POLSKI PRZEGLĄD CHIRURGICZNY 2014, 86, 7, 337–340
10.2478/pjs-2014-0059
Gigantic solitary fibrous tumour of extra-peritoneal space. A case report and review of the literature Tomasz Guzel1, Bohdan Dąbrowski1, Michał Mazurkiewicz2, Marcin Makiewicz1, Ireneusz W. Krasnodębski1 Department of General, Gastroenterological and Oncologic Surgery, Warsaw Medical University1 Kierownik: prof. dr hab. I. W. Krasnodębski Department of Pathomorphology, Warsaw Medical University2 Kierownik: prof. dr hab. B. Górnicka
Solitary fibrous tumour (SFT) is a rare soft tissue tumour which belongs to fibroblastic/myofibroblastic group of tumours. The most often it appears in pleura, also in pericardium, internal organs, peritoneum and extraperitoenal space. SFT was first described as a new type of pleura’s tumour by Klemperer and Rabin in 1931. The histogenesis of SFT’s has been discussed for years suggesting its mesothelial origin. Recently, SFT has been classified as a mesenchymal fibroblastic tumour. We report a very rare case of 71-year old man suffering from gigantic solitary fibrous tumour of extraperitoneal space who underwent curative surgery in the Department of General, Gastroenterological and Oncologic Surgery in 2011. Key words: solitary fibrous tumour, SFT
Solitary fibrous tumour (SFT) is a rare soft tissue tumour which has been described in pleura by Klemperer and Rabin in 1931 (1). According to the WHO classification SFT is a mesenchymaltumour that belongs to fibroblastic/myofibroblastic group. Clinicallyis slow growing neoplasm that appears in mid-aged patients, independently of sex. Course of disease is unpredictable. The most common localisation is pleura but SFT was also described in different localisation such as peritoneum and extarperitoneal space or mesentery (2).
was revealed by ultrasonography, computer tomography (CT) scan confirmed its large diameters: 224x136x256 mm. CT scan also showed necrotic sites and calcinosis, strong impression on abdominal part of aorta (fig. 1). Radiologist’s suggestion was sarcoma with no defined source in abdominal cavity. After on-
Case report 71-years old patient was admitted to the Department of General, Gastroenterologic and Oncologic Surgery because of abdominal mass revealed by palpation of abdominal cavity. The main complain was constipation and leg swelling for about 2 months before admission. Rising abdominal diameter forced patient to perform GP visit. Tumour in abdominal cavity
Fig. 1. Computer tomography of the tumour Unauthenticated Download Date | 5/15/17 6:20 PM
338
T. Guzel et al.
cologic consultation patient was transferred to the Department of General, Gastroenterological and Oncologic Surgery for further examination. On admission he was in good general condition, pain negative, with anemia (Hb 11.6 g/dl) and increased Fibryn level up 505 mg/dl and C-reactive protein (CRP) up 14.3 mg/l. On examination atrial flotation was confirmed. After cardiologic consultation and echocardiography (ECHO) investigation patient was qualified for operation on schedule. Before operation patient got antithrombotic and antibiotic prophylaxis as follows: Deltaparin 5000 iu (1 day before), Cefazolin 1 g (30 minutes before incision). Surgical treatment During operation abdominal mass of about 6-8 kilograms weight filling all abdominal cavity was confirmed (fig. 2). There was no other internal organs infiltration. Tumour’s surface
was coated by many pathologically enlarged vessels with heavy bleeding on touch (fig. 3). Tumour was extirpated, it came out of retroperitoneal space (fig. 4). On the same day, late night, patient was reoperated because of heavy intra abdominal bleeding. On investigation during laparotomy source of bleeding was not found but many vessels were assessed to be at high risk of recurrent bleeding and preserved. Patient got 6 units of fresh frozen plasma and 6 units of packed red blood cells. During postoperative period there was prolonged bowel immobilisation, full oral diet was implemented on day 6th, diet was well tolerated. Patient was discharged on 28th day of hospitalization and 19th day after operation, clinically in very good condition. Histopathological report of excited tumour revealed: solitary fibrous tumour, immunohistochemically CD34(+) (fig. 5), CD117(-), actin (-), desmin (-), S100 (-), CD31(-), CD99(+) (fig. 6), bcl2 (+). Low mithotic activity was confirmed by proliferation index MIB 3%, focuses of necrosis (fig. 7), nuclear polymorphism could indicated malignant disease in this case. Follow-up period is negative, up till April 2013 patient is in good condition, with no complains.
