Benign Lymphangioma Research Articles

Intra-abdominal Cystic Lymphangiomas: The Vanderbilt Experience

IntroductionLymphangiomas are rare, cystic tumors that represent congenital malformation of the lymphatic vessels. We reviewed our institution's experience treating abdominal lymphangiomas with the purpose of describing the clinical features, management, and outcomes of this rare pathology. MethodsThis is a single-institution, institutional review board-approved retrospective review of abdominal lymphangiomas presenting between January 2010 and February 2021. The diagnosis of lymphangioma was made on histopathology from either endoscopic or excisional biopsy of the lesion. Demographics, diagnostic imaging, histopathologic characteristics, and outcomes were analyzed. ResultsWe identified 48 patients, of whom 29 (60%) were female, >18 y (38; 79%), with a mean age of 43 y at the time of diagnosis (range, 4 d-87 y). Tumors ranged in size from <1 cm to 30 cm. Only 1/3 were symptomatic, most commonly with abdominal pain (9; 19%) On preoperative imaging, mural nodules or thickened walls were present in one case, in which pathology was consistent with benign lymphangioma. The majority of lymphangiomas were associated with the small bowel or its mesentery (31; 65%), followed by the colon/omentum (7; 15%). Most patients underwent surgical excision (29; 60%) with incomplete excision in one patient due to extensive local invasion, and three (10%) patients required multivisceral resection. The median duration of the follow-up was 13 mo (range, 1-105 mo), during which time, none of the patients developed malignancy. ConclusionsMost abdominal lymphangiomas arise from the small bowel and are found incidentally and have a favorable prognosis. Resection should be reserved for symptomatic lesions or when there is a diagnostic uncertainty.

S1‐Guideline Cutaneous Angiosarcomas – Update 2021

S1‐Guideline Cutaneous Angiosarcomas – Update 2021

Retroperitoneal Mass-Lesion with Calcificaction; Cystic Lymphangioma; Case Report

Lymphangioma in retroperitoneum is a rare mass-like lesion which presented histopatholigically with single or multi-cystic type and/or cavernous type; and clinically is often asymptomatic. Imaging studies was not enough to conclude exact diagnosis due to wide distribution of differential diagnosis resulted from different organ origination of tumor including of pancreas, liver, gastrointestinal and urologic system. This report described a 36 years old women complaining of 8 months vague abdominal pain underwent surgery in order to trans-abdominal total resection of a cystic mass-like tumor just located between abdominal aorta and inferior vena cava. Pathologic studies revealed benign multi-cystic lymphangioma with calcified foci. Symptoms completely removed after surgery and in follow-up period.

Induction of Lymphangiomas by Freund's Incomplete Adjuvant (FIA) in Fatty Acid Binding Protein (FABP)‐3, 5 and 7 Knock‐out Mice

IntroductionIn an FIA‐induced benign lymphangioma model (Mancardi et al., 1999), we have suggested a sequential transformation from peritoneal mesothelial cells to functional lymphatic vessels via fat storing tumor cells after the FIA stimulation (EB2009 &amp; 2015). However, the actual mechanism of the tumorigenesis and cellular involvement have yet to be established. FABPs are known to be involved in the promotion of cellular uptake and transport of fatty acids and the targeting of them to specific metabolic pathways. The aim of this study is, therefore, to investigate the possible involvement of FABPs during the FIA‐induced tumor development using FABP KO mice.Materials &amp; MethodsLymphangiomas were induced in C57BL/6 (B6) mice and FABP3, 5 and 7 KO mice by an intraperitoneal injection of FIA (0.2 ml emulsion). For histological analyses, tissue cryosections were made and immunostained with various antibodies against FABP3, 4, 5, 7, and various lymphatic markers, such as podoplanin (PDPN), LYVE‐1 and LA102.Results &amp; ConclusionsAfter FIA injection, peritoneal mesothelial cells became tall in height losing their polarity and gradually formed stratified cell masses on the peritoneal surface in B6 mice. The tumors became honeycomb‐like lymphangiomas consisted of ring‐like fat storing cells with various sizes. Some fat storing cells fused with each other and gradually formed either tubular structures like lymphatic vessels or huge follicles. These cells were strongly positive for PDPN, F4/80 and FABP5, but negative for FABP4. The follicular structures mainly consisted of FABP7+ cells. Interestingly, only the functional lymphatic vessel‐like structures expressed LYVE‐1 and LA102. In FABP‐KO mice, there was no significant difference in the incidence of tumors as compared with normal B6 mice. However, tumors developed more vigorously in FABP7 KO mice than other KO strains. Among FABP KO mice, relatively smaller fat storing cells were seen in FABP3 KO mice, whereas follicular type structures were more commonly found in FABP5 KO mice. In contrast, the tumors in FABP7 KO mice showed less fat storing cells with more inflammatory and fibrous components. In conclusion, a close relationship between the extrinsic FIA metabolism and the lymphatic system may be suggested in this tumor model, although the actual role and biological significance of each FABP in the tumorigenesis remain to be further clarified.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

