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Solitary fibrous tumor of all sites: outcome of late recurrences in 14 patients

Solitary fibrous tumor of all sites: outcome of late recurrences in 14 patients Background: We explore the pattern of late recurrence (LR) in solitary fibrous tumor (SFT), focusing on histopathologic characteristics, clinical presentation and patients (pts) outcome. Methods: Clinical records of all pts with confirmed pathologic diagnosis of SFT treated at our Institution from 2005 to 2011 were reviewed. We analysed the data of pts who relapsed ≥10 years after initial diagnosis. Results: A total of 14 pts were identified. The primary site of origin was pleura (5 pts), pelvis (4 pts), head and neck (3 pts) and retroperitoneum (2 pts). Primary tumor was a typical SFT in 5 and a malignant SFT in 7 out of 12 pts whose tumor tissue was available for revision. The median time to first recurrence was 12 years (range 10–23). The first relapse was local in 11 cases, distant in 3. Five pts later developed distant metastases. Four out of 5 cases of typical SFT developed distant metastases in spite of their initial benign aspect. No patient was disease-free at the time of the analyses. Conclusion: Our series suggests that LR can occur in SFT and some cases can behave aggressively even in the absence of any primary morphologic evidence of malignancy. A prolonged follow-up may be advisable. Keywords: Sarcoma, Solitary fibrous tumor, Hemangiopericytoma, Outcome, Late recurrence Introduction entity, the term haemangiopericytoma will be deleted and Solitary fibrous tumor (SFT) is a rare soft tissue neoplasm, SFT will be classified as “typical” or “malignant” based on with an incidence of about 0,2/100.000/years. It was called number of mitosis, cellular atypia, presence of necrosis “haemangiopericytoma” by Stout and Murray in 1942. and hypercellularity [6]. However, the term “SFT” had been introduced by The anatomical origin of SFT is almost ubiquitous, as Klemperer and Rabin in 1931, being regarded as a kind of for soft tissue sarcomas in general. Indeed, SFT was pleural mesothelioma [1]. A consensus on the overlap be- originally described in the pleura and then thought to tween haemangiopericytoma and SFT was then developed originate from serosal surfaces [5,7]. Finally, it was in the ‘90s [2] (though the 2002 WHO “blue books” still reported in a set of other anatomical locations, going retain the two labels separately at least for some anatomical from the meninges to soft tissues [8,9]. locations) [3,4]. In practice, haemangiopericytoma and SFTs are classified in “typical” and “malignant” based on SFT are the same entity, whatever their site of origin, the mitotic count (< and ≥4/10 high-power microscopic and the former term is currently abandoned (the fields, respectively), the presence of necrosis and nuclear haemangiopericytoma-like histological pattern being a polymorphism. However, a strong correlation between non-specific feature shared by many neoplasms) [5]. morphology and clinical course is lacking, so that, as of In this regard, in the last WHO classification of bone today, there is no way to predict the outcome of a SFT and soft tissue sarcoma SFT will be a separate nosological based on its pathologic features. Indeed, we need such prognosticators, since SFT runs a * Correspondence: gbaldi80@gmail.com malignant course at least in 15-30% of cases [8,9]. Further- Department of Cancer Medicine, Adult Sarcoma Medical Oncology Unit, more, SFTs can rarely show an abrupt transition from Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, conventional SFTs to high-grade sarcoma, also called Italy Full list of author information is available at the end of the article © 2013 Baldi et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 2 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 “dedifferentiated” SFTs. These “dedifferentiated” lesions manufacturer’s protocol and antigen retrieval (6 at 95°C are aggressive soft tissue sarcomas [10]. 5 mM citrate buffer ph 6 for the first three Abs and 15 at Relatively few published series about SFT [11-19] are 95°5 mM citrate buffer ph6 for Ki-67). All cases were re- available. In general, they prove complete surgical resec- classified according to the updated criteria for the diagnosis tion, whenever possible, associated with a favourable long- of SFT, used at the time of the analyses [4,6,10,28]. term survival rate as opposed to incomplete excision. Type and timing of follow up examinations varied among Late recurrences are one of the clinical characteristics different Institutions. In the majority of patients, however, of SFT’s [20-27]. Therefore, we decided to search our total body CT-scan were repeated every six months until institutional database and we found 14 SFT patients the fifth year after the primary excision. Then, X-ray and/or who relapsed after ≥10 years from first complete surgery abdominal ultrasound (on the basis of the site of the