title: Desmoid tumor of lung with pleural involvement - the case of unique location of aggressive fibromatosis.
abstract: Desmoid tumors (DTs) are rare mesenchymal neoplasms with unpredictable natural history. There is a high risk of recurrence despite adequate surgical resection, however DTs do not have the capacity to metastasize. The estimated incidence in general population is 2-4 cases/million/year. They may occur at any age but most commonly in the third and fourth decades. Both sexes may be affected, but there is a slight female predominance. DTs can occur at any body site. The exact etiology remains unclear, but trauma, hormonal disturbances, pregnancy, genetic and hereditary factors are postulated to be in association with its' development. Potential to attain large size, infiltration and destruction of adjacent vital structures and tendency to recur are main management problems and important causes of morbidity and mortality. Wide excision is standard first-line treatment of primary or recurrent symptomatic desmoids. We present case of 33-years-old Caucasian female patient admitted to hospital with 2 months history of squeezing pain in right upper quadrant which appeared after meals. The patient was in general good condition. There were no abnormalities on basic laboratory tests on admission. CT of chest revealed hydrothorax to the level of the apex of the right lung and tumor sized 7 × 13 × 13 cm located in the lower lobe of right lung. Histopathological diagnosis of desmoid tumor of right lung was formulated. We report, to our knowledge for the first time in Poland, case of aggressive fibromatosis of lung with invasion of pleura.
authors.affiliation: Department of Pathomorphology Jagiellonian University Medical College, Grzegorzecka 16, Krakow, Poland. tomek.tokarek@gmail.com.
authors.forename: Tomasz
authors.initials: T
authors.lastname: Tokarek
authors.forename: Joanna
authors.initials: J
authors.lastname: Szpor
authors.forename: Juliusz
authors.initials: J
authors.lastname: Pankowski
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Okoń
mesh_terms: D000328:Adult; D005260:Female; D018222:Desmoid Tumors / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D006876:Hydrothorax / Q000000981:diagnostic imaging / Q000209:etiology; D010997:Pleural Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D035583:Rare Diseases; D014057:Tomography, X-Ray Computed
keywords: desmoid; fibromatosis; lung; pleura