弥漫大B细胞淋巴瘤中CD27的表达及临床意义

目的探讨弥漫大B细胞淋巴瘤(diffuse large B cell lymphoma,DLBCL)中CD27的表达及其临床意义。方法采用免疫组化EnVision法检测143例DLBCL组织中CD27蛋白的表达;应用FISH技术检测DLBCL组织中MYC、BCL-2、BCL-6基因重排情况。结果143例DLBCL中,CD27蛋白阳性者46例,阳性率为32.2%。CD27阳性组中BCL-2重排阳性率(17.4%)明显高于CD27阴性组(4.1%),差异有统计学意义(P<0.01)。CD27阳性组病死率(30.4%)明显高于CD27阴性组(15.5%),差异有统计学意义(χ^2=4.326,P=0.038)。CD27阳性者与阴性者的Kaplan-Meier生存曲线差异有显著性(χ^2=4.485,P=0.034),阳性组生存期较短。结论CD27高表达与DLBCL预后密切相关,可作为临床预后评价的指标之一。

Clinical and pathological studies of borderline gastrointestinal stromal tumors

Background Borderline gastrointestinal stromal tumors （GISTs） are intermediate tumors between benign and malignant variants; however, the clinical and pathological features of borderline GISTs remain poorly defined. This study aimed to characterize GISTs and to identify a set of borderline criteria for practical use. Methods Medical records and specimens of 840 patients from 12 hospitals were retrospectively examined. Totally 485 and 76 patients with any of the parameters predictive of either malignant or benign tumors were excluded. The Kaplan-Meier method was used to calculate disease-free survival and overall survival rates. Results Among the remaining 279 borderline GIST patients, 223 were followed up for 1 to 31.48 years. Two patients developed local recurrence, and both were cured by subsequent operations alone. The 5-year disease-free survival and overall survival rates were 99% and 100%, respectively. Morphologically, borderline GISTs typically exhibited moderate cellularity, and subsets of them also showed moderate atypia, low mitotic activities, or large tumor size. According to the National Institutes of Health （NIH） consensus criteria, the risk levels of the 279 GISTs were classified to be very low to high. However, the disease-free survival rates were not significantly different among these risk groups （P=0.681）. Conclusions The proposed borderline GIST criteria in the current study may complement the existing NIH criteria, based primarily on tumor size and mitotic count, in the evaluation of the biological behaviors of GISTs. Since a subset of borderline GISTs with high risk level showed favorable outcome, the introduction of the borderline GIST system may avoid overdiagnosis and over therapy.

Clinical and histopathological alterations of lymphangioleiomyomatosis in 14 Chinese patients

Background Lymphangioleiomyomatosis （LAM） is a rare disease that predominantly affects young females. It is considered as an ＂orphan＂ life-threatening disease of unknown etiology, with uncertain clinical prognosis, and no effective treatment. LAM can arise sporadically or in association with tuberous sclerosis complex （TSC）, an autosomal inherited syndrome characterized by hamartoma-like tumor growth and pathologic features that are distinct from manifestations of pulmonary LAM. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax, and chylous fluid collections. Methods Fourteen cases of LAM from Zhongshan Hospital, Fudan University are reviewed, twelve were confirmed by lung biopsy, one by retroperitoneal lymphangioleiomyoma resection, and one by autopsy. Results All 14 patients were women, aged 18 to 69 years （mean 43.3 years, median 46.5 years）. Haemoptysis （57.1%） and chylothorax （35.7%） were more frequent than those described in previous case series. Extrapulmonary findings such as renal angiomyolipoma （AML）, enlarged abdominal lymph nodes, liver AML and retroperitoneal lymphangioleiomyoma were seen in 21.4%, 14.3%, 7.14% and 7.14% in 14 cases respectively, which is remarkably lower than in the previously reported. Abnormal smooth muscle cells （LAM cells） were found to line the airways, bronchioles, lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts. There were some surprises in the autopsy case as several LAM cell emboli were found in the veins of mediastinum lymph nodes; LAM cells were found to be disseminated in soft tissues adjacent to the ilium. Conclusions Women with unexplained recurrent pneumothorax, tuberous sclerosis, or a diagnosis of primary spontaneous pneumothorax or emphysema in the setting of limited or absent tobacco use should undergo high-resolution computed tomography （HRCT） scan screening for LAM. Routine abdominal and pelvic imaging examinations should be performed to detect extrapulmonary involvement. The autopsy studies histologically suggested that LAM could be a multisystemic disease and LAM cells might possess metastatic potential.

New prognostic parameters for very-low-risk gastrointestinal stromal tumors

Background According to the National Institutes of Health consensus criteria, gastrointestinal stromal tumors （GISTs） smaller than 2 cm in diameter with less than 5 mitotic figures per 50 high-power fields are considered very-low-risk GISTs but these two indices alone cannot reliably predict a benign outcome during long-term follow-ups. Therefore, identification of additional parameters for predicting the clinical behavior of GISTs is necessary. Methods Eighty-eight patients with tumors that meet the very-low-risk GIST criteria were retrospectively investigated and morphological parameters of tumors associated with the biological behavior of very-low-risk GISTs were evaluated in the present study. The Kaplan-Meier method was used to calculate disease-free survival rates. Results Eighty-one patients were followed up for one to 16.3 years. Five cases of relapses were identified in the patients. Distinctive infiltrative growth patterns such as muscularis propria, muscularis mucosa, or nerve infiltration were identified by microscopy in 4 patients with the relapse, including three patients who experienced multiple recurrences. The infiltrative growth features became more obvious in multiple recurrent tumors compared to the single recurrent tumor, while only one developed relapse in 76 patients without infiltration （P 〈0.0001）. Conclusion Microscopic infiltrative growth patterns of the tumor may have clinical significance in predicting the prognosis of very-low-risk GISTs.

Mucins in the diagnosis and differential diagnosis of pancreatic cystic neoplasms: report of 40 cases

Cystic neoplasms of the pancreas account for 10% to 15% of all cystic pancreatic lesions.The majority （85% to 90%） of cystic lesions of the pancreas are pseudocysts. Although cystic neoplasms of the pancreas are rare, they range from benign to malignant neoplasms. The clinical challenge is the differential diagnosis and management of the cystic neoplasms, which represent 10% to 25% of primary pancreatic neoplasms. Pancreatic neoplasms and tumour like lesions with cystic features have been recently reviewed. The incidence of pancreatic cystic neoplasms reported is variable. Because there is no large, systematic study on tne cases from China comparing the incidence and biology of cystic neoplasms of pancreas to that of Western series, we reviewed all the cases of cystic neoplasms from Zhongshan Hospital over 6 years. Most of the neoplasms in our series were classified according to the recent World Health Organization （WHO） classification.

Mucin profile of the pancreatic mucinous cystic neoplasms

Mucinous cystic neoplasms （MCNs） of the pancreas are a distinct entity, account for 1% of pancreatic exocrine tumors. MCNs can be classified histologically as adenomas, borderline tumors, or carcinomas. Because several evidences showing that mucinous cystadenomas are potentially malignant and may transform into cystadenocarcinomas, particularly if treated by drainage, these tumors should be identified accurately.