The Role of Radiosurgery for the Treatment of Pineal Parenchymal Tumors

Neurosurgery ◽  
2002 ◽  
Vol 51 (4) ◽  
pp. 880-889 ◽  
Author(s):  
Toshinori Hasegawa ◽  
Douglas Kondziolka ◽  
Constantinos G. Hadjipanayis ◽  
John C. Flickinger ◽  
L. Dade Lunsford
Keyword(s):  
Complete Remission ◽  
Partial Remission ◽  
Radiation Effects ◽  
Survival Rates ◽  
Tumor Control ◽  
Local Tumor ◽  
Pineal Parenchymal Tumors ◽  
Local Progression ◽  
The Mean

Abstract OBJECTIVE Radiosurgery is an appealing alternative management strategy for selected patients with biopsy-proved pineal parenchymal tumors. The purpose of this report was to clarify its role in conjunction with other surgical, radiation, and medical approaches. METHODS We retrospectively evaluated 16 patients who had undergone radiosurgery as the primary or adjuvant treatment for pineal parenchymal tumors. Ten patients (62.5%) had pineocytomas, two (12.5%) had mixed pineocytoma and pineoblastoma, and four (25%) had pineoblastomas. The mean marginal dose was 15 Gy, and the mean tumor volume was 5.0 cm3. The mean follow-up periods from the time of diagnosis or the time of radiosurgery were 61 and 52 months, respectively. RESULTS The overall actuarial 2- and 5-year survival rates after diagnosis were 75.0 and 66.7%, respectively. In 14 patients who were evaluated with imaging, 4 (29%) demonstrated complete remission, 8 (57%) had partial remission, 2 (14%) had no change, and no patient had local progression. The local tumor control rate (complete remission, partial remission, or no change) was 100%. Five patients died during follow-up. One patient with a pineocytoma and three patients with pineoblastomas died secondary to leptomeningeal or extracranial spread tumor. No cause of death was established for one patient. Two patients developed adverse radiation effects after radiosurgery. CONCLUSION Our initial experience suggests that stereotactic radiosurgery is a valuable primary management modality for patients with pineocytomas. As adjuvant therapy, radiosurgery may be used to boost local tumor dose during multimodality management of malignant pineal parenchymal tumors.

scholarly journals PC3 - 138 Stereotactic Radiosurgery for Intracranial Hemangiopericytomas – A Multicenter Study

2016 ◽  
Vol 43 (S4) ◽  
pp. S21-S21
Author(s):  
O. Cohen-Inbar
Keyword(s):  
Stereotactic Radiosurgery ◽  
Multicenter Study ◽  
Radiation Effects ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Local Tumor ◽  
Who Grade ◽  
Fractionated Radiotherapy ◽  
Local Progression

Hemangiopericytomas (HPC) are widely recognized for their aggressive clinical behavior. We report a large multicenter study, through the International Gamma Knife Research Foundation reviewing management and outcome following stereotactic radiosurgery (SRS) for recurrent or newly-discovered HPC’s. Methods: Eight centers participated, reviewing a total of 90 patients harboring 133 tumors. Prior treatments included embolization (n = 8), chemotherapy (n=2), and fractionated radiotherapy (n=34). The median tumor volume at the time of SRS was 4.9 ml (range 0.2-42.4 ml). WHO-grade II (typical) HPC’s formed 78.9% (n=71) of the cohort. The median margin and maximal doses delivered were 15 Gy (2.8-24) and 32 Gy (8-51), respectively. The median clinical and radiographic follow-up period was 59 months (6-190) and 59 months (6-183), respectively. Results: At last follow-up, 55% of tumors and 62.2% of patients demonstrated local tumor control. New remote intracranial tumors were found in 27.8%. 24.4% of patients developed extra-cranial metastases. Adverse radiation effects were noted in 6.7%. The overall survival was 91.5%, 82.1%, 73.9%, 56.7%, and 53.7% at 2, 4, 6, 8, and 10 years, respectively, after initial SRS. Local progression free survival was 81.7%, 66.3%, 54.5%, 37.2%, and 25.5% at 2, 4, 6, 8, and 10 years, respectively, after initial SRS. In our cohort, 32 patients underwent 48 repeat SRS procedures for 76 lesions. Margin dose greater than 16 Gy (p=0.037) and tumor histology (p=0.006) were shown to influence PFS. Conclusions: SRS provides a reasonable rate of local tumor control and a low risk of adverse effects

