Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion.
Bottom Line: Radiologically it usually occurs as a cystic change of multiple pulmonary nodules, and pneumothorax is the most frequent presenting symptom.We present here a 16-year-old man with recurrent right pneumothorax.The patient had no history of cutaneous fibrohistiocytic lesions.
Affiliation: Division of Thoracic Surgery, and.
Cystic fibrohistiocytic tumor of the lung is a rare neoplasm. In many cases it represents a metastasis from a benign or low-grade fibrohistiocytic tumor of the skin, but occasionally it may be primary. Radiologically it usually occurs as a cystic change of multiple pulmonary nodules, and pneumothorax is the most frequent presenting symptom. We present here a 16-year-old man with recurrent right pneumothorax. The patient had no history of cutaneous fibrohistiocytic lesions. He underwent videothoracoscopic right apical segmentectomy, right lower lobe nodulectomy, and pleuroabrasion. Microscopy of the apical segmentectomy showed a cystic fibrohistiocytic tumor, whereas the nodule of the lower lobe was an intraparenchymal lymph node. The patient is alive with no tumor recurrence. The differential diagnosis includes Langerhans cell histiocytosis, lymphangioleiomyomatosis, pleuropulmonary blastoma, and metastatic endometrial stromal sarcoma. This disease usually occurs with multiple pulmonary cysts and cavitation. This case is the first reported presenting as a single lesion.
No MeSH data available.
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Mentions: Microscopically, in the apical wedge resection the lung parenchyma showed a small subpleural cyst, which opened into the pleural cavity (Figure 1). The cyst was lined by a single row of benign, cuboidal, alveolar epithelial cells, whereas the wall was composed of a thin rim of mesenchymal tissue (Figure 2). The latter consisted of bland, spindle-to-oval cells with scant cytoplasm, regular nuclear chromatin, and inconspicuous nucleoli. A few mitoses were present. Immunohistochemically, the epithelium lining the cyst was diffusely positive for cytokeratin AE1/AE3 and TTF-1, whereas the mesenchymal cells reacted focally only for estrogen receptors and were negative for progesterone receptors, cytokeratin AE1/AE3, TTF-1, S-100 protein, smooth muscle actin, desmin, CD34, CD68/PG-M1, and HMB-45. KI-67/MIB-1 was low (1%) in the mesenchymal component. A diagnosis of cystic fibrohistiocytic tumor was made. The nodule of the lower lobe was a reactive lymph-node.
No MeSH data available.