Unveiling lymphangiomas: Radiologic insights from head to toe

INTRODUCTION

Lymphangiomas are benign vascular lesions with lymphatic differentiation, occurring in both children and adults. About 95% are located in the neck and axilla, while 5% occur in the mesentery, retroperitoneum, abdominal organs, lungs, or mediastinum.^[1]

Lymphangiomatosis, a rare multifocal lymphatic proliferation, usually presents in childhood and involves multiple organs, including the lungs, liver, spleen, bones, and skin. Clinical and radiological features vary from localised swelling to extensive infiltrative abnormalities causing elephantiasis.^[2] Cervicofacial lymphangiomas may lead to disfigurement or airway obstruction, and orbital lesions can impair vision.^[3] Extremity lesions may cause swelling or gigantism with osseous overgrowth.^[4] Though typically slow-growing, rapid enlargement may occur due to infection, hormonal changes, or trauma.^[5] Grossly, lymphangiomas are thin-walled cystic masses—macrocystic (cystic hygroma) or microcystic (cavernous)—containing chylous, serous, or haemorrhagic fluid. Histologically, they consist of dilated lymphatic spaces lined by endothelial cells, filled with protein-rich eosinophilic fluid, and surrounded by collagenous stroma with lymphoid aggregates.^[6]

Ultrasonography (USG), computed tomography (CT), magnetic resonance imaging (MRI), and lymphoscintigraphy are key diagnostic tools. Ultrasound, the first-line modality, shows multilocular cystic lesions with variable septa and predominantly anechoic contents, occasionally hyperechoic due to debris, lipid, infection, or haemorrhage. Solid areas or mixed cystic-solid patterns may also be seen. Doppler may reveal septal vascularity. Besides characterisation, ultrasound is valuable for evaluating the lesion’s size and extent—particularly in superficial cases—and for guiding interventional procedures such as aspiration or sclerotherapy. CT offers detailed anatomy, showing cystic masses with possible wall or septal enhancement, and homogeneous low attenuation, occasionally negative in chylous cases. MRI best defines lesion extent and internal architecture, showing fluid-like T1 hypointense and T2 hyperintense signals, with variations from haemorrhage or infection. The purpose of this essay is to describe and illustrate the imaging features of lymphangiomas at various sites.

Imaging features of lymphangioma at various locations Orbital lymphangioma

Orbital lymphangiomas are rare, predominantly paediatric lesions with infiltrative, trans-spatial growth involving intraconal, extraconal, and pre-/post-septal compartments. Ultrasonography, though radiation-free, has limited posterior orbital penetration and complements MRI and CT.^[7] These unencapsulated lesions contain microcysts and macrocysts; CT may show phleboliths, bony changes, or hyperdense components. MRI best defines anatomical extent, vascular elements, and fluid–fluid levels. Typically, lesions appear isointense to mildly hyperintense on T1 and hyperintense on T2, with septations and variable signals reflecting blood product age [Figure 1].^[8]

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Figure 1: Magnetic resonance imaging fat-sat axial images show a multicystic lesion in the extraconal compartment of the right orbit, which is (a) Hyperintense on T2WI (arrow), (b) Isointense on T1WI (arrow) and, (c) Shows mild enhancement on post-contrast T1WI (arrow). T1WI: T1-weighted image, T2WI: T2-weighted image.

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Cervical lymphangioma

Nearly 90% of lymphangiomas occur in patients under two years of age. Most cervical lesions are unilateral, without gender or side predilection. In children, they often involve the tongue, epiglottis, oropharynx, hypopharynx, and parapharyngeal spaces. Differential diagnoses include branchial cleft cysts, dermoid cysts, and lymph node neoplasms. Ultrasound evaluates superficial lesions, while CT better defines deep or thoracic extensions.^[9] CT typically shows cystic lesions with an “enveloping effect” around neck structures with attenuation values varying by cystic content. MRI offers superior delineation, showing heterogeneous T1 hypointensity and T2 hyperintensity, varying with protein content or haemorrhage [Figures 2 and 3].^[10]

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Figure 2: (a-c) Magnetic resonance imaging - axial images show a large multiseptated cystic lesion (thin arrows) in the left cervical region, which is hyperintense on (a) STIR (thick arrow), (b) T1WI (thick arrow) and, (c) The wall and septations (thick arrow) are showing enhancement on post-contrast T1WI. An ill-defined interface with the left submandibular gland is seen. T1WI: T1-weighted image, STIR: Short tau inversion recovery.

