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Neurosurgical Focus: Video Apr 2024Ganglioneuroma (GN) is a rare solid neoplasm developing from neural crest cells of sympathetic ganglia or adrenal medulla. It usually presents as an asymptomatic mass in...
Ganglioneuroma (GN) is a rare solid neoplasm developing from neural crest cells of sympathetic ganglia or adrenal medulla. It usually presents as an asymptomatic mass in the retroperitoneal space and mediastinum. Resection through open surgery or minimal access is recommended. The video illustrates the case of a 23-year-old female with an incidental finding of thoracic GN. The authors performed a combined, staged approach to ensure complete resection, which involved unilateral T3-4 biportal endoscopy (UBE) for rhizotomy and nerve root decompression, followed by video-assisted thoracoscopic surgery (VATS) for complete excision. The procedure was uneventful, with full recovery and no postoperative complications. The video can be found here: https://stream.cadmore.media/r10.3171/2024.2.FOCVID23210.
PubMed: 38616900
DOI: 10.3171/2024.2.FOCVID23210 -
Critical Reviews in Oncology/hematology May 2024Preoperative biopsy for retroperitoneal sarcoma (RPS) enables appropriate multidisciplinary treatment planning. A systematic review of literature from 1990 to June 2022...
What is the association of preoperative biopsy with recurrence and survival in retroperitoneal sarcoma? A systematic review by the Australia and New Zealand Sarcoma Association clinical practice guidelines working party.
Preoperative biopsy for retroperitoneal sarcoma (RPS) enables appropriate multidisciplinary treatment planning. A systematic review of literature from 1990 to June 2022 was conducted using the population, intervention, comparison and outcome model to evaluate the local recurrence and overall survival of preoperative biopsy compared to those that had not. Of 3192 studies screened, five retrospective cohort studies were identified. Three reported on biopsy needle tract seeding, with only one study reporting biopsy site recurrence of 2 %. Two found no significant difference in local recurrence and one found higher 5-year local recurrence rates in those who had not been biopsied. Three studies reported overall survival, including one with propensity matching, did not show a difference in overall survival. In conclusion, preoperative core needle biopsy of RPS is not associated with increased local recurrence or adverse survival outcomes.
Topics: Humans; Australia; Biopsy; Neoplasm Recurrence, Local; New Zealand; Practice Guidelines as Topic; Preoperative Care; Retroperitoneal Neoplasms; Sarcoma
PubMed: 38614268
DOI: 10.1016/j.critrevonc.2024.104354 -
Cell Death & Disease Apr 2024The primary site of metastasis for epithelial ovarian cancer (EOC) is the peritoneum, and it occurs through a multistep process that begins with adhesive contacts...
The primary site of metastasis for epithelial ovarian cancer (EOC) is the peritoneum, and it occurs through a multistep process that begins with adhesive contacts between cancer cells and mesothelial cells. Despite evidence that Notch signaling has a role in ovarian cancer, it is unclear how exactly it contributes to ovarian cancer omental metastasis, as well as the cellular dynamics and intrinsic pathways that drive this tropism. Here we show that tumor cells produced the Notch ligand Jagged2 is a clinically and functionally critical mediator of ovarian cancer omental metastasis by activating the Notch signaling in single-layered omental mesothelial cells. In turn, Jagged2 promotes tumor growth and therapeutic resistance by stimulating IL-6 release from mesothelial cells. Additionally, Jagged2 is a potent downstream mediator of the omental metastasis cytokine TGF-β that is released during omental destruction. Importantly, therapeutic inhibition of Jagged2-mediated omental metastasis was significantly improved by directly disrupting the Notch pathway in omental mesothelial cells. These findings highlight the key role of Jagged2 to the functional interplay between the TGF-β and the Notch signaling pathways during the metastatic process of ovarian cancer cells to the omentum and identify the Notch signaling molecule as a precision therapeutic target for ovarian cancer metastasis.
