Diagnostic Performance of a Sonographic Volume and Solid Vascular Tissue Score (VSVTS) for Preoperative Risk Assessment of Pediatric and Adolescent Adnexal Masses.

STUDY OBJECTIVE: To evaluate the diagnostic performance of a Volume and Solid Vascular Tissue Score (VSVTS) for preoperative risk assessment of pediatric and adolescent adnexal masses. DESIGN: A retrospective cohort study comprised of all female individuals who presented with an adnexal mass that was managed surgically between April 2011 and March 2016. SETTING: The Hospital for Sick Children (Toronto, Ontario, Canada). PARTICIPANTS: Female individuals 1-18 years of age who presented to a large tertiary pediatric hospital with an adnexal mass that was managed surgically. MAIN OUTCOME MEASURES: Main outcome measures included diagnostic performance of the VSVTS for malignancy via sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+), negative likelihood ratio (LR-), and receiver operating characteristic area-under-the-curve (AUC) analysis. RESULTS: A total of 179 masses in 169 subjects were included. The malignancy rate was 10.6%. The AUC for the VSTVS was 0.919. A VSTVS cut-off value of 4 achieved a sensitivity of 79% (95% CI 0.54-0.93), specificity of 88% (95% CI 0.82-0.93), PPV of 0.44 (95% CI 0.33-0.56), NPV of 0.97 (95% CI 0.94-0.99), LR+ of 6.77 (95% CI 4.18-10.97), and LR- of 0.24 (95% CI 0.10-0.57). CONCLUSIONS: A sonographic scoring system based on the volume and presence of solid vascular tissue improves PPV for preoperative risk stratification of adnexal masses in the pediatric and adolescent population compared to existing ultrasound-only approaches. Further prospective research is needed to determine how best to incorporate components of such scoring systems into clinical management algorithms.

808 nm light triggered lanthanide nanoprobes with enhanced down-shifting emission beyond 1500 nm for imaging-guided resection surgery of tumor and vascular visualization.

Lanthanide based nanoprobe with high efficient down-shifting second near-infrared (NIR-II, 1000-1700 nm) emission has emerged as a promising agent for tumor-associated vascular visualization. However, most of the developed lanthanide-based NIR-II-emissive probes are activated by 980 nm laser, leading to the concern of biological overheating effect. Herein, the high quality 808 nm laser activated NaYF4:Gd/Yb/Er/Nd/Ce@NaYF4:Nd core-shell nanoprobes with significantly improved NIR-II emission beyond 1500 nm and eliminated overheating effect were developed for imaging-guided resection surgery of tumor and vascular visualization. Methods: The core-shell nanoprobe with boosted NIR-II emission and eliminated heating effect was achieved with combination of Nd-sensitizing and Ce-doping strategies. The NIR-II optical imaging and toxicity assessment were demonstrated by in vivo and in vitro experiments. Results: The designed core-shell nanoprobe presented superior NIR-II emission beyond 1500 nm than the core only nanoparticle and NIR-II emission intensity was improved up to 11.0 times by further suppressing the upconversion (UC) pathway through doping Ce3+. More importantly, non-invasive tumor vascular imaging and NIR-II optical imaging-guided surgical resection of tumor were successfully achieved. Conclusion: It is expected that the Nd-sensitized lanthanide-based nanoprobe with significant improvement in NIR-II emission and eliminated overheating effect is a highly promising probe for NIR-II imaging, making it more competitive in non-invasive vascular imaging and imaging-guided tumor resection surgery.

An unusual presentation of pulmonary artery sarcoma: Several pseudoaneurysms with massive hemoptysis.

A 53-year-old woman was admitted to the emergency department with sudden onset of massive hemoptysis. She had previous history of dyspnea and cough for two months. She had no history of chronic disease, smoking, or use of anticoagulant and antiplatelet drugs. On arrival, she was tachycardic and tachypneic, but her body temperature was normal. Chest X-ray showed enlarged right hilus and multiple nodular opacities predominantly in the left lung basis. Computed tomography (CT) scan of the chest demonstrated massive intraluminal filling defect extending from the right pulmonary artery through the main and left pulmonary arteries. Pulmonary artery sarcoma (PAS) was the preliminary imaging-based diagnosis. However, CT also revealed presence of several pseudoaneurysms arising from the distal branches of the left pulmonary artery encased by metastatic nodules. Although hemoptysis is an uncommon presentation for patients with PAS, accompanied pseudoaneurysms were the main reason for massive hemoptysis. Differentiation of PAS from bland thromboembolism could be challenging on CT. Herein reported case provides an additional imaging feature that may utilize differentiating pulmonary artery sarcoma from bland thrombus.

