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Histological examination of liver biopsies and resection specimens remains the gold standard to establish a definitive diagnosis of liver lesions. While hepatocellular carcinoma remains the most commonly encountered liver lesion on mass-directed biopsies, surgical pathologists must be aware of other entities that may pose diagnostic challenges, as an accurate diagnosis is key for patient management. Mesenchymal tumours of the liver are relatively uncommon, therefore many pathologists are unfamiliar with these tumours. While the clinical presentation and radiological features of these lesions often overlap, careful attention to histological clues can assist in weeding out various congeners to arrive at the most accurate diagnosis. An additional challenge when diagnosing mesenchymal tumours is the specimen type, as mass-directed core biopsies are limited and have become standard clinical practice. Besides careful attention to histological features, radiological findings and clinical history, immunohistochemical analysis and molecular studies have become of immense diagnostic value. In this review, we discuss several common and rare mesenchymal hepatic lesions as defined in the current World Health Organization (WHO) classification and most up-to-date literature. We also discuss immunohistochemistry panels and relevant molecular findings that may assist in rendering an accurate diagnosis when encountering these lesions in daily practice., (Copyright © 2021 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

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., (Copyright © 2020 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

In this article, the authors have reviewed all the recent news regarding how the discovery of some novel and recurrent molecular and genetic changes has modified the classification of some entities and have addressed to the description of new variants of vascular tumors. And even more important, the authors also reviewed on how these findings, in addition to gain insight into the tumoral biology, portend significant clinical consequences not only regarding to their diagnosis but also to their management and prognosis because some of these mutations are potential targets for treatment. The authors have also highlighted immunohistochemical markers can help us as a surrogate marker of those molecular alterations.

Purpose: To describe patients with intermediate uveitis complicated by vasoproliferative tumors (VPTs)., Methods: Data were collected at seven Uveitis/Ocular Oncology centers on demographic, ophthalmic findings at baseline and at follow-up, and on imaging. The therapeutic intervention, final visual acuity, and duration of follow-up were recorded., Results: A total of 36 eyes from 34 patients (12 men, 22 women; mean age 35.3 ± 14.2 years) were included in this study. Visual acuity at presentation ranged from 20/40 to counting fingers. At the time of VPT diagnosis, intermediate uveitis was active in all eyes. The mean VPT thickness was 3.06 ± 0.86 mm. Local treatment to the VPT was provide in 22 eyes (61.1%) and no local treatment to the VPT in 14 eyes (38.9%). After the VPT was detected, systemic or local treatment for the inflammation was initiated and on follow-up FAs 94.4% of the eyes showed resolution of the vascular leakage. During follow-up of 35.8 months, the 22 VPTs treated locally had a reduction in the tumor thickness to 1.25 mm, whereas the 14 VPTs untreated remained stable (final mean tumor thickness 2.65 mm)., Conclusion: The presence of active intermediate uveitis accompanied by VPTs suggests the need for an aggressive uveitis treatment.

Aims: Congenital haemangiomas (CHs) can be subdivided into different subtypes [rapidly involuting CHs (RICHs), non-involuting CHs (NICHs), and partially involuting CHs (PICHs)]. During the first few days of life, RICHs may be associated with transient but sometimes marked thrombocytopenia. We sought to assess the histological aspects and clinicopathological correlations of the three subtypes., Methods and Results: We assessed the histopathological features of 10 RICHs, 25 NICHs, and 20 PICHs, described the patients' long-term clinical outcomes, and assessed clinicopathological correlations. All CHs were located in the dermis and hypodermis, and comprised both capillary lobules (with three distinct histopathological patterns) and extralobular large vessels. Most of the extralobular vessels were abnormal veins and abnormal lymphatic vessels. We did not observe significant correlations between the CH subtype, the histopathological pattern, and the time of the histopathological assessment. Interestingly, unexpected intralobular expression of podoplanin was found in neonatal biopsies of five RICHs and PICHs. Four of these five patients had concomitant thrombocytopenia. The podoplanin staining intensity decreased over time as the thrombocytopenia resolved and the tumour shrank., Conclusion: The histopathological features were similar in all three subtypes of CH, and were related to the time since disease onset; we consider that RICH, PICH and NICH form a single entity and differ only in their involuting potential. Along with the transient expression of intralobular podoplanin observed in some specimens from the newborn, the lobular architecture might lead to misdiagnosis of tufted haemangioma or kaposiform haemangioendothelioma., (© 2020 John Wiley & Sons Ltd.)

