Risk factors related to postoperative recurrence of dermatofibrosarcoma protuberans: A retrospective study and literature review

BACKGROUNDDermatofibrosarcoma protuberans (DFSP) is a rare low-grade malignant soft tissue tumor characterized by rosette-like infiltrative growth. Postoperative recurrence of this tumor is very common. AIMTo evaluate the risk factors related to recurrence after wide local excision (WLE) of DFSP and to guide clinical diagnosis and treatment.METHODSThe medical records of 44 DFSP patients confirmed by pathology at our hospital from 2012 to 2019 were retrospectively reviewed. The relationship between clinical features, tumor characteristics, treatment, and recurrence risk were analyzed, and the possible risk factors for postoperative tumor recurrence were evaluated.RESULTSThere were 44 patients in total, including 21 males and 23 females. The median progression free survival was 36 mo (range, 1-240 mo). Twenty patients were treated for the first time, while 24 had previous treatment experience. Forty-two cases were followed for 25.76 ± 22.0 mo, among whom four (9.52%) experienced recurrence after WLE (rate was 9.52%). The recurrence rate in the recurrent group was higher than that in the patients with primary tumor (19.05% vs 0%, P = 0.028). Eighteen cases had a history of misdiagnosis (rate was 40.91%). The recurrence rate among patients with previous experience of misdiagnosis was significantly higher than in patients without (68% vs 36.84%, P = 0.04). The tumor diameter in patients with a history of treatment was larger than in patients treated for the first time (4.75 ± 0.70 cm vs 2.25 ± 0.36 cm, P = 0.004). CONCLUSIONTo sum up, the clinical manifestations of DFSP are not specific and are easily misdiagnosed, thus commonly causing the recurrence of DFSP. After incomplete resection, the tumor may rapidly grow. Previous recurrence history may be a risk factor for postoperative recurrence, and tumor location may have an indirect effect on postoperative recurrence; however, we found no significant correlation between sex, age, course of the disease, or tumor size and postoperative recurrence.     

多层螺旋CT诊断肠系膜侵袭性纤维瘤病

目的 分析肠系膜侵袭性纤维瘤病的多层螺旋CT表现。 方法 6例经手术病理证实的肠系膜侵袭性纤维瘤病患者术前均接受多层螺旋CT平扫和增强扫描。 结果 6例病灶均为单发,最长径平均为15 cm;4例病灶形态规则呈类圆形,2例呈分叶状;4例病灶与周围肠管分界清晰,2例病灶侵犯局部小肠,肿块内可见气体影。CT平扫4例病灶呈均匀软组织密度,2例病灶呈混杂密度,其中1例病灶内有气-液平面,1例病灶内有囊变区。增强扫描病灶以轻-中度强化为主,内见散在小斑片状明显强化区,3例可见肠系膜血管分支进入肿块。所有病灶均无钙化,腹腔及后腹膜无肿大淋巴结、无腹腔积液。 结论 提高对CT征象的认识是正确诊断肠系膜侵袭性纤维瘤病的关键。     

Reconstruction of the chest wall after resection of malignant peripheral nerve sheath tumor: A case report

BACKGROUNDMalignant peripheral nerve sheath tumors (MPNSTs) are a group of rare and aggressive sarcomas that often arise from major peripheral nerves and represent a notable challenge to efficacious treatment. MPNSTs can occur in any body surface and visceral organs with nerve fiber distribution. The treatment options for MPNSTs include surgery, chemotherapy, and adjuvant radiotherapy. CASE SUMMARYA 26-year-old female cellist presented with chest pain on her left side when she squatted to lift the cello. One week later, a chest X-ray was performed and revealed fracture of the fourth rib on the left side. Three months later, the patient inadvertently touched a mass on the left side of the chest wall. Chest computed tomography (CT) three-dimensional reconstruction of the ribs revealed bone destruction of the fourth rib on the left side with a soft tissue mass shadow measuring 5.7 cm × 3.7 cm. CT-guided puncture biopsy of the tumor showed that heterotypic cells (spindle cells) tended to be nonepithelial tumor lesions. PET-CT demonstrated bone destruction and a soft tissue mass with avid 18F-fluorodeoxyglucose activity (SUV_max7.5) in the left fourth rib. The tumor of the left chest wall was resected under general anesthesia, and reconstruction of the chest wall was performed. The postoperative pathological report exhibited an MPNST.CONCLUSIONMPNSTs are relatively chemo-insensitive tumors. The mainstay of treatment for MPNSTs remains resection with tumor-free margins.     

