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

Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment 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 Background Solitary fibrous tumor (SFT) is a rare fibroblastic mesen - *Correspondence: arturo.colon@smqm.es chymal neoplasm (less than 2% of all soft tissue tumors) Department of General and Digestive Surgery Transplant, mainly described in the pleura. Although it is commonly Hepatobiliopancreatic Surgery Unit, Hospital General Universitario considered as intrathoracic, more than 50% of the SFTs Gregorio Marañón, c/Doctor Esquerdo 46, 28007 Madrid, Spain Full list of author information is available at the end of the article arise outside the thorax [1, 2]. They can appear at any © The Author(s) 2020. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creat iveco mmons .org/licen ses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/publi cdoma in/ zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Orue‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 2 of 6 time in life, but they are more frequent in the fifth dec - the subcutaneous lesion located in the left lumbar region ade, without gender difference [3, 4]. These normally and also on one of the focal lesions of the right hepatic arise from inner membranes (pleura, peritoneum and lobe. Histologically, both samples were practically iden- meninges) and deep soft tissues (retroperitoneum and tical, which confirmed that the liver lesions were meta - pelvic soft tissues). Nowadays, the classical thoracic site static. The pathology showed a fusocellular proliferation represents only 30% of the cases, including pleura, lungs of highly cellular mesenchymal lineage, with eosinophilic and mediastinum. Other less frequent locations are cata- cytoplasm cells and ovoid nucleus with finely granular logued as extrapleural solitary fibrous tumor (eSFT) and chromatin, without evident mitotic images. The Ki67 can involve solid organs, head, neck and soft tissues of proliferative index was 20%. Immunohistochemical the abdominal wall and extremities, as the one we pre- staining was positive for STATS6. After a multidiscipli- sent here [3, 5]. nary discussion, the preferred strategy proposed was an The majority of SFTs follow an indolent course and do upfront resection of the primary tumor and liver metas- not recur after the removal. 10-year overall survival in tases, without neoadjuvant chemo or radiotherapy due to these cases is 90%. However, between 10 and 25% of SFTs lack of evidence as do its usefulness. The extend of the may have local recurrence or will present as a dissemi- liver involvement precluded a complete resection in one nate disease. These patients who recur and those who procedure, so we designed a two stage strategy consisting cannot be resected, have a poor prognosis [3, 5]. in resection of the primary tumor and left hepatic metas- Management of SFTs at all sites should be discussed in tases, followed by portal vein embolization and subse- a multidisciplinary committee. The preferred treatment quent right hepatectomy. The liver function test prior to for locally advanced or metastatic SFT is radical surgery the surgery included a direct measure of the portal sys- even if it is necessary to use aggressive surgical strategies temic gradient via transjugular catheter, and indocyanine like two stage hepatectomy as we used in this case [5]. green clearance (IGC) test, both favorable to perform a major resection (4  mmHg gradient; PDR 25; R15: 2). Case presentation The first surgical procedure was carried out as planned, A 53-year-old woman with a long medical history, including metastasectomies of lesions located in seg- including Crohn’s disease with two intestinal surgeries ments IVa; IVb and particularly bulky one in segment (one of them due to an intestinal perforation), psoriatic I, and radiofrequency ablation of a lesion in segment II. arthritis and mild chronic renal failure was referred for Right portal vein embolization (PVE) was performed in consultation because of a solid mass on the left flank. The the same hospital admission by interventional radiology CT scan showed a subcutaneous tumor in the left lateral using cyanoacrylate-lipiodol and particles (PVA: 350– abdominal wall and multiple giant bilobar liver metasta- 500 and 500–700 microns). The postoperative course was ses which consisted in a 13.5  cm tumor in segment VII uneventful and the patient was discharged at 7 postop- and a 9.5  cm tumor in segment IVb (Fig.  