Soft tissue

Vascular

Benign

Cystic / cavernous lymphangioma


Editorial Board Member: Jose G. Mantilla, M.D.
Deputy Editor-in-Chief: Borislav A. Alexiev, M.D.
Laura Warmke, M.D.
Jeanne Meis, M.D.

Last author update: 29 November 2022
Last staff update: 29 November 2022

Copyright: 2002-2023, PathologyOutlines.com, Inc.

PubMed Search: Cystic / cavernous lymphangioma

Laura Warmke, M.D.
Jeanne Meis, M.D.
Page views in 2022: 14,871
Page views in 2023 to date: 1,620
Cite this page: Warmke L, Meis J. Cystic / cavernous lymphangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuelymphangiomacystic.html. Accessed February 3rd, 2023.
Definition / general
  • Benign vascular lesion composed of a collection of dilated lymphatic channels that may be superficial, deep or diffusely involve organ systems
  • First described by Rodender in 1828 (Arch Pathol Lab Med 2015;139:278)
Essential features
  • Benign proliferation of lymphatic vessels
  • Immunohistochemical expression of CD31 and D2-40
Terminology
  • Lymphatic malformation
  • Lymphangioma circumscriptum (superficial cutaneous lymphangioma)
  • Cavernous lymphangioma (deep lymphangioma)
  • Cystic hygroma (cystic lymphangioma)
  • Intra-abdominal cystic lymphangioma
  • Hemangiolymphangioma
  • Lymphangiomatosis (generalized lymphangioma, systemic angiomatosis)
ICD coding
  • ICD-O:
  • ICD-11:
    • LA90.12 & XH9MR8 - lymphatic malformations of certain specified sites & lymphangioma, NOS
    • 2E81.10 - disseminated lymphangiomatosis
Epidemiology
Sites
Pathophysiology
Etiology
  • Early or congenital lesions are favored to be developmental malformations
  • Sequestered lymphatics fail to communicate with normal lymphovascular system
  • Most are considered to be malformations / hamartomas (not true neoplasms)
  • Genetic abnormalities play a role (Virchows Arch 2008;453:1)
Clinical features
  • Superficial lesions may present as multiple small, grouped vesicular lesions involving the skin
  • Tongue lesions present as a mass surfaced by pebbly, vesicle-like nodules said to resemble frog eggs (Head Neck Pathol 2020;14:512)
  • Cystic hygroma usually presents as a unilateral, diffuse, nonpulsatile, painless swelling of the posterior cervical triangle
  • Deeper lesions may present as a large, slow growing painless mass
  • Intra-abdominal lesions can displace organs and cause intestinal obstruction
Diagnosis
  • Swollen soft tissue mass with positive transillumination test
  • Ultrasound can confirm cystic or multicystic lesion
  • Diagnosing these lesions may be very challenging in small biopsies given their cystic nature and bland cytologic features
Radiology description
  • Xray:
  • Ultrasound:
    • Unilocular or multilocular anechoic mass
    • Sharply defined cystic of multicystic mass with internal septations
    • May be diagnosed on prenatal ultrasound (Prenat Diagn 1988;8:405)
  • CT findings:
    • Nonenhancing cystic lesions
    • Septated cystic mass of variable size (Pediatr Radiol 2002;32:88)
    • Contains fluid of homogeneous density
    • May displace adjacent organs
  • MRI findings:
    • T1 weighted image may be hypointense or hyperintense when filled with hemorrhage or proteinaceous material (Magn Reson Imaging 2003;21:81)
    • T2 weighted image shows a multiloculated mass with hyperintense areas
Radiology images

Contributed by Jeanne Meis, M.D.

Radiograph of left lower extremity

Angiogram of
lymphangiomatosis

MRI of left extremity

MRI of lower abdomen

Radiograph of lower extremity


MRI of mediastinal lymphangioma

Radiograph of mediastinal lymphangioma

Arteriogram of lymphangioma



Images hosted on other servers:

CT of splenic lymphangioma

CT of orbital lymphangioma

Ultrasound of mesenteric lymphangioma

Prognostic factors
  • Benign lesions with excellent prognosis
  • Recurrence is high with incomplete removal
  • When involving deeper tissue planes, lesions can recur in up to 20% of patients (J Pediatr Surg 1992;27:220)
  • Complications include infection, hemorrhage, rupture and intestinal obstruction
  • Diffuse lymphangiomatosis with mediastinal or visceral organ involvement can be fatal
  • No malignant transformation has been reported
Case reports
Treatment
  • Surgical resection may be indicated for large, symptomatic lesions
  • Intralesional injection of sclerosing agents, including bleomycin and OK-432
  • Radiofrequency ablation (Int J Pediatr Otorhinolaryngol 2008;72:953)
Clinical images

Contributed by Jeanne Meis, M.D.

