scholarly journals Metastatic cluster 2-related pheochromocytoma/paraganglioma: a single-center experience and systematic review

2021 ◽  
Author(s):  
Sandeep Kumar ◽  
Anurag Ranjan Lila ◽  
Saba Samad Memon ◽  
Vijaya Sarathi ◽  
Virendra A Patil ◽  
...  
Keyword(s):  
Systematic Review ◽  
Metastatic Disease ◽  
Tumor Size ◽  
World Literature ◽  
Metastatic Lesion ◽  
Single Center Experience ◽  
Median Tumor Size ◽  
131I Mibg ◽  
Management Recommendations ◽  
Cluster 2

Objective: Risk of metastatic disease in the cluster 2-related pheochromocytoma/paraganglioma (PPGL) is low. In MEN2 patients, identification of origin of metastases from pheochromocytoma (PCC) or medullary thyroid carcinoma (MTC) is challenging as both are of neuroendocrine origin. We aim to describe our experience and perform a systematic review to assess prevalence, demographics, biochemistry, diagnostic evaluation, management and predictors of cluster 2-related metastatic PPGL. Design: Retrospective study with systematic review of literature. Methods: Data of 3 cases from our cohort and 43 cases from world literature were analyzed. For calculation of prevalence, all reported patients (n=3063) of cluster 2 were included. Results: The risk of metastasis in cluster 2-related PPGL was 2.6% (2% in RET, 5% in NF1, 4.8% in TMEM127 and 16.7% in MAX variation). In metastatic PCC in MEN2, median age was 39 years, bilateral tumors were present in 71% and median tumor size was 9.7 cm (range 4-19) with 43.5% mortality. All patients had primary tumor size ≥4cm. Origin of primary was diagnosed by histopathology of metastatic lesion in 11(57.9%), 131I-MIBG scan in 6(31.6%), and selective venous sampling and computed tomography in one (5.3%) each. In subgroup of NF1, median age was 46 years (range 14-59) with median tumor size 6cm and 57% mortality. Conclusion: The risk of metastatic disease in cluster 2-related PPGL is 2.6%, being especially high in tumors with size ≥4cm and is associated with high mortality. One-third patients of NF1 with metastatic PPGL had presented in 2nd decade of life. Long-term studies are needed to formulate management recommendations.

scholarly journals The characteristics and outcomes of patients with malignant pheochromocytoma and paraganglioma: Single-Center Experience

Acta Medica ◽  
2020 ◽  
Vol 51 (2) ◽  
pp. 25-29
Author(s):  
Asena Gökçay Canpolat ◽  
Demet Çorapçıoğlu
Keyword(s):  
Treatment Outcomes ◽  
Metastatic Disease ◽  
Cytotoxic Chemotherapy ◽  
External Beam Radiation ◽  
Beam Radiation ◽  
Tertiary Referral Center ◽  
Single Center Experience ◽  
Radiological Data ◽  
131I Mibg ◽  
The Mean

ABSTRACT Introduction: Metastatic pheochromocytomas (PCCs) and paragangliomas (PGLs) (PPGLs) are defined by the presence of metastatic disease in nonchromaffin tissue. It is rare, and there is no guidance for clinical, histological, or biochemical predictors of metastatic behavior. So, we aimed to evaluate our PPGL patients and investigate the features and treatment outcomes of our cohort. Methods: Between 2010 and 2019, 35 PCC and 3 PGL were included in the study. The age, clinical features, demographic, biochemical, radiological data, and treatment outcomes of the patients were analyzed. Results: The mean age was 38.3 ± 15.3 years, and the female to male ratio was 22/16. Twenty-one patients had hereditary causes of PCC (Multiple endocrine neoplasia (MEN) type 2A n:17; MEN2B n:4 and Neurofibromatosis n:1). The metastatic disease was seen in four of PCCs and one of PGLs. The metastatic cases were sporadic both in PCC and PGL patients. The mean greatest dimension of the primary tumor of metastatic PPGL cases was 4.5±3.2 cm. The sites for metastasis for PCC were liver, bones, lungs, and lymph nodes in all cases. Surgery was performed, cytotoxic chemotherapy, and 131I-MIBG radionuclide therapy were given for all PCCs and external beam radiation for PGL. Three of the cases were stable with cytotoxic chemotherapy and 131I-MIBG, but one case declined, and after a total of four cycles of 177Lu therapy, she died. Conclusion:: In our tertiary referral center, the majority of the patients who were followed with PGGLs have hereditary/genetic etiology. It is observed that malignant PPGLs constitute 10.5 % of our patients, the same as the literature. Patients should be directed to centers where chemotherapy and theranostic treatments with a multidisciplinary approach are present in cases of metastatic disease in an individualized manner.   Keywords: Pheochromocytoma, paraganglioma, metastatic disease, theranostic treatment                          

