Incremental Value of Live/Real Time Three‐Dimensional Transesophageal Echocardiography over the Two‐Dimensional Modality in the Assessment of Cardiac Lymphoma

We describe a case of cardiac lymphoma where live/real time three‐dimensional transesophageal echocardiography provided additional information compared to two‐dimensional transesophageal echocardiography regarding the extent of tumor infiltration. In addition, it gave a quantitative assessment of the tumor burden by providing its volume.     

The role of FDG‐PET/CT in the evaluation of residual disease in paediatric non‐Hodgkin lymphoma

¹⁸F‐labelled–fluorodeoxyglucose positron emission tomography (FDG‐PET) findings are challenging to interpret for residual disease versus complete response in paediatric patients with non‐Hodgkin lymphoma (NHL). A biopsy is often warranted to confirm the presence or absence of viable tumour if there is clinical or radiographic evidence of residual disease. In this study, we compared conventional imaging and FDG‐PET/computerized tomography (CT) findings with biopsy results in 18 children with NHL. Our goal was to provide additional data to establish more reliable criteria for response evaluation. Residual disease was suspected after conventional imaging alone in eight patients, after FDG‐PET/CT alone in three and after both modalities in seven patients. Biopsy confirmed the presence of viable tumour in two patients. Two additional patients experienced progressive disease or relapse. The sensitivity and negative predictive value of FDG‐PET/CT using the London criteria to indicate residual tumour detectable by biopsy were 100%, but specificity was low (60%), as was the positive predictive value (25%). Thus, in this study, a negative FDG‐PET/CT finding was a good indicator of complete remission. However, because false‐positive FDG‐PET/CT findings are common, biopsy and close monitoring are required for accurate determination of residual disease in individual patients.     

Neovascularized Myxoma‐Causing Abnormal Blood Flow in the Left Atrium Diagnosed by Transesophageal Echocardiography

Myxomas are the most common primary cardiac tumor, and neovascularization of cardiac myxoma has been shown in previous case reports. However, it is seldom reported that abnormal blood flow spurted from a neovascularized myxoma into the cardiac chamber. In this unusual case report, we present a left atrial myxoma in a 44‐year‐old woman with atypical angina. Transesophageal echocardiography (TEE) revealed several small blood streams spurting from the surface of massively vascularized myxoma into the left atrium. Coronary angiography showed a feeding artery which arose from the right coronary artery and supplied the left atrial myxoma, with no evidence of coronary artery stenosis. This case demonstrated that TTE might be helpful for confirming the neovascularization of myxoma in the left atrium and finding the abnormal blood flow that spurts from the neovascularized myxoma. We speculate that the abnormal blood flow spurting from the neovascularized myxoma into the left atrium may have caused a coronary steal phenomenon thus resulting in myocardial ischemia.     

Bone marrow 18F‐fluoro‐2‐deoxy‐d‐glucose positron emission tomography/computed tomography cannot replace bone marrow biopsy in diffuse large B‐cell lymphoma

