title: Lymphangiomatosis: Two cases with unique presentations, salience of nomenclature, and diagnosis.
abstract: We are presenting two unique cases of lymphangiomatosis without visceral and bony involvement and critically discussing the nomenclature used in the extant literature. The first case was a 12-year-old boy with ill-defined mass on the right cheek extending into the ipsilateral orbit leading to conjunctival lesion. The second case was of a 14-week-old infant showing diffuse swelling on nape of the neck. In addition there were raised patches on dorsal aspects of bilateral hands and feet. The biopsies from all the lesions showed similar histopathological features consistent with lymphangiomatosis. We propose that the term lymphangiomatosis should be used only in cases with histological features of lymphangiomatosis. The term should not be used in cases of multiple lymphangiomas. We conclude that the lesions clinically diagnosed as lymphangioma may turn out to be lymphangiomatosis. Extensive lymphangiomatosis without visceral or bony involvement may lead to intrauterine death.
authors.affiliation: Department of Pathology, Dr. Rajender Prasad Government Medical College, Tanda, Himachal Pradesh, India.
authors.forename: Bal
authors.initials: B
authors.lastname: Chander
authors.forename: Sunder Singh
authors.initials: SS
authors.lastname: Dogra
authors.forename: Rashmi
authors.initials: R
authors.lastname: Kaul
authors.forename: Kamal
authors.initials: K
authors.lastname: Preet
authors.forename: Renu
authors.initials: R
authors.lastname: Sharma
authors.forename: Narvir Singh
authors.initials: NS
authors.lastname: Chauhan
mesh_terms: D001706:Biopsy; D002648:Child; D006801:Humans; D007223:Infant; D008202:Lymphangioma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D014057:Tomography, X-Ray Computed