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cystic lymphangioma
Description
What is Cystic Lymphangioma?
Cystic lymphangioma, also known as cystic hygroma, is a rare congenital malformation of the lymphatic system [1][2]. It is characterized by the formation of large macrocystic lymphatic channels with protein-rich fluid [9].
Characteristics and Symptoms
- Cystic lymphangiomas can occur in any part of the body, but most commonly affect the head and neck region [1][3].
- They are usually benign and do not have a family history [1].
- The symptoms due to local compression can occur related to the location and size of the tumor [3].
- Large cystic hygromas of the neck can cause infection, as well as more serious complications such as respiratory distress or difficulty swallowing [13].
Types and Variations
- Cystic lymphangioma is a type of benign vascular lesion composed of dilated lymphatic channels that may be superficial, deep, or diffusely involving organ systems [14].
- It can also be referred to as cystic hygroma, familial nuchal bleb, fetal cystic hygroma (FCH), or hygroma colli [15].
Prevalence and Demographics
- Cystic lymphangioma is a rare condition that mostly affects children under 2 years of age [2][3].
- In adults, it is rarely seen, but can occur in any part of the body [3].
- It occurs more frequently in women [8].
References:
[1] - Search result 1 [2] - Search result 2 [3] - Search result 3 [9] - Search result 9 [14] - Search result 14 [15] - Search result 15
Additional Characteristics
- Cystic lymphangioma
- cystic hygroma
- benign congenital malformation of the lymphatic system
- large macrocystic lymphatic channels with protein-rich fluid
- benign vascular lesion composed of dilated lymphatic channels
- superficial, deep, or diffusely involving organ systems
Signs and Symptoms
Symptoms of Cystic Lymphangioma
Cystic lymphangiomas, also known as cystic hygromas or lymphangiomas, can vary significantly in size and presentation, leading to a range of symptoms. Here are some common signs and symptoms associated with this condition:
- Soft, fluid-filled cysts: Cystic lymphangiomas typically appear as soft, fluid-filled cysts that can be several centimeters in size.
- Asymptomatic or discomforting: These cysts may be asymptomatic or cause considerable discomfort depending on their location and size.
- Swelling and overgrowth: Overgrowth and swelling in affected areas, such as the lips, tongue, jaws, cheeks, arms, legs, fingers, or toes, can occur.
- Redness, pain, and fever: Infection of the cyst can lead to redness, pain, and possibly fever.
- Painless, ill-defined subcutaneous swelling: Cavernous lymphangioma typically presents during infancy as a painless, ill-defined subcutaneous swelling with no changes of the overlying skin.
Variations in Symptoms
Symptoms of cystic lymphangioma can vary based on the size and location of the cyst. Some common variations include:
- Neck mass: A neck mass found at birth or discovered later in an infant after an upper respiratory tract infection.
- Head or neck involvement: Three-quarters of cystic lymphangioma tumors are found on the head or neck, which can lead to specific symptoms such as swelling and overgrowth.
Associated Symptoms
Additional symptoms associated with cystic lymphangioma may include:
- Pruritus (itching)
- Pain
- Burning
- Lymphatic drainage issues
- Infection
- Aesthetic concerns
It's essential to note that the severity of the condition and the associated features can vary from person to person. If you suspect a cystic lymphangioma, it's crucial to consult with a medical professional for proper diagnosis and treatment.
References:
[6] A fluid-filled bump that forms on a baby's neck caused by a blockage in the lymphatic system pathways. [11] Cystic hygroma (cystic lymphangioma): A red to blue swollen, fluid-filled mass often found on the neck, groin or armpit. [12] Cystic Lymphangioma Signs and Symptoms [13] Associated symptoms may include pruritus, pain, burning, lymphatic drainage, infection, and aesthetic concerns. [14] Cystic lymphangiomas can vary significantly in size and presentation. They may be asymptomatic or cause considerable discomfort depending on their location and size. [15] The severity of the condition and the associated features vary from person to person.
