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World Journal of Urology Dec 2022To present the current evidence and the development of studies in recent years on the management of extragonadal germ cell tumors (EGCT).
PURPOSE
To present the current evidence and the development of studies in recent years on the management of extragonadal germ cell tumors (EGCT).
METHODS
A systematic literature search was conducted in Medline and the Cochrane Library. Studies within the search period (January 2010 to February 2021) that addressed the classification, diagnosis, prognosis, treatment, and follow-up of extragonadal tumors were included. Risk of bias was assessed and relevant data were extracted in evidence tables.
RESULTS
The systematic search identified nine studies. Germ cell tumors (GCT) arise predominantly from within the testis, but about 5% of the tumors are primarily located extragonadal. EGCT are localized primarily mediastinal or retroperitoneal in the midline of the body. EGCT patients are classified according to the IGCCCG classification. Consecutively, all mediastinal non-seminomatous EGCT patients belong to the "poor prognosis" group. In contrast mediastinal seminoma and both retroperitoneal seminoma and non-seminoma patients seem to have a similar prognosis as patients with gonadal GCTs and metastasis at theses respective sites. The standard chemotherapy regimen for patients with a EGCT consists of 3-4 cycles (good vs intermediate prognosis) of bleomycin, etoposid, cisplatin (BEP); however, due to their very poor prognosis patients with non-seminomatous mediastinal GCT should receive a dose-intensified or high-dose chemotherapy approach upfront on an individual basis and should thus be referred to expert centers Ifosfamide may be exchanged for bleomycin in cases of additional pulmonary metastasis due to subsequently planned resections. In general patients with non-seminomatous EGCT, residual tumor resection (RTR) should be performed after chemotherapy.
CONCLUSION
In general, non-seminomatous EGCT have a poorer prognosis compared to testicular GCT, while seminomatous EGGCT seem to have a similar prognosis to patients with metastatic testicular seminoma. The current insights on EGCT are limited, since all data are mainly based on case series and studies with small patient numbers and non-comparative studies. In general, systemic treatment should be performed like in testicular metastatic GCTs but upfront dose intensification of chemotherapy should be considered for mediastinal non-seminoma patients. Thus, EGCT should be referred to interdisciplinary centers with utmost experience in the treatment of germ cell tumors.
Topics: Male; Humans; Follow-Up Studies; Neoplasms, Germ Cell and Embryonal; Testicular Neoplasms; Seminoma; Mediastinal Neoplasms; Antineoplastic Combined Chemotherapy Protocols; Neoplasms, Second Primary; Bleomycin
PubMed: 35554637
DOI: 10.1007/s00345-022-04009-z -
Operative Neurosurgery (Hagerstown, Md.) Feb 2022Retroperitoneal nerve sheath tumors present a surgical challenge. Despite potential advantages, robotic surgery for these tumors has been limited. Identifying and... (Review)
Review
BACKGROUND
Retroperitoneal nerve sheath tumors present a surgical challenge. Despite potential advantages, robotic surgery for these tumors has been limited. Identifying and sparing functional nerve fascicles during resection can be difficult, increasing the risk of neurological morbidity.
OBJECTIVE
To review the literature regarding robotic resection of retroperitoneal nerve sheath tumors and retrospectively analyze our experience with robotic resection of these tumors using a manual electromyographic probe to identify and preserve functional nerve fascicles.
METHODS
We retrospectively analyzed the clinical courses of 3 patients with retroperitoneal tumors treated at the National Institutes of Health by a multidisciplinary team using the da Vinci Xi system. Parent motor nerve fascicles were identified intraoperatively with a bipolar neurostimulation probe inserted through a manual port, permitting tumor resection with motor fascicle preservation.
RESULTS
Two patients with neurofibromatosis type 1 underwent surgery for retroperitoneal neurofibromas located within the iliopsoas muscle, and 1 patient underwent surgery for a pelvic sporadic schwannoma. All tumors were successfully resected, with no complications or postoperative neurological deficits. Preoperative symptoms were improved or resolved in all patients.