This case report describes a primary cardiac tumor, classified as venous malformation, diagnosed in an asymptomatic child. The tumor was located in the left atrium near the mitral valve without affecting the mitral valve's functioning. Complete resection of the lesion was performed because of the risk of systemic embolism. The lesion consisted of fibrous tissue with multiple venous vascular channels. The patient did not have similar lesions in other locations. Vascular primary cardiac tumors are extremely rare. Hemangiomas and lymphangiomas have been described previously, but to our knowledge, this is the first primary cardiac tumor identified as a venous malformation., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Vascular anomalies are common in the upper extremities, but there continues to be a relative paucity of information about them in publications dealing with surgery in the hands and upper limbs. The wide spectrum of pathology and an inconsistent use of terminology make vascular anomalies susceptible to incorrect diagnosis and as a result, to misdirected management. This article aims to provide an update on vascular anomalies relevant to the upper limbs, focusing on significant advances in pathogenesis and genetics, classification systems, diagnosis and treatment.

We describe a patient who presented with large cardiac diffuse large B-cell lymphoma (DLBCL) and adrenal masses. The patient also had subcutaneous intravascular lymphoma lesions which were detected by random skin biopsy. Although ambiguous, minimal extravascular location of lymphoma cells is permitted for the diagnosis of intravascular large B-cell lymphoma (IVLBCL) in the WHO definition, a number of rare cases have been reported as having concomitant tumours in other organs, such as the adrenal gland, brain, and penis. We assume that IVLBCL might be a peculiar feature of DLBCL characterised by preferential localisation of lymphoma cells within the capillaries rather than a distinct disease entity of DLBCL.

Background: Epithelioid hemangioendothelioma is an exceedingly rare sarcoma often occurring as an indolent angiocentric vascular tumor at various anatomic sites. Few reports have evaluated large case series of epithelioid hemangioendothelioma., Methods: We conducted a retrospective analysis of the clinical data of 42 consecutive patients with epithelioid hemangioendothelioma who were pathologically diagnosed between 1990 and 2014 at 13 Japanese tertiary hospitals. We analyzed their clinical characteristics, tumor features and prognostic factors., Results: The study included 22 men and 20 women, with a median age of 54 (range, 18-78) years. Pain was the most common symptom, occurring in 15 (68%) of the 22 symptomatic patients. The median maximum tumor diameter was 4.0 (range, 1.0-12.8) cm. The most commonly involved organs were the liver (81%), lungs (57%), and bones (12%). The overall survival rates were 79.5% at 1 year and 72.0% at 5 years. Substantially better survival was observed in asymptomatic patients than in symptomatic patients (P = 0.03), and better survival was also ovserved in patients with Ki-67 index ≤10% than in those with Ki-67 index > 10% (P = 0.04). By multivariate analysis, tumor size > 3.0 cm was associated with decreased survival (P = 0.049, hazard ratio 13.33)., Conclusions: This study showed the clinical characteristics of Japanese patients with epithelioid hemangioendothelioma. Tumor size > 3.0 cm is an independent indicator of a poor prognosis in epithelioid hemangioendothelioma. The presence of symptoms at the time of diagnosis and high Ki-67 index implied poor survival.