Lower extremity



Images hosted on other servers:

Cystic hygroma of neck

Lymphangioma of dorsal tongue

Omentum: 30 cm tumor in 4 year old girl


Scrotal tumor with hemorrhage in 3 year old boy

Abdominal cyst: 25 cm

Broad ligament: 19 kg tumor in 70 year old woman

Mesentery: 1 year old boy


Thigh tumor

Mesenteric tumor: 12 year old girl

Mesenteric tumors


Mesenteric tumors

Gross description
  • Multicystic and spongy lesions
  • Reddish / brown translucent cystic mass
  • Cystic spaces often contain watery, thick or milky fluid (Hum Pathol 2005;36:426)
Gross images

Contributed by Jeanne Meis, M.D.

Resection of lymphangiomatosis

Mediastinal lymphangioma



Images hosted on other servers:

Splenic lymphangioma

Microscopic (histologic) description
  • Variably sized, thin walled, dilated lymphatic vessels lined by flattened endothelium
  • Frequently surrounded by lymphoid aggregates, sometimes with reactive germinal centers
  • Lumina may contain eosinophilic and amorphous proteinaceous fluid with occasional lipid laden macrophages and lymphocytes
  • Longstanding lesions may show interstitial fibrosis
  • Walls of larger vessels may contain smooth muscle
  • Stromal mast cells and hemosiderin deposits are frequently seen
  • Lining of the cysts may rarely form papillary projections
  • Lymphangiomatosis frequently shows an anastomosing growth pattern, dissecting around normal structures
  • Extensive granulation tissue and inflammation may obscure lymphatic nature (Hum Pathol 2005;36:426)
Microscopic (histologic) images

Contributed by Laura Warmke, M.D. and Jeanne Meis, M.D.
Dilated lymphatic spaces

Dilated lymphatic spaces

Focal areas of fibrosis

Focal areas of fibrosis

Proteinaceous fluid Proteinaceous fluid

Proteinaceous fluid

Lymphatic channels

Lymphatic channels

Retroperitoneal lymphanagioma

Retroperitoneal lymphanagioma


Retroperitoneal lymphanagioma Retroperitoneal lymphanagioma

Retroperitoneal lymphanagioma

Lymphangioma circumscriptum

Lymphangioma circumscriptum

Lymphangioma of tongue Lymphangioma of tongue Lymphangioma of tongue

Lymphangioma of tongue


Superficial lymphangioma of vulva Superficial lymphangioma of vulva

Superficial lymphangioma of vulva

Superficial lymphangioma

Superficial lymphangioma

Scattered macrophages

Scattered macrophages

Lymphoid aggregate Lymphoid aggregate

Lymphoid aggregate


Lymphoid aggregates Lymphoid aggregates

Lymphoid aggregates

Calcification

Calcification

Thick wall

Thick wall

Endothelial lining

Endothelial lining

Irregular vascular channels

Irregular vascular channels


Irregular vascular channels

Irregular vascular channels

Trichrome stain

Trichrome stain

D2-40

D2-40

CD31

CD31

Cytokeratin

Cytokeratin

Virtual slides

Images hosted on other servers:

Lymphangioma involving adrenal gland

Buccal lymphangioma

Positive stains
Negative stains
Electron microscopy description
  • May assist in determining endothelial origin of cells lining the cysts
  • Presence of Weibel-Palade bodies, storage granules of endothelial cells
Molecular / cytogenetics description
Videos

Lymphangioma (cystic hygroma)

Sample pathology report
  • Soft tissue, retroperitoneal mass, resection:
    • Cavernous lymphangioma (see comment)
    • Comment: Sections show a lesion composed of dilated lymphatic spaces lined by flattened endothelium without cytologic atypia. Immunohistochemical stains show that the endothelial cells are positive for D2-40 and CD31, supporting a lymphatic nature, while they are negative for cytokeratin. These results, along with the morphologic features, support the above diagnosis.
Differential diagnosis
Board review style question #1

Cystic hygroma (cystic lymphangioma), which frequently involves the posterior triangle of the neck, is associated with which of the following syndromes?

  1. Down syndrome
  2. Klippel-Trenaunay syndrome
  3. Maffucci syndrome
  4. Turner syndrome
Board review style answer #1
D. Turner syndrome

Comment Here

Reference: Cystic / cavernous lymphangioma
Board review style question #2

Which of the following immunohistochemical stains, when positive, is the most helpful in making the diagnosis of lymphangioma?

  1. Calretinin
  2. Cytokeratin
  3. D2-40
  4. GLUT1
Board review style answer #2
Back to top
Image 01 Image 02