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  Table of Contents    
CASE REPORT  
Year : 2012  |  Volume : 55  |  Issue : 2  |  Page : 236-238
Hepatic solitary fibrous tumor: Report of a rare case


1 Department of Pathology, Global Hospital and Health City, Chennai, Tamil Nadu, India
2 Department of Liver Transplantation and Hepatobiliary Surgery, Global Hospital and Health City, Chennai, Tamil Nadu, India

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Date of Web Publication3-Jul-2012
 

   Abstract 

Solitary fibrous tumor (SFT) of the liver is an extremely rare neoplasm of mesenchymal origin. In the English literature, less than 40 cases of SFTs of the liver have been reported. The present case concerns a 34-year-old female who presented to us with complaint of dyspepsia. On examination, there was hepatomegaly. On ultrasound examination, an SOL in the liver was detected. Large tumor measuring 14.5 × 10 × 8 cm was resected. Microscopic evaluation of the tumor showed a well-circumscribed, low to moderately cellular tumor demonstrating spindle- and fibroblast-like cells within the collagenous stroma. Immunohistochemistry revealed diffuse strong cytoplasmic immunopositivity of CD34, Bcl2, and vimentin. A diagnosis of a benign SFT was given. The patient remained well 4 years after surgery. SFT is a rare mesenchymal neoplasm that occasionally involves the liver in adult patients. Most SFTs are benign, but some may have malignant histological features. With less than 40 reported cases in the literature, little can be said regarding its natural history or the benefits of adjuvant radio chemotherapy. Complete surgical resection remains the cornerstone of its treatment.

Keywords: Hepatic, immunohistochemistry, solitary fibrous tumor

How to cite this article:
Patra S, Vij M, Venugopal K, Rela M. Hepatic solitary fibrous tumor: Report of a rare case. Indian J Pathol Microbiol 2012;55:236-8

How to cite this URL:
Patra S, Vij M, Venugopal K, Rela M. Hepatic solitary fibrous tumor: Report of a rare case. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Feb 20];55:236-8. Available from: http://www.ijpmonline.org/text.asp?2012/55/2/236/97892



   Introduction Top


Solitary fibrous tumor (SFT) is a rare spindle cell mesenchymal neoplasm that occurs preferentially in the pleura. It has also been described in meninges, orbit, upper respiratory tract, breast, thyroid, and peritoneum. Rarely, SFT may arise from the liver parenchyma of adult patients. [1] In the English literature, less than 40 cases of SFTs of the liver have been reported. [2],[3],[4],[5],[6] Although most SFT in any location behave in benign fashion, others may possess malignant histologic features, recur locally, and metastasize. [6] Clinical findings are not specific and cannot exclude malignancy. The imaging features of benign and malignant SFT of the liver appear to overlap. Preoperative cytology may be inconclusive or misleading. Histopathology and immunohistochemsitry, including CD34 and Bcl-2, should be used to precisely diagnose SFT. [1],[2],[3],[4],[5] Surgical resection is a plausible therapeutic option in large SFTs of the liver. [7] We report a case of SFT of the liver and review the literature.


