Biomedical subjects

Shih-Sung Chuang

Publications and source records attributed to Shih-Sung Chuang.

32 records · Page 2Linked to original sources

PubMed2004

Angioimmunoblastic T-cell lymphoma with cutaneous involvement: a case report with subtle histologic changes and clonal T-cell proliferation.

Angioimmunoblastic T-cell lymphoma is a nodal peripheral T-cell lymphoma that rarely involves the skin. We describe a 62-year-old Taiwanese man who developed a second relapse of angioimmunoblastic T-cell lymphoma with generalized erythroderma and numerous plaquelike and nodular lesions. Biopsy of the erythematous skin lesion demonstrated mild infiltrate of atypical small lymphocytes, some with clear cytoplasm. The lymphoid infiltrate was located mainly around skin appendages and in the upper dermis without epidermotropism. Immunohistochemically, these atypical lymphocytes expressed CD3. Polymerase chain reaction analysis for T-cell receptor gamma-chain gene rearrangement using paraffin section showed the same-sized monoclonal bands in the skin and 2 previous nodal biopsies. We conclude that the histologic features of angioimmunoblastic T-cell lymphoma involving skin may be very subtle, showing only mild lymphoid infiltrate. Awareness of the history of angioimmunoblastic T-cell lymphoma with ancillary studies, including clonality testing for T-cell receptor gene rearrangement, is crucial for reaching an accurate diagnosis.

Cell Proliferation↗

PubMed2003

Prenatal diagnosis of congenital mesoblastic nephroma in mid-second trimester by sonography and magnetic resonance imaging.

Although congenital mesoblastic nephroma (CMN) is a rare benign congenital renal tumor, it is the most common solid renal tumor in the newborn period. The most common presentation of congenital mesoblastic nephroma is polyhydramnios, and only one case with prenatal fetal hydrops has been previously reported. Prenatal diagnosis of CMN has previously been made on the basis of the findings of sonography in the third trimester, and magnetic resonance imaging (MRI)-based diagnosis has been reported recently. Here we report a case of prenatally diagnosed classical type CMN diagnosed at 22 + 3 weeks of gestation based on the findings of sonography and magnetic resonance imaging. The characteristic imaging findings in this case were fetal hydrops and polyhydramnios. To our knowledge, this is the youngest reported gestational age for prenatal diagnosis of CMN and it is the second case of CMN associated with fetal hydrops detected prenatally.

Adult↗

PubMed2003

T(11;18)(q21;q21) in gastric MALT lymphoma and diffuse large B-cell lymphoma of Chinese patients.

T(11;18)(q21;q21) occurs at variable frequencies in mucosa-associated lymphoid tissue (MALT) lymphomas of different sites. In gastric MALT lymphoma, it occurs in 30% cases and the translocation positive tumours are frequently at advanced stages and do not respond to Helicobacter pylori eradication. Intriguingly, t(11;18)(q21;q21) has been observed only rarely in mucosal diffuse large B-cell lymphomas (DLBCL), despite that at least a proportion of these tumours are believed to be the result of transformation of an MALT lymphoma. We previously examined t(11;18)(q21;q21) in gastric MALT lymphoma of Chinese in Taiwan and found the translocation in one of the three transformed MALT lymphomas studied. To extend this observation, we have examined t(11;18)(q21;q21) in a large cohort of gastric lymphomas of Chinese in China including 39 MALT lymphomas (12 with high-grade components) and 53 DLBCL by reverse transcription and PCR. Of these cases, RNA samples extracted from 36 MALT lymphomas including 10 cases with high-grade component and 46 DLBCL were adequate for detection of the translocation as judged by control RT-PCR of a house-keeping gene. T(11;18)(q21;q21) was found in 8/36 (22%) MALT lymphomas and 1/46 (2%) DLBCL. None of the MALT lymphoma with high-grade component showed t(11;18)(q21;q21). Our results suggest that t(11;18)(q21;q21) occurs only occasionally in transformed MALT lymphoma.

