Successful treatment of primitive neuroectodermal tumor-associated microangiopathy with multiple bone metastases

We report here a 16-year-old male with primitive neuroectodermal tumor (PNET)-associated probable microangiopathy with multiple bone metastases. Laboratory findings excluded the possibility of amegakaryocytic or immune thrombocytopenia and/or disseminated intravascular coagulation. He was first treated with plasma-exchange (PE), followed by platelet transfusions, steroid pulse therapy and combined chemotherapy. PE and steroid pulse therapy reduced his plasma CRP level. Combined chemotherapy drastically increased his platelet count until it had almost normalized without further transfusion. The plasma level of von Willebrand factor-cleaving protease (ADAMTS13) activity measured before PE was not severely deficient (48% of normal) and an unusually large von Willebrand factor multimer (UL-VWFM) was detected. We consider that this therapeutic strategy has the following benefits: (1) reduction of plasma levels of factors that are harmful to both platelet activation and endothelial cell injury; and (2) the safe transfusion of platelet concentrate in thrombotic microangiopathy. This strategy should be confirmed in further cases.     

Surgical outcome of stage III and IV adrenocortical carcinoma

BACKGROUND: Adrenocortical carcinoma (ACC) is a rare tumor usually diagnosed at an advanced stage on invasion of or adherence to adjacent organs. We report surgical outcome of stage III and IV ACCs. METHODS: ACCs from seven patients at clinical stage II (n = 1), III (n = 4), or IV (n = 2) were resected. Combined resection of the liver and inferior vena cava was performed in six patients. Morbidity, mortality, recurrence and survival were analyzed. RESULTS: The pathological stage was stage III in five patients and stage IV in two patients. The mortality was zero and the morbidity was two of seven (29%) patients. The estimated 3-year disease-free and overall survivals for stage III were 20% and 40%, respectively, with a median follow-up of 32 months (range, 11-58). The mean disease-free survival was 21.0 +/- 9.0 months (95% CI: 3.3-38.7). The 3-year disease-free and overall survivals for stage III and IV were 14.3% and 28.6%, respectively. The mean disease-free survival time was 18.6 +/- 6.7 months (95% CI: 5.4-31.8). The most frequent site of metastasis was the lungs, seen in four patients, and liver in three patients. Loco-regional, intra-abdominal lymph node, peritoneum, bone, brain recurrences were also seen in one patient each. The mean survival after recurrence was 19.0 +/- 3.3 months (95% CI: 12.6-25.5), and the 50% survival was 18.4 months with mitotan and cytotoxic drug therapy. CONCLUSIONS: Resection for stage III, IV ACCs affords the possibility of negative margins, acceptable peri-operative morbidity and mortality, and prolongs survival in selected patients.     

Ovarian cancer treatment from the standpoint of the gynecologic oncologist

Ovarian cancer treatment on the standpoint of the gynecologic oncologist: Ovarian cancer is comprehensively treated with combined surgery and anticancer chemotherapy, which influence each other. In the initial surgery, a cancer stage is decided, and in advanced cancer, primary debulking surgery is conducted. The outcome of postoperative chemotherapy has a correlation with the diameter of the postoperative residual tumor. Recently, a new treatment strategy of interval debulking surgery during chemotherapy has been widely conducted to completely remove tumors. Improved diagnostic imaging such as PET enhances aggressive surgery for recurrent cancer.     

Three-weekly docetaxel with prednisone is feasible for Japanese patients with hormone-refractory prostate cancer: a retrospective comparative study with weekly docetaxel alone

BACKGROUND: We previously reported that weekly treatment with docetaxel alone is useful for and well tolerated by patients with hormone-refractory prostate cancer (HRPC). Here, we compare it with the regimen of docetaxel once every 3 weeks (q3w) plus daily prednisone (PSL) based on a TAX 327 trial in order to clarify the efficacy and toxicity of docetaxel regimens in Japan. METHODS: Thirty-two patients with HRPC were treated with docetaxel weekly (regimen 1) or docetaxel q3w plus PSL daily (regimen 2) at Tsukuba University Hospital and the changes in serum prostate-specific antigen (PSA), tumor size and survival were evaluated. The dose of docetaxel in regimen 1 was based on our previous report and that of regimen 2 was modified from a TAX 327 trial. RESULTS: A >50% decrease in PSA was observed in 53% of the patients with a median time to progression of 3.5 months and 69% with 8.5 months with regimens 1 and 2, respectively. Patients who received regimen 2 had a significantly better survival rate than those who received regimen 1. Myelosuppression and neuropathy were statistically more frequent in regimen 2 than in regimen 1. CONCLUSION: A regimen of docetaxel q3w with PSL daily was associated with a high rate of PSA reduction and prolongation of patient survival. Although docetaxel has not been approved in Japan yet, this treatment is considered feasible for Japanese patients with HRPC.     