Radiosurgery for Treatment of Recurrent Intracranial Hemangiopericytomas

Neurosurgery ◽  
2002 ◽  
Vol 51 (4) ◽  
pp. 905-911 ◽  
Author(s):  
Jason Sheehan ◽  
Douglas Kondziolka ◽  
John Flickinger ◽  
L. Dade Lunsford
Keyword(s):  
Stereotactic Radiosurgery ◽  
Survival Rates ◽  
Gamma Knife Radiosurgery ◽  
Residual Tumor ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Local Tumor ◽  
University Of Pittsburgh ◽  
Kaplan Meier

Abstract OBJECTIVE Hemangiopericytomas are highly aggressive meningeal tumors with tendencies for recurrence and metastasis. The purpose of this retrospective, single-institution review was to evaluate the efficacy and role of stereotactic radiosurgery in the management of recurrent hemangiopericytomas. METHODS We reviewed data for patients who underwent stereotactic radiosurgery at the University of Pittsburgh between 1987 and 2001. Fourteen patients underwent radiosurgery for 15 discrete tumors. Prior treatments included transsphenoidal resection (n = 1), craniotomy and resection (n = 27), embolization (n = 1), and conventional radiotherapy (n = 7). Clinical and radiological responses were evaluated. Follow-up periods varied from 5 to 76 months (mean, 31.3 mo; median, 21 mo). The mean radiation dose to the tumor margin was 15 Gy. RESULTS Seventy-nine percent of patients (11 of 14 patients) with recurrent hemangiopericytomas demonstrated local tumor control after radiosurgery. Twelve of 15 tumors (i.e., 80%) dramatically decreased in size on follow-up imaging scans. Regional intracranial recurrences were retreated with radiosurgery for two patients (i.e., 15%); neither of those two patients experienced long-term tumor control. Local recurrences occurred 12 to 75 months (median, 21 mo) after radiosurgery. Local tumor control and survival rates at 5 years after radiosurgery were 76 and 100%, respectively (Kaplan-Meier method). We could not correlate prior irradiation or tumor size with tumor control. Twenty-nine percent of the patients (4 of 14 patients) developed remote metastases. Radiosurgery did not seem to offer protection against the development of intra- or extracranial metastases. CONCLUSION Gamma knife radiosurgery provided local tumor control for 80% of recurrent hemangiopericytomas. When residual tumor is identified after resection or radiotherapy, early radiosurgery should be considered as a feasible treatment modality. Despite local tumor control, patients are still at risk for distant metastasis. Diligent clinical and radiological follow-up monitoring is necessary.

Radiosurgery for chordomas and chondrosarcomas of the skull base

2007 ◽  
Vol 107 (4) ◽  
pp. 758-764 ◽  
Author(s):  
Juan J. Martin ◽  
Ajay Niranjan ◽  
Douglas Kondziolka ◽  
John C. Flickinger ◽  
Karl A. Lozanne ◽  
...  
Keyword(s):  
Skull Base ◽  
Stereotactic Radiosurgery ◽  
Radiation Effects ◽  
Local Tumor Control ◽  
Adjunctive Treatment ◽  
Tumor Control ◽  
Local Tumor ◽  
High Tendency ◽  
Local Progression ◽  
Multimodal Management

Object Chordomas and chondrosarcomas of the skull base are aggressive and locally destructive tumors with a high tendency for local progression despite treatment. The authors evaluated the effect of stereotactic radiosurgery (SRS) on local tumor control and survival. Methods Twenty-eight patients with histologically confirmed chordomas (18) or chondrosarcomas (10) underwent Gamma Knife SRS either as primary or adjuvant treatment. Their ages ranged from 17 to 72 years (median 44 years). The most common presenting symptom was diplopia (26 patients, 93%). In two patients, SRS was the sole treatment. Twenty-six patients underwent between one and five additional surgical procedures. Two underwent an initial trans-sphenoidal biopsy. The average tumor volume was 9.8 cm3. The median dose to the tumor margin was 16 Gy. Results No patient was lost to follow-up. Transient symptomatic adverse radiation effects developed in only one patient. The actuarial local tumor control for chondrosarcomas at 5 years was 80 ± 10.1%. For chordomas both the actuarial tumor control and survival was 62.9 ± 10.4%. Conclusions Stereotactic radiosurgery is an important option for skull base chordomas and chondrosarcomas either as primary or adjunctive treatment. Multimodal management appears crucial to improve tumor control in most patients.

