Systemic Therapy Options for Unresectable and Metastatic Chordomas

Stacchiotti, Silvia; Casali, Paolo G.

doi:10.1007/s11912-011-0176-x

Cited by 44 publications

(50 citation statements)

References 86 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Chemotherapy has been utilized in the treatment of primary chordomas; however, studies documenting a clear benefit are limited [21]. Anecdotal reports of responses to anthracyclines, cisplatin, alkylating agents, and etoposide have been published [21].…”

Section: Discussionmentioning

confidence: 99%

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011)

et al. 2016

View full text Add to dashboard Cite

Background/Aims: Primary chordomas, rare cancers arising from the notochord remnants, are extremely rare in the pediatric population. This study examined a large cohort of primary chordoma patients to determine factors impacting prognosis and survival. Methods: Demographic and clinical data on 1,358 primary chordoma patients (86 pediatric patients ≤19 years of age and 1,272 adult patients ≥20 years of age) were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011). Results: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite the majority of primary chordomas presenting with locoregional involvement (90.4%), pediatric patients had more distant disease (14.8 vs. 9.2%, p < 0.05). Survival among pediatric patients having surgery only was significantly longer than for adults (22.5 vs. 14.3 years, p < 0.001). Overall survival was longer (17.2 vs. 12.6 years) and overall mortality was lower in pediatric patients (38.4 vs. 49.8%), but cancer-specific mortality was higher (37.2 vs. 28.6%, p < 0.005). Conclusions: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite having a higher rate of metastasis, they have prolonged survival compared to adults. Surgical resection significantly improves survival in pediatric primary chordoma patients, and should be considered as first-line therapy in all eligible children.

show abstract

Section: Discussionmentioning

confidence: 99%

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011)

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Reports of tumor responses to regimens, including anthracyclines, cisplatin and alkylating agents, are only anecdotal 40 . Recently, medical oncologists have pointed out the apparent sensitivity of chordoma to new molecular-targeted agents like imatinib, cetuximab and gefitinib 41 . Unfortunately these novel drugs are only accessible in clinical studies, and only for patients with unresecteble or metastatic tumors 42 .…”

Section: Limitation Of the Non-surgical Therapymentioning

confidence: 99%

Surgical treatment of primary malignant tumors of the sacrum

Varga¹,

Szövérfi²,

Lazáry³

2014

Neurological Research

View full text Add to dashboard Cite

show abstract

“…Medical (drug) treatments for chordomas have limited efficacy. 21 Reports of durable tumor control without surgery using high-dose radiation with protons 7 or carbon ions are encouraging. 14 Because various surgical approaches have been used, ranging from intralesional to en bloc, the authors analyzed their influence on LC, particularly in the setting of highdose adjuvant RT.…”

mentioning

confidence: 99%

High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors

Rotondo

Folkert

Liebsch

et al. 2015

SPI

139

119

View full text Add to dashboard Cite

OBJECT Spinal chordomas can have high local recurrence rates after surgery with or without conventional dose radiation therapy (RT). Treatment outcomes and prognostic factors after high-dose proton-based RT with or without surgery were assessed. METHODS The authors conducted a retrospective review of 126 treated patients (127 lesions) categorized according to disease status (primary vs recurrent), resection (en bloc vs intralesional), margin status, and RT timing. RESULTS Seventy-one sacrococcygeal, 40 lumbar, and 16 thoracic chordomas were analyzed. Mean RT dose was 72.4 GyRBE (relative biological effectiveness). With median follow-up of 41 months, the 5-year overall survival (OS), local control (LC), locoregional control (LRC), and distant control (DC) for the entire cohort were 81%, 62%, 60%, and 77%, respectively. LC for primary chordoma was 68% versus 49% for recurrent lesions (p = 0.058). LC if treated with a component of preoperative RT was 72% versus 54% without this treatment (p = 0.113). Among primary tumors, LC and LRC were higher with preoperative RT, 85% (p = 0.019) and 79% (0.034), respectively, versus 56% and 56% if no preoperative RT was provided. Overall LC was significantly improved with en bloc versus intralesional resection (72% vs 55%, p = 0.016), as was LRC (70% vs 53%, p = 0.035). A trend was noted toward improved LC and LRC for R0/R1 margins and the absence of intralesional procedures. CONCLUSIONS High-dose proton-based RT in the management of spinal chordomas can be effective treatment. In patients undergoing surgery, those with primary chordomas undergoing preoperative RT, en bloc resection, and postoperative RT boost have the highest rate of local tumor control; among 28 patients with primary chordomas who underwent preoperative RT and en bloc resection, no local recurrences were seen. Intralesional and incomplete resections are associated with higher local failure rates and are to be avoided.

show abstract

Systemic Therapy Options for Unresectable and Metastatic Chordomas

Cited by 44 publications

References 86 publications

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011)

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011)

Surgical treatment of primary malignant tumors of the sacrum

High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors

Contact Info

Product

Resources

About