[Infantile hemangioma : Clinical manifestation, treatment, and differential diagnoses]. / Infantile Hämangiome : Manifestationsformen, Therapie, Differenzialdiagnosen.

With an incidence of approximately 4% infantile hemangiomas are the most common vascular tumors in children and show characteristic growth dynamics. In order to avoid erroneous treatment, they need to be differentiated from other vascular tumors (granuloma pyogenicum and kaposiform hemangioendothelioma) and vascular malformations. Of all infantile hemangiomas 85% are uncomplicated and undergo spontaneous resolution starting towards the end of the first year of life. First-line treatment for complicated infantile hemangiomas (15%), i.e. those with imminent obstruction (eyes and nose), ulceration or permanent disfigurement, is oral propranolol (2 mg/kg BW and day for at least 6 months).

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

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

Concomitant subcutaneous intravascular lymphoma lesions in a patient with cardiac diffuse large B-cell lymphoma: is intravascular lymphoma a distinct clinical entity?

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.

[Benign aggressive vascular anomalies in children]. / Anomalies vasculaires bénignes agressives de l'enfant et de l'adolescent.

Superficial vascular anomalies constitute a large group of malformative and tumoral conditions developed from all types of vessels. Vascular tumors are the result of cellular hyperplasia, whereas vascular malformations (VMs) are constituted of dysplastic vessels. The classification from International Society for the Study of Vascular Anomalies (ISSVA) is based on this pathogenic difference. The most common vascular tumor is infantile hemangioma, which treatment, when necessary, is propranolol. Congenital hemangiomas and tumors that might be complicated with Kasabach-Merritt phenomenon, i.e. deep thrombocytopenia, are much rarer. Management of Kasabach-Merritt phenomenon is now largely based on sirolimus. Low-flow VMs include capillary, venous and lymphatic malformations; arteriovenous malformations are high-flow malformations. These different types of VMs might be combined. Currently, there is an increasing work in delineating the different entities based on molecular findings. Treatment of VMs depends on the impairment linked to them, and is decided case by case, in pluridisciplinary consultations. Interventional treatments, especially surgery and sclerotherapy, are usually partially efficient, and management of patients with VMs increasingly involves medical drugs. First-line treatment of coagulation disorders associated with venous malformations is based on low molecular weight heparin; sirolimus seems efficient in hemorrhagic complications refractory to usual treatment. Sirolimus is about to become the standard treatment in painful inflammatory manifestations of mixed and/or complicated lymphatic malformations.

Bone marrow-derived cells are recruited by the melanoma tumor with endothelial cells contributing to tumor vasculature

Purpose. Tumor expansion is dependent on neovascularization, a process that requires sustained new vessel formation. Although the critical role of angiogenesis by endothelial sprouting in this process, controversy still prevails on whether angiogenesis involving bone marrow-derived endothelial cells, does contribute to this process. This study aims to evaluate the recruitment of bone marrow-derived cells by the melanoma tumor, including endothelial cells, and if they contribute to angiogenesis. Methods. A chimeric mouse model of GFP bone marrow was used to induce melanoma tumors derived from murine B16-F10 cell line. These tumors were evaluated for the presence of myeloid cells (CD11b), T lymphocytes (CD3, CD4 and CD8) and endothelial cells (VEGFR2 and CD31) derived from bone marrow. Results. Mice transplanted with GFP+ cells showed significant bone marrow chimerism (90.9 ± 0.87 %) when compared to the GFP transgenic mice (90.66 ± 2.1 %, p = 0.83) demonstrating successful engraftment of donor bone marrow stem/progenitor cells. Analysis of the murine melanoma tumor showed the presence of donor cells in the tumors (3.5 ± 1.7 %) and interestingly, these cells represent endothelial cells (CD31+ cells; 11.5 ± 6.85 %) and myeloid cells (CD11b+ cells; 80 ± 21 %), but also tumor-infiltrating lymphocytes (CD8+ T cells, 13.31 ± 0.2 %; CD4+ T-cells, 2.1 ± 1.2 %). Examination of the tumor endothelium by confocal microscopy suggests the presence of donor CD31+/GFP+ cells in the wall of some blood vessels. Conclusion. This study demonstrates that bone marrow-derived cells are recruited by the murine melanoma tumor, with myeloid cells and CD4 and CD8 T lymphocytes migrating as antitumor immune response, and endothelial cells participating of the tumor blood vessels formation (AU)

