Primary cardiac lymphoma initially diagnosed by routine cytology. Case report and literature review

A case of primary cardiac lymphoma initially diagnosed by routine cytologic examination of pericardial fluid is presented. In a 64-year-old woman woman who originally presented with chest pain and heart block, the initial clinical impression was ischemic heart disease. However, coronary angiography failed to reveal significant disease. An echocardiogram demonstrated pericardial fluid, which was drained. A small amount was sent for cytologic examination, and the diagnosis of malignant lymphoma, large cell type, was made. Subsequent radiologic examinations revealed an intracardiac mass involving the atrioventricular canal; surgical biopsy confirmed the diagnosis of a large cell lymphoma. While primary malignant lymphoma of the heart is rare, this case highlights the efficacy of routine cytologic examination of an effusion fluid (often drained therapeutically) in establishing the correct diagnosis.     

Holoprosencephaly

Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10–20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor.     

Cytomorphologic diagnosis of malignant lymphoma arising in the heart: a case report.

BACKGROUND: Primary malignant lymphoma of the heart is extremely rare. Because its clinical signs and symptoms are typically nonspecific, it is often very difficult to detect cardiac involvement while the patient is alive. We describe a case of malignant lymphoma involving predominantly the heart and pericardium and diagnosed by pericardiac effusion cytology antemortem. CASE: An 83-year-old woman presented with dyspnea on exertion. Echocardiography revealed a low-echoic tumor mass close to the right ventricular wall and massive pericardiac effusion. Diagnosis of diffuse large B-cell lymphoma was made by cytomorphologic examination and flow cytometry of the tumor cells obtained from the effusion. Although chemotherapy was instituted immediately, the patient died of progressive heart failure. Diffuse large B-cell lymphoma predominantly involving the intracardiovascular region was confirmed at autopsy. CONCLUSION: From the experience in this case, we conclude that cytopathologic examination of sonographically guided aspiration of the cardiovascular region is very useful for antemortem diagnosis of primary malignant lymphoma of the heart.     

妊娠合并恶性肿瘤疾病的诊疗探讨

目的:探讨妊娠合并恶性肿瘤在诊治过程中的临床表现,探讨其对妊娠结局的影响及预后.方法:回顾性查阅2005年至2011年9月于我院住院治疗患者病历,收集妊娠合并恶性肿瘤疾病患者10例在入院诊断、治疗经过中的相关资料.结果:10例患者中9例在终止妊娠前确诊,3例孕妇死亡,5例在随访中,2例失访;10例胎儿中有生机儿5例,其中1例放弃,在可随访的3例新生儿生存状态良好.结论:妊娠合并恶性肿瘤疾病的临床确诊及及时治疗对妊娠结局会造成影响,妊娠结局与肿瘤分期及孕周相关,应在产前进行常见恶性肿瘤疾病的筛查,早期发现,早期干预.     

Neonatal Cardiac Distress—A Practical Approach to Recognition,Diagnosis, and Management

The death rate among neonates with cardiovascular disease is 50 percent during the first six months, with the majority dying during the first month. With early diagnosis most of these babies could be saved. In approaching the diagnosis of cardiac distress in the newborn, it is important to remember that the types of cardiovascular disease which cause symptoms and death early in life are quite different from those in older children. Lesions such as hypoplasia of the left heart, transposition of the great arteries, endocardial fibroelastosis, pulmonary atresia, mitral atresia, tricuspid atresia and truncus arteriosus are common, not rare, causes of cardiac distress in the newborn.A classification of neonatal cardiovascular diseases into seven pathophysiological groups is presented as a basis for an effective, practical approach to the differential diagnosis of the potentially lethal lesions. This approach is simplified further since over 90 percent of babies with cardiac distress have one of three lesions: (1) Large left-to-right shunt (characterized by the presence of massive plethora on the chest roentgenogram), (2) Large right-to-left shunt (association with intense cyanosis) or (3) Severe obstruction (including hypoplasia of the left heart, which is the most common cause of death due to cardiac distress during the first week of life).     

