Small intestine & ampulla
Benign tumors / tumor-like conditions
Lymphangioma
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PubMed Search: Lymphangioma[TI] small bowel[TIAB]
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Etiology | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Mejbel HA, Al Diffalha S. Lymphangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/smallbowellymphangioma.html. Accessed January 16th, 2025.
Definition / general
- Nonencapsulated benign proliferation of lymphatic vessels
Essential features
- Benign neoplasm of lymphatic origin (proved by immunohistochemical expression of lymphatic markers, such as D2-40)
- Usually detected as an incidental finding during procedures performed for other reasons but can be symptomatic
- Mostly presents as a sporadic single lesion and rarely can be syndromic and diffuse
- Prognosis is excellent; local recurrence may occur, especially for incompletely excised lesions
Terminology
- Lymphangiomatosis (in cases of diffuse organ involvement)
- Lymphatic malformation / hamartoma
- Hemangiolymphangioma
- Simple (capillary) lymphangioma
- Mesenteric lymphangioma
ICD coding
- ICD-10: D18.1 - Lymphangioma, any site
Epidemiology
- Mostly arises in children < 5 years old, with male predominance; rare in adults (Pediatr Radiol 2002;32:88)
- Accounts for 2% of GI tumors in adults and 6% of GI tumors in children
Sites
- Terminal ileum is most common GI site but can arise in any part of the GI tract (Gastrointest Endosc 2005;62:181)
Etiology
- Environmental exposure: as a result of reactive lymphatic proliferation secondary to trauma
- Developmental anomaly: failure of development of lymphatic channels, resulting in lymphatic channel engorgement and dilation
- Genetics: Noonan syndrome, Maffucci syndrome, trisomies 13, 18, 21 and Turner syndrome (cystic hygroma)
Clinical features
- Variable clinical presentation, from asymptomatic to abdominal pain with mass effect (Keio J Med 1993;42:41)
- May present as an incidental finding during surgical procedures performed for other conditions (Int J Surg Case Rep 2020;66:319)
- Rarely in large lesions, complications include obstruction, infarct and perforation (J Clin Diagn Res 2017;11:PD01)
- May be associated syndromes (Turner, Noonan, Maffucci)
- Rarely is large, multiple or involves multiple organs (lymphangiomatosis)
Diagnosis
- Initial examination is by ultrasound and CT; definitive diagnosis by histopathology and ancillary studies
Radiology description
- Ultrasound: cystic lesion; endoscopic ultrasound may be useful to determine depth of lesion
- CT: nonenhancing cystic lesions, multiplication, may have calcifications (Acta Radiol Open 2015;4:2047981614564911)
Prognostic factors
- Incompletely removed lesions may increase recurrence
- Excellent prognosis
Case reports
- 22 year old man with lymphangioma causing intestinal malrotation (BMJ Case Rep 2013;2013:bcr2013008994)
- 37 year old man with recurrent intussusception (Int J Surg Case Rep 2020;75:126)
- 53 year old man with polypoid lymphangioma of the ileocecal valve (Saudi J Gastroenterol 2014;20:262)
- 57 year old woman with small intestinal lymphangioma who presented with persistent gastrointestinal bleeding (World J Gastroenterol 2012;18:2145)
Treatment
- Complete surgical resection, especially for symptomatic lymphangioma
- Endoscopic mucosal resection can be used for mucosal lymphangioma (Ann Surg Treat Res 2018;94:52)
Gross description
- Well defined lesions, polypoid or pedunculated mass with 3.0 cm average size
- Small lesions typically superficial (Gastrointest Endosc 2000;52:255)
- Large lesions may have calcifications and may involve deeper layers (World J Gastroenterol 2012;18:2145)
Microscopic (histologic) description
- Dilated and anastomosing thin walled blood vessels, lined by single layer of flat endothelial cells (Am J Clin Pathol 2015;144:563)
- Blood vessels are surrounded by lymphoid aggregate
- Lymphocytes, eosinophils and proteinaceous material often fill the vascular spaces
- Smooth muscle in blood vessel wall might be seen in large lesions
- No nuclear enlargement, hyperchromasia, pleomorphism or prominent nuclei should be seen in the lining endothelial cells
Microscopic (histologic) images
Contributed by Haider A. Mejbel, M.D.
Lobular contour
Dilated lymphatics
Endothelial cells
Nuclear uniformity
CD31 expression
Positive stains
- Common endothelial markers: CD34, CD31 and factor VIII
- More specific markers: podoplanin (D2-40), VEGFR3 and LYVE1 (J Biol Chem 2001;276:19420)
Negative stains
Sample pathology report
- Small intestine, biopsy:
- Lymphangioma
Differential diagnosis
- Secondary lymphangiectasia:
- Dilated lymphatics arise in response to trauma, obstruction or radiation
- Has identical histology (dilated lymphatics)
- Clinical history and presentation is necessary for distinction (Lymphat Res Biol 2009;7:75)
- Lymphangioma-like Kaposi sarcoma:
- Characteristic area of spindle cells with dissecting blood vessels diagnostic of Kaposi sarcoma should be present and HHV8 is positive
- Hemangioma:
- Smaller in size, has lobular contour and has fewer associated lymphocytes
- Angiosarcoma:
- Angulated and infiltrative blood vessels
- Nuclei of endothelial cells range from hyperchromatic to highly atypical with prominent nucleoli
Board review style question #1
A 34 year old man underwent upper gastrointestinal endoscopy for chronic anemia. A random biopsy from the duodenum shows dilated lymphatics lined by flatted endothelial cells. No atypia or mitosis is seen. Both CD31 and D2-40 are positive, highlighting the endothelial cells lining the lymphatic channels. HHV8 is negative. The most likely diagnosis is
- Angiosarcoma
- Hemangioma
- Kaposi sarcoma
- Lymphangioma
Board review style answer #1
Board review style question #2
What syndrome is associated with the development of large central lower neck lymphangioma (cystic hygroma)?
- Angelman syndrome
- DiGeorge syndrome
- Klinefelter syndrome
- Turner syndrome
Board review style answer #2