and received medical treatment for the disease. This primary tumor) was repeated every year and CT scan was paper reports on these patients. repeated to confirm recurrence. Time to first recurrence was defined as the interval Patients and methods between the excision of the primary tumor and the time of Clinical records of all patients with the diagnosis of SFT first relapse (local and/or metastatic), detected and/or who were treated at the Cancer Medical Department confirmed by CT-scan. We estimated median OS from the of our institution from 2005 to 2011 were reviewed date of diagnosis and from the date of first relapse until retrospectively. We looked for patients who had relapsed death from any cause, using the Kaplan-Meier method [29]. after ≥10 years from initial diagnosis, irrespective of where This retrospective case series analysis was approved by primary tumor surgery was performed. the Institutional Ethics Committee. In addition, in order to estimate the frequency of late relapses in this histological sarcoma subtype, we also Results searched the institutional surgical database for all cases Patient characteristics are displayed in Table 1. of SFT surgically treated from 1995 to 2002. The A total of 9 SFT patients were identified in the institu- number of late relapses is provided in this series, while tional surgical database as having had a relapse at an the analysis of the clinical presentation and outcome of interval of ≥10 years from primary treatment. Only 3 of relapses is confined to patients treated at our institution. them are included in this analysis. The number of We selected only patients with initial complete surgery. patients in this database is 83, so the rate of late relapse We reviewed all initial surgical reports, applying current in our series is 10%. criteria for quality of surgery. Excisions were classified A total of 14 patients relapsing ≥10 years after complete according to the closest surgical margin, defining as R1 surgical resection of the primary tumor were identified those with microscopically infiltrated margins and R0 amongst all soft tissue sarcoma patients treated at our those with microscopically negative margins. institution. In 12 cases, the first pathologic diagnosis was subse- The primary site of origin was pleura in 5 patients, quently confirmed by experienced pathologists specialized pelvis in 4, head and neck in 3 (orbital region, ethmoid, in sarcomas. In 2 cases (case 3 and 14), primary tumor maxillary region), retroperitoneum in 2. No patient had tissuewas unavailablefor review dueto the long time a meningeal presentation, probably due to the referral interval. In all cases, we had a pathologic diagnosis of re- pattern of our institution. lapse (either following surgical excision or biopsy). Review All patients had extra-compartimental disease. Three of the initial tumor was made on tumor tissue samples patients received adjuvant radiotherapy (RT), with a from primary surgery in all cases. With regard to relapses, dose ranging from 45 to 60 Gy. No patient was treated tissue was available for all patients but 7, for whom fine with adjuvant chemotherapy. needle aspiration cytology (FNAC) was done (case 2, 3, 9, The median time to first recurrence was 12 years 10 and 14 for first relapses, and case 5, 7 and 14 for second (range: 10–23 years). Ten patients recurred locally relapses: see Table 1). In 9 cases, unstained sections were without metastatic disease, 1 patient experienced both local available in addition to hematoxilin/eosin (HE), so that the and distant relapse, 3 patients had a distant recurrence morphologic diagnosis was complemented with CD34/bcl2 without local relapse. in3cases,CD34/Ki-67in3,CD34in2.Inall 14 casesop- The 3 patients with distant recurrence only had previ- erated on at our institution (9 at first surgery and 5 at re- ously received adjuvant RT, as opposed to no patient lapse), we assessed CD34 (clone NCL-L-END, Novocastra; among those with local relapses. 1:200), bcl (Clone 124; DakoCytomation; 1:500), CD99 Among 11 patients who recurred locally (70% of the (clone MIC2-12E7; Dako; 1:200) and Ki-67 (clone MIB-1; cases in the current series), 9 were re-treated with surgery, Dakocytomation; 1:200), using Ultra Vision Quanto detec- consisting in a macroscopic complete excision in all cases. tion System HPR (Termo Scientific) according to Postoperative RT was added in 3 cases. