Clinicopathological analysis of malignant lymphoma in Taiwan, defined according to the World Health Organization classification

We report the distribution and clinicopathological characteristics of malignant lymphomas in Taiwan, defined according to the WHO classification. Data including age and gender of the patients, clinical staging and disease courses were collected for 598 cases of malignant lymphomas. The results showed that the epidemiological characteristics of malignant lymphomas in Taiwan are similar to those in other Asian countries except for a lower incidence rate of T/NK cell lymphoma.     

Primary hepatic non-Hodgkin's lymphomas: case report and review of the literature

A 31-yr-old man presented with a 1-wk history of fever, chills, weakness, headaches, and a significant 20-lb weight loss over the preceding 2 months. His past medical history was relevant for liver amebiasis during childhood. Two days before admission, the patient noticed jaundice. He denied abdominal pain or other GI symptoms, and there was no history of alcohol intake, medications, or illicit drugs. His physical examination revealed generalized jaundice, hepatosplenomegaly, and bilateral leg edema. Neurologically, the patient was agitated, with periods of disorientation, and he had bilateral flapping. His blood tests revealed pancytopenia, renal failure, liver failure, and coagulopathy. Because the patient had a fever, hepatosplenomegaly, and pancytopenia, a further workup also included a bone marrow and liver biopsy. No conclusive diagnosis could be made from the above tests, and the patient died 5 days after admission. Postmortem evaluation, including flow cytometry and gene rearrangement in the tissue obtained from the liver, revealed large B cell lymphoma. This case illustrates an unusual presentation of hepatic non-Hodgkin's lymphoma. Current information regarding this entity is scant, mainly owing to its rarity. We present a review of the literature, including the incidence, presentation, treatment, and prognosis of primary hepatic lymphoma.     

Primary tonsillar tuberculosis in a pediatric patient: Case report and literature review

Rationale:Tuberculosis is an entity that usually affects the lungs, although extrapulmonary sites can also be involved. Tonsils are rarely affected, especially in the absence of pulmonary disease, primary tonsillar tuberculosis being a diagnostic challenge for the clinician.Patient concerns:We present the case of a 14-year-old female teenager, presented to our Pediatric Service with a 14-day history of dysphagia, odynophagia and left reflex otalgia associated with a 5 kg weight loss. Clinical examination revealed mild pharyngeal erythema, marked enlargement of the left tonsil infiltrating the lateral pharyngeal wall and the uvula and painful, mobile, nonadherent to deep bilateral latero-cervical adenopathy.Diagnosis:Positive interferon-gamma release assay (QuantiFERON-TB gold). Mantoux test reading was 16 mm.Interventions:During hospitalization, the patient received Clindamycin and Gentamicin for 3 days i.v., with discrete relief of symptoms and inflammatory markers. On the 4th day of hospitalization, treatment with Imipenem/Cilastin is started for 7 days in micro-perfusion, with tonsil hypertrophy decrease in size and favorable clinical evolution.Outcome:Tonsil hypertrophy decreased in size and patient had a favorable clinical evolution. At discharge, the patient was given a 6-month course of anti-tuberculous drug.Lessons:The particularity of this case is represented by the rarity of primary tuberculosis of tonsils in children, with unilateral involvement, displaying at the same time a common issue encountered in the current practice: the limitations and the difficult course of setting the diagnosis due to the involvement of relatives in the medical act.     

Distribution of lymphoid neoplasms in the Republic of Korea: Analysis of 5318 cases according to the World Health Organization classification

Compared with the West, the overall incidence of lymphoid neoplasms is lower, and the subtype distribution is distinct in Asia. To comprehensively investigate the subtype distribution with the age and sex factors, and temporal changes of subtype proportions, we re‐assessed all patients with lymphoid neoplasms diagnosed at a large oncology service in the Republic of Korea from 1989 to 2008 using the World Health Organization classifications. Of the total 5,318 patients, 66.9% had mature B‐cell neoplasms, 12.5% had mature T/natural killer (NK)‐cell neoplasms, 16.4% had precursor lymphoblastic leukemia/lymphoma (ALL/LBL), and 4.1% had Hodgkin's lymphoma. The most common subtypes were diffuse large B‐cell lymphoma (30.5%), plasma cell myeloma (14.0%), extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue type (MALT lymphoma; 12.4%), B‐cell ALL/LBL (11.3%), Hodgkin's lymphoma (4.1%), peripheral T‐cell lymphoma unspecified (4.0%), T‐cell ALL/LBL (3.9%), and extranodal NK/T‐cell lymphoma of nasal type (3.9%). Most subtypes showed male predominance, with an average M/F ratio of 1.3. Most mature lymphoid neoplasms were diseases of adults (mean age, 53.5 yr), whereas ALL/LBLs were of young individuals (mean age, 20.3 yr). When the relative proportion of subtypes were compared between two decades (1989–1998 vs. 1999–2008), especially MALT lymphoma has increased in proportion, whereas T/NK‐cell neoplasms and ALL/LBL have slightly decreased. In summary, the lymphoid neoplasms of Koreans shared some epidemiologic features similar to those of other countries, whereas some subtypes showed distinct features. Although the increase in incidence of lymphoid neoplasms is relatively modest in Korea, recent increase of MALT lymphoma and decrease of T/NK‐cell neoplasms and ALL/LBL are interesting findings. Am. J. Hematol., 2010. © 2010 Wiley‐Liss, Inc.     

