# Malignant Solitary Fibrous Tumor of the Kidney: Report of  the First Case Managed with Interferon

Authors: Javier Cuello; Ricardo Brugés
Journal: Case Reports in Oncological Medicine
 (2013)

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## Abstract

Solitary fibrous tumors of the kidney are extremely rare tumors with unpredictable behavior. We describe a case of a patient with a solitary fibrous tumor of kidney with malignant findings with distant metastasis and nephrectomy managed with subcutaneous interferon achieving 23 months of progression-free survival. To date there is no prospective evaluation of any specific modality of treatment, but the surgical management and long-term followup are the only ones so far recommended strategies in the management of these patients. Studies are awaited with more patients to evaluate the different strategies of systemic therapy reported so far to allow adding survival benefit.


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## Body

## 1. Introduction

Solitary fibrous tumors are rare mesenchymal neoplasms, considered a variant of hemangiopericytomas usually originates in the pleura; however, there have been reports of extrapleural origin (abdomen, retroperitoneum, upper extremities, orbit, cervix, meninges, mediastinum, parotid, nasal cavity, neck, etc.) [1–4]. The location is even more rare urogenital, and according to the literature, only 49 cases of solitary fibrous tumor of the kidney have been reported in the literature [5]. The origin of the majority of cases formed in the kidney is found in the renal capsule tissue or connective tissue interstitial peripelvis. Most cases presented with suspected renal cell neoplasms; however, morphologically, solitary fibrous tumors are characterized by the proliferation of spindle cells with little pattern in their architecture, and the final diagnosis was made with immunohistochemical findings that show staining for CD34 [6–9].


## 2. Case Report

A 49 year old woman with no history of importance who enters the emergency room by 2-months of dyspnea at rest, associated with pleuritic chest pain on right chest, dry cough, no fever. The chest radiograph showed a right pleural effusion and multiple nodular lesions on the pleura, so we decided to perform a CT chest and abdomen in which evidenced free right pleural effusion occupying 80% of the right chest, at least two pleural masses with solid density that capture the contrast, multiple pulmonary nodules with soft tissue density in both lungs and left kidney mass. Carried left nephrectomy with suspected metastatic renal carcinoma, shows renal mass plus liver metastases which were resected. The analysis of the tumor presented as a first option versus solitary fibrous tumor angiomyolipoma. Immunohistochemical studies that showed cell reactivity with CD34, CD99, BCL-2, and vimentin are negative for HMB-45, AMS, CD68, cytokeratin cocktail, and S100, and the Ki67 is not assessable (Figure1). This profile supports the histological diagnosis of solitary fibrous tumor with origin in the kidney.Immunohistochemical evidence CD34 positive cells (panel B), vimentin (panel C), and negative for S100 (panel D).We decided to start treatment with interferon a2b subcutaneous dose, extrapolating the cases of patients with solitary fibrous tumor of the pleura [12]. The dose was adjusted for flu symptoms and she is with stable disease at 23 months follow-up.


