Sarcoma Research Articles

Objective To study the preliminary effect of combining ligament advanced reinforcement system (LARS) and semi-joint replacement for malignant tumor around the knee in children. Methods 9 cases of malignant tumor around the knee (5 boys and 4 girls) from February 2015 to May 2017 were analyzed respectively. The average age was 9.2 years old (ranged from 5 to 12 years). The follow-up time was 6 to 28 months, with an average of 13.5 months. The preoperational biopsy diagnosis respectively were Ewing sarcoma (3 cases) and osteosarcoma (6 cases). According to Enneking staging system, all 9 cases were staged as IIB. The planned courses of standardized preoperative neoadjuvant chemotherapy were successfully given to all patients on time. All patients were given tumor extensive resection and modular prosthesis replacement. Suitable length prosthesis were prepared according to CT and MRI. LARS were annularly bundled to the prosthesis. Then residual patella ligaments, cruciate ligaments, collateral ligaments, capsules and muscles were tightly sutured to LARS. Adjuvant chemotherapy and functional exercise were given after operation. Bone healing, limb discrepancy, and complications were regularly recorded. Functional outcomes were assessed by the system of the Musculoskeletal Tumor Society (MSTS) and the range of motion (ROM) of both knee joints. Results All patients successfully received standardized chemotherapy. In all courses of chemotherapy, bone marrow all restored. No other major complications occurred during chemotherapy. Primary healing of incisions were obtained. No obvious limb discrepancy. The average limb length discrepancy was 2.9±1.8 cm (0.5~6.4 cm), the femur was 1.9±1.0 cm (0.6~3.9 cm), the tibia was 0.8±0.5 cm (0.2~2.0 cm). The distance between the lower limb alignment and the center of the knee was 0.3±0.1 cm (0.2~0.6 cm). MSTS score was 24.6±3.2 of the last follow-up, and 21.4±1.9 of preoperation, the difference was statistically significant (t=2.71, P=0.03). ROM of the knee were 71.7°±18.2° at the last follow-up, and 69.1°±17.9° before operation, and the difference was statistically significant (t=3.261, P=0.01). No infection, snapping knee, limp, dislocation, periprosthesis fractures, prosthetic broken or loosening. 2 case had lung metastasis and still survived. NO local recurrence or other metastasis cases. Conclusion LARS combined with semi-joint replacement for the treatment of malignant tumor around the knee in children have a satisfactory postoperative joint function recovery and simple surgical technique and fewer complications, and preserve the contralateral osteophytes to minimize the occurrence of limb inequality, but the long-term efficacy needs further follow-up. Key words: Sarcoma; Arthroplasty, replacement, knee; Knee prosthesis; Ligaments

Background: Many undifferentiated pleomorphic sarcoma (UPS) patients had lost the opportunity of surgical therapy. Microwave (MW) ablation of UPS can be an effective method for achieving local tumor control while preserving the relative function. We report for the first time the size of the ablation zone and temperature distribution of MW ablation on ex vivo human UPS specimens. Methods: Twenty UPS patients’ specimens were recruited into present experimental study (11 men, 9 women, average age 52±14.7 years). A commercial 2,450 MHz MW ablation apparatus was used throughout this experiment. MW ablations were performed immediately after resection of tissue specimens by using powers of 40, 60, 80 and 100 W for 10 min, and each power setting was performed in five specimens. Coagulation shape, lesion size, and temperature distributions were recorded. Histological examination was performed to assess the pathological changes of the ablation issue. Results: All coagulation geometries were ellipsoidal under the four power settings (40, 60, 80 and 100 W for 10 min) with a ratio of short-axis/long-axis range from 0.80 to 0.88. The short-axis diameters of the coagulation at 40, 60, 80 and 100 W groups were 28.2±1.1, 35.6±1.3, 40.6±1.6, and 46.4±1.7 mm, respectively; and the corresponding long-axis were 33.4±1.5, 41.4±1.7, 47.6±1.8, and 54.2±2.1 mm, respectively. Overall, temperatures at 3 points of distance 10, 20, and 30 mm from antenna increased along with ablation time. Routine histological analysis using haematoxylin-eosin (H&E) showed tissue fixation within the ablation area. Conclusions: The present ex vivo experiment showed that MW ablation by single 2,450 MHz antennae could generate an ellipsoidal coagulation geometry and large ablation zone, which increases with increasing power and time. This initial study provides experimental evidence for clinical MW ablation of UPS.