The clinicopathological analysis of 303 cases with malignant lymphoma classified according to the World Health Organization classification system in a single institute of Taiwan

Several reports have shown a different distribution of malignant lymphoma (ML) in Asian and Western populations. The purpose of our survey was to elucidate whether there are substantial differences in the frequencies of subtypes of ML between different geographical areas. All entities diagnosed as ML between June 1995 and December 2007 were selected according to the 2008 World Health Organization (WHO) classification and searched for clinical outcomes. The cases were retrieved and reviewed by a panel of clinical haematologists and haematopathologists. A total of 303 patients with ML were identified for retrospective analysis. Of the 303 patients with ML, 278 patients (91.7%) had non-Hodgkin’s lymphoma (NHL), and 25 (9.2%) had Hodgkin’s lymphoma. Of the 278 patients with NHL, 223 (73.6%) had lymphoma of B-cell lineage, and 55 (18.1%) had lymphoma of T-cell lineage. One hundred and thirty-seven patients were diagnosed with diffuse large B-cell lymphoma, which was the most common B-cell lineage subtype and accounted for 45.2% of patients with NHL. Peripheral T-cell lymphomas were the most frequent subset of the T-cell neoplasms, comprising 10.6% of ML. Extranodal involvement was found in 125 (44.9%) of the 278 patients with NHL, and the lymph node was the site of primary involvement in 153 patients (55.1%). Fifty-nine (47.2%) of the 125 patients with extranodal presentation had gastrointestinal tract involvement. Outcome was worse in patients with extranodal NHL than in those with nodal NHL through the entire follow-up period; the difference in survival rates was significant. Our findings clarify the applicability and prognostic relevance of the WHO classification system and provide further information about the incidence of various lymphoma subtypes in Taiwan. Primary extranodal NHL was associated with a worse prognosis and distinct characteristics compared with nodal NHL. The outcome of different types of extranodal NHL should be investigated further.     

原发性粒细胞肉瘤1例并文献分析

目的:分析原发性粒细胞肉瘤(GS)的临床特征包括疾病的发病率,诊断流程,预后和治疗策略等。方法:对我院近期内确诊的1例原发于卵巢的GS和近20年MEDLINE文献关于原发性GS的资料87例进行总结分析。结果:分析资料完整的88例患者,其中65例确诊为原发性GS后转化为急性非淋巴细胞白血病(AN-LL),其余23例未发生转化目前仍在随访中。淋巴结,皮肤,泌尿生殖系统和消化系统是GS最常见的髓外发生部位;经典的GS肿瘤细胞形态具有不同阶段髓细胞分化的特点,免疫组化示MPO,NCAE(氯化醋酸AS-D萘酚酯酶),CD43和CD68等阳性;37例患者初诊时被误诊,大多数误诊为恶性淋巴瘤。传统的ANLL化疗方案疗效明显优越于恶性淋巴瘤方案和单独放疗。结论:减少原发性GS向ANLL转化并延长生存期,关键在于早期确诊和早期采用ANLL方案全身化疗。     

The new World Health Organization classification of lung tumours.

Tumour classification systems provide the foundation for tumour diagnosis and patient therapy and a critical basis for epidemiological and clinical studies. This updated classification was developed with the aim to adhere to the principles of reproducibility, clinical significance, and simplicity in order to minimize the number of unclassifiable lesions. Major changes in the revised classification as compared to the previous one (WHO 1981) include the addition of two pre-invasive lesions to squamous dysplasia and carcinoma in situ; atypical adenomatous hyperplasia and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Another change is the subclassification of adenocarcinoma: the definition of bronchioalveolar carcinoma has been restricted to noninvasive tumours. There has been substantial evolution of concepts in neuroendocrine lung tumour classification. Large cell neuroendocrine carcinoma (LCNEC) is now recognized as a histologically high grade non small cell carcinoma showing histopathological features of neuroendocrine differentiation as well as immunohistochemical neuroendocrine markers. The large cell carcinoma class has been enriched with several variants, including the LCNEC and the basaloid carcinoma, both with a dismal prognosis. Finally, a new class was defined called carcinoma with pleomorphic, sarcomatoid, or sarcomatous elements, which brings together a number of proliferations characterized by a spectrum of epithelial to mesenchymal differentiation. Immunohistochemistry and electron microscopy are invaluable techniques for diagnosis and subclassification, but our intention was to render the classification simple and practical to every surgical laboratory, so that most lung tumours could be classified by light microscopic criteria.     