腹部韧带样型纤维瘤病临床特点及治疗探讨

目的探讨腹部韧带样型纤维瘤病（desmoid-type fibromatosis,DTF）的临床特点及治疗方法。方法对我院2006年4月～2011年4月收治的26例腹部DTF临床资料进行回顾性分析。结果本组自行发现或体检时偶然发现腹部肿块24例（92.3%）,腹部不适2例（7.7%）。有腹部手术史10例（38.5%）。术前确诊DTF2例（7.7%）,诊断为腹壁肿瘤20例（76.9%）,余4例分别误诊为原发性肝癌、结肠癌、小肠肿瘤、盆腔肿瘤各1例（各占3.8%）。26例均行手术切除并经病理检查确诊,术后均恢复良好,无术后并发症发生,随访未见肿瘤局部复发及转移。结论 DTF多见于经产妇女,好发于既往有手术史者,以腹壁发病率为最高,肿瘤病理表现虽为良性,但临床上具有浸润性生长和易复发的特点,多发生于深部软组织,手术为该病主要治疗方法。     

骶骨肿瘤的CT影像表现

目的 分析骶骨肿瘤的ＣＴ影像特点。方法回顾分析３０例经是证实的骶骨肿瘤影像学特点。结果 ３０例骶骨肿瘤包括转移瘤１０例,脊索瘤５例,骨巨细胞瘤４例,神经源性肿瘤３例,软骨肉瘤３例,其它５例,其ＣＴ影像学表现各有异同。结论从发病年龄、骶骨骨质破坏的形式、是否伴有硬化边缘,有否钙化、软组织肿块等方面分析,有助于骶骨肿瘤的诊断及鉴别诊断。     

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.     

Pancreas-preserving duodenectomy for treatment of a duodenal papillary tumor: A case report

BACKGROUNDDuodenal papillary tumor is a rare tumor of the digestive tract, accounting for about 0.2% of gastrointestinal tumors and 7% of periampullary tumors. The clinical manifestations of biliary obstruction are most common. Some benign tumors or small malignant tumors are often not easily found because they have no obvious symptoms in the early stage. Surgical resection is the only treatment for duodenal papillary tumors. At present, the methods of operation for duodenal papillary tumors include pancreatoduodenectomy, duodenectomy, ampullectomy, and endoscopic resection.CASE SUMMARYA 47-year-old man was admitted to because of a duodenal mass that had been discovered 2 mo previously. Electronic gastroscopy at another hospital revealed a duodenal papillary mass that had been considered to be a high-grade intraepithelial neoplasia. Therefore, we conducted a multidisciplinary group discussion and decided to perform a pancreas-preserving duodenectomy and a R0 resection was successfully performed. After surgery, the patient underwent a follow-up period of 5 yr. No recurrence or metastasis occurred. CONCLUSIONAccording to our experience with a duodenal papillary tumor, compared with pancreaticoduodenectomy, the use of pancreas-preserving duodenectomy can preserve pancreatic function, maintain gastrointestinal structure and function, reduce tissue damage and complications, and render the postoperative recovery faster. Pancreas-preserving duodenectomy for treatment of a duodenal papillary tumor is feasible under strict control of surgical indications.     

Multiple well-differentiated retroperitoneal liposarcomas with different patterns of appearance on computed tomography: A case report

BACKGROUNDPrimary retroperitoneal liposarcoma (PRPLS) is a rare soft tissue tumor with nonspecific clinical symptoms; it has different computed tomography (CT) image features according to pathological types. Some patients with a single tumor have been previously reported in the literature. We present an exceptional case of a PRPLS patient with multiple large tumors exhibiting different patterns of appearance on CT and confirmed as atypical lipomatous tumor/well-differentiated liposarcoma by postoperative pathology. CASE SUMMARYA 64-year-old man presented with abdominal distension for 1 year. The patient was diagnosed with PRPLS based on physical examination, laparotomy, ultrasonography, CT scan, and surgery. Both of the tumors were completely resected through surgery and confirmed as atypical lipomatous tumor/well-differentiated liposarcoma by postoperative pathology. The postoperative course was uneventful without recurrence or metastasis, as demonstrated by abdominal-pelvic CT during an 18 mo follow-up. CONCLUSIONMultiple large Well-differentiated liposarcomas with different patterns of appearance on CT image can occur simultaneously in the same patient, to which more attention should be paid to make an effective differential diagnosis.     