Diagnostic Tests
Cystic lymphangioma, also known as cystic hygroma, can be diagnosed through various diagnostic tests.
- Prenatal ultrasound: A prenatal ultrasound can detect the cyst before birth, especially if it is large enough to be visible on an ultrasound scan [1].
- Physical examination: After birth, a healthcare provider will examine the cyst and may perform a physical examination to confirm its presence.
- Imaging studies: Imaging studies such as ultrasound, CT scans, and MRI scans can help confirm the diagnosis of cystic lymphangioma. These tests can also help assess the size and location of the cyst [3], [6].
- Dermoscopy and biopsy: In some cases, dermoscopy and biopsy may be used to confirm the diagnosis and rule out other conditions such as squamous cell carcinoma or lymphangiosarcoma [4].
- Incisional biopsy: Incisional biopsy for histological examination is considered the most definitive diagnostic tool for oral lymphangiomas [7].
It's worth noting that a combination of these tests may be used to confirm the diagnosis and rule out other conditions.
References: [1] - Cystic hygroma (cystic lymphangioma): A red to blue swollen, fluid-filled mass often found on the neck, groin or armpit. ... Diagnosis and Tests. [3] - A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. [4] - 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. [6] - MRI is an essential means of differentiating lymphangioma from other cystic lesions due to its excellent spatial resolution. [7] - Incisional biopsy for histological examination remains the most definitive diagnostic tool for oral lymphangiomas.
Treatment
Treatment Options for Cystic Lymphangioma
Cystic lymphangioma, also known as cystic hygroma, can be treated with various methods, including drug treatment. While surgery is often the preferred option, certain medications have been used to manage this condition.
- Sclerotherapy: This involves injecting a solution into the affected area to shrink or close off the abnormal lymphatic channels. Sclerotherapy has been shown to be effective in treating macrocystic lymphangiomas, with studies suggesting that it can reduce symptoms and improve quality of life [4][9].
- Cryotherapy: Another minimally invasive treatment option is cryotherapy, which involves freezing the affected tissue to destroy abnormal cells. However, its effectiveness for cystic lymphangioma is still being researched.
- Radiofrequency therapy: This technique uses heat generated by radio waves to destroy abnormal tissue. It has been used in some cases of cystic lymphangioma, but more research is needed to confirm its efficacy.
Limitations and Considerations
While these drug treatments may offer relief for some patients, it's essential to note that they are not suitable for everyone. The effectiveness of these methods can vary depending on the size, location, and type of lymphangioma, as well as individual patient factors [2][6].
In some cases, surgery remains the best option for treating cystic lymphangioma, especially when symptoms are severe or the condition is causing significant distress.
References
[4] by G Fasching · 2022 — Sclerotherapy with Ok-432 is recommended primary for macrocystic lymphangiomas. The application is safe and without serious side effects. It can be used alone ...
[9] by MT Rahimi · 2024 · Cited by 2 — Sclerotherapy with ethanol 98% seems to be an efficient, safe, and cost-effective treatment for macrocystic lymphangiomas affecting the neck.
Note: The above information is based on a summary of relevant search results.
Recommended Medications
- Cryotherapy
- Sclerotherapy
- Radiofrequency therapy
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Cystic Lymphangioma
Cystic lymphangiomas, also known as cavernous lymphangiomas, are benign vascular lesions that can be challenging to diagnose due to their similarity in appearance with other cystic-like tumors. The differential diagnosis for cystic lymphangioma includes:
- Hemangioma: A well-circumscribed lesion with smaller vascular spaces extensively filled with red blood cells [12].
- Pseudocysts: Can be distinguished from cystic lymphangiomas based on their imaging characteristics, such as the presence of septa, cystic or wall calcification, soft tissue, wall thickness, single or multiple loculation, and dilatation of the pancreas [7][8].
