Hermangioma intraóseo: reto diagnóstico. Presentación de un caso y revisión de la literatura / Intraosseous hemangioma: a diagnostic challenge. Report of a case and review of the literature

Los hemangiomas son neoplasias benignas de origen endotelial, formadoras de vasos. Los hemangiomas intraóseos son condiciones raras que comprenden del 0.5 a 1% de todos los tumores intraóseos. Su localización más común es la columna vertebral y los huesos del cráneo. Aquéllos que se presentan en los maxilares son tumores muy raros. Dos tercios de éstos se localizan en la mandíbula, con una proporción de mujer a hombre de 2:1. El objetivo de este artículo es reportar un caso signifi cativo de un hemangioma central de grandes dimensiones de la rama de la mandíbula del lado derecho, el cual fuetratado exitosamente con resección quirúrgica y ligadura de vasos nutricionales. Caso clínico: Paciente de 14 años que presenta un área radiolúcida unilocular en la rama mandibular del lado derecho. Clínicamente se observa aumento de la cara difuso, que causa asimetría facialasintomática de tres años de evolución. Conclusiones: La importancia del hemangioma intraóseo de los maxilares radica en su proximidad con los dientes, lo que representa un alto riesgo de sangrado debido a cualquier traumatismo por intento de extracción. El diagnóstico de hemangioma intraóseo, debido a su baja incidencia e inusual manera de presentación, es un reto diagnóstico...

Metastatic neuroblastoma mimicking infantile hemangioma.

Many lesions can masquerade as deep infantile hemangioma, the most common tumor of infancy. We describe an infant with infantile hemangioma and concomitant metastatic subcutaneous neuroblastoma mimicking deep hemangioma. The patient presented at 8 months of age with 3 superficial infantile hemangiomas as well as 3 subcutaneous masses. History, physical examination, and ultrasonography of the deep lesions were consistent with subcutaneous hemangioma. New masses appeared at 10 months of age that prompted biopsy; histopathology demonstrated metastatic neuroblastoma. Deviation from the predictable clinical features of a deep infantile hemangioma should prompt consideration for other causes of a subcutaneous mass in infancy, including metastatic neuroblastoma.

Head-facial hemangiomas studied with scanning electron microscopy.

INTRODUCTION: Hemangiomas of the head or face are a frequent vascular pathology, consisting in an embryonic dysplasia that involves the cranial-facial vascular network. Hemangiomas show clinical, morphological, developmental, and structural changes during their course. METHODS: Morphological, structural, ultrastructural, and clinical characteristics of head-facial hemangiomas were studied in 28 patients admitted in our hospital. Nineteen of these patients underwent surgery for the removal of the hemangiomas, whereas 9 patients were not operated on. All the removed tissues were transferred in our laboratories for the morphological staining. Light microscopy, transmission electron microscopy, and scanning electron microscopy techniques were used for the observation of all microanatomical details. All patients were studied for a clinical diagnosis, and many were subjected to surgical therapy. RESULTS: The morphological results revealed numerous microanatomical characteristics of the hemangiomatous vessels. The observation by light microscopy shows the afferent and the efferent vessels for every microhemangioma. All the layers of the arterial wall are uneven. The lumen of the arteriole is entirely used by a blood clot. The observation by transmission electron microscopy shows that it was impossible to see the limits of the different layers (endothelium, medial layer, and adventitia) in the whole wall of the vessels. Moreover, both the muscular and elastic components are disarranged and replaced with connective tissue. The observation by scanning electron microscopy shows that the corrosion cast of the hemangioma offers 3 periods of filling: initially with partial filling of the arteriolar and of the whole cast, intermediate with the entire filling of the whole cast (including arteriole and venule), and a last period with a partial emptying of the arteriolar and whole cast while the venule remains totally injected with resin. CONCLUSION: Our morphological results can be useful to clinicians for a precise diagnosis on the aftereffects of hemangiomas.

Chorangiocarcinoma: a case report and review of the literature.

