Papillary intralymphatic angioendothelioma: Dabska tumor.

Papillary intralymphatic angioendothelioma (Dabska tumor) is a rarely metastasizing lymphatic vascular neoplasm that usually affects children and young adults. The majority of these cases occur in soft tissues of extremities, and to date less than 40 cases have been described. Despite the generally indolent evolution, can be locally invasive with the potential to metastasize. We describe a case of a young woman presenting with a plantar lesion, for 9 months and histological diagnosis of Dabska tumor. This neoplasm should be considered in the differential diagnosis of vascular dermatoses, allowing early diagnosis and treatment. Long-term follow-up should be performed.

Papillary intralymphatic angioendothelioma: Dabska tumor

Abstract Papillary intralymphatic angioendothelioma (Dabska tumor) is a rarely metastasizing lymphatic vascular neoplasm that usually affects children and young adults. The majority of these cases occur in soft tissues of extremities, and to date less than 40 cases have been described. Despite the generally indolent evolution, can be locally invasive with the potential to metastasize. We describe a case of a young woman presenting with a plantar lesion, for 9 months and histological diagnosis of Dabska tumor. This neoplasm should be considered in the differential diagnosis of vascular dermatoses, allowing early diagnosis and treatment. Long-term follow-up should be performed.

[Lymphatic malformations in the head and neck region. Clinical aspects and therapeutic options]. / Lymphatische Malformationen im Kopf-Hals-Bereich. Klinik und therapeutische Möglichkeiten.

Lymphatic malformations are congenital abnormalities of the lymphatic system which occur predominantly in the head and neck region. According to their dominant clinical and morphological characteristics, these are classified into micro- and macrocystic forms. Established therapies for lymphatic malformations include conventional surgery, sclerotherapy and laser treatment. Despite the significant improvements in therapeutic options seen in recent years, treatment of extensive lymphatic malformations remains an interdisciplinary challenge. Close-knit interdisciplinary cooperation is necessary to provide optimized care for affected individuals.

Secondary hypertension caused by massive renal lymphangiomatosis.

Renal lymphangiomatosis is a rare disease characterized by lymphatic vessel proliferation. We present a case of an adult patient with chronic flank pain, hypertension, and a right kidney mass. The magnetic resonance imaging findings were consistent with unilateral renal lymphangiomatosis. Technetium-99m dimercaptosuccinic acid renal scintilography revealed decreased ipsilateral renal function. From these findings, the patient underwent right nephrectomy, which resulted in complete remission of his hypertension and pain.

MR imaging features of nongynaecologic cystic lesions of the pelvis.

OBJECTIVE: The purpose of our article is to review the magnetic resonance imaging (MRI) features of nongynaecologic cystic lesions of the pelvis. CONCLUSION: The rising use of MRI for pelvic exploration will result in an increase in incidental detection of pelvic cystic cysts. Pelvic cysts of non gynecologic origin are less frequent than gynecologic cysts. However, they account for a wide range of abnormalities, and radiologists must be aware of their features and characteristics.

Head and neck lymphatic tumors and bony abnormalities: a clinical and molecular review.

Lymphatic malformations and lymphatic-derived tumors commonly involve the head and neck, where they may be associated with bony abnormalities and other systemic symptoms. The reasons for the association between these disorders and local skeletal changes are largely unknown, but such changes may cause significant disease-related morbidity. Ongoing work in molecular and developmental biology is beginning to uncover potential reasons for the bony abnormalities found in head and neck lymphatic disease; this article summarizes current knowledge on possible mechanisms underlying this association.

Linfangioma progresivo adquirido (linfangioendotelioma benigno) / Acquired Progressive Lymphangioma (Benign Lymphangioendothelioma)

El linfangioma progresivo adquirido es una neoplasia vascular poco frecuente, de comportamiento localmente agresivo. Histológicamente se caracteriza por la proliferación de múltiples vasos dilatados de paredes finas, revestidos con un endotelio plano sin atipias y que aparentemente disecan las fibras de colágeno. Esta entidad suele debutar de forma asintomática como una placa marrón-eritematosa que presenta un crecimiento gradual. Se presenta un caso de linfangioma progresivo adquirido en un varón de 32 años. La tumoración se localizaba en el hipogastrio sobre una lesión vascular congénita diagnosticada previamente de angiomatosis cutánea multifocal. Clínicamente cursaba con intenso dolor e imposibilidad para la deambulación por lo que fue resecada. El linfangioma progresivo adquirido debe ser considerado por dermatólogos y patólogos dado que su tratamiento quirúrgico precoz, cuando la lesión tiene un tamaño limitado, es siempre curativo y evita posteriores complicaciones derivadas de su crecimiento (AU)