scholarly journals The Role of a Navigational Radiofrequency Ablation Device and Concurrent Vertebral Augmentation for Treatment of Difficult-to-Reach Spinal Metastases

2021 ◽  
Vol 28 (5) ◽  
pp. 4004-4015
Author(s):  
Claudio Pusceddu ◽  
Davide De Francesco ◽  
Luca Melis ◽  
Nicola Ballicu ◽  
Alessandro Fancellu
Keyword(s):  
Radiofrequency Ablation ◽  
Pain Relief ◽  
Spinal Metastases ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Vertebral Augmentation ◽  
Local Tumor ◽  
Metastatic Lesions ◽  
The Mean

Aims: The purpose of this study was to assess the effectiveness of a navigational radiofrequency ablation device with concurrent vertebral augmentation in the treatment of posterior vertebral body metastatic lesions, which are technically difficult to access. Primary outcomes of the study were evaluation of pain palliation and radiologic assessment of local tumor control. Materials and Methods: Thirty-five patients with 41 vertebral spinal metastases involving the posterior vertebral body underwent computed tomography-guided percutaneous targeted radiofrequency ablation, with a navigational radiofrequency ablation device, associated with vertebral augmentation. Twenty-one patients (60%) had 1 or 2 metastatic lesions (Group A) and fourteen (40%) patients had multiple (>2) vertebral lesions (Group B). Changes in pain severity were evaluated by visual analog scale (VAS). Metastatic lesions were evaluated in terms of radiological local control. Results: The procedure was technically successful in all the treated vertebrae. Among the symptomatic patients, the mean VAS score dropped from 5.7 (95% CI 4.9–6.5) before tRFA and to 0.9 (95% CI 0.4–1.3) after tRFA (p < 0.001). The mean decrease in VAS score between baseline and one week follow up was 4.8 (95% CI 4.2–5.4). VAS decrease over time between one week and one year following radiofrequency ablation was similar, suggesting that pain relief was immediate and durable. Neither patients with 1–2 vertebral metastases, nor those with multiple lesions, showed radiological signs of local progression or recurrence of the tumor in the index vertebrae during a median follow up of 19 months (4–46 months) and 10 months (4–37 months), respectively. Conclusion: Treatment of spinal metastases with a navigational radiofrequency ablation device and vertebral augmentation can be used to obtain local tumor control with immediate and durable pain relief, providing effective treatment in the multimodality management of difficult-to-reach spinal metastases.

scholarly journals Late Toxicities, Failure Patterns, Local Tumor Control, and Survival of Esophageal Squamous Cell Carcinoma Patients After Chemoradiotherapy With a Simultaneous Integrated Boost: A 5-Year Phase II Study

2021 ◽  
Vol 11 ◽  
Author(s):  
Chuangzhen Chen ◽  
Jianzhou Chen ◽  
Ting Luo ◽  
Siyan Wang ◽  
Hong Guo ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Rates ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Local Tumor ◽  
Integrated Boost ◽  
Grade 3 ◽  
Overall Survival Rates

PurposeWe aimed to evaluate the long-term outcomes of concurrent chemoradiotherapy (CCRT) with a simultaneous integrated boost (SIB) of radiotherapy for esophageal squamous cell carcinoma (ESCC).Methods and MaterialsEighty-seven patients with primary ESCC enrolled in this phase II trial. The majority (92.0%) had locoregionally advanced disease. They underwent definitive chemoradiotherapy. The radiotherapy doses were 66 Gy for the gross tumor and 54 Gy for the subclinical disease. Doses were simultaneously administered in 30 fractions over 6 weeks. The patients also underwent concurrent and adjuvant chemotherapy, which comprised cisplatin and fluorouracil. The study end points were acute and late toxicities, first site of failure, locoregional tumor control, and overall survival rates.ResultsThe median follow-up time was 65.7 (range, 2.2-97.5) months for all patients and 81.5 (range, 19.4-97.5) months for those alive. There were 17 cases (19.5%) of severe late toxicities, including four cases (4.6%) of grade 5 and seven (8.0%) of grade 3 esophageal ulceration, four (4.6%) of grade 3 esophageal stricture, and two (2.3%) of grade 3 radiation-induced pneumonia. Twenty-three (26.4%) patients had locoregional disease progression. Most (86.7%) locally progressive lesions were within the dose-escalation region in the initial radiation plan, while majority of the recurrent lymph nodes were found out-of-field (83.3%) and in the supraclavicular region (75.0%). The 1-, 2-, 3-, and 5-year locoregional tumor control and overall survival rates were 79.2%, 72.4%, 72.4%, 70.8%, and 82.8%, 66.6%, 61.9%, 58.4%, respectively. Incomplete tumor response, which was assessed immediately after CCRT was an independent risk predictor of disease progression and death in ESCC patients.ConclusionsCCRT with SIB was well tolerated in ESCC patients during treatment and long-term follow-up. Moreover, patients who underwent CCRT with SIB exhibited improved local tumor control and had better survival outcomes compared to historical data of those who had standard-dose radiotherapy.