Hepatic small vessel neoplasm (HSVN) is a recently described vascular neoplasm of the adult liver. The neoplastic cells are positive for markers of vascular lineage (CD31, CD34, FLI-1). The distinctive morphology and infiltrative borders separate HSVN from benign vascular tumors such as cavernous hemangioma, while lack of atypical morphologic features, low to absent mitotic activity and low proliferation index distinguish it from malignant vascular tumors such as epithelioid hemangioendothelioma and angiosarcoma. Due to its infiltrative nature and lack of adequate follow-up information, the benign versus low-grade nature of this tumor is currently uncertain. We present a patient with resected HSVN involving all but the right posterior section of the liver, making this case the largest reported in the current literature., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Vascular tumors are benign neoplasms, which result from proliferating endothelial cells. These lesions present during infancy or childhood, may affect any location, and exhibit postnatal growth. Local complications include bleeding, tissue destruction, and pain whereas systemic sequelae include thrombocytopenia, congestive heart failure, and death. Vascular tumors should be differentiated from vascular malformations, which present at birth, have a quiescent endothelium, and grow in proportion to the child. Together, vascular tumors and malformations comprise the field of vascular anomalies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Vascular lesions (VLs) of the breast present a diagnostic challenge on breast core biopsy (BCBx). We report on 27 VLs presenting on BCBx. The mean patient age was 60 years, and mean size was 7.5 mm (range, 1.6 to 16 mm). Presentation included palpable mass in 6 (22%), incidental in 6 (22%), and an imaging abnormality in 15 (56%) cases. Imaging impression included hematoma (24%), lymph node (10%), fat necrosis (10%), tortuous vessel (5%), and not provided in 52%. The lesions were classified on the basis of BCBx or BCBx and excision (available in 16 pts) as follows: 1 low-grade angiosarcoma, 8 angiolipomas, 6 capillary hemangiomas, 4 cavernous hemangiomas, 2 hemangiomas (not otherwise specified), 1 papillary endothelial hyperplasia, and 5 perilobular hemangiomas. The angiosarcoma was 9 mm, detected incidentally by magnetic resonance imaging, and showed dissection of stromal collagen, infiltration of glands, high cellularity, moderate cytologic atypia, scant mitotic activity, and Ki-67 reactivity of 10%. Among the 26 benign VLs, worrisome histologic features were noted in 14 on BCBx, including anastomosing vascular channels in 9, moderate cytologic atypia in 4, high cellularity in 2, Ki-67>10% in 2, mitotic activity in 1, and infiltration of glands in 1. Of the 12 VLs without worrisome features, the lesion extended to edge of core in 8, precluding complete evaluation. BCBx of VLs presents diagnostic challenges due to overlapping clinicopathologic and radiologic features with low-grade angiosarcoma. If completeness of removal is documented on BCBx, and cytoarchitectural changes are not worrisome, follow-up could be considered rather than excision. However, only 4 of these cases fulfilled those criteria.

Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Knowledge of the systemic associations that can occur with different vascular anomalies may reduce life-threatening complications, such as coagulopathy, bleeding, cardiac compromise, and neurologic sequelae. This review provides a synopsis of the epidemiology, pathogenesis, presentation, workup, and treatment of several well-recognized multifocal vascular tumors and malformations., (©2016 Frontline Medical Communications.)

This review provides a clinically-oriented summary of the most commonly encountered overgrowth syndromes associated with vascular malformations. This manuscript will outline morphologic features, clinical evaluation and management of this complex group of patients. Recent genetic advances have aided in classification and help to explain overlapping clinical features in many cases., (©2016 Frontline Medical Communications.)

Myopericytoma are uncommon, slow-growing benign perivascular neoplasms that show hemangiopericytoma-like vascular pattern. We report a 52-year-male patient with a painless palpable nodule in the left thumb for the past 9 months. The mass, on the thenar aspect, was 15x12mm in size. X-ray revealed a soft tissue swelling with no bony association. The excised nodule was a non-capsulated, well-circumscribed vascular neoplasm composed of proliferating spindle to ovoid bland cells with eosinophilic cytoplasm. A concentric perivascular arrangement of the cells was seen interspersed by thin-walled, branching, staghorn blood vessels. Nuclear atypia, mitotic figures and necrosis were not observed. Immunohistochemistry revealed diffuse positivity of the tumour cells for smooth muscle actin while staining negative for CD34 and desmin - features suggestive of origin from the perivascular myoid cell. Morphological features of myopericytoma are shared with hemangiopericytoma, glomus tumors, myofibroma and solitary fibrous tumour which form the important differential diagnoses. It is a relatively newly described disease entity recognized by the World Health Organisation classification of tumours.

Vascular lesions in childhood are comprised of vascular tumors and vascular malformations. Vascular tumors encompass neoplasms of the vascular system, of which infantile hemangiomas (IHs) are the most common. Vascular malformations, on the other hand, consist of lesions due to anomalous development of the vascular system, including the capillary, venous, arterial, and lymphatic systems. Capillary malformations represent the most frequent type of vascular malformation. IHs and vascular malformations tend to follow relatively predictable growth patterns in that IHs grow then involute during early childhood, whereas vascular malformations tend to exhibit little change. Both vascular tumors and vascular malformations can demonstrate a wide range of severity and potential associated complications necessitating specialist intervention when appropriate. Evaluation and treatment of the most common types of vascular lesions are discussed in this article. [Pediatr Ann. 2016;45(8):e299-e305.]., (Copyright 2016, SLACK Incorporated.)

