Annual Meeting Proceedings Part 1 - American Society of Clinical ...

10020 Poster Discussion Session (Board #12), Sat, 1:15 PM-5:15 PM and
4:45 PM-5:45 PM
Phase II study of sorafenib mesylate (So) in patients (pts) with evolutive
and advanced epithelioid hemangioendothelioma (EHE) or hemangiopericytoma/solitary
fibrous tumor (SFT). Presenting Author: Christine Chevreau,
Institut Claudius Regaud, Toulouse, France
Background: There is no standard of care for both rare sarcomas. Regarding,
the important vascularization of EHE and SFT, we explored the activity/
toxicity of So in pts with these sarcomas. Methods: We conducted a
multicenter one-step phase II trial of So (800 mg/d). The primary endpoint
was the 9-months progression-free rate (9-PFR). According to EORTC
criteria, So is considered as promising drug if 6-PFR�14%. All pts have
had documented progressive disease at entry. Results: 20 pts (15 EHE & 5
SFT) were enrolled from June 2009 to February 2011 in the 8 participating
institutions. 12 men and 8 women. Median age was 57. The most common
primaries were superficial trunk (8 cases) and liver (4 cases). PS were 0 in
10 cases, 1 in 7 cases and 2 in 3 cases. 16 pts had metastasic disease ,
especially in lung (15), liver (6) and bone (4). Eight pts received prior
chemotherapy (Doxorubicin : n� 8 cases and taxane : n �3). The median
So treatment duration was 124 days. 9 pts experienced grade-3 toxicities;
the most frequent grade-3 toxic events were hand foot syndrome (5 pts),
myalgia (1), stomatitis (1), anorexia (1), diarrhea (1) and arterial hypertension
(1).Because of this toxicity, treatment discontinuation was necessary
in 6 cases and dose reduction was necessary in 5 pts. The 9-PFR was 6/18
(33.3% [11.5-55.1]). The 2, 4 and 6 PFR were 15/18 (83.3%), 8/18
(44.4%) and 7/18 (38.8) respectively We observed 2 partial responses
lasting 2 and 9 months in 2 pts with EHE. Analysis of the predictive value of
circulating pre-angiogenetic biomarkers is ongoing. Conclusions: According
to the STBSG-EORTC criteria (3-PFR�40% & 6-PFR�14%), So is a
promising drug for EHE and SFT pts. Further trials is needed, especially a
discontinuation randomized trial.
10022 Poster Discussion Session (Board #14), Sat, 1:15 PM-5:15 PM and
4:45 PM-5:45 PM
Tenosynovial giant cell tumor (TGCT)/pigmented villonodular synovitis
(PVNS): Outcome of 313 patients before the era of kinase inhibitors.
Presenting Author: Emanuela Palmerini, Istituto Ortopedico Rizzoli, Bologna,
Italy
Background: Tenosynovial giant cell tumor (TGCT) is a rare, usually benign
neoplasm of synovium and tendon sheath. TGCT is classified as localized or
diffuse according to the extent of synovial involvement. Surgery is the
primary treatment, but the recurrence rate is high, with possible multiple
recurrences, joint function deterioration and decline in quality of life.
Recent data suggest a role for TKIs in advanced disease. In order to identify
prognostic factors for recurrence, a retrospective pooled analysis was
carried out in three institutions (Istituto Ortopedico Rizzoli, Bologna, Italy;
Istituto Nazionale Tumori, Milano, Italy; Memorial Sloan Kettering Cancer
Center, New York, USA). Methods: Clinical charts and pathology reports of
patients (pts) treated in the period 1998-2008 were examined. Results:
The study included 313 pts, 177 F and 136 M; median age: 36 years
(range: 11-89 years). Most (64%) pts had tumors in the knee (15% ankle,
11% hip, 10% other). Tumor size was: �2 cm in 24% of pts, 2-5 cm in
44%, �5 cm in 32%. A diffuse pattern was reported in 69% of pts. The
resection status was available in 289 pts: 51% had R0 surgery, 28% R1
and 21% R2. No metastases were documented. Local recurrence was
reported in 76 pts (median time to recurrence: 15.7 months). With a
median follow-up of 4.2 years, 5-year local recurrence-free survival (LRFS)
was 66% (95% CI: 59 - 73). Size (� 2 cm 80% vs. 2-5 cm 67% vs. �5cm
62%, p�0.04), gender (F 73% vs. M 56%, p�0.02), type (localized 78%
vs. diffuse 61%, p�0.02), and resection status (R0 76% vs. R1 55%, vs.