1). The only erative day. The anatomopathological characteristics of related clinical record was the resection of a lipoma in these samples coincided with the findings described in that same area (left lateral abdominal wall) 12 years ago. the previous biopsies: a neoplasic mesenchymal prolifera- An ultrasound-guided core-biopsy was performed on tion, hypercellular, solid-growth, with a storiform pattern Fig. 1 CT scan (a) primary tumour in left flank. b Giant liver metastases Orue ‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 3 of 6 consisting of spindle cells with imprecise boundaries, staining remained positive for STAT6 and CD34 (Fig. 2). with ovoid nuclei with finely granular chromatin, without A CT scan was performed one month after the PVE atypia, which are accompanied by medium-sized blood and the volume estimate of FLR was insufficient, (35%) vessels, some with deer horn morphology, branched, (Fig.  3). In the meantime the patient had severe Crohnʼs showing thin walls and unaltered endothelium. No vas- exacerbation which coursed with digestive bleeding and cular or perineural invasions were observed. A Ki67 required hospitalisation for more than two weeks, inten- proliferation rate of 15% was also noted, with a low rate sive corticoid treatment and the commencement of bio- of mitosis (2 mitosis/10HPF). Immunohistochemical logical therapy with Adalimumab. It was decided in the Fig. 2 a Relationship between neoplasic mesenchymal proliferation and liver parenchyma; b 2 mitosis/10HPF; c necrosis areas inside tumor; d medium‑sized blood vessels, some with deer horn morphology, branched, showing thin walls and unaltered endothelium; e cytoplasmatic immunohistochemical staining positive for CD34 + ; f immunohistochemical staining positive for STAT6 Fig. 3 Volumetric study. Bilobar giant metastasis. a FLRV before PVE (19%). b FLRV after PVE (35%) Orue‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 4 of 6 multidisciplinary committee to perform a TARE-Y90 for six months until the patient was sufficiently recov - on the right liver in order to avoid tumoral progression ered. In the reevaluation study for the second liver sur- and to try to increase the FRLV (Fig.  4). In the following gery, the CT-scan showed voluminous hepatic masses in months, the patient needed hospital admission due to a the right lobe with diameters up to 10 cm, particularly in septic shock caused by an urinary tract infection. She also the posterior sector with areas of necrosis and reduced suffered malnutrition in the context of Crohn’s disease contrast enhancement as a sign of response follow- and the second stage of the liver surgery was postponed ing modified RECIST criteria [6]. There was no sign of Fig. 4 a, b TARE‑ Y90 procedure. c Increased (50%) FLRV after TARE‑ Y90 Orue ‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 5 of 6 recurrence in the remnant left lobe, nor distant metas- The liver involvement wether it is as a primary or tases. The volumetric study showed an important left secondary location, as our case, is extremely rare with hepatic lobe hypertrophy (FLR 50%) (Fig.  4). With this only a few cases reported in the literature, and should information, a right hepatectomy was planned. The sur - be treated in specialized units of hepatobiliopancreatic gery was particularly difficult mainly due to the adher - surgery [1, 5]. Multiple hepatic tumors, especially if ent syndrome caused by previous surgeries. We utilized they affect both lobes, require careful surgical planning. an anterior approach with hanging maneuver in order to On certain occasions, the surgery cannot be performed avoid tumor spreading during the right liver lobe mobi- in one stage only, and requires combined strategies lization (Fig.  4). The postoperative course was unevent - such as PVE and two-stage hepatectomy [9]. In the ful and the patient was discharged at 7th postoperative literature review, we found several reports of hepatic day. The pathologic study showed images of necrosis SFTs that required major liver resections but as far as representing up to 80% of the tumoral volume. The mes - we know, this is the first case of two-stage hepatectomy enchimal cells had few mitotic images, and were positive using TARE-Y90 in between stages. If the hepatic bilo- for CD34, BCL2, and STAT-6, with a proliferative index, bar involvement precludes surgical removal, Novais measured with Ki67, of 10%. Currently, 30  months after et al. [1] suggest that liver transplantation could be con- the