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 3 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 Table 1 Patient characteristics and pathological/clinical findings Age at Type of surgery Years first Pattern of First relapse Histology Years second Pattern of Second relapse Histology Status Patient Gender Primary site Histology diagnosis (R0/R1/R2) relapse relapse surgery relapse relapse surgery last FU 1 M 52 pelvis MSFT R1 10y local Y MSFT 2y local Y MSFT DOD 2 M 28 head and neck MSFT R1 12y mts N MSFT 1y mts N NA DOD 3 M 48 pleura NA R1 11y mts N MSFT* 1y mts N NA LFU 4 M 31 pelvis MSFT R1 10y local Y MSFT 0y local Y MSFT AWD 5 M 61 pelvis MSFT R1 11y local Y MSFT 2y mts N MSFT* DOD 6 F 36 pleura SFT R1 10y local Y MSFT 3y local Y MSFT LFU 7 F 66 pelvis MSFT R1 11y local Y MSFT 1y mts N MSFT* LFU 8 F 27 head and neck MSFT R1 16y local Y MSFT 11y local Y MSFT AWD 9 F 46 retroperitoneum SFT R1 13y mts N PDSFT* 1y mts N NA DOD 10 F 53 pleura SFT R1 12y local + mts N MSFT* 1y local N NA AWD 11 M 41 pleura MSFT R1 20y local Y MSFT 1y local N NA LFU 12 M 43 pleura SFT R1 13y local Y MSFT 1y local N NA DOD 13 M 58 head and neck SFT R1 10y local Y MSFT 6y mts Y MSFT DOD 14 M 41 retroperitoneum NA R1 23y local N MSFT* 1y mts N MSFT* DOD *Histological diagnosis was performed by FNAC. SFT, typical solitary fibrous tumor; MSFT, malignant solitary fibrous tumor; PDSFT, pleomorphic/dedifferentiated solitary fibrous tumor. NA, not assessed; DOD, dead of disease; LFU, lost to follow-up; AWD, alive with disease. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 4 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 Systemic chemotherapy was administered in 6 patients On review of the 12 available primary tumor specimens, (3 with metastatic disease and 3 with local recurrence), SFT could be diagnosed as “typical” in 5 cases and using a regimen with anthracycline and ifosfamide in 3 “malignant” (MSFT) in 7. Typical cases were collagen-rich cases, high-dose continuous infusion ifosfamide in 2 and hypocellular, had a very low mitotic rate, and lacked cases, and cisplatin plus gemcitabine in 1 case. The best any evidence of cytological atypia or features recalling response by RECIST was a partial response in 1 case lipomatous or giant cell variants of SFT [5]. Four cases of (treated with anthracycline plus ifosfamide), stable typical SFT at first diagnosis had evidence of metastases at disease in 3 cases (treated with anthracycline plus their first or second relapse. ifosfamide and high dose ifosfamide in 2 cases) and pro- All cases with a typical STF at the time of the primary gression in 2 cases (treated with anthracycline plus tumor showed aspects consistent with a MSFT at the ifosfamide and cisplatin plus gemcitabine respectively). time of first and second relapse (Figure 3A-B-C-D), and All patients initially treated with surgery on the first one patient (#9) whose liver metastasis was assessed local recurrence subsequently relapsed, either locally or by FNAC had a dedifferentiated variant of SFT. This distantly in 6 and 3 cases, respectively. tumor had a growth pattern closely resembling an Overall, the median number of relapses per patient all Ewing sarcoma/pPNET and recapitulating the recently over their clinical history was 2.8 (range: 2–4). Six out of described round-cell dedifferentiated variant of SFT [10]. the 14 patients had more than one multifocal loco- The correct diagnosis was rendered after ruling out a regional recurrence (Figure 1A-B-C-D), while 8 devel- diagnosis of pPNET by FISH analysis and reviewing the oped distant metastases during the course of the disease. primary tumor, as we already reported [30]. Median OS from the first diagnosis was 19 years. Figure 2 displays the OS curves from relapse, for patients Discussion with local recurrence only (Figure 2A) and for those with We report on a series of 14 SFT patients who relapsed metastatic ± local recurrence (Figure 2B). after ≥10 years from initial diagnosis (median: 12 years) At the time of the last follow-up, all patients had and were treated thereof at our institution. Their median evidence of disease: 7 had died of disease; 3 were alive OS from first diagnosis was 19 years and median OS after 2, 5 and 14 years from the first relapse, respectively; from first relapse was 8 years. Most patients (70%) 4 patients were lost to follow-up. relapsed at the site of the primary tumor, with only 3 Figure 1 CT scans of primary solitary fibrous tumor of the pleura (1A-B) and the loco-regional relapse in mediastinal pleural (1C-D) after 12 years. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 5 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 patients recurring at distant sites without evidence of local relapse. Six patients developed metastatic disease only as late recurrence. No patient was cured, even amongst those undergoing complete salvage surgery of their first local recurrence. However, median OS was 8 years from relapse for all patients, with no major differ- ence between locally relapsing and metastatic patients. SFT represents a very rare disease and few studies are available on the natural history of this tumor. In particular, few cases of late relapses have been reported [20-26]. They were mostly meningeal and pleural SFTs. Interestingly, none of our patients had a meningeal origin, due to the re- ferral pattern of our institution, and some had other than pleural SFTs. Thus, we can conclude that late relapses are a feature of SFT as such, i.e. they are not confined to men- ingeal or pleural primary