## 3. Discussion

Solitary fibrous tumors are extremely rare tumors, arising mostly at the level of the pleura, and cases arising from the urogenital region are even more rare, with 49 cases reported so far in the literature. The histogenesis of this entity is still unknown, but recent studies suggest a primitive mesenchymal cells or level perivascular [10, 11].The differential diagnosis of these cases includes sarcomatoid variant of renal carcinoma, angiomyolipoma, fibromas, and fibrosarcomas. Table1 presents the cases so far published, reporting the primary source, and histologic variant outcomes.Table 1
Summary of reported cases of renal SFT. (N.A. data not presented in the publication.)
Case
Reference
Age
Sex
Localization
Size (cms)
Subtype
Followup (months)
Outcomes
1
Fain et al. J Urol Pathol 1996; 4: 227–238
45
F
Right kidney
6
Benign
8
Tumor free
2
Fain et al. J Urol Pathol 1996; 4: 227–238
46
F
Right kidney
7,2
Benign
33
Tumor free
3
Fain et al. J Urol Pathol 1996; 4: 227–238
51
M
Left kidney
4,5
Benign
2
Tumor free
4
Gelb et al. Am J Surg Pathol 1996; 20: 1288–1295
48
F
Right kidney
3
Benign
1
Death from other cause  Tumor free
5
Fukunaga and Nikaido Histopathology 1997; 30: 451–456
33
F
Right kidney
3,5
Benign
90
Tumor free
6
Fukunaga and Nikaido Histopathology 1997; 30: 451–456
36
F
Left kidney
2
Benign
12
Tumor free
7
Hasegawa et al. [2]
64
M
Kidney  (laterality not reported)
4,5
Benign
8
Tumor free
8
Leroy et al. Urol Int 2000; 65: 49–52
66
F
Right kidney
9
Benign
9
Tumor free
9
Morimitsu et al. APMIS 2000; 108: 617–625
72
F
Right kidney
8
Benign
10
Tumor free
10
Yazaki et al. Int J Urol 2001; 8: 504–508
70
M
Right kidney
6
Benign
N.A.
N.A.
11
Wang et al. Am J Surg Pathol 2001; 25: 1194–1199
41
M
Right kidney
14
Benign
48
Tumor free
12
Wang et al. Am J Surg Pathol 2001; 25: 1194–1199
72
M
Right kidney
13
Benign
5
Tumor free
13
Cortes-Gutierrez et al. J Urol 2001; 166: 602
28
F
Left kidney
15
Benign
12
Tumor free
14
Magro et al. Pathol Res Pract 2002; 198: 37–43
31
F
Right kidney
8,6
Benign
8
Tumor free
15
Durand et al. Prog Urol 2003;13:491–494
35
M
Right kidney
17
Benign
6
Tumor free
16-17
Llarena Ibarguren et al. Arch Esp Urol 2003; 56: 835–840
51
F
Bilateral
25 (left)2 (right)
Benign
N.A.
N.A.
18
Bugel et al. Prog Urol 2003; 13: 1397–1401
60
F
Right kidney
11
Benign
48
Tumor free
19
Gres et al. Prog Urol 2004; 14: 65–66
82
M
Right kidney
9
Benign
13
Tumor free
20
Yamada et al. Pathol Int 2004; 54: 914–917
59
M
Left kidney
6,8
Benign
N.A.
N.A.
21–27
Pierson et al. Mod Pathol 2005; 18: 159A
Median (52,6) range 29–79)
N.A.
N.A.
median (5,7), range 2,2–10)
Benign
N.A.
N.A.
28
Kawagoe et al. Nishinihon J Urol 2005; 67: 568–571
83
F
Left kidney
11
Benign
20
Tumor free
29
Johnson et al. J Comput Assist Tomogr 2005; 29: 481–483
51
F
Right kidney
11
Benign
N.A.
N.A.
30
Yamaguchi et al. Urology 2005; 65: 175
51
F
Left kidney
10
Benign
N.A.
N.A.
31
Kohl et al. Arch Pathol Lab Med 2006; 130: 117–119
85
F
Left kidney
3,5
Benign
N.A.
N.A.
32
Koroku et al. Hinyokika Kiyo 2006; 52: 705–706
18
F
Left kidney
3,2
Benign
15
Tumor free
33
Provance / Ferrari et al. Clin Pediatr (Phila) 2006; 45: 871–873
4
M
Right kidney
8
Benign
N.A.
N.A.
34
Fine et al. [3]
76
M
Left kidney
12
Malignant
4
Persistent tumor
35
Bozkurt et al. APMIS 2007; 115: 259–262
51
F
Left kidney
4
Benign
10
Tumor free
36
Znati et al. [10]
70
M
Left kidney
15
Benign
6
Tumor free
37
Constantinidis et al. Can J Urol 2007; 14: 3583–3587
26
M
Right kidney
5
Benign
6
Tumor free
38
Hirabayashi et al. Hinyokika Kiyo 2008; 54: 357–359
44
F
Left kidney
5,8
Benign
28
Tumor free
39
Magro et al. [11]
34
F
Left kidney
9
Malignant
15
Tumor free
40
Amano et al. Hinyokika Kiyo 2008; 54: 765–769
67
M
Left kidney
7
Benign
10
Tumor free
41