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Hemangiopericitoma pélvico. Una localización infrecuente de tumor vascular / Pelvic hemangiopericytoma. An unusual location of a vascular tumor

Introducción: el hemangiopericitoma es una neoplasia infrecuente de estirpe vascular. Presentamos un caso clínico de interés dada su escasa frecuencia, tamaño y localización. Caso clínico: se trata de una mujer de 63 años que consultó por dolor abdominal. Se realizaron TAC, RMN abdominal y arteriografía que mostraban una gran masa pélvica que ocupaba el espacio de Douglas y desplazaba útero, vejiga y sigma y presentaba vascularización dependiente de la arteria mesentérica interior y ambas arterias hipogástricas. Se embolizó selectivamente la vascularización propia de la neoplasia y seguidamente se resecó la masa, sin presentarse complicaciones ni intra ni postoperatorias. La anatomía patológica confirmó el diagnóstico de hemangiopericitoma. La paciente sigue controles en consultas externas sin signos de recidiva hasta la fecha. Discusión: el hemangiopericitoma procede de las células del pericito por lo que puede presentarse en cualquier localización. La localización pélvica es excepcional. Puede presentarse como dolor abdominal inespecífico, dar síntomas de compresión de órganos vecinos y, ocasionalmente, asociarse a síndromes paraneoplásicos. El diagnóstico de sospecha es mediante TAC y angiografía aunque la confirmación es histológica tras analizar la pieza de resección. El tratamiento de elección es quirúrgico, precediéndose en algunos casos de embolización preoperatoria de la vascularización de la masa. No hay acuerdo sobre la quimio/radioterapia como tratamiento primario del hemangiopericitoma, aunque se ha descrito la radioterapia adyuvante para mejorar del control local y disminuir las recurrencias. El pronóstico es bueno si se consigue una resección completa, con supervivencias a 5 y 10 años entre el 70 y el 80% según las series (AU)

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Vascular Lesions.

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.].

Actualización en ecografía de las anomalías vasculares / Update on the Use of Ultrasound in Vascular Anomalies

El avance en el conocimiento de la biología y la terapéutica de las anomalías vasculares (AV) han hecho que sean un motivo frecuente de consulta en las consultas de dermatología en la actualidad, y que se hayan creado unidades multidisciplinares para el abordaje de los pacientes con AV complejas. Aunque el diagnóstico de la mayoría de las AV es clínico, a menudo su estudio completo requiere pruebas complementarias de imagen para determinar su naturaleza, extensión y pronóstico. La primera prueba de imagen que se solicita por su rapidez e inocuidad es la ecografía (US). En esta revisión se busca resumir y actualizar los conceptos clave en la ecografía de las AV para su mejor comprensión para los clínicos o especialistas en imagen que tratan a estos pacientes

Advances in our understanding of the biology and therapy of vascular anomalies have made this condition a common reason for consulting a dermatologist. In addition, multidisciplinary units have been created to manage patients with complex vascular anomalies. Although most vascular anomalies are diagnosed based on clinical findings, a thorough evaluation often requires additional imaging tests to determine the nature, extension, and prognosis of these lesions. Because it is fast and noninvasive, ultrasound is usually the first imaging test ordered. In the present review, we provide a state-of-the-art synthesis of key concepts in the ultrasound examination of vascular anomalies so that they are more accessible to clinicians and medical imaging specialists involved in the management of these lesions

Hemangioendotelioma hepático fetal. Reporte de un caso / Fetal hepatic haemangioendothelioma. A case report

Introducción: el hemangioendotelioma hepático es una neoplasia benigna rara de origen vascular que se puede asociar con complicaciones que amenazan la vida. Caso clínico: se presenta el caso de un feto de 30 semanas. El ecograma obstétrico demostró una gran masa abdomino-pélvica con áreas quísticas y calcificaciones puntuales y la evaluación Doppler demostró que la lesión era altamente vascularizada. Discusión: el neonato murió a las 16 horas del nacimiento. El examen microscópico demostró la presencia dentro de la lesión de canales vasculares organizados, tapizados de endotelio, sin signos de atipia celular, compatible con el diagnóstico de hemangioendotelioma hepático (AU)

Background: Hepatic hemangioendothelioma is a rare benign neoplasm of vascular origin that can be associated with life-threatening complications. Case report: We present the case of a 30-week foetus. Obstetric ultrasound showed a large abdomino-pelvic mass with cystic areas and calcifications. Doppler ultrasound demonstrated that the tumour was highly vascularized. Discussion: The newborn died 16 hours after delivery. Microscopic evaluation of the tumour showed the presence of organized vascular channels inside the mass, covered by endothelium, without signs of atypical changes, compatible with a diagnosis of hepatic hemangioendothelioma (AU)

Rare cause of severe anemia due to pyogenic granuloma in the jejunum.

