Huge intermediate-grade myofibroblastic sarcoma in the retroperitoneum revealed by 18F-FDG PET/CT: a case report.

Myofibroblastic sarcoma (MS) is a relatively rare malignant bone and soft tissue tumor, which originates from myofibroblasts, with some characteristics of both smooth muscle cells and fibroblasts. It can develop in individuals at any age and can affect various regions, especially the head and neck; however, it is rarely reported retroperitoneally. Generally, this type of sarcoma is considered a low-grade malignancy, and cases classified as moderate and high-grade malignancy are rare. In this study, we describe a case of intermediate-grade myofibroblastic sarcoma (IGMS) originating from the retroperitoneum, which was confirmed through pathological diagnosis. The 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) scan revealed a large, borderless mass located retroperitoneally with a significantly increased 18F-FDG uptake, accompanied by adjacent visceral and soft tissue infiltration and peripheral lymph node metastasis. The patient received chemotherapy for 3 weeks; however, the tumor did not shrink significantly. Therefore, the patient discontinued the treatment. After 5 months, his condition gradually deteriorated, which eventually led to death. Through this case report, the diagnosis and treatment of moderate malignant retroperitoneal myofibroblastoma were discussed, aiming to increase clinicians' understanding of this disease.

Adrenal Schwannoma Presenting as an Incidentaloma in a Patient with Uterine Fibroids and Cholelithiasis: a Rare Case Report.

Schwannomas, which are benign mesenchymal tumors derived from Schwann cells, are common in the central nervous system. While they are commonly seen in the extremities and head-neck area, their presence in visceral organs, particularly the adrenals, is uncommon. Adrenal schwannomas are frequently discovered incidentally, offering a diagnostic difficulty because of their uncommon presentation. A 46-year-old woman initially sought treatment for symptoms related to uterine fibroids and biliary stones. Diagnostic imaging uncovered an adrenal incidentaloma, necessitating a laparoscopic right adrenalectomy. The mass was determined to be an adrenal schwannoma based on its spindle-shaped cells and S-100 immunohistochemistry positivity. The patient's symptoms improved, and she was discharged with stable vital signs. Preoperative diagnosis of adrenal schwannomas is difficult and requires histological confirmation. When diagnosing non-secreting adrenal tumors with unusual radiology, surgeons should investigate for schwannoma. Post-resection adrenal schwannoma follow-up studies are scarce; however, they imply a low risk of recurrence or metastasis.

Lipoleiomyosarcoma Presenting as Massive Retroperitoneal Mass: An Unusual Soft Tissue Tumor of Abdomen and its Review of Literature.

Lipoleiomyosarcoma is a malignant tumor of both mesenchymal and smooth muscle origin. Lipoleiomyosarcoma is a very rare type of retroperitoneal sarcoma, which by itself is a rare type of soft tissue sarcoma. Lipoleiomyosarcoma is a very rare subtype of liposarcoma, which is the most common type of retroperitoneal sarcoma. To our best review of available literature, this was the first case of lipoleiomyosarcoma presenting as a massive retroperitoneal mass of size 50 × 50 × 30 cm, managed with multi-visceral R0 resection. The computed tomography of the abdomen showed a massive fat density lesion with internal soft density lesions and internal calcifications. Multivisceral resection was planned and executed and achieved R0 resection. We suggest that lipoleiomyosarcoma should be included in differential diagnosis of retroperitoneal tumors, and multi-visceral R0 resection should be considered as an option for lipoleiomyosarcoma, as these tumors have very good prognosis with R0 resection.

Eleven-Year Experience With Midline Extraperitoneal Retroperitoneal Lymph Node Dissection for Germ Cell Tumors.