sites. This conclusion can be made even on a limited series of patients, picked up at a single institution on the basis of their unfavourable out- come: thus, selection biases should be taken into account when looking at this retrospective case series analysis. It is well known that currently available pathologic criteria for defining SFT “malignancy” are not satisfactory. Our series confirms that SFTs can have an aggressive be- haviour even in the absence of any morphologic evidence of malignancy at onset. Interestingly, all our patients showed signs of malignancy on relapse. This points to a pathologic evolution which takes place in relapsing SFTs, even when the relapse occurs late. In this sense, it is clear that we need to refine criteria for SFT classification, al- though available pathologic markers of malignancy clearly Figure 2 OS curves for patients with local recurrence only (2A) correspond to a malignant attitude, having being recorded and for those with metastatic ± local recurrence (2B). in all our relapses. Figure 3 Histopathological pattern of primary typical solitary fibrous tumor (3A) with low expression of Ki-67 (3B) and the malignant counterpart at relapse (3C) with high expression of Ki-67 (3D). Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 6 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 However, aside from those rare cases in which a frank tumor displayed “benign” features on pathologic assess- sarcomatous evolution is seen, the malignant features of ment. Current treatment strategies of relapse are clearly SFT are consistent with a low-aggressiveness tumor. The insufficient, though reports of activity of new targeted clinical counterpart of this conclusion is the long OS of therapies are now available [36-39] so that the outlook relapsing patients in our series, although the long previ- of the limited number of SFT patients who relapse may ous disease-free interval is obviously a bias selecting be due to improve in the next future. more indolent cases. Abbreviations The patterns of relapse we observed emphasize the SFT: Solitary fibrous tumor; MSFT: Malignant solitary fibrous tumor; inherent limitations of surgery in SFT, at least of some FNAC: Fine needle aspiration cytology; pPNET: Peripheral primitive neuroectodermal tumor; FISH: Fluorescence in situ hybridation; RT: Radiation typical anatomical sites. In fact, most of our patients first therapy; OS: Overall survival. recurred with a multinodular loco-regional relapse, and metastases appeared later on. As observed by many [31], Competing interest high rates of local failure are found in epidural, pleural and The authors have no competing interests to declare. pelvic/retroperitoneal SFT, while local failure are much Authors’ contributions rarer with SFT of soft tissues. In other words, SFT arising SS and GGB conceived the study and the design. GGB and ML carried out data in the pleural space, or retroperitoneum, or meninges, may collection and AM performed statistical analysis. VM, PS and APDT performed pathologic review and immunoistochemical analysis. GA and PGC helped to well tend to recur “locally” even when they have a benign draft the manuscript. All authors read and approved the final manuscript. aspect and are apparently resected in a complete manner, simply because of the inherent limitations of surgery in Acknowledgements such anatomical areas. Furthermore, “local” relapse in the We thank the following pathologists who kindly contributed case material: Roberto Fiocca (Istituto di Anatomia Patologica, Università di Genova), Fabio pleural space will inevitably lead to pleural dissemination, Facchetti (Dipartimento di Anatomia Patologica, Spedali Civili, Brescia), thus to a pattern of spread which is very similar to a “meta- Edgardo Bonacina (Dipartimento di Anatomia Patologica, Ospedale di Lecco). static” extent. This explains why none of our patients have We thank Lorenzo Fornaro (U.O. Oncologia, Ospedale Campo di Marte, Lucca) for writing assistance. been cured by salvage treatments, although relapses was loco-regional only in 7. In part, this may also explain why Author details pathologic prognostic criteria are unsatisfactory, the relapse Department of Cancer Medicine, Adult Sarcoma Medical Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, being related to surgical inherent inadequacy much more Italy. Department of Pathology, Experimental Molecular Pathology Unit, than to the tumor inherent aggressiveness. On the other Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, side, tumor relapse, whatever its cause, leads to the expres- Italy. Department of Anatomic Pathology, General Hospital of Treviso, P.zza Ospedale 1, 31100, Treviso, Italy. Department of Surgery, Fondazione IRCCS sion of pathologic markers of higher aggressiveness in all Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, Italy. patients. As said above, this is all the more meaningful in our series, which selected late relapses. 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Abstract

