The embryo-placental CD15-positive "vasculogenic zones" as a source of propranolol-sensitive pediatric vascular tumors.

OBJECTIVE: Propranolol-induced involution is a unique biological feature of some pediatric vascular tumors, for instance infantile hemangioma (IH), cerebral cavernoma or chorioangioma. Currently, the cellular origin of these distinct tumors is unclear. In this study, we tested the hypothesis that propranolol-responsive vascular tumors are derived from common vessel-forming CD15 + progenitor cells which occur in early gestation. The aim of this study was to identify the tumor-relevant CD15 + progenitors at the early stages of embryo-placental development. MATERIALS AND METHODS: Human embryo-placental units of 4-8 weeks gestation and pediatric vascular tumors were tested for expression of the tumor-relevant markers CD15, CD31 and CD34. RESULTS: Placental vessel-forming progenitors were characterized by immunostaining for CD15, CD31, and CD34. In embryonic tissue, a discontinuous CD15+/CD31+/CD34 + progenitors was detected in immature vessels of the skin, neural tube, spinal and cerebral meninges. Similarly, vessels in IH and chorioangioma exhibited a co-expression of CD15, CD31, and CD34. In contrast, the majority of embryonic vessels presented a CD31+/CD34+, but CD15-negative immunophenotypic pattern. DISCUSSION: Our results suggest the existence of a CD15+ "vasculogenic zones" in the embryo-placental unit as well as in IH and chorioangioma. A site-specific correlation between normal embryo-placental and tumoral vessel-forming CD15 + progenitors was demonstrated. CONCLUSION: Hence, site- and stage-specific CD15 + progenitors of vascular wall could be considered as propronalol-sensitive targets and source of pre- and postnatal vascular tumors. We propose, that the CD15+ "vasculogenic zones" are a site-specific reserve of multi-lineage progenitors that could be recruited in pre- and postnatal emergency situations.

Malformación venosa intraósea primaria en hueso cigomático: actualización en criterios diagnósticos / Primary intraosseous venous malformation of the zygoma: update in diagnostic criteria

Las anomalías vasculares incluyen dos entidades bien diferenciadas aunque frecuentemente confundidas en la literatura científica: los hemangiomas y las malformaciones vasculares. La localización primaria intraósea cigomática es muy infrecuente. Además, la mayoría de los casos publicados han sido categorizados de «hemangioma» de forma poco rigurosa. Los autores describen un caso de malformación venosa intraósea cigomática y discuten las características clínicas, histológicas e inmunohistoquímicas distintivas de las anomalías vasculares que conducen a un adecuado diagnóstico y un tratamiento eficaz (AU)

Vascular anomalies include two well-differentiated conditions that are nevertheless often confused in the scientific literature: haemangiomas and vascular malformations. Primary intraosseous involvement of the zygoma is particularly rare. Moreover, most reported cases of zygomatic involvement have been categorised as “haemangioma” without appropriate diagnostic criteria. The authors describe a case of intraosseous venous malformation of the zygoma and discuss the specific clinical, histological and immunohistochemical criteria of vascular anomalies that lead to a correct diagnosis and subsequent effective treatment (AU)

Oro-facial tumours and tumour-like lesions in Greek children and adolescents: an 11-year retrospective study.

OBJECTIVE: To determine the epidemiology, histological diagnosis and treatment outcome of oro-facial tumours and tumour-like lesions in Greek children and adolescents. MATERIAL AND METHODS: The medical records of patients with oro-facial lesions who presented at the Department of Oral and Maxillofacial Surgery at "A. & P. Kyriakou" Children's Hospital from 2000 to 2010 were reviewed. Data was analyzed in relation to age, gender, location, histology, treatment choice and outcome. RESULTS: Two hundred and eleven oro-facial lesions were identified. Age ranged from 14 days to 15 years (mean 8 years); the male-to-female ratio was 1.09: 1; 90.05% of the lesions were benign and 9.95% malignant. Vascular anomalies were the most common benign lesion (22.1%) and rhabdomyosarcoma was the most prevalent malignancy (28.57%). One hundred and ten lesions (52.1%) involved soft tissue, most commonly the tongue and 96 cases (45.5%), involved hard tissue, most frequently the mandible; 5 lesions (2.4%) involved both hard and soft tissue. Surgery was performed under general anaesthesia in 198 cases (93.84%). Some malignant lesions were treated with chemotherapy and/or radiotherapy. CONCLUSIONS: Oro-facial tumours and tumour-like lesions are not uncommon in the Greek paediatric population; although most frequently benign, these may cause considerable morbidity. As such early diagnosis and treatment are imperative.

Vascular tumors of bone: imaging findings.