外周性原始神经外胚层肿瘤的影像学表现

目的探讨外周性原始神经外胚层肿瘤(pPNETs)的影像学表现,以提高对本病的认识。方法回顾性分析15例pPNETs的临床及影像学资料,其中软组织pPNETs6例,骨pPNETs9例;7例行X线检查,12例行CT检查,9例行MRI检查。结果骨pPNETsX线及CT平扫表现为范围较广泛、边缘模糊的溶骨性骨质破坏,周围均见大小不等软组织肿块影,大小为2.5cm×2.8cm~10cm×13cm,平均6.2cm×7.4cm;6例病灶增强扫描后见肿块强化,但强化多不均匀。软组织pPNETsCT平扫肿瘤密度不均匀,实质部分为等密度,内可见不同程度的坏死、囊变区;肿瘤大小2.7cm×4.8cm~12.4cm×14.5cm,平均6.7cm×8.2cm;增强扫描后均呈不均匀强化。MRI示病灶T1WI呈等信号6例,等-低混杂信号1例,中等稍高信号2例;T2WI均呈不规则中、高信号;8例钆喷替酸葡甲胺(Gd-DTPA)增强后均表现为不均匀强化。结论pPNETs多表现为溶骨性骨质破坏和(或)巨大软组织肿块,其内常见不同程度的坏死、囊变。CT、MR能较好显示肿瘤的内部结构、明确肿瘤的范围,有助于本病的诊断及鉴别诊断;对判定手术的可切除性、检出远处转移及评价治疗效果等具有较大的价值。     

腹部外周性原始神经外胚层肿瘤的临床及CT、MR诊断

目的 分析腹部外周性原始神经外胚层肿瘤(pPNET)的临床特点、CT与MRI表现.方法 对有完整影像学及病理学资料的13例腹部pPNET患者进行回顾性分析.结果 本组腹部pPNET的临床特点主要为青少年快速增大的包块伴疼痛,易转移复发,预后差.CT及MRI表现为体积较大、呈浸润性生长的软组织肿块,密度(或信号)不均,常见坏死囊变,无钙化,增强后不均匀强化,可见间隔及网格状强化;病灶对周围正常组织有侵袭,侵犯骨质结构时呈溶骨性骨质破坏,多无骨膜反应及瘤骨.结论 腹部pPNET临床表现及CT、MRI表现无明显特征,但CT与MR能较准确描述肿瘤内部结构、毗邻关系及转移情况,对制订治疗方案和评价疗效有重要价值.     

髋周侵袭性软组织肿瘤的MRI诊断及病理对照

目的总结髋周的侵袭性软组织肿瘤的MRI表现,探讨MRI对髋周软组织肿瘤术前诊断的价值。方法经手术病理证实的19例髋周侵袭性软组织肿瘤,男12例,女7例,平均年龄45．2岁。所有病例均行常规MRI平扫及Gd—DTPA增强扫描,分析其MR表现。结果侵袭性纤维瘤病5例,恶性纤维组织细胞瘤5例,滑膜肉瘤2例,脂肪肉瘤4例,淋巴瘤1例,原始神经外胚层肿瘤1例,转移瘤1例。侵袭性纤维瘤病边界不清,T1WI呈等信号,T2WI呈稍高信号,其内见星芒状低信号带。增强扫描明显均匀强化。恶性纤维组织细胞瘤、脂肪肉瘤T1WI信号呈等或稍低信号,T2WI呈稍高信号或高信号,其内见点状或片状明显高信号区。病灶周围肌肉常有水肿信号。增强扫描肿瘤明显不均匀强化。滑膜肉瘤内见液平,肿块呈多结节样。原始神经外胚层肿瘤、淋巴瘤与转移瘤周围肌肉水肿显著。结论MRI对髋周软组织肿瘤术前诊断有较高的参考价值。     