The aim of the present study was to accurately evaluate the association of Sox2 expression with the survival of patients with digestive tract cancers. Relevant literatures were identified by comprehensively searching databases including the Pubmed, Embase, CBMdisc, and Wanfang (up to October 2014). A meta-analysis was performed to clarify the association between Sox2 expression and overall survival or clinicopathological parameters of patients with digestive tract cancers (esophageal, gastric, and colorectal cancers). The results showed a significant association between high Sox2 expression and poor overall survival in patients with digestive tract carcinomas (HR=1.55, 95% CI=1.04-2.31), especially for patients with esophageal cancer (HR=2.04, 95%CI=1.30-3.22), colorectal cancer (HR=1.40, 95% CI=1.04-1.89), and digestive tract adenocarcinoma (HR=1.80, 95% CI=1.12-2.89), for Europeans (HR=1.98, 95% CI=1.44-2.71) or patients who did not receive neoadjuvant treatment (HR=1.73, 95% CI=1.10-2.72). Furthermore, Sox2 over-expression was highly correlated with vascular invasion (OR=1.86, 95% CI=1.25-2.77) and poor differentiation (OR=1.88, 95% CI=1.14-3.08), especially in esophageal and colorectal cancers. In conclusion, Sox2 expression may serve as a novel prognostic factor for patients with digestive tract cancers. Over-expression of Sox2 that is correlated with vascular invasion and poor differentiation suggests poor outcomes of patients with digestive tract cancers.

Purpose: Vascular lesions of the orbit, although not malignant, can cause morbidity because of their location near critical structures in the orbit. For the same reason, they can be challenging to remove surgically. Anti-vascular endothelial growth factor (VEGF) drugs are increasingly being used to treat diseases with prominent angiogenesis. Our study aimed to determine to what extent VEGF receptors and their subtypes are expressed on selected vascular lesions of the orbit., Design: Retrospective case series of all orbital vascular lesions removed by one of the authors (JAG) at the Mayo Clinic., Participants: A total of 52 patients who underwent removal of vascular orbital lesions., Methods: The pathology specimens from the patients were retrieved, their pathologic diagnosis was confirmed, demographic and clinical information were gathered, and sections from vascular tumors were stained with vascular endothelial growth factor receptor (VEGFR), vascular endothelial growth factor receptor type 1 (VEGFR1), vascular endothelial growth factor receptor type 2 (VEGFR2), and vascular endothelial growth factor receptor type 3 (VEGFR3)., Main Outcome Measures: The existence and pattern of staining with VEGF and its subtypes on these lesions., Results: There were 28 specimens of venous malformations, 4 capillary hemangiomas, 7 lymphatic malformations, and 6 lymphaticovenous malformations. All samples stained with VEGF, 55% stained with VEGFR1, 98% stained with VEGFR2, and 96% stained with VEGFR3. Most (94%) of the VEGFR2 staining was diffuse., Conclusions: Most orbital vascular lesions express VEGF receptors, which may suggest a future target for nonsurgical treatment., (Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

The differential diagnosis of splenic masses is broad and often hinges on the enhancement characteristics of the lesions. Most radiologists are familiar with the differential diagnosis of hypovascular lesions such as fungal infections, sarcoidosis/granulomatous disease, infarctions, and cysts. However, to our knowledge, there is no review article that presents the specific multimodality imaging features of vascular splenic lesions as a group. Vascular splenic lesions may be considered those that enhance more or similarly to the background splenic parenchyma. In this review, we illustrate the spectrum of imaging features of both benign and malignant vascular splenic lesions. The benign lesions include hemangiomas, hamartomas, and sclerosing angiomatoid nodular transformation of the spleen. The malignant lesions are divided into primary and metastatic lesions, ranging from lymphoma, angiosarcoma to pleomorphic sarcoma. While lymphoma and metastases may commonly present as hypoenhancing lesions relative to the background parenchyma, we are addressing them here as their appearance can be varied and hence deserve consideration. Littoral Cell angiomas are discussed separately, as they were originally considered benign, but recent studies have shown that they can have malignant potential.

