Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion

Paci, Massimiliano; Cavazza, Alberto; Annessi, Valerio; Ricchetti, Tommaso; Rapicetta, Cristian; Sgarbi, gGiorgio

doi:10.4081/rt.2010.e14

Cited by 6 publications

(6 citation statements)

References 17 publications

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“…Moreover, cystic PLCH must be differentiated from the rare cystic fibrohistiocytic tumour, both in the primary and in the secondary forms. Pulmonary cysts of this tumour also result from pulmonary nodule transformation [ 32 ]. Histological assessment is required for diagnosis.…”

Section: The Challenging Diagnosis At Initial Patient Presentationmentioning

confidence: 99%

Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

et al. 2014

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ObjectivesPulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs.MethodsThis article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed.ResultsPLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis.ConclusionsThe radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation.Teaching Points• PLCH is a rare interstitial smoking-related disease that usually affects young adults.• The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs.• Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered.• Lung cystic involvement correlates with lung function abnormalities and predicts functional decline.• Integration of the clinical history and imaging results is key to diagnosis.

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Section: The Challenging Diagnosis At Initial Patient Presentationmentioning

confidence: 99%

Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

et al. 2014

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“…On CT scan of the chest, cystic fibrohistiocytic tumours usually appeared as bilateral multiple cystic foci or nodular opacities. In one case, however, the tumour presented as a solitary pulmonary nodule [7]. The overall prognosis was excellent; of 13 patients, all were alive at a median follow-up of 2 years (interquartile range, 1-4.5 years).…”

Section: Discussionmentioning

confidence: 95%

“…Morphological and immunohistochemical evidence suggest that this tumour usually derives from cutaneous cellular fibrous histiocytomas (dermatofibromas) [2][3][4][5][6]. However, there is a small number of cases of cystic fibrohistiocytic tumour of the lung where no skin lesion was identified [1,2,[7][8][9]. These cases can be interpreted as primary pulmonary neoplasms or as cases where the primary skin lesion is occult [1].…”

Section: Discussionmentioning

confidence: 99%

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Cystic fibrohistiocytic tumour of the lung presenting with recurrent pneumothoraces: a case report

Kakos¹,

Lampridis²,

Geropoulos³

et al. 2020

Monaldi Arch Chest Dis

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Cystic fibrohistiocytic tumour of the lung is a very rare pathological entity that occurs either as a primary pulmonary neoplasm or as a metastasis from skin lesions called cellular fibrous histiocytomas. Herein, we present the case of a 19-year old man with a history of recurrent pneumothoraces who was managed surgically and was eventually diagnosed with cystic fibrohistiocytic tumour of the lung. Clinicians should include this disease in the differential diagnosis of pulmonary cystic lesions and be aware of its association with cellular fibrous histiocytoma. Reporting of more cases is warranted to further elucidate the natural course of the disease and optimise its management.

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“…In fact, the differential diagnosis of pulmonary cysts is very broad, including neoplastic and non-tumoral diseases ( Tab. II ) 18-23 . The spindle cell proliferation growing in the cystic wall of cystic monophasic synovial sarcoma may be confused with some benign diseases (e.g., endometriosis) or other neoplastic lesions showing cystic changes, as type 1 pleuropulmonary blastoma (PPB), lymphangioleiomyomatosis (LAM), benign metastasizing leiomyoma, cystic fibrohistiocytic tumor (CFHT), and metastatic low-grade sarcoma 18-23 .…”

Section: Discussionmentioning

confidence: 99%

An unexpected cause of recurrent pneumothorax

Rossi¹,

Farnedi²,

Davoli³

et al. 2022

Pathologica

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Summary The thoracic district is the most frequent visceral location of synovial sarcoma, generally involving lung and pleura as a large solid mass. We present herein a 57-year-old man with recurrent pneumothorax and a localized bulla at the lingula. The lesion was excised by a Video-Assisted-Thoracoscopic-Surgery (VATS) wedge resection and surprisingly consisted of a unilocular cyst with fibrous wall intermingled by a longitudinal proliferation of bland-looking, dense, monomorphic spindle cells diffusely expressing EMA, CD99, CD56 and focally staining with cytokeratins. Fluorescent in situ hybridization demonstrated the presence of SYT rearrangement and a diagnosis of pulmonary cystic monophasic synovial sarcoma was made. Only few similar cases have been reported in literature, mainly occurring in young male adults. A meticulous examination of all resected tissue from pneumothorax is the prerequisite to suspect this extremely challenging condition, while immuno-molecular studies are mandatory to achieve the correct diagnosis.

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Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion

Cited by 6 publications

References 17 publications

Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

Cystic fibrohistiocytic tumour of the lung presenting with recurrent pneumothoraces: a case report

An unexpected cause of recurrent pneumothorax

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