This study aimed to investigate whether visual and quantitative ¹⁸F‐fluoro‐2‐deoxy‐d ‐glucose positron emission tomography/computed tomography (FDG‐PET/CT)‐based bone marrow assessment can replace blind bone marrow biopsy (BMB) in newly diagnosed diffuse large B‐cell lymphoma (DLBCL). This retrospective study included 78 patients with newly diagnosed DLBCL who had undergone both FDG‐PET/CT and BMB. FDG‐PET/CT images were visually evaluated for bone marrow involvement. Patient‐based sensitivity of visual FDG‐PET/CT assessment was calculated using BMB as the reference standard. Metabolically active volume, maximum standardized uptake value, 3D partial volume corrected mean standardized uptake value, and 3D partial volume corrected mean metabolic volume product (cMVP_mean) of FDG‐avid bone marrow lesions were measured. Cox regression analysis was used to determine the influence of (potential) prognostic factors (BMB status, visual [dichotomous] FDG‐PET/CT bone marrow status, metabolically active volume, maximum standardized uptake value, 3D partial volume corrected mean standardized uptake value, 3D partial volume corrected mean metabolic volume product, and International Prognostic Index score) on progression‐free survival and overall survival. FDG‐PET/CT detected bone marrow involvement in 34 (43.6%) cases and BMB in 16 (20.5%) of 78 cases, of whom 11 were also detected by FDG‐PET/CT, resulting in a patient‐based sensitivity of 68.8% (95% confidence interval = 44.2%–86.1%) for FDG‐PET/CT. In the multivariate Cox proportional hazards model, only BMB status was an independent predictive factor of progression‐free survival (P = 0.016) and overall survival (P = 0.004). In conclusion, FDG‐PET/CT misses bone marrow involvement that has been detected by BMB in a non‐negligible proportion of patients. Furthermore, both visual and quantitative FDG‐PET/CT‐based bone marrow assessments are prognostically inferior to BMB. Therefore, FDG‐PET/CT cannot replace BMB in newly diagnosed DLBCL. Am. J. Hematol. 89:726–731, 2014. © 2014 Wiley Periodicals, Inc.     

FDG PET/CT findings in acute adult mononucleosis mimicking malignant lymphoma

We report a case of acute Epstein–Barr virus (EBV) infection in which the clinical syndrome and pattern of F-18-2-fluoro-2-deoxy- d -glucose (FDG) uptake mimicked malignant lymphoma. A 53-yr-old man presented with 2 wk of high fevers, night sweats, sinus congestion and severe fatigue. The patient was treated for 1 wk with broad-spectrum antibiotics for acute sinusitis without any improvement. Persistence of fevers and presence of abnormal lymphadenopathy seen on the abdominal computed tomography (CT) were concerning lymphoma with B symptoms. FDG positron emission tomography (PET) showed avid FDG uptake in numerous abdominal/pelvic nodes, liver, spleen and bone marrow. These findings were highly suspicious for lymphoma. Patient underwent a pelvic lymph node biopsy which showed large granular lymphocytes that were predominantly T cells. Bone marrow biopsy showed diffuse infiltration with plasmocytoid cells that were not kappa lambda restricted. Additional diagnostic workup became available showing positive EBV IgM titers and negative IgG levels indicating acute infectious mononucleosis. Lymph node biopsy stained positively with EBV-encoded RNA. We concluded that patient's abnormal FDG PET was most likely secondary to acute EBV infection. After 2 months, follow-up FDG PET/CT showed marked improvement in lymphadenopathy and decreased hypermetabolic activity in the liver and spleen. Other than EBV, there are many other FDG-avid non-malignant conditions that may lead to false-positive PET scans. FDG accumulates in inflamed and infected lesions with increased glucose metabolism. This case underscores the importance of maintaining a broad differential and restricting use of PET scans for staging.     

Port site and distant metastases of gallbladder cancer after laparoscopic cholecystectomy diagnosed by positron emission tomography

We report port site and distant metastases of unsuspected gallbladder cancer after laparoscopic cholecystectomy diagnosed by positron emission tomography （PET） in two patients. Patient 1, a 72-yearold woman was diagnosed as cholelithiasis and cholecystitis and received laparoscopic cholecystectomy. Unsuspected gallbladder cancer was discovered with histological result of well-differentiated squamous cell carcinoma of the gallbladder infiltrating the entire wall. A PET scan using F-18-fluorodeoxyglucose （FDG- PET） before radical resection revealed residual tumor in the gallbladder fossa and recurrence at port site and metastases in bilateral hilar lymph nodes. Patient 2, a 69-year-old woman underwent laparoscopic cholecystectomy more than one year ago with pathologically confirmed unsuspected adenosquamous carcinoma of stage pTlb. At 7-mo follow-up after surgery, the patient presented with nodules in the periumbilical incision. Excisional biopsy of the nodule revealed adenosquamous carcinoma. The patient was examined by FDG-PET, demonstrating increased FDG uptake in the right lobe of the liver and mediastinal lymph nodes consistent with metastatic disease. This report is followed by a discussion about the utility of FDG-PET in the gallbladder cancer.     

