Lymphangioma - リンパ管腫
リンパ管腫 (Lymphangioma) は、薄壁の嚢胞である病変を特徴とするリンパ系の奇形です。これらの奇形はあらゆる年齢で発生する可能性があり、体のどの部分にも発生する可能性がありますが、90% は 2 歳未満の小児に発生し、頭頸部に発生します。リンパ管腫は通常、胎児超音波検査を使用して出生前に診断されます。後天性リンパ管腫は、外傷、炎症、またはリンパ管閉塞によって生じる可能性があります。悪性化する可能性がないため、リンパ管腫は通常、美容上の理由のみで治療されます。
References
Lymphangioma はリンパ奇形 (LM) としても知られています。生まれつきの血管障害です。出生前後のリンパ組織の異常な増殖が特徴です。 Lymphangioma は約 2000 ～ 4000 人に 1 人が罹患しており、性別や人種間に大きな差はありません。ほとんどの症例 (80 ～ 90%) は 2 歳までに診断されます。症状は局所的な腫れからリンパ管の広範囲にわたる異常まで多岐にわたり、時には象皮病として知られる重度の腫れにつながることもあります。たとえば、首や顔の lymphangioma は顔の腫れを引き起こし、重度の場合は外観を損なう可能性があります。舌に影響を与えると、顎の過成長や歯並びの乱れを引き起こす可能性があります。口や首に発生すると、呼吸障害や生命を脅かす緊急事態を引き起こす可能性があります。目には、視力の低下、目の動きの制限、まぶたの垂れ、目の突出を引き起こす可能性があります。四肢の炎症は、組織や骨の腫れや異常な成長を引き起こす可能性があります。この腫瘍は通常ゆっくりと成長しますが、感染症、ホルモンの変化、または損傷によって急速に成長する可能性があり、緊急の治療が必要な生命を脅かすリスクが生じます。
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.
この後ろ向き研究では、1999 年 1 月から 2004 年 12 月までに lymphangioma を患い、ブレオマイシン溶液の注射による治療を受けた 24 人の子供を調査しました。病変の大部分 (63%) は完全に消失し、21% は良好な反応を得、16% は良好な反応を得ました。うまく反応しませんでした。 2人の患者は後に腫瘍が再発し、他の2人は注射を受けた場所に膿瘍ができた。幸いなことに、他に大きな問題や副作用は見られませんでした。
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 の主な種類は次のとおりです - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma 。これらは小児の良性血管腫瘍の約 26% を占めますが、成人ではあまり一般的ではありません。 lymphangioma circumscriptum は最も頻度の高いタイプで、皮膚から突き出たリンパ管が見られ、カエルの卵に似た透明な液体で満たされた小胞が形成され、組織が腫れています。通常、手足、体幹、脇の下など、リンパネットワークが豊富な領域に発生します。 71歳の女性が、持続する脚の腫れ、性器のピンク色の隆起、かゆみ、リンパ液の漏出を訴えて来院しました。私たちは、クリトリスと四シェットを温存しながら、コーレス筋膜レベルで停止する両側大陰唇切除術と呼ばれる手順ですべての隆起を外科的に除去しました。
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.
