Lymphangioma


Article Author:
Alyssa Miceli


Article Editor:
Kristen Stewart


Editors In Chief:
Amanda Oakley
Jules Lipoff
Shyam Verma


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
5/14/2019 3:02:48 PM

Introduction

Lymphangiomas are uncommon, benign malformations of the lymphatic system that can occur anywhere on the skin and mucous membranes. Lymphangiomas can be categorized as deep or superficial based on the depth and size of the abnormal lymphatic vessels or as congenital or acquired.[1]

The deep forms of lymphangioma include two specific well defined congenital entities: cavernous lymphangiomas and cystic hygromas. [2] Superficial forms of lymphangioma include lymphangioma circumscriptum and acquired lymphangioma, which is also referred to in the literature as lymphangiectasia. Although both entities share similar clinical and histologic features, the term lymphangioma circumscriptum infers lymphatic channel dilation due to a congenital malformation of the lymphatic system. Whereas, the term lymphangiectasia, or acquired lymphangioma, denotes dilated lymphatic channels of previously normal lymphatics that have become obstructed by an external cause.

Etiology

Congenital lymphangiomas form due to blockage of the lymphatic system during fetal development, though the cause remains unknown. Cystic lymphangiomas are associated with genetic disorders, including trisomies 13, 18, and 21, Noonan syndrome, Turner syndrome, and Down syndrome.[3] Acquired lymphangioma circumscriptum occurs in association with chronic lymphedema that leads to disruption of previously normal lymphatic channels.

Epidemiology

Lymphangiomas are rare in the United States. They represent 4% of all vascular tumors and approximately 25% of all benign pediatric vascular tumors. There is no racial or gender predilection. Lymphangiomas typically present at birth or in the first few years of life, while the acquired form of cutaneous lymphangioma circumscriptum often presents in adulthood.[4]

Pathophysiology

Lymphangiomas result from congenital or acquired abnormalities of the lymphatic system. The congenital form typically occurs before the age of 5 years and is due to improper connection of lymphatic channels to the main lymphatic drainage duct.   Acquired lymphangiomas occur as a sequela of any interruption of previously normal lymphatic drainage such as surgery, trauma, malignancy, and radiation therapy.

Histopathology

On histopathologic examination, lesions of superficial lymphangioma consist of a collection of large lymphatic cisterns lying deep in the subcutaneous plane that communicate via dilated dermal lymphatic channels lined with endothelial cells. The overlying epidermis is usually acanthotic or hyperkeratotic and has an irregular elongation of rete pegs. No atypical vascular features, nuclear atypia, mitotic activity, or koilocytic changes typically exist. A mild to the moderate inflammatory infiltrate may be present.[5] Cystic or cavernous lymphangiomas demonstrate large, irregular vascular spaces lined with a single layer of flattened endothelial cells within a fibroblastic or collagenous stroma, which may contain lymphocytes. Penetration of muscle may be seen.

History and Physical

Clinically, lymphangioma circumscriptum appears as multiple, grouped or scattered, translucent or hemorrhagic vesicular papules that resemble frog-spawn. Because the lesions consist of a combination of blood and lymph elements, purple areas can be seen scattered within the vesicle-like papules. In the genital area, the surface can be verrucous, and the lesions may be mistaken for warts. The acquired form is most often found in the axilla, inguinal, and genital areas and there is often coexisting lymphedema. Associated symptoms may include pruritus, pain, burning, lymphatic drainage, infection, and aesthetic concerns.

Cavernous lymphangioma typically presents during infancy as a painless, ill-defined subcutaneous swelling with no changes of the overlying skin that can be several centimeters in size. Rarely, an entire extremity may be affected. Patients may report tenderness upon deep palpation of the area. [6]They are commonly mistaken for cysts or lipomas in clinical practice.  

Cystic hygromas are lymphatic malformations that are clinically more circumscribed than cavernous lymphangioma and typically occur on the neck, axilla, or groin. On physical examination, they are soft, with varying sizes and shapes, and will typically grow if not surgically excised.  When posterior neck lesions are present, there may be an association with Turner syndrome, hydrops fetalis, or other congenital abnormalities.  These lesions can be visualized in utero with the use of transabdominal or transvaginal sonography. MRI can be useful in determining the extent of anatomical involvement of cystic or cavernous lymphangiomas.

Evaluation

In most cases, a clinical diagnosis can be made based on the history and examination findings. As needed, dermoscopy and biopsy can be used to confirm the diagnosis and imaging may be warranted to assess the depth and extent of a lesion.

The dermoscopic examination can be helpful in distinguishing superficial lymphangioma from other cutaneous lesions. Two distinct dermoscopic patterns have been described: yellow lacunae surrounded by pale septa without the inclusion of blood and yellow to pink lacunae alternating with dark-red or bluish lacunae, representing the inclusion of blood. A more recently described dermoscopic finding is a “hypopyon-like” feature which consists of a color transition from dark to light in some lacunae. This occurs due to sedimentation of blood that separates cellular components to the bottom and serum to the upper part of the lacunae.[7]

Treatment / Management

Both superficial and deep lymphangiomas can be difficult to treat. When feasible the treatment of choice for any type of lymphangioma, however, remains surgical excision. Wide local excision of the affected lymphatic channels is necessary as recurrence is common. Recurrence rates for surgical excision of lymphangioma circumscriptum have been reported to be as high 23% in follow-up periods up to 81 months. Surgical success rates are higher for small, superficial lymphangiomas.[8]

Destructive treatments with carbon dioxide (CO2) laser, long-pulsed Nd-YAG laser, and electrosurgery have been reported to improve symptoms. Cryotherapy, superficial radiotherapy, and sclerotherapy with 23.4% hypertonic saline are less commonly used modalities. Direct injection of a sclerosing agent, including 1% or 3% sodium tetradecyl sulfate, doxycycline or ethanol, can be made into lymphatic malformations. Compression may reduce swelling caused by lymphedema. Infection prevention is crucial.[9]