Intracranial intravascular papillary endothelial hyperplasia (IPEH) is very rare, and to our knowledge long-term follow-up results have not been previously published. An 11-year-old boy presented with a 6-month history of progressive visual impairment in the right eye. Magnetic resonance imaging revealed a well-enhanced, large parasellar mass involving the cavernous sinus, right frontal skull base, and ethmoid and sphenoid sinuses. Frontotemporal craniotomy and subtotal resection were performed, and the diagnosis of IPEH was confirmed. The mass increased in size during the following 3 months. A second operation was performed via frontotemporal craniotomy combined with a transsphenoidal approach, and gross-total resection of the tumor was achieved. Adjuvant radiotherapy (5040 cGy) and chemotherapy with interferon were administered. The patient's visual symptoms improved, and there was no recurrence during a 13-year follow-up period. The results of this case indicate that intracranial IPEH can recur with subtotal resection; however, optimal resection with multimodal adjuvant treatment can control the disease for many years, if not permanently.

Objective: The aims of the study were to retrospectively evaluate the imaging characteristics of cavernous sinus angioleiomyoma and explore appropriate surgical approaches., Methods: A total of four cases admitted to our hospital for repeated episodes of headache were enrolled. The common clinical manifestations included decreased vision, diplopia, blepharoptosis and facial numbness. The first three patients were misdiagnosed with cavernous sinus hemangioma, meningioma and chordoma, respectively. The fourth case was suspected to be angioleiomyoma. Imaging examination showed an oval-shaped tumor occupying the cavernous sinus. The tumor was hypointense on T1-weighted magnetic resonance imaging and extremely hyperintense on T2-weighted scan, which was similar to the cerebrospinal fluid, and was progressively enhanced from one side after gadolinium injection, pushing the internal carotid artery toward the inner side., Results: Surgical resection was performed via an epidural approach for these four cases. Clinical symptoms were improved after surgery and no recurrence was observed during follow-up visits (average, 47.5 months)., Conclusions: The initial symptom of cavernous sinus angioleiomyoma was repeated headache. The tumor seems extremely hyperintense on a T2-weighted image and expansive growth is pushing away the internal carotid artery rather than encasing it. It was progressively enhanced from one side after gadolinium injection. Surgical treatment based on an epidural approach had an excellent outcome in tumor resection and nerve protection., (© The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Background: Vascular malformations can mimic malignant tumors, and the coexistence of both types of lesions can limit and interfere with treatment. A consecutive series of vascular malformations that were treated and evaluated in a single institute and cases involving vascular anomalies combined with malignancies or malignancies that were treated as vascular anomalies were analyzed., Objective: Absolute ethanol is used in the treatment and management of vascular malformations and is sometimes administered before a definitive diagnosis has been obtained, despite the fact that some vascular lesions are subsequently revealed to be malignant tumors. This study discusses such cases., Materials and Methods: From January 2006 to August 2012, 139 patients were treated for vascular malformations at Nagasaki University Hospital and were followed up for a minimum of 1 year., Results: Four malignant lesions coexisted with or were misdiagnosed as vascular malformations, including a malignant peripheral nerve sheath tumor located in the chest, a hemangiopericytoma of the palate, an adenoid cystic carcinoma of the cheek, and a squamous cell carcinoma of the cheek. Thus, malignant lesions were detected in 2.88% of cases in which vascular malformations were preoperatively diagnosed., Conclusion: When treating vascular malformations, it is advisable to be aware of the possibility of malignancy.

A 26-year-old patient with recurrent choriocarcinoma of the testis presented with headache and progressive left homonymous hemianopsia. On initial MRI a grade 4 arteriovenous malformation (AVM) was identified in the right occipital lobe, which was further characterized by catheter angiography. Continued worsening of the headache in the following days prompted a follow-up MRI, which revealed a new T2 hypointense nodule and adjacent vasogenic edema in the periphery of the AVM. A follow-up MRI showed a marked increase in the size of the nodule with intrinsic enhancement and worsening perilesional edema. Based on the imaging evolution, the nodule was diagnosed as a metastasis and the patient was started on chemotherapy and radiotherapy. One week after the MRI he developed a sudden hemorrhage within the mass requiring decompression craniectomy and resection of both AVM and tumor. The histopathology of the resected mass confirmed the diagnosis of choriocarcinoma metastasis to the AVM., (2014 BMJ Publishing Group Ltd.)