   Case Report Top


The present case concerns a 34-year-old female who presented to Department of Hepatobiliary Surgery with complaint of dyspepsia. There was no history of jaundice or change in appetite and weight loss. There was no history of blood transfusion. The patient was a known case of asthma. On physical examination, mild abdominal distension was present. Palpation revealed firm mass in the right hypochondrium and epigastrium. There was no evidence of ascites, edema, hepatic failure, or cirrhosis stigmata. On ultrasound examination, a space-occupying lesion measuring 15 cm in greatest dimension was detected in the liver. Computed tomography (CT) scan revealed a large tumor measuring 12.6 × 9.8 cm involving left lobe segments 2 and 3. A diagnosis of large hemangioma with internal hemorrhage was made radiologically. Laboratory investigations, including liver biochemical profile and tumor markers such as Carcinoembryonic antigen (CEA), alpha-fetoprotein, and CA19-9 levels, were in the normal ranges. Routine hematological profile was within normal limits. Magnetic resonance imaging (MRI) and other radiological investigations were not performed due to financial constraints. The patient was undertaken for surgery considering the possibility of internal hemorrhage in the tumor. Preoperative single tumor was identified in segments 2 and 3 of the liver. It was well defined and measured about 15 cm in diameter. No direct invasion of the great vessels or adjacent intraperitoneal structures was seen. The resected specimen was sent to department of histopathology. On gross examination, the specimen measured 20 cm × 11 cm × 8 cm and weighed 850 g. External surface was smooth with few congested vessels [Figure 1]a. On the cut section, a tumor was identified measuring 14.5 × 10 × 8 cm. The tumor was firm, well demarcated, focally encapsulated and grayish-white, with a focal whorled and fasciculated cut surface along with small foci of myxoid degeneration [Figure 1]b. There was no necrosis. The adjacent liver parenchyma measured 7 × 4 × 4 cm and was unremarkable with no evidence of cirrhosis or fibrosis. Surgical resection margin was free of tumor grossly. Microscopic evaluation of the tumor showed a well-circumscribed, low to moderately cellular tumor demonstrating spindle and fibroblast-like cells within the collagenous stroma [Figure 1]c. The vascular pattern of the benign areas of tumor resembled that of hemangiopericytomas, with abundant branching and thin-walled vessels dissecting through the tumor [Figure 1]d. The cells showed spindle to elongated nuclei, clumped chromatin, and variable eosinophilic cytoplasm with indistinct cytoplasmic borders [Figure 2]a. Myxoid degeneration was noted [Figure 2]b. Mitotic figures were not discernible. No pleomorphism was noted. Few small collections of lymphomononuclear inflammatory cell infiltrate were seen. Immunohistochemistry disclosed strong and diffuse CD34, Bcl2, and vimentin positivity, supporting the diagnosis of a benign SFT [Figure 2]c, d. The patient recovered uneventfully after the procedure. She was discharged home on the 7th postoperative day and followed up on an outpatient basis. Four years after surgery, the patient was still doing well with no evidence of recurrence.
Figure 1: (a) Gross specimen of the tumor having smooth external surface with few congested vessels. (b) Grayish-white tumor. (c) Tumour and normal liver interface, (Hematoxylin and Eosin, ×40). (d) Focal capsule with stag horn vasculature (Hematoxylin and Eosin, ×40)

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Figure 2: (a) Low cellular area with thick collagen (Hematoxylin and Eosin, ×20). (b) Spindle-shaped cells in ill-defi ned fascicles in myxoid background (Hematoxylin and Eosin, ×20). (c) Strong CD34
immunopositivity of the tumor cells, (IHC, ×20). (d) Strong Bcl2 immunopositivity of the tumor cells, (IHC, ×20)


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   Discussion Top


SFT (also called localized fibrous tumor) is an uncommon mesenchymal neoplasm composed of spindle cells and interspersed collagen that has characteristic immunohistochemical features. A review of the English literature revealed fewer than 40 reported cases of SFTs of the liver. [1],[2],[3],[4],[5],[6],[7],[8] These tumors show female predilection, with a male:female ratio of 1:2. The mean age was 55 years. [8] The tumor can be found in either the right or the left hepatic lobe. Hepatic SFTs can be asymptomatic or may present with diverse nonspecific clinical manifestations ranging from pain in abdomen, distention, and discomfort, to symptoms such as nausea, vomiting, and weight loss when the tumor grows. [1],[2],[3],[4] Less commonly, jaundice, fever, hypoglycemia, and general fatigue can be present. [5] Some present with alterations of liver function tests and compression of biliary channels leading to cholestasis. In general, the clinical presentation of patients with SFT is usually mild and non specific.

On imaging, SFT of the liver is seen as (i) a large, well-defined solitary mass on cross-sectional imaging, (ii) hyperechoic or hypoechoic on ultrasonography, and (iii) hypodense with or without irregular minimal enhancement on contrast-enhanced CT. [9] Although imaging features may suggest the diagnosis of SFT of the liver, other benign or malignant hepatic tumors, such as sarcoma, leiomyoma, and inflammatory pseudotumor may have similar features. The diagnosis may also be suspected in MRI, especially if the enhancement is hypointense on the T2-weighted MRI and shows progressive enhancement in the delayed phase. Most benign or malignant liver tumors, including hemangiomas, hepatic adenomas, fibrolamellar hepatocellular carcinomas, and peripheral mass-forming cholangiocarcinomas, are predominantly hyperintense on the T2-weighted images, and some have a delayed, persistent enhancement. [6] Fibrolamellar hepatocellular carcinoma which most frequently arises in the noncirrhotic liver of young patients demonstrates a central scar and calcification radiologically. Several authors believe that fine-needle aspiration also helps to establish a diagnosis if the tissue sample is adequate and the fine-needle aspiration can be analyzed for immunomarkers; there has been one successful case report of this practice. Other authors regard fine-needle biopsy to be inaccurate and do not recommend this practice. [6] Definitive diagnosis of SFT of the liver must therefore be made on the basis of histopathologic findings. Therefore, in the present case, the diagnosis of SFT of the liver was based on the association of typical morphological and immunostaining findings. CD34 positivity distinguishes SFT from other spindle cell tumors, and it is necessary to combine other markers for differential diagnosis. In our case, the tumor cells were strongly positive for CD34, Bcl-2, and vimentin, and negative for CD117, CD99, smooth muscle actin (SMA), desmin, S-100, and epithelial membrane antigen (EMA). The characteristic immunohistologic staining pattern differentiates it from other liver tumors, such as sclerosed hemangioma (CD34-positive endothelial cells), leiomyoma (consists of intersecting bundles of smooth muscle cells SMA-positive and CD34-negative), inflammatory pseudotumor (composed of spindle cells admixed with variable amount of proliferating myofibroblasts, fibroblasts, extracellular collagen, lymphocytes, and plasma cells), hepatocellular carcinoma (CD34-negative, Hep Par 1 positive, Glypican 3 positive), fibrosarcoma (highly cellular and forms a "herring bone" pattern), and epithelioid hemangioendothelioma (factor VIII positivity). [8] The histopathologic features described for the present case (low cellular tumor demonstrating hemangiopericytoma-like vascular pattern with keloid type collagen with no atypia, necrosis, and mitotic activity) are typical of a benign SFT of the liver. This was confirmed by the favorable course of the patient, who was alive and disease-free 48 months after surgery.