Pearls and Other Issues

The most common complications associated with lymphangioma circumscriptum include cellulitis and lymphatic fluid leakage. Rare cases of squamous cell carcinoma, verruciform xanthoma, and lymphangiosarcoma arising within lymphangioma have been reported. Large cystic hygromas of the neck can cause infection, as well more serious issues including dysphagia and respiratory problems.[10] [11]

Acquired lymphangiomas are not believed to have malignant potential; however, associated chronic lymphedema places the patient at risk for lymphangiosarcoma, which is an aggressive tumor with a dismal prognosis. Cutaneous angiosarcoma has also been reported in massive localized lymphedema in morbidly obese patients.

Acquired lymphangiectasia can be painful, and poor lymphatic drainage may lead to bacterial infection.

Enhancing Healthcare Team Outcomes

Lymphangiomas are benign lesions that are difficult to diagnose and manage. These lesions are best managed by a multidisciplinary team that includes a pediatrician, surgeon, dermatologist, and the primary care provider. While the treatment of choice is surgical excision, this is not always possible. Recurrence rates in excess of 30% have been reported. In addition, surgery can be associated with complications like lymphatic leaks, fistula formation and chronic wounds. These patients need a wound care nurse as the healing time is often many weeks or months. Parents should be educated about the treatment options because not all are effective or consistently effective.

 

 

 


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Lymphangioma - Questions

Take a quiz of the questions on this article.

Take Quiz
What is the usual treatment for an acquired progressive lymphangioma appearing on the thigh of a 17-year-old male?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Following a complete history and physical examination, as well as an MRI, which of the following is the most important clinical study to aid in the diagnosis of lymphangioma?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 15-year-old female presents with increased snoring and difficulty articulating, which she attributes to her tongue. She states that her tongue has slowly enlarged over the last 5 weeks. Her tongue appears to be enlarged, although it does not protrude from her mouth and has no visually obvious lesions. She denies trauma to the tongue. Given her presentation, which of the following is the most likely etiology for her enlarging tongue?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 44-year-old white female presents with a 5-month history of numerous small bumps on the vulvae bilaterally with occasional oozing of clear fluid. She reports an extensive surgical history, including bilateral medial thighplasty 6 months prior, as well as a history of abdominoplasty, cholecystectomy, sleeve gastrectomy, hemorrhoidectomy, and two cesarean sections. What is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 14-year-old female presents with numerous persistent clear vesicles clustered on her abdomen that increased in size and number after puberty. What is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Lymphangioma - References

References

Stewart CJ,Chan T,Platten M, Acquired lymphangiectasia ('lymphangioma circumscriptum') of the vulva: a report of eight cases. Pathology. 2009;     [PubMed]
Noia G,Maltese PE,Zampino G,D'Errico M,Cammalleri V,Convertini P,Marceddu G,Mueller M,Guerri G,Bertelli M, Cystic Hygroma: A Preliminary Genetic Study and a Short Review from the Literature. Lymphatic research and biology. 2018 Nov 22;     [PubMed]
Sehgal VN,Sharma S,Chatterjee K,Khurana A,Malhotra S, Unilateral, Blaschkoid, Large Lymphangioma Circumscriptum: Micro- and Macrocystic Manifestations. Skinmed. 2018;     [PubMed]
Ersoy AO,Oztas E,Saridogan E,Ozler S,Danisman N, An Unusual Origin of Fetal Lymphangioma Filling Right Axilla. Journal of clinical and diagnostic research : JCDR. 2016 Mar;     [PubMed]
Hara H,Mihara M,Anan T,Fukumoto T,Narushima M,Iida T,Koshima I, Pathological Investigation of Acquired Lymphangiectasia Accompanied by Lower Limb Lymphedema: Lymphocyte Infiltration in the Dermis and Epidermis. Lymphatic research and biology. 2016 Sep;     [PubMed]
Jiao-Ling L,Hai-Ying W,Wei Z,Jin-Rong L,Kun-Shan C,Qian F, Treatment and prognosis of fetal lymphangioma. European journal of obstetrics, gynecology, and reproductive biology. 2018 Dec;     [PubMed]
Zaballos P,Del Pozo LJ,Argenziano G,Karaarslan IK,Landi C,Vera A,Llambrich A,Medina C,Bañuls J, Dermoscopy of lymphangioma circumscriptum: A morphological study of 45 cases. The Australasian journal of dermatology. 2018 Aug;     [PubMed]
Vignes S,Arrault M,Trévidic P, Surgical resection of vulva lymphoedema circumscriptum. Journal of plastic, reconstructive     [PubMed]
Farnoosh S,Don D,Koempel J,Panossian A,Anselmo D,Stanley P, Efficacy of doxycycline and sodium tetradecyl sulfate sclerotherapy in pediatric head and neck lymphatic malformations. International journal of pediatric otorhinolaryngology. 2015 Jun;     [PubMed]
Wilson GR,Cox NH,McLean NR,Scott D, Squamous cell carcinoma arising within congenital lymphangioma circumscriptum. The British journal of dermatology. 1993 Sep;     [PubMed]
Sims SM,McLean FW,Davis JD,Morgan LS,Wilkinson EJ, Vulvar lymphangioma circumscriptum: a report of 3 cases, 2 associated with vulvar carcinoma and 1 with hidradenitis suppurativa. Journal of lower genital tract disease. 2010 Jul;     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Dermatology. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Dermatology, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Dermatology, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Dermatology. When it is time for the Dermatology board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Dermatology.