first liver surgery, the patient is asymptomatic and sidered but to our knowledge, there are no data pub- free of active disease. lished about such an experience. In any case, as with other sarcomas, surgery remains the principal thera- peutic option in order to improve survival rates, so an Discussion and conclusion aggressive approach using complex liver surgery is jus- eSFT with liver metastases is an extremely rare entity tified. Clear criteria for a malignant behavior have not with less than 30 cases reported in the literature [7]. The been well defined, but several risk stratification models most common clinical presentation is as a painless mass have been proposed. Gold et  al., presents a numerous [2]. u Th s, our patient debuted with a tumour on the left series of cases with more than 2-year follow-up, and flank, without any other associated symptoms. Diagnosis proposes the following: extrathoracic tumours bears an is based upon radiographic findings on CT or magnetic increased risk for local recurrence; metastases are more resonance and are similar to those of other soft tissue frequent in tumours larger than 10 cm; positive surgical tumors, without pathognomonic features. Regardless of margins and the presence of histologically aggressive the site of origin, SFTs usually appear as a well-defined features were predicting factors for worse local behav- soft tissue mass, highly vascular, which may be lobulated ior and poorer recurrence-free and metastasis-free and with a heterogeneous enhancement pattern (due survival. Histological criteria favoring recurrence are to different collagen compounds inside). In addition, a pleomorphism, atypia, high cellularity, increased mito- total-body CT is needed for an appropriate staging [4]. sis, and necrosis [6]. Definitive diagnosis of SFT requires histologic examina - Our patient did not receive systemic treatment. The tion of an adequate tissue sample and is based upon rec- reported role of chemotherapy and radiotherapy in this ognition of typical morphologic features in conjunction kind of tumours is still controversial. Systemic thera- with a characteristic immunophenotype. The SFT repre - pies are reserved for cases where the resection has been sents a distinct entity within the wide range of soft tissue incomplete or when there are obvious signs of aggres- tumours, and should be evaluated by a pathologist with sive behavior [5, 8]. ample experience for correct diagnosis. Its cellular origin In conclusion, solitary fibrous tumours with extensive is believed to be fibroblastic in type [2, 3]. hepatic liver metastases are infrequent but when they Once the histological diagnosis is certain, manage- occur modern liver surgery strategies like two stage ment of SFTs at all sites should be discussed in a multi- hepatectomy are the treatment of choice and must be disciplinary tumour committee with the participation carried out in specialized units. The decision making of surgeons, oncologists, radiologists and pathologists. should be done in a multidisciplinary committee. Although there is not a great body of scientific evi - dence, and these are uncommon tumours, radical sur- Abbreviations gery appears to be the treatment of choice [5, 8]. On the PVE: Portal vein embolization; SFT: Solitary fibrous tumor; CT: Computed other hand, such aggressive surgical interventions are tomography; TARE‑ Y90: Transarterial Radiembolization Y90; SFT: Solitary fibrous tumor; eSFT: Extrapleural solitary fibrous tumor; FLRV: Future liver remnant questionable in asymptomatic patients due to the mainly volume. benign or uncertain natural history of this tumour, taking into account the morbidity associated with major surger- Acknowledgements Not applicable. ies [1]. Orue‑Echebarria  et al. Clin Sarcoma Res (2020) 10:23 Page 6 of 6 Authorsʼ contributions 2. Daigeler A, Lehnhardt M, Langer S, Steinstraesser L, Steinau H‑U, Mentzel Surgical interventions: AC, BD‑Z, LR‑B, MIO ‑E: Analysis of the data, review of T, et al. Clinicopathological findings in a case series of extrathoracic the literature, drafting and revision of the manuscript: MIO‑E, AC, LR‑B. solitary fibrous tumors of soft tissues. BMC Surg. 2006;6(1):10. 3. Ronchi A, Cozzolino I, Zito F, Accardo MM, Montella M, Panarese I, et al. Funding Extrapleural solitary fibrous tumor: a distinct entity from pleural solitary We do not have any funding for this work. fibrous tumor. An update on clinical, molecular and diagnostic features. Ann DiagnPathol. 