Acquired lymphangioma circumscriptum secondary to tuberculosis: A rare case report.

Lymphangioma circumscriptum is a benign lymphatic malformation that usually presents at birth or early childhood. Acquired oral lymphangioma has been reported secondary to radiation therapy and denture-induced trauma due to damage to previously normal lymphatics. To the best of our knowledge, this is the first report of acquired oral lymphangioma due to childhood tuberculous adenitis.

Clinical outcomes of primary surgical treatment for acquired vulvar lymphangioma circumscriptum.

OBJECTIVE: To assess the clinical outcomes of surgical treatment for acquired vulvar lymphangioma circumscriptum in patients who received radical surgery and/or adjuvant radiation therapy for cervical cancer. METHODS: A retrospective chart review of eight patients was performed to assess the demographic information, chief complaints, treatment modality for cervical cancer, location, and primary treatment modality for vulvar LC, postoperative changes in symptoms, and/or signs, the development of local recurrence and the outcome of patients. RESULTS: All eight patients were previously diagnosed with cervical cancer FIGO clinical stage IA to IIA and received surgery, radiation therapy, or concurrent chemoradiation therapy. Microscopic examination revealed multiple, dilated, D2-40-positive dermal vascular channels containing eosinophilic proteinaceous material, consistent with LC. Most chief complaints showed considerable improvements on assessment at the outpatient clinic after the primary surgery. No patient showed aggravation of symptoms. Two patients developed local recurrences. One patient developed recurrence on the opposite side 13 months after local excision. We performed a second wide local excision. Another patient developed recurrence 47 months after the primary surgery. Since the lesion was very small and localized, we decided to manage it conservatively, but monitor it very closely. The remaining six patients remained free of recurrence. CONCLUSION: It is not easy for gynecologists to have an initial clinical diagnosis of LC, because there are a number of diseases that exhibit similar clinical manifestation to that of vulvar LC. Even if it is diagnosed correctly, local recurrence often occurs. Relevant symptoms associated with LC are not only distressing, but also affect patients' quality of life. Based on our data, we propose that surgical treatment could provide a more long-lasting answer compared to other treatment modalities, since it is beneficial in terms of clinical outcomes. In the future, a long-term follow-up investigation is required to assess the prognosis and to compare the efficacy and side effects of each modality.

Possible Contribution of a Diverticulum to the Development and Rupture of Colonic Lymphangioma.

A 55-year-old Japanese man with a history of diverticulitis underwent colonoscopy for careful evaluation of progressive anemia. A 5-mm depressed lesion oozing spontaneously was observed at the hepatic flexure. On suspicion of depressed-type of cancer, right-sided hemicolectomy was performed. Histopathological examination indicated a collapsed lymphangioma exactly over a diverticulum, which had previously been complicated diverticulitis. The colonic mucosa and lymphangioma prolapsed beyond the subserosal layer via the muscularis propria defect, resulting in a depressed lesion and mucosal laceration with hemorrhage. This case suggests the contribution of a colonic diverticulum to the development and rupture of lymphangioma, which needed to be distinguished from depressed-type colon cancer.

[A Case of Retroperitoneal Lymphangioma that Was Difficult to Differentiate from Pancreatic Tumor].

We report a rare case of retroperitoneal lymphangioma that was difficult to differentiate from pancreatic tumor. The patient was a 38-year-old woman. In June 2011, CT detected a pancreatic tail tumor. She was expected to undergo radical surgery and was referred to our hospital. The tumor was 55×43×40 mm in size and was described as a multilocular cystic lesion with a partition on CT. MRI demonstrated the tumor as a high-intensity area on T2-weighted images. Sonazoid-enhanced US showed a contrast effect of the partition wall. Therefore, the lesion was diagnosed as a mucinous cystic neoplasm, and the patient underwent surgery. Intraoperatively, the tumor was a white multilocular cystic lesion with a capsule. The cystic lesion was exfoliated from the pancreatic parenchyma. Histologically, the tumor was diagnosed as a cavernous lymphangioma without malignancy. The surgery achieved good progress and the patient was discharged 12 days postoperatively. She is attending our clinic without any recurrence.

Treatment of a tongue lymphangioma with sirolimus after failure of surgical resection and propranolol.

Lymphangiomas of the tongue are rare, and their treatment is problematic. A 10 year-old patient with tongue lymphangioma who was previously treated with surgery and propranolol with no response was treated with sirolimus in our department. We used sirolimus with a dose of 1.6 mg/m(2)/day. After 3 months of treatment, the mass had decreased by more than 60%. We continued the treatment for 1 year with a maximum response of 70% decrease in mass. Disease remained stable 6 months after stopping therapy, the latest time of follow-up. Sirolimus appears to be effective in lymphangioma but requires further study.

[Lymphedema: anatomy, physiology and pathophysiology of lymphedema, definition and classification of lymphedema and lymphatic vascular malformations]. / Das Lymphödem - Anatomie, Physiologie und Pathophysiologie des Lymphödems, Definition und Klassifizierung des Lymphödems und lymphatischer Gefäßmalformationen.

