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The Journal of Clinical Endocrinology... Apr 2021Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas... (Review)
Review
CONTEXT
Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas will exhibit metastatic behavior.
EVIDENCE ACQUISITION
Extensive searches in the PubMed database with various combinations of the key words pheochromocytoma, paraganglioma, metastatic, malignant, diagnosis, pathology, genetic, and treatment were the basis for the present review.
DATA SYNTHESIS
To pinpoint metastatic potential in PPGLs is difficult, but nevertheless crucial for the individual patient to receive tailor-made follow-up and adjuvant treatment following primary surgery. A combination of histological workup and molecular predictive markers can possibly aid the clinicians in this aspect. Most patients with PPGLs have localized disease and may be cured by surgery. Plasma metanephrines are the main biochemical tests. Genetic testing is important, both for counseling and prognostic estimation. Apart from computed tomography and magnetic resonance imaging, molecular imaging using 68Ga-DOTATOC/DOTATATE should be performed. 123I-MIBG scintigraphy may be performed to determine whether 131I-MIBG therapy is a possible option. As first-line treatment in patients with metastatic disease, 177Lu-DOTATATE or 131I-MIBG is recommended, depending on which shows best expression. In patients with very low proliferative activity, watch-and-wait or primary treatment with long-acting somatostatin analogues may be considered. As second-line treatment, or first-line in patients with high proliferative rate, chemotherapy with temozolomide or cyclophosphamide + vincristine + dacarbazine is the therapy of choice. Other therapies, including sunitinib, cabozantinib, everolimus, and PD-1/PDL-1 inhibitors, have shown modest effect.
CONCLUSIONS
Metastatic PPGLs need individualized management and should always be discussed in specialized and interdisciplinary tumor boards. Further studies and newer treatment modalities are urgently needed.
Topics: Abdominal Neoplasms; Adrenal Gland Neoplasms; Animals; Humans; Paraganglioma; Pheochromocytoma
PubMed: 33462603
DOI: 10.1210/clinem/dgaa982 -
Journal of Clinical Oncology : Official... Aug 2022The peritoneum is a common site of metastasis in advanced gastric cancer (GC). Diagnostic laparoscopy is now routinely performed as part of disease staging, leading to... (Review)
Review
The peritoneum is a common site of metastasis in advanced gastric cancer (GC). Diagnostic laparoscopy is now routinely performed as part of disease staging, leading to an earlier diagnosis of synchronous peritoneal metastasis (PM). The biology of GCPM is unique and aggressive, leading to a dismal prognosis. These tumors tend to be resistant to traditional systemic therapy, and yet, this remains the current standard-of-care recommended by most international clinical guidelines. As this is an area of unmet clinical need, several translational studies and clinical trials have focused on addressing this specific disease state. Advances in genomic sequencing and molecular profiling have revealed several promising therapeutic targets and elucidated novel biology, particularly on the role of the surrounding tumor microenvironment in GCPM. Peritoneal-specific clinical trials are being designed with a combination of locoregional therapeutic strategies with systemic therapy. In this review, we summarize the new knowledge of cancer biology, advances in surgical techniques, and emergence of novel therapies as an integrated strategy emerges to address GCPM as a distinct clinical entity.
Topics: Genomics; Humans; Neoplasm Staging; Peritoneal Neoplasms; Peritoneum; Stomach Neoplasms; Tumor Microenvironment
PubMed: 35649219
DOI: 10.1200/JCO.21.02745 -
EBioMedicine Jun 2023Peritoneal metastasis is a challenging aspect of clinical practice for gastric cancer. Animal models are crucial in understanding molecular mechanisms, assessing drug... (Review)
Review
Peritoneal metastasis is a challenging aspect of clinical practice for gastric cancer. Animal models are crucial in understanding molecular mechanisms, assessing drug efficacy, and conducting clinical intervention studies, including those related to gastric cancer peritoneal metastasis. Unlike other xenograft models, peritoneal metastasis models should not only present tumor growth at the transplant site, but also recapitulate tumor cell metastasis in the abdominal cavity. Developing a reliable model of gastric cancer peritoneal metastasis involves several technical aspects, such as the selection of model animals, source of xenograft tumors, technology of transplantation, and dynamic monitoring of the tumor progression. To date, challenges remain in developing a reliable model that can completely recapitulate peritoneal metastasis. Thus, this review aims to summarize the techniques and strategies used to establish animal models of gastric cancer peritoneal metastasis, providing a reference for future model establishment.