2018;34(142):150. Availability of data and materials 4. Wallace SJ, Teixeira R, Miller NF, Raj M, Sheikh H, Sharma R. Extrapleural We have all the data and extra material available in the hospital electronic superficial solitary fibrous tumor on the posterior shoulder: a case report clinic information. and review of the literature. Eplasty. 2018;18:e31. 5. Febrero B, Robles R, Brusadin R, Marín C, López‑ Conesa A, Martínez C, Ethics approval and consent to participate et al. Metástasishepática y pancreáticas de un tumor fibrososolitario. Cir We have ethics approval for the publication of this work. Esp. 2014;92(6):438–41. 6. Lencioni R, Llovet J. Modified RECIST (mRECIST ) Assessment for Hepato ‑ Consent for publication cellular Carcinoma. Semin Liver Dis. 2010;30(01):052–60. We have patientʼs consent for the publication of this work. 7. Gold JS, Antonescu CR, Hajdu C, Ferrone CR, Hussain M, Lewis JJ, et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer. Competing interests 2002;94(4):1057–68. Nothing to disclose. 8. Jakob M. Malignant solitary fibrous tumor involving the liver. World J Gastroenterol. 2013;19(21):3354. Author details 9. Adam R, Laurent A, Azoulay D, Castaing D, Bismuth H. Two‑stage hepa‑ Department of General and Digestive Surgery Transplant, Hepatobiliopan‑ tectomy: a planned strategy to treat irresectable liver tumors. Ann Surg. creatic Surgery Unit, Hospital General Universitario Gregorio Marañón, c/ 2000;232(6):777–85. Doctor Esquerdo 46, 28007 Madrid, Spain. Department of Surgery, School of Medicine, Universidad Complutense de Madrid, Madrid, Spain. Depart‑ Publisher’s Note ment of Radiology, Abdominal Image and Therapeutics Unit, Hospital General 4 Springer Nature remains neutral with regard to jurisdictional claims in pub‑ Universitario Gregorio Marañón, Madrid, Spain. Anatomopatologic Depart‑ lished maps and institutional affiliations. ment, Hospital General Universitario Gregorio Marañón, Madrid, Spain. Received: 25 April 2019 Accepted: 10 November 2020 References 1. Novais P, Robles‑Medranda C, Pannain VL, Barbosa D, Biccas B, Fogaça H. Solitary fibrous liver tumor: is surgical approach the best option? J Gastrointestin Liver Dis. 2010;19(1):81–4. Ready to submit your research ? 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Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment

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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 Background Solitary fibrous tumor (SFT) is a rare fibroblastic mesen - *Correspondence: arturo.colon@smqm.es chymal neoplasm (less than 2% of all soft tissue tumors) Department of General and Digestive Surgery Transplant, mainly described in the pleura. Although it is commonly Hepatobiliopancreatic Surgery Unit, Hospital General Universitario considered as intrathoracic, more than 50% of the SFTs Gregorio Marañón, c/Doctor Esquerdo 46, 28007 Madrid, Spain Full list of author information is available at the end of the article arise outside the thorax [1, 2]. They can appear at any © The Author(s) 2020. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creat iveco mmons .org/licen ses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/publi cdoma in/ zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Orue‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 2 of 6 time in life, but they are more frequent in the fifth dec - the subcutaneous lesion located in the left lumbar region ade, without gender difference [3, 4]. These normally and also on one of the focal lesions of the right hepatic arise from inner membranes (pleura, peritoneum and lobe. Histologically, both samples were practically iden- meninges) and deep soft tissues (retroperitoneum and tical, which confirmed that the liver lesions were meta - pelvic soft tissues). Nowadays, the classical thoracic site static. The pathology showed a fusocellular proliferation represents only 30% of the cases, including pleura, lungs of highly cellular mesenchymal lineage, with eosinophilic and mediastinum. Other less frequent locations are cata- cytoplasm cells and ovoid nucleus with finely granular logued as extrapleural solitary fibrous tumor (eSFT) and chromatin, without evident mitotic images. The Ki67 can involve solid organs, head, neck and soft tissues of proliferative index was 20%. Immunohistochemical the abdominal wall and extremities, as the one we pre- staining was positive for STATS6. After a multidiscipli- sent here [3, 5]. nary discussion, the preferred strategy proposed was an The majority of SFTs follow an indolent course and do upfront resection of the primary tumor and liver metas- not recur after the removal. 