Chordomas and chondrosarcomas of the skull base

2019 ◽  
pp. 189-196
Author(s):  
Rami O. Almefty ◽  
Ossama Al-Mefty
Keyword(s):  
Radiation Therapy ◽  
Skull Base ◽  
Intraoperative Imaging ◽  
Survival Rates ◽  
Radical Resection ◽  
High Dose ◽  
Free Survival ◽  
Endoscopic Techniques ◽  
High Control ◽  
Skull Base Chordomas

Chordomas and chondrosarcomas are rare tumours that occur at the skull base. Chordomas have a benign appearing histology but behave malignantly with a high tendency to invade locally, recur, demonstrate tumour progression by accumulating genetic mutations, metastasize, and surgically implant. Aggressive treatment is necessary and radical resection including of the invaded bone followed by adjuvant high-dose radiation therapy offers the best chance for long-term disease-free survival. This requires initiating treatment at the onset, since once the tumour recurs the outcome is poor. Multiple surgical approaches may need to be employed to achieve the sought-after radical resection in a given patient. Utilizing all of the advances in operative techniques including microscopic and endoscopic techniques, intraoperative imaging, and neuronavigation facilitates this goal. Particle-based radiation has a proven record in a large experience as an adjunct to radical surgical resection. Chondrosarcomas are rarer and carry a better prognosis. Complete surgical removal has excellent recurrence-free survival rates. Radiation therapy has also showed high control rates but may not be necessary given their benign course. Immunohistochemical analysis is essential for ensuring the proper diagnosis.

scholarly journals Skull base chordomas: clinical outcome in a consecutive series of 45 patients with long-term follow-up and evaluation of clinical and biological prognostic factors

2016 ◽  
Vol 125 (2) ◽  
pp. 450-460 ◽  
Author(s):  
Nicola Boari ◽  
Filippo Gagliardi ◽  
Andrea Cavalli ◽  
Marco Gemma ◽  
Luca Ferrari ◽  
...  
Keyword(s):  
Overall Survival ◽  
Multivariate Analysis ◽  
Radiation Therapy ◽  
Clinical Outcome ◽  
Skull Base ◽  
Survival Rates ◽  
Progression Free Survival ◽  
Free Survival ◽  
Skull Base Chordomas

OBJECTIVE Skull base chordomas (SBCs) are rare dysembryogenetic invasive tumors with a variable tendency for recurrence. According to previous studies, the recurrence rate seems to be affected by both clinical variables and tumor biological features. The authors present the results of treatment of SBCs in a large series of patients and investigate the role of 1p36 chromosomal region loss of heterozygosity (LOH) as a prognostic factor. METHODS Between 1990 and 2011, 45 patients were treated for SBCs. The mean follow-up was 76 months (range 1–240 months). An LOH analysis was performed in 27 cases. Survival analysis was performed to determine clinical and biological parameters correlating with clinical outcome. RESULTS The 5- and 10-year overall survival rates were 67% and 57%, respectively. Five- and 10-year progression-free survival rates were 58% and 44%, respectively. Multivariate analysis showed that extent of resection, adjuvant radiation therapy, and absence of rhinopharynx invasion were positive independent predictors of overall survival. The latter 2 variables and a younger patient age were positive independent predictors of progression-free survival. Twenty-one patients showed 1p36 LOH. All events of recurrence and death clustered in the group of patients with 1p36 LOH; however, this biological marker was not statistically significant on multivariate analysis. CONCLUSIONS Resection is the treatment of choice in primary and recurrent SBC. Patient age, rhinopharynx invasion at diagnosis, extent of tumor removal, and postoperative radiation therapy influence SBC prognosis. Genetic analysis, even while showing interesting results, did not reveal 1p36 LOH as an independent predictor of clinical outcome.

Carcinoma of the ampulla of Vater: Patterns of failure after resection and benefit of adjuvant radiotherapy.