Topics: Female; Humans; Carcinoma, Ovarian Epithelial; Cell Line, Tumor; Neoplasm Metastasis; Ovarian Neoplasms; Peritoneal Neoplasms; Retroperitoneal Neoplasms; Signal Transduction; Transforming Growth Factor beta
PubMed: 38575576
DOI: 10.1038/s41419-024-06512-0 -
PloS One 2024Peritoneal sarcomatosis (PS) is a difficult entity to treat with limited options and guarded prognosis. We aimed to determine if the addition of hyperthermic...
Radical resection and hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of high risk recurrent retroperitoneal sarcoma-A pilot study in a tertiary Asian centre.
BACKGROUND
Peritoneal sarcomatosis (PS) is a difficult entity to treat with limited options and guarded prognosis. We aimed to determine if the addition of hyperthermic intraperitoneal chemotherapy (HIPEC) could offer superior local recurrence-free survival in patients with retroperitoneal sarcoma at high risk of developing PS as opposed to extended resection alone.
METHODS
This is a single arm, phase II intervention study where all patients with recurrent localized retroperitoneal sarcoma considered at high risk of developing PS were considered for enrolment (ClinicalTrials.gov identifier: NCT03792867). Upon enrolment, patients underwent vigorous preoperative testing to ensure fitness for the procedure. During surgery, patients underwent extended resection and HIPEC with doxorubicin. Patients were followed-up every 2 weeks (± 10 days) for the first month and subsequently every three months (± 1 month) up to a year post-surgery, and were assessed for potential chemotherapy toxicity and post-treatment complications. After a year from resection and HIPEC, patients were followed-up either during routine clinic review or contacted via telephone every year (± 1 month) for 3 years.
RESULTS
Six patients were recruited but one patient dropped out due to adverse and unexpected intraoperative events. The remaining patients completed the procedure uneventfully. Post-HIPEC, all patients recurred with a disease-free interval ranging from six to 24 months. Three patients died due to complications from recurrent disease whereas the remaining three patients are alive as of their last visit. The overall survival at time at reporting ranged between 22 to 56 months.
CONCLUSION
The procedure is feasible with no major morbidity to patients. However, we are unable to recommend for it to be implemented as a routine procedure at this current stage due to lack of improved survival outcomes. Further multi-institutional studies may be conducted to yield better results.
Topics: Humans; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Pilot Projects; Retroperitoneal Neoplasms; Sarcoma
PubMed: 38574044
DOI: 10.1371/journal.pone.0300594 -
Asian Journal of Surgery Jun 2024
Topics: Humans; Sarcoma, Ewing; Retroperitoneal Neoplasms; Male; Female; Tomography, X-Ray Computed
PubMed: 38565443
DOI: 10.1016/j.asjsur.2024.03.126 -
Journal of Vascular Surgery. Venous and... Jul 2024Primary vascular leiomyosarcomas are incredibly rare and have a poor prognosis. The purpose of this study was to analyze the surgical outcomes of patients with primary...
INTRODUCTION
Primary vascular leiomyosarcomas are incredibly rare and have a poor prognosis. The purpose of this study was to analyze the surgical outcomes of patients with primary inferior vena cava (IVC) leiomyosarcoma.
METHODS
We performed a retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, including 10 women and 3 men. The presenting symptoms, tumor characteristics, operative management, postoperative complications, and survival rates were assessed for each patient.
RESULTS
The median patient age was 59 years (quartile [Q]1, 52 years; Q3, 68 years). The median tumor size was 7.0 cm (Q1, 6 cm; Q3, 12 cm). The median mitotic rate was 6 per 10 high-power fields (Q1, 2.5; Q3, 15.5). All 13 patients underwent grossly negative tumor resection, with 9 (69%) having microscopically negative margins (R0). No patient had lymph node involvement. The IVCs were managed with ligation in four patients for tumors already occluding the IVC and bovine pericardial patch angioplasty in seven patients or primary repair in two patients for patent IVCs. Concomitant right nephrectomy was performed in seven patients. Left renal vein ligation was performed in three patients, but no left nephrectomies were performed. Significant postoperative complications included one patient with lower extremity compartment syndrome, two patients with severe leg swelling, and one patient with arm swelling. The 30-day mortality rate was zero. Using the Kaplan-Meier product limit method, disease-specific survival was estimated to be 93%.