Brief Description of ISSVA Classification for Radiologists.

Ongoing discovery regarding the origin and treatment of vascular anomalies requires standardized nomenclature which itself must undergo iterative updating. This article introduces the 2018 International Society for the Study of Vascular Anomalies (ISSVA) classification, emphasizing the biologic basis of vascular anomalies, summarizing the key features of commonly encountered entities, and serving as a foundation for subsequent articles presented herein. Vascular tumors are discussed to highlight their distinction from vascular malformations which will receive greater attention with respect to management and technical considerations within the issue.

Practical Genetic and Biologic Therapeutic Considerations in Vascular Anomalies.

Vascular anomalies are classified as either tumors or malformations based on clinical findings rendered through radiologic evaluation, physical exam, and histologic interpretation. These findings comprise the phenotype of the disorder. Recently, advances in the molecular genetics of vascular anomalies have shed light on the genotype of these disorders. These phenotype/genotype characterizations will provide a more precise classification of vascular anomalies and identify potential therapeutic targets for expanded treatment options in the future. In this chapter, we will review the phenotype/genotype characterizations and the possible therapeutic pathways for targeted pharmacologic therapy.

Pulmonary vein mass with extension to the left atrium diagnosed by echocardiography.

We report a case of a man aged 65 years presenting with chronic cough, haemoptysis and intermittent atrial tachyarrhythmias on ECG. On 2D transthoracic echocardiography, an incidental left atrial mass was seen, initially thought to be a thrombus predisposed by intermittent atrial fibrillation. Transoesophageal echocardiography confirmed that this left atrial mass originated from a fixed, non-homogenous, right superior pulmonary vein mass with an extracardiac extension. Because of this finding, a thorough search for a primary focus lead to the discovery of a contiguous posterior mediastinal mass, which was a round cell neoplasm on histology. Management was deemed palliative. Although rare, left-sided cardiac masses should prompt the physician to search for a malignancy in the lung in high-risk patients, as haematogenous spread via the pulmonary vein is a potential mechanism for spread.

Myopericytoma occurrence in the liver and stomach space: imaging performance.

BACKGROUND: Myopericytoma is a rare and usually benign tumor, which is even rarer if it occurs in the liver and stomach space. Previous reports of myopericytoma were mostly related to its pathological manifestations, while imaging reports were rare. Here, we report the computed tomography (CT), computed tomography angiography (CTA) and magnetic resonance imaging (MRI) performance for one deep myopericytoma. CASE PRESENTATION: In this study, one deep myopericytoma in the liver and stomach space is reported. A CT that was not contrast-enhanced showed a lobulated tumor with heterogeneous density, and a contrast-enhanced CT showed that the mass had progressive enhancement. CTA showed that the blood-supply of the tumor was supplied by the anterior superior pancreaticoduodenal artery and the left gastric artery. An MRI showed the lesion had isointensity on T1-weighted imaging (T1WI) and slight hyperintensity on T2-weighted imaging (T2WI). The lesion MRI enhancement characteristics were similar to the characteristics from the contrast-enhanced CT. In this case, the enhancement pattern of the tumor was the centrifugal enhancement for both the contrast-enhanced CT and MRI. After surgical resection of the tumor, the pathological diagnosis was myopericytoma, and there was no recurrence in a short-term follow-up. CONCLUSION: The myopericytoma generally has a rich blood supply. When there is necrosis in the center lesion, the lesion has peripheral enhancement. Abdominal myopericytoma could be categorized as having centrifugal enhancement.

"Periportal neoplasms"--a CT perspective: review article.

The periportal space is a potential space surrounding the portal vein and its intrahepatic branches. A variety of neoplasms can involve the periportal region, whether primary or secondary, owing to contiguous spread from surrounding hepatic parenchyma or from adjacent organs. CT plays an important role in not only diagnosing these lesions but also determining the extent of the disease. Most of the malignancies leading to the periportal spread manifest as periportal hypodensity either distinctly or in contiguity with the primary tumour. Even in known malignancies, periportal hypodensity commonly results from non-neoplastic causes like periportal oedema; hence, a knowledge of the imaging findings to ascertain its presence as well as to conclude the definite neoplastic spread is prudent. Periportal spread of neoplasm may suggest locally aggressive or disseminated disease (in extrahepatic malignancies), which may change management accordingly.