Topics: Animals; Humans; Peritoneal Neoplasms; Stomach Neoplasms; Peritoneum; Transplantation, Heterologous; Heterografts; Cell Line, Tumor
PubMed: 37182268
DOI: 10.1016/j.ebiom.2023.104601 -
Molecular Cancer Jun 2023Clinical hyperthermic intraperitoneal chemotherapy (HIPEC) is regarded as a potential treatment that can prolong survival of patients with peritoneal metastases after...
Clinical hyperthermic intraperitoneal chemotherapy (HIPEC) is regarded as a potential treatment that can prolong survival of patients with peritoneal metastases after cytoreductive surgery. However, treated tumor cells are prone to becoming heat resistant to HIPEC therapy through high expression of heat shock proteins (HSPs). Here, a carrier-free bifunctional nanoinhibitor was developed for HIPEC therapy in the management of peritoneal metastases. Self-assembly of the nanoinhibitor was formed by mixing Mn ion and epigallocatechin gallate (EGCG) in a controllable manner. Such nanoinhibitor directly inhibited HSP90 and impaired the HSP90 chaperone cycle by reduced intracellular ATP level. Additionally, heat and Mn ion synergistically induced oxidative stress and expression of caspase 1, which activated GSDMD by proteolysis and caused pyroptosis in tumor cells, triggering immunogenic inflammatory cell death and induced maturation of dendritic cells through the release of tumor antigens. This strategy to inhibit heat resistance in HIPEC presented an unprecedented paradigm for converting "cold" tumors into "hot" ones, thus significantly eradicating disseminated tumors located deep in the abdominal cavity and stimulating immune response in peritoneal metastases of a mouse model. Collectively, the nanoinhibitor effectively induced pyroptosis of colon tumor cells under heat conditions by inhibiting heat stress resistance and increasing oxidative stress, which may provide a new strategy for treatment of colorectal peritoneal metastases.
Topics: Animals; Mice; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; HSP90 Heat-Shock Proteins; Proteolysis; Colon
PubMed: 37316830
DOI: 10.1186/s12943-023-01790-2 -
Current Oncology (Toronto, Ont.) Apr 2023Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease... (Review)
Review
Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease and in the context of a multidisciplinary team of sarcoma specialists. For primary RPS, the goal of surgery is to achieve the complete en bloc resection of the tumor along with involved organs and structures to maximize the clearance of the disease. The extent of resection also needs to consider the risk of complications. Unfortunately, the overarching challenge in primary RPS treatment is that even with optimal surgery, tumor recurrence occurs frequently. The pattern of recurrence after surgery (e.g., local versus distant) is strongly associated with the specific histologic type of RPS. Radiation and systemic therapy may improve outcomes in RPS and there is emerging data studying the benefit of non-surgical treatments in primary disease. Topics in need of further investigation include criteria for unresectability and management of locally recurrent disease. Moving forward, global collaboration among RPS specialists will be key for continuing to advance our understanding of this disease and find more effective treatments.
