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Pulmonary artery intimal sarcoma (PAIS) is a rare and aggressive malignancy originating from the intimal layer of the pulmonary artery with poor prognosis due to its aggressive nature. The management of PAIS poses both diagnostic and therapeutic challenges. It presents with nonspecific symptoms and is often misdiagnosed as pulmonary embolism. While surgical resection is the primary treatment modality, the role of adjuvant chemotherapy and radiotherapy remains uncertain. However, given the high recurrence rate, adjuvant chemotherapy and/or radiotherapy have been utilized in a limited number of case reports. We present the case of a 46-year-old woman who was diagnosed with PAIS and underwent surgical resection followed by adjuvant chemotherapy (ChT) and radiotherapy (RT), demonstrating good tolerance to this multimodal treatment approach., (© 2024. The Author(s).)

Despite significant advances in their molecular pathogenesis, skeletal vascular tumors remain diagnostically challenging due to their aggressive radiologic appearance and significant morphologic overlap. Within the epithelioid category and at the benign end of the spectrum, recurrent FOS/FOSB fusions have defined most epithelioid hemangiomas, distinguishing them from epithelioid hemangioendothelioma and angiosarcoma. More recently, the presence of EWSR1/FUS :: NFATC1/2 fusions emerged as the genetic hallmark of a novel group of unusual vascular proliferations, often displaying epithelioid morphology, with alternating vasoformative and solid growth, variable atypia, reminiscent of composite hemangioendothelioma. In this study, we further our understanding and morphologic spectrum of NFATC -fusion positive vascular neoplasms by describing 9 new cases, including soft tissue locations and novel fusion partners. Combining with the initial cohort of 5 cases, a total of 14 patients were analyzed, showing slight female predilection and an age range of 10 to 66 (mean 42 y). Twelve patients had solitary lesions, while 2 had multifocal polyostotic (pelvic bones) disease. Overall, 12 lesions were intra-osseous and 2 in soft tissue. By targeted RNA Fusion panels or FISH, there were 6 cases of EWSR1::NFATC1 , 4 EWSR1::NFATC2 , 2 FUS::NFATC2 , 1 EWSR1 rearrangement, and 1 with a novel FABP4::NFATC2 fusion. Follow-up was available in 4 patients. One patient experienced 2 local recurrences, 11 and 15 years postdiagnosis, and one patient experienced progressive disease despite multimodality treatment (curettings, embolization, radiation) over 3 years. In summary, our extended investigation confirms that NFATC -related fusions define a distinct group of vascular neoplasms with variable architecture, epithelioid phenotype, and cytologic atypia, commonly located in the bone, occasionally multifocal and with potential for local recurrence and aggressive behavior but no metastatic potential. Molecular analysis is recommended in diagnostically challenging cases with atypical histology to exclude malignancy., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [ Pediatr Ann . 2024;53(4):e129-e137.] .

Purpose: Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare, aggressive, multi-system disease that can affect the eye. We describe the ophthalmic presentation, multimodal imaging and treatment response of uveal IVLBCL., Methods: Review and case report., Results: Twenty-five published cases of IVLBCL involving the eye including our own were identified. Of these, 15 patients (60%) had clinically-detectable intraocular involvement, 6 (24%) had extraocular ophthalmic involvement only and 4 (16%) had subclinical, undiagnosed intraocular involvement that was retrospectively detected on post-mortem ocular histopathology. The male to female ratio was 1.08:1 with a mean presenting age of 65.1 ± 11.7 years (range 38-82 years). The majority of cases had bilateral involvement (21/25 patients, 84%). Extraocular manifestations included diplopia, ptosis and ophthalmoplegia. Intraocular manifestations included serous retinal detachment (13/28, 46%), retinal hemorrhages (9/28, 32%), vascular changes (9/28, 32%), retinal pigment epithelial changes (7/28, 25%), thickened choroid (6/28, 21%), vitritis (5/28, 17%), cotton-wool spots (3/28, 10%), and a subretinal lesion (1/28, 3%). Histopathological diagnosis was most commonly confirmed on post-mortem enucleation (8/25 patients, 32%), skin (6/25 patients, 24%) or brain biopsy (6/25 patients, 24%)., Conclusion: The presence of intra-retinal hemorrhages, cotton wool spots and/or Roth spots help differentiate IVLBCL from other similarly presenting diseases such as central serous chorioretinopathy and Vogt-Koyanagi-Harada disease. New signs not previously described in IVLBCL include macular bacillary layer detachment and hypo-cyanescent spots on ultra-wide field indocyanine green angiography. The diagnosis is elusive and requires tissue biopsy, but systemic chemotherapy and rituximab can lead to rapid improvement of the eye., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Pulmonary artery sarcoma (PAS) is a rare, aggressive cancer originating from the intimal layer of the pulmonary artery (PA), often mistaken for pulmonary thromboembolism. This case report underscores the complex management of PAS and the necessity of a multidisciplinary approach for accurate diagnosis and treatment. A 52-year-old woman with PAS was diagnosed using imaging and therapeutic tests to distinguish it from pulmonary embolism. Primary treatment included surgical resection of the pulmonary trunk, valve, and tumor, followed by reconstruction. Complete resection was impossible due to extensive endocardial infiltration in the right ventricle, precluding cardiac transplant. The patient underwent adjuvant radiotherapy; however, the disease recurred, and she died 3 years post-diagnosis. This case highlights the rarity of an extensive right ventricle invasion, the absence of clear PAS management guidelines, and the limited evidence on the effectiveness of adjuvant therapies. It concludes that multidisciplinary teams are vital for decision-making and stresses the need for further research to establish effective treatment protocols.

Vascular tumors are classified into three categories by the International Society for the Study of Vascular Anomalies (ISSVA): benign, locally aggressive/borderline, and malignant. Many of these tumors are rare, cutaneous in nature, and present in childhood. The characterization and delineation of these distinct vascular tumors is an evolving area of clinical research. The diagnosis of these lesions relies on history and clinical presentation, location, histologic appearance, immunohistochemistry, and more recently, associated genetic mutations. This article provides a brief, yet comprehensive overview of all cutaneous vascular tumors currently recognized by the ISSVA, including presentation, diagnosis, and treatment., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Vascular anomalies are a group of disorders divided into two distinct subtypes: vascular tumors and vascular malformations. Vascular tumors are proliferative in nature, while malformations are nonproliferative. Simple, localized vascular malformations refer to a group of malformations that are localized to a single area of involvement. These simple malformations include capillary, lymphatic, venous, and arteriovenous malformations. The pediatric hematologists and oncologists are becoming increasingly involved in the diagnosis and management of these disorders. This review presents four cases as a means to discuss the diagnosis, clinical and imaging features, and management strategies of simple, localized vascular malformations., (© 2021 Wiley Periodicals LLC.)

