Pharmacological therapies for Liposarcoma

Introduction: Liposarcoma (LS) is one of the most common adult soft tissue sarcomas (STS). For metastatic disease, systemic treatment options were historically represented by standard cytotoxic chemotherapy. More recently, innovative therapies have been introduced and they are currently part of the therapeutic armamentarium, positively impacting disease control and patients’ quality of life. Moreover, in the last decade, a better understanding of the molecular characteristics of each STS subtype allowed to detect new potential targets and develop novel, biology-driven compounds at different stages of testing.

Areas covered: This review is focused on LS, retracing their pharmacological management, starting with a summary of results achieved with standard chemotherapy, then moving to a deeper analysis on data obtained with new, approved therapies and finally reporting an update on ongoing clinical trials, thus providing an overview on the current scenario and outlining how it might evolve in the coming years.

Expert commentary: Important strides have been made in the knowledge and treatment of LS. Peculiar molecular features and fundamental signalling pathways represent nowadays druggable targets for novel therapies. However, predictive biomarkers still need to be identified in order to better select the target population, to possibly test combinations of drugs, with the ultimate goal of improving outcomes.     

Lipoblastoma presenting as a rapidly growing paravertebral mass and masquerading as myxoid liposarcoma on fine needle aspiration cytology

Lipoblastoma is a peculiar variant of lipoma occurring almost exclusively during infancy and early childhood. It is found most commonly in the upper and lower extremities; less common sites are head and neck, trunk, mediastinum, and retroperitoneum. It has a greater predilection for boys and commonly presents as a slowly growing soft‐tissue mass. We present here the case of a five‐ year old female child with a lipoblastoma presenting as a paravertebral mass in the right lower back which progressed rapidly in the previous six months causing diagnostic difficulty on fine needle aspiration cytology. Diagn. Cytopathol. 2016;44:426–429. © 2016 Wiley Periodicals, Inc.     

Magnetic resonance imaging of lipoma and liposarcoma: Potential of short tau inversion recovery as a technique of fat suppression

The present limited retrospective study was performed to assess MR imaging of lipomatous tumours of the musculoskeletal system and to evaluate the potential of the T2 short tau inversion‐recovery (STIR) technique for differentiating lipomas from liposarcomas. Magnetic resonance imaging of 12 patients with lipomatous tumours of the musculoskeletal system (eight benign lipomas, three well‐differentiated liposarcomas and one myxoid liposarcoma) were reviewed. Benign lipomas were usually superficial and showed homogeneity on T1‐ and T2‐weighted spin echo sequences. Full suppression at T2 STIR was readily demonstrated. In contrast, the liposarcomas in the present series were all deep‐seated. Two well‐differentiated liposaromas showed homogeneity at long and short relaxation time (TR) but failed to show complete suppression at T2 STIR. One case of well‐differentiated liposarcoma (dedifferentiated liposarcoma) and one of myxoid liposarcoma showed mild and moderate heterogeneity at T1 and T2, respectively, and posed no difficulty in being diagnosed correctly. In conclusion, short and long TR in combination with T2 STIR show promise in differentiating benign from malignant lipomatous tumours of the musculoskeletal system, when taken in combination with the position of the tumour.     

Personalizing surgical margins in retroperitoneal sarcomas: an update

Introduction: Tumor biology, as well as completeness of surgical resection, are two important prognostic factors when treating retroperitoneal sarcoma (RPS). A frontline extended surgical approach is associated with improved local control and possibly improved survival. However, this approach has to be tailored to each histological subtype, as the patterns of growth and recurrence risks vary significantly among them.

Areas covered: We provide a review of the literature in RPS, describing the behavior of each of the five main histologic subtypes: well-differentiated liposarcoma (WDLPS), dedifferentiated liposarcoma (DDLPS), leiomyosarcoma (LMS), solitary fibrous tumor (SFT) and malignant peripheral nerve sheath tumor (MPNST). The prognostic factors relevant to oncologic outcomes of RPS, the role of margins and the importance of local control are discussed. Finally, a histologic specific surgical approach to RPS is provided in detail.

Expert opinion: While tumor-related factors are paramount, the only intervenable predictive factor is extent and quality of surgery. The extended surgical approach has been advocated for previously and again we describe it in more detail, tailored specifically to the tumor subtype. The aim of this approach is to maximize the possibility of achieving a complete resection through a standardized approach based on histologic behavior and site of origin.     

Combining surgery with 125I brachytherapy for recurrent mediastinal dedifferentiated liposarcoma: A case report and review of literature

BACKGROUND Dedifferentiated liposarcoma in the mediastinum is an extremely rare malignant neoplasm.A few previous case reports indicate that surgical resection is the major treatment,but frequent recurrence occurs locally.Due to its rarity,its clinical characteristics,optimal treatment and clinical outcomes remain unclear.Here,we report a case of multifocal recurrent dedifferentiated liposarcoma in the posterior mediastinum treated by combining surgery with 125I brachytherapy,and summarize its clinical features,treatment and prognosis.CASE SUMMARY A 75-year-old man was admitted to our hospital with a history of gradual dysphagia for one year and aggravated dysphagia for 3 mo.Contrast-enhanced computed tomography(CT)revealed several large cystic-solid masses with lipomatous density,and calcification in the posterior-inferior mediastinum.The patient received a wide excision by video-assisted thoracoscopic surgery.Pathological analysis confirmed the tumors were dedifferentiated liposarcomas.The tumor locally relapsed 24 mo later,and another operation was performed by video-assisted thoracoscopic surgery.Fifteen months after the second surgery,the tumor recurred again,and the patient received CT-guided radioactive seeds 125I implantation.After 8 mo,follow-up chest CT showed an enlarged tumor.Finally,his condition exacerbated with severe dysphagia and dyspnea,and he died of respiratory failure in July 2018.CONCLUSION We reviewed the literature,and suggest that surgical resection provides beneficial effects for dedifferentiated liposarcoma in the mediastinum,even in cases with local recurrence.125I brachytherapy may be beneficial for recurrent unresectable patients.     

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.