CONCLUSION
Resection of retroperitoneal nerve sheath tumors confers an excellent prognosis, although their deep location and proximity to vital structures present unique challenges. Robotic surgery with intraoperative neurostimulation mapping is safe and effective for marginal resection of histologically benign or atypical retroperitoneal nerve sheath tumors, providing excellent visibility, increased dexterity and precision, and reduced risk of neurological morbidity.
Topics: Humans; Nerve Sheath Neoplasms; Neurilemmoma; Neurosurgical Procedures; Retrospective Studies; Robotic Surgical Procedures; United States
PubMed: 35007270
DOI: 10.1227/ONS.0000000000000051 -
World Journal of Urology Dec 2022The optimal treatment for clinical stage (CS) IIA/IIB seminomas is still controversial. We evaluated current treatment options.
PURPOSE
The optimal treatment for clinical stage (CS) IIA/IIB seminomas is still controversial. We evaluated current treatment options.
METHODS
A systematic review was performed. Only randomized clinical trials and comparative studies published from January 2010 until February 2021 were included. Search items included: seminoma, CS IIA, CS IIB and therapy. Outcome parameters were relapse rate (RR), relapse-free (RFS), overall and cancer-specific survival (OS, CSS). Additionally, acute and long-term side effects including secondary malignancies (SMs) were analyzed.
RESULTS
Seven comparative studies (one prospective and six retrospective) were identified with a total of 5049 patients (CS IIA: 2840, CS IIB: 2209). The applied treatment modalities were radiotherapy (RT) (n = 3049; CS IIA: 1888, CSIIB: 1006, unknown: 155) and chemotherapy (CT) or no RT (n = 2000; CS IIA: 797, CS IIB: 1074, unknown: 129). In CS IIA, RRs ranged from 0% to 4.8% for RT and 0% for CT. Concerning CS IIB RRs of 9.5%-21.1% for RT and of 0%-14.2% for CT have been reported. 5-year OS ranged from 90 to 100%. Only two studies reported on treatment-related toxicities.
CONCLUSIONS
RT and CT are the most commonly applied treatments in CS IIA/B seminoma. In CS IIA seminomas, RRs after RT and CT are similar. However, in CS IIB, CT seems to be more effective. Survival rates of CS IIA/B seminomas are excellent. Consequently, long-term toxicities and SMs are important survivorship issues. Alternative treatment approaches, e.g., retroperitoneal lymph node dissection (RPLND) or dose-reduced sequential CT/RT are currently under prospective investigation.
Topics: Male; Humans; Seminoma; Retrospective Studies; Prospective Studies; Neoplasm Staging; Neoplasm Recurrence, Local; Testicular Neoplasms; Neoplasms, Second Primary
PubMed: 34779882
DOI: 10.1007/s00345-021-03873-5 -
European Journal of Radiology Open 2021It is essential to see if MRI can be used as an alternative to CT for the detection of retroperitoneal lymphadenopathy in patients with testicular neoplasms. By doing...
INTRODUCTION
It is essential to see if MRI can be used as an alternative to CT for the detection of retroperitoneal lymphadenopathy in patients with testicular neoplasms. By doing so, the amount of radiation received by these young patients might be reduced.
MATERIAL AND METHODS
A systematic literature review was carried out in 5 databases between January 1984 until December 2020. The articles included were randomized and non-randomized clinical trials, cross-sectional studies, cohort, case and control, and retrospective studies that compare the accuracy of MRI against CT to detect retroperitoneal lymph nodes in patients with testicular neoplasms.
RESULTS
The search string initially retrieved 222 non duplicated papers from which a total of 3 studies of diagnostic accuracy were included for analysis. These articles evaluated a total of 127 patients with testicular neoplasm; the sample size per study ranged from 25 to 52 patients, with a mean age between 29-34 years. MRI presented a sensitivity ranging from 98-80% and specificity of 100 % when read by an experienced radiologist. However, when it was read by a radiologist with 1 year of experience, the sensitivity dropped to 78 % and specificity to 91%.
CONCLUSION
This systematic literature review shows a knowledge gap since not much has been published regarding this topic; therefore, randomized clinical trials are mandatory. Research on when to use MRI over CT is necessary to reduce radiation exposure. The authors strongly suggest that readers start researching on this subject.