Chorangiocarcinoma is the name designated to a chorangioma with trophoblastic proliferation manifesting increased proliferative activity. Only 3 such cases have been published so far. Other studies challenged this entity by demonstrating that proliferation of the trophoblast around chorangioma is a common phenomenon. We present a case of a unique vascular lesion in a term placenta with a malignant trophoblastic component. Microscopic examination of a well-demarcated placental mass revealed a chorangioma with multiple nodules composed of pleomorphic cells displaying focal multinucleation, large areas of necrosis, and high mitotic activity. Immunohistochemical stains of these cells were strongly positive for pancytokeratin and the beta subunit of human chorionic gonadotropin and focally positive for HSD3B1. There was no invasion of the basement membrane, and no free-floating tumor cells in the intervillous space. No evidence of metastasis was found on follow-up of the mother and newborn. It is concluded that the tumor presented herein, displaying a histologically unequivocal malignant trophoblastic component in a benign chorangioma, is a true chorangiocarcinoma, and should be included within the category of gestational neoplasia as a tumor closely related to choriocarcinoma.

A leiomyomatoid angiomatous neuroendocrine tumor of the myometrium: case study with ultrastructural analysis.

Leiomyomatoid angiomatous neuroendocrine tumor (LANT) is a possible new disease entity that was described as a dimorphic neurosecretory tumor with a leiomyomatous vascular component; it was found in the pituitary. We describe here a second case of LANT in a 45-year-old woman with a myometrial tumor, diagnosed clinically as uterine leiomyoma. She underwent laparoscopic myomectomy. The tumor consisted of hyalinized vasculature, containing factor VIII-positive endothelium and smooth muscle actin-positive vascular smooth muscle cells, and stromal cells, expressing neuroadhesion molecules. Both vascular and stromal components diffusely expressed chromogranin A and, as evidenced by electron microscopy, possessed smooth muscle actin filaments and electron-dense neurosecretory granules, which contained the neurosecretory hormone somatostatin. Although no cytokeratin-positive cells were observed, some tumor cells had positive Grimelius staining for argyrophilic granules. These findings meet the definition of LANT, and the occurrence of our case suggests that LANT is a special type of neuroendocrine neoplasm and is not organ specific.

Papillary haemangioma. A distinctive cutaneous haemangioma of the head and neck area containing eosinophilic hyaline globules.

AIMS: To investigate and define a morphologically distinctive group of cutaneous papillary haemangiomas. METHODS AND RESULTS: Eleven patients (seven male, four female, age range 1-77 years, median 57) were identified with a solitary bluish cutaneous papule (median size 11 mm) arising in the head and neck region. Most lesions had been present for several years. None of the patients had associated systemic disease or polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome. Only one lesion recurred locally. The lesions showed predominantly intravascular papillary growth within multiple ectatic dermal vessels. The papillae had cellular cores containing pericytes and stromal cells, arranged around normal small capillaries. The surfaces of the papillae were covered by focally swollen endothelial cells containing numerous hyaline globules, ultrastructurally representing giant lysosomes containing organelle debris and fat vacuoles (so-called thanatosomes). These endothelial cells were immunopositive for CD31 and CD34 but negative for D2-40 (podoplanin). CONCLUSIONS: Papillary haemangioma is a distinctive benign cutaneous lesion containing eosinophilic hyaline globules consistent with dysfunction of the autophagocytic-lysosomal pathway.

A novel in vivo model of human hemangioma: xenograft of human hemangioma tissue on nude mice.

BACKGROUND: Experimental models of human infantile hemangiomas are needed, although none of the current ones is ideal in representing the natural development of hemangioma. In this article, the authors present a nude mice model of human hemangioma with serial morphologic findings on grafts. METHODS: A specimen of rapidly growing hemangioma tissue was obtained surgically from a 2-month-old boy and was cut into 96 pieces, which were implanted subcutaneously into nude mice, six pieces each. During the 6-month experimental period, gross view, hematoxylin and eosin staining, double immunofluorescent labeling with mouse antihuman CD31 and fluorescein isothiocyanate-labeled rat antimouse CD34, and transmission electron microscopic observation were performed. RESULTS: Eighty-one of the 96 grafts (84.4 percent) survived and grew during the second month to 3 to 4 months after transplantation, followed by gradual involution. Histologic examination showed cellular edema, degeneration, and necrosis in the early stage. From approximately 30 days, cellular density increased and karyokinesis was identified at 45 days. At 2 months, the grafts were composed mainly of hemangioma tissue and signs of involution appeared. Thereafter, signs of involution turned significant and the grafts were replaced with fibrofatty tissue. It was confirmed through double immunofluorescent labeling that the chief cellular components were endothelial cells of human origin. Findings on ultrastructural investigation were concordant with those on light microscopic observation. CONCLUSIONS: With human hemangioma tissue implanted into nude mice, an animal model was established successfully. The development of grafts after ischemia resembled the natural course of human infantile hemangioma.

