Soft-tissue sarcoma in adults: An update on the current state of histiotype-specific management in an era of personalized medicine

Soft-tissue sarcomas (STS) are rare tumors that account for 1% of all adult malignancies, with over 100 different histologic subtypes occurring predominately in the trunk, extremity, and retroperitoneum. This low incidence is further complicated by their variable presentation, behavior, and long-term outcomes, which emphasize the importance of centralized care in specialized centers with a multidisciplinary team approach. In the last decade, there has been an effort to improve the quality of care for patients with STS based on anatomic site and histology, and multiple ongoing clinical trials are focusing on tailoring therapy to histologic subtype. This report summarizes the latest evidence guiding the histiotype-specific management of extremity/truncal and retroperitoneal STS with regard to surgery, radiation, and chemotherapy.     

Single Fraction Radiation for Myeloid Sarcoma Is as Effective as Multi-Fraction Regimens for Tumor Regression and Control

Introduction/BackgroundMyeloid sarcoma is a rare extramedullary manifestation of immature myeloid/monocyte cells. Radiotherapy (RT) yields good local control, but data on different fractionation schemes are limited. The goal of this retrospective study was to share our institutional experience and assess volumetric regression with differential fractionation.Materials and MethodsWe evaluated patients treated for myeloid sarcoma between 2000 and 2019 and categorized them into Group A (treated with RT) and Group B (no RT). We assessed local control using cumulative incidence function analysis. Post-treatment imaging sequences were analyzed for volumetric calculations.ResultsForty-four patients with 80 lesions were assessed. Twenty-three patients with 52 lesions received RT (Group A), and 6 lesions received a single fraction of RT. There were 2 instances of local progression in Group A and 8 in Group B, with a cumulative incidence function estimate of local progression in Group A of 2.4% at 1 year and 6.9% at 2 years, significantly reduced compared to 29.7% and 35.5% in Group B, respectively (hazard ratio 0.13 [95% confidence interval 0.030.63], P = .011). No lesion treated with a single fraction of RT developed local progression. Volumetric analysis for 19 chronologically followed lesions (including 3 treated with a single fraction) revealed no difference in regression between single or multi-fraction treatment.ConclusionRT for myeloid sarcoma yields excellent local control and may be as effective in a single fraction as more protracted courses, though this requires validation. For a diagnosis associated with poor survival, a single palliative fraction may be optimal with potential for higher utilization.     

Extraskeletal Ewings sarcoma

A case of Ewings sarcoma arising in the soft tissue of the thumb is described. This appears to be the first case of digital extraskeletal Ewings sarcoma. The soft tissue location, the primary rather than metastatic nature, and the tumor type in this case were well documented.     

Clinicopathologic Features and Clinical Outcome Differences in De Novo Versus Secondary Histiocytic Sarcomas: A Multi-institutional Experience and Review of the Literature

Introduction

Histiocytic sarcoma (HS) is a rare malignant neoplasm that can occur in patients with a history of treatment for hematologic or solid tumors. Because no optimal treatment has been defined and standardized, the treatment modalities used and outcomes reported have been highly variable. In the present study, 3 major institutions explored the clinicopathologic features of de novo and secondary HS.

Historical note on bone and soft tissue sarcoma

Since antiquity, the large bony and fleshy tumors have been called sarcomas. It was not until after the development of cellular pathology during the middle of the 19th century that sarcomas were separated from carcinomas on the basis of their tissues of origin. This paper traces the growth of these ideas as well as the introduction of effective methods of treatment of the sarcomas.     

Nonosseous sarcomas in a military hospital

This is a retrospective review of 67 patients with soft tissue or visceral sarcomas treated during 1973-1982. There were 40 males and 27 females. The mean age was 36 with a range of 1 to 77 years of age. The most common histologic subtypes were rhabdomyosarcoma (19%), malignant fibrohistiocytoma (13%), fibrosarcoma, leiomyosarcoma, dermatofibrosarcoma protuberans (12% each), and liposarcoma (10%). Among the 42 soft tissue sarcomas 33% occurred in the trunk, 35% and 21% in the lower and upper extremities, and 10% in the head and neck region. Among the 25 visceral sarcomas, 16% were in the thorax, 56% involved abdominal or retroperitoneal organs, and 28% were in the head and neck region. Overall, 33 of the 54 patients with early sarcomas were treated by surgical resection alone while 21 others had postoperative adjuvant radiotherapy and/or chemotherapy. Factors influencing survival are presented and patterns of metastasis discussed. Overall, 45% of the patients showed metastatic disease either at diagnosis (15%) or during the follow-up period (30%). The lung was involved in two-thirds of the cases and was the most common site of spread. In patients with rhabdomyosarcoma and malignant fibrohistiocytoma, regional lymph node metastasis was noted in 36% and 17%, respectively.     

Management of recurrent retroperitoneal sarcomas

Recurrent retroperitoneal sarcomas are rare, with patterns of recurrence determined by the histologic subtype. A range of patient characteristics and treatment profiles combined with a myriad of presentations and clinical courses of recurrences make this diverse entity challenging to manage. Although surgical resection improves survival in select patients, the oncological outcomes are inferior to that of primary retroperitoneal sarcomas. Management options for unresectable disease include local ablative therapy, radiation and systemic therapy, with palliative surgery indicated occasionally. Attempts at disease control must be balanced with potential morbidity and impact on the patient's quality of life. This review aims to offer insights into the current understanding of recurrent retroperitoneal sarcomas and provide some guidance on management.     