Safety and Efficacy of Hypo fractionated Stereotactic Radiosurgery in Facial Nerve Schwannoma.

2021 ◽  
Author(s):  
Nida Fatima ◽  
Anna La Dine ◽  
Zachary R Barnard ◽  
Katherine Ko ◽  
Kevin Peng ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Stereotactic Radiosurgery ◽  
Radiation Effects ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Nerve Function ◽  
Local Tumor ◽  
Nerve Preservation ◽  
Fractionated Stereotactic Radiosurgery

Abstract Purpose In the current era of modern neurosurgery, the treatment strategies have been shifted to “nerve-preservation approaches” for achieving a higher facial and hearing function preservation rate following facial nerve tumors. We have conducted this novel report on determining the outcome of patients with facial nerve schwannomas (FNS) treated with hypo fractionated stereotactic radiosurgery (hfSRS). Methods Retrospective chart review of a prospectively maintained database search was conducted. Patients who underwent hfSRS CyberKnife (Accuray Inc, Sunnyvale, California., USA) for FNS were included. Outcomes consisted of tumor control, facial and hearing nerve function as graded by House-Brackmann (HB) and Gardner-Robertson scale, and adverse radiation effects. SPSS 23 was used to perform statistical analysis. Results With an institutional board review approval, we retrospectively identified 5 patients with FNS [4 intracranial (80%) and 1 extracranial (20%)] treated with hfSRS (2011–2019). Patients received definitive SRS in 3 patients (60.0%) wile adjuvant to surgical resection in 2 patients (40.0%). A median tumor volume of 7.5 cm3 (range, 1.5–19.6 cm3) received a median prescription dose of 23.2 Gy (range, 21–25 Gy) administered in median of 3 fractions (range, 3–5 session). With a median radiographic follow-up of 31.4 months (range, 13.0–71.0 months) and clinical follow-up of 32.6 months (range, 15.1–72.0 months), the local tumor control was 100.0%. At last clinical follow-up, the facial nerve function improved or remained unchanged HB I-II in 80.0% of the patients, while the hearing nerve function improved or remained stable in 100.0% (Gardner-Robertson I-II) of the patients. Temporary clinical toxicity was seen in 3 patients (60.0%) which resolved. None of the patient developed adverse radiation effect. Conclusion hfSRS in FNS is safe and efficacious in terms of local tumor control, improved facial and hearing nerve function, and very low incidence of complications.

Stereotactic radiosurgery for intracranial hemangiopericytomas: a multicenter study

2017 ◽  
Vol 126 (3) ◽  
pp. 744-754 ◽  
Author(s):  
Or Cohen-Inbar ◽  
Cheng-Chia Lee ◽  
Seyed H. Mousavi ◽  
Hideyuki Kano ◽  
David Mathieu ◽  
...  
Keyword(s):  
Overall Survival ◽  
Stereotactic Radiosurgery ◽  
Multicenter Study ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Local Tumor ◽  
Who Grade ◽  
Local Progression ◽  
Extracranial Metastases