English

Lymphangiomas are rare tumours of lymphatic vessels, most commonly found in children. We present the unusual case of small bowel obstruction caused by benign lymphangioma of Jejunal mesentery in a young man. KEY WORD: Lymphangioma: Jejunal mesentery. INTRODUCTION: Lymphangiomas are no longer considered to be truly neoplastic but rather may be the result of developmental failure of the original lymphatico-venous system. Three forms of lymphangioma are described: (i) Capillary lymphangioma, (ii) Cavernous lymphangioma, (iii) Cystic lymphangioma. They consist of numerous small or large, thin walled lymphatic spaces. Approximately 50% of the lesions have an associated haemangiomatous element. Lymphangioma are rare benign tumors. They are preferentially located in head, neck and axilla in children. However lymphangiomas in the peritoneal cavity are extremely rare, particularly in adults. This report describes a case of an adult with a large lymphangioma of the jejunal mesentery. CASE REPORT: A 22 year old man was admitted to hospital with small bowel obstruction. On examination - The Abdomen was distended, with a palpable vague lump .It was presumed that his condition was caused by obstruction. X-ray F.P.A.-showed multiple air fluid levels. USG Abdomen-showed a large multilocular solid mass with obscure margins. CECT/MRI of Abdomen was advised, which were not done. Emergency Exploratory Laparotomy was performed. At operation he was found to have a large multiloculated cystic cum solid mass measuring 20cm x 15cm in the mesentery of the small bowel approximately 3 feet from the duodeno-jejunal flexure. The mesentery had undergone torsion through 180 around the mass resulting in obstruction and gangrene of the small bowel. The torsion was reduced and the gangrenous small bowel (upto Ileo-Caecal Junction), and mesenteric lesion were resected (Figure). An Ileo transverse anastomosis was done. Histology showed this to be a benign lymphangioma of the small bowel mesentery with complete excision of the tumour. The tumour consisted of multiple cysts measuring up to 5 cm in diameter separated by fibrous or fibro muscular walls. The lymphangioma extended into the bowel wall, with increased lymphangiomatous spaces particularly prominent in the submucosa.

Lymphangioma of the ovary accompanied by chylous ascites

Lymphangioma of the ovary is very rare, with only 17 cases reported to date. In this report, a 43-year-old woman with lymphangioma of the ovary accompanied by chylous ascites is described. Vaginal ultrasound showed a solid cystic tumor at the right ovary accompanied by ascites. The volume of ascites was changeable. Milky fluid obtained by Douglas pouch aspiration contained numerous mature lymphocytes. She underwent right salpingo-oophorectomy. Histological examination revealed that the excised tumor from the right ovary was consistent with benign lymphangioma. This is the first case report of lymphangioma of the ovary accompanied by chylous ascites. We should discriminate such cases from other malignant tumors in order to avoid overtreatment.