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Figure 3: (a) Grey scale and (b) Color doppler ultrasound images of an 18-year-old male who presented with neck swelling show a cystic lesion with internal echoes (arrow), septations and soft tissue (arrow with notched tail), which is showing vascularity on colour doppler (short arrow). Postoperative histopathology findings were suggestive of cavernous lymphangioma.

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Chest wall lymphangioma

Chest wall lymphangioma is rare, mainly affecting children and young adults. It usually presents as a large, smooth, cystic, non-tender mass, though atypical features like absent cysts, spiculated margins, or calcifications may mimic other conditions such as necrotic tumours, hematomas, or infections. Ultrasonography aids preoperative evaluation, while CT and MRI assess intrathoracic extension; CT helps surgical planning near major vessels, and MRI defines airway, visceral, or bony involvement [Figure 4].^[11]

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Figure 4: (a and b) Grey scale and (c) Color doppler ultrasound images of a 26-year-old male presenting with a swelling in the chest wall shows a multiloculated cystic lesion with internal echoes and septations (arrow). The lesion was not showing vascularity on colour doppler interrogation. “Rib” labels indicate the underlying osseous structures used as anatomical landmarks.

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Breast lymphangioma

Breast lymphangiomas are extremely rare, typically arising in the upper outer quadrant, tail of Spence, or subareolar region. Mammography shows diffuse or localised dense, lobulated masses without calcification. Ultrasound reveals multiloculated cystic lesions with septations and low-level internal echoes suggestive of haemorrhage or proteinaceous content. MRI shows well-defined multilocular or unilocular cystic lesions, sometimes extending to the axillary tail, appearing T1 hypointense and T2 hyperintense, with variable signals in haemorrhage or inflammation [Figure 5].^[12,13]

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Figure 5: (a and b) Grey scale and (c) Color doppler ultrasound images of a 32-year-old woman presenting with a breast lump shows an intramammary multiloculated cystic lesion with internal echoes (thin arrow), septations (short arrow) and vascularity on colour doppler.

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Adrenal lymphangioma

Adrenal lymphangiomas are extremely rare (~0.06% prevalence), usually detected between the 3rd and 6th decades. They appear as cystic, lobulated adrenal masses (~3 cm) with fluid attenuation, thin enhancing walls, sometimes associated with thick curvilinear or scattered punctate septal calcifications. Internal septations may enhance post-contrast. Despite accurate imaging characterisation, differentials include other adrenal cysts, cortical tumours, and pheochromocytomas [Figure 6].^[14,15]

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Figure 6: Ultrasound image of a 36-year-old male (a) Shows a large unilocular anechoic cystic lesion (arrow) superior to the kidney (K); Computed tomography in (b) Axial and (c) Sagittal plane reveals a large, homogeneous, low-attenuation cystic mass with thin mural calcification (thick arrow) in the suprarenal region (arrow) displacing the right kidney (K).

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Renal lymphangioma

Renal lymphangiomas are rare lesions from dilated or abnormal lymphatic channels, presenting as focal cysts or diffuse renal lymphangiomatosis involving perirenal and peripelvic regions. CT and ultrasound show thin-walled, septated cysts, sometimes irregular or calcified. MRI reveals septated masses with low T1 and high T2 signals, varying with protein or haemorrhage. Differentials include benign renal cysts, cystic renal cell carcinoma, cystic nephroma, and abscesses [Figure 7].^[16]

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Figure 7: (a-d) NCCT axial image shows a large, well-defined, multiloculated, low-attenuation cystic lesion with internal fine septations (thin arrow) in the right perinephric space, compressing the adjacent right kidney (K). The lesion is not showing enhancement on CECT. Coronal and sagittal CECT images better demonstrate the extent of the cystic lesion encasing the renal capsule and extending along the perinephric fascia. The lesion was causing compression of the distal ureter, leading to gross hydroureteronephrosis with enhancing residual renal parenchyma (thick arrow). NCCT: Non-contrast computed tomography, CECT: Contrast-enhanced computed tomography.

