Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment
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inical Sarcoma Research Open Access
CASE REPORT
Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment Maitane I. Orue‑Echebarria1, Laura Garciafília1, Luis Rodriguez‑Bachiller1, Benjamín Díaz‑Zorita1, Enrique Velasco1, Enrique Ramón3, Carolina Agra4 and Arturo Colón Rodríguez1,2*
Abstract 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 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 symp‑ toms. 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. Keywords: Solitary fibrous tumor, Extrapleural solitary fibrous tumor, Two-stage hepatectomy, Portal vein emboliztion, TARE-Y90
*Correspondence: [email protected] 1 Department of General and Digestive Surgery Transplant, Hepatobiliopancreatic Surgery Unit, Hospital General Universitario Gregorio Marañón, c/Doctor Esquerdo 46, 28007 Madrid, Spain Full list of author information is available at the end of the article
Background Solitary fibrous tumor
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