Lymphangioma - 림프종
림프종 (Lymphangioma) 은 벽이 얇은 낭종 병변을 특징으로 하는 림프계 기형입니다. 이러한 기형은 모든 연령에서 발생할 수 있으며 신체의 모든 부위에 영향을 미칠 수 있지만 90%는 2세 미만 어린이에게 발생하며 머리와 목에 발생합니다. 림프관종은 일반적으로 태아 초음파 검사를 통해 출생 전에 진단됩니다. 후천성 림프관종은 외상, 염증 또는 림프관 폐쇄로 인해 발생할 수 있습니다. 림프관종은 악성으로 발전할 가능성이 없기 때문에 일반적으로 미용적인 목적으로만 치료됩니다.
References
Lymphangioma 은 림프기형 (LM) 으로도 알려져 있습니다. 이는 태어날 때부터 나타나는 혈관 장애입니다. 출생 전후 림프 조직의 비정상적인 성장이 특징입니다. Lymphangioma 는 2000~4000명 중 약 1명에게 영향을 미치며 성별이나 인종 간에 큰 차이는 없습니다. 대부분의 경우 (80~90%) 는 2세 이전에 진단됩니다. 증상은 국소적인 부종부터 림프관의 광범위한 이상까지 매우 다양하며 때로는 상피병으로 알려진 심각한 부종으로 이어집니다. 예를 들어, 목과 얼굴의 lymphangioma 은 안면 부종을 유발할 수 있으며, 심한 경우에는 외모의 변형을 가져올 수 있습니다. 혀에 영향을 미치면 턱이 과도하게 성장하고 치아가 어긋나게 될 수 있습니다. 입과 목에 발생하면 호흡 문제와 생명을 위협하는 응급 상황이 발생할 수 있습니다. 눈에서는 시력 상실, 안구 운동 제한, 눈꺼풀 처짐, 눈 돌출 등을 유발할 수 있습니다. 사지 침범은 조직과 뼈의 부기와 비정상적인 성장을 유발할 수 있습니다. 이 종양은 일반적으로 천천히 자라지만 감염, 호르몬 변화 또는 부상으로 인해 급속히 성장하여 긴급 치료가 필요한 생명을 위협하는 위험을 초래할 수 있습니다.
Lymphangioma (lymphatic malformation, LM), a congenital vascular disease, is a low-flow vascular abnormality in lymphatic diseases that is characterized by excessive growth of lymphatic tissue during prenatal and postpartum development. The incidence rate of LM is ~1:2000–4000, with no variation between genders and races. Most patients (80–90%) are diagnosed before the age of two. The clinical manifestations of lymphangioma are quite different among patients, varying from local swelling leading to superficial mass to a large area of diffuse infiltrating lymphatic channel abnormalities resulting in elephantiasis. Cervicofacial LM can cause facial elephantiasis, and in some severe cases, it can lead to serious disfigurement of the face. Tongue LM can lead to mandibular overgrowth and occlusal asymmetry, and oral and cervical LM can cause obstructive acute respiratory distress and life-threatening situations. Orbital LM may lead to decreased vision, decreased extraocular muscle movement, ptosis and exophthalmos. LM of the extremities can trigger swelling or gigantism, accompanied by overgrowth of soft tissue and bones. LM usually grows slowly and steadily, but under certain conditions, such as infection, hormonal changes or trauma, it can grow explosively and become a life-threatening disease requiring immediate treatment.
Lymphangioma (lymphatic malformation, LM), a congenital vascular disease, is a low-flow vascular abnormality in lymphatic diseases that is characterized by excessive growth of lymphatic tissue during prenatal and postpartum development. The incidence rate of LM is ~1:2000–4000, with no variation between genders and races. Most patients (80–90%) are diagnosed before the age of two. The clinical manifestations of lymphangioma are quite different among patients, varying from local swelling leading to superficial mass to a large area of diffuse infiltrating lymphatic channel abnormalities resulting in elephantiasis. Cervicofacial LM can cause facial elephantiasis, and in some severe cases, it can lead to serious disfigurement of the face. Tongue LM can lead to mandibular overgrowth and occlusal asymmetry, and oral and cervical LM can cause obstructive acute respiratory distress and life-threatening situations. Orbital LM may lead to decreased vision, decreased extraocular muscle movement, ptosis and exophthalmos. LM of the extremities can trigger swelling or gigantism, accompanied by overgrowth of soft tissue and bones. LM usually grows slowly and steadily, but under certain conditions, such as infection, hormonal changes or trauma, it can grow explosively and become a life-threatening disease requiring immediate treatment.
