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