Sarcoma

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.

This case shows the importance of searching for antineural antibodies in oncologic patients with new neurologic deficits, and of having a judicious workup for occult malignancies in patients with known antineural antibodies.

Our case illustrates the fact that MDS-associated GS can be treated palliatively with radiation and hypomethylating agents in an appropriate setting. With the growing geriatric patient population, effective treatment options are needed in this disease.

Granulocytic sarcomas have been reported in nearly every part of the body, including the gastrointestinal (GI) and genitourinary (GU) tracts, central nervous system (CNS), and respiratory, lymphatic, and skeletal systems. A few case series have been reported through the years.

Several phase III clinical studies have shown significantly better results in treating certain cancers when hyperthermia therapy was added to radiation therapy, as compared to radiation treatments alone.

BSD Medical Corp announced that the results of a 340-patient randomized phase III clinical trial testing the benefit of adding hyperthermia therapy to chemotherapy were presented at the recent annual American Society of Clinical Oncology (ASCO) conference in Chicago.

The Oncologic Drugs Advisory Committee (ODAC) voted 12-to-2 not to recommend that FDA approve Junovan (mifamurtide, IDM Pharma) for treating newly diagnosed, resectable high-grade osteosarcomas in combination with chemotherapy following surgical resection.

Over the past 30 years, there has been a migration away from amputation and radical ablative surgical procedures and toward more conservative, function-preserving surgery combined with radiation to treat extremity and body wall soft-tissue sarcomas. Efforts are now being focused on optimizing and streamlining treatment, including identifying subpopulations of patients who may be adequately treated by surgery alone. The goal of these efforts is to minimize the risks for short- and long-term treatment-related morbidity while maintaining excellent rates of local tumor control. This report will briefly review the progress made in these areas.

Researchers have developed a two-gene test that can accurately distinguish between two common forms of gastrointestinal cancers.

In patients with adult soft-tissue sarcoma (ASTS), the use and timing of adjuvant chemotherapy or chemoradiotherapy remains controversial. The appropriate target population is generally accepted as International Union Against Cancer (UICC)/American Joint Committee on Cancer (AJCC) stage III extremity or trunk sarcomas (ie, > 5 cm, grade 3/4, located deep to the superficial fascia, with no evidence of metastases). After definitive local treatment, the 5-year disease-free and overall survival rates in this population are approximately 52% and 56%.

Preclinical advances offer an opportunity to further reduce morbidity and mortality from sarcomas over the next decade. Since no single institution or North American cooperative oncology group has the expertise or patient resources for histology-specific clinical and translational research on adult sarcomas, efforts have been made to develop funding from the National Cancer Institute (NCI). One such initiative was the Intergroup Coalition Against Sarcomas (ICAS), which, building upon the strengths of the multimodality cooperative oncology groups, provided an infrastructure for broad participation by investigators from all treatment disciplines in protocol development and patient entry. However, despite an excellent evaluation in formal peer review, the Division of Cancer Treatment of NCI has ended this initiative claiming insufficient available funds—to the detriment of adult sarcoma patients now and in the future.

Sarcomas are a group of tumors with highly variable character istics and clinical outcomes. Their locations in almost all body locations present unique challenges for diagnosis and management. These challenges have presented opportunities for evaluation and validation of new imaging techniques. Positron-emission tomography (PET) has been evaluated for use in cancer over the years, and in particular, it has been evaluated in sarcoma diagnosis and treatment evaluation.

We now have multiple novel agents that warrant testing in this disease setting. Clinical trials need not only test interesting drugs, but should also correlate information from tumor specimens and clinical responses.

An innovative cancer agent called PHA-739358, which inhibits one of the aurora proteins, has shown indications of potential benefit in 7 of 36 patients (19.4%) with advanced or metastatic solid tumors who participated in a phase I dosing and toxicity study, Dutch researchers reported at the EORTC-NCI-AACR Symposium on Molecular Targets and Cancer Therapeutics

Patients with metastatic gastrointestinal stromal tumors (GIST) who are in complete remission after surgical resection remain at high risk for relapse and should continue long-term treatment with imatinib (Gleevec), according to Binh Nguyen Bui, MD, of Institut Bergonie, Bordeaux, France. Dr. Bui reported results of the French Sarcoma Group BFR14 randomized phase III trial at the American Society of Clinical Oncology 42nd Annual Meeting (abstract 9501).

Drs. Ephraim S. Casper, Memorial Sloan-Kettering Cancer Center, and George D. Demetri, Dana-Farber Cancer Institute, discussed the 2006 National Comprehensive Cancer Network (NCCN) guidelines on soft-tissue sarcomas at the NCCN 11th Annual Conference. The updated guidelines clarify the follow-up schedule for patients with extremity sarcoma.

Primary nonepithelial malignancies of the breast comprise an importantminority of breast neoplasms, including primary breast sarcomas,therapy-related breast sarcomas, the phyllodes tumors, and primarybreast lymphomas. With widespread mammographic detection ofbreast lesions, these tumors represent critical elements of the differentialdiagnosis of even benign-appearing lesions. Each has a distinctclinical profile, including presentation, available therapeutic options,and prognosis, further underscoring the importance of timely recognition.The increasing incidence of breast carcinomas and the subsequenttherapy thereof may be contributing to an increase in the numberof therapy-related breast tumors. This review discusses various featuresof these uncommon malignancies and their treatment, with thegoal of increasing understanding of their clinical behavior andmanagement.

Medicare will grant limited coverage for the use of positronemissiontomography (PET) for certain of its beneficiariessuffering from thyroid cancer, the Centers for Medicare andMedicaid Services (CMS) recently announced. CMS also said that ithad refused a request to provide PET coverage for soft-tissue sarcomabecause imaging techniques currently covered by Medicare providegood diagnostic results.