Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Additional referencesCite this page: Shankar V. Lymphangioma & lymphangiomatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuelymphangioma.html. Accessed February 4th, 2023.
Definition / general
- May represent malformations due to failure of lymphatics to communicate with venous system
- Most described cases are cystic / cavernous
- Skin tumors are called lymphangioma circumscriptum
- Diffuse / multicentric lymphangiomas are often in thoracic cavity; also bone, extremities, soft tissue, viscera
Clinical features
- Benign
- See also discussion in these chapters: Lymph nodes, Mediastinum, Skin - nonmelanocytic tumors, Spleen
- Lymphangiomatosis:
- Rare, idiopathic, occurs mostly in children
- Cases with visceral involvement are often fatal but cases involving limbs have good prognosis (Am J Surg Pathol 1993;17:329, Am J Surg Pathol 1995;19:125)
- Associated with:
- Kaposiform hemangioendothelioma (Am J Surg Pathol 1993;17:321)
- Bony lytic lesions and chylothorax (J Pediatr Hematol Oncol 2008;30:66)
- Total cavopulmonary connection for complex congenital heart disease (Pediatr Cardiol 2008;29:771)
- Diffuse retroperitoneal cystic abdominal lymphangiomatosis is attributed to congenital malformation of lymphatic ducts; distribution often corresponds to primitive fetal lymphatic sacs; either capillary, cavernous or cystic; may have multisystem involvement involving bone, brain, extremities, liver, pancreas, soft tissue, spleen (Acta Cytol 2009;53:191)
- Gorham disease: diffuse lymphangiomatosis also involving bony tissue
Case reports
- 13 year old girl with isolated laryngeal lymphangioma (Indian J Otolaryngol Head Neck Surg 2011;63:90)
- 15 year old girl with perinephric lymphangiomatosis (Indian J Radiol Imaging 2010;20:224)
- 25 year old woman, with acute presentation of colonic lymphangiomatosis (Int J Surg Case Rep 2012;3:158)
- 25 year old woman with colonic lymphangiomatosis mimicking acute appendicitis (Indian J Cancer 2011;48:139)
- 33 year old woman with fatal case (Am J Med Sci 2008;336:445)
- 35 year old pregnant woman with large cystic tumor in fetal chest (Ultrasound Obstet Gynecol 2008;31:697)
- 39 year old healthy man with recurrent, complicated scrotal lymphangiomatosis (Can Urol Assoc J 2012;6:E11)
- 58 year old man with conjunctival lymphangioma (Case Rep Ophthalmol Med 2012;2012:836573)
- 71 year old man with mesenteric lymphangiomatosis (World J Gastroenterol 2009;15:3947)
- Secondary lymphangiomas of vulva (Indian J Dermatol 2012;57:149)
Treatment
- Excision if possible; also sclerotherapy or observation (Lymphology 2006;39:147)
- Recurrence is minimized by complete excision (Mayo Clin Proc 2006;81:1197)
- Intralesional bleomycin therapy may be an effective alternative to surgery (J Maxillofac Oral Surg 2011;10:152)
Microscopic (histologic) description
- Large lymphatic channels in loose connective tissue stroma
- Focally disorganized smooth muscle in wall of larger channels
- Peripheral lymphoid aggregates
- Often increased mast cells (Mod Pathol 1999;12:569)
- Lymphangiomatosis: increase in size and number of thin walled channels; lymphatic spaces are interconnecting and dilated, lined by a single attenuated layer of endothelial cells involving the dermis, subcutis and occasionally underlying fascia and skeletal muscle
Microscopic (histologic) images
Positive stains
- CD31, CD34, D2-40, (Pediatr Dev Pathol 2005;8:181)
- Patchy Factor VIII, (Am J Clin Pathol 1981;75:167)
- Also CK7 (Hum Pathol 2000;31:1062)
Negative stains
- Ki67 (or low) Arch Pathol Lab Med 1997;121:1055)
Electron microscopy description
- Poorly developed and discontinuous basal lamina; presence of anchoring fibrils (Arch Pathol Lab Med 1980;104:316)