Malignant solitary fibrous tumor of the pancreas with systemic metastasis: A case report and review of the literature

BACKGROUND Pancreatic solitary fibrous tumor(SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Here, we report the first case of malignant pancreatic SFT with typical Doege-Potter syndrome, along with the clinical and pathologic evidence of its systemic metastasis.CASE SUMMARY The patient was a 48-year-old man with a 1-year history of pancreatic and liver masses and refractory hypoglycemia. Increased uptake of the tracer fluorodeoxyglucose(FDG) was found in the liver and bones by fluorine-18 FDG positron emission tomography/computed tomography. After multidisciplinary discussion, a distal pancreatectomy procedure was performed, and histological examination showed a lesion composed of abundant heterogeneous spindle cells with localized necrosis. On immunohistochemistry evaluation, STAT6 was found to be diffusely expressed in the tumor. Based on the overall evidence, the patient was diagnosed with malignant pancreatic SFT with liver and bone metastases.CONCLUSION The diagnosis of malignant SFT requires comprehensive evidence including clinical, immunohistochemistry, and histological features. This case may be presented as a reference for diagnoses and management of malignant pancreatic SFTs with systemic metastasis.     

Malignant solitary fibrous tumor of the greater omentum: A case report and review of literature

BACKGROUNDMalignant solitary fibrous tumors (SFTs) account for 15%-20% of all SFTs, and malignant SFTs arising from the greater omentum are extremely rare. Most malignant SFTs of the greater omentum are diagnosed via pathological examinations after surgery. In this study, we report a case of malignant omental SFT and review the published literature on this rare malignancy.CASE SUMMARYA 64-year-old female presented with an abdominal mass, and underwent exploratory surgery, during which a huge tumor originating from the greater omentum and intraperitoneal implants were identified and resected. The results of the pathological examination, immunohistochemistry staining, and gene sequencing led to the diagnosis of malignant SFT of the greater omentum. The patient died one and a half years later due to tumor recurrence and metastasis.CONCLUSIONThis is the first report of the application of gene sequencing in the diagnosis of malignant SFTs of the greater omentum.     

Dumbbell-shaped solitary fibrous tumor in the parapharyngeal space: A case report

BACKGROUNDSolitary fibrous tumors (SFTs) occurring in the parapharyngeal space are rare, and their final diagnosis depends on pathological and immunohistochemical analyses. Once the tumor is diagnosed, complete resection and regular postoperative follow-up are required.CASE SUMMARYA 40-year-old male patient with a right parotid gland mass discovered 8 years ago was admitted to hospital. The mass showed no tenderness or local skin redness. Imaging was carried out as the patient had stable vital signs and showed that the mass was a dumbbell-shaped tumor comprising a superficial tumor approximately 5 cm long and 3 cm wide in size that compressed the right parotid gland and a deep tumor located in the right parapharyngeal space approximately 4.5 cm long and 2.5 cm wide in size. Both tumors were connected in the middle. Prior to surgery, the tumors were considered to be parapharyngeal schwannomas. During surgical dissection, the tumors were found to be smooth and tough, without obvious adhesion to the surrounding tissues. The tumors were revealed to be a SFT following postoperative pathological analysis.CONCLUSIONSFTs in the parapharyngeal space are rarely reported, and complete resection of such tumor is recommended. Adjuvant chemoradiotherapy is used in patients with extensive tumor invasion to lower the recurrence rate. Postoperative long-term follow-up is required.     

甲状腺孤立性纤维性肿瘤的超声诊断学特征并文献复习

目的探讨甲状腺孤立性纤维性肿瘤的超声诊断学特征。 方法回顾性分析2020年7月29日就诊于济宁医学院附属医院甲状腺外科的1例甲状腺孤立性纤维性肿瘤患者的临床资料和影像学、病理学检查结果，并复习相关文献。 结果患者颈前偏左局限性隆起，甲状腺左叶可触及一明显肿物。超声可见甲状腺左叶一不均质回声团块，大小约5.8 cm×4.0 cm×2.7 cm，界清，形态规则，后方回声衰减，内可及较丰富的分支状血流信号。甲状腺CT平扫及增强扫描可见甲状腺左叶内略低密度影，增强后呈不均匀性延迟强化，呈"快进慢出"的特点。术后病理诊断为孤立性纤维性肿瘤。 结论超声对甲状腺孤立性纤维性肿瘤的大小、形态、内部回声、血流情况具有一定特征性，结合其他检查可提高该病的临床诊断率。     

Successful laparoscopic treatment of a giant solitary fibrous tumor of the mesorectum: A case report and literature review

A solitary fibrous tumor is a ubiquitous mesenchymal fibroblastic tumor that was previously considered limited to the pleural cavity. Here, we report a rare case of a large solitary fibrous tumor of the mesorectum, which was successfully resected laparoscopically. A 56‐year‐old woman was referred to our hospital for a giant pelvic mass. Pelvic MRI showed a well‐circumscribed mass, 12 cm in diameter, with heterogeneous signal intensity on T₂‐weighted images. It was diagnosed as a benign mesorectal tumor of unknown origin. We successfully resected the entire tumor laparoscopically. Histological examination revealed it to be an extrapleural solitary fibrous tumor. For large tumors in the pelvis, the laparoscopic approach is preferable in terms of intraoperative hemorrhage, as long as they do not invade surrounding tissues.     