A hepatic SFT is often a benign tumor that has a good prognosis. Radical surgical removal of the tumor with clear margins of resection is the mainstay of treatment. Most authors believe that postoperative adjuvant chemotherapy or radiotherapy should be reserved for incomplete resections and/or histopathological features of malignancy. [8],[10] Some authors consider reoperation in case of positive margins. There are no data about liver transplantation in patients with SFT.

In conclusion, SFT of the liver is an uncommon neoplasm with various clinical presentations. This entity should be included in the differential diagnosis of a single, large, well-circumscribed, hepatic lesion. Correct interpretation of unique pathological findings and CD34 and Bcl-2 immunoreactivity plays a significant role in differentiating from other spindle cell neoplasms of the liver. A complete surgical resection is the treatment of choice and is curative in most cases.

 
   References Top

1.Neeff H, Obermaier R, Technau-Ihling K, Werner M, KurtzC, Imdahl A, et al. Solitary fibrous tumour of the liver: Case report and review of the literature. Langenbecks Arch Surg 2004;389:293-8.  Back to cited text no. 1
    
2.Changku J, Shaohua S, Zhicheng Z, Shusen Z. Solitary fibrous tumor of the liver: Retrospective study of reported cases. Cancer Invest 2006;24:132-5.  Back to cited text no. 2
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3.Vennarecci G, Ettorre GM, Giovannelli L, Del Nonno F, Perracchio L, Visca P, et al. Solitary fibrous tumor of the liver. J Hepatobiliary Pancreat Surg 2005;12:341-4.  Back to cited text no. 3
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4.Ji Y, Fan J, Xu Y, Zhou J, Zeng HY, Tan YS. Solitary fibrous tumor of the liver. Hepatobiliary Pancreat Dis Int 2006;5:151-3.  Back to cited text no. 4
    
5.Guglielmi A, Frameglia M, Iuzzolino P, Martignoni G, DeManzoni G, Laterza E, et al. Solitary fibrous tumor of the liver with CD 34 positivity and hypoglycemia. J Hepatobiliary Pancreat Surg 1998;5:212-6.  Back to cited text no. 5
    
6.Peng L, Liu Y, Ai Y, Liu Z, He Y, Liu Q. Skull base metastases from a malignant solitary fibrous tumor of the liver. A case report and literature review. Diagn Pathol 2011;6:127.  Back to cited text no. 6
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7.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;19:81-4.  Back to cited text no. 7
    
8.Korkolis DP, Apostolaki K, Aggeli C, Plataniotis G, Gontikakis E, Volanaki D, et al. Solitary fibrous tumor of the liver expressing CD34 and vimentin: A case report. World J Gastroenterol 2008;14:6261-4.  Back to cited text no. 8
    
9.Fuksbrumer MS, Klimstra D, Panicek DM. Solitary fibrous tumor of the liver: Imaging findings. AJR Am J Roentgenol 2000;175:1683-7.  Back to cited text no. 9
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10.Archontaki M, Korkolis D, Arnogiannaki N, Hatzijiannis S, Dendrinos P, Megapanos C, et al. Histologically malignant solitary fibrous tumour of the anterior thoracic wall: A case report and review of the literature. Case Report Med 2010;2010:257167.Epub 2010 Jun 20.  Back to cited text no. 10
    

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Correspondence Address:
Sushma Patra
Senior Consultant, Department of Pathology, Global Hospital and Health City, Chennai, Tamil Nadu - 600 100
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.97892

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