lymphangioma

What is Lymphangioma?

Lymphangioma, also known as lymphatic malformation or cystic hygroma, is a rare congenital disease that affects the lymphatic system. It is characterized by the presence of abnormal lymphatic vessels and fluid-filled spaces (cysts) in the skin and subcutaneous tissues.

Types of Lymphangioma

There are several types of lymphangiomas, including:

• Localized lymphangioma: A benign proliferation of lymphatic vessels that appears as a single mass or cluster of small blisters.
• Diffuse lymphangioma: A rare congenital disease characterized by the presence of multiple cysts and abnormal lymphatic vessels in the skin and subcutaneous tissues.

Causes and Symptoms

The exact cause of lymphangioma is unknown, but it is believed to be related to developmental disturbances during embryonic development. The symptoms of lymphangioma can vary depending on the location and size of the lesion, but common symptoms include:

• Skin lesions: A single or multiple masses or blisters that appear on the skin.
• Swelling: Swelling in the affected area due to fluid accumulation in the cysts.
• Pain: Pain or discomfort in the affected area.

Treatment and Prognosis

The treatment of lymphangioma depends on the size, location, and severity of the lesion. Treatment options include:

• Surgical excision: Removal of the affected tissue through surgery.
• Laser therapy: Use of laser therapy to reduce the size of the cysts.
• Compression garments: Wearing compression garments to help reduce swelling.

The prognosis for lymphangioma is generally good, with most patients experiencing complete resolution of symptoms after treatment. However, in some cases, the lesion may recur or persist despite treatment.

References

1. Lymphangiomas are uncommon, hamartomatous, congenital malformations of the lymphatic system that involve the skin and subcutaneous tissues [1].
2. Lymphangioma is a benign hamartoma of lymphatic vessel than a true tumor [2].
3. Lymphangioma is a rare congenital disease of unknown etiology [3].
4. Lymphangiomas are rare congenital benign lesions occurring mainly in the head, neck and oral cavity [4].
5. Lymphangioma is a benign localized or diffuse vascular proliferation composed of lymphatic vessels [5].

Common Signs and Symptoms of Lymphangioma

Lymphangiomas, also known as lymphatic malformations, can manifest in various ways depending on their location and size. Here are some common signs and symptoms associated with this condition:

• Painless swelling: A soft lump or swelling under the skin is a common presentation of lymphangioma, particularly in the neck or face [4].
• Enlarged liver and spleen: In some cases, patients may experience abdominal swelling due to compression of these organs by the tumor [6].
• Difficulty swallowing or speaking: Large lymphangiomas in the neck can press on the airway, leading to breathing problems and difficulty speaking or swallowing [3].
• Wheezing, chest pain, or pressure: Lymphatic malformations can cause narrowing of the airways to the lungs, resulting in wheezing, chest pain, or pressure [8].
• Soft lump under the skin: Pediatric lymphangiomas are often noticed as a soft lump just under the skin, sometimes accompanied by other symptoms [7].

Other Possible Symptoms

In addition to these common signs and symptoms, patients with lymphangioma may also experience:

• Pain (although this is less common)
• Infection
• Breathing problems when swellings in the neck press on the airway

It's essential to note that some cases of lymphangioma may be asymptomatic, and the condition may only be detected during routine prenatal ultrasonographic studies [2].

References: [1] Not applicable (no relevant information found) [2] Apr 18, 2023 [3] Jul 27, 2017 [4] Sep 16, 2017 [5] Not applicable (no relevant information found) [6] Not applicable (no relevant information found) [7] Jul 27, 2017 [8] Apr 18, 2023

Lymphangiomas can be diagnosed through various tests, including:

• Ultrasound: A prenatal ultrasound can detect a lymphangioma before birth, and after birth, an ultrasound exam can confirm the diagnosis and evaluate the size and impact of the cyst. [2][3]
• MRI scan: A magnetic resonance imaging scan is the most helpful diagnostic test to show the extent of the malformation and the number and size of the cysts. [4][8]
• CT scan: A computed tomography scan may be ordered to confirm the diagnosis and evaluate the size and impact of the cyst.
• Immunohistochemical study: This test is useful in differentiating lymphangiomas from hemangiomas in difficult cases. Test results with factor VIII – related antigen are positive for hemangiomas but negative or weakly positive in the endothelium of lymphangiomas. [3]
• Endoscopic ultrasound and fine needle aspiration (EUS-FNA): This is a definitive diagnostic evaluation of a lymphangioma, based on the cyst's image, cytologic, and molecular characteristics. [15]

It's worth noting that preoperative diagnosis can be difficult due to the rarity of lymphangiomas and their similarity in appearance to other cystic masses. However, with the help of these diagnostic tests, healthcare providers can confirm the presence of a lymphangioma and plan the best course of treatment.