OBJECTIVE Hemangiopericytomas (HPCs) are rare tumors widely recognized for their aggressive clinical behavior, high recurrence rates, and distant and extracranial metastases even after a gross-total resection. The authors report a large multicenter study, through the International Gamma Knife Research Foundation (IGKRF), reviewing management and outcome following stereotactic radiosurgery (SRS) for recurrent or newly discovered HPCs. METHODS Eight centers participating in the IGKRF participated in this study. A total of 90 patients harboring 133 tumors were identified. Patients were included if they had a histologically diagnosed HPC managed with SRS during the period 1988–2014 and had a minimum of 6 months' clinical and radiological follow-up. A de-identified database was created. The patients' median age was 48.5 years (range 13–80 years). Prior treatments included embolization (n = 8), chemotherapy (n = 2), and fractionated radiotherapy (n = 34). The median tumor volume at the time of SRS was 4.9 cm3 (range 0.2–42.4 cm3). WHO Grade II (typical) HPCs formed 78.9% of the cohort (n = 71). The median margin and maximum doses delivered were 15 Gy (range 2.8–24 Gy) and 32 Gy (range 8–51 Gy), respectively. The median clinical and radiographic follow-up periods were 59 months (range 6–190 months) and 59 months (range 6–183 months), respectively. Prognostic variables associated with local tumor control and post-SRS survival were evaluated using Cox univariate and multivariate analysis. Actuarial survival after SRS was analyzed using the Kaplan-Meier method. RESULTS Imaging studies performed at last follow-up demonstrated local tumor control in 55% of tumors and 62.2% of patients. New remote intracranial tumors were found in 27.8% of patients, and 24.4% of patients developed extracranial metastases. Adverse radiation effects were noted in 6.7% of patients. During the study period, 32.2% of the patients (n = 29) died. The actuarial overall survival was 91.5%, 82.1%, 73.9%, 56.7%, and 53.7% at 2, 4, 6, 8, and 10 years, respectively, after initial SRS. Local progression–free survival (PFS) was 81.7%, 66.3%, 54.5%, 37.2%, and 25.5% at 2, 4, 6, 8, and 10 years, respectively, after initial SRS. In our cohort, 32 patients underwent 48 repeat SRS procedures for 76 lesions. Review of these 76 treated tumors showed that 17 presented as an in-field recurrence and 59 were defined as an out-of-field recurrence. Margin dose greater than 16 Gy (p = 0.037) and tumor grade (p = 0.006) were shown to influence PFS. The development of extracranial metastases was shown to influence overall survival (p = 0.029) in terms of PFS; repeat (multiple) SRS showed additional benefit. CONCLUSIONS SRS provides a reasonable rate of local tumor control and a low risk of adverse effects. It also leads to neurological stability or improvement in the majority of patients. Long-term close clinical and imaging follow-up is necessary due to the high probability of local recurrence and distant metastases. Repeat SRS is often effective for treating new or recurrent HPCs.

Gamma Knife surgery combined with resection for treatment of a single brain metastasis: preliminary results

2010 ◽  
Vol 113 (Special_Supplement) ◽  
pp. 90-96 ◽  
Author(s):  
M. Yashar S. Kalani ◽  
Aristotelis S. Filippidis ◽  
Maziyar A. Kalani ◽  
Nader Sanai ◽  
David Brachman ◽  
...  
Keyword(s):  
Survival Data ◽  
Initial Treatment ◽  
Median Survival Time ◽  
Local Tumor Control ◽  
Survival Duration ◽  
Tumor Control ◽  
Local Tumor ◽  
Systemic Control ◽  
Cranial Metastasis

Object Resection and whole-brain radiation therapy (WBRT) have classically been the standard treatment for a single metastasis to the brain. The objective of this study was to evaluate the use of Gamma Knife surgery (GKS) as an alternative to WBRT in patients who had undergone resection and to evaluate patient survival and local tumor control. Methods The authors retrospectively reviewed the charts of 150 patients treated with a combination of stereotactic radiosurgery and resection of a cranial metastasis at their institution between April 1997 and September 2009. Patients who had multiple lesions or underwent both WBRT and GKS were excluded, as were patients for whom survival data beyond the initial treatment were not available. Clinical and imaging follow-up was assessed using notes from clinic visits and MR imaging studies when available. Follow-up data beyond the initial treatment and survival data were available for 68 patients. Results The study included 37 women (54.4%) and 31 men (45.6%) (mean age 60 years, range 28–89 years). In 45 patients (66.2%) there was systemic control of the primary tumor when the cranial metastasis was identified. The median duration between resection and radiosurgery was 15.5 days. The median volume of the treated cavity was 10.35 cm3 (range 0.9–45.4 cm3), and the median dose to the cavity margin was 15 Gy (range 14–30 Gy), delivered to the 50% isodose line (range 50%–76% isodose line). The patients' median preradiosurgery Karnofsky Performance Scale (KPS) score was 90 (range 40–100). During the follow-up period we identified 27 patients (39.7%) with recurrent tumor located either local or distant to the site of treatment. The median time from primary treatment of metastasis to recurrence was 10.6 months. The patients' median length of survival (interval between first treatment of cerebral metastasis and last follow-up) was 13.2 months. For the patient who died during follow-up, the median time from diagnosis of cerebral metastasis to death was 11.5 months. The median duration of survival from diagnosis of the primary cancer to last follow-up was 30.2 months. Patients with a pretreatment KPS score ≥ 90 had a median survival time of 23.2 months, and patients with a pretreatment KPS score < 90 had a median survival time of 10 months (p < 0.008). Systemic control of disease at the time of metastasis was not predictive of increased survival duration, although it did tend to improve survival. Conclusions Although the debate about the ideal form of radiation treatment after resection continues, these findings indicate that GKS combined with surgery offers comparable survival duration and local tumor control to WBRT for patients with a diagnosis of a single metastasis.