磁共振成像对乳腺肿瘤的诊断价值

乳腺癌是危及女性健康的常见恶性肿瘤之一,病死率较高,且发病年龄呈年轻化趋势。目前临床对乳腺疾病的检查方法很多,既往检查主要包括钼靶、超声等,因价格便宜、操作方便,已成为常规的乳腺疾病检查方法,但两者的敏感性和特异性较低并有自身的局限性。CT软组织分辨率较高,但检查过程中的X线剂量较大,并且动态增强时间较长,故作为乳腺钼靶的补充检查手段。这些检查方法对乳腺疾病均有不同的诊断意义,在当前众多诊断乳腺疾病方法中,具有无辐射,较高软组织分辨力及可多方位多层面成像的乳腺磁共振(MRI)成像有其独到的优势,某些方面能弥补超声和钼靶检查的局限性,乳腺磁共振可提供病灶形态学和增强血流动力学表现,可用于常规检查方法不能确诊病灶的鉴别诊断。乳腺肿瘤MRI成像对临床诊断、鉴别诊断及手术方案的选择有着极其重要的作用。本文就乳腺MRI影像技术、MRI影像学表现及其临床应用予以综述,探讨MRI在乳腺肿瘤中的应用。     

Thoracic lymphatic disorders

Thoracic complications of lymphatic disorders can culminate in respiratory failure and death and should be considered in any patient with a lymphatic disease and clinical or radiographic evidence of chest disease. Congenital lymphatic disorders are being increasingly recognized in the adult population. The spectrum of thoracic manifestations of lymphatic disorders ranges from incidental radiographic findings to diffuse lymphatic disease with respiratory failure. This article serves to review some recent advances that allow improved diagnosis and management of thoracic lymphatic disorders. Herein, we describe their anatomical and physiologic effects, the time course of their progression, and the therapies that are currently available. The management of malignant (cancerous) lymphatic disorders of the thorax is beyond the scope of this paper.     

Malignant phyllodes tumor of the breast metastatic to the parotid gland diagnosed by fine needle aspiration biopsy. A case report

BACKGROUND: Phyllodes tumor (cystosarcoma phyllodes) is a rare fibroepithelial neoplasm of the breast. Malignant phyllodes tumor is characterized by an infiltrative border and marked degree of hypercellular stromal overgrowth with > 5 mitoses per 10 high-power fields. Distant metastasis occurs in 10-20% of patients with malignant phyllodes tumor. The most common sites of distant metastases are the lungs, bone and abdominal viscera. Although theoretically any organ may have metastasis, the parotid gland has not been documented before in the English-language literature. CASE: A 40-year-old, Caucasian woman with a history of malignant phyllodes tumor of the left breast presented with a mass on the right side of the parotid gland. Fine needle aspiration biopsy of the mass revealed abundant discohesive spindle cells showing moderate nuclear pleomorphism with occasional mitoses. No epithelial elements were seen. A diagnosis of malignant spindle cell tumor consistent with metastatic malignant phyllodes tumor was made. Histology confirmed the cytologic diagnosis. CONCLUSION: Fine needle aspiration biopsy is accurate and efficient in conjunction with clinical information in the diagnosis of malignant phyllodes tumor of the breast metastatic to the parotid gland.     

Life-long tailoring of management for patients with hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, characterised by complex pathophysiology and extensive genetic and clinical heterogeneity. In most patients, HCM is caused by mutations in cardiac sarcomere protein genes and inherited as an autosomal dominant trait. The clinical phenotype ranges from severe presentations at a young age to lack of left ventricular hypertrophy in genotype-positive individuals. No preventative treatment is available as the sequence and causality of the pathomechanisms that initiate and exacerbate HCM are unknown. Sudden cardiac death and end-stage heart failure are devastating expressions of this disease. Contemporary management including surgical myectomy and implantable cardiac defibrillators has shown significant impact on long-term prognosis. However, timely recognition of specific scenarios – including transition to the end-stage phase – may be challenging due to limited awareness of the progression patterns of HCM. This in turn may lead to missed therapeutic opportunities. To illustrate these difficulties, we describe two HCM patients who progressed from the typical hyperdynamic stage of asymmetric septal thickening to end-stage heart failure with severely reduced ejection fraction. We highlight the different stages of this complex inherited cardiomyopathy based on the clinical staging proposed by Olivotto and colleagues. In this way, we aim to provide a practical guide for clinicians and hope to increase awareness for this common form of cardiac disease.     