- Cystadenoma: A type of congenital cyst that can be distinguished from cystic lymphangiomas based on their imaging characteristics and histopathology [6][9].
- Pseudocyst: Can be distinguished from cystic lymphangiomas based on their imaging characteristics, such as the presence of septa, cystic or wall calcification, soft tissue, wall thickness, single or multiple loculation, and dilatation of the pancreas [3][7].
- Cystic metastasis: A type of metastatic tumor that can be distinguished from cystic lymphangiomas based on their imaging characteristics and histopathology [5].
- Echinococcal cysts: A type of parasitic cyst that can be distinguished from cystic lymphangiomas based on their imaging characteristics and histopathology [5].
- Mesenchymal tumors: A type of tumor that can be distinguished from cystic lymphangiomas based on their imaging characteristics and histopathology [5].
It is essential to consider the location or site of origin and imaging features when formulating a differential diagnosis for a cystic-appearing lesion in the abdomen or pelvis. Other diagnostic hypotheses should also be considered, such as lipoma, branchial cleft cyst, thyroglossal duct cyst, tumors of major salivary gland, hematoma, carotid body tumors, soft tissue sarcomas, and thyroid masses [15].
References:
[1] Cystic lymphangiomas arise in areas such as neck and axilla, where loose connective tissue allows expansion of the endothelial-lined channels; cavernous lymphangiomas develop in the mouth, lips, cheek, tongue or other areas where dense connective tissue and muscles prevent expansion.
[2] Lymphangioma circumscriptum may resemble warts, metastatic carcinoma of the skin, lymphangiectasis, herpes simplex, and herpes zoster.
[3] Differential diagnosis of cystic lymphangiomas includes pseudocysts, which can be distinguished based on their imaging characteristics.
[4] Cystic / cavernous lymphangioma is a benign vascular lesion composed of a collection of dilated lymphatic channels that may be superficial, deep or diffusely involving organ systems.
[5] Differential diagnosis for cystic lymphangiomas includes cystic metastasis, echinococcal cysts, and mesenchymal tumors.
[6] Cystadenoma is a type of congenital cyst that can be distinguished from cystic lymphangiomas based on their imaging characteristics and histopathology.
[7] Pseudocysts can be distinguished from cystic lymphangiomas based on their imaging characteristics, such as the presence of septa, cystic or wall calcification, soft tissue, wall thickness, single or multiple loculation, and dilatation of the pancreas.
[8] Cystadenoma is a type of congenital cyst that can be distinguished from cystic lymphangiomas based on their imaging characteristics and histopathology.
[9] Pseudocysts can be distinguished from cystic lymphangiomas based on their imaging characteristics, such as the presence of septa, cystic or wall calcification, soft tissue, wall thickness, single or multiple loculation, and dilatation of the pancreas.
[10] Cystic / cavernous lymphangioma is a benign vascular lesion composed of a collection of dilated lymphatic channels that may be superficial, deep or diffusely involving organ systems.
[11] Large cystic hygromas of the neck can cause infection, as well more serious complications.
[12] Hemangioma: A well-circumscribed lesion with smaller vascular spaces extensively filled with red blood cells.
[13] This review describes the epidemiology, histopathology, biomedicine, clinical manifestations, radiological features, differential diagnosis, and management of this lesion.
[14] Formulate a differential diagnosis for a cystic-appearing lesion in the abdomen or pelvis, considering its location or site of origin and imaging features.
[15] On patient evaluation, various diagnostic hypotheses should be considered such as lipoma, branchial cleft cyst, thyroglossal duct cyst, tumors of major salivary gland, hematoma, carotid body tumors, soft tissue sarcomas and thyroid masses.
Additional Differential Diagnoses
- benign peritoneal mesothelioma
- appendix adenocarcinoma
- Mesenchymal tumors
- Pseudocysts
- Cystic metastasis
- Echinococcal cysts
- Pseudocyst
- hemangioma
- cystadenoma
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