R2 57%, p�0.002) influenced 5-year LRFS, whereas age, tumor location
and bone involvement did not. The 5-year 2nd LRFS was 43% (95% CI: 28 -
59). Multiple (2 to 5) local recurrences were observed in 39% of relapsed
patients. Conclusions: The study confirms TGCT propensity to multiple local
recurrences. Diffuse type, suboptimal surgery, male gender and larger
tumors increase the recurrence risk. In order to improve the probability of
local control, studies addressing the role of TKIs could be considered in
subsets of patients.
Sarcoma
635s
10021 Poster Discussion Session (Board #13), Sat, 1:15 PM-5:15 PM and
4:45 PM-5:45 PM
Optimizing the therapy of desmoplastic small round cell tumor: Combined
experience from the two major cancer centers. Presenting Author: Vivek
Subbiah, Division of Cancer Medicine, University of Texas M. D. Anderson
Cancer Center, Houston, TX
Background: Desmoplastic small round cell tumor (DSRCT) is a rare
translocation-positive (EWS-WT1)sarcoma subtype that often presents as
diffuse peritoneal sarcomatosis in male adolescents and young adults. The
objective of this study was to assess the survival of patients with DSRCT
from the two major cancer centers. Methods: A multi-center retrospective
review was performed of patients diagnosed with DSRCT treated at The
University of Texas MD Anderson Cancer Center (MDACC) and Memorial
Sloan-Kettering Cancer Center (MSKCC). Results: Of the 197 pts evaluated
at both cancer centers (109 from MSKCC and 88 from MDACC) 87 % were
male. The mean age was 25 � 11 years; 139 (70.6%) underwent surgery;
38 ( 19.6%) had debulking surgery, 30 received radiation therapy, and 27
underwent hyperthermic chemotherapy after debulking (CHPP), 11 had a
stem cell transplant. 112 patients were seen after 2003 in both the centers
vs 85 before 2003. Neoadjuvant chemotherapy included Ewing’s-like
chemotherapy with MSKCC’s P6 regimen (�18 yrs of age) and VAI in
adults. We further analyzed the MDACC cohort. In univariate analysis
radiotherapy, surgery, CHPP, removal of primary mass � metastases, age �
30 and patients treated after 2003 were associated with significantly
improved overall survival. Multi-variate analysis showed that patients
treated after 2003 had 59% lesser hazard (HR�0.41) than those treated
earlier (p�0.04). Comparing 1990-2003 vs. 2004-2010 survival �3 yr
with DSRCT was �25 % and is now �50 %, a significant improvement.
Conclusions: We present long-term outcomes for the largest series of
patients diagnosed with DSRCT. A multidisciplinary approach to DSRCT
treatment is required to prolong 3- and 5-yr overall survival. Our recent
improved outcomes required not only aggressive chemotherapy but also
regional local control measures. Best adjuvant therapy remains to be
determined. A treatment algorithm will be proposed.
10023 Poster Discussion Session (Board #15), Sat, 1:15 PM-5:15 PM and
4:45 PM-5:45 PM
Advanced chondrosarcomas: Role of chemotherapy and survival. Presenting
Author: Binh Bui Nguyen, Institut Bergonie, Bordeaux, France
Background: There are no data about the role of chemotherapy in patients
with advanced chondrosarcomas. Methods: From 2000 to 2011, 98
patients with a confirmed diagnosis of advanced chondrosarcomas were
referred to one of the 8 participating institutions, and their medical records
reviewed. Results: Median age was 46 years (range 16-82). The most
frequent histological subtype was conventional chondrosarcoma (n�60,
61%). 83 patients (85%) had metastatic disease ( lung n�71, bone n�23,
liver n�6) and 15 patients (15%) had locoregional unresectable disease.
30 patients (31%) had � 2 metastatic sites. 63 patients (64%) received
1st-line combination agent chemotherapy. 70 patients (71%) received a
1st-line anthracycline-containing regimen (doxorubicin � cisplatin �/ifosfamide:
n�30, doxorubicin or pegylated liposomal doxorubicin: n�18,
doxorubicin � ifosfamide �/- dacarbazine: n�16, others� 6). RECIST
objective response was observed in 14 patients (14%), all but two treated
with anthracyclines. 30 patients (31%) had stable disease � 6 months.
Median progression-free survival (PFS) and overall survival (OS) were 5.3
months (95% CI: 2.8-7.7) and 19 months (95% CI: 14-24) respectively.
Performance status (PS) � 2 was the sole factor significantly associated
with OS. Conclusions: Chemotherapy is associated with a 6-month nonprogression
rate of about 45% in patients with advanced chondrosarcoma.
Best supportive care should be considered in patients with poor PS. These
data should be used as a reference for response and outcome in the
assessment of investigational drugs in advanced chondrosarcoma.
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