PURPOSE: A midline extraperitoneal approach for retroperitoneal lymph node dissection (EP-RPLND) has been associated with decreased morbidity compared to the transperitoneal approach. We aimed to review our 11-year experience in patients with germ cell tumors (GCTs) who underwent EP-RPLND at a single institution. MATERIALS AND METHODS: All patients with GCT who underwent EP-RPLND between 2010 and 2021 were reviewed. Surgical, perioperative, and oncologic outcomes were reported. A logistic regression model was developed to evaluate variables predictive of early discharge. Oncologic outcomes included 2-year recurrence-free survival (RFS) and recurrence patterns, which were analyzed according to pathology. RESULTS: Overall, 237 patients underwent EP-RPLND, of which 72% were administered in the postchemotherapy (PC) setting. Median follow-up was 16.7 months (interquartile range [IQR] 3.9-39.6). Median size of retroperitoneal disease was 2.8 cm (IQR 1.8-5.4), of which 16 cases were ≥ 10 cm. There were no cases of postoperative ileus or readmission due to small-bowel obstruction. Median hospital stay was 2 days (IQR 1-3). From 2020 to 2021, 73% of patients were discharged on postoperative day 1 and 89% by postoperative day 2. Thirty-one complications occurred, including 4% grade III to IV complications. In the primary setting, 2-year RFS for seminoma and nonseminomatous GCT was 0.93 (95% CI 0.84-1.00) and 0.85 (95% CI 0.72-1.00), respectively. In the PC setting, 2-year RFS for seminoma and nonseminomatous GCT was 0.88 (95% CI 0.74-1.00) and 0.88 (95% CI 0.81-0.95), respectively. Overall, only 7 patients had in-field recurrence. CONCLUSIONS: Midline EP-RPLND is safe and associated with rapid gastrointestinal recovery, short hospital stay, and low complication rates. It also demonstrates acceptable oncologic outcomes in the primary and PC settings, with low rates of in-field relapse.

Mesenchymal Tumor Management: Integrating Surgical and Non-Surgical Strategies in Different Clinical Scenarios.

Mesenchymal tumors originate from mesenchymal cells and can be either benign or malignant, such as bone, soft tissue, and visceral sarcomas. Surgery is a cornerstone treatment in the management of mesenchymal tumors, often requiring complex procedures performed in high-volume referral centers. However, the COVID-19 pandemic has highlighted this need for alternative non-surgical approaches due to limited access to surgical resources. This review explores the role of non-surgical treatments in different clinical scenarios: for improving surgical outcomes, as a bridge to surgery, as better alternatives to surgery, and for non-curative treatment when surgery is not feasible. We discuss the effectiveness of active surveillance, cryoablation, high-intensity focused ultrasound, and other ablative techniques in managing these tumors. Additionally, we examine the use of tyrosine kinase inhibitors in gastrointestinal stromal tumors and hypofractionated radiotherapy in soft tissue sarcomas. The Sarculator tool is highlighted for its role in stratifying high-risk sarcoma patients and personalizing treatment plans. While surgery remains the mainstay of treatment, integrating advanced non-surgical strategies can enhance therapeutic possibilities and patient care, especially in specific clinical settings with limitations. A multidisciplinary approach in referral centers is vital to determine the optimal treatment course for each patient.

Advancing treatment efficacy: combined therapy of eribulin, anlotinib, and camrelizumab in advanced or metastatic retroperitoneal liposarcoma.

Background: Retroperitoneal liposarcoma (RLPS) typically shows limited response to standard chemotherapy, presenting a challenge in treating advanced or metastatic RLPS. Objective: This study aimed to evaluate the potential advantages of a combined therapeutic strategy utilizing eribulin, anlotinib, and camrelizumab. Design: Between December 2020 and March 2023, this retrospective study enrolled patients with advanced or metastatic RLPS who received treatment at Peking University Cancer Hospital Sarcoma Center. The treatment regimen involved eribulin plus anlotinib and camrelizumab administered every 3 weeks (Q3W). Methods: Efficacy was assessed following the Response Evaluation Criteria in Solid Tumors version 1.1, while safety was evaluated using the Common Terminology Criteria for Adverse Events version 5.0. Results: The study included 47 patients with RLPS with a median age of 55.5 years. Patients received a median of 4.5 (range, 2-21) cycles of treatment. Notably, partial response was observed in 8 patients (18.2%), while 25 (56.8%) exhibited stable disease. The objective response rate (ORR) and disease control rate were 18.2% and 75%, respectively. Significant differences in ORR were observed among histological subtypes (well-differentiated vs de-differentiated vs myxoid: 0 vs 17.9% vs 50%; p = 0.039). Six patients underwent surgery before disease progression, and one patient with myxoid liposarcoma (MLPS) had a pathological complete response. With a median follow-up of 21.8 (range, 2.7-30.7) months, the median progression-free survival (mPFS) was 6.9 (95% confidence interval (CI), 4.7-9.1) months, and the 6-month PFS rate was 60.5%. Based on various histological subtypes, the mPFS was 8.4 (95% CI, 4.1-12.7) months with well-differentiated liposarcoma, 5.8 (95% CI, 3.3-8.3) months with de-differentiated liposarcoma and not reached with MLPS, respectively. Treatment-related adverse events (TRAEs) of any grade occurred in 36 (76.6%) patients, with grade 3 or higher TRAEs in 21 (44.7%) patients. The most common TRAEs were neutropenia (53.2%), proteinuria (21.3%), and anorexia (21.3%). Conclusion: The combined treatment strategy involving eribulin, anlotinib, and camrelizumab showed promising efficacy and manageable safety in patients with advanced or metastatic RLPS, particularly in those with MLPS.

