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Ganesh M. Shankar, Michelle J. Clarke, Tamir Ailon, Laurence D. Rhines, Shreyaskumar R. Patel, Arjun Sahgal, Ilya Laufer, Dean Chou, Mark H. Bilsky, Daniel M. Sciubba, Michael G. Fehlings, Charles G. Fisher, Ziya L. Gokaslan and John H. Shin

OBJECTIVE

Primary osteosarcoma of the spine is a rare osseous neoplasm. While previously reported retrospective studies have demonstrated that overall patient survival is impacted mostly by en bloc resection and chemotherapy, the continued management of residual disease remains to be elucidated. This systematic review was designed to address the role of revision surgery and multimodal adjuvant therapy in cases in which en bloc excision is not initially achieved.

METHODS

A systematic literature search spanning the years 1966 to 2015 was performed on PubMed, Medline, EMBASE, and Web of Science to identify reports describing outcomes of patients who underwent biopsy alone, neurological decompression, or intralesional resection for osteosarcoma of the spine. Studies were reviewed qualitatively, and the clinical course of individual patients was aggregated for quantitative meta-analysis.

RESULTS

A total of 16 studies were identified for inclusion in the systematic review, of which 8 case reports were summarized qualitatively. These studies strongly support the role of chemotherapy for overall survival and moderately support adjuvant radiation therapy for local control. The meta-analysis revealed a statistically significant benefit in overall survival for performing revision tumor debulking (p = 0.01) and also for chemotherapy at relapse (p < 0.01). Adjuvant radiation therapy was associated with longer survival, although this did not reach statistical significance (p = 0.06).

CONCLUSIONS

While the initial therapeutic goal in the management of osteosarcoma of the spine is neoadjuvant chemotherapy followed by en bloc marginal resection, this objective is not always achievable given anatomical constraints and other limitations at the time of initial clinical presentation. This systematic review supports the continued aggressive use of revision surgery and multimodal adjuvant therapy when possible to improve outcomes in patients who initially undergo subtotal debulking of osteosarcoma. A limitation of this systematic review is that lesions amenable to subsequent resection or tumors inherently more sensitive to adjuvants would exaggerate a therapeutic effect of these interventions when studied in a retrospective fashion.

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Dean Chou, Mark H. Bilsky, Alessandro Luzzati, Charles G. Fisher, Ziya L. Gokaslan, Laurence D. Rhines, Mark B. Dekutoski, Michael G. Fehlings, Ravi Ghag, Peter Varga, Stefano Boriani, Niccole M. Germscheid, Jeremy J. Reynolds and the AOSpine Knowledge Forum Tumor

OBJECTIVE

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft-tissue sarcomas. Resection is the mainstay of treatment and the most important prognostic factor. However, complete resection of spinal MPNSTs with tumor-free margins is challenging due to the likelihood of residual tumor cells. The objective of this study was to describe whether the type of Enneking resection in the management of spinal MPNSTs had an effect on local recurrence and survival.

METHODS

The AOSpine Knowledge Forum Tumor developed a multicenter database that includes demographic, diagnostic, therapeutic, local recurrence, and survival data on patients with primary spinal column tumors. Patients who had undergone surgery for a primary spinal MPNST were included and were analyzed in 2 groups: 1) those undergoing Enneking appropriate (EA) resections and 2) those undergoing Enneking inappropriate (EI) resections. EA surgery was performed if there was histopathological evidence of an intact tumor pseudocapsule and at least a marginal resection on a vital structure. EI surgery was performed if there was an intentional or inadvertent transgression of the margin.