Adaptor Proteins, Signal Transducing↗

PubMed2003

High frequency of t(11;18) in gastric mucosa-associated lymphoid tissue lymphomas in Taiwan, including one patient with high-grade transformation.

t(11;18)(q21;q21), the most frequent chromosomal aberration of mucosa-associated lymphoid tissue (MALT) lymphoma, occurs in 30% of gastric patients. Although the translocation is often associated with an 'aggressive' course, it has not been described in transformed MALT lymphomas. We screened 15 gastric MALT lymphomas [three with concurrent or subsequent high-grade transformation and 11 diffuse large B-cell lymphomas (DLBCLs)] in Chinese patients for t(11;18). t(11;18) was found in 9/15 (60%) MALT lymphomas, but not in any DLBCLs. One patient, with subsequent high-grade transformation, showed the translocation in low- and high-grade lesions. t(11;18) was frequent in Chinese gastric MALT lymphomas and unusually one transformed lymphoma carried the translocation.

Adult↗

PubMed2003

Immunophenotyping of angioimmunoblastic T-cell lymphomas by multiparameter flow cytometry.

Angioimmunoblastic T-cell lymphoma (AITL) is a distinct form of peripheral T-cell lymphoma (PTCL) frequently involving lymph nodes, spleen and bone marrow, and is associated with systemic symptoms. Its histologic features may be subtle at an early phase and difficult to diagnose. Despite the success of flow cytometry (FCM) in diagnosing B-cell neoplasm, FCM has not been widely accepted as a useful method for establishing the diagnosis of PTCL. Recently, the neoplastic T-cells in AITL have been shown to express CD10. We prospectively applied multiparameter FCM immunophenotyping to three cases of histologically confirmed AITL and identified a small (5-7%) population of CD4+/CD10+ T-cells in two cases. In one case, the CD4+/CD10+ population lacked surface signals of CD3 and CD7, but strongly expressed CD2, whereas CD45 expression was very weak; partial loss of surface CD3 was observed in the other. None of the lymph nodes with reactive hyperplasia, B-cell lymphomas, or Hodgkin's lymphoma studied during the same time period contained the CD4+/CD10+ population. These findings suggest that addition of CD4/CD10 and CD3/CD10 to FCM immunophenotyping panels is useful in the diagnosis of AITL. To the best of our knowledge, this is the first report to demonstrate CD10-expressing T-cells in AITL by FCM.

Aged↗

PubMed2003

Ectopic cervical thymoma: a mimic of T-lymphoblastic lymphoma.

Ectopic cervical thymoma is a rare tumor that probably arises from ectopic thymic tissue trapped during migration of thymic primordia in the embryonic stage. To the best of our knowledge, only 20 cases have been reported. Our patient was a 68-year-old woman who had been suffering from a nodular lesion in the anterior neck for several years. She underwent nearly total right thyroidectomy. Eight years and nine months after surgery, she is free of tumor recurrence or metastasis. The tumor was nodular with fibrous septa, and was composed of varying numbers of epithelial cells and lymphocytes, corresponding to AB thymoma as classified by WHO. Using lymphoid markers only, it was initially misdiagnosed as T-lymphoblastic lymphoma. The lymphocytes expressed T-cell antigens and terminal deoxynucleotidyl transferase and exhibited high proliferation fraction. Awareness of ectopic cervical thymoma, careful microscopic examination, and a proper selection of antibodies for immunohistochemistry will lead to the correct diagnosis.

Aged↗

PubMed2003

Detecting clonal rearrangement in non-Hodgkin's lymphomas in Taiwan by polymerase chain reaction.

