Abnormal venous structures in salivary gland tumors: vasculature characteristics of pleomorphic adenoma

To clarify the diagnostic significance of abnormal venous structures present in salivary gland tumors, we examined 21 pleomorphic adenomas, 14 Warthin tumors, 1 oncocytic adenoma, 3 myoepitheliomas, 7 basal cell adenomas, 5 mucoepidermoid carcinomas, and 6 adenoid cystic carcinomas. Verhoeffvan Gieson staining was carried out and the morphology of the veins within the tumors was observed microscopically. Branching veins, thickened intima of the veins, discontinuous elastic membrane and multilayered elastic membrane were seen in 71.4%, 76.2%, 47.6% and 85.7% of pleomorphic adenomas, respectively, and were abundant and easily found in most cases. The abnormal venous structures were also found in other salivary gland tumors examined, but they were few in number and lacked variety. Elastic fibers extending radially into the surrounding stroma were seen in 66.7% of pleomorphic adenomas, and were not seen in other salivary gland tumors. Our results showed that a variety of abnormal venous structures are more abundant and more easily found in pleomorphic adenoma compared with other salivary gland tumors, and, in particular, that perivascular radiating elastic fibers are characteristic of pleomorphic adenoma. We emphasize that the presence of perivascular radiating elastic fibers may be helpful in diagnosing pleomorphic adenoma in small biopsy specimens.     

含破骨巨细胞样细胞的胰腺多形性癌1例及文献复习

目的 探讨含破骨巨细胞样细胞的胰腺多形性癌的临床病理特征。方法 对1例含破骨巨细胞样细胞的胰腺多形性癌进行临床、病理组织学和免疫组织化学观察。结果 患者为58岁女性，因中上腹部持续性隐痛2个月余就诊，B超、CT检查发现胰腺占位。切除标本示实性结节状肿块，灰白、灰黄色，存灶性坏死。镜下见肿瘤由低分化腺癌伴梭形肉瘤样细胞构成，两种细胞均示CK、Vim、α-AT阳性。可见多灶性巨细胞瘤样结构，由单核瘤细胞和形态上良性的破骨巨细胞样细胞构成；前者见CK散在阳性，Vim和α-AT阳性；后者仅见CD68和Vim阳性。结论 含破骨巨细胞样细胞的胰腺多形性癌是多形性胰腺癌的一种亚型，破骨巨细胞为反应性细胞，非肿瘤成分。     

Spontaneous infarction of a pleomorphic adenoma in parotid gland: diagnostic problems and review

Although infarction of parotid gland pleomorphic adenoma (PA) following fine-needle aspiration (FNA) has been well-documented, spontaneous infarction of PA has remained as an uncommon entity in the literature. To our knowledge, we report the second case of spontaneous infarction occurring in a parotid gland PA. A 44-yr-old man presented with a 2-yr history of slowly enlarging right parotid mass, which had become painful 1 mo before performing FNA biopsy. Smears revealed abundant necrotic debris, atypical squamous cells, and small cells with dark nuclei suggestive of a carcinoma. Histologic examination of the tissue fragments demonstrated degenerated clusters of cells and chondromyxoid matrix. The parotidectomy specimen had features consistent with those of an infarcted PA. Although appears to be rare, spontaneous infarction of PA should be considered in the differential diagnosis, since necrosis may mimic carcinoma and cause misinterpretation of necrosis as an indication of malignancy, in an otherwise benign salivary gland neoplasm.     

3种恶性成分的腮腺区多形性腺瘤癌变1例报告及文献复习

目的：探讨1例含有3种恶性成分的腮腺区多形性腺瘤癌变（CXPA）患者的诊断和预后,提高临床医生对该病的认识。方法：患者因左腮腺区肿物30年余伴疼痛1个月入院,经腮腺区彩超检查后行左腮腺区肿物切除术。结果：该患者手术过程顺利,术后病理诊断为CXPA,包含非特异性腺癌、导管癌和肌上皮癌3种恶性成分。结论：同时含有多种恶性成分的CXPA临床罕见,诊断困难,预后差,临床医师应提高对该病的认识。     

自噬调控因子mTOR和Beclin1在良恶性多形性腺瘤中的表达及意义

目的 研究自噬调控中的主要因子西罗莫司靶蛋白(mTOR)及Beclin1在良、恶性多形性腺瘤中的表达及意义.方法 采用免疫组织化学显色检测Beclin1在35例腮腺瘤多形性腺瘤(CPA),37例腮腺良性多形性腺瘤(PA)和20例正常腮腺组织中的表达情况.用蛋白免疫印迹法(Western blot)测定p-mTOR、Beclin1在CPA、PA及正常腮腺组织中的表达情况.结果 p-mTOR在正常腮腺组织,PA,CPA中的阳性表达呈递增的趋势,三者间差异有统计学意义(P<0.05);Beclin1在3种组织中表达呈递减趋势,三者间差异也有统计学意义(P<0.05).结论 mTOR信号因子在CPA组织中过度表达,而Beclin1在CPA组织中的阳性表达受到抑制.     

Radiation-induced undifferentiated pleomorphic sarcoma after radiation therapy for a desmoid tumour

Radiation-induced sarcoma is a long-term complication of radiation therapy. The most common secondary neoplasia is the undifferentiated pleomorphic sarcoma, which is usually described in the deep soft tissue of the trunk or extremities. Radiation-induced sarcomas have a poor prognosis. An early diagnosis and management are needed to improve the survival rate of such patients. We presently report a case of a radiation-induced undifferentiated pleomorphic sarcoma of the left gluteus maximus muscle, which developed 25 years after an initial diagnosis of aggressive fibromatosis and 21 years after a tumour recurrence. This case study illustrates the risk of developing a sarcoma in a radiation field and the need for long-term follow-up after radiation therapy. Unnecessary radiation therapy, in particular in the case of benign conditions in young patients, should be avoided.     

Pharmacological therapies for Liposarcoma

Introduction: Liposarcoma (LS) is one of the most common adult soft tissue sarcomas (STS). For metastatic disease, systemic treatment options were historically represented by standard cytotoxic chemotherapy. More recently, innovative therapies have been introduced and they are currently part of the therapeutic armamentarium, positively impacting disease control and patients’ quality of life. Moreover, in the last decade, a better understanding of the molecular characteristics of each STS subtype allowed to detect new potential targets and develop novel, biology-driven compounds at different stages of testing.

Areas covered: This review is focused on LS, retracing their pharmacological management, starting with a summary of results achieved with standard chemotherapy, then moving to a deeper analysis on data obtained with new, approved therapies and finally reporting an update on ongoing clinical trials, thus providing an overview on the current scenario and outlining how it might evolve in the coming years.

Expert commentary: Important strides have been made in the knowledge and treatment of LS. Peculiar molecular features and fundamental signalling pathways represent nowadays druggable targets for novel therapies. However, predictive biomarkers still need to be identified in order to better select the target population, to possibly test combinations of drugs, with the ultimate goal of improving outcomes.