Imprint cytology of primary cardiac sarcomas: a report of 3 cases

Primary cardiac sarcomas are rare instances and only occasionally documented in the cytologic literature. Usually, the diagnosis of these rare lesions can be made at echocardiography, aspiration biopsy cytology, cardiac biopsy, and open cardiac surgery (intraoperative diagnosis). In this study, cytologic configurations and immunohistochemistry for 3 primary cardiac sarcomas (rhabdomyosarcoma, angiosarcoma, and malignant fibrous histiocytoma) were revealed. In rhabdomyosarcoma (right ventricle), the tumor cells exhibited an anisocytotic spindle-shaped nuclei with hyperchromasia and an obscure cytoplasmic margin. Vimentin and myosin were positive throughout the cytoplasm for the tumor cells. In angiosarcoma (right atrium), small clusters of anisocytotic spindle-shaped tumor cells appeared as vascular-like structures and hemosiderin-laden macrophages in many erythrocyte-rich backgrounds. Nuclei showed round to oval shape with hyperchromasia and prominent large nucleoli. Cytoplasm was obscure and elongated. Factor VIII related antigen and CD34 were strongly positive throughout the cytoplasm for the tumor cells. In malignant fibrous histiocytoma (right ventricle), the tumor cells exhibited oval to spindle-shaped and elongated nuclei and coarse granular chromatins with hyperchromasia. The nuclear margin was thin. A few small round nucleoli appeared. Elongated obscure and foamy cytoplasm was stained pale blue. Vimentin and α₁-antitrypsin were positive throughout the cytoplasm for the tumor cells. This study elucidated the cellular characteristics and immunohistochemistry for cardiac sarcomas using imprint smears as an aid to cytopathologic diagnosis.     

Bronchiectasis in children: 10-year experience at a single institution

Purpose

Bronchiectasis in children is still one of the most common causes of childhood mortality in developing countries. The aim of this study was to investigate the epidemiological characteristics, clinical features, underlying etiologic factors, and distinct change in the management of patients with bronchiectasis at Asan Medical Center Children''s Hospital of Seoul.

Methods

A retrospective study of children diagnosed with bronchiectasis was conducted between January 1999 and December 2008. All patients underwent a comprehensive examination to identify etiologic factors. Data analysis in terms of age at onset, initial presenting symptoms, underlying etiology, distinct change in treatment, distribution of pulmonary involvement on computed tomography (CT), and causative microbiological flora triggering secondary infections was performed.

Results

The median age at the time of the diagnosis of bronchiectasis was 7.6 years (range, 2 months to 18 years). Persistent coughing was the most common symptom. The underlying etiologies identified in 79 patients (85.8%) included bronchiolitis obliterans (32.6%), childhood respiratory infection (20.6%), interstitial lung disease (17.3%), immunodeficiency (8.6%), and primary ciliary dyskinesia (4.3%). In 53 children (67%), the identified cause led to a distinct and individualized change in management. The distribution of CT abnormalities had no correlation with the underlying cause of bronchiectasis.

Conclusions

Selected Korean children with bronchiectasis were reviewed to identify diverse underlying etiologies. All children with bronchiectasis should be comprehensively investigated because identifying underlying causes may have a major impact on their management and prognosis.     

Primary and metastatic cardiac sarcomas: a 12-year experience at a German heart center

Background: Primary and metastatic cardiac sarcomas represent a heterogeneous group of rare neoplasms with varying clinical course and diverse histogenetic line of differentiation. To date, there exist no uniform guidelines for their surgical and/or oncological treatment. Methods: We evaluated retrospectively all patients undergoing cardiac surgery for primary or secondary cardiac sarcoma in the period 1999-2011 at the Erlangen Heart Centre to analyze their clinicopathological spectrum, treatment and outcome. Results: Five patients (3 women & 2 men; mean age: 46 years; range: 28-81 years) had primary cardiac sarcomas (6.7% of primary cardiac tumors) and 4 had cardiac metastasis from soft tissue sarcoma (1 case each of osteosarcoma, myxoid liposarcoma, alveolar soft part sarcoma and pleomorphic spindle cell sarcoma). Primary sarcomas were located in the left atrium (n=3), left ventricle (n=1) and right atrium (n=1). Histological types were myxosarcoma (3), pleomorphic undifferentiated sarcoma (1) and angiosarcoma (1). Four patients died at 2-64 months (mean, 24.5 months). Sarcoma metastasis to the heart developed at a mean of 109.5 months from initial diagnosis (range, 5-240 months). Three of them died of disease at a mean of 14 months after cardiac surgery and one is disease free 34 months after heart transplantation for metastasis. Conclusions: Primary and metastatic cardiac sarcomas are very heterogeneous in their histological appearance, clinical presentation and course of the disease. Radical surgery combined with chemoradiation is promising in patients with resectable disease and may significantly prolong survival. Cardiac transplantation represents an emerging strategy for patients with isolated unresectable cardiac involvement.     

Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases

AIMS: Primary cardiac sarcomas are rare and aggressive tumours. The aims of this study were to precisely classify cardiac sarcomas according to their pathology, and to determine their clinicopathological features and prognosis. METHODS AND RESULTS: Twenty-four primary cardiac sarcomas were studied. Clinical features and follow-up of all patients were collected. Histological diagnoses were obtained by combining both morphological features as described in soft tissue counterparts and the immunoprofile of the tumours. The 24 cases were classified as undifferentiated sarcoma (nine cases), angiosarcoma (six cases all located in the right atrium), leiomyosarcoma (six cases), malignant fibrous histiocytoma (one pleomorphic and one giant cell type) and synovial sarcoma (one case). Patients included 14 men and 10 women, with a mean age of 46 years. Clinical manifestations were protean, often delaying the diagnosis. Dyspnoea resulting from obstruction of the cardiac chambers was the most common symptom. Echocardiography and magnetic resonance imaging were useful respectively to detect and to evaluate tumour extension. Complete macroscopic resection was possible in only 33% of patients. The most common cause of death was local recurrence of the tumours (50%), even in the cases of complete macroscopic resection. Whatever the treatment, the prognosis was poor with a mean survival of 16.5 months after diagnosis. CONCLUSIONS: All types of sarcomas may be observed in the heart with a predominance of undifferentiated sarcomas. Histological grading, unlike histological type, seems to correlate with survival which remains extremely poor.     

Recurrence of synovial sarcoma of the abdomen in a 10-year old child

The case of a 10 year old child with a synovial sarcoma of the left iliac fossa, which recurred 12 months after simple large surgical excision, is reported here. No related lesions and, in particular, no lesion of the articular capsule of the left hip could be found, either on X-ray or at the time of the two excisions. Microscopically, the tumor showed, in all the areas examined, the existence of two characteristic morphological aspects, associating epithelial like type cells and elongated fusiform cells. These features correspond exactly to the classic "biphasic" type of synovial sarcoma. This location of a synovial sarcoma is an uncommon one, both in children and in adults.     

Anal canal carcinoma: experience from a single Korean institution

PURPOSE: The clinical features, treatment modality approaches in clinical practice, and prognostic factors for anal canal carcinoma patients were retrospectively analyzed. MATERIALS AND METHODS: Between October 1994 and December 2005, 50 patients with anal canal cancer were treated at Samsung Medical Center, Seoul, Korea. RESULTS: After a median follow up of 37.8 months (range, 6.6-136.1 months), the 5-year and 10-year survival rates for the 38 patients with early and locally advanced squamous and cloacogenic carcinoma (squamous cell carcinoma and cloacogenic carcinoma) were 74.8% and 66.5%, respectively. The 5-year survival and disease-free survival rates (DFS) of the 31 patients who received chemoradiation therapy (CRT) were 83.6% and 74.3%, respectively. The overall and DFS could not be determined for the adenocarcinoma group due to the small number of cases (n=8). Univariate analysis showed that tumor size (p=0.04) and inguinal node status (p=0.04) significantly influenced patient survival in patients with squamous cell and cloacogenic carcinomas. Furthermore, univariate analysis also showed that, inguinal node status influenced patient survival in the adenocarcinoma group. Multivariate analysis showed that inguinal node metastasis is a single independent prognostic variable for survival (p=0.04) in patients with squamous cell and cloacogenic carcinomas. CONCLUSION: Combined CRT has been adopted as standard treatment with outcomes that are comparable to those reported in randomized clinical trials. Due to the rarity and complexity of anal canal carcinoma, interdepartmental cooperation is required for disease treatment. Thus, proper treatment of patients should incorporate a team-approach and should be available to as many patients as possible.     

Incidence of pulmonary non-epithelial tumors: 18 years’ experience at a single institute

Primary and metastatic non-epithelial tumors of the lung and pleura (NETs-LP) are presumed to represent a small proportion of pulmonary neoplasm, and their epidemiologic characterization is lacking. Moreover, classification of pulmonary mesenchymal tumor according to ‘WHO Classification of Tumors of Lung and Pleura’ (2004) is simple and necessary to give a supplementation like new ‘WHO Classification of Tumors of Soft Tissue and Bone’ (2013).     