报道1例发生于舌部及心脏的髓系肉瘤(MS)患者，对MS的治疗及预后进行文献复习。显示发生于舌部及心脏的MS很罕见，联合化疗应为首选治疗，预后不佳，心脏MS预后极差，多因心包填塞、心力衰竭猝死。

Endometrial stromal sarcoma involve inferior vena cava complicateol with lower limb venous thrombosis: report of one case

Background: Surgical techniques for resection of tumors at proximal humerus and scapula has been described in literature along with different classification systems, however , these techniques have not been revised for a while and the classification systems didn’t respect the difference between bone and soft tissue tumors, or humerus vs. scapula locations. Material and methods: The author operated on 32 patients with shoulder girdle tumors, all are bone tumors, Ewings sarcoma (n=12), Osteosarcoma (n=7), Metastatic tumors (n=7), GCT (n=3), Chondrosarcoma (n=3). We assigned two separate classifications to humerus and scapula resection, since surgical techniques, mechanics and reconstruction is totally different for the both sites. Resection of the humerus classified into: Type I to Type IV, A: is added to the type when the majority of Deltoid is preserved, and B: when it is sacrificed. Type I: Intra articular proximal humerus resection Type II: Extra articular proximal humerus resection Type III: Intra articular total humerurs resection Type IV: Extra articular total humerus resection And we classify the scapula resection into: Type I to Type III Type I: Partial Scapular Resection Type II: Intra articular Total Scapular Resection Type III: Extra articular Scapular Resection In extra articular humerus resection, we found that sacrificing the acromion and coracoid process is not needed as a routine part of the extra articular resection of the proximal humerus and preservation of these structures can improve the cosmetic outcome of the shoulder., and for all tumors with no huge medial component, in our techniques there is no need to detach the muscle attachment from the coracoid process and so post operatively elbow extension as tolerated can be started immediately. Endoprosthesis was used in 23 patients for reconstruction, osteoarticular allograft was used in 3 patients, and Tichoff Lindberg technique for 3 patients. Results: At 30 month mean follow up period, 2 patients developed local recurrence (osteosarcoma n=1, Ewing Sarcoma n=1), and 2 patients infection, one patient stem loosening, the average MSTS functional score for all patient was 83%. Conclusion: The modification of surgical techniques saved structures which were unnecessarily resected, and kept the integrity of more muscular tissue and attachments which were detached in previous described techniques with no obvious advantage leading to less restriction during the rehabilitation process. The new classification system is realistic, easy to be recalled and applicable to all patients.

Carcinosarcoma is a malignant tumor composed of both epithelial and mesenchymal malignant tumor components. A 78-year-old man was transferred to our hospital because of hematemesis and tarry stool. An emergency gastrointestinal endoscopic examination revealed active bleeding from an ulcerative lesion on the posterior wall of the gastric body; endoscopic hemostasis was successfully performed. A gastrointestinal endoscopy performed two months later showed a polypoid lesion at the same place where the ulcer had been. The biopsy specimen was histologically diagnosed as well to moderately differentiated tubular adenocarcinoma. The patient underwent a laparoscopic distal gastrectomy with D2 lymph node dissection under a diagnosis of gastric adenocarcinoma. A 28 × 15 mm polypoid tumor was resected from the gastric body, and was found on microscopic examination to consist of both carcinoma and sarcoma components, showing atypical spindle cells, which were positive for α-smooth muscular actin, calponin, and h-caldesmon, but negative for CD34, CD117 (c-kit), desmin, and dog 1. These findings led to a diagnosis of gastric carcinosarcoma. The tumor was confined to the mucous membrane. Lymph node metastasis was found in one node and contained only the carcinoma component. The postoperative course was uneventful. The patient lived without recurrence for 2 years. Carcinosarcoma of the stomach is a rare tumor with high malignant potential and poor prognosis. Careful follow up is required for early detection of any recurrence.

Cancer immunotherapies using gene-engineered T cells comprise adoptive transfer of T-cell receptor (TCR) and chimeric antigen receptor (CAR) gene-transduced T cells. Although CD19-targeting CAR-T cell therapy is the most progressed, wherein B-cell malignancy is treated efficiently, it also induces cytokine release syndrome and neurotoxicity, which frequently leads to serious adverse events. Of note, TCR-T cell therapy has been primarily used to target melanoma, resulting in 30%-50% of tumor responses. In clinical trials that target NY-ESO-1-expressing synovial sarcoma, a high efficacy of 50%-60% has been obtained. To date, no specific clinical efficacy has been reported for epithelial tumors. Serious on-target adverse effects in normal tissues have been reported when using affinity-enhanced TCR of mutated or mouse-derived ones. Furthermore, there are potential risks in using high-affinity TCRs and in targeting tumor antigens that may also be expressed in normal tissues.