Fig. 2. Tumour after skin incision
Fig. 3. Enlarged blood vesels on tumour’s surface
Fig. 4. Cross section of the tumour
Fig. 5. Histopathology: CD34 positive Unauthenticated Download Date | 5/15/17 6:20 PM
Gigantic solitary fibrous tumour of extra-peritoneal space. A case report and review of the literature
339
Fig. 7. Histopathology: focuses of necrosis Fig. 6. Histopathology: CD99 positive
Discussion First histopathological solitary fibrous tumour characteristics as a pleural tumour was published in 1870 by Wagner. In 1931 Klemperer and Rabin described SFT as differentiated serosal origin and in 1942 Stout and Murray as mesothelial tumour. Today, it is widely confirmed that SFT is fibroblastic mesodermal neoplasm with its characteristic branched vessels that makes it similar to hemagiopericitoma (HPC) tumours. Despite similar immunophenotype SFT is histhopathologically different than HPC, has no organized architecture. Typical for SFT is glazing in matrix and around vessels, rich presence of colagen fibre. Similar to HPC it shows expression of CD34 (80-90%), CD99 (70%), rarely bcl-2 (30%), epithelial membrane antigen (EMA, 30%) and smooth muscle actin (SMA, 20%). Usually, there is no expression of S100 protein, desmin andcytokeratin. Probably, because of unclear SFT and HPC differentiation previously, many HPC tumours would be recognised as SFT today. During last 10 years, because of high interest in these types of tumours, they became better recognized, new classifications, subclassification and prognostic markers were established. There were also different types of SFT distinguished, such as fibrous, celullar, adipose or gigantocelullar tumour (3). SFT origin can exist not only in serosal localisation like pleura, pericardium or peritoneum but in soft tissues and visceral organs also (4). During last 20 years this kind of tumour was diagnosed in extra-pleural localisation mainly (5).
Clinically, SFT of soft tissue is a slow-rising tumour, expressed in medium-aged person, with no sex relationship (6). It can reach over 10cm diameter, that can cause pressure and dysfunction of different organs. Sometimes it can give paraneoplastic signs as hypoglicemy because of insulin-like growth factor (IGF) secretion (7). The most common extra-pleural localisation is retroperitoneal and peritoneal space, soft tissues of extremities and head. Disease progression remains to be unpredictable. 10-15% of all cases aremalignant disease, with metastases to lungs, liver and bones. Malignancy signs of tumour are diameter over 5 cm, high proliferation index MIB (over 2%), nuclear polymorphism, focuses of necrosis, high mithotic index (over 4 mitoses/10 FOV). Unfortunately, in described case histhopathological investigation revealed almost all of these signs what claim to be a negative prognostic factor. Solitary fibrous tumours are published rarely. Yamashita et al. described tumour 15x14x10 cm confirmed as SFT. There was low nuclear polymorphism and mitotic activity (0-2 mitoses/10 FOV), proliferation index 2. During 26 moths follow-up period authors didn’t confirmed recurrent or metastatic disease (8). Similar tumour, in 24-years old woman described Cristi et al. (9). Despite benign neoplasm confirmed by histopthology examination authors proposes very strict and long lasting follow-up period because of unpredictable disease progress and high risk of very late recurrence rate. Lau et al described 53-years old man who was suffering from groin pain, with nausea, lack of appetite, diarrhoea. CT scan revealed 22x18x11 cm size tumour in right iliac fossa. During operation investigation showed tumour in distal ileum mesentery (10). Unauthenticated Download Date | 5/15/17 6:20 PM
340
T. Guzel et al.
Similar tumour was described by Bouhabel et al. in 71-years old woman. Both authors maintain that these were two first publications about SFT in mesenteric localisation. Thanks to improvement of diagnostic methods there are much higher extra-pleural SFT identification. Solitary fibrous tumour is a low-
signed and potentially benign neoplasm but there is no assessed malignancy conversion rate, metastases appearance and recurrence rate risk. Patients after curative SFT operation should be under strict oncologic supervision, in the same way as patients with malignant disease confirmation.