BACKGROUND: Pyogenic granuloma (PG) is a polypoid lobular capillary hemangioma rarely observed in the gastrointestinal tract. Only a few cases in the small bowel have been described in the literature. CASE PRESENTATION: A 58-year-old man had been suffering from general fatigue and severe anemia. Esophagogastroduodenoscopy and colonoscopy did not reveal any significant bleeding. Abdominal computer tomography revealed a hypervascular tumor in the small intestine. Oral double-balloon endoscopy (DBE) detected a polypoid lesion (2 cm in diameter) in the jejunum. We performed laparoscopic-assisted partial resection of the jejunum. The histological features of the tumor were consistent with PG. The patient's anemia gradually improved without the need for oral iron after surgery. CONCLUSION: In this case report, we present a case of pyogenic granuloma in in the jejunum that was detected by DBE.

Diagnosis and surgical treatment of cavernous sinus angioleiomyoma: a report of four cases.

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.

Hemorrhagic collision metastasis in a cerebral arteriovenous malformation.

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.

Atypical teratoid rhabdoid tumor in the cavernous sinus of a toddler presenting with oculomotor nerve palsy.

INTRODUCTION: Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant, and aggressive tumor of infancy. Although the prognosis of ATRT has been extremely poor, recently, the first prospective study for ATRT demonstrated improvement of prognosis. On the other hands, oculomotor nerve palsy is rare in children and the most frequent etiology is congenital. To our knowledge, only a few ATRT cases presenting with oculomotor nerve palsy have been reported, but ATRT originating from the cavernous sinus (CS) has not yet been reported. CASE REPORT: An 18-month-old girl with right oculomotor nerve palsy was admitted, and a small mass in the right CS was detected with brain MRI. Although she received steroid pulse therapy and antimicrobial therapy, the mass continued to enlarge. One month after admission, the mass was partially resected and diagnosed as ATRT. Multimodal therapy including anthracycline-based chemotherapy, intrathecal therapy, and cranial irradiation was performed. Twenty-nine months after resection, she was alive without tumor relapse, but the oculomotor nerve palsy persisted. CONCLUSION: This is the first reported case of ATRT located in the CS presenting with oculomotor nerve palsy. This case was successfully treated with partial removal of the tumor, a new chemotherapy regimen for ATRT and cranial X-ray irradiation.

Placental mesenchymal dysplasia and fetal hematologic disorder.

Placental mesenchymal dysplasia (PMD) is a rare, recently recognized placental vascular anomaly. About 20% of patients with this placental anomaly have Beckwith-Wiedemann syndrome. We report a case of a phenotypically normal neonate with anemia and thrombocytopenia associated with PMD. Histologic examination of the placenta showed findings consistent with PMD, including chorangioma. The patient's hematologic abnormalities resolved during the week following birth. Normal phenotypic fetuses with PMD seem to exhibit hematologic disorders at birth in some cases, especially in the presence of chorangioma.

Bases patogénicas de la epileptogénesis en los cavernomas cerebrales / Pathogenetic bases of epileptogenesis in cerebral cavernomas