RESULTS

Between 1993 and 2012, 29 primary spine MPNSTs were identified in 12 (41%) females and 17 (59%) males with a mean age at diagnosis of 40 ± 17 years (range 5–74 years). The median patient follow-up was 1.3 years (range 42 days to 11.2 years). In total, 14 (48%) patients died and 14 (48%) patients suffered a local recurrence, 10 (71%) of whom died. Within 2 years after surgery, the median survival and local recurrence were not achieved. Data about Enneking appropriateness of surgery were available for 27 patients; 9 (33%) underwent an EA procedure and 18 (67%) underwent an EI procedure. Enneking appropriateness did not have a significant influence on local recurrence or survival. Twenty-two patients underwent adjuvant treatment with combined chemo- and radiotherapy (n = 7), chemotherapy alone (n = 3), or radiotherapy alone (n = 12). Adjuvant therapy had no significant influence on recurrence or survival.

CONCLUSIONS

The rates of recurrence and survival were similar for spinal MPNSTs regardless of whether patients had an EA or EI resection or received adjuvant therapy. Other factors such as variability of pathologist interpretation, PET CT correlation, or neurofibromatosis Type 1 status may play a role in patient outcome. Nonetheless, MPNSTs should still be treated as sarcomas until further evidence is known. The authors recommend an individualized approach with careful multidisciplinary decision making, and the patient should be informed about the morbidity of en bloc surgery when considering MPNST resection.

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Mark B. Dekutoski, Michelle J. Clarke, Peter Rose, Alessandro Luzzati, Laurence D. Rhines, Peter P. Varga, Charles G. Fisher, Dean Chou, Michael G. Fehlings, Jeremy J. Reynolds, Richard Williams, Nasir A. Quraishi, Niccole M. Germscheid, Daniel M. Sciubba, Ziya L. Gokaslan, Stefano Boriani and The AOSpine Knowledge Forum Tumor

OBJECTIVE

Primary spinal osteosarcomas are rare and aggressive neoplasms. Poor outcomes can occur, as obtaining marginal margins is technically demanding; further Enneking-appropriate en bloc resection can have significant morbidity. The goal of this study is to identify prognostic variables for local recurrence and mortality in surgically treated patients diagnosed with a primary osteosarcoma of the spine.

METHODS

A multicenter ambispective database of surgically treated patients with primary spine osteosarcomas was developed by AOSpine Knowledge Forum Tumor. Patient demographic, diagnosis, treatment, perioperative morbidity, local recurrence, and cross-sectional survival data were collected. Tumors were classified in 2 cohorts: Enneking appropriate (EA) and Enneking inappropriate (EI), as defined by pathology margin matching Enneking-recommended surgical margins. Prognostic variables were analyzed in reference to local recurrence and survival.

RESULTS

Between 1987 and 2012, 58 patients (32 female patients) underwent surgical treatment for primary spinal osteosarcoma. Patients were followed for a mean period of 3.5 ± 3.5 years (range 0.5 days to 14.3 years). The median survival for the entire cohort was 6.7 years postoperative. Twenty-four (41%) patients died, and 17 (30%) patients suffered a local recurrence, 10 (59%) of whom died. Twenty-nine (53%) patients underwent EA resection while 26 (47%) patients underwent EI resection with a postoperative median survival of 6.8 and 3.7 years, respectively (p = 0.048). EI patients had a higher rate of local recurrence than EA patients (p = 0.001). Patient age, previous surgery, biopsy type, tumor size, spine level, and chemotherapy timing did not significantly influence recurrence and survival.

CONCLUSIONS

Osteosarcoma of the spine presents a significant challenge, and most patients die in spite of aggressive surgery. There is a significant decrease in recurrence and an increase in survival with en bloc resection (EA) when compared with intralesional resection (EI). The effect of adjuvant and neoadjuvant chemotherapeutics, as well as method of biopsy, requires further exploration.

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Ziya L. Gokaslan, Patricia L. Zadnik, Daniel M. Sciubba, Niccole Germscheid, C. Rory Goodwin, Jean-Paul Wolinsky, Chetan Bettegowda, Mari L. Groves, Alessandro Luzzati, Laurence D. Rhines, Charles G. Fisher, Peter Pal Varga, Mark B. Dekutoski, Michelle J. Clarke, Michael G. Fehlings, Nasir A. Quraishi, Dean Chou, Jeremy J. Reynolds, Richard P. Williams, Norio Kawahara and Stefano Boriani

OBJECT

A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI.