Establishment of lymphangioma model and a study on the promoting effect of murine melanoma cell B16-F1 on the lymphangiogenesis in vitro

To establish an animal model of benign lymphangiomas of C57BL/6 mouse in vitro and to observe the effect of mouse ascites melanoma cell B16-F1 on the lymphangiogenesis, 16 C57BL/6 mice aged 8 weeks were given two intraperitoneal injections of incomplete Freund's adjuvant at a 15-day interval. The induced neoplasms were studied histopathologically and the neoplasms specimens were immunohistochemically examined for the expressions of VEGF-C (vascular endothelial growth factor-C) and Flt-4 (VEGFR-3, vascular endothelial growth factor receptor-3). The neoplasms were harvested and embedded in fibrin gel for culture in conditioned medium of B16-F1 cells in vitro and observed under inverted microscope. Our results showed that white solid tumor masses developed in peritoneal cavity after the induction. The tumors were confirmed to be lymphangioma by gross and histological examination. The tumor cells expressed both VEGF-C and Flt-4. Lymphatic capillaries coming from lymphangioma specimen grew into the gel and the conditioned medium of B16-F1 cells was found to be able to promote the growth of the vessels. It is concluded that intraperitoneal injection of incomplete Freund's adjuvant is a good method for inducing benign lymphangiomas in mouse and B16-F1 cells can promote lymphangiogenesis.

Generation and characterization of a mouse lymphatic endothelial cell line

Lymphatic vessels, by channeling fluid and leukocytes from the periphery into lymph nodes, play a central role in the development of the immune response. Despite their importance in homeostasis and disease, the difficulties in enriching and culturing lymphatic endothelial cells limit studies of their biology. Here, we report the isolation, stabilization, and characterization of a mouse lymphatic endothelial cell line (MELC) and the generated clones thereof. Cells were isolated from benign lymphangiomas induced by intraperitoneal injections of incomplete Freund's adjuvant. The MELC line expressed molecules typical of lymphatic endothelium, including VEGFR3/Flt-4, podoplanin, Prox-1, and D6, but not LYVE-1. It also expressed CD34, ICAM-1, VCAM, and JAM-A, but not CD31, VE-cadherin, E-selectin, or CX3CL1/fractalkine (both TNFalpha-induced), at variance with vascular endothelial cells tested in parallel. The inflammatory cytokines TNFalpha and IL-4 regulated production of selected adhesion molecules (VCAM), cytokines (IL-6), and chemokines (CCL2/JE). Whole genome transcriptional profiling identified a set of 150 known genes differentially expressed in MELC versus vascular endothelial cells. Thus, the MELC line may represent an invaluable source of lymphatic endothelium.

El linfangioma cavernoso de los genitales externos

PurposeFluctuating tumours of the scrotal and penile basis can be caused by benign lymphangiomas. These rare malformations can preferently be seen in children. Case ReportWe report about a 10 year old boy, who was presented in our department with a bluish, indolent, 4x2 cm sized, fluctuating tumor of the scrotum and penile basis. Magnetic resonance imaging could demonstrate the whole anatomic extension of the sonographically hypoechoic lesion. Operative removal of the tumour was performed without complication. Histologically, the specimen was diagnosed as cavernous lymphangioma. To date, there is no sign of recurrence (follow-up period: 6 months). ConclusionA cavernous lymphangioma of the scrotum or penis is very uncommon. We report, to the best of our knowledge, the 2nd case reported in literature. Even lymphangiomas with other histological features rarely affect this region. Lymphangiomas are benign tumours of lymphatic vessels. The surrounding tissue sometimes shows a perifocal inflammation and scarification. About 50% of the cases are considered congenital. 9/10 lymphangiomas become obvious during the first 2 postnatal years. Therapy of choice is the complete excision.

Effect of hepatoma H22 on lymphatic endothelium in vitro

To determine the effect of metastatic hepatoma cells on lymphangioma-derived endothelium, and to establish in vitro model systems for assessing metastasis-related response of lymphatic endothelium. Benign lymphangioma, induced by intraperitoneal injection of the incomplete Freund's adjuvant in BALB/c mice, was embedded in fibrin gel or digested and then cultured in the conditioned medium derived from hepatoma H22. Light and electron microscopy, and the transwell migration assay were used to determine the effect of H22 on tissue or cell culture. Expressions of Flt-4, c-Fos, proliferating cell nuclear antigen (PCNA), and inducible nitric oxide synthase (iNOS) in cultured cells, and content of nitric oxide in culture medium were also examined. The embedded lymphangioma pieces gave rise to array of capillaries, while separated cells from lymphangioma grew to a cobblestone-like monolayer. H22 activated growth and migration of the capillaries and cells, induced expressions of Flt-4, c-Fos, PCNA and iNOS in cultured cells, and significantly increased the content of NO in the culture medium. Lymphangioma-derived cells keep the differentiated phenotypes of lymphatic endothelium, and the models established in this study are feasible for in vitro study of metastasis-related response of lymphatic endothelium.