Retroperitoneal cystic lymphangioma coexisting with a uterine fibroid in a 42-year-old woman: A case report.

Lymphangiomas are rare benign neoplasms traditionally thought to result from congenital lymphatic channel malformations, though they may also be associated with other conditions. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and fewer than 200 cases have been reported. A 42-year-old woman was admitted with symptoms of abdominal pain and distension. A computerized tomography (CT) scan showed an abdomino-pelvic mass and a giant uterine myoma. The patient underwent explorative laparotomy and the whole cyst mass was removed along with the uterine myoma. Cystic lymphangiomas are often misdiagnosed because of the vague symptoms and the absence of obvious etiology. A provisional diagnosis can be made with CT but histological examination confirms the diagnosis. Cystic lymphangioma should be included in the differential diagnosis of an ovarian cystic mass. Complete resection can be curative.

Retroperitoneal vs. Transperitoneal Approach for Non-Ruptured Open Conversion after Endovascular Aneurysm Repair.

OBJECTIVE: Several studies comparing the transperitoneal (TP) and retroperitoneal (RP) approach for AAA repair suggest that the RP approach may result in lower rates of perioperative mortality and morbidity. However, data comparing these approaches for open conversion are lacking. This study aims to evaluate the association between the type of approach and outcomes following open conversion after EVAR. METHODS: We included all patients who underwent open conversion after EVAR between 2010-2022 in Vascular Quality Initiative (VQI). Patients presenting with rupture were excluded. The primary outcome was perioperative mortality. The secondary outcomes included perioperative complications and 5-year mortality. Inverse probability weighting (IPW) was used to adjust for factors with statistical or clinical significance. Logistic regression was used to assess perioperative mortality and complications in the weighted cohort. 5-year mortality was evaluated using Kaplan-Meier and Cox regression. RESULTS: We identified 660 patients (39% RP) who underwent open conversion after EVAR. Compared with TP, RP patients were older (75 [IQR, 70-79] years vs. 73.5 [IQR, 68-79] years, p<.001), and more frequently had prior myocardial infarction (33% vs. 22%, p=.002). Compared with the TP approach, the RP approach was less frequently used in case of associated iliac aneurysm (19% vs. 27%, p=.026), but more frequently with associated renal bypass (7.8% vs. 1.7%, p<.001) and by high volume physicians (highest quintile, >7 AAA annually: 41% vs. 17%, p<.001) and in high volume centers (highest quintile, >35 AAA annually: 36% vs. 20%, p<.001). RP patients, compared with TP patients, were less likely to have external iliac or femoral distal anastomosis (8.2% vs. 21%, p<.001), and an infrarenal clamp (25% vs. 36%, p<.001). Unadjusted perioperative mortality was not significantly different between approaches (RP vs. TP: 3.8% vs. 7.5%; p=.077). After risk adjustment, RP patients had similar odds of perioperative mortality (aOR, 0.49; 95%CI, 0.22-1.10; p=.082), and lower odds of intestinal ischemia (aOR, 0.26; 95%CI, 0.08-0.86; p=.028) and in-hospital reintervention (aOR, 0.43; 95%CI, 0.22-0.85; p=.015). No significant differences were found in the other perioperative complications, and 5-year mortality (aHR, 0.79; 95%CI, 0.47-1.32; p=.37). CONCLUSIONS: - Our findings suggest that the RP approach may be associated with lower adjusted odds of perioperative complications compared with the TP approach. The RP approach should be considered for open conversion after EVAR when feasible.

Comparison of Superselective Renal Artery Embolization versus Retroperitoneal Laparoscopic Partial Nephrectomy in Ruptured Hemorrhagic Renal Angiomyolipoma: A Single-Center Study.