2011 ◽  
Vol 29 (4_suppl) ◽  
pp. 254-254
Author(s):  
M. Palta ◽  
C. G. Willett ◽  
P. Patel ◽  
D. S. Tyler ◽  
H. E. Uronis ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Local Control ◽  
Survival Rates ◽  
Radical Resection ◽  
Ampulla Of Vater ◽  
Ampullary Carcinoma ◽  
Local Failure ◽  
Term Survival ◽  
Free Survival ◽  
Significant Difference

254 Background: Ampullary carcinoma is a rare malignancy. Despite radical resection, survival rates remain low with high rates of local failure. To define the role of radiation therapy and chemotherapy with surgery, we performed a single institution analysis of treatment- related outcomes. Methods: A retrospective analysis was performed of all patients undergoing potentially curative therapy for adenocarcinoma of the ampulla of Vater at Duke University Hospitals between 1975 and 2009. Local control (LC), overall survival (OS), disease-free survival (DFS), and metastases-free survival (MFS) were estimated using the Kaplan-Meier Method. Results: One hundred thirty-seven patients with ampullary carcinoma underwent potentially curative pancreaticoduodenectomy. Sixty-one patients undergoing resection received adjuvant (n= 43) or neoadjuvant (n=18) radiation therapy with concurrent chemotherapy (CRT). Patients receiving radiotherapy were more likely to have poorly differentiated tumors. Median radiation dose was 50 Gy. Median follow up was 8.8 years. Of patients receiving neoadjuvant therapy, 67% were downstaged on final pathology with 28% achieving pathologic complete response. Three-year local control was significantly improved in patients receiving CRT (88% vs. 55% p= 0.001) with trend toward a 3-year OS benefit in patients receiving CRT (62% vs. 46% p=0.074). Despite this, there was no significant difference in 3-year DFS (66% CRT vs 48% surgery alone p=0.09) or MFS (69% CRT vs 63% surgery alone p=0.337). Conclusions: Long term survival rates are low. Local failure rates are high following radical resection alone and improved with CRT. Despite more adverse pathologic features in patients receiving CRT, survival outcomes were at least equivalent with a trend toward statistical significance. Given the patterns of relapse with surgery alone and local control benefit in patients receiving CRT, the use of chemoradiotherapy in selected patients should be considered. No significant financial relationships to disclose.

Phase II trial of chemotherapy alone for primary CNS and intraocular lymphoma.

1998 ◽  
Vol 16 (9) ◽  
pp. 3000-3006 ◽  
Author(s):  
V Sandor ◽  
V Stark-Vancs ◽  
D Pearson ◽  
R Nussenblat ◽  
S M Whitcup ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Response Rate ◽  
Survival Rates ◽  
Primary Cns Lymphoma ◽  
Progression Free Survival ◽  
Complete Response ◽  
Intraocular Lymphoma ◽  
Cns Lymphoma ◽  
High Dose ◽  
Free Survival

PURPOSE Primary CNS lymphoma (PCNSL) and primary intraocular lymphoma (IOL) are usually treated with radiation therapy alone or in combination with chemotherapy. The neurotoxicity of these treatments can be substantial. This study attempts to define the toxicity and efficacy of the treatment of this disease with chemotherapy alone. PATIENTS AND METHODS Fourteen nonimmunocompromised patients were accrued to a chemotherapy regimen that incorporated a 24-hour infusion of high-dose methotrexate total dose of 8.4 g/m2 with leucovorin rescue; thiotepa 35 mg/m2; vincristine 1.4 mg/m2; dexamethasone; and intrathecal cytarabine (Ara-C) and methotrexate (MTV) administered in 21-day cycles. Seven patients were prospectively followed up with formal neuropsychologic assessments for evidence of CNS toxicity. RESULTS The response rate was 100% with 11 (79%) complete responses and three (21%) partial responses. Cumulative survival and progression-free survival rates at more than 4.5 years were 68.8% and 34.3%, respectively. Median survival has not been reached, and median progression-free survival was 16.5 months. Toxicity included severe leukoencephalopathy that was clearly attributable to chemotherapy (two patients), grade 3 or 4 neutropenia in 50% of the cycles administered, ileus (one patient), and seizures (two patients). Mucositis and renal and hepatic toxicity were mild and not therapy limiting. CONCLUSION The MTV regimen is generally well tolerated and produces a high complete response rate. Chemotherapy alone should be investigated further in this disease to assess the necessity of initial radiation therapy, either alone or in combined modality regimens, for the achievement of optimal response and survival.