METHODS

Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling.

RESULTS

A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local recurrence and 57 (34%) patients died. Median survival was 7.0 years postoperative: 8.4 years postoperative for EA patients and 6.4 years postoperative for EI patients (p = 0.023). The multivariate analysis showed that the EI cohort was significantly associated with an increased risk of local recurrence in comparison with the EA cohort (HR 7.02; 95% CI 2.96–16.6; p < 0.001), although no significant difference in survival was observed.

CONCLUSIONS

EA resection plays a major role in decreasing the risk for local recurrence in patients with chordoma of the mobile spine.

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Daniel M. Sciubba, Mohamed Macki, Mohamad Bydon, Niccole M. Germscheid, Jean-Paul Wolinsky, Stefano Boriani, Chetan Bettegowda, Dean Chou, Alessandro Luzzati, Jeremy J. Reynolds, Zsolt Szövérfi, Patti Zadnik, Laurence D. Rhines, Ziya L. Gokaslan, Charles G. Fisher and Peter Paul Varga

OBJECT

Clinical outcomes in patients with primary spinal osteochondromas are limited to small series and sporadic case reports. The authors present data on the first long-term investigation of spinal osteochondroma cases.

METHODS

An international, multicenter ambispective study on primary spinal osteochondroma was performed. Patients were included if they were diagnosed with an osteochondroma of the spine and received surgical treatment between October 1996 and June 2012 with at least 1 follow-up. Perioperative prognostic variables, including patient age, tumor size, spinal level, and resection, were analyzed in reference to long-term local recurrence and survival. Tumor resections were compared using Enneking appropriate (EA) or Enneking inappropriate surgical margins.

RESULTS

Osteochondromas were diagnosed in 27 patients at an average age of 37 years. Twenty-two lesions were found in the mobile spine (cervical, thoracic, or lumbar) and 5 in the fixed spine (sacrum). Twenty-three cases (88%) were benign tumors (Enneking tumor Stages 1–3), whereas 3 (12%) exhibited malignant changes (Enneking tumor Stages IA–IIB). Sixteen patients (62%) underwent en bloc treatment—that is, wide or marginal resection—and 10 (38%) underwent intralesional resection. Twenty-four operations (92%) followed EA margins. No one received adjuvant therapy. Two patients (8%) experienced recurrences: one in the fixed spine and one in the mobile spine. Both recurrences occurred in latent Stage 1 tumors following en bloc resection. No osteochondroma-related deaths were observed.

CONCLUSIONS

In the present study, most patients underwent en bloc resection and were treated as EA cases. Both recurrences occurred in the Stage 1 tumor cohort. Therefore, although benign in character, osteochondromas still require careful management and thorough follow-up.

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Charles G. Fisher, Tony Goldschlager, Stefano Boriani, Peter Paul Varga, Laurence D. Rhines, Michael G. Fehlings, Alessandro Luzzati, Mark B. Dekutoski, Jeremy J. Reynolds, Dean Chou, Sigurd H. Berven, Richard P. Williams, Nasir A. Quraishi, Chetan Bettegowda and Ziya L. Gokaslan

Object

The National Institutes of Health recommends strategies to obtain evidence for the treatment of rare conditions such as primary tumors of the spine (PTSs). These tumors have a low incidence and are pathologically heterogeneous, and treatment approaches are diverse. Appropriate evidence-based care is imperative. Failure to follow validated oncological principles may lead to unnecessary mortality and profound morbidity. This paper outlines a scientific model that provides significant evidence guiding the treatment of PTSs.