The efficacy of transcatheter embolization of severe arterioportal shunts in hepatocellular carcinoma

Transcatheter arterial embolizations of severe arterioportal shunt (A-P shunt) were performed with steel coils in 3 patients with hepatocellurlar carcinoma (HCC) as shown below. Case 1: A 56-year-old man with HCC associated with portal hypertension (esophageal varices and ascites abnominal pain), portal vein tumor thrombus and severe A-P shunt was performed in critical conditions. Case 2: A 51-year-old man with HCC, lung and adrenal gland metastases was accompanied with severe portal hypertention caused by A-P shunt and was in a harmful condition similar to case 1. Case 3: A 68-year-old woman with HCC associated with autoimmune hepatitis was performed a hepatic resection. Then multiple intrahepatic recurrences appeared 6 months later. A-P shunt made impossible to detect the feeding artery of tumor. After embolization of A-P shunt, esophageal varices and ascites resolved, and abdominal pain improved in cases 1 and 2. In addition, embolization enabled to perform transcatheter arterial chemoembolization in case 3. This procedure is a useful tool to improve various symptoms due to A-P shunt and to continue treatments for HCCs.     

Short-term gefitinib treatment brought about a long-term regression of bronchioloalveolar carcinoma without EGFR gene alterations: a case report

The tyrosine kinase inhibitor (TKI) of the epidermal growth factor receptor (EGFR) gefitinib has beneficial effect in some patients with refractory advanced non-small cell lung cancer (NSCLC). However, the majority of responders eventually develop acquired resistance during the course of prolonged continuous treatment. Here we present a case of 76-year-old Japanese female, who had never smoked, with poor performance status from bronchioloalveolar carcinoma (BAC), in whom a brief initial 5-week administration of gefitinib resulted in dramatic antitumor effects that lasted approximately 8.5 months after cessation of the treatment. Furthermore, the relapsed tumor later regressed again by re-treatment with the TKI. She survived 26 months since she first took gefitinib. Unexpectedly, neither sensitizing mutations for EGFR-TKIs nor increased copy numbers were detected in EGFR gene of her BAC cells. This case suggests that, in some patients with NSCLC, even short-term administration of gefitinib may bring about clinical benefits and disease response comparable to the standard long-term daily dosing schedule. Short-term use of gefitinib will also be able to minimize the expensive medical cost of the TKI. The potential role of short-term or pulse-dose therapy with EGFR-TKIs should be clarified in further prospective studies. Moreover, it is urgent to develop better strategies by which we could distinguish responders to the TKIs from nonresponders among patients who do not have any EGFR gene alterations.     

Repeat reduction surgery after an initial hepatectomy for patients with colorectal cancer

A hepatectomy is the only treatment offering long-term survival in patients with colorectal liver metastases. However, 70-80% of the patients with a complete resection develop recurrent disease after an initial hepatectomy. Sixty-one patients who underwent metastases from colorectal carcinoma with a curative hepatectomy were entered into this study. Recurrence after hepatectomy was observed in 41 patients (67.2%). We reviewed the outcome of these 41 patients. Repeat reduction surgery was performed on 16 out of 41 patients (39.0%). According to a multivariate analysis, repeat reduction surgery and tumor size were found to be independent prognostic factors for the survival rate (p=0.007, p=0.018). Furthermore, in the group that underwent repeat reduction surgery, the rate of positive lymph nodes was significantly lower in the primary lesions, and the disease-free interval (DFI) was also significantly longer than in the group that did not undergo repeat reduction surgery (p=0.023, p=0.045), respectively. Repeat reduction surgery was found to be the most important prognostic factor. Patients with a longer DFI and with negative lymph node findings at the primary site may therefore be considered to be good candidates for repeat reduction surgery.     

Geriatric nutritional risk index as an easy-to-use assessment tool for nutritional status in hepatocellular carcinoma treated with atezolizumab plus bevacizumab

Aim

The present study focused on Geriatric Nutritional Risk Index (GNRI), which is based on bodyweight and serum albumin, and known as an easy-to-use nutritional assessment tool in clinical settings, to elucidate the prognostic predictive ability of GNRI in patients treated with atezolizumab plus bevacizumab (Atez/Bev) for hepatocellular carcinoma (HCC).

Methods

A total of 525 HCC patients treated with Atez/Bev, based on their classification of unsuitable status for curative treatments and/or transarterial catheter chemoembolization, were enrolled (Child–Pugh A:B:C = 484:40:1, Barcelona Clinic Liver Cancer stage 0:A:B:C:D = 7:25:192:283:18). Prognosis was evaluated retrospectively using GNRI.

Results

Atez/Bev was used in 338 of the present cohort as first-line systemic chemotherapy (64.4%). Median progression-free survival based on GNRI indicating normal, mild decline, moderate decline, and severe decline was 8.3, 6.7, 5.3, and 2.4 months, respectively, whereas median overall survival was 21.4, 17.0, 11.5. and 7.3 months, respectively (both p < 0.001). The concordance index (c-index) values of GNRI for predicting prognosis (progression-free survival/overall survival) were superior to those of Child–Pugh class and albumin-bilirubin grade (0.574/0.632 vs. 0.527/0.570 vs. 0.565/0.629). As a subanalysis, muscle volume loss was observed in 37.5% of 256 patients with computed tomography data available. Along with GNRI decline, frequency of muscle volume loss became progressively larger (normal vs. mild vs. moderate vs. severe = 17.6% vs. 29.2% vs. 41.2% vs. 57.9%, p < 0.001), and a GNRI value of 97.8 was predictive of its occurrence (AUC 0.715, 95% CI 0.649–0.781; specificity/sensitivity = 0.644/0.688).

Conclusion

These findings indicate that GNRI is an effective nutritional prognostic tool for predicting prognosis and muscle volume loss complication in HCC patients treated with Atez/Bev.