Introduction: Primary intravenous leiomyosarcomas are rare vascular tumors with aggressive disease biology. The diagnosis and management have been challenging as little data exist from large databases., Methods: A literature search was done to identify all cases of primary leiomyosarcomas in the last five years. Clinicopathological features and management strategies were evaluated., Results: The median age was 53 years, predominantly females (2.5:1), presenting as metastases in up to 12.1% cases. Most tumors were locally advanced with a median size of 10cm. Inferior vena cava involvement from renal veins to infrahepatic veins remains the most frequent site (57.1%cases) while nearly half (52.8%) proceeded for surgery without histological proof. Most patients could undergo upfront resection (88.0%) with few patients receiving neoadjuvant chemotherapy (4.3%) or neoadjuvant radiotherapy (2.2%). Significant multivisceral resections included right nephrectomy (41.3%), liver resection (25.7%) and left nephrectomy (2.2%). Most patients (91.8%) needed an inferior vena cava graft placement with remarkable microscopically negative margins (85.5% cases). Doxorubicin and ifosfamide were the most frequently used combination chemotherapy regimens in both pre and postoperative settings with partial responses. The median overall and disease free survival among operated patients was 60 months and 28 months respectively. In multivariate analysis large tumor, extensive inferior vena cava involvement, and need for adjuvant chemotherapy appeared significant predictors for overall survival., Conclusions: Aggressive upfront surgical resection with clear margin remains the key for long-term survival. Doxorubicin-based regimens were preferred as neoadjuvant chemotherapy while adjuvant treatment with chemotherapy, radiotherapy, or both may be considered in high-risk patients.

Objectives: A retrospective cohort study was designed to describe the clinical features and outcomes of pulmonary artery sarcoma (PAS)., Methods: Twenty-two (22) consecutive patients diagnosed with PAS by pathological examination were enrolled and followed up until they died or until January 2020. The medical records were retrospectively reviewed to evaluate the clinical characteristics, image findings, and outcomes., Results: 1) Twenty-one (21, 95.5%) patients were firstly misdiagnosed. Dyspnoea was the most common presenting symptom (19 of 22, 86.4%). 2) Filling defects in the right pulmonary artery were seen in 17 patients (77.3%) with computed tomography pulmonary angiography or magnetic resonance pulmonary angiography. Among those patients, 14 underwent positron emission tomography-computed tomography detection and 13 (92.9%) were found to have increased uptake value in the pulmonary artery. 3) The median survival (from diagnosis to death or January 2020) of the total series was 11.6 months (range, 0.7-68.5 months). The estimated cumulative survival rates at 1, 2, and 3 years were 52.6%, 32.8%, and 19.7%, respectively. Patients who received surgery and/or chemo-radiotherapy treatment had a better survival rate compared with patients without treatment (the estimated cumulative survival rates at 1, 2, and 3 years were 60.3%, 39.1%, and 29.3%, respectively, vs 33.3%, 16.6%, and 0, accordingly) and better survival time (median survival 17.02 vs 3.16 months, respectively) (p=0.025)., Conclusions: Pulmonary artery sarcoma is easily misdiagnosed, as the symptoms and routine image detection are nonspecific. Positron emission tomography-computed tomography may be helpful in diagnosis. Surgery and/or chemo-radiotherapy offer a chance for better outcomes., (Copyright © 2021 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objective: We sought to compare overall survival (OS) and disease control for patients with localized pancreatic ductal adenocarcinoma (PDAC) treated with ablative dose radiotherapy (A-RT) versus resection., Summary Background Data: Locoregional treatment for PDAC includes resection when possible or palliative RT. A-RT may offer durable tumor control and encouraging survival., Methods: This was a single-institution retrospective analysis of patients with PDAC treated with induction chemotherapy followed by A-RT [≥98 Gy biologically effective dose (BED) using 15-25 fractions in 3-4.5 Gy/fraction] or pancreatectomy., Results: One hundred and four patients received A-RT (49.8%) and 105 (50.2%) underwent resection. Patients receiving A-RT had larger median tumor size after induction chemotherapy [3.2 cm (undetectable-10.9) vs 2.6 cm (undetectable-10.7), P < 0.001], and were more likely to have celiac or hepatic artery encasement (48.1% vs 11.4%, P <0.001), or superior mesenteric artery encasement (43.3% vs 9.5%, P < 0.001); however, there was no difference in the degree of SMV/PV involvement (P = 0.123). There was no difference in locoregional recurrence/progression at 18-months between A-RT and resection; cumulative incidence was 16% [(95% confidence interval (CI) 10%-24%] versus 21% (95% CI 14%-30%), respectively (P= 0.252). However, patients receiving A-RT had a 19% higher 18-month cumulative incidence of distant recurrence/progression [58% (95% CI 48%-67%) vs 30% (95% CI 30%-49%), P= 0.004]. Median OS from completion of chemotherapy was 20.1 months for A-RT patients (95% CI 16.4-23.1 months) versus 32.9 months (95% CI 29.7-42.3 months) for resected patients (P < 0.001)., Conclusion: Ablative radiation is a promising new treatment option for PDAC, offering locoregional disease control similar to that associated with resection and encouraging survival., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: This study aimed to assess the feasibility of preoperative chemoradiotherapy using gemcitabine plus nab-paclitaxel (GnP) and to determine the recommended dose (RD) of nab-paclitaxel for patients with localized pancreatic ductal adenocarcinoma (PDAC)., Methods: The participants had localized PDAC with contact or invasion to major arteries. They received GnP on days 1, 15, 29, and 43. The dose of gemcitabine was fixed at 600 mg/m2, whereas that of nab-paclitaxel was at 3 dose levels in accordance with a standard 3 + 3 dose escalation scheme. Three-dimensional radiotherapy was administered concurrently to a total dose of 50.4 Gy per 28 fractions., Results: The study cohort comprised 15 patients. Grade 3 or 4 neutropenia was observed in 4 (26.7%), leukopenia in 1 (6.7%), biliary infection in 2 (13.3%), appetite loss and nausea in 1 (6.7%), and anaphylaxis in 1 (6.7%). The RD was determined as level 2 (gemcitabine, 600 mg/m2; nab-paclitaxel, 100 mg/m2). Three patients underwent pancreatectomy after additional chemotherapy and achieved R0 resection., Conclusions: The RD of nab-paclitaxel in our chemoradiotherapy protocol using GnP was 100 mg/m2 with gemcitabine 600 mg/m2 and 3-dimensional conformal radiotherapy to a total dose of 50.4 Gy per 28 fractions for patients with localized PDAC., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: We aimed to assess the clinical efficacy of bypass grafting in recurrent nasopharyngeal carcinoma patients with internal carotid artery invasion., Methods: A retrospective analysis was performed involving 51 patients either operated by bypass grafting (n = 22) or treated with repeated chemo-radiotherapy (n = 29)., Results: Four patients in the bypass grafting group died 3-5 months after the operation due to epistaxis and pulmonary infection (4/22, 18.2%), and three more patients exhibited a modified Rankin Scale (mRS) ≥ 2 during the follow-up (3/22, 13.6%). In the repeated chemo-radiotherapy group, 8 patients died (8/29, 27.6%), including seven patients dying within 2-3 months due to epistaxis and pulmonary infection (7/29, 24.1%). One more patient died of epistaxis after 9 months. The difference in mortality between the two treatment groups within 3 months of treatment was statistically significant (P = 0.038)., Conclusions: Cerebrovascular reconstruction after detailed collateral flow assessment is an effective treatment for recurrent NPC patients with internal carotid artery invasion., (Copyright © 2020. Published by Elsevier Inc.)