10-year overall survival in tases, without neoadjuvant chemo or radiotherapy due to these cases is 90%. However, between 10 and 25% of SFTs lack of evidence as do its usefulness. The extend of the may have local recurrence or will present as a dissemi- liver involvement precluded a complete resection in one nate disease. These patients who recur and those who procedure, so we designed a two stage strategy consisting cannot be resected, have a poor prognosis [3, 5]. in resection of the primary tumor and left hepatic metas- Management of SFTs at all sites should be discussed in tases, followed by portal vein embolization and subse- a multidisciplinary committee. The preferred treatment quent right hepatectomy. The liver function test prior to for locally advanced or metastatic SFT is radical surgery the surgery included a direct measure of the portal sys- even if it is necessary to use aggressive surgical strategies temic gradient via transjugular catheter, and indocyanine like two stage hepatectomy as we used in this case [5]. green clearance (IGC) test, both favorable to perform a major resection (4  mmHg gradient; PDR 25; R15: 2). Case presentation The first surgical procedure was carried out as planned, A 53-year-old woman with a long medical history, including metastasectomies of lesions located in seg- including Crohn’s disease with two intestinal surgeries ments IVa; IVb and particularly bulky one in segment (one of them due to an intestinal perforation), psoriatic I, and radiofrequency ablation of a lesion in segment II. arthritis and mild chronic renal failure was referred for Right portal vein embolization (PVE) was performed in consultation because of a solid mass on the left flank. The the same hospital admission by interventional radiology CT scan showed a subcutaneous tumor in the left lateral using cyanoacrylate-lipiodol and particles (PVA: 350– abdominal wall and multiple giant bilobar liver metasta- 500 and 500–700 microns). The postoperative course was ses which consisted in a 13.5  cm tumor in segment VII uneventful and the patient was discharged at 7 postop- and a 9.5  cm tumor in segment IVb (Fig.  1). The only erative day. The anatomopathological characteristics of related clinical record was the resection of a lipoma in these samples coincided with the findings described in that same area (left lateral abdominal wall) 12 years ago. the previous biopsies: a neoplasic mesenchymal prolifera- An ultrasound-guided core-biopsy was performed on tion, hypercellular, solid-growth, with a storiform pattern Fig. 1 CT scan (a) primary tumour in left flank. b Giant liver metastases Orue ‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 3 of 6 consisting of spindle cells with imprecise boundaries, staining remained positive for STAT6 and CD34 (Fig. 2). with ovoid nuclei with finely granular chromatin, without A CT scan was performed one month after the PVE atypia, which are accompanied by medium-sized blood and the volume estimate of FLR was insufficient, (35%) vessels, some with deer horn morphology, branched, (Fig.  3). In the meantime the patient had severe Crohnʼs showing thin walls and unaltered endothelium. No vas- exacerbation which coursed with digestive bleeding and cular or perineural invasions were observed. A Ki67 required hospitalisation for more than two weeks, inten- proliferation rate of 15% was also noted, with a low rate sive corticoid treatment and the commencement of bio- of mitosis (2 mitosis/10HPF). Immunohistochemical logical therapy with Adalimumab. It was decided in the Fig. 2 a Relationship between neoplasic mesenchymal proliferation and liver parenchyma; b 2 mitosis/10HPF; c necrosis areas inside tumor; d medium‑sized blood vessels, some with deer horn morphology, branched, showing thin walls and unaltered endothelium; e cytoplasmatic immunohistochemical staining positive for CD34 + ; f immunohistochemical staining positive for STAT6 Fig. 3 Volumetric study. Bilobar giant metastasis. a FLRV before PVE (19%). b FLRV after PVE (35%) Orue‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 4 of 6 multidisciplinary committee to perform a TARE-Y90 for six months until the patient was sufficiently recov - on the right liver in order to avoid tumoral progression ered. In the reevaluation study for the second liver sur- and to try to increase the FRLV (Fig.  4). In the following gery, the CT-scan showed voluminous