A locally aggressive solitary fibrous tumor of the leg: Case report and literature review

INTRODUCTIONThe solitary fibrous tumor (SFT) is a rare soft tissue tumor with a substantially benign clinical behavior. However, malignant neoplasms with local recurrence or distant metastases have been reported.PRESENTATION OF THE CASEThe authors present a case of an aggressive SFT of the leg, in a 55 years old Caucasian man. Radiological, histological and molecular findings are reported. The differential diagnosis, therapy and outcome of this rare tumor are also discussed.DISCUSSIONAn extensive review of literature showed SFT's clinical behavior as substantially benign, anyway aggressive or malignant neoplasms have been described. The potential risk of local recurrence and distant metastasis thus suggests wide surgical resection and careful long-term follow-up. Differential diagnosis may be quite laborious as SFT can mimic a variety of benign and malignant mesenchymal tumors; immunohistochemical analysis for CD34, CD99, vimentin and bcl-2 is then mandatory.CONCLUSIONOur clinical experience confirmed that SFT may have an aggressive behavior, however, conservative surgical treatment may be successful in the long term.     

Solitary fibrous tumor in the pelvic space

A case of a solitary fibrous tumor (SFT) of the pelvic space in a 64-year-old man is reported herein. Computed tomography (CT) of the pelvis showed a large mass enhanced heterogeneously left paracentral and posterior to the bladder and intimately associated with prostate. The site of origin of the mass could not be defined on CT because margins blended with the bladder, prostate, and rectum. A tumorectomy was performed and has remained well with no evidence of recurrence during the last 3 months. The tumor was 12.5×9.5×8.3 cm in size, solid with a fibromuscular capsule, and gray-tan in color. Histologically, the neoplasms were well circumscribed and composed of short spindle cells arranged without an obvious pattern. Immunohistochemically, these cells were strongly positive for CD 34 and negative for S-100, alpha SMA, and AE1/AE3.     

Solitary fibrous tumor of the thoracic spine

Intraspinal solitary fibrous tumors are rare: to our knowledge, the literature reports only 27 cases. We present a histologically and immunohistochemically confirmed solitary fibrous tumor involving the intradural extramedullary compartment of the thoracic spine. Microsurgical gross-total resection was achieved. A definitive role for adjuvant treatments in this type of tumor has not been established and therefore, they were not used. The patient was well, without clinical or radiological recurrence, 18 months after surgery.     

Large Solitary Fibrous Tumor of the Retroperitoneum: Report of a Case

We report an unusual case of a large solitary fibrous tumor (SFT) in the retroperitoneum. A 53-year-old man was referred to our hospital for surgical treatment of a swelling in the right flank with dull pain. Abdominal computed tomography (CT) and echograms showed a large encapsulated tumor compressing the right kidney and liver. At laparotomy, the tumor was found to be encapsulated but fixed to the capsule of the right kidney within a small area. Therefore, complete removal was achieved. The resected specimen was an encapsulated elastic hard tumor, 14 × 13 × 10cm in size. Immunohistochemical studies revealed reactivity for CD34 and vimentin, but no staining for keratin, S-100, or -smooth muscle actin, confirming a diagnosis of SFT. Although SFT is usually associated with a favorable prognosis, close follow-up is recommended because of the limited information on its long-term behavior.     

Dumbbell-type solitary fibrous tumor in the cervical spine

 We report a dumbbell-type solitary fibrous tumor in the cervical spine. Eight spinal solitary fibrous tumors have been reported previously, but the cervical location is rare and this may be the first report of a dumbbell-type tumor. Histopathological examination showed that the tumor was composed of spindle cells in a collagen-rich matrix, although regional variability was noted. Diffuse immunostaining for CD34 and vimentin was noted in the cytoplasm of the tumor cells. This rare tumor should be recognized in the differential diagnosis of cervical spinal tumors. Received: October 8, 2002 / Accepted: January 22, 2003 RID="*" ID="*" Offprint requests to: K. Endo     

前列腺孤立性纤维性肿瘤一例报告

孤立性纤维性肿瘤是一种罕见的起源于间充质梭形细胞的肿瘤,发生于前列腺者很少见。本院收治1例,年龄30岁,以排尿困难就诊,行经尿道前列腺钬激光剜除术,术后病理诊断为前列腺孤立性纤维性肿瘤,术后患者排尿症状得到明显改善。     

胸腔非典型孤立性纤维肿瘤1例

正患者男,43岁,2个月前无明显诱因出现胸闷、气促,偶有咳嗽、咳痰,近2天出现左侧胸痛。CT增强示左侧胸腔内下份见混杂密度肿块(图1),病灶中心层面大小约10.3cm×23.1cm,病灶与膈肌分界不清;增强后肿瘤呈不均匀强化。考     

A solitary fibrous tumor in the perianal region with a 13-year follow-up: Report of a case

(Received for publication on June 5, 1998; accepted on Nov. 6, 1998)     