Abstract: Prostate-specific membrane antigen (PSMA) is expressed in the tumor-associated endothelial neovasculature of various nonprostatic benign and malignant neoplasms. A 25-year-old man with recurrent sinonasal glomangiopericytoma underwent whole-body 68Ga PSMA PET/CT to explore its theranostic role. There was intense PSMA uptake (SUVmax = 23.9) noted in the tumor. The uptake was more than that of the salivary glands, lacrimal glands, aorta, spleen, and the liver. Performance of PSMA PET/CT in sinonasal glomangiopericytoma opens up new frontiers concerning radiological imaging, early recurrence identification, and perhaps even radioligand therapy of residual/recurrent tumors., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Aims: To characterise the karyotypic abnormalities and heterogeneities in intravascular lymphoma (IVL)., Methods: G-banded karyotyping was performed on biopsy specimens from a single-centre IVL cohort comprising intravascular large B-cell lymphoma (IVLBCL, n=12) and NK/T-cell lymphoma (IVNKTCL, n=1)., Results: Five IVLBCL cases and one IVNKTCL case (total 46%) were found to have normal karyotypes, and the cytogenetic abnormalities observed in the other seven IVLBCL cases (54%) were investigated further. These seven karyotypes were uniformly complex with an average of 13 aberrations. The seven cases all had abnormalities involving chromosome 6, with 57% involving structural abnormalities at 6q13, and chromosome 8, with 43% involving abnormalities at 8p11.2. In addition, 71% had aberrations at 19q13. On average, 4.4 chromosomal gains and losses were detected per case. Cytogenetic heterogeneities were observed in six cases (86%) and tetraploidy in three cases (43%). There was no significant difference in progression-free survival (p=0.92) and overall survival (p=0.61) between the IVLBCL cases with complex and normal karyotypes., Conclusion: Approximately half of IVLBCL cases had a highly heterogeneous pattern of karyotypes with different clonal numerical and structural chromosome aberrations., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Leiomyosacroma of the inferior vena cava is an extremely rare malignancy originating from the tunica media of the venous wall. Its symptoms and radiomorphological signs do not always lead to an accurate diagnosis. Histological sampling can be dangerous due to its location. Therefore the diagnosis is often a challenge for clinicians. Its treatment is primarily surgical, supplemented by radiotherapy and chemotherapy applied together or in monotherapy. In our case, an asymptomatic 74-year-old female patient was diagnosed with a tumor of the inferior caval vein located just above the right renal vein and involving the right adrenal gland. As serum cortisol, epinephrine, norepinephrine, adrenocorticotropic hormone (ACTH), total and free testosterone, dehydroepiandrosterone sulfate (DHEA-S), sex hormone binding globulin (SHBG) was at normal level, the tumor showed no hormone secretion. Primary surgical resection was planned. Preoperative biopsy was not performed due to its high risk of complications. During operation, tumor resection was performed by resection of the venous wall, removal of the right adrenal gland and neoimplantation of the right renal vein. For better exposure, cholecystectomy was also performed at the beginning of surgery. There were no postoperative complications. The patient received adjuvant radiation therapy. Thanks to the early diagnosis of the tumor and the complete resection, we significantly increased the patient’s chances of total recovery. With the radiotherapy performed, we reduced the likelihood of tumor recurrence.