Topics: Humans; Neoplasm Recurrence, Local; Sarcoma; Treatment Outcome; Retroperitoneal Neoplasms; Soft Tissue Neoplasms
PubMed: 37232807
DOI: 10.3390/curroncol30050349 -
Abdominal Radiology (New York) Aug 2022Involvement of the abdomen and pelvis is common in lymphoma. Nodal and extranodal abdominal and pelvic lymphoma may present with various complications. Complications are... (Review)
Review
Involvement of the abdomen and pelvis is common in lymphoma. Nodal and extranodal abdominal and pelvic lymphoma may present with various complications. Complications are most common in high-grade lymphomas, especially diffuse large B-cell lymphoma. Complications may occur as the initial manifestation of lymphoma, during treatment course, or late following complete disease remission. Most complications are associated with worse prognosis and increased mortality. Imaging is essential in evaluation of disease extent and diagnosis of complications. Therefore, radiologists should be familiar with the clinical context and imaging features of abdominal and pelvic lymphoma complications. We provide a comprehensive, organ system-based approach, and clinical and imaging review of complications of abdominal and pelvic lymphoma along with radiologic images of illustrated cases of the most commonly encountered complications.
Topics: Abdomen; Abdominal Neoplasms; Humans; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Non-Hodgkin; Pelvis; Prognosis
PubMed: 35690955
DOI: 10.1007/s00261-022-03567-5 -
Journal of Surgical Oncology Jun 2022Retroperitoneal and abdominopelvic sarcomas are rare heterogeneous malignancies. The only therapy proven to improve disease-free survival (DFS) is R0/R1 surgical...
BACKGROUND AND OBJECTIVES
Retroperitoneal and abdominopelvic sarcomas are rare heterogeneous malignancies. The only therapy proven to improve disease-free survival (DFS) is R0/R1 surgical resection. We sought to analyze whether additional factors such as radiation and systemic therapy were associated with DFS and abdominal recurrence-free survival (RFS).
METHODS
Retrospective review of adults (≥18) with resectable abdominopelvic and retroperitoneal sarcomas who underwent intent-to-cure surgery at a high-volume tertiary referral center between 1998 and 2015. The main outcome measures were DFS and abdominal RFS.
RESULTS
Overall, 159 patients met the criteria for inclusion. Median follow-up was 4.8 years (range 0.1-18.9 years). The most common histology was liposarcoma (49%). Systemic therapy was administered to 48% of patients and was not associated with improved outcomes. The neoadjuvant radiotherapy group (11%) had improved adjusted DFS (5.46 years, 95% CI [3.68, 7.24] vs. 3.1 years, 95% CI [2.48, 3.73]) and abdominal RFS (6.14 years, 95% CI [4.38, 7.89] vs. 3.22 years, 95% CI [2.61, 3.84]). The adjuvant radiotherapy group (19%) had no improvement.
CONCLUSIONS
In a cohort of patients undergoing resection for retroperitoneal or abdominopelvic sarcoma, neoadjuvant radiation improved DFS and abdominal RFS. A follow-up of over three years was needed to appreciate a difference in outcomes.
Topics: Adult; Disease-Free Survival; Humans; Liposarcoma; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms
PubMed: 35239187
DOI: 10.1002/jso.26828 -
The British Journal of Surgery Dec 2021This study aimed to determine the impact of pulmonary complications on death after surgery both before and during the severe acute respiratory syndrome coronavirus 2... (Comparative Study)
Comparative Study
BACKGROUND
This study aimed to determine the impact of pulmonary complications on death after surgery both before and during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic.
METHODS
This was a patient-level, comparative analysis of two, international prospective cohort studies: one before the pandemic (January-October 2019) and the second during the SARS-CoV-2 pandemic (local emergence of COVID-19 up to 19 April 2020). Both included patients undergoing elective resection of an intra-abdominal cancer with curative intent across five surgical oncology disciplines. Patient selection and rates of 30-day postoperative pulmonary complications were compared. The primary outcome was 30-day postoperative mortality. Mediation analysis using a natural-effects model was used to estimate the proportion of deaths during the pandemic attributable to SARS-CoV-2 infection.