Vascular anomalies can be subdivided into vascular tumours and vascular malformations (VMs). While most VMs are present at birth and do not exhibit significant postnatal growth, vascular tumours are characterized by their dynamics of growth and (sometimes) spontaneous regression. This review focuses on benign vascular tumours other than infantile haemangiomas (IHs), namely pyogenic granuloma, eruptive pseudoangiomatosis, glomangioma, rapidly involuting and noninvoluting congenital haemangioma, verrucous haemangioma and spindle cell haemangioma. While some of them bear clinical resemblance to IH, they can be separated by age of appearance, growth characteristics and/or negative staining for glucose transporter 1. Separation of these tumours from IH is necessary because their outcome and therapeutic options are different. Semimalignant and malignant vascular tumours will be addressed in a separate review., (© 2013 British Association of Dermatologists.)

Aim: To evaluate the efficacy of intravitreal bevacizumab in the treatment of retinal vasoproliferative tumours (VPT)., Materials and Methods: Six eyes of 6 patients with VPT who received intravitreal bevacizumab were retrospectively reviewed. All patients received between one and three injections of intravitreal bevacizumab depending upon response to treatment. Best-corrected visual acuity (BCVA), tumour size, and presence of co-pathology or sequelae were noted pre- and postoperatively and then analysed. Subsequent retreatments were performed in patients with recurrent or persistent VPT according to the ophthalmologist's discretion. Retreatments included photodynamic therapy with verteporfin, ruthenium-106 plaque brachytherapy, or endoresection of tumour., Results: The mean follow-up duration was 33.3 months (range 10-66 months). At baseline, the mean logMAR BCVA was 1.45 (Snellen equivalent of 6/165); range 0.10-1.90 (6/8-CF). Following bevacizumab treatment the mean logMAR BCVA was 0.98 (Snellen equivalent of 6/57); range 0.5-1.9 (Snellen equivalent of 6/19 to CF). Therefore, there was no statistically significant change in visual acuity. The mean tumour thickness reduced from 2.4 to 2.1 mm following treatment with bevacizumab. However, this did not reach the statistical significance of P<0.05. Despite the visual improvement following bevacizumab therapy, five out of six patients had recurrence of tumour activity during the follow-up period and required further intervention in order to achieve sustained regression., Conclusions: Intravitreal bevacizumab appeared to result in temporary reduction of tumour thickness in 3 out of 6 VPT patients. However, neither the reduction in tumour thickness nor the change in visual acuity were statistically significant and intravitreal bevacizumab monotherapy had limited effectiveness in causing long-term regression of the lesions. Additional therapy was indicated in five out of six patients to establish long-term regression. The efficacy of bevacizumab as an adjunct is as yet undetermined and further studies are needed. Presently, we recommend other treatment modalities in the long-term management of VPTs.

Purpose: To evaluate epiretinal membrane (ERM) response after cryotherapy for retinal vasoproliferative tumors (VPTs)., Method: Retrospective interventional case series., Results: Of 16 eyes with VPT and ERM, the tumor was classified as primary in 12 (75%) eyes or secondary in 4 (25%) eyes. The median patient age was 44 years (mean, 43 years; range, 9-70 years). The tumor was located in extramacular zone (n = 16, 100%) and inferotemporal quadrant (n = 12, 75%). The mean tumor base was 6 mm, and thickness was 3 mm. The ERM involved the macula in 12 (75%) eyes and extramacular zone in 4 (25%) eyes, with best-corrected visual acuity of 20/40 or better in 6 (38%) eyes. Associated features included cystoid macular edema (n = 8, 50%), subretinal fluid (n = 10, 63%), vitreous cells (n = 9, 56%), and vitreous hemorrhage (n = 7, 44%). Single-session cryotherapy (double freeze-thaw) to the VPT was performed in each case. Over mean follow-up of 68 months (median, 54 months; range, 8-252 months), tumor regression was documented in 16 (100%) cases, with ERM release in 10 (63%) cases. After ERM release, the foveal anatomy was normal in 12 (75%) eyes. Final visual acuity improved (n = 5, 31%), remained stable (n = 9, 56%), or worsened (n = 2, 13%). Posttreatment best-corrected visual acuity was 20/40 or better in 10 (63%) eyes., Conclusion: Cryotherapy is remarkably effective for VPT of 6 mm or less in basal dimension. After cryotherapy, VPT-related ERM spontaneously released in 63% of the cases, without the need for surgical intervention.