Acquired progressive lymphangioma is a rare vascular tumor with a locally aggressive behavior. Histologically it is characterized by a proliferation of numerous, dilated, thin-walled vessels lined by flat endothelial cells with no nuclear atypia. The vessels appear to dissect between the collagen fibers. It usually presents as an asymptomatic, slow-growing, reddish-brown plaque. We present the case of a 32-year-old man with acquired progressive lymphangioma. The tumor was in the hypogastric region and had arisen on a congenital vascular lesion previously diagnosed as multifocal cutaneous angiomatosis. It was very painful and impeded walking, for which reason it was decided to perform excision. Dermatologists and pathologists must be aware of acquired progressive lymphangioma as early surgical treatment, while the lesion is still small, is curative and prevents subsequent complications due to growth (AU)

Linfangiomas de cavidad oral en edad pediátrica / Lymphangiomas of the oral cavity in childhood

Los linfangiomas son tumores raros y benignos producidos por el desarrollo anómalo de los vasos linfáticos. Presentamos un caso de linfangioma quístico asintomático localizado en paladar duro; se trata de una localización atípica, puesto que la mayoría de esas lesiones se localizan en la lengua a nivel de cavidad oral. Se realizó una resección con láser de CO2, sin que se evidenciase recidiva tras dos años de seguimiento. Actualmente se acepta adoptar una actitud expectante o el tratamiento con agentes esclerosantes que reduzcan su tamaño o los eliminen (AU)

Lymphangiomas are rare, benign tumors produced by the abnormal development of the lymphatic vessels. We present a case of asymptomatic cystic lymphangioma localized in the hard palate. This is an uncommon location since most of these lesions are found in the tongue at the level of the oral cavity. Resection was performed with CO laser, and there was no evidence of recurrence after two years of follow-up. At the present time, the approaches most widely accepted are expectant treatment or administration of sclerosing agents that reduce the size of the lesions or eliminate them (AU)

Pseudotumor inflamatorio multicéntrico con presentación asincrónica en meninges, hígado, bazo y ganglios linfáticos en una paciente con espondiloartropatía seronegativa. Presentación de un caso y revisión de la bibliografía / Multicentric inflammatory pseudotumor with asynchronic presentation in meninges, liver, spleen and lymph nodes in a patient with seronegative spondiloarthropathy. Case report and review of the literature

Introducción. El pseudotumor inflamatorio es un proceso reactivo de etiología y patogenia no bien definida que sepuede encontrar en cualquier localización. Los casos con afección del sistema nervioso central se han descrito en meninges, parénquima cerebral, plexos coroides y nervios craneales y espinales. Se han descrito casos multicéntricos, tanto sincrónicos como asincrónicos. Caso clínico. Mujer de 45 años portadora de una enfermedad reumatológica (espondiloartropatía seronegativa) a quien se le encontró un pseudotumor inflamatorio multicéntrico con afección del bazo, el hígado y los ganglios linfáticos paraaórticos asociado a fiebre de origen desconocido en 1995, y seis años después presentó una lesión similar en las meninges de la convexidad frontoparietal derecha, la cual se acompañó de fiebre, ataque al estado general, incremento de la velocidad de sedimentación globular, anemia microcítica hipocrómica y trombocitosis, que remitieron al resecar la lesión.Conclusión. Se revisan las características clinicopatológicas de esta entidad, así como las diferentes teorías que se han propuesto en relación con la etiología y patogenia, incluyendo las referentes a las manifestaciones sistémicas y alteraciones enlos exámenes de laboratorio asociadas a estas lesiones, que se presentan en un 15-30% de los casos y que están mediadas por citocinas producidas por las células inflamatorias del tumor