Surgical removal of intra- and extraoral cavernous lymphangiomas using intraoperative-assisted intralesional fibrin glue injections.

Benign lymphangiomas are encountered frequently in the head and neck region. They may be well circumscribed or diffusely invasive, involving subcutaneous tissue and underlying muscle, and consist of dilated, thin-wall sinuses. Surgical therapy with excision as wide as possible, while taking care to avoid rupturing the lesion, is the treatment of choice. To simplify complete excision, the authors introduce an intraoperative technique--intralesional injection of fibrin glue. Using this technique over period of 10 years in 27 patients (age range, 7-24 years) they removed successfully intra- and extraoral lymphangiomas. There have been no recurrences. This technique helps avoid rupture of the epithelium and bleeding, and makes it easier to remove lymphangiomas surgically and as completely as possible. This technique reduces recurrence remarkably and ensures successful surgical excision.

Malignant Chylothorax Treated by Talc Pleurodesis: A Case Report and Review of the Literature

Malignant Chylothorax Treated by Talc Pleurodesis: A Case Report and Review of the Literature

Benign lymphangioma of the jejunal mesentery: an unusual cause of small bowel obstruction.

Lymphangiomas are rare tumours of lymphatic vessels, most commonly found in children. We present the unusual case of small bowel obstruction caused by benign lymphangioma in a middle aged woman.

Surgical treatment of bilateral chylothorax due to benign lymphangioma of the thoracic duct

We present a female patient in whom after an 8-year history of recurrent bilateral pleural effusions, a benign lymphangioma of the thoracic duct was diagnosed and successfully treated by ligation of the duct, resection of the tumour and simultaneous decortication of the right pleural space and lung. This observation supports surgical intervention in this condition even in patients of advanced age.

Beware of overtreating children!

Some years ago I witnessed a clinical catastrophe involving a 4-year-old boy. A history of headaches led to skull roentgenograms that disclosed a large pituitary fossa. This triggered a series of clinical decisions that were climaxed by a carotid hemorrhage during transsphenoid biopsy. Autopsy revealed an empty sella and a benign lymphangioma in adjacent bone. The case was a source of fascination and much earnest and perplexed discussion. The consultants had little experience with similar cases and it was not entirely clear who was in charge. The cause of the headaches in this otherwise-normal boy was never known, and the long-range outlook without intervention was strictly speculative. However, it was an avoidable catastrophe and the case sensitized me to the factors that cause overtreatment of children. Four recent journal articles have crystallized some random thoughts about this problem. Stickler 1 discussed two infants who underwent unnecessary surgery that completed a

Acquired Lymphangioma (Lymphangiectasis)

Lymphangiectases are considered acquired dilatations of lymphatics as distinguished from new growths. Lymphangiomas are almost always congenital growths appearing soon after birth and containing mainly lymph as compared with hemangiomas that are new growths made up of bloodcontaining vessels. Lymphangiosarcoma (Stewart-Treves) is a malignant growth usually occurring in the lymphedematous ipsilateral extremity of a patient who has had a radical mastectomy. Verrucose lymphangioma-like lesions appeared on the vulva of an elderly woman several years after irradiation for squamous cell carcinoma of the cervix uteri. The lesions have the appearance of benign lymphangioma cutis but are located bilaterally and have developed late in life. The alternate diagnosis of lymphangiectasis was made despite the lack of preceding or accompanying edema. This condition can be compared with lymphangiosarcoma.

Malignant lymphangioma of the ovary

We have presented a case of an apparently morphologically benign lymphangioma of the ovary in which a resection was done. Six months later a local recurrence, peritoneal dissemination, and hepatic metastasis had taken place, and a new growth in the opposite ovary was present. A rather profound morphologic difference was evident between the primary and disseminated neoplasm, consisting of proliferation of the endothelium and changes in the individual cells. However, because of the marked necrosis of the primary together with marked hepatic necrosis present at autopsy make us feel that the tumor was malignant at its inception in spite of the regular benign morphology it presented.