CONCLUSIONS
Surgical resection is a feasible and effective oncologic treatment option for patients with IVC leiomyosarcoma. The IVC can be safely managed by ligation, primary repair, or patch angioplasty, depending on the prior patency of the IVC.
Topics: Humans; Leiomyosarcoma; Vena Cava, Inferior; Male; Middle Aged; Female; Retrospective Studies; Vascular Neoplasms; Aged; Treatment Outcome; Time Factors; Postoperative Complications; Risk Factors; Vascular Surgical Procedures; Nephrectomy; Ligation; Tumor Burden; Margins of Excision; Adult
PubMed: 38552955
DOI: 10.1016/j.jvsv.2024.101885 -
Medicine Mar 2024Retroperitoneal dedifferentiated liposarcoma (RPDDL) is an uncommon malignancy, which often remains undetected for many years due to having adequate space in the... (Review)
Review
RATIONALE
Retroperitoneal dedifferentiated liposarcoma (RPDDL) is an uncommon malignancy, which often remains undetected for many years due to having adequate space in the retroperitoneal cavity and lacking clinical manifestations in the early stage of the disease. Surgical procedure is usually used as the first choice for treatment. However, it is prone to local recurrence after the operation, resulting in an unfavorable prognosis. Our aim is to draw useful lessons from the new case and provide some experience for management of the disease.
PATIENT CONCERNS
We describe a 55-year-old male patient who was admitted for a 3-week history of persistent dull ache of the left waist. A large mass of the left upper abdomen was palpated in physical examination. Moreover, the imaging examination revealed that the diameter of the mass was about 21 cm, and some adjacent vital organs were invaded, which brought great challenges to complete surgical resection.
DIAGNOSIS
The postoperative pathological results confirmed that the mass was RPDDL with invasion of the surrounding vital structures including pancreas, spleen, left adrenal gland, left kidney, and vasculature with tumor emboli.
INTERVENTIONS
Surgical resection of the mass was performed by our multidisciplinary team. The patient received chemotherapy 1 month after surgery.
OUTCOMES
The effect of chemotherapy seemed to be unsatisfactory. Local multifocal recurrence of the tumor was considered about 2 months after surgery. Finally, he gave up any treatments and died of the disease.
LESSONS
Regular physical examination and ultrasound screening may detect the disease as early as possible, especially for high-risk group aged 60 to 70, which should be popularized. Incomplete resection, vascular invasion, and interruption of postoperative treatment may lead to an unfavorable prognosis. Therefore, we think that patients with the disease may benefit from complete surgical resection and uninterrupted adjuvant therapy.
Topics: Male; Humans; Middle Aged; Retroperitoneal Neoplasms; Retroperitoneal Space; Liposarcoma; Kidney
PubMed: 38552050
DOI: 10.1097/MD.0000000000037604 -
Frontiers in Oncology 2024Erdheim-Chester disease (ECD) is a rare disease that belongs to the group of Dendritic and histiocytic neoplasms. Only 2000 cases have been reported worldwide. It can...
INTRODUCTION
Erdheim-Chester disease (ECD) is a rare disease that belongs to the group of Dendritic and histiocytic neoplasms. Only 2000 cases have been reported worldwide. It can present with a wide range of symptoms, making a differential diagnosis especially difficult. The primary and most important diagnostic tool is a biopsy of the affected organ/tissue. Nowadays the analysis of different mutations affecting the BRAF and MAPK pathways makes it possible to use targeted treatments, such as vemurafenib, dabrafenib, or cobimetinib.
OBJECTIVE
Our aim is to present the results of three male patients treated in our hematology department.