PURPOSE: To analyze the clinical efficacy of superselective renal artery embolization and retroperitoneal laparoscopic partial nephrectomy for the treatment of ruptured hemorrhagic renal angiomyolipoma and to provide a reference for the selection of treatment methods for ruptured hemorrhagic renal angiomyolipoma. METHODS: A retrospective analysis was conducted on the clinical data of 24 patients who were diagnosed with ruptured hemorrhagic renal angiomyolipoma at the Second Hospital of Tianjin Medical University between January 2019 and December 2021. Among them, 10 patients were treated with superselective arterial embolization (SAE), and 14 patients were treated with retroperitoneal laparoscopic part nephrectomy (RLPN). The differences between the two treatment methods in terms of hospital stay, hospital costs, anesthesia method, operation time, intraoperative blood loss, postoperative bed rest time, antibiotic dosage, postoperative complication rate, tumor diameter changes, creatinine value changes, hemoglobin value changes, tumor recurrence rate, and reoperation rate were compared. RESULTS: All patients completed the treatment and were discharged. There were no significant differences in length of hospital stay, hospital costs, creatinine change values, or postoperative complication rates between the two groups (p > 0.05). However, there were statistically significant differences (p < 0.05) in surgical time (85.50 ± 19.94 min vs. 141.07 ± 76.33 min), intraoperative blood loss (21.50 ± 14.72 mL vs. 153.57 ± 97.00 mL), postoperative bed rest time (22.7 ± 1.56 h vs. 41.21 ± 3.57 h), preoperative hemoglobin levels (94.7 ± 23.62 g/L vs. 113.79 ± 17.83 g/L), and hemoglobin changes (-6.60 ± 10.36 g/L vs. -15.21 ± 8.79 g/L) between the two groups. Both groups of patients had an average follow-up period of 22 months, and patients in the SAE group had a mean reduction of 3.33 cm in tumor diameter within the follow-up period compared with the pre-embolization period (p < 0.05). None of the patients in the SAE group experienced rebleeding, and there was no tumor recurrence in either group. CONCLUSION: SAE and RLPN are effective treatments for ruptured renal angiomyolipoma with good outcomes. Furthermore, compared to RLPN, SAE offers advantages such as simplicity of operation, minimal trauma, shorter surgical time, minimal impact on hemoglobin levels, shorter bed rest time, faster postoperative recovery, and maximal preservation of renal units.

Challenges in Diagnosis and Management: A Case of Abdominal Wall Liposarcoma.

This case report presents the rare occurrence of a large dedifferentiated liposarcoma originating from the abdominal wall in a 57-year-old male. The patient's initial complaint was the gradual development of an abdominal mass over six months without associated urinary or digestive symptoms. Clinical examination revealed a firm, non-mobile mass in the left lumbar region, prompting further investigation. Imaging studies confirmed the presence of a sizable soft tissue mass with calcifications, suggestive of a sarcoma. Preoperative biopsy indicated a malignant mesenchymal tumor, leading to surgical intervention. Intraoperative findings revealed characteristics consistent with a low-grade malignancy, prompting complete tumor resection with flap reconstruction. Subsequent histopathological analysis confirmed the diagnosis of dedifferentiated liposarcoma with negative surgical margins. The patient was referred for palliative chemotherapy due to the aggressive nature of the tumor. This case underscores the diagnostic challenges and therapeutic considerations associated with rare abdominal wall liposarcomas, emphasizing the importance of a multidisciplinary approach in their management.

A rare case of giant pelvic retroperitoneal schwannoma.

This report highlights the successful treatment of large pelvic schwannomas and underscores the importance of preoperative embolization. A 40-year-old male presented with a lower abdominal mass and reported pain and numbness in the left lower limb attributed to nerve compression. Preoperative embolization of the main feeding vessels was performed to mitigate intraoperative bleeding. The tumor was completely excised during surgery, with preservation of the hypogastric nerve. Histopathological examination confirmed the diagnosis of schwannoma. We underscore the significant role of preoperative embolization in effectively managing large pelvic tumors, such as schwannomas, by reducing the risk of excessive bleeding.

Literature review and robotic management of a rare case of primary retroperitoneal mucinous cystadenoma.