Treatment of epidemic Kaposi's sarcoma with etoposide or a combination of doxorubicin, bleomycin, and vinblastine.

1984 ◽  
Vol 2 (10) ◽  
pp. 1115-1120 ◽  
Author(s):  
L J Laubenstein ◽  
R L Krigel ◽  
C M Odajnyk ◽  
K B Hymes ◽  
A Friedman-Kien ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
Complete Remission ◽  
Partial Remission ◽  
Kaposi's Sarcoma ◽  
Opportunistic Infections ◽  
Kaposi’S Sarcoma ◽  
Response Duration ◽  
Median Response ◽  
Stage Disease

An epidemic of disseminated Kaposi's sarcoma in male homosexuals has recently been described. Forty-one evaluable patients with epidemic Kaposi's sarcoma were treated with etoposide. The majority of these patients had early stage disease, no prior opportunistic infections, and no prior therapy. Twelve patients (30%) achieved complete remission, 19 (46%) partial remission, and ten (24%) no response. With follow-up time to 31 months, the median response duration is nine months. The median survival of patients with complete and partial remissions has not been reached. A combination of doxorubicin (Adriamycin, Adria Laboratories, Columbus, Ohio), bleomycin, and vinblastine (ABV) was used in 31 evaluable patients with epidemic Kaposi's sarcoma. The majority of these patients had late stage disease, prior opportunistic infections, or had failed prior treatment. Seven patients (23%) achieved complete remission, 19 (61%) partial remission, and five (61%) no response. With follow-up time to 24 months, the median response duration is eight months. The projected median survival for all patients treated with ABV is nine months. Both regimens were well tolerated, with an overall response rate of 76% for etoposide and 84% for ABV. However, while successfully treating the Kaposi's sarcoma, the underlying immune deficiency in these patients has persisted. Future treatments of Kaposi's sarcoma will need to focus on reversing the underlying immune incompetence as well as controlling the malignant manifestations of Kaposi's sarcoma arising in relation to the acquired immune deficiency syndrome.

scholarly journals Surgical treatment of pulmonary artery sarcoma: a report of 17 cases

2021 ◽  
Vol 11 (1) ◽  
pp. 204589402098639
Author(s):  
Wu Song ◽  
Long Deng ◽  
Jiade Zhu ◽  
Shanshan Zheng ◽  
Haiping Wang ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Survival Rates ◽  
Tumor Resection ◽  
Long Term Results ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Artery Sarcoma ◽  
The Mean ◽  
Operative Findings

Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis is often delayed, and optimal treatment remains unclear. The aim of this study is to report our experience in the surgical management of this disease. Between 2000 and 2018, 17 patients underwent operations for PAS at our center. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings, the postoperative outcomes, and the long-term results. The mean age at operation was 46.0 ± 12.4 years (range, 26–79 years), and eight (47.1%) patients were male. Six patients underwent tumor resection alone, whereas the other 11 patients received pulmonary endarterectomy (PEA). There were two perioperative deaths. Follow-up was completed for all patients with a mean duration of 23.5 ± 17.6 months (1–52 months). For all 17 patients, the median postoperative survival was 36 months, and estimated cumulative survival rates at 1, 2, 3, and 4 years were 60.0%, 51.4%, 42.9%, and 21.4%, respectively. The mean survival was 37.0 months after PEA and 14.6 months after tumor resection only ( p = 0.046). Patients who had no pulmonary hypertension (PH) postoperatively were associated with improved median survival (48 vs. 5 months, p = 0.023). In conclusion, PAS is often mistaken for chronic pulmonary thromboembolism. The prognosis of this very infrequent disease remains poor. Early detection is essential for prompt and best surgical approach, superior to tumor resection alone, and PEA surgery with PH relieved can provide better chance of survival.

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