PubMed: 34458506
DOI: 10.1016/j.ejro.2021.100372 -
Cancer Control : Journal of the Moffitt... 2021There is currently no consensus on optimal management of patients with primary or recurrent non-resectable/residual retroperitoneal sarcomas (RPS). The objective of this...
BACKGROUND
There is currently no consensus on optimal management of patients with primary or recurrent non-resectable/residual retroperitoneal sarcomas (RPS). The objective of this study was to document the outcomes of patients with primary or recurrent non-resectable/residual RPS treated in our center with definitive radiotherapy (RT) and to perform a systematic review on the topic.
METHODS
A retrospective analysis of consecutive RPS patients treated in our center between 2000 and 2019 was performed. All consecutive patients who underwent definitive conformal RT with image guidance for primary or recurrent non-resectable or macroscopically residual RPS were included. Additionally, a systematic review compliant with the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses was performed.
RESULTS
The study enrolled 14 patients who met the aforementioned criteria. Data on clinicopathological characteristics, RT and response to treatment were assessed. RT allowed achieving prolonged local control of the disease, i.e. no local progression of the disease for more than 12 months after RT in 10 patients. Local control lasted more than 24 months in 6 cases, with minimal or no toxicity. A systemic review of 11 studies revealed concordance of our results with previous reports of primary or recurrent non-resectable/residual RPS.
CONCLUSIONS
RT provided satisfactory local disease control with acceptable treatment tolerance in patients with primary or recurrent non-resectable/residual RPS and represents a valuable treatment modality in the selected group of patients. Additional RT modalities i.e. BT, particle therapy, MRI-guided RT, or GRID/Lattice RT may be introduced to improve local control and further minimize toxicity.
Topics: Adult; Aged; Cohort Studies; Female; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Neoplasm, Residual; Radiotherapy Dosage; Radiotherapy, Image-Guided; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma; Treatment Outcome
PubMed: 33567904
DOI: 10.1177/1073274820983028 -
Frontiers in Oncology 2020To compare perioperative, functional and oncological outcomes between transperitoneal robotic partial nephrectomy (TRPN) and retroperitoneal robotic partial nephrectomy...
BACKGROUND
To compare perioperative, functional and oncological outcomes between transperitoneal robotic partial nephrectomy (TRPN) and retroperitoneal robotic partial nephrectomy (RRPN).
METHODS
A literature searching of Pubmed, Embase, Cochrane Library and Web of Science was performed in August, 2020. Pooled odds ratios (ORs) or weighted mean differences (WMDs) with 95% confidence intervals (CIs) were estimated using fixed-effect or random-effect model. Publication bias was evaluated with funnel plots. Only comparative studies with matched design or similar baseline characteristics were included.
RESULTS
Eleven studies embracing 2,984 patients were included. There was no significant difference between the two groups regarding conversion to open (P = 0.44) or radical (P = 0.31) surgery, all complications (P = 0.06), major complications (P = 0.07), warm ischemia time (P = 0.73), positive surgical margin (P = 0.87), decline in eGFR (P = 0.42), CKD upstaging (P = 0.72), and total recurrence (P = 0.66). Patients undergoing TRPN had a significant higher minor complications (P = 0.04; OR: 1.39; 95% CI, 1.01-1.91), longer operative time (P < 0.001; WMD: 21.68; 95% CI, 11.61 to 31.76), more estimated blood loss (EBL, P = 0.002; WMD: 40.94; 95% CI, 14.87 to 67.01), longer length of hospital stay (LOS, P < 0.001; WMD: 0.86; 95% CI, 0.35 to 1.37). No obvious publication bias was identified.
CONCLUSION
RRPN is more favorable than TRPN in terms of less minor complications, shorter operative time, less EBL, and shorter LOS. Methodological limitations of the included studies should be considered while interpreting these results.
PubMed: 33489891
DOI: 10.3389/fonc.2020.592193 -
World Journal of Surgical Oncology Sep 2020Ganglioneuromas (GNs) are extremely rare, slowly growing, benign tumors that can arise from Schwann cells, ganglion cells, and neuronal or fibrous tissues. Due to their...