hepatic masses in months, the patient needed hospital admission due to a the right lobe with diameters up to 10 cm, particularly in septic shock caused by an urinary tract infection. She also the posterior sector with areas of necrosis and reduced suffered malnutrition in the context of Crohn’s disease contrast enhancement as a sign of response follow- and the second stage of the liver surgery was postponed ing modified RECIST criteria [6]. There was no sign of Fig. 4 a, b TARE‑ Y90 procedure. c Increased (50%) FLRV after TARE‑ Y90 Orue ‑Echebarria et al. Clin Sarcoma Res (2020) 10:23 Page 5 of 6 recurrence in the remnant left lobe, nor distant metas- The liver involvement wether it is as a primary or tases. The volumetric study showed an important left secondary location, as our case, is extremely rare with hepatic lobe hypertrophy (FLR 50%) (Fig.  4). With this only a few cases reported in the literature, and should information, a right hepatectomy was planned. The sur - be treated in specialized units of hepatobiliopancreatic gery was particularly difficult mainly due to the adher - surgery [1, 5]. Multiple hepatic tumors, especially if ent syndrome caused by previous surgeries. We utilized they affect both lobes, require careful surgical planning. an anterior approach with hanging maneuver in order to On certain occasions, the surgery cannot be performed avoid tumor spreading during the right liver lobe mobi- in one stage only, and requires combined strategies lization (Fig.  4). The postoperative course was unevent - such as PVE and two-stage hepatectomy [9]. In the ful and the patient was discharged at 7th postoperative literature review, we found several reports of hepatic day. The pathologic study showed images of necrosis SFTs that required major liver resections but as far as representing up to 80% of the tumoral volume. The mes - we know, this is the first case of two-stage hepatectomy enchimal cells had few mitotic images, and were positive using TARE-Y90 in between stages. If the hepatic bilo- for CD34, BCL2, and STAT-6, with a proliferative index, bar involvement precludes surgical removal, Novais measured with Ki67, of 10%. Currently, 30  months after et al. [1] suggest that liver transplantation could be con- the first liver surgery, the patient is asymptomatic and sidered but to our knowledge, there are no data pub- free of active disease. lished about such an experience. In any case, as with other sarcomas, surgery remains the principal thera- peutic option in order to improve survival rates, so an Discussion and conclusion aggressive approach using complex liver surgery is jus- eSFT with liver metastases is an extremely rare entity tified. Clear criteria for a malignant behavior have not with less than 30 cases reported in the literature [7]. The been well defined, but several risk stratification models most common clinical presentation is as a painless mass have been proposed. Gold et  al., presents a numerous [2]. u Th s, our patient debuted with a tumour on the left series of cases with more than 2-year follow-up, and flank, without any other associated symptoms. Diagnosis proposes the following: extrathoracic tumours bears an is based upon radiographic findings on CT or magnetic increased risk for local recurrence; metastases are more resonance and are similar to those of other soft tissue frequent in tumours larger than 10 cm; positive surgical tumors, without pathognomonic features. Regardless of margins and the presence of histologically aggressive the site of origin, SFTs usually appear as a well-defined features were predicting factors for worse local behav- soft tissue mass, highly vascular, which may be lobulated ior and poorer recurrence-free and metastasis-free and with a heterogeneous enhancement pattern (due survival. Histological criteria favoring recurrence are to different collagen compounds inside). In addition, a pleomorphism, atypia, high cellularity, increased mito- total-body CT is needed for an appropriate staging [4]. sis, and necrosis [6]. Definitive diagnosis of SFT requires histologic examina - Our patient did not receive systemic treatment. The tion of an adequate tissue sample and is based upon rec- reported role of chemotherapy and radiotherapy in this ognition of typical morphologic features in conjunction kind of tumours is still controversial. Systemic thera- with a characteristic immunophenotype. The SFT repre - pies are reserved for cases where the resection has been sents a distinct entity within the