The detection of monoclonal expansions of the immunoglobulin heavy chain (IgH) or the T-cell receptor-gamma (TCRgamma) chain genes is an important supplement for the diagnosis of the non-Hodgkin's lymphomas (NHLs). Detection of monoclonality by polymerase chain reaction (PCR) method has offered an efficient approach for rapid diagnosis and monitoring of the therapeutic effects. Here we conducted a retrospective PCR clonality study on 49 cases of NHLs including 23 B-cell lymphomas (BCLs), 20 peripheral T-cell lymphomas (PTCLs), 6 natural killer (NK)/T-cell lymphomas and 3 reactive lymphoid tissues from southern Taiwan. Genomic DNAs from paraffin sections were extracted and analyzed by the IgH- and TCR-specific PCR reactions. The results showed that 20 of 23 (87.5%) BCLs exhibited IgH gene rearrangements and were all germline for TCRgamma. 15 of 20 (75.0%) PTCLs exhibited TCRgamma gene rearrangements while 1 case (5%) was positive for IgH gene rearrangement. The 6 NK/T-cell lymphomas and 3 reactive lymphoid tissues were all germline for either IgH or TCRgamma genes. Our results were similar to other Western reports in terms of sensitivity and cell-lineage specificity. This is the first large series of PCR clonality study of IgH and TCRgamma gene rearrangements on NHLs from Taiwan. We have confirmed that this rapid method is a sensitive diagnostic tool for NHLs.

Clone Cells↗

PubMed2003

Anaplastic large cell lymphoma--a rare disorder in southern Taiwan.

Anaplastic large cell lymphoma (ALCL) is a subgroup of non-Hodgkin's lymphomas with large lymphoma cells expressing CD30 antigen. This entity has rarely been reported in Taiwan. We performed a retrospective clinicopathologic study in a medical center in southern Taiwan during a 13-year period and identified 13 cases. There were 10 males and 3 females with a median age of 49 years old. Seven presented with pure nodal disease and 5 had bony involvement. The staging results were stage I (5 patients), II (1), III (1), and IV (4). The pathologic subtypes were common variant (10), lymphohistiocytic variant (2), and small cell variant (1). Eleven tumors were of T-cell lineage; 2, null-cell. Immunohistochemically, 5 tumors (38.5%) expressed cytotoxic markers, T-cell intracellular antigen-1 and/or granzyme B. Two tumors (15.4%) expressed anaplastic lymphoma kinase (ALK). Long-term follow-up information was available in 8 patients. The 2 patients with ALK-expressing tumors (37 and 49 years old) were free of disease for 61 and 54 months, respectively. The other 6 patients were either died of disease (5 patients) or experienced relapse with progressive disease (1). In conclusion, we reported the largest series of ALCL in Taiwan. We confirmed ALK-expressing ALCL carries favorable prognosis and ALK-negative ALCL has similar poor prognosis as non-anaplastic T-cell lymphoma. As compared to the previous reports from the West, our ALK positive rate was lower and the age of our ALK-positive patients was older. A larger national or multi-institutional study is needed for further characterization of ALCL in Taiwan.

Adolescent↗

PubMed2002

High incidence of t(11;18)(q21;q21) in Helicobacter pylori-negative gastric MALT lymphoma.

In approximately 5% to 10% of gastric mucosa-associated lymphoid tissue (MALT) lymphomas, evidence of Helicobacter pylori infection is absent, and their pathogenesis is poorly understood. We reviewed the clinical data and histology, and we examined t(11;18)(q21;q21) and BCL10 expression pattern in 17 such cases. In each case, the absence of H pylori was confirmed by negative serology and histology/immunohistochemistry. Five cases with stage I(E) disease were first treated with antibiotics, and none of them showed any endoscopic or histologic response. Review of the histology failed to identify any apparent difference between gastric MALT lymphomas with and without H pylori infection. Reverse transcription-polymerase chain reaction (RT-PCR) showed t(11;18)(q21;q21) in 9 (53%) of 17 cases, more frequent in lymphomas at stage II(E) or above (5 of 6) than those at stage I(E) (3 of 10). Two t(11;18)(q21;q21)-positive lymphomas were treated by partial gastrectomy and more than 16 years later showed lymphoma relapse in the gastric stump with dissemination to other mucosal sites, poorly responsive to therapy. BCL10 nuclear expression was observed in 7 of 8 t(11;18)(q21;q21)-positive cases and 4 of 7 t(11;18)(q21;q21)-negative cases, including one case suspicious for a BCL10-involved chromosomal translocation. Our results show that t(11;18)(q21;q21) occurs at a high frequency in H pylori-negative gastric MALT lymphomas. Translocation-positive gastric MALT lymphomas tend to be aggressive, and patients with such lymphomas might benefit from prompt treatment and close follow-up.