Mass Spectrometric Identification of Ancient Proteins as Potential Molecular Biomarkers for a 2000-Year-Old Osteogenic Sarcoma

Osteosarcoma is the most common primary malignant tumor of bone usually occurring in young adolescent and children. This disease has a poor prognosis, because of the metastases in the period of tumor progression, which are usually developed previous to the clinical diagnosis. In this paper, a 2000-year-old ancient bone remain with osteogenic sarcoma was analyzed searching for tumor biomarkers which are closely related to this disease. After a specific extraction SDS-PAGE gel electrophoresis followed by tryptic digestion was performed. After the digestion the samples were measured using MALDI TOF/TOF MS. Healthy bone samples from same archaeological site were used as control samples. Our results show that in the pathological skeletal remain several well known tumor biomarkers are detected such as annexin A10, BCL-2-like protein, calgizzarin, rho GTPase-activating protein 7, HSP beta-6 protein, transferrin and vimentin compared to the control samples. The identified protein biomarkers can be useful in the discovery of malignant bone lesions such as osteosarcoma in the very early stage of the disease from paleoanthropological remains.     

补肾益气活血汤对小鼠血清雌、孕激素浓度 及其受体表达的影响

目的：探讨中药补肾益气活血汤促进着床的机理。方法：在以吲哚美辛建立小鼠着床障碍模型的基础上,将动物分为正常组,吲哚美辛组,中药组和黄体酮组,用放射免疫、免疫组织化学及Westem—blot方法检测各组小鼠妊娠d5（围着床期）血清皿,P4浓度及子宫内膜ER,PR表达。结果：补肾益气活血中药对E2、P4水平无明显影响,对PR表达也无明显影响,但有轻度促进ERα表达作用,此外,实验发现治疗对照组注射黄体酮虽然使P4水平升高,但ERα、PR表达却都有一定下降。结论：提示中药补肾益气活血汤可能主要通过雌激素样活性加强E2与其受体作用而有利于发动蜕膜化从而促进着床,黄体酮辅助生殖治疗失败可能与孕酮一定程度上抑制ERα和PR表达有关。     

Sensitivity and positive predictive values of presurgical clinical diagnosis of excised benign and malignant skin tumors: a prospective study of 835 lesions in 778 patients.

This article reports on the sensitivity and positive predictive value of clinical diagnosis of benign and malignant skin tumors by expert plastic surgeons in an Israeli clinic. Most published reports have focused on the sensitivity of clinicians' diagnoses, a general measure of the physician's skill that does not predict the rate of accuracy of a physician's diagnoses. Our study of 835 lesions in 778 patients, one of the largest Israeli series, assesses the clinical diagnosis of malignant and benign skin tumors and is one of the few that provide information on the positive predictive value, the measure that is of interest to both physicians and patients. The majority of tumors were benign (56.8 percent), 31.6 percent were malignant, and 11.6 percent were premalignant. Among the 474 benign lesions, 46 percent were nevi. The most common nevi subclass was compound nevi (53 percent), 9 percent of the nevi were dysplastic, and 5 percent were blue nevi. The most common malignant tumor was basal cell carcinoma, accounting for 78 percent of malignant tumors.Although sensitivity for clinical diagnosis of malignancy was 91.3 percent, the positive predictive value for clinical diagnosis of malignancy was 71.3 percent. The sensitivity rate for clinically diagnosing premalignant tumors was 42.3 percent, whereas the positive predictive value for these diagnoses was higher (64.1 percent). The sensitivity rate for diagnosis of all benign lesions was 85.9 percent, and the positive predictive value was 94.2 percent. The sensitivity rate for diagnosis of all nevi was 87.6 percent, and the positive predictive value was 85.7 percent: i.e., only seven of the 218 pathologically proven diagnoses of nevi (3.2 percent) were falsely diagnosed as malignant lesions. Even more interestingly, five of the 223 clinical diagnoses of nevi (2.2 percent) were pathologically proven to be malignant melanomas, and seven were found to be premalignant lesions (3.1 percent). It was concluded that publications which report only on the sensitivity neglect to provide information of interest regarding the positive predictive value. Often, positive predictive value is qualitatively different from the sensitivity, and thus relying only on the sensitivity may lead to incorrect evaluation of a clinical judgment, which may result in erroneous surgical decisions.     

The therapeutic potential of stem cells in heart disease

Abstract.  Coronary heart disease and chronic heart failure are common and have an increasing frequency. Although interventional and conventional drug therapy may delay ventricular remodelling, there is no basic therapeutic regime available for preventing or even reversing this process. Chronic coronary artery disease and heart failure impairs quality of life and are associated with subsequent worsening of the cardiac pump function. Numerous studies within the past few years have been demonstrated, that the intracoronary stem cell therapy has to be considered as a safe therapeutic procedure in heart disease, when destroyed and/or compromised heart muscle must be regenerated. This kind of cell therapy with autologous bone marrow cells is completely justified ethically, except for the small numbers of patients with direct or indirect bone marrow disease (e.g. myeloma, leukaemic infiltration) in whom there would be lesions of mononuclear cells. Several preclinical as well as clinical trials have shown that transplantation of autologous bone marrow cells or precursor cells improved cardiac function after myocardial infarction and in chronic coronary heart disease. The age of infarction seems to be irrelevant to regenerative potency of stem cells, since stem cells therapy in old infarctions (many years old) is almost equally effective in comparison to previous infarcts. Further indications are non-ischemic cardiomyopathy (dilative cardiomyopathy) and heart failure due to hypertensive heart disease.     

