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At least 19 recordsLinked to original sources

Lymphangioma circumscriptum associated with paravesical cystic retroperitoneal lymphangioma.

A boy with long-standing congenital cutaneous lymphangioma circumscriptum (LC), presented at the age of 6 years with an episode of painless haematuria, subsequently attributed to bladder wall involvement of a paravesical cystic retroperitoneal lymphangioma. Magnetic resonance imaging demonstrated this lymphangioma to be in communication with the cutaneous lesions. Deep lymphatic and visceral involvement is an extremely unusual occurrence in association with LC. This is the first report of an association between LC and cystic retroperitoneal lymphangioma. LC occasionally heralds a deeper, more clinically significant lymphangioma which may not become apparent until many years after the initial dermatological presentation. We report this case to raise awareness of this potential association and to demonstrate the usefulness of magnetic resonance imaging in the assessment of the deep component of cutaneous lymphangiomas.

Child↗

Co-expression of VEGF-C and its receptors, VEGFR-2 and VEGFR-3, in endothelial cells of lymphangioma. Implication in autocrine or paracrine regulation of lymphangioma.

Lymphangioma has long been thought of as congenital malformations resulting from the failure of lymphatic vessels communicating with the venous system in the fetal period. Alternatively, it is proposed to be true neoplasm originated from the transformation of lymphatic endothelia. To extend the molecular basis of the pathogenesis of lymphangioma, we have characterized the expression of vascular endothelial growth factor (VEGF) and VEGF receptors (VEGFR) in 29 cases of lymphangioma by RNA in situ hybridization. Endothelial cells of lymphangioma co-express transcripts of VEGF-C and its receptors VEGFR-3 (Flt4) and VEGFR-2 (Flk1), which are not detectable in the adjacent connective tissue. In contrast, there is little or no expression of VEGF-C, VEGFR-3, and VEGFR-2 mRNA in endothelial cells of hemangiomas, angiosarcomas, or normal lymphatic vessels of the small or large intestines. The results suggest that VEGF-C and its receptors may take active parts in the formation of lymphangioma by autocrine or paracrine regulation.

Adolescent↗

Lymphangioma tuberosum multiplex: an unusual variant of lymphangioma.

Lymphangioma tuberosum multiplex is a variant of lymphangioma in which there is an abundant proliferation of endothelial cells and dilated lymphatic vessels forming a large, lobulated tumor. A 57-year-old black man developed lymphangioma tuberosum multiplex progressively over a 30-year period following the removal of a smaller tumor from the right posterior flank.

Humans↗

Lymphangiomas of the larynx in infants and children. A survey of pediatric lymphangioma.

Lymphangiomas are uncommon benign congenital lymphatic tumors of childhood. They are found in all parts of the body structure, but rarely occur in the larynx. This paper reviews charts of 160 patients with cystic hygroma (lymphangiomata) of whom ten had extensive involvement of the larynx. A benign but nevertheless challenging tumor, its treatment demands the patience and skill of the surgeon. The lesion is more common in the white patient, and 40% appear in the newborn. Fifty percent present by the end of the first year of life, and 75% by the end of the second. The onset is uncommon in the older child and a rare occurrence in the adult. The treatment of lymphangiomata is surgical excision, and is more difficult when in the larynx. Laser surgery has been most helpful when the lesion is in the laryngeal complex. Considering the extreme difficulties which lymphangioma present to the child and to the physician, the ultimate results of all forms of conservative surgical therapy can be rewarding. Excessive or radical surgery will not necessarily guarantee complete elimination of disease and may be harmful.

Child↗

A clinical evaluation of lymphangioma of the large intestine: a case presentation of lymphangioma of the descending colon and a review of 279 Japanese cases.

With the development and widespread use of colonoscopy, lymphangioma of the large intestine has recently been reported frequently. This paper presents some findings from a review of 279 cases of this disease in Japan, including a typical case that we encountered. A 69-year-old female was diagnosed as having lymphangioma of the descending colon based on the findings of a barium enema and a colonoscopy, and the lesion was successfully removed by an endoscopic resection. In the published reports, the etiology of this disease is not clear yet but the age at onset range shows a tendency toward a higher incidence in comparatively older patients and the male-to-female ratio indicates a higher incidence in males. If there is no complication, endoscopic treatment seems to be the preferable procedure for this disease.

Aged↗

Lymphangiomas in infancy and childhood.