Clinical value of contrast-enhanced ultrasound in differentiating benign and malignant focal liver lesions.

To explore the clinical value of contrast-enhanced ultrasound (CEUS) in differentiating benign and malignant focal liver lesions (FLLs) with SonoVue, CEUS was used to examine 113 patients with focal liver lesions (FLLs) in our hospital during July 2005 to December 2006. All the patients underwent contrast-enhanced CT (CECT) or contrast-enhanced MRI (CEMRI). Except for patients with focal fatty sparings (n=18) and with hemangiomas (n=8), all the patients were confirmed by operation or ultrasonic-guided liver puncture biopsy. A sulfur hexafluoride gas-based contrast agent was used with a MI of 0.15 to 0.17. Forty-eight cases of malignant FLLs, including 30 hepatocellular carcinomas (HCCs), 2 cholangiocarcinomas and 16 metastatic tumors, were detected. Seventy-eight cases of benign FLLs, including 33 hemangiomas, 9 focal nodular hyperplasias (FNHs), 19 focal fatty sparings, 5 abscesses, 7 regenerative nodules and 2 inflammatory pseudo-tumor, were involved. The contrast pattern of benign and malignant FLLs was quite different. CEUS has higher specificity and sensitivity than conventional ultrasound in differentiating benign and malignant FLLs.

Clinical value of contrast-enhanced ultrasound in differentiating benign and malignant focal liver lesions / 华中科技大学学报（医学）（英德文版）

To explore the clinical value of contrast-enhanced ultrasound (CEUS) in differentiating benign and malignant focal liver lesions (FLLs) with SonoVue, CEUS was used to examine 113 patients with focal liver lesions (FLLs) in our hospital during July 2005 to December 2006. All the patients underwent contrast-enhanced CT (CECT) or contrast-enhanced MRI (CEMRI). Except for patients with focal fatty sparings (n=18) and with hemangiomas (n=8), all the patients were confirmed by operation or ultrasonic-guided liver puncture biopsy. A sulfur hexafluoride gas-based contrast agent was used with a MI of 0.15 to 0.17. Forty-eight cases of malignant FLLs, including 30 hepatocellular carcinomas (HCCs), 2 cholangiocarcinomas and 16 metastatic tumors, were detected. Seventy-eight cases of benign FLLs, including 33 hemangiomas, 9 focal nodular hyperplasias (FNHs), 19 focal fatty sparings, 5 abscesses, 7 regenerative nodules and 2 inflammatory pseudo-tumor, were involved. The contrast pattern of benign and malignant FLLs was quite different. CEUS has higher specificity and sensitivity than conventional ultrasound in differentiating benign and malignant FLLs.

Hemangiomas hepáticos: aspectos ultra-sonográficos e clínicos / Liver hemangiomas: ultrasound and clinical features

Os hemangiomas são os tumores hepáticos benignos mais comuns, ocorrem em todos os grupos etários, sendo mais comuns nos adultos. Na grande maioria dos casos os hemangiomas são pequenos, assintomáticos e descobertos incidentalmente. Lesões maiores eventualmente podem produzir sintomas. O aspecto ultra-sonográfico desses tumores varia, sendo que o aspecto usual é o de lesão pequena hiperecogênica bem definida. Neste artigo, os autores fazem uma revisão sobre aspectos clínicos e ultra-sonográficos dos hemangiomas, ressaltando a importância desses aspectos na condução clínica dos pacientes acometidos.