Lymphedema is a chronic disease associated with a congenital or acquired disorder of the lymphatic vessels or lymph nodes. Untreated lymphedema can lead to complications and disability. Clinical Lymphology deals not only with lymphedema of the extremities but also of the head, the genitals and the internal organs (lymphostatic enteropathy, chylaskos, chylothorax, chylopericard etc). Symptoms of this disorder are often misdiagnosed or not recognized. Ignorance and trivialization of lymphedema causes insufficient treatment, which then is not carried out to the extent as it is possible today by scientific findings. Even today delayed or not optimal treatment causes a long ordeal for many patients.The fact that lymphedema for those affected is a major psychological and social burden, which is limiting the quality of life, has also often been unregarded. The knowledge of anatomy, physiology and pathophysiology as well as the knowledge of causes are necessary for diagnosis, so that early treatment can be initiated.

Human safety risk assessment of lymph node angiomas observed in 2-year carcinogenicity studies in rats.

The occurrence of mesenteric lymph node angiomas (benign vascular neoplasms including lymphangioma and hemangioma) in untreated control rats in 2-year carcinogenicity studies can range from rare to common depending on the strain used. This lesion is most common in male rats. Factors and conditions that may contribute to the etiopathogenesis of lymph node angiomas in rats include: (1) genetic drift, (2) congenital/developmental malformation, (3) sinus vascular transformation/venous obstruction of outflow, (4) "inflammatory" pseudo-tumors, and/or (5) defects of endothelial lymphatic vascular secretion/permeability. Lymph node angiomas in humans are extremely rare, not reported in mesenteric lymph nodes, and more common in females than males. The evaluation of increased mesenteric lymph node angiomas in rats for overall human safety risk assessment of novel pharmaceutical therapeutics should consider: genotoxicity of the test article, occurrence of vascular neoplasms in other locations in rats and in mice, occurrence of proliferative vascular lesions in nonclinical toxicology studies in non-rodent species, dose/exposure response, and pathophysiologic/morphologic differences and similarities of lymph node angiomas between rats and humans. Angiomas are independent lesions from angiosarcomas and are not precursors for angiosarcomas in either humans or animals. Mesenteric lymph node angiomas in rats are unlikely to be relevant for human risk assessment of pharmaceutical agents.

Digging deeper into lymphatic vessel formation in vitro and in vivo.

BACKGROUND: Abnormal lymphatic vessel formation (lymphangiogenesis) is associated with different pathologies such as cancer, lymphedema, psoriasis and graft rejection. Lymphatic vasculature displays distinctive features than blood vasculature, and mechanisms underlying the formation of new lymphatic vessels during physiological and pathological processes are still poorly documented. Most studies on lymphatic vessel formation are focused on organism development rather than lymphangiogenic events occurring in adults. We have here studied lymphatic vessel formation in two in vivo models of pathological lymphangiogenesis (corneal assay and lymphangioma). These data have been confronted to those generated in the recently set up in vitro model of lymphatic ring assay. Ultrastructural analyses through Transmission Electron Microscopy (TEM) were performed to investigate tube morphogenesis, an important differentiating process observed during endothelial cell organization into capillary structures. RESULTS: In both in vivo models (lymphangiogenic corneal assay and lymphangioma), migrating lymphatic endothelial cells extended long processes exploring the neighboring environment and organized into cord-like structures. Signs of intense extracellular matrix remodeling were observed extracellularly and inside cytoplasmic vacuoles. The formation of intercellular spaces between endothelial cells led to tube formation. Proliferating lymphatic endothelial cells were detected both at the tips of sprouting capillaries and inside extending sprouts. The different steps of lymphangiogenesis observed in vivo are fully recapitulated in vitro, in the lymphatic ring assay and include: (1) endothelial cell alignment in cord like structure, (2) intracellular vacuole formation and (3) matrix degradation. CONCLUSIONS: In this study, we are providing evidence for lymphatic vessel formation through tunneling relying on extensive matrix remodeling, migration and alignment of sprouting endothelial cells into tubular structures. In addition, our data emphasize the suitability of the lymphatic ring assay to unravel mechanisms underlying lymphangiogenesis.

Lymphangiogenesis: in vitro and in vivo models.

Lymphangiogenesis, the formation of new lymphatic vessels from preexisting ones, is an important biological process associated with diverse pathologies, such as metastatic dissemination and graft rejection. In addition, lymphatic hypoplasia characterizes lymphedema, usually a progressive and lifelong condition for which no curative treatment exists. Much progress has been made in recent years in identifying molecules specifically expressed on lymphatic vessels and in the setting up of in vitro and in vivo models of lymphangiogenesis. These new tools rapidly provided an abundance of information on the mechanisms underlying lymphatic development and the progression of diseases associated with lymphatic dysfunction. In this review, we describe the common in vitro and in vivo models of lymphangiogenesis that have proven suitable for investigating lymphatic biology and the interactions occurring between lymphatic vessels and other cells, such as immune cells and cancer cells. Their rationales and limitations are discussed and illustrated by the most informative findings obtained with them.