Improved, streamlined total syntheses of natural tubulysins such as V (Tb45) and U (Tb46) and pretubulysin D (PTb-D43), and their application to the synthesis of designed tubulysin analogues (Tb44, PTb-D42, PTb-D47-PTb-D49, and Tb50-Tb120), are described. Cytotoxicity evaluation of the synthesized compounds against certain cancer cell lines revealed a number of novel analogues with exceptional potencies [e.g., Tb111: IC50 = 40 pM against MES SA (uterine sarcoma) cell line; IC50 = 6 pM against HEK 293T (human embryonic kidney cancer) cell line; and IC50 = 1.54 nM against MES SA DX (MES SA with marked multidrug resistance) cell line]. These studies led to a set of valuable structure-activity relationships that provide guidance to further molecular design, synthesis, and biological evaluation studies. The extremely potent cytotoxic compounds discovered in these investigations are highly desirable as potential payloads for antibody-drug conjugates and other drug delivery systems for personalized targeted cancer chemotherapies.

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid follicles. FDCS has been increasingly reported in recent years. However, data on FDCS are mostly based on single case reports or case series and its natural history and standard treatment are not clear. To increase the understanding of this rare disease, we report our experience of three cases of FDCS with an analysis of the morphological and immunophenotypic characteristics, clinical course, treatment options and response to treatment. In addition, we reviewed the literature on FDCS.

We report a rare case of Ewing's sarcoma (ES)/primitive neuroectodermal tumor (PNET) arising from the adrenal gland. A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 × 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma. Resection of the tumor was performed. We obtained the final diagnosis of ES/PNET by immunohistochemical molecular study with positive staining for the MIC2 gene product (CD99) and a Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement. Local recurrence was observed one month after the surgery. The patient was then treated with systemic chemotherapy and localized radiotherapy.

We describe two paradigmatic cases where metronomic antitumor chemotherapy was successfully employed in patients not suitable for standard treatments. The first patient was affected by advanced soft tissue sarcoma but she also had ischemic cardiopathy. She received oral cyclophosphamide 50 mg once daily and methotrexate 2.5 mg bid twice weekly, obtaining a significant clinical response with a progression-free survival of 7 months. The second patient was over 70 years of age and suffered from metastatic gastric cancer. Because of his poor performance status he was given capecitabine 1500 mg daily, achieving a complete remission with a current disease-free survival of 13 months. In both cases no significant toxicities were observed.

Important role of gemcitabine in the treatment of classic Kaposi's sarcoma.

This article focuses on a rare case of primary renal angiosarcoma in a 56-year-old man with a previous clinical history of stage III Hodgkin's lymphoma treated with supra- and subdiaphragmatic radiotherapy, splenectomy in association with vincristine-based chemotherapy and thermochemotherapy, and subsequent thymectomy. The patient was referred to the department of urology from the department of cardiology, where he had been seen for right coronary stenosis after the incidental finding on an abdominal scan of a large left renal mass. There was no family history of renal cancer. Diagnosis was high-grade angiosarcoma, extensively necrotic and hemorrhagic, involving the renal parenchyma and perirenal soft tissue. Taking into account tumor histology, grade, size and site as well as patient's age and general condition, a therapeutic program was planned comprising surgery followed by chemotherapy (epirubicin 60 mg/m² and ifosfamide 3000 mg/m²). Adjuvant radiotherapy, normally delivered to the site of surgery, was not considered necessary. Unfortunately the patient died 4 months after surgery, before chemotherapy was started. Our paper highlights the extreme rarity and aggressiveness of renal sarcoma, its poor prognosis, and the fact that there is no one, accepted approach to its treatment.

Malignant fibrous histiocytoma is an aggressive tumor, the most common soft-tissue sarcoma of adult age. It is usually located in the extremities and retroperitoneum, and very rarely there is skeletal involvement. Surgery is the preferred treatment in early disease; in advanced disease, chemotherapy is the main therapeutic strategy. We present a 25-year-old female patient diagnosed with a vertebral mass in T5 with a severely compromised spinal cord. She underwent surgical decompression and the pathological findings were consistent with malignant fibrous histiocytoma. After several surgical treatments she had pulmonary progression and was therefore started on chemotherapy. She had a very poor response to most of the administered regimens until she initiated trabectedin 1 mg/m 2 every three weeks. She showed a significant improvement with a major response of the lung metastases. This report indicates that trabectedin is an active drug in advanced, previously treated metastatic malignant fibrous histiocytoma.