BACKGROUND
Ganglioneuromas (GNs) are extremely rare, slowly growing, benign tumors that can arise from Schwann cells, ganglion cells, and neuronal or fibrous tissues. Due to their origin from the sympathetic neural crest, they show neuroendocrine potential; however, most are reported to be hormonally inactive. Nevertheless, complete surgical removal is recommended for symptom control or for the prevention of potential malignant degeneration.
CASE REPORT
A 30-year-old female was referred to our oncologic center due to a giant retroperitoneal and mediastinal mass detected in computed tomography (CT) scans. The initial symptoms were transient nausea, diarrhea, and crampy abdominal pain. There was a positive family history including 5 first- and second-degree relatives. Presurgical biopsy revealed a benign ganglioneuroma. Total resection (TR) of a 35 × 25 × 25 cm, 2550-g tumor was obtained successfully via laparotomy combined with thoracotomy and partial incision of the diaphragm. Histopathological analysis confirmed the diagnosis. Surgically challenging aspects were the bilateral tumor invasion from the retroperitoneum into the mediastinum through the aortic hiatus with the need of a bilateral 2-cavity procedure, as well as the tumor-related displacement of the abdominal aorta, the mesenteric vessels, and the inferior vena cava. Due to their anatomic course through the tumor mass, the lumbar aortic vessels needed to be partially resected. Postoperative functioning was excellent without any sign of neurologic deficit.
CONCLUSION
Here, we present the largest case of a TR of a GN with retroperitoneal and mediastinal expansion. On review of the literature, this is the largest reported GN resected and was performed safely. Additionally, we present the first systematic literature review for large GN (> 10 cm) as well as for resected tumors growing from the abdominal cavity into the thoracic cavity.
Topics: Adult; Female; Ganglioneuroma; Humans; Mediastinal Neoplasms; Prognosis; Retroperitoneal Neoplasms; Retroperitoneal Space; Tomography, X-Ray Computed
PubMed: 32948207
DOI: 10.1186/s12957-020-02016-1 -
Journal of Cancer Research and Clinical... Nov 2020Testicular granulosa cell tumors (tGrCT) are rare sex cord-stromal tumors. This review aims to synthesize the available evidence regarding the clinical presentation and...
PURPOSE
Testicular granulosa cell tumors (tGrCT) are rare sex cord-stromal tumors. This review aims to synthesize the available evidence regarding the clinical presentation and clinicopathological characteristics, treatment and outcomes.
METHODS
We conducted a systematic literature search using the most important research databases. Whenever feasible, we extracted the data on individual patient level.
RESULTS
From 7863 identified records, we included 88 publications describing 239 patients with tGrCT. The majority of the cases were diagnosed with juvenile tGrCT (166/239, 69%), while 73/239 (31%) patients were diagnosed with adult tGrCT. Mean age at diagnosis was 1.5 years (± 5 SD) for juvenile tGrCT, and 42 years (± 19 SD) for adult tGrCT. Information on primary treatment was available in 231/239 (97%), of which 202/231 (87%) were treated with a radical orchiectomy and 20/231 (9%) received testis sparing surgery (TSS). Local recurrence after TSS was observed in 1/20 (5%) cases. Metastatic disease was never observed in men with juvenile tGrCT but in 7/73 (10%) men with adult tGrCT. In 5/7 men with metastatic tGrCT, metastases were diagnosed at initial staging, while 2/7 patients developed metastases after 72 and 121 months of follow-up, respectively. Primary site of metastasis is represented by the retroperitoneal lymph nodes, but other sites including lungs, liver, bone and inguinal lymph nodes can also be affected. In comparison with non-metastatic adult tGrCT, men with metastatic adult tGrCT had significantly larger primary tumors (70 vs 24 mm, p 0.001), and were more likely to present with angiolymphatic invasion (57% vs 4%, p 0.002) or gynecomastia (29% vs 3%, p 0.019). In five out of seven men with metastatic disease, resection of metastases or platinum-based chemotherapy led to complete remission.
CONCLUSION
Juvenile tGrCT represent a benign entity whereas adult tGCTs have metastatic potential. Tumor size, presence of angiolymphatic invasion or gynecomastia represent risk factors for metastatic disease. The published literature supports the use of testis sparing surgery but there is only limited experience with adjuvant therapies. In the metastatic setting, the reviewed literature suggests that aggressive surgical and systemic treatment might cure patients.