肺孤立性纤维性肿瘤肉瘤变一例报告及文献复习

目的:探讨肺孤立性纤维性肿瘤(SFT)肉瘤变的临床病理学特征、诊断与鉴别诊断以及治疗和预后。方法:对1例肺SFT伴肉瘤变的标本进行临床病理学观察,并结合国内外文献进行复习。结果:患者男,57岁。因畏寒、发热2个月余,干咳10余天入院,胸部CT示左下肺病灶,体积9.5 cm×6.3 cm×5.5 cm,境界清楚。术中见肿瘤位于左下肺脏层胸膜下,表面光滑,质地较硬。镜检:肿瘤大部分区域由梭形细胞、大片玻璃样变的胶原和分支状血管构成,瘤细胞围绕血管周围排列成血管外皮瘤样,部分区域细胞丰富、密集,异型明显,病理性核分裂象>4/10HPF,并见坏死。免疫组织化学检查示,Vimentin+++、CD34++、CD99+++、Bcl-2++、CD117-、Des-、SMA-、S-100-、CKpan-,Ki-67增殖指数细胞密集区高达50%。病理诊断为肺SFT伴局部肉瘤变。术后随访16个月,一般状况良好。结论:SFT肉瘤变确诊依靠病理组织形态学和免疫组织化学检查,并应与肺部其他恶性间叶源性肿瘤进行鉴别。     

Diagnosis of solitary fibrous tumor in the submandibular gland: Case report and literature review

Solitary fibrous tumors (SFTs) rarely occur in the head and neck area. Imaging findings are nonspecific, and immunohistochemical (IHC) analysis is necessary for a definitive diagnosis. We report the case of a patient with a mass in the submandibular region that was initially diagnosed as basal cell adenoma. After excision and IHC examinations, a SFT was diagnosed.     

盆腔巨大型孤立性纤维瘤1例

患者,男,25岁,因车祸入院,行超声检查发现盆腔巨大占位.MRI平扫示盆腔内前列腺右前侧巨大的椭圆形稍长T1稍长T2异常信号,大小约9 cm×12 cm×12 cm,境界清晰,信号均匀,内可见血管流空信号影,增强扫描肿块明显均匀强化(图1、2),膀胱及前列腺明显受压变扁,膀胱向左上移位,邻近腰大肌及骨质信号未见明显异常;IVP示盆腔隐约可见巨大椭圆形占位,膀胱受压明显上移,左侧输尿管轻度扩张;CT平扫示盆腔内巨大软组织块影,密度均匀,境界尚清晰,膀胱明显受压向左上方移位(图3).影像诊断为间叶组织起源恶性肿瘤可能.     

左心室及心包恶性孤立性纤维性肿瘤1例

患者男,24岁,因发热,上腹部不适9天,胸闷、气促5天来诊。查体:胸骨左缘3~4肋间可闻及Ⅱ/6级收缩期杂音。MRI检查:升主动脉右侧心包内占位;双侧胸腔积液。超声见:左室流出道内等回声团,约32mm×22mm,其蒂似附着于主动脉瓣无冠瓣,瘤体随心脏舒缩在左室流出道及主动脉根部作往复活     

肾原发性孤立性纤维性肿瘤临床病理观察

目的 探讨原发于肾的孤立性纤维性肿瘤的临床病理特点及鉴别诊断要点.方法 复习2例肾孤立性纤维性肿瘤患者的临床资料,并对其进行组织学观察和免疫组化标记.结果 2例肾原发性孤立性纤维性肿瘤患者均为女性,年龄分别为33岁和63岁.临床主要表现为肾区钝痛,无尿频、尿急、尿痛,无血尿.CT检查示肾占位.组织学特征为瘤组织呈束状、波纹状排列,瘤细胞呈梭形,局部富于瘤细胞,间质血管丰富,部分呈血管瘤样或血管外皮瘤样结构,有明显的束状胶原.免疫表型：瘤细胞CD34、CD99和bcl-2（＋）,SMA局灶性（＋）;而HMB45和CD10（-）.结论 孤立性纤维性肿瘤具有低度恶性潜能,发生在肾极少见,以手术治疗为主,预后较好.确诊主要依靠组织病理学,并辅以免疫组化标记.     