Innovative chest wall reconstruction with a locking plate and cement spacer after radical resection of chondrosarcoma in the sternum: A case report

BACKGROUNDChondrosarcoma, a cartilage matrix producing tumor, is the second most commonly observed primary bone tumor after osteosarcoma, accounting for 15% of all chest wall malignancies. We herein report the case of a patient with chondrosarcoma of the sternum and our management of the chest wall defects that presented following radical tumor resection.CASE SUMMARYA 31-year-old patient presented to our hospital with dull pain and a protruding mass overlying the chest for 3 mo. The presence of nocturnal pain and mass size progression was reported, as were overhead arm elevation-related limitations. Computed tomography showed a focal osteoblastic mass in the sternum with bony exostosis and adjacent soft tissue calcification. Positron emission tomography-computed tomography revealed hypermetabolic activity with a mass located over the upper sternum. Magnetic resonance imaging showed a focal ill-defined bony mass of the sternum with cortical destruction and periosteal reaction. Preoperative biopsy showed a consistent result with chondrosarcoma with immunohistochemical positivity for S100 and focal positivity for IDH-1. The grade II chondrosarcoma diagnosis was confirmed by postoperative pathology. The patient underwent radical tumor resection and chest wall reconstruction with a locking plate and cement spacer. The patient was discharged 1 wk after surgery without any complications. At the 1-year follow-up, there was no local recurrence on imaging. The functional scores, including Constant Score, Nottingham Clavicle Score, and Oxford Shoulder Score, showed the absence of pain in the performance of daily activities or substantial functional disabilities.CONCLUSIONThe diagnosis of chondrosarcoma must be considered when chest wall tumors are encountered. The surgical reconstructive materials, with a locking plate and cement spacer, used in our study are cost-effective and readily-available for the sternum defect.     

99Tcm-MDP骨显像评估脊柱成骨细胞瘤

目的观察脊柱成骨细胞瘤^99 Tc^m-MDP骨显像表现,探讨骨显像用于术后评估的价值。方法回顾性收集24例病理证实的脊柱成骨细胞瘤影像学及临床资料,分析其术前、术后骨显像及同期CT特点,对比骨显像及CT诊断成骨细胞瘤术后复发的价值。结果14例接受术前骨显像,病灶均呈显著浓聚,其中13例后位像显示较前位像清晰;11例接受同期CT扫描,病变长径1.3~4.2 cm,9例见膨胀性溶骨性破坏,内部可见钙化或骨样密度影;2例呈单纯骨样密度。12例接受术后骨显像,6例显示病变复发,其中5例呈显著浓聚、1例轻度浓聚,5例接受同期CT,其中4例呈骨样密度影,1例软组织密度影伴钙化;6例未见复发,3例骨显像呈放射性减低伴周围浓聚,1例轻度浓聚、1例放射性减低、1例未见异常,5例接受同期CT,1例示术区软组织影、2例碎骨片影、2例未见异常。结论原发及复发性脊柱成骨细胞瘤骨显像多呈显著放射性浓聚。骨显像可用于脊柱成骨细胞瘤术后评估及指导临床决策,术后骨显像阴性可排除肿瘤复发。     

Adult rhabdomyosarcoma originating in the temporal muscle,invading the skull and meninges: A case report

BACKGROUNDRhabdomyosarcoma (RMS) is a rare malignant tumor of mesenchymal origin that mainly affects children. Spindle cell/sclerosing RMS (SSRMS) is even rarer. It is a new subtype that was added to the World Health Organization disease classification in 2013. To the best of our knowledge, this is the first reported case of adult SSRMS disease classification originating in the temporal muscle.CASE SUMMARYSSRMS originating in the temporal muscle of a male adult enlarged rapidly, destroyed the skull, and invaded the meninges. The tumor was completely removed, and the postoperative pathological diagnosis was SSRMS. Postoperative recovery was good and chemotherapy and radiotherapy were given after the operation. Followed up for 3 mo, no tumor recurred.CONCLUSIONRMS is one of the differential diagnoses for head soft tissue tumors with short-term enlargement and skull infiltration. Preoperative computed tomography or magnetic resonance imaging is necessary for early detection of tumor invasion of the skull and brain tissue.     

