VIP (etoposide, ifosfamide, cisplatin) in adult patients with recurrent or refractory Ewing sarcoma family of tumors.

Despite the fact that Ewing sarcoma family of tumors (ET) is chemosensitive, long-term survival is extremely rare for patients with primary refractory or recurrent disease. There is no standard salvage chemotherapy regimen available in this context. In this study the authors reviewed their experience with the combination of etoposide, ifosfamide, and cisplatin in adult patients with recurrent or refractory disease. From February 1997 through December 2001, they evaluated the efficacy of etoposide (75 mg/m2/day for 5 days), ifosfamide (1,200 mg/m2/day for 5 days), and cisplatin (20 mg/m2/day for 5 days) combination chemotherapy (VIP regimen), as second-line salvage therapy in 27 patients with recurrent or refractory ET. All patients were evaluated for response, time to progression, and overall survival. Twenty-one male and 6 female patients with recurrent (n = 14) and refractory (n = 13) disease were treated with the VIP regimen. Median age was 18 years (range, 16-34 years). Twenty-two patients were previously treated with vincristine, Adriamycin, ifosfamide, and actinomycin-D; and 5 patients were treated with cyclophosphamide, Adriamycin, and vincristine. Sites of recurrent or progressive disease included local (n = 3), distant (n = 11), and both local and distant (n = 13). A total of 129 cycles of VIP were given (median, 5 cycles/patient; range, 1-14 cycles/patient). One patient (4%) had a complete response (CR) and 8 patients (30%) had a partial response (PR), for an overall response rate of 34%. The median number of cycles given to patients with CR + PR was 6 (range, 3-14 cycles). Nine patients (33%) had stable disease and 9 (33%) had disease progression. Median time to progression and median overall survival were 6.6 months and 8.1 months respectively for all patients, and 12.8 months and 14.2 months respectively for responders. There were no toxic deaths. Major toxicities included grade IV granulocytopenia in 19 patients and grades III/IV thrombocytopenia in 15 patients. At a median follow-up of 8 months (range, 2-56 months), 24 patients died of disease progression, 2 patients are alive with disease, and 1 patient is alive with no evidence of disease. The authors conclude that the VIP combination is active in patients with recurrent/refractory ET, with acceptable toxicity, and offers good palliation. Cisplatin-based combination chemotherapy merits further investigation, possibly as first-line treatment in this disease.

18-Fluorodeoxyglucose-positron emission tomography in inflammatory breast cancer.

Inflammatory breast cancer (IBC) is the most aggressive form of locally advanced breast cancer. It can be diagnosed based on a clinical or pathologic basis. We evaluated the usefulness of (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) scans for diagnosing and staging IBC. We retrospectively reviewed the medical records of seven consecutive patients with IBC who underwent FDG-PET scanning for the initial staging. Four patients had follow-up PET scans after chemotherapy. All seven patients presented with diffuse breast enlargement, redness, and peau d'orange for 1 to 5 months' duration. In addition, four patients had a palpable breast mass, and three had axillary lymph node enlargement. Mammography showed diffuse, increased parenchymal density and skin thickening in 85% and parenchymal distortion in 43%. There was no evidence of distant metastasis on computed tomography of the chest or abdomen. Pathologic examination of breast biopsy specimens showed infiltrating ductal carcinoma in six patients, and one had lobular carcinoma. All patients had prechemotherapy whole-body PET scans that showed diffuse FDG uptake in the breast with superimposed intense foci in the primary tumor. Furthermore, there was skin enhancement in 100%, axillary lymph node in 85%, and skeletal metastases in 14% of the patients, confirmed by bone scintigraphy. Postchemotherapy FDG-PET scans performed in four patients showed response in the primary tumor, axillary lymph nodes, and skeletal metastases. The FDG-PET scan is thus useful for displaying the pattern of FDG breast uptake that reflects the extent of the pathologic involvement in IBC (i.e., diffuse breast involvement and dermal lymphatic spread). It can also detect the presence of lymph node and skeletal metastases, demarcating the extent of the disease locally as well as distally.