Skull base chordomas: a management challenge

1997 ◽  
Vol 86 (2) ◽  
pp. 182-189 ◽  
Author(s):  
Ossama Al-Mefty ◽  
Luis A. B. Borba
Keyword(s):  
Radiation Therapy ◽  
Skull Base ◽  
Photon Beam ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Surgical Removal ◽  
High Dose ◽  
Previous Surgery ◽  
The Mean ◽  
Skull Base Chordomas

✓ Because of their critical location, invasive nature, and aggressive recurrence, skull base chordomas are challenging and, at times, frustrating tumors to treat. Both radical surgical removal and high-dose radiation therapy, particularly proton beam therapy, reportedly are effective in tumor control and improve survival rates. The authors posit that these tumors are best treated with radical surgery and proton—photon beam therapy. During the last 5 years, they treated 25 patients (15 females and 10 males) who harbored pathologically diagnosed skull base chordomas. The mean age of the patients was 38.4 years (range 8–61 years). Previous surgery or radiation therapy was performed at other institutions in seven and two patients, respectively. The authors performed 33 surgical procedures on 23 patients. Radical removal (defined as absence of residual tumor on operative inspection and postoperative imaging) was achieved in 10 patients; subtotal resection (defined as resection of > 90% of the tumor) was achieved in 11 patients; and partial resection (defined as resection of < 90% of the tumor) was achieved in two patients. Radical surgical removal included not only the excision of soft-tumor tissue, but also extensive drilling of the adjacent bone. Adjuvant therapy consisted of postoperative combined proton—photon beam therapy (given to 17 patients and planned for one patient) and conventional radiation therapy (two patients); three patients received no adjunct therapy. To date, four patients have died. One patient who had undergone previous surgery and sacrifice of the internal carotid artery died postoperatively from a massive stroke; one patient died from adenocarcinoma of the pancreas without evidence of recurrence; and two patients died at 25 and 39 months of recurrent tumor. Permanent neurological complications included third cranial nerve palsy (one patient) and hemianopsia (one patient); radiation necrosis occurred in three patients. Of the 21 patients followed for more than 3 months after surgery, 16 have had no evidence of recurrence and five (including the two mortalities noted above) have had recurrent tumors (four diagnosed clinically and one radiologically). The mean disease-free interval was 14.4 months. A longer follow-up period will, hopefully, support the early indication that radical surgical removal and postoperative proton—photon beam therapy is an efficacious treatment. The use of skull base approaches based on the tumor classification introduced in this paper is associated with low mortality and morbidity rates.

Preliminary experience in treating skull base chordomas with high-dose hyperfractionated stereotactic photon radiation therapy

2013 ◽  
Vol 3 (1) ◽  
pp. 57-64 ◽  
Author(s):  
Arshin Sheybani ◽  
Raheel Ahmed ◽  
Mindi TenNapel ◽  
Edward C. Pennington ◽  
Daniel E. Hyer ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Skull Base ◽  
Photon Radiation ◽  
High Dose ◽  
Skull Base Chordomas

scholarly journals Surgical approaches and strategies for skull base chordomas

2001 ◽  
Vol 10 (3) ◽  
pp. 1-7 ◽  
Author(s):  
Norihiko Tamaki ◽  
Tatsuya Nagashima ◽  
Kazumasa Ehara ◽  
Yasuhiko Motooka ◽  
Kanak Kanti Barua
Keyword(s):  
Quality Of Life ◽  
Skull Base ◽  
Radical Resection ◽  
Surgical Approaches ◽  
Recurrence Free Survival ◽  
Total Removal ◽  
Free Survival ◽  
Skull Base Chordomas

Object The management of chordomas involving the skull base continues to present a number of treatment-related problems. Recently, both radical resection and charged-particle irradiation or stereotactic radiosurgery have reportedly been found effective for tumor control and for promoting a better quality of life in patients. In this article the authors analyzed the outcomes in 17 patients with skull base chordomas who were surgically treated at Kobe University Hospital between 1972 and 2000. Methods Preoperative radiological examinations included magnetic resonance imaging, computerized tomography, angiography, and balloon occlusion test of the internal carotid artery. Among the various surgical approaches used to remove the tumor were the frontoorbitozygomatic, transmaxillary, transcondylar, transsphenoidal, and the transbasal. Total removal was achieved in two (12%), near-total removal in three (18%), subtotal removal in nine (52%), and partial removal in three patients (18%). Since 1990, chordomas have been radically resected via various skull base approaches; the combined total or near-total removal rate has been 80% in this period. Radical removal of the tumors has not led to an increased risk. At the final follow-up review (mean 59.5 month), 75% of the patients were still alive, and 25% had died of chordoma recurrence. The overall recurrence-free survival rate was 82% at 3 years and 51% at 5 years. The 5-year recurrence-free survival rate in the five patients who underwent the operation during the past decade was 77% (mean follow up of 5.2 years). In two patients with recurrent tumors who underwent radiosurgery, no evidence of tumor regrowth was demonstrated at 3 years posttreatment. Conclusions The authors suggest that for the treatment of skull base chordomas radical resection is a key factor for longer survival and improved quality of life. Patients with sufficiently small tumors, which show a favorable configuration and location, can be suitable candidates for stereotactic radiosurgery.