Vascular tumors are a part of the vascular anomalies spectrum. Vascular malformations are congenital vascular lesions, originating from a mesenchymal stem cell defect and distinguished from vascular tumors by their low cell turnover and lack of invasiveness. They tend to grow in proportion to the child. Vascular tumors are proliferative and range from benign proliferation to malignant tumors. The appropriate differential diagnosis is imperative. Infantile hemangioma can be diagnosed clinically and rarely requires therapy; more rare tumors are difficult to diagnose and treat. This review provides an overview of vascular tumors seen in the neonatal period and summarizes treatment options., Competing Interests: Disclosure Dr D. Adams is a consultant for Venthura and Novartis., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background and Aims: Sorafenib is a proven first-line treatment recommended for hepatocellular carcinoma (HCC) patients with portal vein invasion (PVI). However, multiple treatment modalities are used in clinical practice as a first-line option. This study is a prospective, observational, multicenter, cohort study evaluating patterns of treatment modalities and outcomes for HCC patients with PVI., Methods: The baseline characteristics, treatment modalities, and outcomes were prospectively collected for 287 newly diagnosed HCC patients with PVI between August 2015 and July 2016 from 16 sites in Korea., Results: During a median 7.8 months of follow-up (range 0.3-24.6 months), mortality was observed in 123 (42.9%) patients. Decision tree analysis classified patients into five subgroups with different outcomes. The patterns of treatment were very heterogeneous, and there was no dominant treatment modality. The most commonly used treatment modality was transarterial chemoembolization (TACE) (20.2%) followed by TACE plus external beam radiation therapy (17.8%) and sorafenib (12.5%). When stratified according to the extent of PVI, sorafenib treatment showed comparable outcomes when the PVI extent was lobal or main/bilateral, yet showed worse outcomes when the PVI extent was limited to the segmental level compared to those who received treatment other than sorafenib., Conclusions: HCC patients with PVI comprise a heterogeneous population and are treated with various treatment modalities with diverse clinical outcomes in clinical practice. Subclassification of HCC patients with PVI is required to minimize heterogeneity and should be considered for the selection of treatment modalities and future clinical trials.

Vascular anomalies are composed of tumors and malformations and with overlapping histologies, thus are often misdiagnosed or labeled with imprecise terminology. Lesions are common and usually diagnosed during infancy or childhood; the estimated prevalence is 4.5%. Vascular tumors rapidly enlarge postnatally and demonstrate endothelial proliferation. Malformations are errors in vascular development with stable endothelial turnover; they are typically named based on the primary vessel that is malformed (capillary, arterial, venous, lymphatic). This article reviews the pathologic and molecular genetic characteristics for select recently described vascular anomalies., Competing Interests: Disclosure The authors declare that they have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Vascular liver tumors in the pediatric population can present a diagnostic dilemma. The most common hepatic vascular tumors are hepatic hemangiomas; however the differential diagnosis can also include other benign lesions and malignant masses. Management is unique to the type and nature of the specific lesion. Thus, correct diagnosis and timely intervention is critical. The work up, diagnosis, and management of the different hepatic lesions are discussed in this paper., (Copyright © 2020. Published by Elsevier Inc.)

Vascular tumors are a rare subset of vascular anomalies. These are classified based on their malignant potential or local destruction potential. Classification has been historically difficult and treatment recommendations are based on case series. The purpose of this chapter is to review the presentation, pathologic and imaging characteristics. Treatment recommendations are summarized based on the current literature. Congenital and infantile hemangiomas are covered separately in a separate chapter in this issue., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose, (Copyright © 2020. Published by Elsevier Inc.)

Introduction: The diagnosis and management of vascular lesions of the neck is a challenging task that requires a multidisciplinary approach. This retrospective study assesses the single center experience of vascular tumors of the neck., Materials and Methods: Patients diagnosed with a vascular tumor and/or a mass in close proximity to the carotid artery were identified from our records over a 10-year period. The demographic characteristics, clinical features, surgical approach, and outcomes were reviewed., Results: Surgical excision of 17 vascular lesions were performed in 16 patients with a mean age of 51.56 ± 17.35 years at the time of operation. Intra- and/or postoperative clinical and histological assessment revealed unilateral glomus caroticum (N = 11), glomus vagale (N = 2), bilateral glomus caroticum (N = 1), cavernous hemangioma (N = 1), and carotid sheath tumor (N = 1). In three patients, internal carotid artery, common carotid artery and vagal nerve were sacrificed to facilitate complete tumor excision. During the follow-up period, no tumor recurrences were observed, and the morbidity and mortality were minimal., Conclusion: Preoperative evaluation concerning the size, extent, and anatomical relationships of the tumor thoroughly should be investigated. Multidisciplinary approach involving vascular surgery, otolaryngology, and radiology is preferred to treat these patients for better outcomes. Preoperative embolization in selected cases may decrease estimated blood loss and operative time.

Objective: Microvascular invasion (MVI) has been associated with a poor prognosis for hepatocellular carcinoma (HCC) patients. This study aimed to evaluate the efficacy and safety of adjuvant transarterial chemoembolization (TACE) after curative hepatectomy for HCC with MVI., Methods: An online search on Embase and Ovid MEDLINE(R) was conducted to identify the appropriate articles published prior to March 11, 2019. The primary endpoint was the overall survival (OS) of patients treated using adjuvant TACE after hepatectomy (HTAT) versus hepatectomy (HT) alone for HCC with MVI. The secondary endpoints were disease-free survival (DFS) and safety., Results: Seven studies with 1869 patients were included in this analysis. Meta-analyses demonstrated that HTAT was superior to HT in OS (Hazard Ratio [HR]: 0.67, 95%CI: 0.58-0.77, P<0.001) and DFS (HR: 0.71, 95%CI: 0.62-0.81, P<0.001) for treating HCC with MVI. Subgroup analysis revealed that for early-stage HCC, HTAT was associated with longer OS (P=0.009) and DFS (P=0.066) as compared with HT. For HCC larger than 5cm, HTAT also prolonged the DFS (P=0.008) of patients, but the difference in OS was not statistically significant (P=0.266). Adjuvant TACE commonly caused nausea and vomiting, liver dysfunction, leucopenia, pain, and fever., Conclusions: Adjuvant TACE after hepatectomy is effective and safe for patients with HCC accompanied by MVI. However, the benefit of adjuvant TACE in patients who have HCC with a diameter >5cm is not clear. Further randomized controlled studies are warranted to test these conclusions., (Copyright © 2019. Published by Elsevier Masson SAS.)

Intravascular lymphoma is a rare disease that progresses to multiple organ dysfunction caused primarily by tumor cell proliferation in small blood vessels. Few studies have investigated critical care management of intravascular lymphoma. We describe a rare case of multiple organ failure due to intravascular lymphoma with severe lactic acidosis in a patient who survived. A 64-year-old man with impaired consciousness was diagnosed as having intravascular large B-cell lymphoma by means of a random skin biopsy. The patient arrived at our hospital's intensive care unit (ICU) with impaired consciousness, respiratory failure that required mechanical ventilation, and lactic acidosis that required renal replacement therapy. Mechanical ventilation and renal replacement therapy were continued in the ICU, and his respiratory status and circulatory dynamics eventually stabilized. However, his impaired consciousness and hyperlactatemia did not improve until after the start of chemotherapy with doxorubicin, cyclophosphamide, vincristine, prednisolone, and rituximab. Although he developed tumor lysis syndrome immediately after chemotherapy, his systemic condition was gradually stabilized by continued critical care management primarily comprising renal replacement therapy. He was weaned from ventilator support after a tracheotomy and moved to the general ward. Hematopoietic malignancy with hyperlactatemia has a very poor prognosis; however, hyperlactatemia and impaired consciousness were dramatically improved in this patient by critical care management and chemotherapy.

