Solitary fibrous tumour of the liver with IGF-II-related hypoglycaemia. A case report

Introduction We report a rare case of solitary fibrous tumour (SFT) of the liver associated with non-islet cell tumour hypoglycaemia (NICTH), which caused a hypoglycaemic coma due to over-production of big-insulin-like growth factor-II. Discussion Although generally benign, SFTs are better characterised, lately due to Western blot, but less than 40 cases have been reported where the liver is the target organ. In only two benign cases has hypoglycaemia been a feature. Conclusion We report for the first time a demonstrable relationship between benign liver SFT and NICTH.     

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.     

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。     

Solitary fibrous tumour of the liver: case report and review of the literature

Background Solitary fibrous tumours (SFTs) of the liver are very rare entities. Although firstly described to be tumours of pleural origin, SFTs have been reported in various organs such as the meninges, orbit, upper respiratory tract, thyroid, peritoneum, retroperitoneum and soft tissues. Histologically, this tumour often shows alternating cellular and relatively acellular areas. The cellular areas show a wide variety of patterns, making it difficult for it to be differentiated from other mesenchymal tumours. Its immunohistochemical positivity for CD34 and vimentin is believed to be unique. Histiogenesis of SFTs, however, is not yet fully understood. They are known to be usually benign, with only few reports indicating their ability to metastasize.Patients and methods We review the literature on SFTs of the liver and report on the case of a 63-year-old female patient with a large SFT of the right liver.Conclusions Surgical resection seems to be the treatment of choice. Local recurrence is scarce. Due to the very limited number of cases, data regarding the long-term survival of patients are lacking.     

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.     

Solitary fibrous tumour of the orbit: clinical and histological evidence

Solitary fibrous tumour (SFT) is rarely found in the orbit; it can occur within a wide age range (14–80 years). A case of a 59-year-old man with unilateral exophthalmos is presented. After CT and MRI investigations, the cause was seen to be an orbital mass that, after surgical removal, was diagnosed histologically as a solitary fibrous tumour. In the orbit, the SFT has a histological appearance identical to that of homonymous pleural lesion. It is characterized by alternating hypercellular and hypocellular areas, the collagen bundles having a keloid-type quality. Immunohistochemically, it showed a strong and widespread reactivity for CD68 and CD34. Correct diagnosis of this infrequent neoplasm needs not only clinical observation but also requires radiological examination (CT, MRI) and, above all, histological assessment. Various primary mesenchymal tumours of vascular and fibrohistiocytic type can mimic the symptoms of SFT, thus only the immunohistochemical profile and the ultrastructural features of the lesion allows it to be diagnosed definitively.     

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.     

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 recurrent solitary fibrous tumor of the thigh with malignant transformation: A case report

IntroductionWe describe an unusual case of a uniformly high-grade malignant solitary fibrous tumor (SFT) of the thigh with recurrence after wide resection in a 31-year-old man.Presentation of caseOur current case showed a long-term benign course before the operation, although the subcutaneous tumor was larger than 10 cm at presentation. The SFT was diagnosed by needle biopsy, and wide resection was performed. Histological findings showed proliferation of capillaries surrounded by masses of spindle-shaped cells without any cytologic atypia, and the percentage of MIB-1-positive nuclei was 2.1%. However, a rapidly enlarging recurrent tumor was observed 11 months after the operation. A second wide resection for the recurrent tumor was performed. Histologically, the tumor cells uniformly displayed significant cytologic atypia and pleomorphism, and had 40–50 mitoses per 10 high-power fields. The proportion of MIB-1-positive nuclei was 48%. Consequently, the tumor was diagnosed as a SFT with malignant transformation.DiscussionThe malignant transformation described in past studies showed high-grade areas within benign, low-grade, or intermediate-grade SFTs. Therefore, in contrast to our case, uniformly high-grade malignant histological findings at recurrence were not described.ConclusionEven if a tumor is non-malignant during the clinical course, as confirmed by tissue biopsy, the possibility of tumor progression to high-grade sarcoma at recurrence should be considered, and the treatment strategy should be determined carefully.     

Surgical treatment of retroperitoneal leiomyosarcoma invading the inferior vena cava: report of three cases

Retroperitoneal leiomyosarcoma is a rare neoplasm for which complete surgical removal provides the only effective treatment, as local recurrence adversely affects prognosis. However, invasion of major vessels may occur, making complete resection difficult. This report describes the cases of three patients who required concomitant resection of parts of the inferior vena cava because of direct tumor invasion. The major vessels should be isolated in preference to the tumor capsule during surgery to prevent sudden exsanguination or incomplete tumor resection. Resection of a recurrent sarcoma or a solitary metastasis can be effective in selected patients. Received: September 20, 2001 / Accepted: May 7, 2002     

Recurrence rates of surgically resected solitary fibrous tumours of the pleura: a systematic review and meta-analysis