Clinical usefulness of fine needle aspiration cytology in patients less than 20 years old: a 10-year experience at a single institution

The purpose of this study was to identify the spectrum of cytological diagnoses and evaluate the diagnostic effectiveness of fine needle aspiration cytology (FNA) in patients less than 20 years old. The subjects were selected by retrospectively reviewing records from 1999 to 2009. Selected patients less than 20 years old underwent FNA. Cytological and histological slides of samples from the subjects were reviewed. Our study included a total of 909 subjects with a mean age of 14.6 years. The majority of the FNA samples were taken from lymph nodes (n = 448, 49.3%), with the remaining aspirates obtained from the thyroid gland (n = 247, 27.2%), soft tissues of head and neck masses (n = 106, 11.7%), salivary glands (n = 75, 8.3%), breasts (n = 18, 1.9%), skins (n = 9, 1.0%) and soft tissues of extremity (n = 6, 0.7%). The majority (87.6%, n = 796) of the FNA samples were categorized as ‘benign’, with the remaining designated as ‘atypical lesion’ (n = 18, 2.0%), ‘malignant’ (n = 24, 2.6%), or ‘inadequate specimen’ (n = 71, 7.8%). FNA accuracy was 92% for diagnosing cancer. Specificity and sensitivity were 99% and 63%, respectively. Our study first revealed that FNA has a high specificity for diagnosing cancer in various anatomical locations in young patients and can be confidently used as an effective tool for diagnosing malignancies in young individuals with a clinically suspicious lesion.     

Gastrointestinal stromal tumors--frequency, malignancy, and new prognostic factors: the experience of a single institution

Gastrointestinal stromal tumors (GISTs) are c-Kit-positive neoplasms of the digestive tract. Few studies have reported their real incidence and malignancy. Two systems of risk assessment of aggressive behavior were recently proposed. The aims of our study were (1) to ascertain the frequency of GISTs and their clinical behavior in a large series of cases with a long-term follow-up, (2) to evaluate the prognostic value of two well-known risk assessment classifications, and (3) to propose an alternative prognostic index based on our data. Statistical analyses were performed to identify possible predictors of malignant behavior. One hundred and eighteen out of 169 (70%) mesenchymal tumors were GISTs. They were located in the stomach (57%), small intestine (31%), colon/rectum (6%), and omentum/mesentery (6%). Eighteen cases (16%) showed malignant behavior, with local recurrence in eight cases and distant metastases in 11 cases. Fifteen of 114 (13%) patients died of disease within 74 months, whereas 63 (55%) patients were still alive after a median period of 78 months. At multivariate analysis, high-risk category (according to Fletcher's criteria), omental/colorectal site, and younger patient age were independent predictors of malignant behavior. In addition to the evaluation of risk category, tumor site and patient age helped to better identify patients requiring stricter monitoring.     

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival

Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.     

Well-differentiated papillary mesothelioma: A 17-year single institution experience with a series of 75 cases

We present our experience with 75 cases of well-differentiated papillary mesothelioma (WDPM) that were diagnosed at our institution between 2000 and 2017. The patients included 58 females and 17 males with age ranging from 18 to 69 years (mean, 42 years). Clinically, the vast majority of WDPMs were incidental findings during laparotomy or laparoscopic surgery for a variety of benign or malignant disease. The lesion manifested as either a small solitary nodule or multiple miliary nodules on the peritoneum or serosal surfaces of internal organs. Histologically, 67 cases were consistent with a classical WDPM, of which 6 cases contained microinvasive foci and 1 case had malignant transformation. Eight cases were hybrid tumors with variable combined component of adenomatoid tumor (n = 4), multicystic mesothelioma (n = 2), and both (n = 2). By immunohistochemistry, besides calretinin, D2-40, CK5/6 and WT1, 94% (29/31) of cases also showed immunostaining for PAX8. In comparison, PAX8 staining was only present in 12% (6/50) of epithelioid malignant mesothelioma selected as control cases. Follow-up information available in 46 cases revealed no signs of tumor progression or local recurrence except for the case that showed transformation to a fully malignant mesothelioma after a period of 15 years. Our comprehensive study further expanded the clinical and histopathological spectrum of WDPM. Compared with epithelioid malignant mesothelioma, PAX8 staining is highly sensitive and specific for WDPM (P < 0.001).     

粒细胞肉瘤38例临床病理分析

目的 探讨粒细胞肉瘤的临床病理特征及鉴别诊断.方法 对38例粒细胞肉瘤患者的病理组织进行HE及免疫组织化学(EnVision法)染色观察,结合临床资料进行分析,并复习相关文献.结果 患者发病年龄2～77岁,平均年龄43.3岁,男23例,女15例.临床主要表现为体表淋巴结肿大、局部软组织肿块及疼痛.其中随访18例,死亡14例,平均生存时间16.9个月.组织学表现为肿瘤细胞弥漫成片,小～中等大小,形态较一致,胞质少而淡染,核呈圆形或不规则形,可见核仁,核分裂象易见.部分肿瘤具有"列兵样"组织结构,肿瘤细胞间可见散在分布的幼稚嗜酸性粒细胞.免疫组织化学染色显示瘤细胞表达抗髓过氧化物酶、CD43,部分表达CD68、溶菌酶、CD99和末端脱氧核苷酸转移酶,而CD3、CD20、CD79a、AE1/AE3和胎盘碱性磷酸酶阴性.结论 粒细胞肉瘤临床少见,形态学上易误诊为非霍奇金淋巴瘤、Ewing肉瘤/PNET、胚胎性横纹肌肉瘤等,免疫组织化学技术对于确诊本病具有重要价值.     