A case of psoriasis complicated by bullous pemphigoid with secondary Kaposi sarcoma

目的 通过学习前列腺髓系肉瘤探讨其临床特点及诊疗策略等。 方法 回顾性报道1例前列腺髓系肉瘤患者的临床表现、治疗方案和治疗效果，并复习相关文献资料。 结果 患者行DA方案化疗，3个月后患者复查急性粒细胞白血病复发，1年后死亡。 结论 前列腺髓系肉瘤常为急性粒细胞性白血病的局部表现之一，或为其复发的早期症状，需要早期给予急性白血病化疗方案进行化疗，结合局部手术治疗、外周血造血干细胞移植、放疗及靶向治疗等治疗。总体来说，髓系肉瘤预后差。

Detection of V-raf murine sarcoma viral oncogene homolog B1 (BRAF) V600E mutation is important for clinical diagnosis, treatment decision-making, effect judgement and prognosis evaluation in papillary thyroid carcinoma (PTC). Research has shown that peripheral blood BRAF V600E is closely related to the histological level of BRAF V600E. Detection of peripheral blood BRAF V600E mutation will be less traumatic, more convenient and dynamic, indicating that it will be the future development trend of PTC research. Key words: V-raf murine sarcoma viral oncogene homolog B1 oncogene; Circulating tumor DNA; Papillary thyroid carcinoma; Progress

Malignant tumors of extremity are mostly of mesenchymal origin and arise from bone or extraskeletal soft tissue. Combination chemotherapy has greatly increased the survival of non-metastatic localized osteosarcoma and Ewing's sarcoma; however, there is conflicting evidence regarding the role of neoadjuvant and adjuvant chemotherapy in soft tissue sarcoma due to the rarity and heterogeneous population. Despite intensive multimodal therapy and valiant efforts, most of the patients with relapsed and metastatic sarcoma will succumb to their disease. Molecular studies like next-generation sequencing have revealed various targetable biomarkers which can be further explored in the therapeutic landscape with specific targeted therapies. We reviewed all ongoing and completed clinical trials involving chemotherapy, targeted therapy, and immunotherapy valid for patients with extremity sarcoma. We present the current viable therapeutic options for such a cohort of patients in routine clinical practice.

Objective To analyze the efficacy of total claviculectomy in the treatment of Ewing's sarcoma and to discuss the impact of clavicular reconstruction on the outcome. Methods The clinical records of a 13-year-old male patient with left clavicular Ewing's sarcoma were reviewed. The subject received 2 cycles (at an interval of 2 weeks) of neoadjuvant chemotherapy comprising cisplatin 120 mg/m2 (1 d), adriomycin 30 mg/m2 (3 d), and ifosfamide 2.0 g/m2 (5 d). Total claviculectomy was carried out without clavicular reconstruction and the patient underwent 9 cycles of subsequent chemotherapy. The appearance and function of the operated limb, radiological examinations of the surgical site, ECT bone scans and pulmonary CT were followed up. The literatures regarding the total and subtotal excision of the clavicle were reviewed to investigate the importance of clavicular reconstruction. Results After 23-months of follow-up, the patient was subjectively free of limb function compromise. Physical examinations indicated drooping of the operated shoulder by 2 cm, whereas the appearance was similar between both shoulders. Functionality of the operated limb was recovered. The motion of the left shoulder: abduction 90°, adduction 40°, anterior flexion 90°, extension 45°, internal rotation 80°, external rotation 60°, and no restriction of limb elevation. Manual muscle strength tests showed V/V muscle strength of the operated limb. American Shoulder and Elbow Surgeons' form (ASES) and Musculoskeletal Tumor Society (MSTS) score were 96 and 30, respectively. Postoperative X-ray films demonstrated mild thoracic scoliosis. The patient did not appear recurrence and metastasis. Conclusion Total claviculectomy is efficacious for treating clavicular Ewing's sarcoma. Clavicular reconstruction may not be imperative for treatment of clavicular malignancy because of the increased technical difficulty and great risk of complications. Key words: Sarcoma, Ewing; Clavicle; Surgical procedures; Bone remodeling; Recovery of function

Case reportEwing’s sarcoma is a highly malignant, poorly differentiated bone tumour of childhood and adolescence primarily involving diaphysis of long bones; femur, tibia, fibula, and humerus and may also occur in other bony structures and cartilage tissue. It is a very clinically aggressive tumour