Persistent truncus arteriosus in a rhesus monkey (Macaca mulatta)

A 47-day-old female rhesus macaque (Macaca mulatta) was examined because of a severe holosystolic heart murmur (grade 5/6) and signs of congestive heart failure. Results of physical examination, thoracic radiography, and cardiac ultrasonography confirmed an advanced stage of congestive heart failure. Due to the animal's age and clinical signs of disease, a congenital heart defect was suspected. Necropsy revealed a rare congenital heart defect known as persistent (common) truncus arteriosus.     

Fine needle aspiration biopsy of primary mucinous carcinoma of the skin: a case report

BACKGROUND: Primary mucinous carcinoma of the skin is a rare neoplasm of sweat gland origin. To date there are only 2 case reports in English describing its features on fine needle aspiration biopsy (FNAB). We describe an additional case and review the literature regarding this entity. To the best of our knowledge, this is the first reported case with a sentinel lymph node biopsy. CASE: A 78-year-old woman presented with a 3-cm left scalp mass at an outside institution. Following incomplete excision, multiple subcentimeter nodules developed in the skin adjacent to the biopsy site. FNAB of the nodules confirmed a recurrence of mucinous carcinoma. Clinical examination and extensive radiographic studies did not reveal primary disease elsewhere, thus supporting a diagnosis of primary mucinous carcinoma of the skin. At the time of wide excision of the residual tumor, sentinel lymph node biopsy revealed a single focus of micrometastasis. The patient declined adjuvant therapy and was disease free 6 months after the initial diagnosis. CONCLUSION: Cutaneous mucinous carcinoma is a tumor characterized by bland histocytologic features and abundant extracellular pools of mucin. Without a high index of suspicion, this rare entity may be overlooked or misdiagnosed. Numerous benign and malignant mucin-producing primary and secondary mimics exist, and immunohistochemistry offers limited benefits in differentiating them. Cytologic diagnosis of primary mucinous carcinoma of the skin is possible; however, correlation of clinical, radiologic and pathologic features is necessary to arrive at an accurate diagnosis.     

Inflammatory mediators and the failing heart: a translational approach

Recent studies have identified the importance of proinflammatory mediators in regulating cardiac structure in health and disease. Recent studies suggest that cytokines that are expressed within the myocardium in response to a environmental injury, namely tumor necrosis factor-alpha (TNF), interleukin-1 (IL-1) and the interleukin-6 (IL-6) family of cytokines play an important role in initiating and integrating homeostatic responses within the heart. However, these "stress-activated" cytokines all have the potential to produce cardiac decompensation when expressed at sufficiently high concentrations. Indeed, there is now a growing appreciation that these molecules may play an important role in mediating disease progression in the failing heart. The growing appreciation of the pathophysiological consequences of sustained expression of proinflammatory mediators in pre-clinical and clinical heart failure models culminated in a series of multicenter clinical trials that utilized "targeted" approaches to neutralize tumor necrosis factor (TNF) in patients with moderate to advanced heart failure. However, these targeted approaches have resulted in worsening heart failure, thereby raising a number of important questions about what role, if any, proinflammatory cytokines play in the pathogenesis of heart failure. This review will summarize the tremendous growth of knowledge that has taken place in this field, with a focus on what we have learned from the negative clinical trials, as well as the potential direction of future research in this area.     