Adaptor Proteins, Signal Transducing↗

PubMed2002

Primary bony peripheral T-cell lymphoma mimicking nasal type NK/T-cell lymphoma: a case report.

Primary bony lymphomas are rare, and nearly all are high-grade B-cell lymphomas. Natural killer (NK)/T-cell lymphomas are highly aggressive lymphomas of NK- or T-cell lineage with predominant extranodal presentation and are divided into nasal and nasal-type (extra-nasal). We report a primary bony peripheral T-cell lymphoma mimicking NK/T-cell lymphoma, nasal type. A 22-year-old Taiwanese male presented with a frontal skull bone mass noted for 3 weeks, and received craniectomy with tumor removal. His tumor showed extensive coagulative necrosis with angioinvasion by large lymphoma cells expressing CD2, CD8, CD16, CD43, CD45, CD45RO, CD56, T-cell intracellular antigen-1, and granzyme B, but not CD3, CD4, CD20, CD57, CD68, and betaF1. In situ hybridization for Epstein-Barr virus-encoded mRNA was negative. Polymerase chain reaction study of formalin-fixed tissue showed clonal rearrangement of the T-cell receptor-gamma chain gene. The diagnosis was peripheral T-cell lymphoma, unspecified subtype. The initial stage was I(EA). His lymphoma was refractory to chemotherapy, and bony metastases developed in the right iliac bone 2 months later. He died of disease after 6 months without autopsy. We emphasize the importance of detailed immunohistochemical and gene rearrangement studies for the classification of malignant lymphomas via a very rare primary bony lymphoma of peripheral T-cell subtype.

Adult↗

PubMed2002

Clinicopathological features of primary intestinal lymphoma in Taiwan: a study of 21 resected cases.

In Taiwan, primary intestinal lymphomas (PIL) have rarely been reported and characterized. Using WHO classification, we retrospectively studied the clinicopathological features of PIL cases surgically resected in Taiwan. There were 21 cases, 14 males and seven females, with a median age of 66. The most common symptom at presentation was abdominal pain (n = 14; 66.7%). Six (28.6%) cases showed perforation and two (9.5%) intussusception. Two patients had multicentric tumors. The most common location was ileum (n = 11, 52.4%). Twenty cases (95.2%) were of B-cell lineage, and one (4.8%) was of T-cell lineage. These cases were classified as diffuse large B-cell lymphoma (DLBL) (n = 18; 85.7%), Burkitt lymphoma (n = 2; 9.5%), and enteropathy-type T-cell lymphoma (EATL) (n = 1; 4.8%). One case was lost to follow-up. The 1- and 2-yr survival rates of the remaining 20 patients were 44.4% and 26.7%, respectively. In conclusion, we describe the clinicopathological findings of a rare case of appendiceal DLBL and another one of ileal EATL, that have never been reported in Taiwan previously. We found that in Taiwan PIL occurred in the elderly, with a male predominance, showing a relatively aggressive clinical course, and a pattern similar to that seen in western countries, except for the absence of multiple lymphomatous polyposis.

Adult↗

PubMed2002

Primary brain lymphoma: a report of eight cases from a medical center in southern Taiwan.

