Lymphangioma - 淋巴管瘤
淋巴管瘤 (Lymphangioma) 是以薄壁囊腫病灶為特徵的淋巴系統畸形。此類畸形可發生於任何年齡，且可能累及身體任何部位，但 90% 發生在 2 歲以下的兒童，主要涉及頭頸部。後天性淋巴管瘤可能因創傷、發炎或淋巴阻塞而產生。因為淋巴管瘤不會惡化為惡性腫瘤，通常僅因美觀需求而接受治療。
relevance score : -100.0%
ReferencesLymphangioma 也稱為淋巴管畸形 (LM)。這是一種先天性血管疾病，特徵是出生前後淋巴組織異常增生。Lymphangioma 的發生率約為 1/2000 至 1/4000 人，性別與種族無顯著差異。大多數病例（80‑90%）在兩歲之前即被診斷。臨床表現多樣，從局部腫脹到廣泛的淋巴管異常，有時會出現嚴重腫脹，稱為像皮病。例如，頸部和臉部的 lymphangioma 會導致面部腫脹，嚴重時可能造成毀容。當它影響舌頭時，可能導致下頜過度生長與牙齒錯位。口腔和頸部受累時，可能引起呼吸困難，甚至危及生命的緊急情況。眼部受累可導致視力喪失、眼球運動受限、眼瞼下垂與眼球突出。四肢受累則會造成軟組織與骨骼腫脹及異常生長。此腫瘤通常生長緩慢，但感染、荷爾蒙變化或外傷可能促使其快速增大，增加危及生命的風險，需緊急處理。
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.
在這項回顧性研究中，我們回顧了 1999 年 1 月至 2004 年 12 月期間，24 名患有 lymphangioma 並接受 bleomycin(博來黴素)溶液注射治療的兒童。結果顯示，84% 獲得良好反應，16% 未獲得良好反應。兩名患者的腫瘤後來復發，另有兩名患者在注射時出現膿瘍。幸運的是，我們未觀察到其他重大問題或副作用。
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 的主要類型包括：lymphangioma circumscriptum、cavernous lymphangioma、cystic hygroma、lymphangioendothelioma。這些腫瘤約佔兒童良性血管腫瘤的 26%，在成人中較少見。 其中最常見的類型是 lymphangioma circumscriptum，表現為淋巴管穿過皮膚突出，形成類似青蛙卵的透明充液囊泡，伴隨組織腫脹。它常出現在淋巴網絡豐富的區域，如四肢、軀幹和腋窩。 一位 71 歲的女性來診，她的腿部持續腫脹，生殖器出現粉紅色腫塊，伴有搔癢與淋巴液滲漏。我們以雙側大陰唇切除術切除所有腫塊，止於科爾斯筋膜水平，並保留陰蒂。
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.