Primary cardiac sarcoma: report of 3 cases and review of the literature

Primary cardiac sarcoma is a rare tumor with a poor prognosis. We report 3 cases with a review of literature about this disease. There were 2 males and 1 woman. The main symptoms were thoracic pain. The clinical features were various and the thoracic ultra sonography exam allowed the diagnosis in the 3 cases. All patients had surgical remove of their cardiac tumor followed by chemotherapy. All of them died within 13 to 36 months after the diagnosis. Primary cardiac sarcoma has a poor prognosis with a mean survival less than 12 months.     

原发于肺的淋巴瘤六例临床分析并文献复习

目的：探讨原发于肺的淋巴瘤的临床特点、诊断、治疗方法，以提高其诊断率。方法：回顾性地总结算1989年－2000年收住北京协和医院的原发于肺的淋巴瘤6例，并结合文献对其临床表现、影像学特点、支气管镜下表现、诊断及治疗方法进行分析。结果：原发于肺的淋巴瘤为少见的淋巴瘤，临床表现不特异，很难确诊。原发于肺的淋巴瘤主要症状为咳嗽（4／6）、发热（2／6）等。影像学可表现为单发或多发的结节或团块影、实变影等，早期无肺门和纵隔淋巴结肿大。支气管镜检查可见支气管狭窄，慢性炎症或大致正常。最终确诊需通过开胸手术、胸腔镜及经皮肺穿获取病灶组织，并结合病理及免疫组化检查。主要治疗手段为手术切除辅以放化疗。预后取决于淋巴瘤的恶性程度。结论：原发于肺的淋巴瘤临床表现不典型，易误诊，及时行必要的有创检查获取组织病理标本有利于早期诊断。     

Primary pancreatic paraganglioma:A report of two cases and literature review

Paraganglioma is a rare tumor of paraganglia,derived from neural crest cells in sympathetic or parasympathetic ganglions.Primary paraganglioma originating from the pancreas is rare.We report two patients with paraganglioma in the head of the pancreas,in whom computed tomography showed a sharply marginated,hypervascular tumor with cystic areas.Significant intratumoral vessels and early contrast filling of the draining veins from the mass were not found.Although the pancreatic paraganglioma was located at the pancreatic head,the bile ducts often revealed no dilation,and sometimes the main pancreatic duct was mildly dilated.These findings are helpful in differentiating pancreatic paraganglioma from other pancreatic neoplasms.It is often difficult to distinguish between nonfunctional pancreatic paragangliomas and pancreatic endocrine tumors.In many reports,pancreatic paragangliomas show the retroperitoneal extension of a paraganglioma into the pancreas rather than a true pancreatic neoplasm.In surgical treatment,we could select simple excision of the tumor rather than radical surgery.     

Primary hepatic angiosarcoma:A report of two cases and literature review

Primary hepatic angiosarcoma(PHA) is a rare malignancy that carries a poor prognosis. Of 1500 patients who underwent hepatectomy for primary hepatic tumors between 1994 and 2013 at our center,two patients were pathologically diagnosed with PHA. Clinical characteristics, treatment modalities,and outcomes of the two patients were collected and analyzed. Both patients underwent hepatectomy and had a postoperative survival time of 8 and 16 mo, respectively. A search of PubMed yielded eight references reporting 35 cases of PHA published between 2004 and 2013. On the basis of the presented cases and review of the literature, we endorse complete surgical resection as the mainstay definitive treatment of PHA, with adjuvant postoperative chemotherapy potentially improving survival. Palliative chemotherapy is an option in advanced hepatic angiosarcoma.     

Primary hepatic amyloidosis: a mini literature review and five cases report

Primary hepatic amyloidosis (PHA) is characterized by abnormal deposition of monoclonal immunoglobulin light chains (AL) in the liver. This rare condition is frequently undiagnosed or misdiagnosed and can be associated with poor prognosis. At present, the precise pathogenesis is not fully understood. Despite that hepatomegaly and elevated alkaline phosphatase (ALP) are present in most patients with PHA, no specific clinical markers have been identified. Staining of hepatic tissues with Congo Red is often regarded as the "gold standard". Pharmacological therapy should aim to rapidly reduce the supply of misfolded amyloidoge- nic AL. High-dose intravenous melphalan (HDM) and autologous stem cell transplantation (ASCT) appear to be the most appropriate therapy but controversies still exist.     