PURPOSE: To identify radiological features of malignant vascular tumors of bone, which can be used to avoid erroneously diagnosing metastases based on radiological multifocality, and histological epitheloid phenotype. MATERIALS AND METHODS: From the databases of the Bologna & Netherlands Committee on Bone Tumors, 63 patients with a histological diagnosis of malignant vascular tumor of bone were retrieved. Epidemiological and imaging characteristics were recorded on a case record form. RESULTS: In 63 patients, 185 lesions were detected by radiographs (61 patients) and/or CT (30 patients) and/or MRI (19 patients). Multifocality was observed in 25 patients (40%), in these patients most lesions were located in the femur. Typically lesions were well-defined, osteolytic, had a geographically pattern of destruction and were also located in the femur. Most lesions showed cortical destruction (118 lesions). No periosteal reaction was seen in most cases (121 lesions). In 13 of 39 patients (33%) tumor extension was more advanced and/or (additional) lesions (29 lesions; 17%) were visible on MRI and CT. In 20 cases (51%) cortex destruction was better shown on CT or MRI. In six patients (15%) periosteal reaction was only seen on MRI or CT and not on radiographs. In 16 (41%) cases soft tissue extension was only seen on MRI or CT, and not on radiographs. Extensive reactive changes on T2-weighted images were seen in 11 patients (58%). CONCLUSION: When single, or regional multifocal osteolytic, well-marginated lesions with cortical destruction are seen, in the femur, and with marked reactive soft tissue changes on MRI, a diagnosis of malignant vascular tumor should trigger the use of additional immunohistochemistry to confirm the vascular nature of the tumor. CLINICAL RELEVANCE STATEMENT: Because of epithelioid phenotype at histology, radiological signs are key in entertaining a diagnosis of malignant vascular tumor of bone which should trigger the use of appropriate immunohistochemical stainings.

[Children's tumors of the hand]. / Tumeurs de la main chez l'enfant.

Soft-tissue and osseous tumors of the hand in children differ considerably from those of adults, not only in frequency but also in terms of anatomic distribution, histologic type and prognosis. Malignant tumors are rare in children, the most common being rhabdomyosarcoma for soft-tissue tumors, and osteosarcoma and Ewing sarcoma for osseous tumors. Hemangioma is the most common soft-tissue tumor in infancy and childhood. Other vascular abnormalities are capillary malformation, venous malformation. Many other benign soft-tissue and cutaneous tumors can be seen (ganglion cyst, naevus, fibroma...). The surgeon must know the signs, evolution and the treatment of these tumors.

Familial blood vessel tumors and subsequent cancers.

BACKGROUND: Population-based data on the familial risk for vascular tumors are largely lacking. Such data are important for clinical counseling and cancer genetics. METHODS: We used the Swedish Family-Cancer Database to calculate standardized incidence ratios for specific subtypes of vascular tumors in offspring using parents as probands. In addition, risks for second cancers were analyzed. RESULTS: Offspring hemangioblastoma in the nervous system was associated with parental kidney cancer and nervous system hemangioblastoma and hemangioma. Offspring nervous system hemangiopericytoma was associated with parental pituitary adenomas. Offspring angiosarcoma in the trunk and extremities was associated with maternal breast cancer. Second Kaposi's sarcoma, non-Hodgkin's lymphoma, Hodgkin's disease and myeloma were increased following primary skin Kaposi's sarcoma. Kidney and endocrine gland tumors and nervous system hemangioblastomas and hemangiomas were in excess following primary nervous system hemangioblastoma and hemangioma. CONCLUSIONS: Our data showed that familial clustering of nervous system hemangioblastoma and hemangioma and the risks of subsequent cancers were primarily related to von-Hippel-Lindau disease. As a novel association, offspring nervous system hemangiopericytomas were in excess when parents were diagnosed with pituitary adenoma. Similarly, offspring angiosarcoma is associated with maternal breast cancer. Immunodeficiency may explain the excess of lymphoproliferative diseases after skin Kaposi's sarcoma.

Sarcoma de Kaposi clásico / Classical Kaposi´s sarcoma

El Sarcoma de Kaposi es un tumor vascular relativamennte infrecuente que puede afectar piel, mucosas y órganos internos. Se distinguen diferentes formas clínicas: clásica, endémica, epidémica y iatrogénica (secundario a tratamiento inmunosupresor), que poseen raíces epidemiológicas bien definidas y comparten características etiológicas, clínicas e histológicas. De todas ellas, la más conocida en la actualidad es la forma epidémica, dada su particular e histórica relación con el SIDA y quizá, por ello, es interesante recordar su variedad clásica, que por afectar de forma característica a varones mayores de 60 años en el área mediterránea describimos a continuación (AU)