Controversies on the prognostic value of interim FDG‐PET in advanced‐stage Hodgkin lymphoma

Hodgkin lymphoma, even in advanced‐stage, is a highly curable malignancy, but treatment is associated with short‐term toxicity and long‐term side effects. Early predictive markers are required to identify those patients who do not require the full‐length standard therapy (and thus qualify for therapy de‐escalation) and those patients who will not be cured by standard therapy (and thus qualify for therapy escalation). Multiple trials have assessed the value of ¹⁸F‐fluoro‐2‐deoxy‐d ‐glucose positron emission tomography (FDG‐PET) after a few cycles of chemotherapy (also known as ‘interim FDG‐PET’) in predicting outcome in advanced‐stage Hodgkin lymphoma. Furthermore, multiple interim FDG‐PET‐adapted trials, in which patients with positive interim FDG‐PET scans are assigned to escalated therapies, and patients with negative interim FDG‐PET scans are assigned to de‐escalated therapies, have recently been published or are currently ongoing, with generally heterogeneous results. The present article reports the currently available evidence (and controversies) on the prognostic value of interim FDG‐PET in advanced‐stage Hodgkin lymphoma in patients with positive and negative interim FDG‐PET findings following continuation of standard chemotherapy or escalated/de‐escalated therapy.     

Incremental Value of Live/Real Time Three‐Dimensional Transesophageal Echocardiography over the Two‐Dimensional Technique in the Assessment of Primary Cardiac Malignant Fibrous Histiocytoma

We describe a case of primary cardiac malignant fibrous histiocytoma where live/real time three‐dimensional transesophageal echocardiography added incremental value to the two‐dimensional modalities. Specifically, the three‐dimensional technique allowed us to delineate the true extent and infiltration of the tumor, to identify characteristics of the tumor mass suggestive of its malignant nature, and to quantitatively assess the total tumor burden.     

A retrospective observational study of benign anthracotic lymphadenitis and its association with PET avid lymph nodes in patients undergoing cancer evaluation

BackgroundAccurate staging of newly diagnosed or recurrent malignancy is essential for effective treatment. An important first step in staging involves the use of PET/CT to identify areas of FDG avidity. PET/CT however has limitations, including false positive FDG uptake from benign causes. In this paper we characterize an uncommon yet clinically important cause of false positive PET/CTs, that of benign anthracotic lymphadenitis (BAL). We examine the clinical, radiographic and histologic characteristics of BAL in patients referred for endobronchial ultrasound (EBUS) guided biopsies and discuss its context in relation to existing literature.MethodsWe performed a retrospective observational case series of 20 patients who were referred for EBUS guided biopsies of PET positive mediastinal and hilar lymph nodes during the work-up or treatment of suspected malignancy.ResultsTo be included, all patients received PET imaging as well as an EBUS guided biopsy of FDG avid lymph nodes which demonstrated anthracotic pigment as the only histologic abnormality. The key findings were that 90% of patients in this cohort were born outside of the US, 90% had bilateral FDG avid lymph nodes with an average standardized uptake value (SUV) of 7.9±2.2. Most patients, based on their history, had a likely exposure to biomass fuel or urban pollution.ConclusionsBAL may be an underrecognized cause for PET positive lymph nodes in patients undergoing work-up for malignancy. These findings support the importance of sampling mediastinal and hilar lymph nodes even when SUVs are highly suggestive of malignancy.     

Use of echocardiography as an essential tool for targeted transcatheter biopsy of cardiac masses

Echocardiography has emerged as an essential tool to guide targeted, transcatheter biopsy of cardiac masses. Options for imaging include transthoracic or transesophageal echocardiography and intracardiac echocardiography, with appropriate use being dictated by specific patient characteristics and institutional experience. The authors present a case of three‐dimensional (3‐D) transesophageal echocardiography‐guided transcatheter biopsy of a right ventricular mass and review the current use of echocardiography to guide these procedures.     