 Open in a separate windowOBJECTIVESRecurrence rates of solitary fibrous tumours of the pleura (SFTP) after surgical resection vary widely in the published literature. Our objective was to systematically review the existing literature to determine an accurate estimate of SFTP recurrence rates after surgical resection and to determine risk factors associated with recurrence.METHODSPubMed, EMBASE and the Cochrane library were systematically searched for randomized controlled trials and observational studies (prospective or retrospective) through 20 June 2020 that reported the recurrence rates after surgical resection. The outcome of interest was recurrence.RESULTSOf the 23 included studies comparing 1262 patients, the overall recurrence of SFTP in patients who underwent surgical resection was 9% [95% confidence interval (CI) 7–12%; I² = 52%]. In addition, pooled benign and malignant recurrence rates were 3% (95% CI 2–5%; I² = 8%) and 22% (95% CI 15–32%; I² = 52%), respectively. A benign SFTP was associated with a significantly lower recurrence rate than a malignant SFTP [odds ratio (OR) 0.11; 95% CI 0.06–0.20; I² = 0%]. There was no significant difference in the recurrence rates between lesions originating from parietal versus visceral pleura (OR 1.30; 95% CI 0.28–6.02; I² = 59%). Female sex was associated with increased recurrence (OR 5.29; 95% CI 1.66–16.92; I² = 0%).CONCLUSIONSCollectively, this systematic review demonstrated a 9% SFTP post-resection recurrence rate. Furthermore, the recurrence rates for benign and malignant SFTP were 3% and 22%, respectively. Histological malignancy and female sex were associated with higher risk.     

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.     

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.     

Malignant solitary fibrous tumor of the lumbar spinal root mimicking schwannoma: a case report

Background contextMalignant solitary fibrous tumors (SFTs) arising from the spinal cord are extremely rare and poorly understood mesenchymal neoplasms. To date, only one malignant SFT located in the spinal canal of the sacrum has been described, but none arising from the lumbar nerve root have been reported. Although most SFTs with benign histological features can be treated by complete surgical excision alone, malignant SFTs may require adjuvant therapy. However, systemic chemotherapy and radiotherapy have not been shown effective in patients with malignant SFTs.PurposeTo describe a patient with a malignant SFT arising from the lumbar nerve root.Study designA case report and review of literature.MethodsWe describe the clinical course of the patient and the radiological and pathological findings of the tumor. The effect of systemic chemotherapy was evaluated and the relevant literature was reviewed. This work has no disclosure of funding and was approved by the Institutional Review Board of Gifu University.ResultsThe tumor had been resected previously at another hospital, but it recurred and showed multiple metastatic lesions on both lungs within 3 months. Although the patient received systemic chemotherapy, both primary and metastatic lesions were found to be stable disease according to Response Evaluation Criteria in Solid Tumors. The patient died due to cachexia 6 months after her first visit.ConclusionThis patient presented with a highly unusual tumor. Even if a tumor is a dumbbell-shaped mass, similar to a neural tumor, SFT should be considered in the differential diagnosis.     

腹膜后肿瘤伴下腔静脉癌栓的外科处理

目的：探讨腹膜后肿瘤伴下腔静脉癌栓患者的分期和分型及外科处理。方法：2例右肾癌伴下腔静脉癌栓和1例右肾上腺皮质癌伴下腔静脉癌栓，分别行右肾癌或右肾上腺皮质癌根治性下腔静脉部分切除或切开取栓术。结果：2例术后分别存活48个月和12个月，1例术中死亡。结论：根据患者的分期和分型采用不同的手术方法，是治疗本病的有效途径。     

Post traumatic inferior vena cava thrombosis: A case report and review of literature

IntroductionPost traumatic inferior vena cava (IVC) thrombosis is a rare and not well described entity with nonspecific clinical presentation. It remains a therapeutic challenge in traumatic context because of haemorrhagic risk due to anticoagulation.Presentation of caseWe report a case of IVC thrombosis in an 18 year-old man who presented with liver injury following a traffic crash. The thrombosis was incidentally diagnosed on admission by computed tomography. The patient was managed conservatively without anticoagulation initially considering the increasing haemorrhagic risk. IVC filter placing was not possible because of the unusual localization of the thrombus. Unfractionated heparin was started on the third day after CT scan control showing stability of hepatic lesions with occurrence of a pulmonary embolism. The final outcome was good.DiscussionThe management of post traumatic IVC thrombosis is not well described. Medical approach consists in conservative management with anticoagulation which requires the absence of active bleeding lesions. Surgical treatment is commonly based on thrombectomy under extracorporeal circulation. Interventional vascular techniques have become an important alternative approach for the treatment of many vessel lesions. Their main advantages are the relative ease and speed with which they can be performed.ConclusionPost traumatic IVC thrombosis is a rare condition. Its management is not well defined. Early anticoagulation should be discussed on a case-by-case basis. Other alternatives such IVC filter or surgical thrombectomy may be used when the bleeding risk is increased. The most serious risk is pulmonary embolism. Outcome can be favorable even with non surgical approaches.     

Phlegmasia Cerulea Dolens of the Lower Extremities Secondary to Thrombosis of an Inferior Vena Caval Aneurysm

We present the clinical case of a 34-year-old male with an aneurysm of the inferior vena cava in which thrombosis led to a picture of bilateral phlegmasia cerulean dolens of the lower extremities. A clear and precise diagnosis was achieved by angio CT which allowed initial conservative management with good immediate and short term outcome.To our knowledge only 23 cases of aneurysm of the inferior vena cava have been reported in the literature worldwide making it difficult to establish a diagnostic and therapeutic algorithm for these cases. Nine cases were presented with thrombosis and in the other 14 the diagnosis was incidental. They mimick a pararenal mass requiring differential diagnosis with retroperitoneal neoplasms.