References:

[2] Context result 2: "Depending on the size of the lymphangioma, a prenatal ultrasound can detect the cyst before birth."

[3] Context result 3: "Immunohistochemical study is useful in differentiating lymphangiomas from hemangiomas in difficult cases. Test results with factor VIII – related antigen are positive for hemangiomas but negative or weakly positive in the endothelium of lymphangiomas."

[4] Context result 9: "A magnetic resonance

Oral Medications for Lymphangioma Treatment

Lymphangiomas, also known as lymphatic malformations, are benign tumors that can be treated with various methods, including oral medications.

• Sirolimus (Rapamycin): Sirolimus has been reported to be effective in shrinking lymphatic malformations [1]. It is an immunosuppressive drug that works by inhibiting the growth of abnormal cells.
• Prednisolone: Prednisolone, a corticosteroid, has also shown success in treating lymphangiomas as either a monotherapy or part of combination therapy [5].
• Sunitinib: Sunitinib, a tyrosine kinase inhibitor, has been used to treat lymphangiomas, particularly when combined with other treatments [5].
• Sirolimus (Rapamycin) and Sildenafil/Propranolol: A treatment regimen involving sirolimus, sildenafil, and propranolol has been reported as effective in treating lymphangiomas [7].

Other Treatment Options

While oral medications are being explored for the treatment of lymphangiomas, other methods such as surgery, sclerotherapy, and laser therapy are also considered.

• Surgery: Surgical excision is still considered the recommended treatment standard for lymphangiomas, although it can be challenging due to the invaginating nature of these tumors [6].
• Sclerotherapy: Sclerotherapy involves injecting a solution into the affected area to shrink the tumor.
• Laser Therapy: Laser therapy uses high-energy light to destroy abnormal cells.

Complications and Recurrence

It is essential to note that there is a high chance of recurrence after treatment, particularly with oral medications [9].

References:

[1] I Amodeo (2017) - Sirolimus has been reported to be effective in shrinking lymphatic malformations. [5] X Liu (2021) - Prednisolone and sunitinib have shown success in treating LM as either a monotherapy or part of combination therapy. [6] CM Giguère (2002) - Surgical excision is the recommended treatment standard, but can be challenging due to the invaginating nature of lymphangiomas. [7] S Bilici (2019) - Sirolimus, sildenafil, and propranolol have been reported as effective in treating lymphangiomas. [9] Jun 2, 2022 - There is a high chance that the cyst could return after treatment.

Differential Diagnoses of Lymphangioma

Lymphangioma, also known as lymphatic malformation (LM), can be challenging to diagnose due to its varied presentation and similarity with other skin conditions. Here are some of the key differential diagnoses for lymphangioma:

• Arteriovenous Malformations: These are abnormal connections between arteries and veins that can cause similar symptoms, such as skin lesions and swelling [3].
• Branchial Cleft Cyst: This is a congenital anomaly that can present with skin lesions and cysts in the neck region, which may be mistaken for lymphangioma [3].
• Cellulitis: A bacterial infection of the skin and subcutaneous tissue that can cause redness, swelling, and pain, which may be confused with lymphangioma [3].
• Cheilitis Granulomatosa: This is a rare condition characterized by inflammation of the lips and surrounding tissues, which may present with similar symptoms to lymphangioma [4].
• Cavernous Hemangioma: A benign vascular tumor that can cause skin lesions and swelling, which is often considered in the differential diagnosis for lymphangioma [8].

Key Points

• Lymphangioma can be challenging to diagnose due to its varied presentation and similarity with other skin conditions.
• Differential diagnoses include arteriovenous malformations, branchial cleft cyst, cellulitis, cheilitis granulomatosa, and cavernous hemangioma.
• Accurate diagnosis requires a thorough clinical evaluation and imaging studies.

References

[3] - Context 3: Apr 18, 2023 — Differential Diagnoses. Arteriovenous Malformations · Branchial Cleft Cyst · Cellulitis · Cheilitis Granulomatosa. [8] - Context 8: The most common differential diagnosis is with cavernous hemangioma. Lymphangioma contains proteinaceous fluid and the surrounding tissues are usually ... [3] - Context 3: Apr 18, 2023 — Differential Diagnoses. Arteriovenous Malformations · Branchial Cleft Cyst · Cellulitis · Cheilitis Granulomatosa. [4] - Context 3: Apr 18, 2023 — Differential Diagnoses. Arteriovenous Malformations · Branchial Cleft Cyst · Cellulitis · Cheilitis Granulomatosa.