Background: We explore the pattern of late recurrence (LR) in solitary fibrous tumor (SFT), focusing on histopathologic characteristics, clinical presentation and patients (pts) outcome. Methods: Clinical records of all pts with confirmed pathologic diagnosis of SFT treated at our Institution from 2005 to 2011 were reviewed. We analysed the data of pts who relapsed ≥10 years after initial diagnosis. Results: A total of 14 pts were identified. The primary site of origin was pleura (5 pts), pelvis (4 pts), head and neck (3 pts) and retroperitoneum (2 pts). Primary tumor was a typical SFT in 5 and a malignant SFT in 7 out of 12 pts whose tumor tissue was available for revision. The median time to first recurrence was 12 years (range 10–23). The first relapse was local in 11 cases, distant in 3. Five pts later developed distant metastases. Four out of 5 cases of typical SFT developed distant metastases in spite of their initial benign aspect. No patient was disease-free at the time of the analyses. Conclusion: Our series suggests that LR can occur in SFT and some cases can behave aggressively even in the absence of any primary morphologic evidence of malignancy. A prolonged follow-up may be advisable. Keywords: Sarcoma, Solitary fibrous tumor, Hemangiopericytoma, Outcome, Late recurrence Introduction entity, the term haemangiopericytoma will be deleted and Solitary fibrous tumor (SFT) is a rare soft tissue neoplasm, SFT will be classified as “typical” or “malignant” based on with an incidence of about 0,2/100.000/years. It was called number of mitosis, cellular atypia, presence of necrosis “haemangiopericytoma” by Stout and Murray in 1942. and hypercellularity [6]. However, the term “SFT” had been introduced by The anatomical origin of SFT is almost ubiquitous, as Klemperer and Rabin in 1931, being regarded as a kind of for soft tissue sarcomas in general. Indeed, SFT was pleural mesothelioma [1]. A consensus on the overlap be- originally described in the pleura and then thought to tween haemangiopericytoma and SFT was then developed originate from serosal surfaces [5,7]. Finally, it was in the ‘90s [2] (though the 2002 WHO “blue books” still reported in a set of other anatomical locations, going retain the two labels separately at least for some anatomical from the meninges to soft tissues [8,9]. locations) [3,4]. In practice, haemangiopericytoma and SFTs are classified in “typical” and “malignant” based on SFT are the same entity, whatever their site of origin, the mitotic count (< and ≥4/10 high-power microscopic and the former term is currently abandoned (the fields, respectively), the presence of necrosis and nuclear haemangiopericytoma-like histological pattern being a polymorphism. However, a strong correlation between non-specific feature shared by many neoplasms) [5]. morphology and clinical course is lacking, so that, as of In this regard, in the last WHO classification of bone today, there is no way to predict the outcome of a SFT and soft tissue sarcoma SFT will be a separate nosological based on its pathologic features. Indeed, we need such prognosticators, since SFT runs a * Correspondence: gbaldi80@gmail.com malignant course at least in 15-30% of cases [8,9]. Further- Department of Cancer Medicine, Adult Sarcoma Medical Oncology Unit, more, SFTs can rarely show an abrupt transition from Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, conventional SFTs to high-grade sarcoma, also called Italy Full list of author information is available at the end of the article © 2013 Baldi et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 2 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 “dedifferentiated” SFTs. These “dedifferentiated” lesions manufacturer’s protocol and antigen retrieval (6 at 95°C are aggressive soft tissue sarcomas [10]. 5 mM citrate buffer ph 6 for the first three Abs and 15 at Relatively few published series about SFT [11-19] are 95°5 mM citrate buffer ph6 for Ki-67). All cases were re- available. In general, they prove complete surgical resec- classified according to the updated criteria for the diagnosis tion, whenever possible, associated with a favourable long- of SFT, used at the time of the analyses [4,6,10,28]. term survival rate as opposed to incomplete excision. Type and timing of follow up examinations varied among Late recurrences are one of the clinical characteristics different Institutions. In the majority of patients, however, of SFT’s [20-27]. Therefore, we decided to search our total body CT-scan were repeated every six months until institutional database and we found 14 SFT patients the fifth year after the primary excision. Then, X-ray and/or who relapsed after ≥10 years from first complete surgery abdominal ultrasound (on the basis of the site of the and received medical treatment for the disease. This primary tumor) was repeated every year and CT scan was paper reports on these patients. repeated to confirm