Introduction. Inflammatory pseudotumor is a reactive process in which the etiology and pathogenesis are not well defined, that can be found in any location. The cases with central nervous system affection have been described in meninges, brain, choroid plexus and cranial and spinal nerves. Multicentric cases, synchronous and asynchronous have been described.Case report. A 45 years-old woman with a rheumatologic disease (a seronegative spondiloarthropathy) who developed an inflammatory pseudotumor in spleen, liver and abdominal lymph nodes in 1995, associated to fever of unknown origin, six years later she presented with an inflammatory pseudotumor of the meninges in the convexity of the right frontoparietal region, with fever, malaise, and increase of globular sedimentation rate, microcytic hypochromic anemia and thrombocytosis.Conclusion. The clinicopathologic features of this lesion are revised, including the different theories in regard to the etiology and pathogenesis, and the role of cytokines produced by inflammatory cells in the tumor

Analysis of children with peripheral lymphadenopathy.

In this study, the clinical and laboratory features of children with lymphadenopathy were evaluated. Over a 3-year period, 126 patients were referred to the clinic for lymphadenopathy. Twenty-eight of cases have diseases mimicking lymphadenopathy; 98 (mean age: 86+/-55 months) have lymphadenopathy. Localized, limited, and generalized involvement was found in 52%, 30%, and 18% of patients. The most common localization was the head and neck region. The causes of lymphadenopathy were benign diseases in 75 patients. Sixty percent were reactive lymphadenopathy, 39% were lymphadenitis. Lymphadenitis was more frequently localized and bigger than 3 cm compared with reactive adenopathy (p=.02, p=.004). Twenty-three patients have malignant diseases whose mean age was higher than others (p=.002). The enlargement of supraclavicular nodes was more likely due to malignant disease (p=.001). The risk of malignant disease was higher in patients who had generalized lymphadenopathy, lymph nodes bigger than 3 cm, hepatosplenomegaly, and high lactate dehydrogenase levels. In conclusion, this study pointed out the important clues for the differential diagnosis, which were present in the history, physical, and laboratory findings.

Childhood cervical lymphadenopathy.

Cervical lymphadenopathy is a common problem in children. The condition most commonly represents a transient response to a benign local or generalized infection, but occasionally it might herald the presence of a more serious disorder. Acute bilateral cervical lymphadenopathy usually is caused by a viral upper respiratory tract infection or streptococcal pharyngitis. Acute unilateral cervical lymphadenitis is caused by streptococcal or staphylococcal infection in 40% to 80% of cases. The most common causes of subacute or chronic lymphadenitis are cat scratch disease, mycobacterial infection, and toxoplasmosis. Supraclavicular or posterior cervical lymphadenopathy carries a much higher risk for malignancies than does anterior cervical lymphadenopathy. Generalized lymphadenopathy is often caused by a viral infection, and less frequently by malignancies, collagen vascular diseases, and medications. Laboratory tests are not necessary in the majority of children with cervical lymphadenopathy. Most cases of lymphadenopathy are self-limited and require no treatment. The treatment of acute bacterial cervical lymphadenitis without a known primary source should provide adequate coverage for both Staphylococcus aureus and group A beta hemolytic streptococci.

Carcinoma erysipelatoides-cutaneous lymphatic vessel spread of a poorly differentiated naso-pharyngeal carcinoma.

Carcinoma erysipelatoides, which is frequently called inflammatory carcinoma, is an uncommon form of skin metastasis. It originates most commonly from breast carcinoma and less often from endometrial or bronchogenic carcinoma. The naso-pharynx is an unusual source for carcinoma erysipelatoides. We present a case of carcinoma erysipelatoides due to poorly differentiated naso-pharyngeal carcinoma.

Thrombomodulin as a marker of vascular and lymphatic tumours.

The aim of this study was to evaluate the utility of a new commercially available antibody to thrombomodulin as an endothelial marker in formalin-fixed paraffin-embedded tissue. The expression of thrombomodulin in a variety of 50 vascular and lymphatic neoplasms and malformations was compared to the expression of von Willebrand factor, QBend 10 (CD34) and JC70 (CD31). We showed that thrombomodulin was the best marker of lymphatic endothelium and also stained a higher percentage of malignant vasoformative tumours when compared to the other markers. We recommend the assessment of thrombomodulin expression in the differential diagnosis of malignant vasoformative neoplasms and in the detection of lymphatic endothelium for evidence of tumour permeation.