A 26-year-old male suffering from sudden right lower abdominal pain and lumbago was referred to our hospital. Enhanced computed tomography demonstrated bilateral kidneys and spleen infarctions, and a large tumour was found occupying the aortic arch and thoracic descending aorta. We suspected that these infarctions were due to tumour embolization. The aortic arch and thoracic descending aorta were resected with the tumour and then reconstructed using the L-incision technique. A microscopic examination revealed the presence of an intimal sarcoma. The patient was treated with adjuvant chemotherapy and showed a good postoperative course. Neither recurrence nor metastasis has been observed during the 3 years since the operation.

Angiosarcoma (AS) is a rare malignant vascular tumor, whereas epithelioid hemangioendothelioma (EHE) is a vascular tumor of low-grade malignancy. CD30 is a member of the tumor necrosis factor receptor superfamily, member 8 (TNFRSF8). Although the expression of CD30 is most commonly associated with lymphoid malignancies or germ cell tumors, occasional ASs have been reported as CD30 positive. However, there are limited data to evaluate its role definitively in malignant vascular tumors. In this study, we evaluated 91 ASs, 30 EHEs from various sites, and 25 Kaposi sarcomas. Overall, CD30 was expressed in 31/91 cases (34%) of AS, in 7/30 cases (30%) of EHE, but in none of the Kaposi sarcomas. CD30 was expressed in a membranous staining pattern and positivity in tumor cells varied from focal to diffuse. The positive ASs included vasoformative more differentiated tumors and also solid, undifferentiated, lymphoma-like examples, one of which was classified as lymphoma before the era of immunohistochemistry. The CD30 expression was seen in >50% of tumor cells in a majority of ASs but only in 7% of EHEs. None of the 55 ASs studied were immunohistochemically positive for TIA-1 or Granzyme B, antigens used as more specific markers for anaplastic large-cell lymphoma. Compared with AS, normal vascular endothelia of capillaries and muscular vessels showed variable positivity. Among hemangiomas, cavernous and spindle cell hemangiomas showed most frequent endothelial CD30 positivity, whereas in most other hemangiomas, CD30 positivity was scant. In conclusion, CD30 expression occurs in a significant subset of ASs and EHEs and needs to be included in the differential diagnosis with other CD30-positive malignancies to avoid a diagnostic pitfall. It remains to be determined whether patients with strongly CD30-positive ASs could be candidates for targeted therapy using the recently introduced CD30 antibody drug conjugates.

Podoplanin is a type I integral membrane glycoprotein that, because it is expressed in lymphatic endothelium, but not in vascular blood vessel endothelial cells, is commonly used in the identification of lymphatic endothelial differentiation in vascular endothelial neoplasms and lymphatic invasion by tumor. Because podoplanin is also expressed in mesothelial cells and fetal gonocytes, it has proved to be a useful marker for assisting in the differential diagnosis of mesotheliomas and germ cell tumors, particularly seminomas/dysgerminomas. Podoplanin expression has also been reported in a wide variety of other neoplasms, including hemangioblastomas, meningiomas, cartilaginous tumors, and follicular dendritic cell neoplasms. This article reviews the information that is currently available on the application of podoplanin immunostaining in diagnostic pathology.

The incidence rate of pulmonary artery sarcoma is very low, but its prognosis is extremely poor. In this case report, after various initial diagnoses at the early stage, pulmonary artery sarcoma was confirmed by surgery. 1 year later, the tumor recurred. After chemotherapy, the patient showed improvement of the subjective complaint of tightness in the chest, and radiological lesion decreased in size. The survival time was extended by 2.5 years. This is the first case report of pulmonary artery sarcoma with such chemo-sensitivity.

Objective: The purpose of this article is to review new terminology to diagnose, classify, and refer patients with vascular anomalies for additional imaging, intervention, and treatment., Conclusion: In recent decades, much has been learned regarding the histopathology, cause, and treatment of vascular anomalies. As information has been gleaned, a new classification system has emerged that divides vascular anomalies into neoplasms and malformations. Its utility is based on accurate initial diagnosis that correlates consistently with clinical presentation, disease course, and treatment.