Only a few hundred cases of intimal sarcomas of pulmonary artery (ISPA) were reported on the literature. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of ISPA. Treatment strategies rely initially on an early aggressive surgery aiming for complete surgical resection with clear margins while no clear recommendations guiding the choice for additional drug therapy or radiotherapy exist. In this article, we perform an extensive review of the literature on ISPA with details on the clinical presentation, diagnosis and management strategies. An additional goal of this paper is to make practicing oncologists aware of this rare entity with clear idea on the initial management., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020. Published by Elsevier B.V.)

Background/objectives: Congenital hemangiomas (CH) are a group of benign vascular tumors that are present at birth and exhibit variable involution during infancy. Congenital hemangiomas that do not involute are typically solitary patch or plaque-type tumors that grow proportionally with somatic growth. We report a case series of 9 patients with persistent CH, which exhibited uncommon features including segmental involvement, recurrent or severe pain, or growth via volumetric increase in size or apparent increased extent of anatomic involvement over time., Methods: Via retrospective chart review, we included patients with persistent CH and atypical presentations. Available data regarding clinical characteristics, natural history, histopathology, imaging, and genetic tests were collected., Results: Data on 9 patients were collected, including 7 noninvoluting CH and 2 partially involuting CH. Three of the 9 cases had segmental distribution, 6 had apparent growth or clinical evolution, and 4 were symptomatic with pain. One also had marked localized intravascular coagulopathy., Conclusions: Ongoing or recurrent pain and large extent of anatomic involvement can be features of CH, albeit uncommon ones, and can pose both diagnostic and management challenges. Tissue genomic studies can offer a novel tool for CH diagnosis., (© 2019 Wiley Periodicals, Inc.)

Purpose: In view of a recent meta-analysis reporting increased mortality following angioplasty with paclitaxel-coated devices in peripheral arteries, we performed a patient-level 2-year mortality analysis based on pooled original data of four randomized controlled trials (THUNDER, FEMPAC, PACIFIER and CONSEQUENT)., Methods and Results: Clinical data of four randomized controlled trial were pooled to assess 2-year mortality following paclitaxel-coated balloon (PCB) angioplasty compared to angioplasty without paclitaxel (control group). A logistic regression model was applied to identify potential predictors of mortality. At two years, 13 of 185 (7.0%) patients had died in the control group and 16/184 (8.7%) in the PBC group, p = 0.55. Kaplan-Meier analysis revealed no significant difference from all-cause death at 2 years (log rank p = 0.54). Causes of death were well balanced between the groups with no pattern or trend in favour of any specific causes in the PBC group. Logistic regression revealed that treatment groups (controls or PBC) were not a predictor of 2-year mortality. The only predictor for mortality was patient age ≥ 75 years. The delivered paclitaxel doses per patient were not significantly different in patients that died and those who did not die during the 24-month follow-up (5.300 ± 4.224 μg vs. 6.248 ± 4.629 μg, p = 0.433)., Conclusions: Based on original patient-level data of four pooled randomized controlled trials, we found no increase in 2-year mortality in patients treated with PCB compared to control patients treated with uncoated balloons. Causes of death were well balanced between PCB and control patients.

BACKGROUND Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma characterized by involvement of lymphoma cells in the lumina of small blood vessels in various organs. Although many studies have claimed that timely diagnosis and early initiation of chemotherapy are important, the literature on the successful treatment of IVLBCL in elderly patients over the age of 80 years is scarce. CASE REPORT An 82-year-old man presented with abnormal elevation of lactate dehydrogenase at 1605 IU/L and altered mental status, which manifested as forgetfulness, abnormal behavior, and worsening performance status for 2 days. There was no evidence of lymphadenopathy or skin lesions, but a computed tomography scan of the chest showed bilateral diffuse ground-glass opacities. With a primary consideration of IVLBCL, random skin biopsy and transbronchial lung biopsy were performed. Immediately after the pathologic findings on hematoxylin-eosin staining alone revealed abnormal large cells in the small vessels, chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) at half the usual dose was started; later on, immunostaining revealed CD20 expression in the abnormal lymphoid cells. Immediately after CHOP administration, his mental and physical symptoms rapidly resolved. After 8 cycles of dose-reduced CHOP with rituximab, he remained free from disease progression 2 years after the diagnosis. CONCLUSIONS We presented a case of successful early chemotherapy for IVLBCL in an elderly patient with poor performance status. Early chemotherapy with CHOP, even before obtaining a definitive diagnosis based on immunostaining, may contribute to good outcomes.

Leiomyosarcomas of vascular origin are uncommon. They arise from the smooth muscles of tunica media of major blood vessels. Most of the cases are present in women. We report one such case of external iliac vein leiomyosarcoma in a 77-year-old female and discuss the clinical, radiological, and histopathological findings and the available treatment options., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Pulmonary artery intimal sarcoma is a rare aggressive intraluminal tumor often misdiagnosed as acute or chronic pulmonary thromboembolism due to its clinical presentation and radiological findings. Thus early diagnosis is very crucial and may improve patient outcome. There is limited literature on diagnosis of pulmonary artery sarcoma by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). Herein, we report a case of mass-like lesion in the PA diagnosed on cytological material obtained by EBUS-TBNA with rapid on-site evaluation (ROSE). The aspirate showed pleomorphic malignant spindled cells arranged in loosely cohesive clusters. The intraluminal origin of PAIS was supported by radiographic findings. Subsequently, the patient received preoperative chemotherapy and underwent tumor resection with reconstruction. This report describes the cytomorphologic features of this rare intravascular tumor and demonstrates how limited cytological sample obtained from EBUS-TBNA with ROSE can be triaged efficiently for ancillary studies like immunohistochemistry and MDM2 amplification, thus expediting the management., (© 2018 Wiley Periodicals, Inc.)

