An unusual case of incomplete Carney triad: an 18-year-old girl suffering from multiple benign tumors

Abstract

Carney triad is a rare syndrome that involves gastrointestinal stromal tumor (GIST), pulmonary chondroma and extra-adrenal paraganglioma. Patients presenting GIST and pulmonary chondroma account for 72.7% of all incomplete Carney triad cases. Clinically, it is mainly diagnosed by radiological images and pathological results. Some studies have elucidated the pathogenesis of Carney triad. Surgical resection is the preferred treatment for Carney triad. Generally speaking, the prognosis of patients with Carney triad has been satisfied. According to current reports, the rate of 40-year survival is up to 73%. The case reports an 18-year-old girl suffering from pulmonary chondroma and posterior mediastinal paraganglioma, simultaneously with pulmonary hamartoma, breast fibroma and lower limb chondroma.

Keywords: Carney triad; incomplete; multiple benign tumors.

Figure 1

Chest X-ray shows two calcified shadows. One is about 5.2 cm × 4.5 cm near the right lung hilum and the other is about 0.9 cm × 0.7 cm in right lower lung. Moreover, there is a hummocky shadow about 2.9 cm × 1.7 cm on the right side of the spine.

Figure 2

Chest CT shows that there is a soft and multiple calcified tissue mass about 5.8 cm × 3.1 cm near the bronchus of the right lower lung, and its edge is clear and smooth. It is clinically diagnosed pulmonary hamartoma.

Figure 3

Chest CT shows that there is a soft tissue mass with blood vessels likely connected to the descending aorta on the right side of fifth thoracic near the spine. Its unenhanced CT value is 42 HU and enhanced CT value is about 247 HU respectively. Finally, it is diagnosed hypervascular neurogenic tumor located in posterior mediastinum.

Figure 4

Chest CT reveals a calcified nodule about 1.0 cm in basal segment of the right lower lung, which is considered pulmonary chondroma or pulmonary hamartoma.

Figure 5

Paraganglioma and pulmonary hamartoma were clearly exposed to the surgeons’ field of vision. (A) Paraganglioma (between fifth and sixth thoracic, near right side of spine); (B) pulmonary hamartoma (located in dorsal segment of right lower lung).

Figure 6

Three benign tumors were completely excised and neatly placed on surgical table. (A) Pulmonary hamartoma (7 cm × 6.5 cm × 4.5 cm); (B) pulmonary chondroma (1.5 cm × 1.0 cm × 0.5 cm); (C) paraganglioma (2.5 cm × 1.5 cm × 0.5 cm).

Figure 7

Pulmonary hamartoma (×100): osteoblasts, chondrocytes, adipocytes and respiratory epithelial cracks (Hematoxylin staining).

Figure 8

Pulmonary chondroma (×100): well-differentiated chondrocytes (Hematoxylin staining).

Figure 9

Paraganglioma (×100): cellular nests, full of thin-walled vessels, abundant cytoplasm of tumor cells, slightly eosinophilic, round nuclei. Immunohistochemistry: s-100 sertoli cells (+) (Hematoxylin staining).