Austrian syndrome: report of two new cases and literature review

In April 2004, Rammeloo et al. reported a child-aortic-valve endocarditis following pneumococcal meningitis. Classically, the association of pneumococcal pneumonia along with meningitis and infectious endocarditis (IE) is known as Austrian syndrome. We assessed the incidence, clinical manifestations and follow-up of patients diagnosed with Austrian syndrome in Lugo (Northwest Spain) between 1987 and 2002. A computerized MEDLINE search was also performed for literature review. During the study period 165 Lugo patients met criteria for definite IE. Two of the 3 patients with definite streptococcus pneumoniae IE also met definitions for Austrian syndrome. The incidence of this syndrome in Lugo patients with definite IE was 1.2%. In the literature review 48 cases, including our two patients, were found. Complete clinical information was only available on 16 patients. Alcoholism constituted the most common predisposing factor. Aortic valve involvement and high grade of valve regurgitation leading to cardiac failure and the need of cardiac surgery during the admission were common in these patients. This review confirms that Austrian syndrome is a rare but severe disease. Early recognition of these patients is required to avoid ominous complications.     

Long-term outcomes for unselected patients with acute myeloid leukemia categorized according to the World Health Organization classification: a single-center experience

The actual utility of a new classification system of acute myeloid leukemia (AML) recently introduced by the World Health Organization (WHO) has not been thoroughly investigated yet. In this study, we evaluated long-term outcomes of unselected AML patients categorized according to the new WHO classification. Between 1990 and 2002, 109 adult AML cases were referred to our hospital. For the entire population, the median survival duration was 1.2 yr with a 5-yr survival rate of 31%. AML with recurrent genetic abnormalities accounted for 26%, AML with multilineage dysplasia for 29%, therapy-related AML for 13%, and AML not otherwise categorized for 32% of classifiable cases. Among the four groups, a significant difference was observed in terms of overall survival (P < 0.0001). Univariate analysis showed that six variables affected survival: cytogenetic risk, age, multilineage dysplasia, prior chemo/radiotherapy, type of treatment (intensive or palliative), and transplantation. However, in multivariate analysis no adverse prognostic impact of multilineage dysplasia and prior chemo/radiotherapy was detected (P = 0.4979 and 0.8702), whereas cytogenetic risk and patient age maintained their prognostic value (P = 0.0005 and 0.0100). These results indicate that outcomes for AML patients appear to be distinguished on the basis of the WHO classification, but the prognostic significance of multilineage dysplasia and prior therapy is lost after adjusting for cytogenetic risk and age. Our findings suggest that the WHO classification may be strengthened by greater emphasis on genetic/cytogenetic information.     

Case-control study of risk factors of myelodysplastic syndromes according to World Health Organization classification in a Chinese population

Risk factors of mydelodysplastic syndromes (MDS) remain largely unknown. We conducted a hospital-based case-control study consisting of 403 newly diagnosed MDS patients according to World Health Organization classification and 806 individually gender and age-matched patient controls from 27 major hospitals in Shanghai, China, to examine relation of lifestyle, environmental, and occupational factors to risk of MDS. The study showed that all MDS (all subtypes combined) risk factors included anti tuberculosis drugs [odds ratio (OR)(adj) = 3.15; 95% confidence interval (CI) = 1.22-8.12] as an independent risk factor, benzene (OR(adj) = 3.73; 95% CI = 1.32-10.51), hair dye use (OR = 1.46; 95% CI = 1.03-2.07), new building and renovations (OR = 1.69; 95% CI = 1.11-2.00), pesticides (OR = 2.16; 95% CI = 1.22-3.82), and herbicides (OR = 5.33; 95% CI = 1.41-20.10) as relative risk factors. Risk factors of MDS subtype refractory cytopenia with multiple dysplasia (RCMD) were benzene (OR(adj) = 5.99; 95% CI = 1.19-30.16) and gasoline (OR(adj) = 11.44; 95% CI = 1.31-100.03) as independent risk factors, and traditional Chinese medicines (OR = 2.17; 95% CI = 1.15-4.07), pesticides (OR = 2.92; 95% CI = 1.37-6.25), and herbicides (OR = 12.00; 95% CI = 1.44-99.67) as relative risk factors. Smoking tobacco was significantly associated with refractory anemia with excess of blasts (RAEB) (OR(adj) = 2.43; 95% CI = 1.02-5.77). Education is shown as an independent protective factor against all MDS (OR(adj) = 0.90; 95% CI = 0.83-0.99) and RCMD (OR(adj) = 0.89; 95% CI = 0.79-0.99). These findings suggest that multiple modifiable behavioral, environmental, and occupational factors play a role in MDS etiology, and various MDS subtypes may have different susceptibility.