Background: Patients with hepatocellular carcinoma (HCC) and microvascular invasion (mVI) have shown dismal postoperative prognosis; however, whether adjuvant transarterial chemoembolization (TACE) can improve their outcomes remains unclear., Materials and Methods: We retrospectively identified 549 eligible patients to form the crude cohort and adopted propensity score matching method to assemble another cohort of 444 patients with similar baseline characteristics. We assessed the effects of adjuvant TACE by stratified analyses and multivariate Cox analyses in two cohorts., Results: There was significant interaction between tumor size and adjuvant TACE with respect to overall survival (OS; p = .006 for interaction). In the matched cohort, patients who received adjuvant TACE showed higher rates of 5-year OS (72.4% vs. 50.9%, p = .005) and 5-year recurrence-free survival (50.5% vs. 36.4%, p = .003) in the tumor ≤5 cm subgroup, but not in the tumor >5 cm subgroup (32.3% vs. 24.9%, p = .350 and 18.8% vs. 19.7%, p = .180). The independent protective role of adjuvant TACE on OS was observed in patients with tumor ≤5 cm (adjusted odds ratio [OR] = 0.59, 95% confidence interval [CI] 0.36-0.97) but not in patients with tumor >5 cm (adjusted OR = 1.17, 95% CI 0.84-1.62). The effects of adjuvant TACE did not change materially while the analysis was performed in the crude cohort., Conclusion: For patients with HCC and mVI, adjuvant TACE was associated with improved outcomes, but not for those with tumor >5 cm, according to the current protocol., Implications for Practice: The outcomes of patients with hepatocellular carcinoma and microvascular invasion who received adjuvant transarterial chemoembolization were inconsistent in this study. According to the current protocol, adjuvant transarterial chemoembolization was associated with improved prognosis in patients with microvascular invasion, except for those with tumor >5 cm. Multivariate Cox models confirmed adjuvant transarterial chemoembolization was an independent protective factor in the tumor ≤5 cm subgroup but not in the tumor >5 cm subgroup., Competing Interests: Disclosures of potential conflicts of interest may be found at the end of this article., (© AlphaMed Press 2018.)

Angiosarcoma (AS) is a type of malignancy within sarcomas affecting soft tissue and bone. It presents a very heterogeneous distribution in the human body, and it can appear in multiple locations, from breast to liver or skin. The incidence of this type of tumor is low; however, it is aggressive and requires an early diagnosis to initiate the treatment as soon as possible. In the present article, we report the case of a male patient who was diagnosed with epithelioid AS of popliteal artery after an aneurysm popliteal surgery, as well as its treatment and evolution. This kind of tumors has controversial treatment and poor survival rates in the early years., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

A 64-year-old woman was diagnosed with diffuse large B-cell lymphoma (DLBCL) in 2013. After eight courses of R-CHOP therapy followed by local irradiation of the remaining retroperitoneal soft tissue shadow, complete response was confirmed on 18F-2-fluoro-2-deoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT). Early in 2016, patient's serum LDH and soluble IL-2 receptor levels elevated. With suspected recurrence of DLBCL, FDG-PET/CT was performed that showed no lymphadenopathy or abnormal FDG uptake. By the end of July 2016, the patient developed fever and night sweating. Intravascular large B-cell lymphoma (IVLBCL) was suspected, and the patient underwent random skin biopsies, which revealed large atypical cells infiltrating peripheral and intravascular regions of the subcutaneous adipose tissue. Cell morphology, immunostaining, and PCR analysis of the immunoglobulin heavy chain gene suggested the recurrence of DLBCL. Despite salvage chemotherapy and autologous peripheral stem cell transplantation with high-dose chemotherapy, approximately 15 months later, DLBCL recurred and involved the lungs. The patient again received chemotherapy and achieved a second remission. Because DLBCL may recur like intravascular lymphoma, the same tests used for IVLBCL diagnosis are required in cases of suspected recurrence of DLBCL based on clinical and laboratory findings.

Vascular leiomyosarcoma is a very rare soft tissue neoplasma. We are reporting a vascular leiomyosarcoma case arising from an unusual site: the radiocephalic vein. Despite a poor prognosis, after surgery and radiotherapy, the patient was alive without signs of recurrence two years later., (Copyright © 2018. Published by Elsevier Masson SAS.)

Background: Lateral nodal disease in rectal cancer remains a subject of debate and is treated differently in the East and the West. The predictive value of lateral lymph node and MRI-detected extramural vascular invasion (mrEMVI) features on oncological outcomes was assessed in this study., Methods: In this retrospective cohort study, data on patients with cT3-4 rectal cancer within 8 cm from the anal verge were considered over a 5-year period (2009-2013). Lateral lymph node size, malignant features and mrEMVI features were evaluated and related to oncological outcomes., Results: In total, 192 patients were studied, of whom 30 (15·6 per cent) underwent short-course radiotherapy and 145 (75·5 per cent) received chemoradiotherapy. A lateral lymph node short-axis size of 10 mm or more was associated with a significantly higher 5-year lateral/presacral local recurrence rate of 37 per cent, compared with 7·7 per cent in nodes smaller than 10 mm (P = 0·041). Enlarged nodes did not result in a higher 5-year rate of distant metastasis (23 per cent versus 27·7 per cent in nodes smaller than 10 mm; P = 0·563). However, mrEMVI positivity was related to more metastatic disease (5-year rate 43 versus 26·3 per cent in the mrEMVI-negative group; P = 0·014), but not with increased lateral/presacral recurrence. mrEMVI occurred in 46·6 per cent of patients with nodes smaller than 10 mm, compared with 29 per cent in patients with nodes of 10 mm or larger (P = 0·267)., Conclusion: Although lateral nodal disease is more a local problem, mrEMVI mainly predicts distant recurrence. The results of this study showed an unacceptably high local recurrence rate in patients with a short axis of 10 mm or more, despite neoadjuvant (chemo)radiotherapy., (© 2018 BJS Society Ltd Published by John Wiley & Sons Ltd.)