references 1. Klemperer P, Rabin CB: Primary neoplasm of the pleura: a report of five cases. Arch Pathol 1931; 11: 385-412. 2. Bouhabel S, Leblanc G, Ferreira J et al.: Solitary fibrous tumour arising in the mesentery: a case report. W J Surg Oncol 2011; 9: 140. 3. Gengler C, Guillou L: Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology 2006: 48: 63-74. 4. Hasegawa T, Matsuno Y, Shimodfa T et al.: Extrathoracic solitary fibrous tumours: their histological variabilityand potentially aggressive behaviour. Human Pathol 1999; 30, 12: 1464-73. 5. Musyoki FN, Nahal A, Powell TI: Solitary fibrous tumour: an update on the spectrum of extrapleural manifestations. Skeletal Radiol 2012; 41: 5-13.
6. Pływaczewski R, Hawryłkeiwcz I, Langfort R i wsp.: Odosobniony guz włóknisty opłucnej u 75letniej chorej. Pneumonol Alergol 2004; 72: 32-36. 7. Nagase T, Adachi I, Yamada T et al.: Solitary fibrous tumour in the pelvic cavity with hypoglicemia: report of a case. Surg Today 2005; 35: 181-84. 8. Yamashita S, Tochigi T, Kawamura S et al.: A case of retroperitoneal solitary fibrous tumour. Act Urol 2007; 53: 477-80. 9. Cristi E, Perrone G, Battista C et al.: A rare case of solitary fibrous tumor of the pre-sacral space: morphological and immunohistochemical features. In Vivo 2005; 19: 777-80. 10. Lau MI, Foo FJ, Sissons MC et al.: Solitary fibrous tumour of small bowel mesentery: a case report and review of the literature. Tumori 2010; 96: 1035-39.
Received: 5.08.2013 r. Adress correspondence: 02-097 Warszawa, ul. Banacha 1a e-mail: [email protected]
Unauthenticated Download Date | 5/15/17 6:20 PM
10.2478/pjs-2014-0059
Gigantic solitary fibrous tumour of extra-peritoneal space. A case report and review of the literature Tomasz Guzel1, Bohdan Dąbrowski1, Michał Mazurkiewicz2, Marcin Makiewicz1, Ireneusz W. Krasnodębski1 Department of General, Gastroenterological and Oncologic Surgery, Warsaw Medical University1 Kierownik: prof. dr hab. I. W. Krasnodębski Department of Pathomorphology, Warsaw Medical University2 Kierownik: prof. dr hab. B. Górnicka
Solitary fibrous tumour (SFT) is a rare soft tissue tumour which belongs to fibroblastic/myofibroblastic group of tumours. The most often it appears in pleura, also in pericardium, internal organs, peritoneum and extraperitoenal space. SFT was first described as a new type of pleura’s tumour by Klemperer and Rabin in 1931. The histogenesis of SFT’s has been discussed for years suggesting its mesothelial origin. Recently, SFT has been classified as a mesenchymal fibroblastic tumour. We report a very rare case of 71-year old man suffering from gigantic solitary fibrous tumour of extraperitoneal space who underwent curative surgery in the Department of General, Gastroenterological and Oncologic Surgery in 2011. Key words: solitary fibrous tumour, SFT
Solitary fibrous tumour (SFT) is a rare soft tissue tumour which has been described in pleura by Klemperer and Rabin in 1931 (1). According to the WHO classification SFT is a mesenchymaltumour that belongs to fibroblastic/myofibroblastic group. Clinicallyis slow growing neoplasm that appears in mid-aged patients, independently of sex. Course of disease is unpredictable. The most common localisation is pleura but SFT was also described in different localisation such as peritoneum and extarperitoneal space or mesentery (2).