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Splenic lymphangioma

Primary splenic lymphangiomas are rare, occurring alone or with systemic or hepatosplenic lymphangiomatosis. They typically arise subcapsularly, appearing as thin-walled cysts or diffuse infiltrative lesions causing splenomegaly, sometimes with mural or septal calcifications. Differentials include haemangioma, infarction, embolism, lymphoma, and metastasis. Rarely, they associate with Klippel-Trenaunay syndrome, showing vascular-lymphatic malformations and tissue hypertrophy [Figures 8 and 9].^[17]

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Figure 8: NCCT abdomen image (a) Shows a thin-walled, multiloculated low attenuation cystic lesion (arrow) on the anterosuperior aspect of the spleen (S), (b and c) Did not show enhancement on the CECT scan (arrow). NCCT: Non-contrast computed tomography, CECT: Contract-enhanced computed tomography, LK: Left kidney.

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Figure 9: (a) NCCT images, (b and c) CECT images in sagittal and coronal plane shows a large, homogenous low attenuation non-enhancing cystic lesion (thin arrows) with few foci of wall calcifications (thick arrow) arising from the spleen (S), smooth splenic contour expansion and mass effect over adjacent organs. NCCT: Non-contrast computed tomography, CECT: Contrast-enhanced computed tomography, K: Kidney.

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Mesenteric lymphangioma

Mesenteric lymphangiomas are the most common abdominal lymphatic malformations and may cause haemorrhage, ischemia, obstruction, or volvulus. Ultrasound shows multilocular anechoic cystic masses with possible echogenic debris; CT reveals homogeneous low-attenuation fluid, while MRI shows T1 hypointense and T2 hyperintense fluid-like signals [Figure 10].

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Figure 10: Imaging features of mesenteric lymphangioma in a 22-year-old male patient. (a) Barium meal follow-through examination revealed downward displacement of small bowel loops and extrinsic compression (thin arrow), (b) NCCT image, and (c-e) CECT images demonstrate a well-defined, multiloculated cystic mass (arrows) with mildly enhancing thin septations (arrows with black outline) in the mesentery, causing bowel displacement towards the right side and inferiorly. NCCT: Non-contrast computed tomography, CECT: Contrast-enhanced computed tomography.

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Retroperitoneal lymphangioma

Retroperitoneal lymphatic malformations are rare, accounting for <1% of abdominal cases, and usually occur in older children and adults. They may present as an abdominal mass, pain, obstruction, or haematuria, or be incidental. Imaging resembles mesenteric lymphangiomas, with a tendency to extend across multiple compartments. Retroperitoneal cystic teratoma is a key differential [Figures 11 and 12].

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Figure 11: (a) NCCT images, (b and c) CECT images of the abdomen demonstrating a thin-walled, low attenuation non-enhancing cystic lesion (arrows) in the retroperitoneal plane in the left pelvic region. The lesion is abutting left iliac vessel medially (thick arrow). NCCT: Non-contrast computed tomography, CECT: Contrast-enhanced computed tomography.

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Figure 12: Contrast-enhanced computed tomography images in (a) Axial and (b) Coronal planes show a thin-walled, low attenuation non-enhancing cystic lesion (arrows) in the left para-aortic retroperitoneum. Ao: Aorta and LK: Left kidney.

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Pelvic lymphangioma

Pelvic lymphangiomas may be isolated or linked to syndromes like Klippel-Trénaunay-Weber. Usually asymptomatic, they can cause compressive symptoms or palpable masses, with pain or fever from haemorrhage, rupture, or infection. CT shows uni- or multilocular thin-walled cystic spaces, rarely calcified. MRI reveals low T1, high T2 signals, minimal enhancement, and trans-compartmental spread, distinguishing them from other cystic pelvic masses [Figure 13].

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Figure 13: (a-f) Magnetic resonance imaging of the pelvis in a 19-year-old male demonstrating a huge multiloculated cystic lesion (arrows) involving the pelvic, perineal and left gluteal region. The lesion is hypointense on T1WI hyperintense on T2WI and STIR and shows fluid-fluid levels (arrow with black outline). Post contrast T1WI images shows mural and septal enhancement. T1WI: T1-weighted image, T2WI: T2-weighted image, STIR: Short tau inversion recovery.

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TAKE HOME MESSAGE

Lymphangiomas are rare benign lymphatic malformations with variable presentations depending on location. Imaging is crucial for diagnosis and differentiation from other lesions. Ultrasound, CT, and MRI reveal their cystic nature, extent, and features, enabling accurate assessment and guiding optimal management and treatment decisions.