A case of extensive acquired progressive lymphangioma

Abstract

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.

Giant benign lymphangioendothelioma with positive expression of Wilms tumor 1: A case report

“…5 The clinical differential diagnosis of BL includes ecchymosis, vascular malformation, Kaposi sarcoma, and low-grade angiosarcoma. 1 The definitive diagnosis requires histopathological examination, which usually shows thin-walled vessels in the dermis and subcutaneous tissue. 1 The endothelial cells of well-differentiated angiosarcoma exhibit atypical cytologic features such as hobnailing, hyperchromasia, and nuclear enlargement, 2 which were not present in this case.…”

“…1 The definitive diagnosis requires histopathological examination, which usually shows thin-walled vessels in the dermis and subcutaneous tissue. 1 The endothelial cells of well-differentiated angiosarcoma exhibit atypical cytologic features such as hobnailing, hyperchromasia, and nuclear enlargement, 2 which were not present in this case. Since the patient neither report previous radiation therapy nor exhibited signs of chronic lymphedema, immunohistochemical staining for Ki-67 and c-myc was not performed.…”

“…Benign lymphangioendothelioma (BL, acquired progressive lymphangioma) is a rare, slow-growing lymphatic tumor, first described 40 years ago, with fewer than 50 published cases. 1 Typically, it affects young adolescents but may arise in adulthood, with no sex predilection. 2 It presents as an asymptomatic macule or plaque of red or bruise-like coloration, with possible superimposed papules.…”

A rare case of thigh asymmetry in an infant

“…Literature review reveals varied presentation of the entity such as juxta-articular lymphangioma of the extremity, 5 retroperitoneal and genital lymphangioma, 6 and even acquired cases of progressive lymphangioma. 7 Previously published case reports documented abdominal lymphatic malformations extending to the extremities, mostly in autopsy specimens of preterm infants after termination of pregnancy. 8 This case illustrates an uncommon yet important cause for unilateral extremity swelling of congenital origin.…”

American Thoracic Society 2019 Pediatric Core Curriculum

“…Channel type disorders result from failure to clear lymph due to channel obstruction, dilation, or dysmotility, causing chylothorax, pulmonary lymphangiectasia, and edema of the extremities or body wall. Acquired progressive lymphatic anomaly or so called progressive lymphangioma is a rare benign LM appearing as a solitary plaque on the trunk and limbs possibly as response to injury . Primary lymphedema leads to abnormal accumulation of interstitial fluid as a result of developmental defects affecting maturation and function of the lymphatic system.…”