scholarly journals Robotic vs. Transsternal Thymectomy: A Single Center Experience over 10 Years

2021 ◽  
Vol 10 (21) ◽  
pp. 4991
Author(s):  
Luis Filipe Azenha ◽  
Robin Deckarm ◽  
Fabrizio Minervini ◽  
Patrick Dorn ◽  
Jon Lutz ◽  
...  
Keyword(s):  
Tumor Size ◽  
Survival Rates ◽  
Tumor Resection ◽  
Median Sternotomy ◽  
Good Alternative ◽  
En Bloc ◽  
Thymic Tissue ◽  
Single Center Experience ◽  
Median Tumor Size ◽  
Bovine Pericardial Patch

Introduction: Thymomas are the most common tumors of the mediastinum. Traditionally, thymectomies have been performed through a transsternal (TS) approach. With the development of robot-assisted thoracic surgery (RATS), a promising, minimally invasive, alternative surgical technique for performing a thymectomy has been developed. In the current paper, the oncological and surgical outcomes of the TS vs. RATS thymectomies are discussed. Methods: For the RATS thymectomy, two 8 mm working ports and one 12 mm camera port were used. In the transsternal approach, we performed a median sternotomy and resected the thymic tissue completely, in some cases en bloc with part of the lung and/or, more frequently, a partial pericardiectomy with consequent reconstruction using a bovine pericardial patch. The decisions for using the TS vs. RATS methods were mainly based on the suspected tumor invasion of the surrounding structures on the preoperative CT scan and tumor size. Results: Between January 2010 and November 2020, 149 patients were submitted for an anterior mediastinal tumor resection at our institution. A total of 104 patients met the inclusion criteria. One procedure was performed through a hemi-clamshell incision. A total of 81 (78%) patients underwent RATS procedures, and 22 (21.1%) patients were treated using a transsternal (TS) tumor resection. Thymoma was diagnosed in 53 (51%) cases. In the RATS group, the median LOS was 3.2 ± 2.8 days and the median tumor size was 4.4 ± 2.37 cm compared to the TS group, which had a median LOS of 9 ± 7.3 days and a median tumor size of 10.4 ± 5.3 cm. Both differences were statistically significant (p < 0.001). Complete resection was achieved in all patients. Conclusion: While larger and infiltrating tumors (i.e., thymic carcinomas) were usually resected via a sternotomy, the RATS procedure is a good alternative for the resection of thymomas of up to 9.5 cm, and the thymectomy is a strong approach for myasthenia gravis. The oncological outcomes and survival rates were not influenced by the chosen approach.

scholarly journals Primary Synovial Sarcomas of the Mediastinum: A Systematic Review and Pooled Analysis of the Published Literature

ISRN Oncology ◽  
2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Samer Salah ◽  
Ahmed Salem
Keyword(s):  
Systematic Review ◽  
Tumor Size ◽  
Descriptive Analysis ◽  
Locally Advanced ◽  
Pooled Analysis ◽  
Related Factors ◽  
Median Tumor Size ◽  
Pubmed Database ◽  
Complete Surgical Resection ◽  
Synovial Sarcomas

