Sarcoma

Patients with retroperitoneal sarcomas who are unable to obtain complete resection after initial resection are left with few treatment options for palliative therapy, according to the results of a recent study.

Patients with heavily pretreated metastatic gastrointestinal stromal tumors were able to tolerate combined treatment with the pan-deacetylase inhibitor panobinostat and imatinib, according to the results of a small phase I study.

A phase II trial showed that patients with advanced gastrointestinal stromal tumors had a marginal response to pazopanib when assigned to the treatment after two or more failed therapies.

Results of a small study indicate that patients with gastrointestinal stromal tumors had increased activity of Src, but unlike in other tumors, it may be associated with longer overall survival.

The Akt/mammalian target of rapamycin (mTOR) pathway is often activated in patients with synovial sarcoma, and activation of the pathway is associated with worse outcomes, according to a new study.

The combination of palifosfamide and doxorubicin was no better than doxorubicin and placebo in the phase III PICASSO 3 trial of patients with metastatic soft-tissue sarcoma.

Carbon ion radiotherapy is a safe and effective method for treating unresectable spinal sarcomas, according to a retrospective analysis from one center in Japan.

The multikinase angiogenesis inhibitor pazopanib was well tolerated and exhibited some responses in pediatric patients with soft-tissue sarcomas and other refractory solid tumors in a phase I trial.

Almost half of the resections performed for deep and malignant extremity soft-tissue sarcomas are done by non-oncology surgeons including orthopedic surgeons and general surgeons, according to a new study.

Surgery is the mainstay of treatment for localized soft-tissue sarcoma (STS). It consists primarily of resection of the tumor along with a cuff of surrounding healthy tissue. In limb and trunk wall sarcomas, this basically implies resection of the surrounding soft tissues, which are mainly muscles, subcutaneous fat, and skin.[1] In the retroperitoneum, this necessarily should imply resection of adjacent viscera, even when they are not overtly involved.[2] This is the only way to avoid/minimize the presence of tumor cells at the cut surface (ie, positive microscopic surgical margins). Positive microscopic surgical margins are associated with a higher risk of local failure, distant metastases, and death.[3-6] Moreover, for STS located at critical sites, such as retroperitoneal sarcoma (RPS), positive surgical margins may have a direct impact on survival, favoring the development of inoperable local recurrences.[7] Indeed, unlike with STS arising in the extremities and trunk wall, local control in RPS poses a significant challenge and remains the leading cause of death, particularly in patients with low- to intermediate-grade tumors-roughly 75% of all cases.[8-13] Extending the resection to adjacent uninvolved viscera for primary RPS is the only way to minimize the presence of microscopic surgical margins and hence maximize the chance of cure. In essence, this strategy should often include ipsilateral nephrectomy and colectomy; locoregional peritonectomy and myomectomy (partial/total) of the muscle of the lateral/posterior abdominal wall (usually the psoas) (see Figure); splenectomy and left pancreatectomy, for tumors located on the left upper side; occasionally pancreaticoduodenectomy or hepatectomy, for tumors located on the right side; and vascular and bone resection only if vessels/bone are overtly infiltrated.[2]

Recently controversy has emerged regarding the extent of resection that constitutes optimal surgical management of retroperitoneal soft-tissue sarcoma.

The addition of adjuvant chemotherapy using doxorubicin, ifosfamide, and cisplatin, to pelvic radiation therapy for the treatment of uterine sarcomas increased 3-year disease-free survival in a group of women with localized disease, but also resulted in two toxic deaths among the study group.

Decreasing the time between cycles of standard chemotherapy for Ewing’s sarcoma from 3 weeks to 2 weeks increased event-free survival without an associated increase in toxicity, according to the results of a Children’s Oncology Group report.

Despite its place in standard practice, the performance of pulmonary metastasectomy in patients with sarcoma and lung metastases may not improve the survival rate of these patients, according to information from a systematic review of studies investigating the procedure in this patient population.

Treatment of gastrointestinal stromal tumor (GIST) with regorafenib after prior treatment failure with both imatinib and sunitinib resulted in a PFS survival benefit for patients across all prespecified subgroups.

Results from a phase Ib/II trial presented at the ESMO 2012 Congress found that a tumor-targeting doxorubicin conjugate, aldoxorubicin (INNO-206), showed activity in relapsed soft-tissue sarcoma patients.

Treating resected grade II-III soft-tissue sarcoma with an adjuvant chemotherapy regimen of doxorubicin, ifosfamide, and lenograstim did not result in any increase in relapse-free survival or overall survival.

Earlier today the FDA approved pazopanib (Votrient) to treat patients with advanced soft-tissue sarcoma who have previously received chemotherapy. More than 20 subtypes of sarcoma were included in the clinical trial that led to the approval.

A combination of cixutumumab, a type 1 insulin-like growth factor receptor inhibitor, and temsirolimus, a mammalian target of rapamycin (mTOR) inhibitor, showed evidence of activity in refractory Ewing’s sarcoma tumors as well as small-round-cell tumors in a phase I multicenter clinical study.

Isolated pulmonary metastases (PM) represent a unique manifestation of the myriad presentations of systemic spread from a primary neoplasm.

In this issue of ONCOLOGY, Kon and Martin review the difficult issue of lung metastasis from sarcoma and its management.

Surgical resection of isolated pulmonary metastases has been incorporated into the management of cancer for more than 70 years. However, many questions still remain concerning indications, technique, and efficacy for this approach.

In this article, we provide an extensive review of patient selection criteria and surgical approaches, as well as of controversies regarding resection for metastatic sarcoma.

This rare cancer finally sheds its FDA designation as an orphan disease.

Using FDG-PET/CT, a multidisciplinary team has been able to determine within a short period of time whether neoadjuvant therapy for soft-tissue sarcoma is working.