Study Design: A retrospective study of 46 patients with malignant epithelioid vascular tumors (MEVT) in the spine was performed., Objective: The purpose of this study was to illustrate the clinic characteristics of MEVT in the spine and to discuss prognostic factors by survival analysis., Summary of Background Data: MEVT in the spine is relatively uncommon, and there is little published information regarding this subject. Therefore, prognostic factors of this disease are still controversial., Method: The univariate and multivariate analysis of various clinic characteristics were performed to identify the independent factor that affects prognosis for patients with MEVT in the spine who underwent surgical treatment in Changzheng Hospital Orthopedics Oncological Center (CHOOC) between 2005 and 2015. Disease-free survival (DFS) and overall survival (OS) were estimated by Kaplan-Meier method to identify potential prognostic factors. Factors with P values of 0.1 or less were subjected to multivariate analysis by Cox regression analysis. P values of 0.05 or less was considered statistically significant., Result: A total of 46 patients with MEVT in the spine were included in the study. The mean follow-up period was 42.6 months (range, 5-143 mo). Recurrence was detected in 22 patients after initial surgery in our center, whereas death in 18 patients. The univariate analysis suggested that age (<30 yr/≥30 yr), metastasis, preoperative Frankel score, postoperative Frankel score, malignant grade and resection mode were potential prognostic factors for DFS and OS. However, as they were submitted to multivariate analyses, only malignant grade and resection mode were independent prognostic factors., Conclusion: Total en bloc spondylectomy can significantly improve DFS for MEVT in the spine and low-grade malignancy was favorable factor for DFS. Total spondylectomy and low-grade malignancy considerably improve OS for patients with MEVT in the spine., Level of Evidence: 4.

Background:  Vascular anomalies are a diagnostic and therapeutic challenge. They require dedicated interdisciplinary management. Optimal patient care relies on integral medical evaluation and a classification system established by experts in the field, to provide a better understanding of these complex vascular entities., Method:  A dedicated classification system according to the International Society for the Study of Vascular Anomalies (ISSVA) and the German Interdisciplinary Society of Vascular Anomalies (DiGGefA) is presented. The vast spectrum of diagnostic modalities, ranging from ultrasound with color Doppler, conventional X-ray, CT with 4 D imaging and MRI as well as catheter angiography for appropriate assessment is discussed., Results:  Congenital vascular anomalies are comprised of vascular tumors, based on endothelial cell proliferation and vascular malformations with underlying mesenchymal and angiogenetic disorder. Vascular tumors tend to regress with patient's age, vascular malformations increase in size and are subdivided into capillary, venous, lymphatic, arterio-venous and combined malformations, depending on their dominant vasculature. According to their appearance, venous malformations are the most common representative of vascular anomalies (70 %), followed by lymphatic malformations (12 %), arterio-venous malformations (8 %), combined malformation syndromes (6 %) and capillary malformations (4 %)., Conclusion:  The aim is to provide an overview of the current classification system and diagnostic characterization of vascular anomalies in order to facilitate interdisciplinary management of vascular anomalies., Key Points:   · Vascular anomalies are comprised of vascular tumors and vascular malformations, both considered to be rare diseases.. · Appropriate treatment depends on correct classification and diagnosis of vascular anomalies, which is based on established national and international classification systems, recommendations and guidelines.. · In the classification, diagnosis and treatment of congenital vascular anomalies, radiology plays an integral part in patient management.., Citation Format: · Sadick M, Müller-Wille R, Wildgruber M et al. Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies. Fortschr Röntgenstr 2018; 190: 825 - 835., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor affecting infants and young children. Although benign, it can be associated with an aggressive locally growing tumor and/or a life-threatening Kasabach-Merritt phenomenon (KMP). To date, only reviews of limited cases have been performed. We, therefore, conducted a comprehensive literature search to collect relevant data and make recommendations for future treatment trials., Methods: Review of the available literature between 1993 and 2017 revealed a total of 105 publications involving 215 patients of less than 21 years of age. To this, we added 12 from our department and 4 from the Cooperative Weichteilsarkomstudie database., Results: We found that KMP was present in 79% of the infants, in 47% of the 1-5-year olds, in 43% of the 6-12-year olds, and in 10% of the 13-21-year-old patients. KMP was present in nearly all (94%) patients with retroperitoneal tumors and in all patients with extra-regional tumors. The median size of a KHE without KMP was 12 cm 2 as compared to 49 cm 2 when associated with a KMP. With complete (not further classifiable if R0 or R1) resection, all patients were cured. If inoperable, response regarding KMP/regression of tumor size was seen in 29/28% with steroid-, 47/39% with vincristine-, 44/43% with interferon alpha-, 65/61% with anti-platelet agents-, and in 97/100% with sirolimus-containing therapies., Conclusions: Patients with progressive KHE should undergo resection whenever it is considered a safe option. If inoperable, sirolimus should be the first choice for treating KMP and reducing tumor size.

