. In case 2 an 84-year-old man, who developed rapidly progressing dementia and gait disturbance, the MRI study revealed an intracranial-extraaxial right frontal tumor with an extracranial extension resulting in skull osteolysis. Pathological examination of the totally resected tumor identified meningothelial meningioma, but MIB-1 index of the intracranial portion of the tumor was less than 0.1%, while that of the extracranial portion was approximately 15%. Although the meningiomas presently reported failed to show histological features of malignancy, the high MIB-1 index indicated that they were rapidly growing tumors. In the present report it is considered that meningioma cells that invade the skull and extracranial tissue are biologically aggressive and require total resection, as long as the condition of the patients is feasible for surgery. [source] Blastic natural killer cell lymphoma arising from the mediastinum with terminal deoxynucleotidyl transferase expressionPATHOLOGY INTERNATIONAL, Issue 1 2001Kouichi Isobe Blastic natural killer (NK) cell lymphoma/leukemia is a relatively rare NK cell malignancy. We report the second case of blastic NK cell lymphoma arising from the mediastinum with an aggressive clinical course. The patient was a 63-year-old Japanese man with an anterior mediastinum tumor. The biopsy specimen showed diffuse proliferation of tumor cells with frequent mitotic figures and apoptotic bodies. Both angiocentric features and small foci of coagulative necrosis were found in this section. The tumor cells had medium to large nuclei with a fine chromatin pattern, inconspicuous nucleoli and scanty cytoplasm. The nuclear contour was oval to moderately irregular, showing slight pleomorphism as compared with typical lymphoblastic lymphoma. The tumor cells were positive for CD2, CD56 and terminal deoxynucleotidyl transferase, but negative for other T-cell antigens, B-cell antigens and myeloid markers. In situ hybridization for Epstein,Barr virus encoded small ribonucleic acid 1 was negative. [source] Pediatric ALK+ anaplastic large cell lymphoma with t(3;8)(q26.2;q24) translocation and c-myc rearrangement terminating in a leukemic phaseAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2007Sara Monaco Abstract Pediatric ALK-positive anaplastic large cell lymphoma (ALK+ ALCL) is usually associated with a favorable prognosis. ALK+ ALCL associated with a leukemic phase is uncommon, but has been associated with an aggressive clinical course and unfavorable prognosis. Overexpression of c-myc has been shown to be a consistent finding in ALK+, but not ALK-negative ALCL (ALK, ALCL), and the c-myc gene is considered a downstream target of deregulated ALK signaling. We describe a pediatric ALK+ ALCL with a leukemic phase at relapse. Similar to other rare cases described in the literature, it followed an aggressive clinical course despite multiple regimens of chemotherapy and bone marrow transplantation. Lymphoma cells showed aberrant ALK expression and c-myc overexpression. In addition to the characteristic t(2;5)(p23;q35) translocation, a t(3;8)(q26.2;q24) translocation was also present, and c-myc gene rearrangement was confirmed by FISH analysis. The findings in this case demonstrate the association of peripheral blood leukemic involvement and aggressive clinical course, and suggest that other factors, such as c-myc rearrangement, may be responsible for the aggressive clinical behavior in ALK+ ALCL. Am. J. Hematol., 2006. © 2006 Wiley-Liss, Inc. [source] Chordoma and chondrosarcoma: Similar, but quite different, skull base tumorsCANCER, Issue 11 2007Kaith Almefty BBA Abstract BACKGROUND Chordoma and chondrosarcoma of the skull base are frequently amalgamated because of similar anatomic location, clinical presentation, and radiologic findings. The chondroid chordoma variant has been reported to carry a better prognosis. The objective of the current study was to investigate the distinctions between these 3 entities. METHODS The data concerning 109 patients with chordoma, chondroid chordoma, and chondrosarcoma who were treated by a single surgeon with maximum surgical resection and frequently by adjunct proton beam radiotherapy between 1990 and 2006 were analyzed retrospectively. Pathologic distinction was established by cytokeratin and epithelial membrane antigen staining. Clinical, radiologic, pathologic, and cytogenetic studies were analyzed in relation to disease recurrence and death. RESULTS The average follow-up was 48 ± 37.5 months (range, 1,191 months). There were no reliable distinguishing clinical or radiologic features noted between the groups. Chondrosarcoma patients had a significantly better outcome compared with chordoma patients with regard to survival and recurrence-free survival (P = .028 and P < .001, respectively), whereas patients with chondroid chordoma had a poor outcome similar to chordoma patients with