Introducción. Los cavernomas cerebrales son un tipo de malformación arteriovenosa que cursa clínicamente con crisis epilépticas, déficits neurológicos focales y hemorragias intraparenquimatosas. Se cree que las situaciones de hipoxia, neovascularización y algunas endoproteasas están implicadas en la fisiopatología de las crisis. Nuestro estudio pretende valorar esta posible relación, analizando con métodos inmunohistoquímicos la presencia de la subunidad 1α del factor inducible por hipoxia (HIF-1α) y la metaloproteasa de matriz 9 (MMP-9). Pacientes y métodos. Se seleccionaron 17 muestras consecutivas con diagnóstico anatomopatológico de cavernoma en un período de nueve años sobre las que se realizó una tinción inmunohistoquímica para HIF-1α y MMP-9, valorando la relación con las crisis epilépticas según el grado de captación del anticuerpo de los diferentes tejidos encontrados alrededor de las muestras de cavernoma. Resultados. En aquellos pacientes que presentaron crisis, se observó tinción inmunohistoquímica para HIF-1α en el 31% de las muestras en el endotelio vascular, el 17% en el tejido fibroso y el 34% en el tejido inflamatorio. También se observó tinción positiva para MMP-9 en el 86% del endotelio vascular, el 100% del tejido fibroso y el 43% del tejido cerebral. Analizando la muestra, se observa una tendencia positiva en presencia de crisis epilépticas en los pacientes que muestran la presencia de HIF-1α y MMP-9 en el endotelio vascular, tejido fibroso y tejido cerebral, no siendo así para el tejido inflamatorio. Conclusión. La expresión de HIF-1α y MMP-9, evaluada por métodos inmunohistoquímicos, se relaciona positivamente con la presencia de complicaciones de tipo epiléptico (AU)

Introduction. Brain cavernoma are a type of arteriovenous malformation that clinically presenting seizures, neurological deficit or bleeding. Hypoxia, neoangiogenesis and metalloproteasas seems to be involved in seizures physiopathology. Our study aims to assess this potential relation by immunohistochemical methods, analyzing hypoxia inducible factor (HIF-1alpha) and metalloproteasa (MMP-9) in tissue surrounding cavernoma. Patients and methods. We selected 17 consecutive cases anatomopathologically diagnosed as cavernoma during 9 years. Immunohistochemical staining was performed for HIF-1alpha and MMP-9. We evaluated the relation between seizures and the scale of uptake of different tissues surrounding cavernoma. Results. Cases with seizures had HIF-1alpha positive uptake in vascular endothelium in 31%, 17% in fibrous tissue and 34% in inflammatory tissue. Besides, it also shows MMP-9 positive uptake in vascular endothelium in 86%, 100% in fibrous tissue and 43% of brain tissue. Statistical analysis by chi-square and odds ratio shows a positive trend towards seizures and the presence of HIF-1alpha and MMP-9 in vascular tissue, fibrous tissue and brain tissue, but no for inflammatory tissue. Conclusion. HIF-1alpha and MMP-9, valued by immunohistochemical methods, are related to complications as seizures (AU)

Cerebral vascular hamartoma with thrombosis in a dog.

A cerebral vascular hamartoma was identified in the frontal lobe, striatum and thalamus of the right side of the brain of a male, 7-year-old Shih Tzu. Histologically, the lesion consisted of thin-walled vessels, which showed various sizes and occasionally contained fibrin thrombi. These vascular walls were composed of a single layer of fibromuscular tissue lined by flat endothelium with various amount of collagen, but devoid of large coat of smooth muscles and elastic tissue. Immunohistochemically, the lining endothelial cells were positive for von Willebrand Factor antibody. Neuropil between the vessels was stained with Klüver-Barrera stain, and positive for synaptophysin and GFAP antibodies. Based on these findings, the lesion was diagnosed as vascular hamartoma, which might resemble venous malformation in humans.

Special considerations in vascular anomalies: hematologic management.

Proper care of the patient with a vascular anomaly requires the expertise of multiple specialists. Because of the need for an interdisciplinary approach, several vascular anomalies centers have now been developed across the world. A hematologist/oncologist provides clinical acumen in establishing a correct diagnosis and guiding the medical management of these patients. These patients can have complicated coagulopathies and need medical therapy. This article emphasizes the hematologic complications and management of these patients.

Sclerosing angiomatoid nodular transformation of the spleen in an adolescent with chronic abdominal pain.

Sclerosing angiomatoid nodular transformation (SANT) is a relatively new, benign neoplasm arising within the red pulp of the spleen. The lesion is often identified incidentally on imaging, and the diagnosis is confirmed on pathologic assessment of the resected spleen. Although there have been several reports of SANT in the adult population, data on this lesion in the pediatric population are exceedingly rare. We present a case of SANT in an adolescent male with chronic abdominal pain and discuss the management issues that arise in treating this condition in the pediatric population.