Acute myeloid leukaemia presenting with mediastinal myeloid sarcoma: report of three cases and review of literature

Although myeloid sarcomas (MS) are frequently associated with acute myeloid leukaemia (AML), the occurrence of mediastinal MS is a much rarer event. The authors describe a distinct group of three AML patients with mediastinal MS and complex cytogenetics presenting at their centre over a 7-year period. Clinical features consistent with superior vena caval obstruction were noted at presentation in two of the three patients. Mediastinal mass was detected on routine chest radiography, and biopsies confirmed the diagnosis of MS. One patient relapsed after consolidation chemotherapy and died from progressive disease. Two patients underwent allogeneic haemopoietic stem cell transplant, but succumbed to transplant related complications. Review of mediastinal MS over the last 20 years shows that a significant proportion of patients have complex cytogenetic abnormalities and a poor long-term prognosis. Early and accurate diagnosis is essential and patients should be managed along the lines of high risk AML.     

Surgical treatment for small round cell sarcoma of the bone

Background. The role of surgical intervention for the treatment of small round cell sarcoma of bone, has become important. However, there have been few reports concerning the surgical safety margin for this disease. An appropriate surgical margin should be discussed in relation to the effects of preoperative treatment, local recurrence rate, and prognosis. Methods. Twenty-eight patients with small round cell sarcoma of the bone were studied. Nineteen patients were stage M0 (of whom 18 were operated) and 9 were stage M1 (of whom 4 were operated). Preoperative treatment was evaluated by histological examination of the surgical specimen. The surgical margin was evaluated as curative, wide, marginal, or intralesional, by methods accepted by the Japanese Orthopedic Association. Results. (1) Of the 18 operated patients in stage M0, 2 showed local recurrence because of an inappropriate surgical margin or poor response to preoperative treatment. (2) Patients who had a wide margin procedure and who responded to preoperative treatment did not show local recurrence. (3) Patients who had a curative procedure did not show local recurrence regardless of the effect of preoperative treatment. (4) Patients with surgical speciment histologically evaluated as showing over 90% necrosis showed no local recurrences and had favorable prognoses with wide or curative procedures. Conclusion. The effects of preoperative treatment and surgical margins were important prognostic factors for local control in patients with small round cell sarcoma of the bone. Received: July 2, 1998 / Accepted: August 17, 1999     

Ewing’s Sarcoma: Experience with 12 Cases

Twelve patients with localized Ewing's sarcoma were treated between 1980-1990 at the Istanbul School of Medicine, Department of Pediatric Oncology-Hematology, Oncology Research and Treatment Center and Our Children Leukemia Foundation. There were 8 boys and 4 girls, with a mean age of 8.1 (range 3-17) years. The tumors were in the femur in 3 patients, in the humerus and rib in 2 patients each and in the tibia, radius, vertebra, clavicula and pelvis in 1 patient each. Chemotherapy alone was applied in 2 patients, 1 patient had chemotherapy and radiotherapy. The remaining 9 cases were treated with Chemotherapy and radiotherapy (during the chemotherapy). The chemotherapy protocols were: VAC (n = 5), VACA (n = 3), IVAD (n = 3) and T.9 (n=1). One patient died from the disease itself. Remissions were achieved in the other 11 patients. After 5 to 95 months (mean: 22 months) 7 patients had relapsed (4 had local and 3 had distant metastases). Three patients were not able to be followed, 3 died due to additional problems (infection, cardiotoxicity). The best prognosis was achieved when Ewing's sarcoma initiated in the long bones, with less than 100 ml tumor volumes and patients were under 5 years old. There were no significant differences among chemotherapy protocols.     

容易误诊的椎管内原始单核细胞肉瘤的临床病理分析

目的：原发性髓系肉瘤,很容易造成临床和病理的误诊,尤其是发生在罕见部位.本文就2例发生在椎管内的髓系肉瘤进行报道分析.方法：对2例椎管内硬膜外髓系肉瘤患者的临床情况、病理诊断及随访结果,结合文献,进行临床和病理分析.结果：2例患者均为年轻人,临床出现脊髓压迫症状,肿物切除后经病理及免疫组化证实,诊断为髓系原始单核细胞肉瘤.结论：椎管内这一罕见部位可以发生髓系肉瘤;病理图象上见到弥漫排列的单一性的小圆细胞肿瘤,要考虑髓系肉瘤的可能性;免疫组化在诊断中起重要作用.     

Retroperitoneal sarcomas: A review of disease spectrum,radiological features,characterisation and management

Retroperitoneal sarcomas are a rare disease. The overall 5‐year survival rate for these lesions remains low, and surgical management offers the only option for effective treatment and potential for cure. Radiotherapy is increasingly being employed in addition to standard surgical treatment. Improvements in cross‐sectional imaging have also facilitated better characterisation of lesions, preoperative planning and long‐term follow‐up. This article reviews the current literature and documents the various types of retroperitoneal sarcomas with a particular approach to their imaging features. We also highlight the pathology, diagnostic methods and most current management of these tumours.     

Retroperitoneal malignant fibrous histiocytoma presenting with inferior vena caval obstruction

A 54-year-old-man presented with inferior vena caval obstruction. At autopsy, a retroperitoneal malignant fibrous histiocytoma was noted to have invaded the inferior vena cava. Such a clinical presentation is quite unusual for a retroperitoneal sarcoma.