Natural course of cholestasis in neonates on extracorporeal membrane oxygenation (ECMO): 10-year experience at a single institution

The study objectives were to confirm the incidence of cholestasis and determine factors that contribute to its development and the natural course of cholestasis in neonates treated with extracorporeal membrane oxygenation (ECMO). This was a retrospective chart review including all patients receiving ECMO between 1995 and 2005 at Kosair Children's Hospital. Neonates were grouped as having cholestasis or no cholestasis. A total of 211 patients underwent ECMO between 1995 and 2005. Thirty patients (14%) developed cholestasis. There was no difference in demographics or diagnoses between patients developing cholestasis on ECMO vs. those who did not. The development of cholestasis on ECMO was related to run time, 148 hours +/- 111 (no cholestasis group) vs. 252.8 hours +/- 187 (cholestasis group), p < 0.001 respectively. The majority of patients received veno-arterial ECMO, but there was no difference in cholestasis related to type of ECMO support. Direct hyperbilirubinemia and elevation of hepatic enzymes resolved in all but one patient by the time of discharge. Our incidence of cholestasis (14%) on ECMO is lower than previously reported series. Longer duration of ECMO and other complications on ECMO (renal, infectious, and metabolic) are more likely in the patients developing cholestasis. Follow-up showed resolution of cholestasis in all patients without hepatic sequelae.     

Diagnostic telepathology: long-term experience of a single institution

Objectives The paper reviews the development of the application of telepathology in a department of surgical pathology between 1991 and 2003. The goal of the efforts during this time was to give up the concept of programming a single application, available only between two fixed workstations with sophisticated devices and special software, and to find the virtual largest common denominator for implementing as many different applications as possible with the same basic system.Methods A new telepathology system was designed as a client–server system with a relational database at its centre. The clients interact together by transferring the questions (texts and images) to a record (case) in the database on the server and by transferring the answers to the same record on the database.Results The new open telepathology system iPath () has been very well accepted by many groups around the world. The main application fields are: consultations between pathologists and medical institutions without a pathologist (e.g. for frozen section diagnoses or for surgical diagnoses in hospitals in South Asia or Africa), tumour boards, field studies and distance education ().Conclusions Having observed that with iPath we have succeeded in satisfying all our telepathology needs, we are inclined to put the emphasis on the nature of the tasks being performed, as opposed to the methods or technical means for performing a given task. The three organisation models proposed by Weinstein et al. (2001) [24] can be reduced to only two models: the model of discussion groups and the model of expert groups (virtual institutes).     

Primary sarcomas of the lung: a clinicopathological and immunohistochemical study of 14 cases

The clinicopathological features and immunohistochemical findings in 14 primary sarcomas of the lung collected over a 30-year-period are presented. This represents one sarcoma per 550 bronchogenic carcinomas undergoing resection in this centre. The study group comprised six leiomyosarcomas, five malignant peripheral nerve sheath tumours, two haemangiopericytomas and one epithelioid haemangioendothelioma. The majority of cases occurred in men (nine males: five females), with mean age at presentation of 54 years for men and 47 years for women. All leiomyosarcomas were seen in men, whereas malignant peripheral nerve sheath tumours showed no particular sex preponderance. Leiomyosarcomas were larger tumours than malignant peripheral nerve sheath tumours, mean tumour diameter 15 cm (range 10–25 cm) compared to 9.5 cm (7–15 cm), respectively. All leiomyosarcomas were situated intraparenchymally whereas two of the five malignant peripheral nerve sheath tumours were endobronchial in site. Extrathoracic metastates were seen at death in two of the six leiomyosarcomas but not in any of the malignant peripheral nerve sheath tumours. Overall survival was 28 months although for the leiomyosarcoma/malignant peripheral nerve sheath tumour group alone survival was 8 months. Tumour grading appeared to be a more useful prognostic factor than tumour site (endobronchial/parenchymal) or tumour size. Haemangiopericytoma and epithelioid haemangioendothelioma were associated with a more favourable prognosis.     

Cardiac papillary fibroelastoma: Retrospective clinicopathologic study of 17 tumors with resection at a single institution and literature review

Cardiac papillary fibroelastomas (PFEs), which are mainly found in the valves, are rare benign tumors that can cause embolism. Single-center surgical experience in the treatment of this tumor is uncommon.