Many dermatologic conditions may be present on a newborn infant's upper extremity that can evoke concern for parents and/or primary caregivers. Although the pediatrician typically remains the first care provider, often these children are referred to specialists to diagnose and treat these lesions. Hand surgeons should be familiar with different infantile skin lesions on an upper extremity. Some lesions are best observed, whereas others require treatment with nonoperative measures, lasers, or surgical interventions. A 2-part series is presented to aid the hand surgeon in becoming familiar with these lesions. This part 1 article focuses on vascular neoplasms and malformations. Particular attention is paid to the multiple types of hemangiomas and hemangioendotheliomas, telangiectasias, angiokeratomas, as well as capillary, venous, and lymphatic malformations. Diagnostic tips and clinical photographs are provided to help differentiate among these lesions. In addition, the recommended treatment for each is discussed., (Copyright © 2013 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Benign conjunctival tumors include congenital, melanocytic, epithelial and stromal lesions. Clinical observation often fails to make the diagnosis, and complete excisional biopsy must be performed as soon as growth is suspected. Histology remains the best way to exclude malignancy or lesions with a risk of transformation, because malignant lesions carry a poor prognosis and require specific therapy and follow-up. Onco-ophthalmologists must have a perfect knowledge of benign conjunctival tumors, since during adulthood, nevi can be confused with melanomas, and epithelial or stromal lesions with carcinomas. For children and teenagers, surgery is only recommended when growth or functional problems are observed., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

We present the case of a rare vulva tumour, in a 33 years Cameroonian old woman and managed in Obstetrics and Gynecology Unit of Yaoundé Central Hospital in Cameroon. It was a painless pedunculated vulva tumour which developed over a period of six months. This gigantic rapidly growing tumour, was treated with simple surgical resection. After surgical resection, histology confirmed an angioneurofibroma hamartoma. There has been no recurrence and presently the patient is symptom-free.

Venous thromboembolism remains one of the important causes of pulmonary hypertension in children. The causes of venous thromboembolism are varied and include antiphospholipid antibody syndrome (APS). Catastrophic APS (CAPS) is a severe variant of APS characterised by disseminated thrombosis and microangiopathy resulting in multiorgan failure. CAPS can occur independently (primary APS) but mainly occurs in association with systemic lupus erythematosus including the paediatric population. We report a young girl with primary CAPS who presented with pulmonary hypertension and an inferior vena cava mass masquerading as a tumour.

In 1982, vascular anomalies were classified as either vascular tumors or vascular malformations. Hemangiomas were identified as benign tumors that undergo a phase of active growth characterized by endothelial proliferation and hypercellularity, followed by gradual tumor regression over the first decade. Vascular malformations were described as structural congenital anomalies derived from capillaries, veins, lymphatic vessels, arteries, or a combination of these. Unlike vascular tumors, vascular malformations were shown to have normal levels of endothelial turnover and to grow proportionately with the child. This article describes the most common types of vascular anomalies and available treatment modalities., (Copyright © 2013 Elsevier Inc. All rights reserved.)

The most common intraocular vascular tumours are choroidal haemangiomas, vasoproliferative tumours, and retinal haemangioblastomas. Rarer conditions include cavernous retinal angioma and arteriovenous malformations. Options for ablating the tumour include photodynamic therapy, argon laser photocoagulation, trans-scleral diathermy, cryotherapy, anti-angiogenic agents, plaque radiotherapy, and proton beam radiotherapy. Secondary effects are common and include retinal exudates, macular oedema, epiretinal membranes, retinal fibrosis, as well as serous and tractional retinal detachment, which are treated using standard methods (ie, intravitreal anti-angiogenic agents or steroids as well as vitreoretinal procedures, such as epiretinal membrane peeling and release of retinal traction). The detection, diagnosis, and monitoring of vascular tumours and their complications have improved considerably thanks to advances in imaging. These include spectral domain and enhanced depth imaging optical coherence tomography (SD-OCT and EDI-OCT, respectively), wide-angle photography and angiography as well as wide-angle fundus autofluorescence. Such novel imaging has provided new diagnostic clues and has profoundly influenced therapeutic strategies so that vascular tumours and secondary effects are now treated concurrently instead of sequentially, enhancing any opportunities for conserving vision and the eye. In this review, we describe how SD-OCT, EDI-OCT, autofluorescence, wide-angle photography and wide-angle angiography have facilitated the evaluation of eyes with the more common vascular tumours, that is, choroidal haemangioma, retinal vasoproliferative tumours, and retinal haemangioblastoma.