Successful treatment of primary cardiac angiosarcoma with docetaxel and radiotherapy

A 49-year-old man was admitted for primary cardiac angiosarcoma with a cardiac tamponade. Transthoracic echocardiography and contrast-enhanced computed tomography scan demonstrated a large mass in the right atrium and thickening of the right ventricular wall. 18F-labeled deoxyglucose (FDG) positron emission tomography (PET) scan showed increased FDG uptake in the mediastinum and over the heart. The patient responded to combination therapy with docetaxel and radiotherapy and tolerated the treatment well, except for radiation esophagitis, which required a soft diet and resolved 1 month after treatment. This combination therapy resulted in a minimal response with slight regression in the tumor size, but FDG-PET initially showed an increase in FDG uptake by the tumor that was no longer seen after combination therapy. There is no evidence of progression or metastasis even at 12 months after diagnosis.     

FDG‐PET/CT in the management of lymphomas: current status and future directions

FDG‐PET/CT is the current state‐of‐the‐art imaging in lymphoma and plays a central role in treatment decisions. At diagnosis, accurate staging is crucial for appropriate therapy selection: FDG‐PET/CT can identify areas of lymphoma missed by CT alone and avoid under‐treatment of patients with advanced disease stage who would have been misclassified as having limited stage disease by CT. Particularly in Hodgkin lymphoma, positive interim FDG‐PET/CT scans are adversely prognostic for clinical outcomes and can inform PET‐adapted treatment strategies, but such data are less consistent in diffuse large B‐cell lymphoma. The use of quantitative FDG‐PET/CT metrics using metabolic tumour volume, possibly in combination with other biomarkers, may better define prognostic subgroups and thus facilitate better treatment selection. After chemotherapy, FDG‐PET/CT response is predictive of outcome and may identify a subgroup who benefit from consolidative radiotherapy. Novel therapies, in particular immunotherapies, exhibit different response patterns than conventional chemotherapy, which has led to modified response criteria that take into account the risk of transient pseudo‐progression. In relapsed lymphoma, FDG‐PET/CT after second‐line therapy and prior to high‐dose therapy is also strongly associated with outcome and may be used to guide intensity of salvage therapy in relapsed Hodgkin lymphoma. Currently, FDG‐PET/CT has no role in the routine follow‐up after complete metabolic response to therapy, but it remains a powerful tool for excluding relapse if patients develop clinical features suggestive of disease relapse. In conclusion, FDG‐PET/CT plays major roles in the various phases of management of lymphoma and constitutes a step towards the pursuit of personalized treatment.     

Primary cardiac angiosarcoma: A case report

Cardiac angiosarcomas are the most common primary malignant cardiac tumors in adults. The diagnosis is often delayed due to nonspecific clinical symptoms at presentation. The cornerstones of diagnosis are echocardiography and the histological evaluation of the cardiac biopsy. The knowledge on the treatment is limited; the outcomes of chemotherapy, radiotherapy, complete surgical removal, and heart transplantation are controversial. We report a 38‐year‐old woman with a primary heart tumor which infiltrated the right atrial wall and the pericardium and caused pericardial effusion. Angiosarcoma was verified histologically. The surgical excision could not be radical, and the patient died 3 months from diagnosis.     