Yoneyama et al. Hinyokika Kiyo 2009; 55: 479–481
76
F
Right kidney
2,2
Benign
48
Tumor free
42
Hirano et al. [6]
75
M
Left kidney
4,5
Benign
9
Tumor free
43
Taxa et al. Actas Urol Esp 2010; 34: 568–570
39
F
Left kidney
2,5
Benign
12
Tumor free
44
Yamaguchi et al. Hinyokika Kiyo 2010; 56: 435–438
39
F
Left kidney
20
Benign
6
Tumor free
45
Marzi et al. Minerva Urol Nefrol 2011; 63: 109–113
72
F
Left kidney
19
Malignant
N.A.
N.A.
46
Hsieh et al. [8]
50
F
Right kidney
9
Malignant
30
Tumor free
47
De Martino et al. [5]
68
F
Left kidney
7
Malignant
5
Death by the disease
48
Caso actual
49
F
Left kidney
9,8
Malignant
23
Stable diseaseThese results show a relatively rare entity, with peak presentation in the fifth decade of life, arising mostly in the renal parenchyma unilaterally (Table2).Table 2
Clinicopathologic features and outcomes of the 49 cases reported with solitary fibrous tumors of the kidney.
Median age in years (range)
51 (4–85)
Sex
Male
14
Female
28
Unknown
7
Location
Left kidney
23
Right kidney
17
Bilateral
1
Unknown
8
Site
Kidney
33
Renal capsule
6
Peripelvis
3
Pelvis
1
Unknown
6
Medium size in cm (range)
7,6 (2–20)
Histology
Benign
42
Malignant
7
Treatment
Tumor resection
2
Nephrectomy
41
Unknown
6
Subcutaneous interferon
1
Outcome
No evidence of disease
25
Metastasis
4
Unknown
20About 14% of patients have aggressive behavior where common management strategy is nephrectomy with complete resection of the lesions. The pathological findings that have been correlated with aggressive behavior are pleomorphism, increased cellularity and mitotic activity (>4 mitosis/10 high-power fields), necrosis, hemorrhage, and atypical sites (parietal pleura, lung parenchyma) [13]. However, even the clinical behavior can not accurately predict the histopathological findings, as some cases with results suggesting benign disease may show aggressive behavior and vice versa, so it is necessary that these patients have an indefinite period of observation [14–19].In relation to the management of this condition, there is as yet no prospective evaluation of any specific treatment modality; however, case reports and retrospective case series suggest that complete surgical resection and long-term followup are generally most recommended strategies. In cases such as the present, which present with metastatic disease, there is no clearly defined systemic therapy. Metastasectomy is thought to improve progression-free survival, but in many cases like this, this strategy is not feasible.In case reports hemangiopericytomas, entity closely related to solitary fibrous tumors, has achieved stable disease with the use of interferon with or without thalidomide. So far, this is the first case reported in the literature in which benefit is demonstrated with the use of interferon in a patient with a malignant variant renal solitary fibrous tumor with metastatic disease, achieving stable disease for about 20 months.Some authors suggest the use of antiangiogenic therapies (bevacizumab, sunitinib, pazopanib, etc.), based on the findings of high vascularity and a possible origin of pericytes at this entity [15]. The combination of bevacizumab associated with temozolomide is a potentially promising scheme for patients with solitary fibrous tumors. A series of 14 patients with solitary fibrous tumor unresectable or metastatic, were treated with temozolomide 150 mg/m2 orally on days 1–7 and days 15–21 and bevacizumab 5 mg/kg intravenously on days 8 and 22, with cycles every 28 days. In this study, 11 patients (79%) achieved partial response assessed by Choi criteria with 2 cases (14%) with stable disease. The median progression-free survival was 8.6 months [12, 19, 20].



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