Cardiac toxicity of lung cancer radiotherapy

Radical radiotherapy of lung cancer with dose escalation has been associated with increased tumor control. However, these attempts to continually improve local control through dose escalation, have met mixed results culminating in the findings of the RTOG trial 0617, where the heart dose was associated with a worse overall survival, indicating a significant contribution to radiation-induced cardiac morbidity. It is, therefore, very likely that poorly understood cardiac toxicity may have offset any potential improvement in overall survival derived from dose escalation and may be an obstacle that limits disease control and survival of patients. The manifestations of cardiac toxicity are relatively common after high dose radiotherapy of advanced lung cancers and are independently associated with both heart dose and baseline cardiac risk. Toxicity following the treatment may occur earlier than previously thought and, therefore, heart doses should be minimized. In patients with lung cancer, who not only receive substantial heart dose, but are also older with more comorbidities, all cardiac events have the potential to be clinically significant and life-threatening.Sophisticated radiation treatment planning techniques, charged particle therapy, and modern imaging methods in radiotherapy planning, may lead to reduction of the heart dose, which could potentially improve the clinical outcomes in patients with lung cancer. Efforts should be made to minimize heart radiation exposure whenever possible even at doses lower than those generally recommended. Heart doses should be limited as much as possible.A heart dosimetry as a whole is important for patient outcomes, rather than emphasizing just one parameter.     

Dermatoscopy and High Frequency Sonography: Two Useful Non-Invasive Methods to Increase Preoperative Diagnostic Accuracy in Pigmented Skin Lesions

Dermatoscopy and high frequency sonography have recently been combined to increase diagnostic preoperative accuracy in the treatment of pigmented skin lesions. In this monocentric study 80 patients with pigmented skin lesions were evaluated clinically, by dermatoscopy, and 20 MHz-sonography followed by dermatohistopathological evaluation; 39 malignant melanomas, 37 common nevi, 3 dysplastic nevi, and 1 nevus Spitz were diagnosed histologically. In 72 of the 80 cases (91.3%) dermatoscopical diagnoses were confirmed by histopathology, compared to only 79% correct clinical diagnoses. For the mere clinical diagnosis of melanoma sensitivity was 79%, specificity was 78% and diagnostic accuracy was 65%. All diagnostic values increased by dermatoscopy: sensitivity reached 90%, specificity was 93%, and diagnostic accuracy was 83%. In order to determine tumor thickness preoperatively tumor thickness was measured by 20 MHz sonography. The correlation of tumor thickness between histometric and sonographic results was determined for nevi (r = 0.93) and melanoma (r = 0.95); 74.3% of melanomas were diagnosed correctly within an 0.2 mm range. Regarding the clinical important limit of 1 mm tumor thickness, 87.2% were diagnosed in accordance with histometric evaluation. An increase of 18% in diagnostic accuracy by dermatoscopy and 87.2% of correctly diagnosed cases of tumor thickness of malignant melanoma by high frequency sonography clearly demonstrate that these methods should be considered standard procedures in the diagnosis of pigmented skin lesions and will facilitate the decision on necessary surgical treatment.     

Intraoperative cytologic diagnosis of unsuspected cardiac myxoma: a case report

BACKGROUND: Atrial myxoma is the most common primary intracardiac tumor. The diagnosis is generally primary intracardiac tumor, based on classical clinical findings coupled with echocardiographic or magnetic resonance image findings demonstrating a cardiac mass. CASE: Unsuspected atrial myxoma was found in a woman who had been diagnosed with invasive lobular carcinoma of the breast. The echocardiographic findings in the presence of fever favored a clinical working diagnosis of infective endocarditis complicating a suspected cardiac metastasis. While intraoperative frozen section examination could not rule out metastatic invasive lobular carcinoma, cytologic touch imprint findings were diagnostic of myxoma. This appears to be the first report of concurrent breast carcinoma and atrial myxoma. To our knowledge, this is also the first report of intraoperative cytologic diagnosis of cardiac myxoma. CONCLUSION: In myxoma cases with a complicated clinical setting in which frozen section examination may be equivocal, intraoperative cytologic examination may be a useful diagnostic tool.     

乳腺肿块X线检查常见漏、误诊原因的病例分析

目的:分析乳腺X线检查的误漏诊原因,提高诊断准确性。方法:选择2011年3月至2013年12月来我院就诊的135例经乳腺X线摄影和病理检查证实的乳腺肿瘤患者为研究对象,将X线摄影结果与病理检查结果对比,进行回顾性的分析。结果:病理诊断72例良性肿瘤而X线误诊为恶性7例(误诊率9.72%);63例恶性肿瘤而X线误诊为良性5例(漏诊率7.93%)。结论:乳腺X线误诊与乳腺致密程度、患者年龄以及肿瘤形态相关。掌握拍片技术减少技术性误差,提高影像质量,诊断时仔细阅片并熟知各类型乳腺疾病的特征性X影像表象,并与临床相结合,增强责任心,可减少乳腺X线检查的误漏诊。