OBJECTIVE: Lymphangiomas are rare congenital malformations, commonly seen in the head and neck. This is a review of our experience in the management of 22 children with lymphangiomas. METHODS: The medical records of children with lymphangioma admitted to Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia over a period of 10 years from August 1989 to July 2000 were retrospectively reviewed for age at diagnosis, gender, mode of presentation, site of lymphangioma, method of treatment and outcome. RESULTS: We treated 22 children (12 females and 10 males) with lymphangioma. Their ages ranged from birth to 12 years, but majority (73%) were 4 years of age or younger. In 10 (45.5%), the lymphangioma involved the neck, 5 of them presented with sudden neck swelling as a result of hemorrhage into a lymphangioma, which caused diagnostic confusion. One patient had extensive lymphangioma involving the floor of the mouth, tongue, and left parotid gland. The remaining 11 patients had lymphangioma involving the parotid gland in 2, floor of the mouth in 3, and one each in the abdominal wall, above the right knee, mediastinum, breast, scrotum, and mesentery. All were treated surgically except 3 who were treated with intralesional bleomycin and showed complete disappearance of their lesions. There was recurrence in the child with mediastinal lymphangioma and a small recurrence in the child with bilateral lesions in the floor of the mouth. CONCLUSION: Lymphangiomas are relatively rare, involving mainly the head and neck, but they can be rarely seen at other sites. An important observation is the sudden appearance of cervical lymphangioma as a result of hemorrhage, which should be kept in mind. Our experience in the treatment of lymphangiomas using bleomycin is limited to draw any conclusions. We therefore considered surgery as treatment of choice for lymphangiomas. However, sclerotherapy can be used when there is a risk of damaging surrounding structures, and also to obviate the poor cosmetic results.

Child↗

Lymphangioma colli--a new classification contributing to prognosis.

The aim of the study was to introduce a classification of lymphangioma colli, which allows us to predict the expected morbidity and prognosis with surgical treatment. We defined 4 types, dependent on the volume of the lymphangioma as assessed by prenatal ultrasound or clinically: type I included tumors which had no or only a minimal effect on the contour of the neck; type II lymphangiomas were smaller than a line drawn at the lateral border of the head; type III tumors exceeded this line; in type IV the lymphangioma extended beyond the midline of the body. We report on the pre- and postoperative findings in 36 cases operated on between 1988 and 2000, mean follow-up was 4.2 years. 25 % of the cases were classified as type I, 36 % as type II, 22 % as type III, and 17 % as type IV. Cesarean section was carried out for 79 % of type III and IV lymphangiomas following prenatal diagnosis (71 %) and for 14 % of type I and II. MRT was indicated in 38 % of type II compared to 86 % in type III and IV lymphangiomas. Endoscopy was necessary in 25 % of type III and in 66 % of type IV lymphangiomas. In 2 cases (type I and IV), imaging revealed an intrathoracic portion. In 64 % of type I and II cases, resection was complete compared to 21 % in infants with more extended tumors. In all type IV cases, at least one further operation was required, likewise in 25 % of type III lymphangiomas. Postoperative effusion and infection occurred significantly more often in group IV than in groups I to III. 5 nerval lesions remained in children with operated type IV lymphangioma and 2 in type III cases. A visible lack of facial symmetry was evident at the follow-up examination in 83 % of type IV cases and moderate asymmetry was found in 19 % of all type I and II cases. Tracheostomy and gastrostomy were required only in type IV. The introduced classification of lymphangioma colli enables predictive statements to be made concerning the morbidity and prognosis with surgical therapy. The prognosis in type I is excellent; in type II, a cosmetically significant facial asymmetry remains in only 15 %. In type III lymphangiomas postoperative complications occurred in 38 % of cases, the necessity for reoperation with definitive lesions occurred in only 25 %. Morbidity gets significantly higher and the prognosis is worse if the lymphangioma exceeds the midline of the body.

Decompression, Surgical↗

Cutaneous lymphangioma.

Cutaneous lymphangiomas from 158 patients were studied clinically and pathologically. Lymphangiomas show a predilection for the neck and axilla, breasts and chest, and buttocks and thighs, but may occur on almost any area of skin. They show highest incidence of onset in infancy, the majority are present by age 5 years, but they may appear spontaneously in adolescence or adult life. No correlation among cutaneous lymphangioma, mucous membrane lymphangioma, internal lymphangioma, or lymphangiosarcoma was found. No familial histories of lymphangioma were elicited. Single surgical excision cured 75% of cutaneous lymphangiomas and reexcision cured an additional 12%. The cutaneous lymphangiomas are divided into superficial "lymphangioma circumscriptum" and deep "lymphangioma cavernosum." No specific histologic criteria could be found to differentiate lymphangioma from "bloodless" hemangioma, primary from secondary lymphangioma (lymphangiectasia), or cystic cavernous lymphangoma from cystic hygroma.