Multimodality Imaging in the Assessment of Cardiac Lymphoma Presented as New‐Onset Atrial Fibrillation

Cardiac involvement by non‐Hodgkin's lymphoma is not uncommon, however rarely diagnosed during life due to nonspecific clinical presentation. We report a case of secondary cardiac lymphoma in patient who presented with new‐onset atrial fibrillation. Cardiac lymphoma was in a form of bulky right atrial mass, infiltrating the atrial septum and cavo‐atrial junction with concomitant mild pericardial effusion. In the present case, we illustrate complementary role of transthoracic, transesophageal echocardiography and multislice CT scan with three‐dimensional reconstruction, in detection and evaluation of secondary cardiac tumor. Usefulness of echocardiography to follow up the effects of chemotherapy is also shown. (Echocardiography 2010;27:332‐336)     

Asymptomatic metastatic osteosarcoma to the right ventricle: Case report and review of the literature

Metastatic cardiac tumors are far more common than primary tumors, and benign primary cardiac tumors are common than malignant tumors. We report a 22-year-old Saudi woman with right femur osteosarcoma who was found to have a large right ventricular mass by transthoracic and transesophageal echocardiography. Diagnosis was highly suggestive by cardiac magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan. We performed a review of the literature for metastatic osteosarcoma of the right ventricle.     

Assessment of Residual Viability by Enoximone Echocardiography in Patients with Previous Myocardial Infarction Correlation with Positron Emission Tomographic Studies and Functional Follow‐Up

Background: The aim of this study was to evaluate enoximone echocardiography (EE) for the identification of residual myocardial viability in postinfarction patients. Findings obtained during EE were compared with those acquired by myocardial uptake of fluorine‐18 fluorodeoxyglucose (¹⁸F‐FDG) positron emission tomography (PET) and functional follow‐up results. Methods: Twenty‐five patients underwent EE and PET ¹⁸F‐FDG studies. An asynergic segment was considered as having contractile enhancement when the wall motion score decreased by ≥1 grade during EE and was defined as viable if ¹⁸F‐FDG uptake score was ≥2 grade on PET. Results: Of 293 dysfunctional segments at baseline, 139 (47%) were viable by PET criteria; 117 (40%) had contractile enhancement induced by enoximone (P = 0.07). Agreement between EE and PET was found in 75% of involved segments (K = 0.46, P < 0.001). The majority of discrepancies (65%, P < 0.01) were mainly due to discordant segments in which PET revealed evidence of ¹⁸F‐FDG uptake but EE showed no change in wall motion. In 179 revascularized segments, negative predictive value for functional recovery of both tests reached the same value (89% for both), whereas positive predictive value was 82% for EE and 68% for PET, respectively (P < 0.05). Sensitivity was 85% for EE and 88% for PET (P = ns); specificity was 87% and 70%, respectively (P < 0.01). Conclusions: EE yields a fair concordance with PET study. Compared with PET, despite a similar negative accuracy, EE shows a greater specificity for prediction of function recovery after revascularization. (Echocardiography 2010;27:544‐551)     

Added Value of Real Time Three‐Dimensional Echocardiography in the Diagnosis of an Apical Right Ventricular Metastasis from Malignant Melanoma

In a man presenting to the emergency room with dyspnea and atypical chest pain irradiated among the scapulae, with new‐onset diffuse negative T‐waves on the ECG, the first clinical and diagnostic hypothesis was pulmonary embolism (PE). However, computed tomography (CT) performed in emergency was negative for PE, showing instead a marked defect in right ventricle (RV) filling. For this reason, echocardiography was performed to better investigate the nature of the space‐occupying lesion, and several echocardiographic modalities were used (two‐dimensional transthoracic and transesophageal echocardiography and three‐dimensional [3D] transthoracic echocardiography). They revealed the presence of a mass attached to the apex of the RV, partially obstructing the inflow and outflow tracts. Cardiac magnetic resonance imaging was also performed, confirming the findings of 3D echocardiography. After that, several other diagnostic imaging techniques were used for disease staging, since the patient had a history of surgical excision of a malignant melanoma of the skin several years before. Whole‐body CT, soft tissue echography and positron emission tomography revealed the widespread diffusion of the primary tumor to distant organs. For this reason, we suspected that the RV mass could also be an intracardiac metastasis from malignant melanoma, and did not perform biopsy given the bad clinical conditions and the worse prognosis of the patient. However, he was entered in an experimental therapeutic protocol with Vemurafenib because he showed B‐RAF gene mutation at molecular gene analysis.