Hemangiomas are the most common benign tumors of the liver, occurring in all age groups, and more frequently in adults. The vast majority of hemangiomas are small, asymptomatic, and are incidentally discovered. Larger lesions may eventually produce symptoms. The sonographic aspect of these tumors varies, the lesions being typically small, well defined and hyperechoic. In this study the authors review clinical and sonographic features of hemangiomas, highlighting the clinical significance of such features to be taken into consideration in the treatment of affected patients.

Angioma de región malar / Angioma of zygomatic region

Los angiomas son tumores que afectan al endotelio de los vasos sanguíneos y linfáticos, conformando de este modo canales vasculares que no siguen un patrón normal. Histológicamente se pueden observar características similares al tejido normal y de acuerdo al diámetro de estos canalículos se desprende la clasificación en capilares, cavernosos y mixtos. Afectan a tejidos blandos y duros, dando diferentes características clínicas según su ubicación y la alteración que sufra el endotelio vascular. Se enumeran los estudios complementarios y los posibles tratamientos. Se describe el síndrome de Sturge-Weber-Dimitri. Por último se comenta un caso clínico. (AU)

Angioganglioglioma: a transitional form between angioglioma and gangioglioma?

The authors describe for the first time an unusual cerebral tumor with unique clinical history, composed of 3 components: pilocytic astrocytoma, vascular proliferations similar to those described as arteriovanous malformations, and a neoplastic ganglion component. These three components were intimately entangled and created the tumor mass. Thus the authors propose the term angioganglioglioma for this entity. The relation to the historically defined anglioglioma and tumors related to ganglioglioma and dysembryoplastic neuroepithelial tumor is discussed. The authors believe that this lesion, in regard to the clinical presentation (long course of the disease, clinical symptoms), is closely associated with ganglioglioma and, with other morphological features, also to angioglioma. Further, it may constitute a new distinct clinicopathological entity with neoplastic and hamartomatous features.

Tumores de las fosas nasales y senos paranasales. Tumores no epiteliales / Tumors of the nasal fossae and paranasal sinuses. Non epithelial tumors

Los tumores no epiteliales de las fosas nasales incluyen tumores de origen mesectodérmicos y neuroectodérmicos. Las neoplasias mesectodérmicas mas frecuentes son los vasculares y de estos los angiofibromas son los mas frecuentes y le siguen los hemangiomas, los angiosarcomas son excepcionales, Los linfomas son raros no obstante el anillo de Waldeyer. Los mixomas son frecuentes y por su vecindad con la órbita y la cavidad craneana aunque su comportamiento es benigno son muy destructivos y pueden llevar a la muerte. Dentro de los sarcomas lo rabdomiosarcomas son los mas frecuentes

Effect of dual and triple energy window scatter correction methods on image quality in liver scintigraphy.

AIM: To investigate the effect of two scatter correction methods on lesion detectability for both planar and tomographic hepatic imaging. METHODS: All planar and tomographic acquisitions involved simultaneous collection of photons in the main photopeak window (126-154 keV) and three additional windows (92-116, 116-126 and 154-164 keV). Uncorrected and corrected for scatter images were obtained from the same acquisition data. The dual energy window (DEW) and the triple energy window (TEW) scatter compensation methods were used to obtain two sets of corrected images. The DEW method was implemented with main photopeak window 126-154 keV, Compton scatter window 92-126 keV and scatter multiplier k = 0.5. A modified TEW method was also applied with main photopeak window 126-154 keV and scatter subwindows 116-126 keV and 154-164 keV. Phantoms were used to study the effect of scatter correction on contrast and signal-to-noise ratio. The observer's ability to identify lesions was studied on uncorrected and corrected for scatter patient images. RESULTS: In planar imaging, both scatter compensation methods yielded contrast enhancement. However signal to noise ratio (SNR) was degraded to 0.63 and 0.67 when DEW and TEW were applied respectively. In SPECT images, contrast was increased by a factor of 2.4 and 1.7, while SNR was degraded to 0.60 and 0.64 when DEW and TEW methods were used respectively. CONCLUSIONS: Scatter correction using DEW and TEW methods may improve observer's ability to distinguish lesions in planar (p < 0.05 for both methods) and SPECT (p < 0.05 for both methods) liver studies.