RESULTS
Our BRAF mutation-positive patient presented with retroperitoneal tissue proliferation and diabetes insipidus. The initial therapy of choice was dabrafenib. After 3 months of treatment, F-fluoro-deoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT) scans showed regression, and after 2 years of treatment, no disease activity was detected. In our second patient, a recurrent febrile state (not explained by other reasons) and diabetes insipidus suggested the diagnosis. A femoral bone biopsy confirmed BRAF-negative ECD. The first-line therapy was interferon-alpha. After 3 months of treatment, no response was observed on FDG-PET/CT, and treatment with cobimetinib was started. The control FDG-PET/CT imaging was negative. Our third patient was evaluated for dyspnea, and a CT scan showed fibrosis with hilar lymphadenomegaly. A lung biopsy confirmed BRAF-negative ECD. We started treatment with interferon-alpha, but unfortunately, no improvement was observed. Second-line treatment with cobimetinib resulted in a partial metabolic response (PMR) according to control FDG-PET/CT.
CONCLUSIONS
Our results demonstrate that an appropriately chosen treatment can lead to a good therapeutic response, but dose reduction may be necessary due to side effects. With advanced targeted therapeutic treatment options, survival and quality of life are significantly improved.
PubMed: 38549927
DOI: 10.3389/fonc.2024.1305518 -
Medicina (Kaunas, Lithuania) Feb 2024: Our aim was to clarify the oncological outcomes of the two different approaches to laparoscopic nephroureterectomies (LNUs) in Japan, and to examine whether there were...
: Our aim was to clarify the oncological outcomes of the two different approaches to laparoscopic nephroureterectomies (LNUs) in Japan, and to examine whether there were any significant differences between the transperitoneal approach and the retroperitoneal approach. : We retrospectively evaluated patients who underwent an LNU for upper tract urothelial carcinoma (UTUC) from January 2013 to December 2022. We identified 52 patients who underwent a transperitoneal LNU (tLNU) and 93 who underwent a retroperitoneal LNU (rLNU). We adopted age, smoking, and pT-stage matching, and 43 patients were classified in each group. We investigated the time from surgery to recurrence (RFS: recurrence-free survival), the time to death (OS: overall survival), and the time to non-urothelial-tract recurrence-free survival (NUTRFS). A Cox regression analysis was performed to evaluate the risk factors that influenced recurrence. : There were no significant differences in the RFS, OS, and NUTRFS between the two matched groups. In the multivariate Cox regression analysis, the pT stage (pT3≥ vs. pT2≤) had an HR = 2.09 and a = 0.01, and was an independent prognostic risk factor regarding cancer recurrence. : There were no significant differences in the oncological outcomes between the tLNU and rLNU groups. It is suggested that the transperitoneal approach should be selected for LNUs.
Topics: Humans; Nephroureterectomy; Carcinoma, Transitional Cell; Treatment Outcome; Urinary Bladder Neoplasms; Retrospective Studies; Ureteral Neoplasms; Nephrectomy; Laparoscopy; Neoplasm Recurrence, Local
PubMed: 38541147
DOI: 10.3390/medicina60030421 -
Rare Tumors 2024The existence of both neuroendocrine and non-neuroendocrine histology in variable proportion in a lesion has been described by the World Health Organisation (WHO) as...
The existence of both neuroendocrine and non-neuroendocrine histology in variable proportion in a lesion has been described by the World Health Organisation (WHO) as mixed neuroendocrine and non-neuroendocrine neoplasm (MiNEN). The pathogenesis of this tumour remains controversial but molecular studies point towards a common monoclonal origin. Tumours are classified as functioning and nonfunctioning based on substances secreted. The nonfunctioning tumours may be discovered due to its local effect. Presented is a 66-year-old male with an intra-abdominal mass, underwent laparotomy and excision biopsy with transient right lower limb lymphoedema. Histology confirmed retroperitoneal MiNEN with no evidence of tumour recurrence 12 months following surgery. MiNENs should be considered as a differential diagnosis in patients with intra-abdominal mass. Surgical resection is recommended as this may offer the best treatment option.
PubMed: 38525086
DOI: 10.1177/20363613241242383