Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS)
Autops. Case Rep
;
9(3): e2019110, July-Sept. 2019. tab, ilus
Article
Dans Anglais
| LILACS
| ID: biblio-1017374
ABSTRACT
Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Sarcomes
/
Tumeurs du poumon
Limites du sujet:
Adulte très âgé
/
Humains
/
Mâle
langue:
Anglais
Texte intégral:
Autops. Case Rep
Thème du journal:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Année:
2019
Type:
Article
Pays d'affiliation:
États-Unis d'Amérique
Institution/Pays d'affiliation:
University of California/US
/
University of California, San Francisco/US
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