recurrence. Time to first recurrence was defined as the interval Patients and methods between the excision of the primary tumor and the time of Clinical records of all patients with the diagnosis of SFT first relapse (local and/or metastatic), detected and/or who were treated at the Cancer Medical Department confirmed by CT-scan. We estimated median OS from the of our institution from 2005 to 2011 were reviewed date of diagnosis and from the date of first relapse until retrospectively. We looked for patients who had relapsed death from any cause, using the Kaplan-Meier method [29]. after ≥10 years from initial diagnosis, irrespective of where This retrospective case series analysis was approved by primary tumor surgery was performed. the Institutional Ethics Committee. In addition, in order to estimate the frequency of late relapses in this histological sarcoma subtype, we also Results searched the institutional surgical database for all cases Patient characteristics are displayed in Table 1. of SFT surgically treated from 1995 to 2002. The A total of 9 SFT patients were identified in the institu- number of late relapses is provided in this series, while tional surgical database as having had a relapse at an the analysis of the clinical presentation and outcome of interval of ≥10 years from primary treatment. Only 3 of relapses is confined to patients treated at our institution. them are included in this analysis. The number of We selected only patients with initial complete surgery. patients in this database is 83, so the rate of late relapse We reviewed all initial surgical reports, applying current in our series is 10%. criteria for quality of surgery. Excisions were classified A total of 14 patients relapsing ≥10 years after complete according to the closest surgical margin, defining as R1 surgical resection of the primary tumor were identified those with microscopically infiltrated margins and R0 amongst all soft tissue sarcoma patients treated at our those with microscopically negative margins. institution. In 12 cases, the first pathologic diagnosis was subse- The primary site of origin was pleura in 5 patients, quently confirmed by experienced pathologists specialized pelvis in 4, head and neck in 3 (orbital region, ethmoid, in sarcomas. In 2 cases (case 3 and 14), primary tumor maxillary region), retroperitoneum in 2. No patient had tissuewas unavailablefor review dueto the long time a meningeal presentation, probably due to the referral interval. In all cases, we had a pathologic diagnosis of re- pattern of our institution. lapse (either following surgical excision or biopsy). Review All patients had extra-compartimental disease. Three of the initial tumor was made on tumor tissue samples patients received adjuvant radiotherapy (RT), with a from primary surgery in all cases. With regard to relapses, dose ranging from 45 to 60 Gy. No patient was treated tissue was available for all patients but 7, for whom fine with adjuvant chemotherapy. needle aspiration cytology (FNAC) was done (case 2, 3, 9, The median time to first recurrence was 12 years 10 and 14 for first relapses, and case 5, 7 and 14 for second (range: 10–23 years). Ten patients recurred locally relapses: see Table 1). In 9 cases, unstained sections were without metastatic disease, 1 patient experienced both local available in addition to hematoxilin/eosin (HE), so that the and distant relapse, 3 patients had a distant recurrence morphologic diagnosis was complemented with CD34/bcl2 without local relapse. in3cases,CD34/Ki-67in3,CD34in2.Inall 14 casesop- The 3 patients with distant recurrence only had previ- erated on at our institution (9 at first surgery and 5 at re- ously received adjuvant RT, as opposed to no patient lapse), we assessed CD34 (clone NCL-L-END, Novocastra; among those with local relapses. 1:200), bcl (Clone 124; DakoCytomation; 1:500), CD99 Among 11 patients who recurred locally (70% of the (clone MIC2-12E7; Dako; 1:200) and Ki-67 (clone MIB-1; cases in the current series), 9 were re-treated with surgery, Dakocytomation; 1:200), using Ultra Vision Quanto detec- consisting in a macroscopic complete excision in all cases. tion System HPR (Termo Scientific) according to Postoperative RT was added in 3 cases. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 3 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 Table 1 Patient characteristics and pathological/clinical findings Age at Type of surgery Years first Pattern of First relapse Histology Years second Pattern of Second relapse Histology Status Patient Gender Primary site Histology diagnosis (R0/R1/R2) relapse relapse surgery relapse relapse surgery last FU 1 M 52 pelvis MSFT R1 10y local Y MSFT 2y local Y MSFT DOD 2 M 28 head and neck MSFT R1 12y mts N MSFT 1y mts N NA DOD 3 M 48 pleura NA R1 11y mts N MSFT* 1y mts N NA LFU 4 M 31 pelvis MSFT R1 10y local Y MSFT 0y local Y MSFT AWD 5 M 61 pelvis MSFT R1 11y local Y MSFT 2y mts N MSFT* DOD 6 F 36 pleura SFT R1 10y local Y MSFT 3y local Y MSFT LFU 7 F 66 pelvis MSFT R1 11y local Y MSFT 1y mts N MSFT* LFU 8 F 27 head and neck MSFT R1 16y local Y MSFT 11y local Y MSFT AWD 9 F 46 retroperitoneum SFT R1 13y mts N PDSFT* 1y mts N NA DOD 10 F 53 pleura SFT R1 12y local + mts N MSFT* 1y local N NA AWD 11 M 41 pleura MSFT R1 20y local Y MSFT 1y local N NA LFU 12 M 43 pleura SFT R1 13y local Y MSFT 1y local N NA DOD 13 M 58 head and neck SFT R1 10y local Y MSFT 6y mts Y MSFT DOD 14 M 41 retroperitoneum NA R1 23y local N MSFT* 1y mts N MSFT* DOD *Histological diagnosis was performed by FNAC. SFT, typical solitary fibrous tumor; MSFT, malignant solitary fibrous tumor; PDSFT, pleomorphic/dedifferentiated solitary fibrous tumor. NA, not assessed; DOD, dead of disease; LFU, lost to follow-up; AWD, alive with disease. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 4 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 Systemic chemotherapy was administered in 6 patients On review of the 12 available primary tumor specimens, (3 with metastatic disease and 3 with local recurrence), SFT could be diagnosed as “typical” in 5 cases and using a regimen with anthracycline and ifosfamide in 3 “malignant” (MSFT) in 7. Typical cases were collagen-rich cases, high-dose continuous infusion ifosfamide in 2 and hypocellular, had a very low mitotic rate, and lacked cases, and cisplatin plus gemcitabine in 1 case. The best any evidence of cytological atypia or features recalling response by RECIST was a partial response in 1 case lipomatous or giant cell variants of SFT [5]. Four cases of (treated with anthracycline plus ifosfamide), stable typical SFT at first diagnosis had evidence of metastases at disease in 3 cases (treated with anthracycline plus their first or second relapse. ifosfamide and high dose ifosfamide in 2 cases) and pro- All cases with a typical STF at the time of the primary gression in 2 cases (treated with anthracycline plus tumor showed aspects consistent with a MSFT at the ifosfamide and cisplatin plus gemcitabine respectively). time of first and second relapse (Figure 3A-B-C-D), and All patients initially treated with surgery on the first one patient (#9) whose liver metastasis was assessed local recurrence subsequently relapsed, either locally or by FNAC had a dedifferentiated variant of SFT. This distantly in 6 and 3 cases, respectively. tumor had a growth pattern closely resembling an Overall, the median number of relapses per patient all Ewing sarcoma/pPNET and recapitulating the recently over their clinical history was 2.8 (range: 2–4). Six out of described round-cell dedifferentiated variant of SFT [10]. the 14 patients had more than one multifocal loco- The correct diagnosis was rendered after ruling out a regional recurrence (Figure 1A-B-C-D), while 8 devel- diagnosis of pPNET by FISH analysis and reviewing the oped distant metastases during the course of the disease. primary tumor, as we already reported [30]. Median OS from the first diagnosis was 19 years. Figure 2 displays the OS curves from relapse, for patients Discussion with local recurrence only (Figure 2A) and for those with We report on a series of 14 SFT patients who relapsed metastatic ± local recurrence (Figure 2B). after ≥10 years from initial diagnosis (median: 12 years) At the time of the last follow-up, all patients had and were treated thereof at our institution. Their median evidence of disease: 7 had died of disease; 3 were alive OS from first diagnosis was 19 years and median OS after 2, 5 and 14 years from the first relapse, respectively; from first relapse was 8 years. Most patients (70%) 4 patients were lost to follow-up. relapsed at the site of the primary tumor, with only 3 Figure 1 CT scans of primary solitary fibrous tumor of the pleura (1A-B) and the loco-regional relapse in mediastinal pleural (1C-D) after 12 years. Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 5 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 patients recurring at distant sites without evidence of local relapse. Six patients developed metastatic disease only as late recurrence. No patient was cured, even amongst those undergoing complete salvage surgery of their first local recurrence. However, median OS was 8 years from relapse for all patients, with no major differ- ence between locally relapsing and metastatic patients. SFT represents a very rare disease and few studies are available on the natural history of this tumor. In particular, few cases of late relapses have been reported [20-26]. They were mostly meningeal and pleural SFTs. Interestingly, none of our patients had a meningeal origin, due to the re- ferral pattern of our institution, and some had other than pleural SFTs. Thus, we can conclude that late relapses are a feature of SFT as such, i.e. they are not confined to men- ingeal or pleural primary sites. This conclusion can be made even on a limited series of patients, picked up at a single institution on the basis of their unfavourable out- come: thus, selection