肢体和躯干软组织肿块的CT评价

目的探讨肢体和躯干软组织肿块的ＣＴ征象在诊断中的价值。方法分析２０例经手术病理证实的肢体及躯干软组织肿块增强前后的ＣＴ资料。２例恶性肿瘤,１８例良性病变。结果良性病变中囊肿４例,３例为边缘光滑的水样密度,１例有出血感染密度增高,无可化;脂肪瘤３例,有特征性,为边界清楚的ＣＴ值－５０～－１３０Ｈｕ的低密度灶;血肿２例和软组织感染１例均有明显的临床病史,病灶边缘不规则,界限不清。侵袭性血管瘤１例,增强     

Paratesticular liposarcoma: Two case reports

BACKGROUNDParatesticular liposarcoma accounts for approximately 7% of scrotal tumors. They are rare lesions of the reproductive system with approximately 90% of the lesions originating from the spermatic cord. Surgery, with the goal of complete resection, is the mainstay for treatment of this disease. However, treatment consisting of extended resection to decrease local recurrence remains controversial.CASE SUMMARYWe report the cases of two patients with paratesticular liposarcomas who were treated with radical testicular tumor resection without adjuvant therapy. Follow-up investigations at 9 mo showed no sign of recurrence.CONCLUSIONSurgery is the first-line treatment, regardless of whether it is a recurrent or primary tumor. Extended resection carries a higher risk of complications and should not be performed routinely. Preoperative radiotherapy can reduce the local recurrence rate without affecting the overall survival.     

Malignant giant cell tumor in the left upper arm soft tissue of an adolescent: A case report

BACKGROUNDGiant cell tumor of soft tissue (GCT-ST) is an extremely rare low-grade soft tissue tumor that is originates in superficial tissue and rarely spreads deeper. GCT-ST has unpredictable behavior. It is mainly benign, but may sometimes become aggressive and potentially increase in size within a short period of time.CASE SUMMARYA 17-year-old man was suspected of having a fracture, based on radiography following left shoulder trauma. One month later, the swelling of the left shoulder continued to increase and the pain was obvious. Computed tomography (CT) revealed a soft tissue mass with strip-like calcifications in the left shoulder. The mass invaded the adjacent humerus and showed an insect-like area of destruction at the edge of the cortical bone of the upper humerus. The marrow cavity of the upper humerus was enlarged, and a soft tissue density was seen in the medullary cavity. Thoracic CT revealed multiple small nodules beneath the pleura of both lungs. A bone scan demonstrated increased activity in the left shoulder joint and proximal humerus. The mass showed mixed moderate hypointensity and hyperintensity on T1-weighted images, and mixed hyperintensity on T2-weighted fat-saturated images. The final diagnosis of GCT-ST was confirmed by pathology.CONCLUSIONGCT-STs should be considered in the differential diagnosis of soft tissue tumors and monitored for large increases in size.     

韧带样型纤维瘤病的影像表现及其病理基础

目的 探讨韧带样型纤维瘤（DF）的影像学特征及病理学基础.方法 回顾性分析经手术病理证实的韧带样型纤维瘤14例,6例行CT检查,其中 3 例增强检查;8例行 MRI 检查,其中7例增强.分析CT及MRI表现,并对肿瘤的病理特点进行分析.结果 肿瘤平均直径6.2cm.肿瘤形态为不规则形或分叶状11例（11/16）.肿瘤与周围结构分界不清或部分分界不清的9例（9/16）.7个肿瘤在CT上呈稍低或等密度,3例增强后呈中度或高度强化.9例病灶在T1WI图像上呈等或稍低信号,在T2WI上呈稍高信号,8个病灶呈中度或高度强化.9个病灶内有条索状、片状的长T1、短T2区域,增强后未见强化.光镜下瘤细胞长梭形,无明显异型性,核分裂少见,呈侵袭性生长,浸润周围的横纹肌及脂肪组织,伴胶原纤维增生及程度不等的透明变性、黏液变性.结论 韧带样型纤维瘤影像学变现具有一定的特异性,认识其病理学改变有助于解释该病的影像学表现,有助于提高术前诊断率,磁共振对手术切除范围的判断具有优势.