Telangiectatic Carcinoma - Like Lymphangioma Circumscriptum. A Rare Form of Cutaneous Metastasis of Breast Carcinoma: Case Report.

INTRODUCTION: The Breast cancer is the most common malignancy in middle-aged women and that causes skin metastasis. Skin metastasis in internal cancer cases is a very rare condition and may be difficult to diagnose and have poor prognostic marker. Cutaneous metastasis of breast carcinoma is mostly seen as direct invasion and/or local infiltration. However, in addition to the well-known types, cutaneous metastases may mimic many benign skin lesions and therefore may be difficult to diagnose. CASE REPORT: In this article we present a 36-year-old woman with telangiectatic carcinoma-like lymphangioma circumscriptum, a rare form of cutaneous metastasis skin metastases. It can be the first sign of internal malignancies, so early diagnosis is very important at this stage. CONCLUSION: Therefore, solitary lesions or benign dermatoses seen in the skin and not associated with specific disease should be considered as tumor metastasis especially in female patients with a history of breast cancer and differential diagnosis must be made.

Papules arising after radiotherapy for rhabdomyosarcoma.

Radiation therapy, even at low doses, can induce a wide spectrum of vascular skin proliferations ranging from nonmalignant ones, such as benign lymphangiomatous papules (BLAP), to frankly malignant pathologies, such as angiosarcoma. We describe a 50-year-old Caucasian woman with a past history of uterine rhabdomyosarcoma, treated 22 years prior with surgical excision, chemotherapy, and radiotherapy. She presented with a few skin-colored papules and a clear discharge located in the previously irradiated area (right inguinal region). Histopathology showed a proliferation of irregular, interanastomosing vascular channels, thin walled and lined by prominent endothelial cells with focally hobnail features. Cytological atypia of endothelial cells, mitotic figures, hemorrhagic areas, and necrosis were not observed. The endothelial cells expressed D2-40 and CD31. A diagnosis of BLAP following radiotherapy for uterine rhabdomyosarcoma was made. The patient was treated with complete excision using electrodessication. At the 20-month follow-up visit the patient was still free of recurrence.

Penile Lymphangioma Secondary to Sclerosing Lymphangitis/Mondor's Disease in an HIV-Positive Patient.

A 34-year-old man presented with a painless lesion of the glans present for more than 4 years. The patient became HIV-positive in 2011, and he has been treated with tenofovir, emtricitabine, and efavirenz. A CD4 count performed 4 months prior was 570 cells/mL3; syphilis, hepatitis B, and hepatitis C serologies performed on the same date were non-reactive.

Pathogenesis of lymphangiomas.

Based on various hypotheses concerning lymphangiogenesis published in the literature, different putative mechanisms of lymphangioma development are discussed including failure of the lymphatic system to connect with or separate from the venous system, abnormal budding of the lymphatic system from the cardinal vein, or acquired processes such as traumata, infections, chronic inflammations, and obstructions. Increasingly, the possible influence of lymphangiogenic growth factors on the development of lymphangiomas is discussed. The proved expression of different growth factors in the endothelium of lymphangiomas leads to new hypotheses regarding the pathogenesis of lymphangiomas. Thus, further studies on the lymphangiogenesis and the development of lymphangiomas will have to clarify as to whether lymphangiomas are true malformations or neoplastic in nature.

Oral lymphangiectasias and Crohn's disease: two case reports.

Lymphangiectasias, or acquired lymphangiomas, are rare in the oral cavity, more typically occurring on the skin or the genital area and, to our knowledge, have not been reported previously in association with Crohn's disease. Lymphangiectasias can occur at any age and develop secondary to chronic obstruction of the lymphatics. This differentiates them from congenital lymphangiomas, which are congenital malformations of the lymphatic system. We present 2 cases of oral lymphangiectasias associated with Crohn's disease, one of which was treated successfully with cryotherapy.

Lymphangioma Circumscriptum Post Radiotherapy for Penile Cancer Treated with CO2 Laser.

Lymphangioma circumscriptum (LC) is a rare, benign condition, predominantly characterized by the malformation of lymphatic skin vessels. Its onset may be congenital or due to secondary causes such as radiotherapy, infections, or surgical procedures. We present the case of a 55-year-old patient with a pathologic history of squamous cell carcinoma of the penis followed by radical penectomy. Due to metastasis to the locoregional lymph nodes, the entire affected area was subsequently treated with radiation therapy, receiving a total dose of 55.8 Gray. Eight years after this treatment, translucent vesicles filled with a clear liquid appeared on the scrotum. Histopathology confirmed the diagnosis of LC and therapy with CO2 laser was applied, resulting in a favorable outcome. LC of the scrotum may present a long-term radiotherapy-induced complication of this site. Our clinical experience showed that the CO2 laser was the therapy of choice as the vesicles entirely disappeared and healed as white scar-like lesions.