肾孤立性纤维瘤1例报告并文献复习

目的 分析肾孤立性纤维瘤的临床病理特征及诊治方式.方法 回顾性分析我院诊治的1例肾孤立性纤维瘤的病理特征、临床表现、影像学检查、治疗方式和术后随访状况.结果 肾孤立性纤维瘤是肾脏较罕见的一种间叶性肿瘤,良性居多,少数为恶性.病理特征多为VIM(+)、CD34(+)、BcL-2(+)、CD99(+).影像学检查显示为类圆形、包膜完整的肿物,与周围组织界限清楚,CT增强后呈现不同程度欠均匀强化.本例患者行机器人辅助腹腔镜下肾部分切除术,术后病理诊断为肾孤立性纤维瘤(中间性),术后随访3月余,未见肿瘤复发、进展和转移.结论 肾孤立性纤维瘤属临床上较罕见的肿瘤,确诊主要通过病理诊断,治疗上首选手术切除肿瘤.     

Malignant solitary fibrous tumor with superior vena cava syndrome

Mediastinal solitary fibrous tumors, particularly those that are malignant, are rare. We report a case of malignant solitary fibrous tumor with superior vena cava syndrome and highly aggressive behavior. Postoperative radiotherapy was performed due to incomplete resection, but the tumor relapsed within 4 months. Gemcitabine-based chemotherapy was ineffective.     

Solitary fibrous tumour of the urinary bladder in a young woman presenting with haemodynamic-relevant gross haematuria

A 24-yr-old woman presented with gross haematuria and a huge tumour of the right bladder wall. At transurethral resection, a solid tumour was seen covered with normal mucosa. The pathological evaluation revealed a solitary fibrous tumour (SFT) of the urinary bladder. For final treatment, a partial cystectomy was performed; tumour-free margins were ensured by frozen-section analysis. This is the first case in the literature presenting intravesically in a young woman. Due to the difficulty in discriminating between malignant and benign growth pattern of this tumour entity, a regular follow-up after conservative treatment is mandatory.     

Intrapulmonary solitary fibrous tumor with bronchial involvement: a rare case report in a child

Intrapulmonary solitary fibrous tumor is a very rare neoplasm. Our review of the English literature suggests that it has not previously been reported in children younger than 10 years. Herein, the occurrence of such a rare lesion invading the tracheobronchial tree is reported in a 7-year-old boy.     

Malignant solitary fibrous tumor of the nasal cavity.

Solitary fibrous tumors (SFTs) are unusual mesenchymal tumors that were first described as primary spindle-cell neoplasms of the pleura. These tumors have been described in many other locations, including the urogenital system, orbit, mediastinum, and upper respiratory tract. Twenty-two cases of an SFT of the paranasal sinuses and nasal cavity have been reported, but none described a malignant SFT extending through the anterior skull base. A 70-year-old man had a 6-month history of unilateral left-sided epiphora and nasal obstruction. Computed tomography and magnetic resonance imaging showed a large left-sided nasal cavity mass with extension into the left extraconal orbit and intracranial extension through the left cribriform plate and ethmoid roof. The patient underwent preoperative embolization of the internal maxillary artery and a subsequent anterior craniofacial resection via a midfacial degloving approach and a left anterior craniotomy. Histopathological analysis of the specimen was consistent with a malignant SFT.     

Mediastinal solitary fibrous tumor with right diaphragm invasion: Report of a case

Mediastinal solitary fibrous tumors (SFTs) are rarely found in adults and there are few reports describing primary mediastinal SFT invading the diaphragm. We report the case of a 47-year-old woman with a large right inferior mediastinal SFT. Magnetic resonance imaging showed the tumor invading the right lower lobe of the lung and the right hemidiaphragm, with displacement of the inferior vena cava (IVC) and right lobe of the liver. Angiogram showed IVC stenosis. To our knowledge, this is the first report of complete resection of the tumor combined with right lower lobectomy of the lung and partial resection and reconstruction of the right diaphragm with a Dacron flap.     

Retroperitoneal malignant solitary fibrous tumor of the small pelvis causing recurrent hypoglycemia by secretion of insulin-like growth factor 2

A 28-yr-old man presented with recurrent reduced consciousness, generalized seizures of unknown etiology, recurrent hypoglycemia, psychomotor retardation, and grade 2 ectasia of the left kidney. Abdominal computed tomography (CT) and positron emission tomography (PET) scans demonstrated a well-circumscribed suprapubic pelvic mass, measuring 18 x 15 x 11 cm, with involvement of para-aortic lymph nodes and dilatation of the left ureter suggestive of an extragonadal testicular tumor. We excised the tumor by laparotomy, and it was confirmed to be a solitary fibrous tumor (SFT). After surgery and R0 tumor resection, the patient had no further evidence of hypoglycemia or of recurrence.     

Solitary fibrous tumor of the perithyroidal soft tissue mimicking substernal goiter

Solitary fibrous tumor is an uncommon neoplasm. We present a case of solitary fibrous tumor of the perithyroidal soft tissue mimicking a substernal goiter.