Background. The aim of this systematic review is to attempt to provide a descriptive analysis for cases of synovial sarcoma (SS) arising in the mediastinum and to analyze prognostic factors. Methods. We performed PubMed database search in July 2013. Twenty-two studies, which included 40 patients, form the basis of this review. Demographic and disease-related factors were analyzed for possible influence on survival. Findings were compared with extremity SS studies reported in literature. Results. Sixteen cases (40%) presented with locally advanced unresectable disease, 2 (5%) with metastatic disease, and 22 (55%) with localized resectable disease. Median tumor size was 11 cm (range: 5–20 cm). Thirty patients were assessable for survival and had a 5-year OS of 36%. Completeness of resection was the only factor associated with significant improvement in OS (5-year survival of 63% and 0% in favor of complete resection, P=0.003). Conclusion. Mediastinal SS is associated with poor prognosis as more cases are diagnosed at an advanced stage and with larger tumor size compared to extremity SS. Complete surgical resection is the only identified factor associated with better prognosis and may result in survival outcomes that are comparable with those for localized SS of the extremity.

scholarly journals Diagnostic Role of 18F-PSMA-1007 PET/CT in Prostate Cancer Staging: A Systematic Review

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 552
Author(s):  
Salam Awenat ◽  
Arnoldo Piccardo ◽  
Patricia Carvoeiras ◽  
Giovanni Signore ◽  
Luca Giovanella ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Systematic Review ◽  
Diagnostic Accuracy ◽  
Metastatic Disease ◽  
Cancer Staging ◽  
Relevant Information ◽  
Cochrane Library ◽  
Number Of Patients ◽  
Pet Ct

Background: The use of prostate-specific membrane antigen (PSMA)-targeted agents for staging prostate cancer (PCa) patients using positron emission tomography/computed tomography (PET/CT) is increasing worldwide. We performed a systematic review on the role of 18F-PSMA-1007 PET/CT in PCa staging to provide evidence-based data in this setting. Methods: A comprehensive computer literature search of PubMed/MEDLINE and Cochrane Library databases for studies using 18F-PSMA-1007 PET/CT in PCa staging was performed until 31 December 2020. Eligible articles were selected and relevant information was extracted from the original articles by two authors independently. Results: Eight articles (369 patients) evaluating the role of 18F-PSMA-1007 PET/CT in PCa staging were selected. These studies were quite heterogeneous, but, overall, they demonstrated a good diagnostic accuracy of 18F-PSMA-1007 PET/CT in detecting PCa lesions at staging. Overall, higher primary PCa aggressiveness was associated with higher 18F-PSMA-1007 uptake. When compared with other radiological and scintigraphic imaging methods, 18F-PSMA-1007 PET/CT had superior sensitivity in detecting metastatic disease and the highest inter-reader agreement. 18F-PSMA-1007 PET/CT showed similar results in terms of diagnostic accuracy for PCa staging compared with PET/CT with other PSMA-targeted tracers. Dual imaging with multi-parametric magnetic resonance imaging and 18F-PSMA-1007 PET/CT may improve staging of primary PCa. Notably, 18F-PSMA-1007-PET/CT may detect metastatic disease in a significant number of patients with negative standard imaging. Conclusions: 18F-PSMA-1007 PET/CT demonstrated a good accuracy in PCa staging, with similar results compared with other PSMA-targeted radiopharmaceuticals. This method could substitute bone scintigraphy and conventional abdominal imaging for PCa staging. Prospective multicentric studies are needed to confirm these findings.

