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.

[Medication-based therapy of infantile hemangioma and lymphatic malformations]. / Medikamentöse Behandlungsansätze für infantile Hämangiome und lymphatische Malformationen.

The therapy of vascular tumors and malformations should be interdisciplinary and performed according to available guidelines. Infantile hemangiomas (IH) are the most frequent vascular tumors of childhood and do not require treatment in most cases. If the IH is complicated by its location (e.g. facial or genital) or if the lesion threatens to cause loss of function, small localized IH should be treated by laser- or cryotherapy. If the IH is diffuse or rapidly growing it can be successfully treated using the ß blocker propranolol. The mechanism underlying the efficacy of this medication-based therapy is not completely understood and this still represents an experimental therapy. The results of molecular studies on vascular malformations have indicated new strategies for medical therapies. However, lymphatic malformations (LM) are still treated by surgery where possible, or sclerotherapy. Further investigations are necessary to determine whether new drugs such as the mTOR inhibitor rapamycin may be effective for treatment of diffuse LM. First case reports seem to be promising.

[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.

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.

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.

Possible role of EBV in breast cancer and other unusually EBV-associated cancers.

Epstein-Barr virus (EBV) a ubiquitous gamma herpesvirus persists for life, generally without health consequences. However, it is associated with several well-recognized malignancies, such as Burkitt's lymphoma and nasopharyngeal carcinoma. A growing list of malignancies has been proposed to be EBV-associated: most of which are consistently EBV-positive whereas others show inconsistent results. The possible contribution of EBV to the development and/or progression of different "non-classical" tumors is discussed in terms of putative "non-traditional'' infection in EBV-related tumors.

Increased vascular endothelial growth factor-C expression is insufficient to induce lymphatic metastasis in human soft-tissue sarcomas.

PURPOSE: Unlike carcinomas, soft-tissue sarcoma (STS) rarely exhibit lymphatic spread. Consequently, we examined expression and function of vascular endothelial growth factor (VEGF)-C and STS-associated lymphatic vessel density (LVD) components of this process. EXPERIMENTAL DESIGN: VEGF-C and VEGF-A mRNA and VEGF-C protein expression were evaluated in STS, STS cell lines, and breast cancers (reverse transcription-PCR, quantitative reverse transcription-PCR, and ELISA). STS cell conditioned medium after VEGF-C knockdown was examined for endothelial cell proliferation and migration effects (MTS and migration assays). Paraffin-embedded human lymph node-negative and lymph node-positive STS and lymph node-negative and lymph node-positive breast cancers were examined for VEGF-C, D2-40, and CD31 expression (immunohistochemistry). LVD differences were analyzed by Wilcoxon rank-sum tests. RESULTS: STS and breast cancer VEGF-C expression was comparable and higher than normal tissue levels. STS cells secreted functional VEGF-C: STS conditioned medium induced lymphatic endothelial cell proliferation and migration, which was abrogated by STS cell VEGF-C knockdown. STS and breast cancer intratumoral LVD was similar. STS peritumoral LVD (PT-LVD) was reduced versus breast cancer PT-LVD (P < 0.001). Significantly higher PT-LVD was observed in lymph node-positive versus lymph node-negative STS; lymphatic spreading STS subtypes also had higher LVD. STS VEGF-C expression and PT-LVD lacked correlation, and many lymph node-negative STS had high PT-LVD, suggesting complexity in this metastatic process. CONCLUSIONS: Compared with breast cancers, STS exhibited lower PT-LVD independent of VEGF-C expression, which may underlie STS lymph node metastasis rarity. Moreover, lymphatic vessels appear necessary but not sufficient to sustain STS lymphatic spread. Examining STS "nonlymphatic" dissemination may help elucidate mechanisms of lymphatic spread, insights critically important to cancer metastasis control.

[Unusual metastasis of renal carcinoma]. / Metastases inhabituelles du cancer du rein.

Renal carcinoma, the third most common urological cancer, induces presence of metastases in 75% of cases. The most affected sites for metastasis are the lungs, the lymphatic system, bones, the liver, adrenal glands and the brain with sometimes a cancer free period of several years prior to evolutionary recurrence of the disease. The aim of this literature review is to report on secondary uncommon renal localizations by underlining their clinical significance, as well as main characteristics, in order to provide guidelines for effective patient diagnosis and therapeutic management.

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.

Identification of lymphatic endothelium in pediatric vascular tumors and malformations.

The distinction between lymphatic and other vascular vessels on microscopic sections is a challenging task. D2-40, a novel antibody, has been reported to be selective for lymphatic endothelium. We studied the specificity and sensitivity of D2-40 in pediatric vascular tumors and malformations. Fourteen lymphatic and 11 vascular lesions were randomly selected and stained with D2-40 and CD31 antibodies. The lymphatic lesions included 6 lymphatic malformations, 5 cystic hygromas (macrocystic lymphatic malformation), 2 lymphovenous malformations, and 1 lymphangioma, and the vascular lesions comprised 3 infantile hemangiomas, 3 Kaposiform hemangioendotheliomas, 2 tufted angiomas, 1 pyogenic granuloma, 1 arteriovenous, and 1 venulocapillary malformations. The staining patterns of the vascular channels were compared. In all lesions D2-40 labeled only the endothelium of thin-walled vascular channels morphologically consistent with lymphatic vessels (25 of 25). No staining of the vascular lesions (0 of 11) or of arteries and veins (0 of 25) was observed. All lymphatic lesions had D2-40-positive vessels; however, the percentage of vessels that stained varied. Five lymphatic lesions showed more than 75% D2-40-positive channels, 5 lesions had approximately 50%, and 4 cases showed fewer than 25% D2-40-positive channels. There was a tendency of more consistent D2-40 staining of small versus large lymphatic channels. CD31 constantly labeled arteries, veins, capillaries, and lymphatics in all lesions and all endothelial cells in the vascular lesions. D2-40 is a very specific antibody for lymphatic endothelium, with variable sensitivity. CD31 more reliably identifies lymphatic endothelium. Currently, D2-40 appears to be a good marker to identify lymphatic vessels in pediatric vascular tumors and malformations.

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.

Visualizing function in the tumor-associated lymphatic system.

The recent surge of interest in the lymphatic system can be attributed to two factors: the discovery of cytokines that induce the growth of new lymphatic capillaries and the identification of lymphatic endothelial-specific markers. In contrast to the above, there is a paucity of techniques for studying lymphatic function in vivo. This article reviews imaging and other techniques that allow the assessment of lymphatic function, particularly in the tumor microenvironment, and proposes novel solutions for probing the same in vivo.