Topics: Adolescent; Adult; Child; Child, Preschool; Granulosa Cell Tumor; Humans; Infant; Male; Middle Aged; Risk Factors; Testicular Neoplasms; Treatment Outcome; Young Adult
PubMed: 32719989
DOI: 10.1007/s00432-020-03326-3 -
Radiology and Oncology Feb 2020Background There is no clear evidence on whether radiotherapy (RT) improves treatment result in patients with retroperitoneal sarcomas (RPS). Methods A systematic... (Meta-Analysis)
Meta-Analysis
Background There is no clear evidence on whether radiotherapy (RT) improves treatment result in patients with retroperitoneal sarcomas (RPS). Methods A systematic literature search was performed using PubMed, Scopus and CENTRAL databases. Data were retrieved from published comparatives studies in patients with RPS undergoing surgery alone or RT plus surgery. The primary endpoints were the 5-year OS and the median OS. The secondary endpoints were the recurrence-free survival (RFS) and the R0-resection rate. Continuous outcomes were calculated by means of weighted mean difference (WMD). Results Ten out of 374 articles were analyzed. The median OS and the 5-year survival were significantly increased in patients treated with RT and surgery, compared to patients treated with surgery alone (p < 0.00001, p < 0.001). Median RFS was significantly increased in patients treated with either preoperative (p < 0.001) or postoperative (p = 0.001) RT compared to patients that underwent surgery alone. Finally, median R0-resection rate was similar between the two groups (p = 0.56). Conclusion RT along with radical surgery could be the standard of care in at least a subgroup of patients with RPS.
Topics: Combined Modality Therapy; Disease-Free Survival; Humans; Margins of Excision; Publication Bias; Retroperitoneal Neoplasms; Sarcoma; Time Factors
PubMed: 32114526
DOI: 10.2478/raon-2020-0012 -
The Oncologist Jul 2020Sertoli cell tumors (SCTs) of the testes are rare, and the literature provides only weak evidence concerning their clinical course and management. The objective of this... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Sertoli cell tumors (SCTs) of the testes are rare, and the literature provides only weak evidence concerning their clinical course and management. The objective of this study was to summarize evidence on SCTs' clinical presentation, clinicopathological risk factors for malignancy, treatment options, and oncological outcomes.
MATERIALS AND METHODS
Data sources included Medline, Embase, Scopus, the Cochrane Database of Systematic Reviews, and Web of Science. Published case reports, case series, and cohorts were included. Data on clinicopathological variables, treatment of local or metastatic disease, site of metastasis, or survival were extracted from each study considered in this paper, and associations between clinicopathological variables and metastatic disease were analyzed. Whenever feasible, data on individual patients were collected.
RESULTS
Of the 435 patients included, only one (<1%) showed local recurrence after testis-sparing surgery (TSS). Three patients underwent adjuvant retroperitoneal lymphadenectomy. Fifty patients presented with metastases, located in the retroperitoneal lymph nodes (76%), lungs (36%), and bones (16%); median time to recurrence was 12 months. Risk factors for metastatic disease included age, tumor size, necrosis, tumor extension to the spermatic cord, angiolymphatic invasion, and mitotic index. Patients with metastases had a median life expectancy of 20 months. In six patients, metastasectomy resulted in complete remission.
CONCLUSION
Our findings suggest that few local recurrences result after TSS, and no adjuvant therapy can be regarded as a standard of care. Several risk factors are predictive of metastatic disease. Surgery leads to remission in metastatic disease, whereas systemic treatment alone does not result in long-term remission.
IMPLICATIONS FOR PRACTICE
Testicular Sertoli cell tumors usually present without metastatic disease and show low local recurrence rates after testis-sparing surgery; no adjuvant therapy option can be regarded as a standard of care. Patients with risk factors should undergo staging investigations. Those with metastatic disease have poor prognoses, and metastasectomy may be offered in selected cases.
Topics: Humans; Lymph Node Excision; Male; Neoplasm Recurrence, Local; Neoplasm Staging; Sertoli Cell Tumor; Systematic Reviews as Topic; Testicular Neoplasms
PubMed: 32043680
DOI: 10.1634/theoncologist.2019-0692