biases should be taken into account when looking at this retrospective case series analysis. It is well known that currently available pathologic criteria for defining SFT “malignancy” are not satisfactory. Our series confirms that SFTs can have an aggressive be- haviour even in the absence of any morphologic evidence of malignancy at onset. Interestingly, all our patients showed signs of malignancy on relapse. This points to a pathologic evolution which takes place in relapsing SFTs, even when the relapse occurs late. In this sense, it is clear that we need to refine criteria for SFT classification, al- though available pathologic markers of malignancy clearly Figure 2 OS curves for patients with local recurrence only (2A) correspond to a malignant attitude, having being recorded and for those with metastatic ± local recurrence (2B). in all our relapses. Figure 3 Histopathological pattern of primary typical solitary fibrous tumor (3A) with low expression of Ki-67 (3B) and the malignant counterpart at relapse (3C) with high expression of Ki-67 (3D). Baldi et al. Clinical Sarcoma Research 2013, 3:4 Page 6 of 7 http://www.clinicalsarcomaresearch.com/content/3/1/4 However, aside from those rare cases in which a frank tumor displayed “benign” features on pathologic assess- sarcomatous evolution is seen, the malignant features of ment. Current treatment strategies of relapse are clearly SFT are consistent with a low-aggressiveness tumor. The insufficient, though reports of activity of new targeted clinical counterpart of this conclusion is the long OS of therapies are now available [36-39] so that the outlook relapsing patients in our series, although the long previ- of the limited number of SFT patients who relapse may ous disease-free interval is obviously a bias selecting be due to improve in the next future. more indolent cases. Abbreviations The patterns of relapse we observed emphasize the SFT: Solitary fibrous tumor; MSFT: Malignant solitary fibrous tumor; inherent limitations of surgery in SFT, at least of some FNAC: Fine needle aspiration cytology; pPNET: Peripheral primitive neuroectodermal tumor; FISH: Fluorescence in situ hybridation; RT: Radiation typical anatomical sites. In fact, most of our patients first therapy; OS: Overall survival. recurred with a multinodular loco-regional relapse, and metastases appeared later on. As observed by many [31], Competing interest high rates of local failure are found in epidural, pleural and The authors have no competing interests to declare. pelvic/retroperitoneal SFT, while local failure are much Authors’ contributions rarer with SFT of soft tissues. In other words, SFT arising SS and GGB conceived the study and the design. GGB and ML carried out data in the pleural space, or retroperitoneum, or meninges, may collection and AM performed statistical analysis. VM, PS and APDT performed pathologic review and immunoistochemical analysis. GA and PGC helped to well tend to recur “locally” even when they have a benign draft the manuscript. All authors read and approved the final manuscript. aspect and are apparently resected in a complete manner, simply because of the inherent limitations of surgery in Acknowledgements such anatomical areas. Furthermore, “local” relapse in the We thank the following pathologists who kindly contributed case material: Roberto Fiocca (Istituto di Anatomia Patologica, Università di Genova), Fabio pleural space will inevitably lead to pleural dissemination, Facchetti (Dipartimento di Anatomia Patologica, Spedali Civili, Brescia), thus to a pattern of spread which is very similar to a “meta- Edgardo Bonacina (Dipartimento di Anatomia Patologica, Ospedale di Lecco). static” extent. This explains why none of our patients have We thank Lorenzo Fornaro (U.O. Oncologia, Ospedale Campo di Marte, Lucca) for writing assistance. been cured by salvage treatments, although relapses was loco-regional only in 7. In part, this may also explain why Author details pathologic prognostic criteria are unsatisfactory, the relapse Department of Cancer Medicine, Adult Sarcoma Medical Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, being related to surgical inherent inadequacy much more Italy. Department of Pathology, Experimental Molecular Pathology Unit, than to the tumor inherent aggressiveness. On the other Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, side, tumor relapse, whatever its cause, leads to the expres- Italy. Department of Anatomic Pathology, General Hospital of Treviso, P.zza Ospedale 1, 31100, Treviso, Italy. Department of Surgery, Fondazione IRCCS sion of pathologic markers of higher aggressiveness in all Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milan, Italy. patients. As said above, this is all the more meaningful in our series, which selected late relapses. 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Clinical Sarcoma ResearchSpringer Journals

Published: Apr 3, 2013

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