巨大壁层胸膜孤立性纤维性肿瘤一例

孤立性纤维性肿瘤（SFT）是一种来源于间叶组织的梭形细胞性肿瘤。该病早期临床症状隐匿,常因肿瘤增大压迫周围组织而被发现,CT和（或）MRI是诊断SFT的常用手段,确诊需行病理活组织检查及免疫组织化学检查。该文报告l例巨大SFT患者,其以大量胸腔积液为首发表现且伴胸闷、呼吸困难等症状。行胸腔引流术后完善胸部CT检查,结果提示为左侧胸腔顶部肿物,由左锁骨下动脉供血,血供较丰富,考虑为壁层胸膜恶性肿瘤。其后行肿瘤血管栓塞术,待肿瘤缩小后行手术将其完全切除,术后病理活组织检查结果为SFT,患者术后恢复良好。由此可见,手术是治疗SFT的首选治疗方法,由于SFT血供常较丰富,故肿瘤较大时可于术前行肿瘤血管栓塞术,待肿瘤缩小后再行手术切除,以降低手术难度及风险,提高手术成功率。     

眼眶孤立性纤维性肿瘤超声表现1例

患者女,25岁,已婚,因发现"左侧眼球突出"9个月入院.眼科检查:VOD 1.0、VOS 0.3,右眼球突出16 mm,左眼球突出23 mm,左侧上眼眶皮下未扪及包块,无红肿、压痛,左眼球向外、向上运动受限;眼前节(-),眼底(-),眼压:正常(Tn).入院诊断:左眼眶肿瘤?超声表现:双侧晶状体及玻璃体未见明显异常.左眼球后软组织内可见一大小约2.5 cm×1.8 cm×1.3 cm的实性低回声肿块(图1),形态欠规则,边界欠清楚,内部回声不均质.彩色多普勒血流显像:包块内可见较丰富的血流信号(图2),频谱多普勒探测为动脉频谱,RI:0.57.     

Primary leiomyosarcoma of the thyroid gland with prior malignancy and radiotherapy:A case report and review of literature

BACKGROUND Leiomyosarcoma(LMS) of the thyroid gland is a rarely presented tumor that offers poor prognosis. To the best of the authors' knowledge, there currently exist only 28 known cases described in the literature(limited to English).CASE SUMMARY Herein a case is reported of a 60-year-old female patient who had an LMS of the thyroid, which was accompanied by periodic dysphonia and breathing disorder as well as the feeling of pressure in the chest and neck. At the time the disease was diagnosed, no metastases were detected. Prior to the diagnosis, the patient experienced a uterine adenocarcinoma that had been treated by surgical procedure and radiotherapy. For the LMS, a total thyroidectomy was performed,followed by radiotherapy. Since metastases were also discovered in the lungs,sternum, and femur, chemotherapy was administered as well.Immunohistochemically, the tumor cells in the thyroid indicated positively for alpha smooth muscle actin, calponin, and H-caldesmon, but were negative for CD34, p63, estrogen receptor, progesterone receptor, and Epstein-Barr virus.CONCLUSION Although the etiology of the LMS is as of yet unknown, prior malignancy and radiation should be considered as risk factors.     

胸腔孤立性纤维瘤伴阵发性低血糖症1例报道并文献复习

目的 研究胸腔孤立性纤维性肿瘤(PSFT)伴阵发性低血糖的病理特点和鉴别诊断。方法 通过组织形态学和免疫组化对胸腔孤立性纤维性肿瘤进行病理分析并复习文献。结果 肿瘤细胞由纤维母细胞样梭形细胞构成，束状交错排列及片状分布，局部呈血管外皮瘤形态。间质内有大量胶原纤维，部分区域黏液变性。在细胞密集区，可见核分裂象3个／10HPF。CD34、vimentin、somatotastin和insulin( )；CK、HBME-1，calvetinin、EMA、AE1／AE3、Cam5．2和p53(-)表达。结论 PSFT确能合成和分泌胰岛素样物质及生长抑素，但低血糖症则由多种因素造成。vimentin和CD34阳性以及上皮性标记阴性有助于诊断。     

Calcitonin-negative neuroendocrine tumor of the thyroid with metastasis to liver-rare presentation of an unusual tumor: A case report and review of literature

BACKGROUND Neuroendocrine tumors mainly occur in the stomach,intestine,pancreas,and lung and are rarely detected in the thyroid.Thyroid neuroendocrine tumors,designated medullary thyroid carcinoma,generally present with elevated calcitonin.Calcitonin-negative neuroendocrine tumors of the thyroid are extremely rare.CASE SUMMARY Here,we present a case report of a 56-year-old female patient with a neck pain complaint.Total thyroidectomy was conducted after comprehensive evaluation,and diagnosis was confirmed as calcitonin-negative neuroendocrine tumor of the thyroid.Two months later,liver metastasis was detected,and transcatheter arterial chemoembolization was subsequently performed to control growth.However,the curative effect was unsatisfactory and multiple intrahepatic metastases occurred after 3 mo.CONCLUSION Owing to the rarity of this disease,no clear guidelines are available for treatment.In addition to reporting this rare case,we have reviewed and summarized associated medical literature with an aim to provide a comprehensive reference platform for subsequent research.