Epithelioid angiomatous nodule of the nasal cavity: Report of 2 cases.

Epithelioid angiomatous nodule (EAN) is a novel clinicopathological entity characterized by a benign proliferation of endothelial cells with prominent epithelioid features. It can arise from any region of the body although it is increasingly being recognized in the head and neck. This paper presents two cases of EAN arising in the nasal cavity where the clinical presentations were recurrent unilateral epistaxis. In both cases, the lesions were excised endoscopically. Histopathologic analysis showed well-circumscribed proliferation of solid sheets of epithelioid endothelial cells and vascular channels in a background of mild chronic inflammatory infiltrate. Cytologic atypia was absent with rare mitotic figures seen. Immunohistochemical studies with CD31 and CD34 were strongly positive, thus confirming a vascular origin. Postoperative recovery was uneventful and no recurrence was seen in the follow-up period. EAN can be differentiated from other lesions demonstrating epithelioid vascular proliferation by means of clinical and histopathological characteristics. The present series seeks to inform that EAN should be regarded as a possible, albeit rare, cause of recurrent unilateral epistaxis. We advocate for endoscopic excision of EAN of the nasal cavity for diagnostic and therapeutic purposes.

Sonographic features of rare posterior fetal neck masses of vascular origin.

The purpose of this series is to describe the grayscale and color Doppler sonographic characteristics as well as the histopathologic features of rare solid posterior neck masses identified on prenatal sonography in pregnant patients. We conducted a retrospective review of detailed fetal sonographic examinations of second- and third-trimester pregnancies referred to the Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia for suspected fetal neck masses from June 1998 to December 2011. Eight predominately solid posterior neck masses were identified on 139 studies performed during the study period. Of the 7 cases in which follow-up was available, 6 were confirmed as hemangiomas, and 1 was confirmed as a kaposiform hemangioendothelioma with Kasabach-Merritt syndrome. The most common sonographic features were hypervascularity (7) and calcifications (5). Posterior solid fetal neck masses are rare anomalies. Hemangioma is the most common etiology and should be suggested as the likely diagnosis rather than teratoma, even in the presence of calcifications.

Management of intravenous leiomyomatosis with intracaval and intracardiac extension.

OBJECTIVE: To report the results of management of intravenous leiomyomatosis with intracaval and intracardiac extension at Peking Union Medial College Hospital. METHODS: We reviewed a cohort of 20 patients with intravenous leiomyomatosis extending to the inferior vena cava and heart, focusing on the clinical characteristics, the results of surgical management, and prognosis. RESULTS: The mean age of the patients was 42.4 ± 7.0 years. The clinical manifestations of intravenous leiomyomatosis are various and nonspecific, including pelvic mass, chest tightness and shortness of breath, swelling in the lower extremity, abdominal distension, palpitation, syncope, hypermenorrhea, and skelalgia. All the patients had history of uterine leiomyoma and 16 patients (80%) had undergone uterine leiomyoma operation. After careful preoperative evaluation, nine patients underwent one-stage operations (cardiac surgery, vascular and gynecologic surgery together) and 11 patients underwent two-stage operations (cardiac surgery first, then vascular and gynecologic surgery). There was no significant difference in the postoperative complication rate (33.3% compared with 27.3%; P>.99) between one-stage and two-stage operations. All operations were performed without severe surgical-related complications or death. Approximately 78% of patients had complete resection of tumor and 22.2% of the patients experienced incomplete resection. Eleven (55%) patients received hormone therapy postoperatively. During mean follow-up time of 20.5 months, recurrence occurred in five patients (27.8%) but all the patients survived. CONCLUSIONS: Precise and full-scale preoperative evaluation, complete tumor resection, and multidisciplinary cooperation are crucial for successful treatment.

Mushroom-shaped secondary vasoproliferative tumor.

The authors report a clinical and echographic correlation of a retinal vasoproliferative tumor that assumed a mushroom shape. A 64-year-old man with a history of retinal detachment repair presented with vitreous hemorrhage. A clinical examination including standardized ultrasound was performed, which revealed a mushroom-shaped tumor with high reflectivity. The patient underwent pars plana vitrectomy with fine needle aspiration biopsy. Histopathology of the biopsy specimen demonstrated vascular proliferation. The lesion was diagnosed to be a retinal vasoproliferative tumor. Clinicians should be aware that a mushroom-shaped configuration can be seen in such cases, although it is common for uveal melanoma.

