应用最大似然法鉴别诊断周围型肺癌、肺错构瘤和结核球的初步研究

目的:探讨应用最大似然法鉴别周围性肺癌、肺错构瘤及结核球3种孤立性肺结节(SPN)的诊断价值。方法:对150例经临床和手术病理证实的SPN(周围性肺癌、肺错构瘤、结核球各50例)的HRCT影像学征象进行分类统计分析,运用最大似然判别法,将其各种征象转化为记分值,以数值的大小来判定肺结节所属的类型。结果:最大似然法对周围型肺癌、肺错构瘤及结核球的诊断正确率分别为86%、92%及90%,平均诊断正确率为89.3%,高于常规阅片法的82%,但两组间差异无统计学意义(χ2=2.434,P>0.05)。最能提示为肺癌的征象依次为空泡征、分叶征、支气管充气征和血管集束征;最能提示为肺错构瘤的征象依次为脂肪、边缘清楚及钙化;最能提示为结核球的征象依次为空洞、卫星灶、钙化及胸膜凹陷征。结论:最大似然法对常见SPN的鉴别诊断正确率有所提高,是一种辅助影像学诊断的有价值的方法。     

Hamartoma of the parotid gland: A case report with immunohistochemical and electron microscopic study

Summary A case of a solid parotid tumour in a 16-year-old boy is presented. Histologically, the tumour demonstrated some peculiar findings. An acinar pattern was predominant although every component seen in the normal salivary gland was present, namely, serous and mucous gland acini, ducts, myoepithelial cells, adipose and lymphoid tissue. Large eosinophilic granules were abundant in the large acinar cell cytoplasm. Immunohistochemically, the tumour demonstrated the proteins which are present in the normal parotid gland, for example, amylase, lactoferrin and lysozyme. Electron microscopic features were quite similar to those of normal parotid tissue except for accumulation of a large number of cytoplasmic granules in the acinar cells. There has been no previous report of a tumour with the same features as seen in this case. Our pathological diagnosis is hamartoma, although the possibility of hyperplasia or neoplasia can not be excluded.     

结节性硬化症多器官损害7例报告

目的探讨结节性硬化症多器官损害的临床特点以提高诊断治疗水平。方法回顾性分析7例结节性硬化症伴皮肤、大脑、肾脏、肝脏等多器官损害患者的临床资料,探讨其特征性临床表现及影像学改变。结果7例患者均有多器官损害,累及两个器官3例,3个及以上器官损害4例;皮肤损害主要为面部血管纤维瘤6例,皮肤色素脱失斑7例,鲨鱼皮斑3例,趾甲下纤维瘤1例;癫痫发作6例,智力低下4例,颅脑CT或MRI检查提示室管膜下结节4例,皮质结节2例;4例合并双侧肾脏多发错构瘤,1例合并肝脏错构瘤。结论特殊的皮肤损害、癫痫发作、智力低下,脑CT或MRI检查提示室管膜下结节或皮质结节或内脏多发性错构瘤为本病的主要临床特征,提高本病的认识有助于早期诊断和治疗。     

Respiratory Epithelial Adenomatoid Hamartoma Originating from Nasal Septum

Respiratory epithelial adenomatoid hamartoma (REAH) is a rare benign lesion in nasal cavity. We report two cases of REAH of the nasal cavity arising from nasal septum. The etiology of REAH is unknown although inflammation may induce gland proliferation observed in hamartomas. One of our cases was associated with nasal polyposis. REAH is a self-limiting disease, so it is important to differentiate REAH from other pathologic process, including inverted papilloma and low-grade adenocarcinoma. The treatment of choice is complete excision through a conservative approach.     

Breast hamartoma: A report of 14 cases of an under-recognized and under-reported entity

ObjectivesMammary hamartoma is a rare benign lesion accounting for approximately 4.8% of all benign breast masses. It is often underdiagnosed and therefore is underreported mostly due to lack of awareness of the characteristic clinical and histological features. Raising awareness of this poorly recognized benign entity is of utmost significance as it clinically mimics other breast tumors including both benign and malignant ones. This study is to report and present our experience of breast hamartomas from Johns Hopkins Aramco Healthcare in the Eastern province of Saudi Arabia from which there have not been previous studies in literature.MethodA retrospective review of our pathology files was done from 1994 to 2014 for cases diagnosed as breast hamartoma during this 20 year period.ResultsA total of 14 cases with diagnosis of breast hamartoma were identified in our institute. Histologically the lesion is mostly sharply demarcated showing a mixture of varying proportions of fibrous, adipose, and glandular tissue. 13 cases were seen in females (93%) and only one rare occurrence in a male patient (7%). The age ranges quite vastly from 18 to 51 years (mean 33 years). Two-third of these lesions were seen involving the right breast (9 cases/64.3%) and only one-third in the left side (5 cases/35.7%). 13 out of 14 patients had a well circumscribed lesion (92.9%) while only 1 case showed irregular borders (7.1%). The size varied from 1.4 to 9.5 cm. Three cases (21.4%) showed evidence of myoid differentiation, a histopathologic variance which is important to identify however has no clinical significance. 3 cases had associated epithelial ductal hyperplasia of the usual type varying from mild (2 cases) to moderate (1 case); with two of these cases exhibiting additional features of fibrocystic mastopathy including adenosis, apocrine metaplasia, and cyst formation. None of our cases showed any malignancy or pseudoangiomatous stroma hyperplasia (PASH).     

成人肝错构瘤恶变血管肉瘤1例

1．临床资料:患者女性,33岁,因右上腹胀痛,发现肝脏多发占位8年,出现腹水半年入院。患者无肝炎后肝硬化病史。8年前因右上腹胀痛查CT示多发占位,行肝穿刺活检为肝     

Red flags for early recognition of adult patients with PTEN Hamartoma Tumour Syndrome

Patients with PTEN Hamartoma Tumour Syndrome (PHTS) are at increased risk of developing cancer. Many adult PHTS patients are not recognized as such and do not receive the cancer surveillance they need. Our aim was to define phenotypic characteristics that can easily be assessed and manifest by early adulthood, and hence could serve as red flags (i.e. alerting signals) for early recognition of adult patients at high risk of PHTS. Phenotypic characteristics including macrocephaly, multinodular goitre (MNG), and oral features were examined in 81 paediatric and 86 adult PHTS patients by one of two medical experts during yearly surveillance visits at our Dutch PHTS expert centre between 1997 and 2020. MNG was defined as signs of thyroid nodules and/or goitre. Oral features included gingival hypertrophy, high palate (adults only) and oral papillomas. Based on the characteristics’ prevalence in different age groups, combinations of phenotypic characteristics were defined and evaluated on their potential to recognize individuals with PHTS. Macrocephaly was present in 100% of paediatric and 67% of adult patients. The prevalence of MNG was ∼50% in paediatric and gradually increased to >90% in adult patients. Similar percentages were observed for any of the oral features. Scoring two out of three of these characteristics yielded a sensitivity of 100% (95%CI 94–100%) in adults. The presence of the combination macrocephaly, MNG, or multiple oral features could serve as a red flag for general practitioners, medical specialists, and dentists to consider further assessment of the diagnosis PHTS in adults. In this way, recognition of adult PHTS patients might be improved and cancer surveillance can be offered timely.