Methods

A four-stage approach was used: 1) planning: data from large-volume centers were reviewed to provide insight; 2) recruitment: centers were enrolled and provided the necessary infrastructure; 3) retrospective stage: existing medical records were reviewed and completed with survival data; and 4) prospective stage: prospective data collection has been implemented. The AOSpine Knowledge Forum Tumor designed six modules: demographic, clinical, diagnostic, therapeutic, local recurrence, survival, and perioperative morbidity data fields and provided funding.

Results

It took 18 months to implement Stages 1–3, while Stage 4 is ongoing. A total of 1495 tumor cases were captured and diagnosed as one of 18 PTS histotypes. In addition, a PTS biobank network has been created to link clinical data with tumor pathology and molecular analysis.

Conclusions

This scientific model has not only aggregated a large amount of PTS data, but has also established an international collaborative network of spine oncology centers. Access to large volumes of data will generate further research to guide and enhance PTS clinical management. This model could be applied to other rare neoplastic conditions. Clinical trial registration no.: NCT01643174 (ClinicalTrials.gov).

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Camilo A. Molina, Christopher P. Ames, Dean Chou, Laurence D. Rhines, Patrick C. Hsieh, Patricia L. Zadnik, Jean-Paul Wolinsky, Ziya L. Gokaslan and Daniel M. Sciubba

Object

Chordomas involving the mobile spine are ideally managed via en bloc resection with reconstruction to optimize local control and possibly offer cure. In the cervical spine, local anatomy poses unique challenges, limiting the feasibility of aggressive resection. The authors present a multi-institutional series of 16 cases of cervical chordomas removed en bloc. Particular attention was paid to clinical outcome, complications, and recurrence. In addition, outcomes were assessed according to position of tumor at the C1–2 level versus the subaxial (SA) spine (C3–7).

Methods

The authors reviewed cases involving patients who underwent en bloc resection of cervical chordoma at 4 large spine centers. Patients were included if the lesion epicenter involved the C-1 to C-7 vertebral bodies. Demographic data and details of surgery, follow-up course, exposure to adjuvant therapy, and complications were obtained. Outcome was correlated with presence of tumor in C1–2 versus subaxial spine via a Student t-test.

Results

Sixteen patients were identified (mean age at presentation 55 ± 14 years). Seven cases (44%) cases involved C1–2, and 16 involved the subaxial spine. Median survival did not differ significantly different between the C1–2 (72 months) and SA (60 months) groups (p = 0.65). A combined (staged anteroposterior) approach was used in 81% of the cases. Use of the combined approach was significantly more common in treatment of subaxial than C1–2 tumors (100% vs 57%, p = 0.04). En bloc resection was attempted via an anterior approach in 6% of cases (C1–2: 14.3%; SA: 0%; p = 0.17) and a posterior approach in 13% of cases (C1–2: 29%; SA: 0%; p = 0.09). The most commonly reported margin classification was marginal (56% of cases), followed by violated (25%) and wide (19%). En bloc excision of subaxial tumors was significantly more likely to result in marginal margins than excision of C1–2 tumors (C1–2: 29%; SA: 78%; p = 0.03). C1–2 tumors were associated with significantly higher rates of postoperative complications (C1–2: 71%; SA: 22%; p = 0.03). Both local and distant tumor recurrence was greatest for C1–2 tumors (local C1–2: 29%; local SA: 11%; distant C1–2: 14%; distant SA: 0%). Statistical analysis of tumor recurrence based on tumor location was not possible due to the small number of cases. There was no between-groups difference in exposure to postoperative adjuvant radiotherapy. There was no difference in median survival between groups receiving proton beam radiotherapy or intensity-modulated radiotherapy versus no radiation therapy (p = 0.8).

Conclusions

Compared with en bloc resection of chordomas involving the subaxial cervical spine, en bloc resection of chordomas involving the upper cervical spine (C1–2) is associated with poorer outcomes, such as less favorable margins, higher rates of complications, and increased tumor recurrence. Data from this cohort do not support a statistically significant difference in survival for patients with C1–2 versus subaxial disease, but larger studies are needed to further study survival differences.