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Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion

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 pneumothora...

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Autores principales: Paci, Massimiliano, Cavazza, Alberto, Annessi, Valerio, Ricchetti, Tommaso, Rapicetta, Cristian, Sgarbi, Giorgio
Formato: Texto
Lenguaje:English
Publicado: PagePress 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2994490/
https://www.ncbi.nlm.nih.gov/pubmed/21139943
http://dx.doi.org/10.4081/rt.2010.e14
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author Paci, Massimiliano
Cavazza, Alberto
Annessi, Valerio
Ricchetti, Tommaso
Rapicetta, Cristian
Sgarbi, Giorgio
author_facet Paci, Massimiliano
Cavazza, Alberto
Annessi, Valerio
Ricchetti, Tommaso
Rapicetta, Cristian
Sgarbi, Giorgio
author_sort Paci, Massimiliano
collection PubMed
description 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.
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spelling pubmed-29944902010-12-07 Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion Paci, Massimiliano Cavazza, Alberto Annessi, Valerio Ricchetti, Tommaso Rapicetta, Cristian Sgarbi, Giorgio Rare Tumors Case Report 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. PagePress 2010-03-31 /pmc/articles/PMC2994490/ /pubmed/21139943 http://dx.doi.org/10.4081/rt.2010.e14 Text en ©Copyright M. Paci et al., 2010 This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0). Licensee PAGEPress, Italy
spellingShingle Case Report
Paci, Massimiliano
Cavazza, Alberto
Annessi, Valerio
Ricchetti, Tommaso
Rapicetta, Cristian
Sgarbi, Giorgio
Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
title Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
title_full Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
title_fullStr Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
title_full_unstemmed Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
title_short Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
title_sort cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2994490/
https://www.ncbi.nlm.nih.gov/pubmed/21139943
http://dx.doi.org/10.4081/rt.2010.e14
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