Lymphangioma cavernosum - Altmeyers Encyclopedia

Synonym(s)

cystic lymphangioma; Hygroma cysticum; lymphangioma cysticum

Definition
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The lymphatic malformations affecting the dermatological specialty are mostly subcutaneously or cutaneously-subcutaneously localized lymphatic malformations(lymphangioma), which consist of a bundle of large-volume, cystically expanded lymphatic vascular spaces

The other cavernous lymphatic malformations concern different specialties. They are diagnosed as non-symptomatic (tumor masses) and affect different localizations such as mediastinum, ovaries, kidneys, genitals, etc.

Manifestation
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From birth or in early infancy.

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Localization
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Ubiquitous on skin and mucous membranes, especially in the neck, head and extremities.

Clinical features
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Skin-coloured, more rarely bluish (caused by haemorrhaging), cushion-like, succulent, usually blurred, painless tumour of varying size. Depending on the size of the lymphangioma, elephantiasis, macrochelia, macroglossia are possible.

Histology
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Differently dilated and calibrated lymph vessels with optically empty lumina. Transitions between thin- and thick-walled, muscularly dilated vessel walls, lymphocytic infiltrate and lymph follicles.
Immunohistology: Endothelia pos. for CD31.

Diagnosis
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Clinic, isotope lymphography.

Differential diagnosis
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Deep-seated infantile hemangioma; lymphangioma-like variant of angiosarcoma; lymphangioma-like Kaposi sarcoma.

Therapy
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Surgical removal if necessary.

Literature
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Bieniasz A et al (1961) A case of lymphangioma cavernosum of the ovary.ginekol Pol 32:667-669.
Chirino-Romo J et al (2014) Unusual diagnosis of an intrathoracic tumor, cavernosum lymphangioma. Case report. Circle 82:537-540.
Khanna S et al (1978) Lymphangioma cavernosum of the ovary. Int Surgery 63:104-105.
Malik NM (1951) Congenital lymphangioma cavernosum. (Report of a case). Indian J Surge 13:82-84.
Rodríguez Reina G et al (1998) Intrascrotal lymphangioma: report of 2 cases and review of the literature. Actas Urol Esp 22:55-59.