Solitary Fibrous Tumor of the Liver

international journal of surgery 6 (2008) 396–399

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Review Solitary ﬁbrous tumor of the liver: Report of a rare case and review of the literature

M.V. Perini*, P. Herman, L.A.C. D’Albuquerque, W.A. Saad

University of Sao Paulo School of Medicine, Department of Gastroenterology, Digestive Surgery Division, Sa˜o Paulo, Sa˜o Paulo, Brazil article info abstract

Article history: Solitary ﬁbrous tumor of the liver is extremely rare, with only 38 cases reported in the lit- Received 15 September 2007 erature. We present one case of a SFT originating from the caudate lobe of the liver, treated Received in revised form 30 by surgical resection and review the previous reported cases. September 2007 ª 2007 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. Accepted 3 October 2007 Published online 25 October 2007

Keywords: Fibroma Liver neoplasm Surgery

1. Introduction 2. Case report

Solitary fibrous tumors (SFTs) also known as localized fibrous A 40-year-old woman with six months history of mild upper mesothelioma, localized fibroma, localized fibrous tumor or abdominal pain was referred for evaluation. At physical and solitary fibrous tumor, are rare tumors with a controversial or- laboratory examination no abnormality was found. Ultrasound 1,2 igin. Most often the origin is the pleura but occasionally the (US) and computed tomographic scan (CT) disclosed a big mass, mediastinum, peritoneum or mesentery. SFTs of the liver are 10 cm in diameter, hyperechoic at US and hypodense at CT, extremely rare and in a literature review, only 38 cases have involving the caudate lobe and segments II and III of the 1–29 been reported (Table 1). Pre-operative diagnosis is very dif- liver (Fig. 1). Hepatic scintigraphy with DISIDA showed a non- ficult and sometimes surgery is necessary to exclude malig- uptaking area in the left lobe of the liver. Surgery was indicated nant disease or to relief symptoms. Herein, we present a case to relief symptoms and to preclude a malignant mass. of a solitary fibrous tumor originating from the caudate lobe Laparotomy revealed a large, hard and well delimited tu- of the liver, successfully treated by surgical resection. mor arising from the caudate lobe, compressing the left lateral

* Corresponding author. Marcos Vinicius Perini, M.D.R. Pernambuco, 120 apto 5, 01240-020 Sao Paulo, Brazil. Tel.: þ55 1181797885. E-mail address: [email protected] (M.V. Perini). 1743-9191/$ – see front matter ª 2007 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.ijsu.2007.10.004 international journal of surgery 6 (2008) 396–399 397

Table 1 – Data from the previous reported cases in the literature, including the present case N Ref. Year Age/gender Treatment Size (cm) Lobe IH Follow-up

1 Edmondson 1958 16/f Res 23 R na 24 ms 2 Edmondson 1958 na Res 5 R na na 3 Nevius 1959 56/m Rdt 15 R na 2 days 4 Ishak 1976 62/m Res 24 L na da 5 Ishak 1976 62/f Res 23 L na Died 6 Kim 1983 27/f Res 27 L na 6 ms 7 Kottke-Marchand 1989 84/f Res 15 L na 29 ms 8 Kasano 1991 39/f Res 18 L na 53 ms 9 Barnoud 1996 50/m Res 17 R Cd34þ 38 ms 10 Levine 1997 57/m Res 18 L Cd34þ na 11 Gugliemi 1998 61/f Res 26 R Cd34þ 72 12 Moran 1998 62/f Res 23 na Cd34þ na 13 Moran 1998 34/f No 2 na Cd34þ Autopsy 14 Moran 1998 57/f Res 24 na Cd34þ na 15 Moran 1998 32/m Res 12 na Cd34þ na 16 Moran 1998 68/f Res 17 na Cd34þ 2d 17 Moran 1998 83/f Res 18 R Cd34þ 6d 18 Moran 1998 72/f Res 9 L Cd34þ 12 ms 19 Moran 1998 62/m Res 24 L Cd34þ na 20 Moran 1998 50/f Res 3 L Cd34þ na 21-23 Fuksbrumer 2000 40/71/80 f/f/m Res/res/no 14–17 R Cd34þ na 24 Yilmaz 2000 25/f Res 32 R Vimentinþ 6ms 25 Lin 2001 75/m Res 21 R Cd34þ 11 ms 26 Saint-Marchand 2002 69/f Res 23 R Cd34þ 15 ms 27 Chithriki 2004 75/f Res 20 L Cd34þ 11 ms 28 Neef 2004 63/f Res 30 R Cd34þ 6ms 29 Venarrecci 2005 65/f Res 30 R Cd34þ 30 ms 30 Ji 2006 46/f Res 6 R Cd34þ na 31 Lehmann 2006 63/f Res na R Cd34þ 8y 32 Tsuro 2006 54/m No na D Cd34þ na 33 Nath 2006 65/f Res 30 R Cd34þ 10 ms 34 Terkivatan 2006 74/m Res 24 L Cd34þ 12 ms 35–37 Weitz 2007 na Res/no/no na na na na 38 Obuz 2007 52/m Res 12 L Cd34þ 22 ms 39 Present case 2007 40/f Res 10 L Cd34þ 49 ms

