Wednesday, August 19, 2020

Lymphangioma Circumscriptum

The patient is an active 10-year-old boy who was seen today for evaluation of a vascular anomaly on his right knee that has been present since birth.  It was biopsied in March 2017 and the histologic diagnosis was "lymphatic malformation consistent with a lymphangioma." 

Occasionally, his knee will swell and he will get some bleeding spots.  He is learning boxing and that can precipitate swelling. He never has pain.  Has not had any infection.  I referred him to a pediatric dermatologist 3 years ago but they were out of network and he never went. 

The lesion measures 7 cm in diameter on the right knee.  There are areas of dark purplish pigmentation in confluent vesicles. 

Clinical photos:

Pathology: There were multiple dilated irregular thin walled vessels lined by a single layer of bland endothelial cells.

IMPRESSION:  Lymphangioma circumscriptum.  I expect there is a deep component.  The patient has no lymphedema.  Note: The new terminology for this is "Microcystic Lymphatic Malformation (Ref 4.)

Question:  Should this tumor be evaluated and if so how?  Would any therapy be better than active surveillance?


1. Lymphangioma

Alyssa Miceli, Kristen M. Stewart  In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan. 2020 Aug 12. Free Full Text.


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. The deep forms of lymphangioma include two specific well defined congenital entities: cavernous lymphangiomas and cystic hygromas. 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.

2. The Possible Role of Color Doppler and Pulse Wave Doppler Ultrasound in the Diagnosis of Cutaneous Lymphangioma Circumscriptum. Yang Sun, et. al. J Craniofac Surg. Jan/Feb 2020
: Cutaneous lymphangioma circumscriptum (CLC) is an uncommon congenital lymphatic malformation. Its dermoscopic features have been reported, however, if blood has infiltrated the lacunas, a pink hue overwhelms the lacunar structures, rendering dermoscopic features indistinguishable. In addition, dermoscopy cannot assess the subcutaneous extent of the lesion before surgery. The high-frequency array transducer Sonography with a high-frequency transducer is excellent in its resolution to evaluate skin lesions and is unaffected by infiltration of blood. Here, the authors report the use of ultrasound (color Doppler and pulse wave Doppler) for the diagnosis and management of CLC.An 18-year-old man presented 10 years previously with an asymptomatic 0.5 × 0.7 cm cluster of dark reddish vesicular lesions on his buttock. The lesions had recently increased in size and number. There was associated pachyderma. The lesions were hyperechoic and well defined on grayscale ultrasound and hypervascular on color Doppler ultrasound. Cluster cystics of lymphatic spaces were found throughout the whole dermis, especially in the papillary dermis. The lymphatic channels extended downwards to the reticular dermis and the superficial layer of the subcutaneous tissue. The pathological findings revealed lymphangiomas invading the subcutaneous tissue. The flat endothelial cells were partly positive for D2-40, which is a marker of lymphatic endothelial cells. Ultrasound was able to delineate the margins of the lesions. Diagnosis of CLC using preoperative color Doppler and pulse wave Doppler could reduce unnecessary repeated pre- and postoperative biopsies.

3. Lynphangioma Overview National Organization for Rare Disorders.

4. Micro- and Macrocystic Lymphatic Malformation. Lindsay McCormack, Krystal Jones, Jennifer T Huang. J Pediatr. 2020 Apr;219:275-276.  Free Full Text


5. Treatment of superficial vascular anomalies with topical sirolimus: A multicenter case series.  Melissa Dodds, et. al. Pediatr Dermatol. 2020 Mar;37(2):272-277.

Abstract: Background: Systemic sirolimus (rapamycin) has recently been found effective in treating complex vascular anomalies by reducing the size and associated complications. Many vascular anomalies have a cutaneous component, and thus, we sought to determine whether topical administration of sirolimus may be an effective therapy, as data on the use of topical sirolimus are limited.

Results: Eleven patients had combined venous lymphatic malformations, three had tufted angiomas, two had a lymphatic malformation, one had a venous malformation, and one had a verrucous venous malformation. All (100%) patients reported some degree of improvement and 50% of patients reported marked improvement in one or more symptoms, most commonly blebs and lymphatic drainage, and bleeding.

Conclusion: Topical sirolimus appears to be a safe and useful non-invasive therapy that is well-tolerated in the treatment of the cutaneous portion of a variety of vascular anomalies.





  1. Krystal Jones, M.D. pediatric dermatologist, Wyoming: "It does look like microcystic lymphatic malformation to me. The question is if there is a deep component and where that is. When you describe knee swelling — does the joint itself swell or just locally on top? It is hard to tell based on photos but it seems as though the LM is more proximal to the knee?

    We would probably start with ultrasound but move to MRI if there is concern it is in the knee joint. If it is in the joint space and causing a lot of symptoms I suppose we would consider oral sirolimus. If there is a macrocystic component and it is not in the joint space IR can do sclerotherapy for treatment. If it is mostly superficial and microcystic you could treat with topical sirolimus (Compounded at chemistry rx in Philadelphia).

    Based on photos it seems pretty localized and pretty superficial — starting with an ultrasound to determine depth seems reasonable and if fairly superficial I would offer topical rapamycin. It’s not a cure - will recur when they stop applying."

  2. Julianne Mann, MD, pediatric dermatologist, Hanover, NH: "Clinically, this could certainly be a microcystic lymphatic malformation. Lymphatic stains (D-240) done on the biopsy that would support this diagnosis. Would also consider verrucous hemangioma in the DDx, and far less likely but still worth considering would be an AVM with an overlying vascular stain. Although clinically this looks like it is likely limited to the skin/sub Q, I do think that imaging should be done.
    Ultrasound with Doppler for flow would rule out an underlying AVM, and then MRI w/wo contrast would help delineate size, depth, and would identify any macrocystic areas if they were present. I would probably start with compounded topical rapamycin. Pulsed dye laser may be worth considering as well. Surgical excision is obviously not a great choice given the large size and the location of the birthmark."

  3. Professor Khalifa Sharquie, Baghdad: "Yes the picture is that of lymphangioma but warty hemangioma could not be excluded as I can not see well the content of lumen.
    For the last 7 years I am using inderal tab to treat these cases and there is good response even in lymphangioma a study going to be published.
    Please try this therapy and response will be seen within short time and inform me."

  4. Prof. Bhushan KumarAugust 28, 2020

    No investigations and no treatments are effective. Surgery is disappointing because the extent is deeper. It is least likely that the malformation has gone into the knee joint. However, if a must- than only the MRI can give the required information.


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