Imaging of primary malignant bone tumors (nonhematological).

Primary malignant bone tumors are uncommon and are diagnosed typically based on radiographic and microscopic findings combined with clinical and demographic features. CT and MR imaging scans are useful in further staging the tumors by determining intraosseous and extraosseous spread.

Soft tissue masses in children.

The diagnosis of soft tissue masses in children can be challenging because there is a broad spectrum of entities that can present as such. Ultrasonography is the primary diagnostic imaging modality in the pediatric age and is especially useful for small and superficial lesions. MR imaging can be helpful, particularly in the evaluation of large and deep soft tissue lesions. Correlation of the imaging findings with the clinical information is crucial in the diagnostic work-up. This article reviews the most common causes of soft tissue masses that require imaging in children.

Management of angiomyolipoma vena cava thrombus during pregnancy.

BACKGROUND: Angiomyolipoma can worsen during pregnancy. Inferior vena cava thrombus of renal angiomyolipoma during pregnancy is rare, and threatens to cause massive emboli. We report a case of vena cava tumor thrombectomy during the second trimester of pregnancy with delayed renal tumorectomy. CASE: A 34-year-old woman with bilateral known angiomyolipomas presented asymptomatic at 30 weeks of gestation with an inferior vena cava thrombus at renal follow-up ultrasonography. Retro-hepatic thrombus had fatty signal on magnetic resonance imaging (MRI). The woman had renal vein and vena cava tumor initial thrombectomy, and had normal cesarean delivery at 39 weeks of gestation. The tumor was treated by postpartum right partial nephrectomy. CONCLUSION: Successful angiomyolipoma isolated thrombectomy during pregnancy with delayed partial nephrectomy is possible. Angiomyolipoma needs follow-up during pregnancy with repeated renal ultrasonography.

Pars plana vitrectomy and endoresection of a retinal vasoproliferative tumor.

A healthy 31-year-old woman developed decreased vision due to vitreous hemorrhage from 2 retinal vascular lesions. Because of the patient's desire for visual rehabilitation and the concern for von Hippel-Lindau (VHL) disease-associated retinal hemangioblastomas, pars plana vitrectomy and endoresection of both lesions were performed. Surgical techniques included the use of chandelier illumination to enable bimanual manipulation of tissue, endolaser around the tumor prior to resection, endodiathermy to cauterize the tumor's feeder vessels, and long-acting gas tamponade following the retinectomy. Histopathology showed a vascular anomaly within the retina that consisted of multiple large vessels with multiple lumens and no evidence of VHL-associated retinal hemangioblastoma. Long-term follow-up revealed no evidence of recurrent disease. This surgical technique may be valuable in select patients to differentiate between retinal vasoproliferative tumors and VHL disease-associated retinal hemangioblastomas for diagnostic, therapeutic, and counseling purposes.

Retinal vascular tumor and peripheral retinal vasculitis in the setting of systemic tuberculosis.

Tuberculosis commonly affects the eye by causing neovascularization, peripheral vasculitis, and choroidal tubercles. The authors describe a 28-year-old man with systemic tuberculosis who presented with a retinal vascular tumor, peripheral retinal vasculitis, retinal neovascularization, and vitreous hemorrhage causing acute vision loss. He was successfully treated with systemic anti-tuberculosis medications, retinal photocoagulation, and focal ablative diode laser to the tumor. Ophthalmologists should consider performing a purified protein derivative test and a chest x-ray for any patient with a history suspicious for tuberculosis who presents with a vascular tumor.

Radiological localizing techniques in adrenal tumors.

The characterisation of adrenal lesions is a common radiological dilemma. Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical. The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT. Such lesions are likely to be seen with increasing frequency given the expanding use of radiological imaging in clinical practice. Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%. Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas. This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases. We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.

Primary angiosarcoma of the brain in a child.

We describe a pediatric case of primary angiosarcoma of the brain displaying striking intravascular papillary pattern, consistent with the "Dabska tumor," often in continuity with a massive, multifocal intravascular papillary endothelial hyperplasia. The tumor contained small hemangioma and obliterated dysplastic arteries as well as very large thin-walled veins. The surrounding brain tissue showed scattered telangiectasias, conglomerates of calcified dysplastic arteries, old hemorrhages and gliosis. Colocalization of these lesions suggests the development of a papillary angiosarcoma in the pre-existing vascular malformation. Although never reported, the possibility of a malignant transformation of endothelial papillary hyperplasia also should be considered in this case.