na, not available; rdt, radiation; res, resection; no, not operated; f, female; m, male; r, right; l, left; d, difuse; ms, months; y, years; died, died in the operation room. segment of the liver. The mass vascular supply was from the spindle cell tumor, with no pleomorphism, mitosis or inva- left hepatic artery. A left lateral segment and caudate lobe of sion. Immunohistochemical evaluation disclosed positivity the liver resection were performed. The tumor cut surface for vimentin, CD 34 and was negative for keratin. The diagno- showed a light-yellow colored fibrous tissue with a thin sis was of a solitary fibrous tumor of the liver. Postoperative capsula. Microscopic examination revealed a fibroblast and course was uneventful, with hospital discharge at the ninth day. After 49 months of follow-up, the patient is disease free.

3. Discussion

Solitary fibrous tumor (SFT) most frequently originates from the pleura and occasionally from the peritoneum, mediastinum or mesentery.1 Almost all cases are benign but malignant behavior has been reported. SFTs are defined as large, multi- nodular benign spindle cell tumors but there is a lack of an accurate classification due to the rarity of the disease. In the English literature, there are only 38 reported cases of SFT originating from the liver. Recent studies with immunohistochemical evaluation suggest that SFT is a primitive mesenchymal cell neoplasm with multidirectional differentiation. Some Fig. 1 – CT scan depicts a hypodense well delimited mass authors suggested that liver irradiation can induce fibrotic compromising the caudate lobe of the liver. transformation into SFT.26 Gross examination shows firm 398 international journal of surgery 6 (2008) 396–399

large tumors with a nodular surface covered by an external At histologic evaluation it is possible to predict a likelihood capsula and at cut surface whorls of fibrous gray to white tis- of recurrence and metastasis based on mitosis count: more sue are found. Microscopic examination reveals fibrous tissue than 4/10 HPF suggests malignancy and high rate of metasta- with fibroblast-like cells, within collagenous strands. Small ses.1,2 Recurrence rate cannot be exactly determined due to and spindle cells with tapered nuclei arranged in bundles the rarity of the disease. Tumor size has been suggested as are also found. Mitotic figures are occasionally present but an important factor affecting recurrence.1,2 Intra-thoracic there is no evidence of pleomorphism or nuclear atypia.6 Im- SFT presents local recurrence and distant metastatic rates of munohistochemical evaluation shows positivity for vimentin up to 15%; extra-thoracic tumors with malignant behaviour (a marker for mesenchymal origin cells), CD 34 (a marker for can be found in approximately 6% of the cases.1,10,20 Long pluripotent stem cells) and neurofilament 68 kDa.9 term follow-up, looking for local and distant metastasis, is al- Data from reported cases are summarized in Table 1. The ways necessary.1 tumor was observed predominantly in middle age females (mean age ¼ 50 years), arising mainly from the left lobe of Conflict of interest the liver. The female:male ratio is 2.2:1 and, in all cases tumors The authors have no financial and personal relationships with were large, measuring from 5 to 33 cm. 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