J Korean Med Sci.  2010 Sep;25(9):1387-1389. 10.3346/jkms.2010.25.9.1387.

The First Korean Case of Cutaneous Lung Tissue Heterotopia

• Jeon GW
• Han SW
• Jung JM
• Kang MS
• Sin JB

• KMID: 1785924
• DOI: http://doi.org/10.3346/jkms.2010.25.9.1387

Abstract

Cutaneous lung tissue heterotopia is a very rare disorder where mature lung tissues develop in the skin. This is only the second known report of cutaneous lung tissue heterotopia, with the first by Singer et al. in 1998. A newborn infant had a hemangioma-like, freely movable mass connected to the anterior aspect of the sternal manubrium. Pathologic findings showed mature lung tissues with bronchi, bronchioles, and alveoli through the dermis and subcutis, and it was diagnosed as cutaneous lung tissue heterotopia. Cutaneous lung tissue heterotopia is hypervascular, so grossly it looks like a hemangioma. It can be differentiated from pulmonary sequestration, teratoma, bronchogenic cyst, and branchial cleft cyst by histology and the location of the mass. We describe the clinical, radiologic, and pathologic findings of a cutaneous lung tissue heterotopia, the first reported in Korea.

Keyword

Choristoma; Bronchogenic Cyst; Branchioma; Skin

MeSH Terms

Branchioma/pathology/surgery
Bronchogenic Cyst/pathology/surgery
Choristoma/*pathology/surgery
Humans
Infant, Newborn
*Lung
Magnetic Resonance Imaging
Male
Republic of Korea
Skin Diseases/*pathology/surgery
Skin Neoplasms/pathology
Tomography, X-Ray Computed

Figure

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  Fig. 1 Reddish brown hamangioma-like mass on the sternal manubrium. (A, B) 4.2×3.5×1.0 cm hemangioma-like protruding mass was connected to the middle of sternal manubrium by a stalk. The surface of the mass was wrinkled and vessel-like tissue was visible under the surface. The mass was freely movable and soft.

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  Fig. 2 Chest CT images. (A) Chest CT showed the mass was lobulated, hypervascular, and highly enhanced, and contained two arteries and veins (arrow) that cross the middle of the sternal manubrium. (B) These vessels were connected to the internal mammary arteries and veins (arrow). A similar 1.1×2.3 cm highly enhancing mass (arrowhead) was located in the anterior mediastinum.

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  Fig. 3 Histologic findings. (A) Lobulated lung parenchymal tissue (arrow) and a dilated bronchus (arrowhead) with mature cartilages are seen in dermis and subcutis (H&E, ×40). (B) Lung parenchymal tissue is composed of bronchioles lined by columnar epithelia and alveolar spaces containing alveolar macrophages (H&E, ×200). (C) The columnar epithelia and alveolar lining cells are TTF-1 positive.

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