Angiosarcoma is an exceedingly rare and aggressive neoplasm. Owing to early metastasis even in low-grade tumors, it is associated with poor survival. We report a late development of an angiosarcoma in a saphenous vein graft after a femoral artery to above-knee popliteal artery bypass performed for disabling claudication. Chronic inflammation, lymphatic disruption, and thrombosis of the graft may have contributed to the malignant transformation, similar to cases described as occurring after arteriovenous fistulae creation. This case illustrates that angiosarcoma may be a rare, late complication of autogenous vascular bypass., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Risk of recurrent disease for men with clinical stage 1 high-risk nonseminomatous germ cell testicular cancer (CS1 NSGCT) with lymphovascular invasion (LVI) after orchiectomy is 50% and current treatment options (surveillance [S], retroperitoneal lymph node dissection [RPLND], or 1 cycle of BEP [BEP ×1]) are associated with a 99% disease specific survival, therefore practice patterns vary. We performed a decision analysis using updated data of long-term complications for men with CS1 NSGCT with LVI to quantify and assess relative treatment values., Methods: Decision analysis included previously defined utilities (via standard gamble) for posttreatment states of living from 0 (death from disease) to 1 (alive in perfect health) and updated morbidity probabilities. We quantified the values of S, RPLND, and BEP ×1 via the rollback method. Sensitivity analyses including a range of orchiectomy cure rates and utility values were performed., Results: Estimated probabilities favoring treatment with RPLND (0.97) or BEP ×1 (0.97) were equivalent and superior to surveillance (0.88). Sensitivity analysis of orchiectomy cure rates (50%-100%) failed to find a cure rate that favored S over BEP ×1 or RPLND. Varying utility values for cure after S from 0.92 (previously defined utility) to 1 (perfect health), failed to find a viable utility state favoring S over BEP ×1 or RPLND. An orchiectomy cure rate of ≥82% would be required for S to equal treatment of either type., Conclusions: We demonstrate that for surveillance to be superior to treatment with BEP ×1 or RPLND, the orchiectomy cure rate must be at least 82%, which is not expected in a patient population with high-risk CS1 NSGCT., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Rationale: Gallbladder cancer (GBC) is a highly fatal malignancy. Due to its invasiveness and delayed diagnosis, many GBC patients are diagnosed with synchronous liver and hepatoduodenal ligament involvement. In our case, we report a gallbladder cancer with portal vein thrombus., Patient Concerns: A 60-year-old woman presented with persistent upper abdominal dull pain for 2 months., Diagnoses: Ultrasound examination showed gallbladder carcinoma invading liver segment IV, and a tumor thrombus in the left and right main portal trunk. Ultrasonography and contrast-enhanced magnetic resonance imaging (MRI) showed gallbladder carcinoma with invasion of adjacent liver, and tumor thrombus in the right branch of the portal vein and intrahepatic bile duct. Abdominal computed tomography angiography (CTA) revealed no hepatic artery invasion., Interventions: We made a decision to perform extended right lobectomy. Twenty-six days later, the patient underwent intravenous infusion port implantation for S-1 plus oxaliplatin (SOX) therapy., Outcomes: After treatment, the patient has been doing very well and no recurrence has been found for 5 months., Lessons: The patient with gallbladder cancer and tumor thrombus in the portal vein described in this report provides a reminder for surgeons of the importance of early diagnosis, and adequate surgical and adjuvant treatment. Multi-disciplinary treatment is significantly beneficial for the overall survival of patients with advanced GBC.

Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent vascular tumor. Extensive cases are treated with amputation as chemotherapy seems to be ineffective. Recently, promising results were published using mammalian target of rapamycin (mTOR) inhibitors in tumors of vascular origin. Here, we present a case of a child with advanced PMH relapsing after surgery and chemotherapy. Sirolimus achieved significant clinical improvement and stabilization of the lesions without any remarkable toxicity. This case contributes to the growing evidence regarding the efficacy of mTOR inhibitors, such as sirolimus, in multifocal PMH., (© 2017 Wiley Periodicals, Inc.)

Vascular neoplasms of the head and neck present with a wide spectrum of signs and symptoms. Diagnosis requires a high index of suspicion and is usually made after tumors are large enough to be visually apparent or cause symptoms. This article discusses the most common acquired benign and malignant vascular tumors, with an emphasis on their evaluation and treatment., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Congenital and acquired vascular anomalies are common in childhood. In addition to predominantly harmless vascular skin alterations there are others which must be immediately treated due to the potentially threatening complications. As examples three anomalies and the typical complications are presented. Hemangiomas in infancy can make urgent treatment necessary because they can be complicated by obstruction or painful ulceration. Kaposiform hemangioendothelioma can lead to a life-threatening consumption coagulopathy. Klippel-Trenaunay syndrome can be complicated by thrombosis, localized coagulopathy, and superinfections.

Tumors of the hand comprise a vast array of lesions involving skin, soft tissue and bone. The majority of tumors in the hand are benign. Malignant tumors, although rare, do occur and frequently have unique characteristics in this specific anatomic location. Careful staging, histological diagnosis and treatment are essential to optimize clinical outcome. However, straightforward most of the time, hand tumor management does have pitfalls; caution is advised, as a missed or delayed diagnosis or an improperly executed biopsy may have devastating consequences. This article reviews the clinical spectrum of the most common benign and malignant bone and soft tissue tumors of the hand and discusses the clinicopathological findings, imaging features and current concepts in treatment for these tumors.

Objective: Vascular anomalies in the neonatal period are a diagnostic challenge for the lack of evident signs, symptoms and follow-up, and the convenience of restricting aggressive diagnostic tests. The aim of this work is to review the characteristics of neonatal cases presented to our Vascular Anomalies Unit in the last 5 years., Materials and Methods: All cases of suspected vascular anomaly presented to our unit before 1 month of age between 2010 and 2015 were reviewed, diagnostic tests and treatments carried out with chronology were analyzed. Presumptive diagnosis and final diagnosis (when available) were compared., Results: Fifteen vascular tumors were found, 2 with visceral involvement: 6 infantile hemangiomas (IH), 3 NICH, 4 RICH, 1 tufted hemangioma, 1 unspecified liver vascular tumor, 3 venous malformations (2 equivocal MRI and a hyperkeratotic venous malformation), 4 lymphatic malformations, 3 of them macrocystic, and 2 vascular lesions that were diagnosed of fibrosarcoma and sclerema neonatorum and they were not vascular anomalies. Only 3 patients with macrocystic lymphatic malformations had prenatal diagnosis., Conclusion: Accurate diagnosis of vascular anomalies during the first month of life is difficult, even with MRI. Only in a few cases early treatment is needed, so it is worth taking time to follow-up. Different types of treatment (observation, propranolol, biopsy, laser, embolization, and resection) will depend on the condition to be treated. A continuous observation can avoid unnecessary procedures and risks.

Superficial vascular anomalies are a heterogeneous group of diseases, developed from various types of vessels and differentiated by their clinical presentation, progression, and prognosis. Their classification was first published by Mulliken and Glowacki in 1982, then endorsed by the International society of the study of vascular anomalies (ISSVA) in 1996 and updated in 2014. It separates vascular anomalies into two categories: vascular tumors characterized by cellular proliferation and vascular anomalies that are structural anomalies of blood vessels. Infantile hemangiomas (IHs) are the most frequent childhood vascular tumor. In most cases, IHs are small, involute progressively with or without a few sequelae, and do not require any treatment. However, in particular localizations or in complicated hemangiomas, treatment is essential to limit tumor growth and accelerate their involution. Propranolol is now the first-line treatment. Its efficacy and safety have been clearly demonstrated., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)