was revealed by ultrasonography, computer tomography (CT) scan confirmed its large diameters: 224x136x256 mm. CT scan also showed necrotic sites and calcinosis, strong impression on abdominal part of aorta (fig. 1). Radiologist’s suggestion was sarcoma with no defined source in abdominal cavity. After on-
Case report 71-years old patient was admitted to the Department of General, Gastroenterologic and Oncologic Surgery because of abdominal mass revealed by palpation of abdominal cavity. The main complain was constipation and leg swelling for about 2 months before admission. Rising abdominal diameter forced patient to perform GP visit. Tumour in abdominal cavity
Fig. 1. Computer tomography of the tumour Unauthenticated Download Date | 5/15/17 6:20 PM
338
T. Guzel et al.
cologic consultation patient was transferred to the Department of General, Gastroenterological and Oncologic Surgery for further examination. On admission he was in good general condition, pain negative, with anemia (Hb 11.6 g/dl) and increased Fibryn level up 505 mg/dl and C-reactive protein (CRP) up 14.3 mg/l. On examination atrial flotation was confirmed. After cardiologic consultation and echocardiography (ECHO) investigation patient was qualified for operation on schedule. Before operation patient got antithrombotic and antibiotic prophylaxis as follows: Deltaparin 5000 iu (1 day before), Cefazolin 1 g (30 minutes before incision). Surgical treatment During operation abdominal mass of about 6-8 kilograms weight filling all abdominal cavity was confirmed (fig. 2). There was no other internal organs infiltration. Tumour’s surface
was coated by many pathologically enlarged vessels with heavy bleeding on touch (fig. 3). Tumour was extirpated, it came out of retroperitoneal space (fig. 4). On the same day, late night, patient was reoperated because of heavy intra abdominal bleeding. On investigation during laparotomy source of bleeding was not found but many vessels were assessed to be at high risk of recurrent bleeding and preserved. Patient got 6 units of fresh frozen plasma and 6 units of packed red blood cells. During postoperative period there was prolonged bowel immobilisation, full oral diet was implemented on day 6th, diet was well tolerated. Patient was discharged on 28th day of hospitalization and 19th day after operation, clinically in very good condition. Histopathological report of excited tumour revealed: solitary fibrous tumour, immunohistochemically CD34(+) (fig. 5), CD117(-), actin (-), desmin (-), S100 (-), CD31(-), CD99(+) (fig. 6), bcl2 (+). Low mithotic activity was confirmed by proliferation index MIB 3%, focuses of necrosis (fig. 7), nuclear polymorphism could indicated malignant disease in this case. Follow-up period is negative, up till April 2013 patient is in good condition, with no complains.
Fig. 2. Tumour after skin incision
Fig. 3. Enlarged blood vesels on tumour’s surface
Fig. 4. Cross section of the tumour
Fig. 5. Histopathology: CD34 positive Unauthenticated Download Date | 5/15/17 6:20 PM
Gigantic solitary fibrous tumour of extra-peritoneal space. A case report and review of the literature
339
Fig. 7. Histopathology: focuses of necrosis Fig. 6. Histopathology: CD99 positive
Discussion First histopathological solitary fibrous tumour characteristics as a pleural tumour was published in 1870 by Wagner. In 1931 Klemperer and Rabin described SFT as differentiated serosal origin and in 1942 Stout and Murray as mesothelial tumour. Today, it is widely confirmed that SFT is fibroblastic mesodermal neoplasm with its characteristic branched vessels that makes it similar to hemagiopericitoma (HPC) tumours. Despite similar immunophenotype SFT is histhopathologically different than HPC, has no organized architecture. Typical for SFT is glazing in matrix and around vessels, rich presence of colagen fibre. Similar to HPC it shows expression of CD34 (80-90%), CD99 (70%), rarely bcl-2 (30%), epithelial membrane antigen (EMA, 30%) and smooth muscle actin (SMA, 20%). Usually, there is no expression of S100 protein, desmin andcytokeratin. Probably, because of unclear SFT and HPC differentiation previously, many HPC tumours would be recognised as SFT today. During last 10 years, because of high interest in these types of tumours, they became better recognized, new classifications, subclassification and prognostic markers were established. There were also different types of SFT distinguished, such as fibrous, celullar, adipose or gigantocelullar tumour (3). SFT origin can exist not only in serosal localisation like pleura, pericardium or peritoneum but in soft tissues and visceral organs also (4). During last 20 years this kind of tumour was diagnosed in extra-pleural localisation mainly (5).
