Lymphangioma - Linfangioma
Linfangioma (Lymphangioma) sono malformazioni del sistema linfatico caratterizzate da lesioni costituite da cisti a parete sottile. Queste malformazioni possono comparire a qualsiasi età e coinvolgere qualsiasi parte del corpo, ma il 90 % si manifesta nei bambini di età inferiore a 2 anni, interessando soprattutto testa e collo. I linfangiomi sono spesso diagnosticati prima della nascita mediante ecografia fetale. I linfangiomi acquisiti possono derivare da traumi, infiammazioni o ostruzione linfatica. Poiché non hanno alcuna possibilità di trasformarsi in maligni, vengono solitamente trattati solo per ragioni estetiche.
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relevance score : -100.0%
ReferencesLymphangioma è anche conosciuta come malformazione linfatica (LM). È un disturbo vascolare presente dalla nascita e si caratterizza per una crescita anormale del tessuto linfatico, sia prenatale che postnatale. Lymphangioma colpisce circa 1 persona su 2.000‑4.000, senza differenze significative tra i sessi o le etnie. La maggior parte dei casi (80‑90 %) viene diagnosticata prima dei due anni. I sintomi variano ampiamente, da un gonfiore localizzato a estese anomalie nei canali linfatici, che talvolta portano a un grave gonfiore noto come elefantiasi. Per esempio, un lymphangioma al collo o al viso può causare gonfiore facciale e, nei casi più gravi, deformità. Quando coinvolge la lingua, può provocare una crescita eccessiva della mascella e il disallineamento dei denti. Nella bocca e nel collo può determinare problemi respiratori e situazioni potenzialmente letali. Negli occhi, può causare perdita della vista, limitazione dei movimenti oculari, ptosi palpebrale e protrusione degli occhi. Il coinvolgimento degli arti può provocare gonfiore e crescita anormale di tessuti e ossa. Questo tumore di solito cresce lentamente, ma infezioni, cambiamenti ormonali o lesioni possono indurre una crescita rapida, con rischi mortali che richiedono un trattamento urgente.
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.
In questo studio retrospettivo abbiamo esaminato 24 bambini affetti da lymphangioma, trattati con iniezioni di soluzione di bleomicina dal gennaio 1999 al dicembre 2004. La maggior parte delle lesioni (63 %) è scomparsa completamente; il 21 % ha mostrato una buona risposta e il 16 % non ha risposto adeguatamente. Due pazienti hanno avuto una recidiva del tumore in seguito, e altri due hanno sviluppato ascessi nel sito di iniezione. Fortunatamente non sono stati riscontrati altri problemi rilevanti o effetti collaterali.
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.
I principali tipi di linfangioma sono i seguenti: lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Questi costituiscono circa il 26 % dei tumori vascolari benigni nei bambini, ma sono meno comuni negli adulti. Lymphangioma circumscriptum, il tipo più frequente, presenta dotti linfatici che sporgono attraverso la pelle, formando vescicole trasparenti piene di liquido, simili a uova di rana, accompagnate da edema dei tessuti. Compare tipicamente su aree con una ricca rete linfatica, come gli arti, il tronco e le ascelle. Una donna di 71 anni si è presentata alla nostra clinica con gonfiore persistente alle gambe, protuberanze rosa sui genitali, prurito e perdita di liquido linfatico. Abbiamo rimosso chirurgicamente tutte le protuberanze con una procedura chiamata labiectomia maggiore bilaterale, intervenendo fino alla fascia di Colles e preservando il clitoride e la forchetta clitoridea.
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.