BACKGROUND: Primary brain lymphoma (PBL) in Taiwan has been reported only in three series with very limited immunophenotypic characterization. METHODS: We retrospectively studied PBL cases with history review, immunohistochemistry, and in situ hybridization (ISH) for Epstein-Barr virus-encoded mRNA (EBER) from a single institution in southern Taiwan during 1989-2000. RESULTS: We found eight cases of PBL including four males and four females with mean age of 64.1 years and median of 63.0. The major presenting symptoms were headache, poor memory, slurred speech, and hemiplegia in three patients each. All patients had stage I solitary tumor. Half of the patients received tumor excision, the other half, stereotactic biopsy. Seven cases were of diffuse large B-cell type (DLBL), with expression of bcl-2 in six cases. They were all negative for CD5, CD10, bcl-6, and EBER. The eighth patient had anaplastic large cell lymphoma (ALCL) of T-cell phenotype with expression of cytotoxic markers and was positive for EBER. Two were lost to follow up. The median follow-up time for the remaining six was 11.2 months (range, 5.5 - 25.0). They all received radiotherapy with initial complete remission. Two died of the disease, another of cardiopulmonary failure, and the other of stroke or recurrence. The remaining two were free of disease for 9.6 and 25.0 months after radiotherapy alone. The 1-year survival rate was 60%. CONCLUSIONS: We have fully characterized eight cases of PBL, including seven DLBLs and one ALCL, in southern Taiwan that occurred in an older age group. Old age, immunophenotype (bcl-2-positivity and bcl-6-negativity), and lack of systemic chemotherapy were probably responsible for the shorter survival as compared to other studies. Radiotherapy seems to be effective for inducing complete remission and even long-term survival in some patients, however, systemic chemotherapy should be administered to prevent recurrence and to achieve long-term survival.

Aged↗

PubMed2002

High dose chemotherapy and allogenic peripheral blood stem cell transplantation for multiple myeloma evolving from intra-abdominal plasmacytoma.

Solitary plasmacytomas include extramedullary plasmacytomas and those found in the bone. Seventy percent of patients are male and the median age is 50-55 years, younger than that for plasma cell myeloma. Most solitary plasmacytomas of bone eventually evolve to plasma cell myeloma within 2-10 years, while the extramedullary ones do so infrequently. We present an unusual case of intra-abdominal plasmacytoma in a young woman which was misdiagnosed and treated as T cell lymphoma initially. Typical manifestations of plasma cell myeloma appeared one year later. High dose chemotherapy followed by allogeneic peripheral stem cell blood transplantation (allo-PBSCT) was given. Relapse in skin occurred one year after allo-PBSCT, and was treated with wide excision and local irradiation. The patient was well and alive without evidence of disease 4 years after wide excision of the recurrence of chest wall solitary plasmacytoma and local radiotherapy.

Abdominal Neoplasms↗

PubMedDate not supplied

Acute myelomonocytic leukemia with abnormal eosinophils: a case report with multi-modality diagnostic work-up.

Acute myeloid leukemia (AML) with recurrent genetic abnormalities often carries a favorable prognosis. AML with inv(16)(p13q22) occurs predominantly in younger patients and usually shows granulocytic and monocytic differentiation with abnormal eosinophils. It is referred to as acute myelomonocytic leukemia with abnormal eosinophils (AMML Eo). We report a case in a 27-year-old man with leukocytosis (10.6 x 10(3)/microL with 34% blasts), thrombocytopenia and splenomegaly. Marrow aspiration showed 47% blasts and 33% eosinophils, of which 19% were morphologically abnormal with both eosinophilic and basophilic cytoplasmic granules. Cytochemically, the blasts were positive for myeloperoxidase while the granules of abnormal eosinophils were positive for naphthol ASD chloroacetate esterase. With flow cytometric immunophenotyping the blasts expressed CD13, CD33, CD117, myeloperoxidase and CD34. Marrow trephine showed 90% cellularity with 40% blasts expressing CD34, CD117, and myeloperoxidase on immunohistochemistry. Chromosomal analysis reveled a karyotype of 46, XY, inv(16)(p13q22). This case illustrates a typical AMML Eo confirmed by a multi-modality diagnostic approach including morphology, cytochemistry, flow cytometry, immunohistochemistry, and conventional cytogenetic study.

Adult↗