Adolescent↗

[A study of effects of pingyangmycin injection on treatment of lymphangiomas in oral, maxillofacial and cervical regions].

OBJECTIVE: The purpose of this study was to investigate the indication and therapeutic effects of Pingyangmycin injection as a primary therapy of lymphangiomas in oral, maxillofacial and cervical region. METHODS: A total of 195 patients (106 males and 89 females) with lymphangiomas in oral and maxillofacial regions were treated in the affiliated dental hospital of Sichuan University from May 1990 to December 2000. The patients' ages ranged from 0.5 to 46 years. The tongue was the most commonly involved site, followed by the cheek and the neck. The 200 lymphangiomas (5 patients had 2 lymphangiomas in different sites) underwent the therapy of Pingyangmycin, which was injected as with 1 mg/ml in saline. The total dose of Pingyangmycin ranged from 5 mg to 70 mg and 5 to 58 times, 1 time per 2-4 weeks. RESULTS: The curative rate of cystic-type lymphangiomas was the highest. Of the 51 cystic lymphangiomas, 110 capillary lymphangiomas, 18 cavernous lymphangiomas and 21 combinations of capillary and cavenous lymphangiomas, the curative rates were respectively 100% (51), 46.36% (51), 16.16% (3) and 19.05% (4), which showed a significant therapeutic effect, respectively. And 40(78.43%), 19(17.27%), 2(11.11%) and 0(0%) of them completely disappeared. There was no serious side effect with Pingyangmycin-injection treatment, such as pulmonary fibrosis. CONCLUSION: The treatment of injection of Pingyangmycin is a selective primary method of lymphangiomas, which can reduce the size of lymphangiomas, and make them completely disappeared.

Adolescent↗

Correlation of surgical outcome with neuroimaging findings in periocular lymphangiomas.

PURPOSE: To evaluate the results of surgical intervention and rate of recurrence in patients with periocular lymphangioma with respect to the anatomic extent of tumor involvement determined by neuroimaging studies. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Eighteen patients with periocular lymphangioma. METHODS: All patients underwent complete ocular examination and preoperative orbital imaging with either computed tomography or magnetic resonance imaging. Tumor location in the orbit was defined as intraconal, extraconal, or diffuse. For extraconal cases, a further classification was made as intraorbital, extraorbital, or mixed. All patients underwent orbitotomy via either a conjunctival or a skin-crease approach, depending on the tumor location. The indications for surgical intervention were varied and included preservation of visual function or cosmetic appearance in some cases and exploration of an unidentified orbital mass in others. The diagnosis of lymphangioma was established histopathologically in each case. Cases that showed recurrence at follow-up also were treated using an orbitotomy approach. MAIN OUTCOME MEASURES: Anatomic extent of tumor location in the orbit as determined by neuroimaging studies, completeness of surgical excision, pathological findings, recurrence, and visual outcome. RESULTS: Seven patients had extraconal tumors; 4, intraconal tumors, and 7, diffuse tumors. Of the patients with extraconal tumors, 4 had mixed lymphangioma and 3 had extraorbital lymphangioma. In 5 cases, the extraconal lymphangiomas were totally resected. In the remaining 13 cases with diffuse, intraconal, and infiltrating extraconal lymphangiomas, the tumors were treated with a subtotal resection procedure. At a mean follow-up of 29.4 months (range: 3-77), 2 patients with diffuse tumors developed 4 recurrences and underwent further surgical intervention for 3 recurrent events. The mean interval between treatment and recurrence was 16 months (range: 12-20). Two patients experienced decreased visual acuity due to compressive optic neuropathy in one case and operative damage in the other. CONCLUSIONS: Surgically well-delineated extraconal lymphangiomas can be resected totally with no clinical recurrence at short-term follow-up. Although intraconal, diffuse, and some extraconal lymphangiomas were treated with a subtotal resection procedure, many such cases did not demonstrate recurrence. These results are encouraging because they demonstrate that many periocular lymphangiomas can be treated successfully with good visual outcome and satisfactory cosmesis.

Adolescent↗