regard to survival and recurrence-free survival (P = .337 and P = .906, respectively). CONCLUSIONS Chordoma and chondrosarcoma differ with regard to their origin and histology, and differ markedly with regard to outcome. Chondroid chordomas behave in a manner that is clinically similar to chordomas, with the same prognosis. Both chordoma types demonstrate an aggressive clinical course and poor outcome after disease recurrence. The optimal treatment for all groups of patients involves radical surgical resection followed by high-dose radiotherapy in patients with chordomas. Radiotherapy may not be necessary in patients with low-grade chondrosarcoma. Cancer 2007. © 2007 American Cancer Society. [source] Breast carcinoma in pregnant womenCANCER, Issue 5 2003Assessment of clinicopathologic, immunohistochemical features Abstract BACKGROUND Breast carcinoma is one of the most common carcinomas in pregnant women. The incidence of breast carcinoma may increase in the future because of the trend toward delayed childbearing and increased screening. However, very few contemporary studies have attempted to identify the combined histopathologic and immunohistochemical features of breast carcinoma in these patients. METHODS The authors evaluated 39 patients with breast carcinoma occurring coincident with pregnancy. This was comprised of a critical histologic review and immunohistochemical evaluation to determine the status of prognostic and predictive markers including estrogen receptor (ER), progesterone receptor (PR), HER-2/neu, Ki-67, and p53. RESULTS The mean age at presentation was 33 years (range, 24,44 years). Densities and/or masses were noted on mammograms in 14 of 16 patients with available radiographic information. The primary tumors were a mean of 4.5 cm in greatest dimension (range, 0.1,13.5 cm). Two of the 39 patients had clinical (American Joint Committee on Cancer) Stage I disease, 19 patients had Stage II disease, 16 had Stage III disease, and 2 patients had Stage IV disease at the time of presentation. Histologically, high-grade invasive ductal carcinomas were found in 32 of 38 patients. The primary tumor was not available for review in one patient. A predominantly solid pattern of growth was observed in nine patients. Lymphovascular invasion was identified in 61% of cases. Ductal carcinoma in situ was identified in 72% of tumors and was high grade in all cases. Of the 25 patients tested, ER positivity was found in 7 patients, PR positivity was found in 6 patients, HER-2/neu positivity was found in 7 patients, and p53 positivity was found in 12 patients. The proliferation rate as shown by Ki-67 staining was high in 60% of the cases. Follow-up information was available for 35 patients and the mean follow-up period was 43 months (range, 2,163 months). Distant metastasis occurred in seven patients. The mean time to disease recurrence was 20.4 months (range, 10,33 months). Of 35 patients, 4 have died, 22 were alive with no evidence of disease, and 9 were alive with disease at the last follow-up. The remaining four patients died of unknown causes. CONCLUSIONS Pregnant women with breast carcinomas generally present with advanced-stage disease and the tumors have poor histologic and prognostic features. The findings from the follow-up indicated that these tumors do not follow a very aggressive clinical course as was proposed in earlier reports. Breast carcinomas occurring during pregnancy share many histologic and prognostic similarities with breast carcinoma occurring in other young women. Cancer 2003;98:1055,60. © 2003 American Cancer Society. DOI 10.1002/cncr.11614 [source] Staging and management of cutaneous T-cell lymphomaCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2006J. J. Scarisbrick Summary Cutaneous T-cell lymphoma (CTCL) accounts for two-thirds of cases of primary cutaneous lymphoma. Most variants of CTCL are indolent lymphoma, the most common being mycosis fungoides. In addition, Sézary syndrome, the leukaemic variant, has an aggressive clinical course. Accurate diagnosis and staging is critical in determining the prognosis of those with CTCL. The tumour, node, metastasis and blood stage needs to be documented and used to determine an overall stage from IA to IVB. Management of patients should be carried out by a multidisciplinary team. A full clinical examination should be made at all visits. Thorough investigations are needed at diagnosis and should be repeated during disease progression to allow initial staging and restaging. Treatment of patients with early-stage disease (IA,IIB) should be limited to skin-directed therapy. More advanced or resistant disease may be treated with systemic therapies such as extracorporeal photopheresis, immunotherapy, monoclonal antibody therapy, novel retinoids or chemotherapy, and where possible, patients should be entered into clinical trials. [source] | |||||||||||||||||||||||||