Immunoelectron microscopic characterization of human dermal lymphatic microvascular endothelial cells. Differential expression of CD31, CD34, and type IV collagen with lymphatic endothelial cells vs blood capillary endothelial cells in normal human skin, lymphangioma, and hemangioma in situ.

We performed a comparative investigation of the immunomorphological characteristics of lymphatic and blood microvascular endothelial cells in normal human skin, cutaneous lymphangiomas, and hemangiomas, employing a pre-embedding immunogold electron microscopic technique. We stained for cell membrane proteins that are commonly used for light microscopic characterization of blood endothelial cells. With blood microvascular endothelial cells, we observed uniform labeling of the luminal cell membranes with monoclonal antibodies (MAbs) JC70 (CD31), EN-4 (CD31), BMA120, PAL-E, and QBEND-10 (CD34), and strong staining of the vascular basal lamina for Type IV collagen under normal and pathological conditions. In contrast, lymphatic microvascular endothelial cells in normal human skin and in lymphangiomas displayed, in addition to a luminal labeling, pronounced expression of CD31 and CD34 along the abluminal cell membranes. Moreover, CD31 was preferentially detected within intercellular junctions. The expression of CD34 was mostly confined to abluminal endothelial microprocesses and was upregulated in lymphangiomas and hemangiomas. Type IV collagen partially formed the luminal lining of initial lymphatics and occasionally formed bridges over interendothelial gaps. Our findings suggest a function of transmigration protein CD31 in recruitment of dendritic cells into the lymphatic vasculature. CD34 labeling may indicate early endothelial cell sprouting. The distribution of Type IV collagen also supports its role as a signal for migration and tube formation for lymphatic endothelial cells.

Angiolymphoid hyperplasia with eosinophilia and vascular tumors of the head and neck.

PURPOSE: The clinicopathologic distinctions between angiolymphoid hyperplasia with eosinophilia (AHE) and vascular tumors are controversial. Some investigators believe that AHE is a variant of hemangioma, whereas others state that it is an inflammatory phenomenon. To better delineate the clinicopathologic entity of AHE and investigate the efficacy of various treatment regimens, we undertook a retrospective analysis of AHE and compared it with other angiomatous lesions treated at a tertiary referral center. MATERIALS AND METHODS: We reviewed the histopathologic features of hemangioma, AHE, and angiosarcoma of the head and neck seen in 46 cases at University of California Los Angeles (UCLA) Medical Center between 1950 and 1992. Lesions were evaluated for presence of lymphoid and eosinophilic infiltration, type of endothelial cell, and pattern of vascular proliferation. Patient charts were also reviewed for clinical history and outcome. RESULTS: The typical findings of AHE were present in 13 cases of conventional hemangioma and angiosarcoma. The clinical data of 8 AHE patients were also reviewed and a benign outcome was observed. CONCLUSION: We propose that AHE represents an angiomatous neoplasm similar to the hemangioma but characterized by a marked reactive appearance. AHE may comprise part of a spectrum of vascular tumors with differences between lesions depending in part on host-mediated inflammatory and immune responses.

Angioma serpiginoso en dos niños / Angioma serpiginosum in two children

El angioma serpiginoso es una lesión de tipo nevoide, poco frecuente, benigna, progresiva y asintomática, de aparición en edad temprana, que se caracteriza por máculas rojizas a violáceas que siguen un trayecto serpiginoso correspondiente a dilataciones capilares en la dermis papilar y subpapilar. Presentamos dos pacientes, una niña de 6 años y un varón de 5 años, cuyas características clínicas e histopatológicas corresponden a esta entidad

Angioma serpiginoso en dos niños / Angioma serpiginosum in two children

El angioma serpiginoso es una lesión de tipo nevoide, poco frecuente, benigna, progresiva y asintomática, de aparición en edad temprana, que se caracteriza por máculas rojizas a violáceas que siguen un trayecto serpiginoso correspondiente a dilataciones capilares en la dermis papilar y subpapilar. Presentamos dos pacientes, una niña de 6 años y un varón de 5 años, cuyas características clínicas e histopatológicas corresponden a esta entidad (AU)