wide range of soft tissue incomplete or when there are obvious signs of aggres- tumours, and should be evaluated by a pathologist with sive behavior [5, 8]. ample experience for correct diagnosis. Its cellular origin In conclusion, solitary fibrous tumours with extensive is believed to be fibroblastic in type [2, 3]. hepatic liver metastases are infrequent but when they Once the histological diagnosis is certain, manage- occur modern liver surgery strategies like two stage ment of SFTs at all sites should be discussed in a multi- hepatectomy are the treatment of choice and must be disciplinary tumour committee with the participation carried out in specialized units. The decision making of surgeons, oncologists, radiologists and pathologists. should be done in a multidisciplinary committee. Although there is not a great body of scientific evi - dence, and these are uncommon tumours, radical sur- Abbreviations gery appears to be the treatment of choice [5, 8]. On the PVE: Portal vein embolization; SFT: Solitary fibrous tumor; CT: Computed other hand, such aggressive surgical interventions are tomography; TARE‑ Y90: Transarterial Radiembolization Y90; SFT: Solitary fibrous tumor; eSFT: Extrapleural solitary fibrous tumor; FLRV: Future liver remnant questionable in asymptomatic patients due to the mainly volume. benign or uncertain natural history of this tumour, taking into account the morbidity associated with major surger- Acknowledgements Not applicable. ies [1]. Orue‑Echebarria  et al. Clin Sarcoma Res (2020) 10:23 Page 6 of 6 Authorsʼ contributions 2. Daigeler A, Lehnhardt M, Langer S, Steinstraesser L, Steinau H‑U, Mentzel Surgical interventions: AC, BD‑Z, LR‑B, MIO ‑E: Analysis of the data, review of T, et al. Clinicopathological findings in a case series of extrathoracic the literature, drafting and revision of the manuscript: MIO‑E, AC, LR‑B. solitary fibrous tumors of soft tissues. BMC Surg. 2006;6(1):10. 3. Ronchi A, Cozzolino I, Zito F, Accardo MM, Montella M, Panarese I, et al. Funding Extrapleural solitary fibrous tumor: a distinct entity from pleural solitary We do not have any funding for this work. fibrous tumor. An update on clinical, molecular and diagnostic features. Ann DiagnPathol. 2018;34(142):150. Availability of data and materials 4. Wallace SJ, Teixeira R, Miller NF, Raj M, Sheikh H, Sharma R. Extrapleural We have all the data and extra material available in the hospital electronic superficial solitary fibrous tumor on the posterior shoulder: a case report clinic information. and review of the literature. Eplasty. 2018;18:e31. 5. Febrero B, Robles R, Brusadin R, Marín C, López‑ Conesa A, Martínez C, Ethics approval and consent to participate et al. Metástasishepática y pancreáticas de un tumor fibrososolitario. Cir We have ethics approval for the publication of this work. Esp. 2014;92(6):438–41. 6. Lencioni R, Llovet J. Modified RECIST (mRECIST ) Assessment for Hepato ‑ Consent for publication cellular Carcinoma. Semin Liver Dis. 2010;30(01):052–60. We have patientʼs consent for the publication of this work. 7. Gold JS, Antonescu CR, Hajdu C, Ferrone CR, Hussain M, Lewis JJ, et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer. Competing interests 2002;94(4):1057–68. Nothing to disclose. 8. Jakob M. Malignant solitary fibrous tumor involving the liver. World J Gastroenterol. 2013;19(21):3354. Author details 9. Adam R, Laurent A, Azoulay D, Castaing D, Bismuth H. Two‑stage hepa‑ Department of General and Digestive Surgery Transplant, Hepatobiliopan‑ tectomy: a planned strategy to treat irresectable liver tumors. Ann Surg. creatic Surgery Unit, Hospital General Universitario Gregorio Marañón, c/ 2000;232(6):777–85. Doctor Esquerdo 46, 28007 Madrid, Spain. Department of Surgery, School of Medicine, Universidad Complutense de Madrid, Madrid, Spain. Depart‑ Publisher’s Note ment of Radiology, Abdominal Image and Therapeutics Unit, Hospital General 4 Springer Nature remains neutral with regard to jurisdictional claims in pub‑ Universitario Gregorio Marañón, Madrid, Spain. Anatomopatologic Depart‑ lished maps and institutional affiliations. ment, Hospital General Universitario Gregorio Marañón, Madrid, Spain. Received: 25 April 2019 Accepted: 10 November 2020 References 1. Novais P, Robles‑Medranda C, Pannain VL, Barbosa D, Biccas B, Fogaça H. Solitary fibrous liver tumor: is surgical approach the best option? J Gastrointestin Liver Dis. 2010;19(1):81–4. Ready to submit your research ? 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