Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment

Maitane I. Orue-Echebarria, Laura Garciafília, Luis Rodriguez-Bachiller, Benjamín Díaz-Zorita, Enrique Velasco, Enrique Ramón, Carolina Agra, Arturo Colón Rodríguez
2020 Clinical Sarcoma Research  
Background Solitary fibrous tumor is an unusual fibroblastic mesenchymal neoplasm typically described in the pleura. It may appear anywhere with a varied anatomic distribution and essentially it can develop from any soft tissue or visceral location. Its course is usually indolent and it rarely causes distant metastases, so it has a prolonged survival rate. It sometimes presents itself as a disseminate disease being the liver the most frequently involved location. In these occasions, the
more » ... asions, the management should be discussed in a multidisciplinary tumor committee formed by surgeons, oncologists and radiologists. Surgery remains the gold standard for treatment. Case representation We present the case of a woman with a tumor in the left abdominal wall and bilobar massive liver metastases, both locations histologically diagnosed as solitary fibrous tumor. She receives biological treatment for a severe case of Crohn´s disease. Evaluated in a multidisciplinary committee, surgery was recommended for both the primary lesion and the liver metastases. The hepatobiliary surgeons considered a two-stage hepatectomy with portal vein embolization (PVE) as the best strategy. After the first procedure consisting in cleaning the left hepatic lobe followed by PVE the future liver remnant volume (FLRV) was considered inadequate, so the patient was also treated with right transarterial radioembolizacion with yttrium 90 (TARE-Y90) intending a double goal: to treat the tumor and to increased the FLRV. Furthermore, a severe flare of Crohn´s disease forced us to intensify the patient's treatment with the addition of biological agents (infliximab and adalimumab) until complete remission of the symptoms. The second stage of the liver surgery had to be postponed for more than 6 months and could finally be carried out without complications, achieving an R0 resection. The postoperative course was uneventful and the follow up has showed no recurrence to date. Conclusion Solitary fibrous tumours with extensive liver metastases are infrequent but when they appear modern surgical strategies like two stage hepatectomy are the treatment of choice and must be carried out by specialised units. The therapeutic decisions should be guided by a multidisciplinary committee.
doi:10.1186/s13569-020-00146-4 pmid:33292480 fatcat:s3eneu6kn5b5xnxd7h77wdr5si