Clinically, SFT of soft tissue is a slow-rising tumour, expressed in medium-aged person, with no sex relationship (6). It can reach over 10cm diameter, that can cause pressure and dysfunction of different organs. Sometimes it can give paraneoplastic signs as hypoglicemy because of insulin-like growth factor (IGF) secretion (7). The most common extra-pleural localisation is retroperitoneal and peritoneal space, soft tissues of extremities and head. Disease progression remains to be unpredictable. 10-15% of all cases aremalignant disease, with metastases to lungs, liver and bones. Malignancy signs of tumour are diameter over 5 cm, high proliferation index MIB (over 2%), nuclear polymorphism, focuses of necrosis, high mithotic index (over 4 mitoses/10 FOV). Unfortunately, in described case histhopathological investigation revealed almost all of these signs what claim to be a negative prognostic factor. Solitary fibrous tumours are published rarely. Yamashita et al. described tumour 15x14x10 cm confirmed as SFT. There was low nuclear polymorphism and mitotic activity (0-2 mitoses/10 FOV), proliferation index 2. During 26 moths follow-up period authors didn’t confirmed recurrent or metastatic disease (8). Similar tumour, in 24-years old woman described Cristi et al. (9). Despite benign neoplasm confirmed by histopthology examination authors proposes very strict and long lasting follow-up period because of unpredictable disease progress and high risk of very late recurrence rate. Lau et al described 53-years old man who was suffering from groin pain, with nausea, lack of appetite, diarrhoea. CT scan revealed 22x18x11 cm size tumour in right iliac fossa. During operation investigation showed tumour in distal ileum mesentery (10). Unauthenticated Download Date | 5/15/17 6:20 PM
340
T. Guzel et al.
Similar tumour was described by Bouhabel et al. in 71-years old woman. Both authors maintain that these were two first publications about SFT in mesenteric localisation. Thanks to improvement of diagnostic methods there are much higher extra-pleural SFT identification. Solitary fibrous tumour is a low-
signed and potentially benign neoplasm but there is no assessed malignancy conversion rate, metastases appearance and recurrence rate risk. Patients after curative SFT operation should be under strict oncologic supervision, in the same way as patients with malignant disease confirmation.
references 1. Klemperer P, Rabin CB: Primary neoplasm of the pleura: a report of five cases. Arch Pathol 1931; 11: 385-412. 2. Bouhabel S, Leblanc G, Ferreira J et al.: Solitary fibrous tumour arising in the mesentery: a case report. W J Surg Oncol 2011; 9: 140. 3. Gengler C, Guillou L: Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology 2006: 48: 63-74. 4. Hasegawa T, Matsuno Y, Shimodfa T et al.: Extrathoracic solitary fibrous tumours: their histological variabilityand potentially aggressive behaviour. Human Pathol 1999; 30, 12: 1464-73. 5. Musyoki FN, Nahal A, Powell TI: Solitary fibrous tumour: an update on the spectrum of extrapleural manifestations. Skeletal Radiol 2012; 41: 5-13.
6. Pływaczewski R, Hawryłkeiwcz I, Langfort R i wsp.: Odosobniony guz włóknisty opłucnej u 75letniej chorej. Pneumonol Alergol 2004; 72: 32-36. 7. Nagase T, Adachi I, Yamada T et al.: Solitary fibrous tumour in the pelvic cavity with hypoglicemia: report of a case. Surg Today 2005; 35: 181-84. 8. Yamashita S, Tochigi T, Kawamura S et al.: A case of retroperitoneal solitary fibrous tumour. Act Urol 2007; 53: 477-80. 9. Cristi E, Perrone G, Battista C et al.: A rare case of solitary fibrous tumor of the pre-sacral space: morphological and immunohistochemical features. In Vivo 2005; 19: 777-80. 10. Lau MI, Foo FJ, Sissons MC et al.: Solitary fibrous tumour of small bowel mesentery: a case report and review of the literature. Tumori 2010; 96: 1035-39.
Received: 5.08.2013 r. Adress correspondence: 02-097 Warszawa, ul. Banacha 1a e-mail: [email protected]
Unauthenticated Download Date | 5/15/17 6:20 PM