RESULTS
This study included 7402 patients from 50 countries; 3031 (40.9 per cent) underwent surgery before and 4371 (59.1 per cent) during the pandemic. Overall, 4.3 per cent (187 of 4371) developed postoperative SARS-CoV-2 in the pandemic cohort. The pulmonary complication rate was similar (7.1 per cent (216 of 3031) versus 6.3 per cent (274 of 4371); P = 0.158) but the mortality rate was significantly higher (0.7 per cent (20 of 3031) versus 2.0 per cent (87 of 4371); P < 0.001) among patients who had surgery during the pandemic. The adjusted odds of death were higher during than before the pandemic (odds ratio (OR) 2.72, 95 per cent c.i. 1.58 to 4.67; P < 0.001). In mediation analysis, 54.8 per cent of excess postoperative deaths during the pandemic were estimated to be attributable to SARS-CoV-2 (OR 1.73, 1.40 to 2.13; P < 0.001).
CONCLUSION
Although providers may have selected patients with a lower risk profile for surgery during the pandemic, this did not mitigate the likelihood of death through SARS-CoV-2 infection. Care providers must act urgently to protect surgical patients from SARS-CoV-2 infection.
Topics: Abdominal Neoplasms; Aged; COVID-19; Cohort Studies; Elective Surgical Procedures; Female; Humans; Male; Middle Aged; Pandemics; Postoperative Complications; Respiration, Artificial; Respiratory Distress Syndrome
PubMed: 34871379
DOI: 10.1093/bjs/znab336 -
Radiographics : a Review Publication of... 2020Abdominal wall masses, masslike lesions, and diffuse processes are common and often incidental findings at cross-sectional imaging. Distinguishing among these types of... (Review)
Review
Abdominal wall masses, masslike lesions, and diffuse processes are common and often incidental findings at cross-sectional imaging. Distinguishing among these types of masses on the basis of imaging features alone can be challenging. The authors present a diagnostic algorithm that may help in distinguishing different types of abdominal wall masses accurately. Hernias may mimic discrete masses at clinical examination, and imaging is often ordered for evaluation of a possible abdominal wall mass. Once a discrete mass is confirmed to be present, the next step is to determine if it is a fat-containing, cystic, or solid mass. The most common fat-containing masses are lipomas. Fluid or cystic masses include postoperative abscesses, seromas, and rectus sheath hematomas. Solid masses are the most common abdominal wall masses and include desmoid tumors, sarcomas, endometriomas, and metastases. Multiple masses and other diffuse abdominal wall processes are often manifestations of an underlying condition or insult. The most frequently found diffuse processes are multiple injection granulomas from administration of subcutaneous medication. This article offers an algorithmic approach to characterizing abdominal wall masses on the basis of their composition and reviews abdominal wall diffuse processes. RSNA, 2020.
Topics: Abdominal Neoplasms; Abdominal Wall; Cysts; Diagnosis, Differential; Endometriosis; Female; Hematoma; Hernia, Abdominal; Humans; Incidental Findings; Lipoma; Male; Postoperative Complications; Seroma; Soft Tissue Neoplasms
PubMed: 32330085
DOI: 10.1148/rg.2020190170 -
BMJ Case Reports Jun 2020There are less than 150 cases of non-functioning retroperitoneal abdominal schwannoma (NRS) reported. Hormonal assay is crucial in confirming the diagnosis of NRS, as...
There are less than 150 cases of non-functioning retroperitoneal abdominal schwannoma (NRS) reported. Hormonal assay is crucial in confirming the diagnosis of NRS, as manipulation of a functional retroperitoneal paraganglioma will instigate an abrupt liberation of catecholamines, resulting in devastating consequences. We report the case of 42-year-old woman who presented with headache, night sweats and abdominal discomfort. Cross-sectional imaging demonstrated a retroperitoneal mass adherent to the aorta and inferior vena cava but biochemical testing of blood and urine was negative for metanephrines and normetanephrines. She underwent successful tumour resection via laparotomy, as location increased the complexity and risk of laparoscopic resection.
Topics: Abdominal Neoplasms; Adult; Female; Humans; Neurilemmoma; Retroperitoneal Neoplasms
PubMed: 32554461
DOI: 10.1136/bcr-2019-233371