Solitary intrapulmonary cystic lymphangioma in an infant: a case report with literature review.
Lymphangioma rarely presents as a solitary pulmonary lesion. We encountered a case of solitary cystic lymphangioma and present its clinicopathologic and immunohistochemical findings. A 2-month-old boy was referred to the hospital after developing a persistent cough. Chest X-ray showed a large cyst in the right lung. Under the preoperative diagnosis of bronchogenic cyst, he underwent right lower lobectomy at the age of 11 months. The resected specimen contained a 5.5-cm septate cystic lesion. Microscopically, the lesion consisted of a large cystic space and interconnected slit-like spaces surrounding bronchovascular islands. The cyst was lined by a monolayer of flat cells with focal multinucleated giant cells. Immunohistochemically, the cells lining the cystic lesion were positive for D2-40, Prox1, CD34, and CD31, and weakly positive for VEGFR-3, but were negative for AE1/3, HMB45, VEGF-A, VFGF-C, VEGFR-1. Differential diagnoses included lobar or interstitial emphysema, bronchogenic cyst, congenital pulmonary airway malformation and alveolar adenoma. D2-40 and Prox1 were useful in differentiation and in determining the extent of the lesion. A review of the literature found only 15 cases of solitary pulmonary lymphangioma. In younger patients, the lesions tend to occupy more of the lung. Focal giant cell reaction has not been described in the reported papers.
Minato
Hiroshi
H
Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Kahoku, Ishikawa, Japan. hminato@kanazawa-med.ac.jp
Kaji
Sachiko
S
Kinoshita
Eriko
E
Kurose
Nozomu
N
Nojima
Takayuki
T
Kohno
Miyuki
M
Konuma
Kunio
K
Ikawa
Hiromichi
H
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018952:Antigens, CD34; D001994:Bronchogenic Cyst / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D003371:Cough; D003937:Diagnosis, Differential; D015726:Giant Cells / Q000473:pathology; D018398:Homeodomain Proteins; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D018191:Lymphangioma, Cystic / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D011656:Pulmonary Emphysema / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed; D025521:Tumor Suppressor Proteins; D000098551:Prospero-Related Homeobox 1 Protein
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018952:Antigens, CD34; D018398:Homeodomain Proteins; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D025521:Tumor Suppressor Proteins; C500987:monoclonal antibody D2-40; D000098551:Prospero-Related Homeobox 1 Protein