Proton radiation therapy for chordomas and chondrosarcomas of the skull base

1999 ◽  
Vol 91 (3) ◽  
pp. 432-439 ◽  
Author(s):  
Eugen B. Hug ◽  
Lilia N. Loredo ◽  
Jerry D. Slater ◽  
Alexander Devries ◽  
Roger I. Grove ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Skull Base ◽  
Survival Rates ◽  
Tumor Control ◽  
High Dose ◽  
Proton Radiation ◽  
Content Type ◽  
Proton Radiation Therapy ◽  
Acceptable Risks ◽  
Fine Print

Object. Local tumor control, patient survival, and treatment failure outcomes were analyzed to assess treatment efficacy in 58 patients in whom fractionated proton radiation therapy (RT) was administered for skull base chordomas and chondrosarcomas.Methods. Between March 1992 and January 1998, a total of 58 patients who could be evaluated were treated for skull base tumors, 33 for chordoma and 25 for chondrosarcoma. Following various surgical procedures, residual tumor was detected in 91% of patients; 59% demonstrated brainstem involvement. Target dosages ranged from 64.8 and 79.2 (mean 70.7) Co Gy equivalent. The range of follow up was 7 to 75 months (mean 33 months).In 10 patients (17%) the treatment failed locally, resulting in local control rates of 92% (23 of 25 patients) for chondrosarcomas and 76% (25 of 33 patients) for chordomas. Tumor volume and brainstem involvement influenced control rates. All tumors with volumes of 25 ml or less remained locally controlled, compared with 56% of tumors larger than 25 ml (p = 0.02); 94% of patients without brainstem involvement did not experience recurrence; in patients with brainstem involvement (and dose reduction because of brainstem tolerance constraints) the authors achieved a tumor control rate of 53% (p = 0.04). Three patients died of their disease, and one died of intercurrent disease. Actuarial 5-year survival rates were 100% for patients with chondrosarcoma and 79% for patients with chordoma. Grade 3 and 4 late toxicities were observed in four patients (7%) and were symptomatic in three (5%).Conclusions. High-dose proton RT offers excellent chances of lasting tumor control and survival, with acceptable risks. In this series all small- and medium-sized tumors with no demonstrable brainstem involvement have been controlled; all such patients are alive. Surgical debulking enhanced delivery of full tumoricidal doses, but even patients with large tumors and disease abutting crucial normal structures benefited.

Combined endoscopy-assisted cranionasal approach for resection of infantile myofibromatosis of the ethmoid and anterior skull base

2008 ◽  
Vol 2 (1) ◽  
pp. 58-62 ◽  
Author(s):  
Ercole Galassi ◽  
Ernesto Pasquini ◽  
Giorgio Frank ◽  
Gianluca Marucci
Keyword(s):  
Skull Base ◽  
Skull Base Surgery ◽  
Radical Resection ◽  
Permanent Tooth ◽  
Anterior Skull Base ◽  
Facial Growth ◽  
Endoscopic Endonasal ◽  
Skull Base Reconstruction ◽  
Endoscopic Techniques ◽  
Tooth Roots

The advent and widespread development of endonasal endoscopic techniques have recently expanded the frontiers of skull base surgery. The reduced invasiveness, wider and adjustable visualization of the operative field, and lack of postoperative cosmetic defects are well-known advantages of the endonasal endoscopic approaches compared with traditional surgical exposures both in adults and in children. The need to avoid disruption of facial growth centers and permanent tooth roots represents a further special consideration in favor of these endoscopic techniques in children. The authors report on a case of solitary myofibroma involving the ethmoid, mesial orbits, and anterior skull base with intracranial intradural expansion in a 17-month-old girl. The occurrence of such proliferative disease along the skull base is exceedingly rare. The tumor was successfully excised via an endoscopy-assisted cranionasal approach in which a transcranial microsurgical exposure was combined with endonasal endoscopic access to ensure a radical resection and optimize skull base reconstruction. To the authors' knowledge, the patient in this case is the youngest reported patient in the literature who has undergone treatment with this surgical strategy. The outcome in this patient underscores the feasibility and safety of endoscopic endonasal surgery even in toddlers and early childhood.

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