Indian Journal of Pathology and Microbiology

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Year
: 2021  |  Volume : 64  |  Issue : 4  |  Page : 847--849

A case of solitary fibrous tumor of urinary bladder


Suganthi Krishnamurthy1, Maya Menon1, Ashok Parameswaran1, Ananthakrishnan Sivaraman2,  
1 Department of Histopathology and Cytology, Apollo Main Hospitals, Chennai, Tamil Nadu, India
2 Department of Urology, Chennai Urology and Robotics Institute, Chennai, Tamil Nadu, India

Correspondence Address:
Suganthi Krishnamurthy
Department of Histopathology and Cytology, Mezzanine Floor, Apollo Hospitals, 21, Greams Lane, off Greams Road, Chennai - 600 006, Tamil Nadu
India




How to cite this article:
Krishnamurthy S, Menon M, Parameswaran A, Sivaraman A. A case of solitary fibrous tumor of urinary bladder.Indian J Pathol Microbiol 2021;64:847-849


How to cite this URL:
Krishnamurthy S, Menon M, Parameswaran A, Sivaraman A. A case of solitary fibrous tumor of urinary bladder. Indian J Pathol Microbiol [serial online] 2021 [cited 2022 Jan 21 ];64:847-849
Available from: https://www.ijpmonline.org/text.asp?2021/64/4/847/328582


Full Text



Mesenchymal tumors of the bladder are rare, and among these, Solitary fibrous tumors are distinctly unusual. SFTs were first described by Klemperer and Rabin et al. as pleural-based lesion.[1] The recognition of lesions with similar morphology in extra-pleural sites led to documentation of their presence in the deep soft tissue, pelvis, retroperitoneum, serosal surfaces, and in the head and neck region including the orbit and meninges. The overlapping morphology with “hemangiopericytoma'' and the common CD34 positivity on immunohistochemistry led to the realization that these were two ends of the spectrum of a common entity.[1] Westra W H et al. in the year 2000, described five cases of SFT in the lower genital tract of which two cases were in the urinary bladder. These were the first w cases in the urinary bladder.[2] Thereafter, only 30 urinary bladder SFTs have been reported in the bladder in the English literature.[3]

A 30-year-old lady was referred to the urologist by her family physician for evaluation of a bladder mass detected in the course of a routine pelvic ultrasound. The patient reported that she had had urgency and straining to void urine on and off for the past 2 month. Computed tomography (CT) showed a hypodense lesion in the urinary bladder with intense postcontrast enhancement, along the right lateral wall, measuring 3.0 × 2.3 cm [Figure 1]. On MRI, the lesion was well defined in T1-weighted images, hyperintense in T2, and measured 2.6 × 2.0 cm, indenting the serosa. A CT-guided biopsy of the lesion was performed. Microscopic examination revealed a cellular spindle cell lesion arranged in short fascicles and with a pericytomatous vasculature. The tumor cells exhibited ovoid bland nuclei, wavy eosinophilic cytoplasm, and interstitial collagenous stroma; and scattered ectatic blood vessels were noted [Figure 2]a. Mitoses were not evident. The histopathology was reported as a spindle cell lesion with the possibility of solitary fibrous tumor being raised. Immunohistochemistry showed strong and diffuse positivity for CD34, diffuse nuclear staining for STAT6, and membranous staining pattern for CD99 [Figure 2]b, [Figure 2]c, [Figure 2]d. Desmin S100 and c-Kit (CD117) were negative.{Figure 1}{Figure 2}

The patient underwent a robotic partial cystectomy; the mass was noted to involve the right lateral wall of urinary bladder [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. Gross examination of the excision specimen revealed a tan brown nodular mass covered by mucosa on one aspect and adherent perivesical fat on the other. On sectioning, a circumscribed whitish firm lesion measuring 2.4 × 2.3 × 2.0 cm was noted. Histology revealed urinary bladder wall with a circumscribed nodular lesion interdigitating between the smooth muscle bundles composed of spindle-shaped cells with interstitial hyalinization with a “patternless pattern” [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. Few storiform foci were observed. Ectatic blood vessels with a branched appearance were seen. No cellular atypia, necrosis, or increase in mitoses was noted. The lesion extended from the submucosa into the perivesical adipose tissue. Circumferential and perivesical margins were free of tumor. The postoperative course was uneventful at 6 months follow-up.{Figure 3}{Figure 4}

 Discussion



Histological features that typify SFTs are variable cellularity including both fibrous and cellular areas, a “patternless pattern” with interstitial collagenization and hemangiopericytoma type “staghorn” vasculature.[1] The criteria for malignancy is similar in pleural and extrapleural sites, which includes high cellularity, mitoses of four or more per ten high-power fields, hemorrhage, necrosis, and cellular pleomorphism.[4] The differential diagnosis is broad and includes many benign and malignant mesenchymal tumors. Based on the hemangiopericytomatous pattern of vasculature, alternating hypo and hypercellular areas, and the site, gastrointestinal stromal tumor, low-grade dedifferentiated liposarcoma, deep fibrous histiocytoma, and vascular lesions enter into the differential diagnosis. Most of the SFTs are positive for CD34 (90–95%) and CD99 (70%),[5] but are generally negative for S-100, actin, desmin, and keratin. Molecular genetics studies have identified NAB2-STAT6 gene fusion in a majority of SFTs. STAT 6 by immunohistochemistry, a surrogate marker for this fusion, is a highly sensitive and specific marker.[5] In a study by Doyle et al., which included 231 cases of benign and malignant tumors including 60 SFTs, 98% of SFTs showed strong and diffuse nuclear expression of STAT6. All the other tumors were negative with the exception of three dedifferentiated liposarcomas and one deep fibrous histiocytoma, which though positive had a weak staining intensity.[6] Our case had typical features of SFT, lacking atypia or pleomorphism and with diffuse and strong expression of CD34 and STAT 6 on immunohistochemistry. The tumor cells were negative for S100, C kit (CD117), and desmin. According to a risk stratification model in a study by Demicco EG et al., age less than 55 years, size less than 5 cm and absence of mitosis are predictive of low risk tumors with metastases free and disease specific survival rate at 5 and 10 years of 100%.[7] Our patient falls under this low-risk category. The most common approach in the management of SFT is complete resection with tumor-free margins.[5] Hence, a conservative minimally invasive robotic treatment approach was justified in this patient as the tumor lent itself to complete resection (partial cystectomy). The diagnosis on needle biopsy enabled planning the appropriate surgery. Although uncommon, the possibility of SFT should be considered in the differential of spindle cell lesions of the urinary bladder. Long-term follow-up is recommended in these patients as 5–10% of histologically benign cases behave aggressively, with recurrence and metastases.[5]

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