본 후향적 연구에서는 1999년 1월부터 2004년 12월까지 lymphangioma 을 앓고 블레오마이신 용액 주사 치료를 받은 24명의 어린이를 검토했습니다. 대부분의 병변 (63%) 이 완전히 사라졌고, 21%는 좋은 반응을 얻었고, 16%는 잘 반응하지 않았습니다. 두 명의 환자는 나중에 종양이 재발했고, 다른 두 명은 주사를 맞은 부위에 농양이 생겼습니다. 다행히 다른 큰 문제나 부작용은 발견되지 않았습니다.
This is a retrospective study of 24 children diagnosed with lymphangioma and treated with intralesional injection of bleomycin aqueous solution from January 1999 to December 2004. Complete resolution was seen in 63% (15/24) of lesions, 21% (5/24) had good response and 16% (4/24) had poor response. The tumour recurred in 2 patients. Two other patients had abscess formation at the site of injection. No other serious complications or side effects were observed.
This is a retrospective study of 24 children diagnosed with lymphangioma and treated with intralesional injection of bleomycin aqueous solution from January 1999 to December 2004. Complete resolution was seen in 63% (15/24) of lesions, 21% (5/24) had good response and 16% (4/24) had poor response. The tumour recurred in 2 patients. Two other patients had abscess formation at the site of injection. No other serious complications or side effects were observed.
Lymphangioma 의 주요 유형은 다음과 같습니다 - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. 이는 어린이 양성 혈관 종양의 약 26%를 차지하지만 성인에서는 덜 흔합니다. 가장 흔한 유형인 lymphangioma circumscriptum 은 림프관이 피부를 통해 돌출되어 개구리 알과 유사한 투명한 액체로 채워진 소포를 형성하고 조직 부종을 나타냅니다. 일반적으로 팔다리, 몸통, 겨드랑이와 같이 림프 네트워크가 풍부한 부위에 나타납니다. 71세 여성이 지속적인 다리 부기, 생식기의 분홍색 돌기, 가려움증, 림프액 누출로 우리 병원에 왔습니다. 우리는 음핵과 포체트를 보존하면서 콜스의 근막 수준에서 멈추는 양측 주요 음순절제술이라는 절차를 통해 모든 돌기를 외과적으로 제거했습니다.
The predominant types of lymphangioma are lymphangioma circumscriptum (LC), cavernous lymphangioma, cystic hygroma, and lymphangioendothelioma. These entities account for approximately 26% of benign vascular tumors in children but are rarer in adults. LC is the most common form of cutaneous lymphangioma and is characterized by superficial lymphatic ducts protruding through the epidermis. This condition results in clusters of clear fluid-filled vesicles resembling frog spawn and associated tissue edema. It is usually found on the proximal extremity, trunk, and axilla, which has an abundant lymphatic system. A 71-year-old female presented to our outpatient clinic with persistent edema of both lower limbs, clusters of pink labial papules, pruritus, and watery lymph oozing. We removed all the papules by performing bilateral major labiectomy down to the level of Colles' fascia, sparing the clitoris and fourchette.
The predominant types of lymphangioma are lymphangioma circumscriptum (LC), cavernous lymphangioma, cystic hygroma, and lymphangioendothelioma. These entities account for approximately 26% of benign vascular tumors in children but are rarer in adults. LC is the most common form of cutaneous lymphangioma and is characterized by superficial lymphatic ducts protruding through the epidermis. This condition results in clusters of clear fluid-filled vesicles resembling frog spawn and associated tissue edema. It is usually found on the proximal extremity, trunk, and axilla, which has an abundant lymphatic system. A 71-year-old female presented to our outpatient clinic with persistent edema of both lower limbs, clusters of pink labial papules, pruritus, and watery lymph oozing. We removed all the papules by performing bilateral major labiectomy down to the level of Colles' fascia, sparing the clitoris and fourchette.
