9170/0 Lymphangioma, NOS


Synonyms

Lymphangioendothelioma, NOS

Definitions

Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22

Lymphangioma is a rare benign tumour that is usually observed in children and adolescents. It may occur as a solitary mass or more commonly as multiple masses composed of multiple endothelial- lined spaces that vary in size from capillary channels to large, cystic spaces containing clear pink-staining lymph. The vascular spaces are lined by a single layer of endothelial cells. The supporting stroma is usually scanty and may contain lymphoid tissue. Painless swelling is the usual clinical manifestation.

Hepatic lymphangiomas extremely rarely occur in isolation; they are often accompanied by lymphangiomatosis of the spleen, skeleton, and other tissues, and may represent a malformation syndrome. Diffuse lymphangiomatosis involving the liver and multiple organs is associated with a poor prognosis 1
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Datz C, Graziadei IW, Dietze O, Jaschke W, Königsrainer A, Sandhofer F, Margreiter R (2001)
Massive progression of diffuse hepatic lymphangiomatosis after liver resection and rapid deterioration after liver transplantation.
Am J Gastroenterol 96: 1278-81



. Single lesions have been successfully resected 2
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Shahi KS, Geeta B, Rajput P (2009)
Solitary hepatic lymphangioma in a 22-day-old infant.
J Pediatr Surg 44: E9-11



3
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

A benign neoplasm composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33

A benign neoplasm composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.



Lip, Oral Cavity and Oropharynx
ICD-O-3 topography code: C00-C06, C09-C10, C14

A benign neoplasm composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.



Kidney
ICD-O-3 topography code: C64

Lymphangioma is a benign cavernous/cystic vascular lesion composed of dilated lymphatic channels. They constitute common paediatric lesions, which most often present at birth or during first years of life. Cavernous/cystic lymphangioma of head and neck are most common. Some lymphangiomas occur in the setting of Turner syndrome and other malformative syndromes
4
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



. Painless swelling is the usual clinical manifestation.

Lymphangioma of the kidney is rare and usually presents as a peripelvic or renal sinus mass. Renal lymphangiomas are encapsulated, diffusely cystic lesions ranging from small well-delineated tumours to large lesions that may replace the entire renal parenchyma. The cysts communicate, contain clear fluid, and are composed of fibrous septae lined by flattened endothelium
5
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



.



Esophagus
ICD-O-3 topography code: C15

A benign neoplasm composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.

Oesophageal ymphangiomas vary from small mucosal lesions to pedunculated polyps and large masses that may involve surrounding structures extending into the mediastinum. Most have occurred in older adults, arising in the middle and lower third underneath an intact mucosa, and many have been characterized as cavernous lymphangiomas. Some cystic lymphangiomas of the neck in infants have involved the upper oesophagus
6
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Scarpis M, De Monti M, Pezzotta MG, Sonnino D, Mosca D, Milani M (1998)
Endoscopic resection of esophageal lymphangioma incidentally discovered.
Diagn Ther Endosc 4: 141-7



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Pancreas
ICD-O-3 topography code: C25

A benign neoplasm composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.

A small number of lymphangiomas of the pancreas have been reported, most commonly centred in the peripancreatic tissues. These neoplasms often occur in young adults and tend to be cystic
7
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Igarashi A, Maruo Y, Ito T, Ohsawa K, Serizawa A, Yabe M, Seki K, Konno H, Nakamura S (2001)
Huge cystic lymphangioma of the pancreas: report of a case.
Surg Today 31: 743-6



8
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Sun LF, Ye HL, Zhou QY, Ding KF, Qiu PL, Deng YC, Zhang SZ, Zheng S (2009)
A giant hemolymphangioma of the pancreas in a 20-year-old girl: a report of one case and review of the literature.
World J Surg Oncol 7: 31



. Lymphangiomas should be distinguished from serous cystadenomas of the pancreas. Serous cystic neoplasms will express keratins 7, 8, 18 and 19, while lymphangiomas express CD319
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Skin
ICD-O-3 topography code: C44

/span> benign neoplasm composed of dilated lymphatic channels. Painless swelling is the usual clinical manifestation.
In the skin, two variants are distinghuished:

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