A case of extensive acquired progressive lymphangioma.

Acquired progressive lymphangioma (benign lymphangioendothelioma) is a rare lymphatic anomaly of unclear pathogenesis. Excision is generally advised for local disease, although other therapies have been tried. This report describes a unique case of extensive acquired progressive lymphangioma involving the abdomen, genitalia, and lower extremity of a 1-year-old boy. Rapid progression and multisite involvement required exploration of nonsurgical options for management.

Benign lymphangioendothelioma presenting as a giant flank mass.

Benign lymphangioendothelioma is a rare lesion of controversial etiology and a histopathologic mimic of Kaposi sarcoma and so-called 'well-differentiated' angiosarcoma. Its most typical clinical presentation is as a slowly expanding, erythematous patch or plaque; it rarely presents as a large mass. We report the second case of a giant benign lymphangioendothelioma, which arose as a serpiginous mass involving most of the flank of an elderly male with no prior radiation exposure and with a remote history of herpes zoster infection. A biopsy revealed numerous anastomosing vascular channels extending from the superficial dermis to the subcutis that were dilated to progressively slit-like in architecture. The endothelial cells lacked cytologic atypia, hobnailing, or significant mitotic activity, and human herpesvirus-8 expression was absent. Positivity for podoplanin (D2-40) was observed in the endothelial cells, supporting a lymphatic phenotype. Furthermore, the lesional cells lacked immunohistochemical expression of Wilms tumor 1, providing further support of a malformative - rather than neoplastic - pathogenesis.

Inflammatory Nasal Polyp With Benign Lymphangioendothelioma/Acquired Progressive Lymphangioma in a Young Man.

A 17-year-old Japanese male presented with a nasal polyp and neck lymphadenopathy. He was referred to our hospital for diagnosis and treatment of neck lymphadenopathy. Grossly, a whitish pedunculated nasal polyp was observed on the right side of the nasal septum. Computed tomography confirmed the presence of a nasal polyp and two nodular lesions in the right maxillary sinus. All three lesions showed no contrast enhancement. Clinical examination revealed neck lymphadenopathy, suggestive of Kikuchi disease. The nasal polyp was resected, and pathological examination revealed an inflammatory nasal polyp with benign lymphangioendothelioma, also known as acquired progressive lymphangioma. The right maxillary sinus nodules were followed up. The nasal occurrence of benign lymphangioendothelioma is markedly unusual, as the lesion typically occurs in the skin. To the best of our knowledge, this is the first reported case of nasal benign lymphangioendothelioma in our review of the PubMed database.

Benign lymphangioendothelioma: a clinical, histopathologic and immunohistochemical analysis of four cases.

BACKGROUND: Benign lymphangioendothelioma represents a rare lymphatic vascular proliferation characterized by proliferation of irregular and thin-walled vessels dissecting amongst dermal collagen. Immunohistochemical analysis has been lacking in most previously reported cases. METHODS: Herein, we report the clinical and histopathologic characteristics of four cases of benign lymphangioendothelioma. Immunohistochemical study was completed for all lesions. RESULTS: All lesions presented as large, red to brown patches or plaques. Three lesions were located on the thigh and one lesion was located on the neck. Histopathologically, all lesions showed proliferation of anastomotic or retiform thin-walled vessels with a single layer of endothelial cells that dissect the dermis. D2-40 and Prox1 immunostains were positive and Wilms tumor 1 (WT-1) immunostain was negative in all cases. CONCLUSION: Benign lymphangioendothelioma represents a lymphatic vascular proliferation. A lack of expression of WT-1 suggests it represents a lymphatic vascular malformation.

Surgical Treatment for Benign Lymphangioendothelioma After Two Incomplete Excisions: A Case Report and Literature Review.

Benign lymphangioendothelioma (BL) is a rare, poorly identified, slow-growing benign vascular lesion characterized by asymptomatic, solitary, well-demarcated macules, or by mildly infiltrated plaque. We report a case of an atypical BL that arose as a tender, protuberant, flesh-colored mass with cyanotic vesicles, and then progressed to a persistent exudative wound after two incomplete excisions. The patient was also diagnosed with thoracic duct narrowing. Although the stenosis was removed by surgery, the right lower extremity ulceration and exudation did not improve. Thus, we performed a thorough excision and split-thickness skin graft transplant following vacuum sealing drainage, and eventually the patient had a favorable functional and cosmetic outcome. A biopsy revealed irregular, dilated vascular spaces lined with a single layer of flat endothelial cells extending from the superficial dermis to the subcutis that did not reach the striated muscles. Additionally, by reviewing the literature on BL, in this paper we summarize the diverse pathogenic, morphological, and immunohistochemical presentations for this rare disease, as well as the histopathological differential diagnosis of lymphangiomatosis, Kaposi's sarcoma, and angiosarcoma.

Benign Lymphangioendothelioma - A Case Report.

Benign lymphangioendothelioma is an uncommon locally infiltrative lymphatic tumour, presenting as a slow-growing, asymptomatic, reddish-violaceous macule or plaque. Histopathologically, it is characterized by thin-walled endothelial-lined spaces that are interspersed between strands of collagen. It must be recognized and differentiated from angiosarcoma, early Kaposi's sarcoma, in view of major differences in treatment and prognosis. A 24-year-old female presented with a raised lesion over the left leg since 2 years which was associated with minimal itching. Biopsy of the lesion showed thin walled vascular channels lined by single layer of bland endothelial cells at the dermo-epidermal junction, few vessels in the dermis.