Systematic Review Comparing Open versus Endoscopic Surgery in Clival Chordomas and a 10-Year Single-Center Experience

2021 ◽  
Author(s):  
Ravindran Visagan ◽  
Asfand Baig Mirza ◽  
Mohamed Okasha ◽  
Timothy Martyn Boardman ◽  
Eleni Maratos ◽  
...  
Keyword(s):  
Systematic Review ◽  
Surgical Management ◽  
Open Surgery ◽  
Literature Search ◽  
Optimal Management ◽  
Total Resection ◽  
Single Center Experience ◽  
Institutional Experience ◽  
Open Versus ◽  
Slow Growing

Abstract Objectives Chordomas are rare, slow-growing, and osteo-destructive tumors of the primitive notochord. There is still contention in the literature as to the optimal management of chordoma. We conducted a systematic review of the surgical management of chordoma along with our 10-year institutional experience. Design A systematic search of the literature was performed in October 2020 by using MEDLINE and EMBASE for articles relating to the surgical management of clival chordomas. We also searched for all adult patients surgically treated for primary clival chordomas at our institute between 2009 and 2019. Participants Only articles describing chordomas arising from the clivus were included in the analysis. For our institution experience, only adult primary clival chordoma cases were included. Main Outcome Measures Patients were divided into endoscopic or open surgery. Rate of gross total resection (GTR), recurrence, and complications were measured. Results Our literature search yielded 24 articles to include in the study. Mean GTR rate among endoscopic cases was 51.9% versus 41.7% for open surgery. Among the eight cases in our institutional experience, we found similar GTR rates between endoscopic and open surgery. Conclusion Although there is clear evidence in the literature that endoscopic approaches provide better rates of GTR with fewer overall complications compared to open surgery. However, there are still situations where endoscopy is not viable, and thus, open surgery should still be considered if required.

scholarly journals Pituitary apoplexy induced by gonadotropin-releasing hormone (GnRH) agonist administration for treatment of prostate cancer: a systematic review

2021 ◽  
Author(s):  
Rishi Raj ◽  
Ghada Elshimy ◽  
Aasems Jacob ◽  
P. V. Akhila Arya ◽  
Dileep C. Unnikrishnan ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Systematic Review ◽  
Pituitary Adenoma ◽  
Gnrh Agonist ◽  
Tumor Size ◽  
Pituitary Apoplexy ◽  
Clinical Presentation ◽  
Pituitary Surgery ◽  
Laboratory Evaluation ◽  
The Mean

Abstract Objective We aimed to review of literature on the clinical presentation, management and outcomes of pituitary apoplexy following gonadotrophic release hormone (GnRH) agonist administration for the treatment of prostate cancer. Methods We used PRISMA guidelines for our systematic review and included all English language original articles on pituitary apoplexy following GnRH agonist administration among prostate cancer patients from Jan 1, 1995 to Dec 31, 2020. Data on patient demographics, prostate cancer type, Gleason score at diagnosis, history of pituitary adenoma, clinical presentation, GnRH agonist, interval to pituitary apoplexy, laboratory evaluation at admission, radiologic findings, treatment of pituitary apoplexy, time to surgery if performed, pathology findings, and clinical/hormonal outcomes were collected and analyzed. Results Twenty-one patients with pituitary apoplexy met our inclusion criteria. The mean age of patients was 70 (60–83) years. Leuprolide was the most common used GnRH agonist, used in 61.9% of patients. Median duration to symptom onset was 5 h (few minutes to 6 months). Headache was reported by all patients followed by ophthalmoplegia (85.7%) and nausea/vomiting (71.4%). Three patients had blindness at presentation. Only 8 cases reported complete anterior pituitary hormone evaluation on presentation and the most common endocrine abnormality was FSH elevation. Tumor size was described only in 15 cases and the mean tumor size was 26.26 mm (18–48 mm). Suprasellar extension was the most common imaging finding seen in 7 patients. 71.4% of patients underwent pituitary surgery, while 23.8% were managed conservatively. Interval between symptoms onset to pituitary surgery was 7 days (1–90 days). Gonadotroph adenoma was most common histopathologic finding. Clinical resolution was comparable, while endocrine outcomes were variable among patients with conservative vs surgical management. Conclusion Although the use of GnRH agonists is relatively safe, it can rarely lead to pituitary apoplexy especially in patients with pre-existing pituitary adenoma. Physicians should be aware of this complication as it can be life threatening. A multidisciplinary team approach is recommended in treating individuals with pituitary apoplexy.

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