Multiple Cystic Fibrohistiocytic Tumors of the Lung: Report of Two Cases

Abstract

We describe two adults with multiple cystic fibrohistiocytic tumors of the lung that manifested as bilateral nodular opacities, cystic lesions, or both on chest roentgenograms. One patient had recurrent episodes of pneumothorax and intermittent shortness of breath; the other was asymptomatic. Open-lung biopsy specimens showed identical histologically benign fibrohistiocytic proliferations associated with formation of cysts that were lined by metaplastic bronchiolar, squamous, or type II alveolar epithelium and old hemorrhage in the cysts. In both patients, the lesions grew slowly over a period of years. These unusual multiple bilateral fibrohistiocytic tumors of the lung should be included in the differential diagnosis of bilateral cystic lesions in the lung.

Metastasizing Fibrous Histiocytoma of the Skin: A Clinicopathologic and Immunohistochemical Analysis of Three Cases

“…In most cases, there is a long delay (range, 4 to 228 months; median, 40 months) between the excision of the primary skin lesion and the emergence of metastases. Actually, long-term follow-up shows that those metastases, whatever their location, are characteristically slow-growing lesions, which justifies the iterative metastasectomy approach in case of pulmonary involvement (7,8). This is exemplified by Patient 1 of our series in whom a progressive inguinal lymph node enlargement was noticed as long as 13 years before inguinal lymphadenectomy and also by Patient 2 of Colome-Grimmer and Evans's report (7), who survived 8 years after his lung metastases had been detected.…”

“…In both cases, the tumor metastasized to regional lymph nodes and lungs, in one case after repeated recurrences. Two potential additional cases of metastasizing dermatofibroma have been found in the literature (8,9).…”

Imaging of macrophage-related lung diseases

“…The tumor is composed of multiple nodules with varying degrees of thin-walled cystic changes and multifocal proliferation of fibrohistiocytic cells in the intervening stroma. Hemosiderin-laden macrophages and multinucleated giant cells containing cholesterol clefts are found within the cysts [129]. Cyst formation probably reflects chronic macrophage involvement of the alveolar parenchyma.…”

“…Cystic fibrohistiocytic tumor of the lung Cystic fibrohistiocytic tumors are exceedingly rare, with only eight cases (seven males, one female) having been reported as such [127][128][129][130]. The tumor is composed of multiple nodules with varying degrees of thin-walled cystic changes and multifocal proliferation of fibrohistiocytic cells in the intervening stroma.…”

“…All patients with follow-up (n=10) showed enlargement of the lesions but were alive with disease at an average of 5 years [127][128][129][130]. Chest radiography depicted bilateral cystic lung nodules or thin-walled cavities.…”