Primary retroperitoneal mucinous cystic tumors (PRMCT) are divided into 3 groups: benign, borderline malignancy, and malignant. We report a rare case of benign retroperitoneal mucinous cystadenoma of a 59-year-old Caucasian female who presented to our clinic with moderate intermittent left upper quadrant abdominal pain for several months, accompanied by early satiety, and unintentional weight loss of 10 pounds. An abdominal contrast-enhanced computed tomography (CT) scan indicated the presence of a 6.5 × 8.8 cm multilobulated mass in the left upper quadrant, characterized by a homogenous appearance with smooth margins. Upper endoscopic ultrasound and fine needle aspiration were performed. Cytology and histology results yielded rare inflammatory cells and debris with no cytological evidence of malignancy. The case was discussed at the hepatopancreatobiliary conference; the patient underwent a robotic resection of the mass with a gastric rim. Primary retroperitoneal mucinous cystadenomas are commonly managed by complete surgical excision. Robotic excision presents an alternative approach for handling this uncommon neoplastic lesion.

Localized retroperitoneal mass suspected malignancy: A rare case of unicentric Castleman's disease.

Key Clinical Message: Castleman's Disease should be considered in the differential diagnosis of retroperitoneal masses, especially in equivocal cases. Clinician should not presume all cases of retroperitoneal masses as a malignancy. Abstract: Castleman's Disease is a heterogeneous group of lymphoproliferative disorders, that can develop in lymph nodes or in extranodal sites. It has three distinct histological subtypes; hyaline vascular, plasma cell or mixed. It can be unicentric or multicentric, and sometimes oligocentric or regional. In this article, we report a case of a 30-year-old male who presented with a palpable left lumbar mass, clinically suspected as sarcoma vs GIST, which was surgically excised and pathologically examined revealing a rare condition of intra-abdominal unicentric Castleman's Disease with good prognosis. Castleman's Disease should be considered in the differential diagnosis of retroperitoneal masses, especially in equivocal cases.

Clinical features and outcomes of retroperitoneal unicentric Castleman disease resected as sarcomas: insights from a high-volume sarcoma center.

Background: Castleman disease (CD) is a rare lymphoproliferative disorder that can occur anywhere along the lymphatic pathway. Retroperitoneal unicentric Castleman disease (UCD) is an extremely rare manifestation. This study aims to explore the clinical features and surgical treatment of retroperitoneal UCD. Methods: We retrospectively reviewed patients who underwent retroperitoneal tumor surgery and were diagnosed with CD based on postoperative pathology before December 31, 2022. Data from these patients were collected and analyzed. Results: A total of 15 patients were included in the final analysis. All patients underwent radical resection under general anesthesia. Two out of 15 patients (13.3%) experienced serious complications but recovered well. There were no perioperative deaths. The median follow-up time was 78.5 months (range: 18-107.5 months), and no deaths or recurrences occurred during this period. Conclusions: Surgical treatment for retroperitoneal UCD is safe. Patients with retroperitoneal UCD can achieve long-time survival through complete resection.

The Use of Palliative Therapy in Patients With Advanced Retroperitoneal Sarcoma.

INTRODUCTION: Advanced retroperitoneal sarcoma (RPS) can include unresectable locoregional disease, systemic or multifocal intra-abdominal metastasis, or abdominal sarcomatosis, all of which are associated with high morbidity and may be addressed through palliative therapy. Current trends in the use of palliative therapy and factors associated with its use in patients with advanced RPS remain largely unexplored. The objectives of this study are to (1) describe the temporal trend in the use of palliative therapy and (2) identify factors associated with its use in patients with advanced RPS in the United States from 2004 to 2020. METHODS: This study is a retrospective cohort study using the National Cancer Database. We identified adult patients who were diagnosed with advanced RPS (American Joint Committee on Cancer stages III and IV) from 2004 to 2020. We performed a trend analysis to describe the use of palliative therapy over time, followed by univariable and multivariable logistic regression analyses to identify predictors of palliative therapy use in this patient population. RESULTS: A total of 6149 patients with advanced RPS were identified, of which only 383 used palliative therapy, including surgery (n = 28), radiation therapy (n = 87), systemic therapy (n = 115), pain management (n = 61), combination therapy (n = 55), or other palliative therapy (n = 37). The proportion of patients using palliative therapy increased significantly from 2.6% in 2004 to 6.5% in 2020 (Ptrend < 0.001). On multivariable logistic regression, age (odds ratio [OR] 1.03, 95 confidence interval [CI] 1.01-1.04), year of diagnosis (OR 1.05, 95 CI 1.02-1.08), lack of insurance (OR 2.18, 95 CI 1.17-4.04), community cancer program status (OR 1.83, 95 CI 1.05-3.19), stage IV disease (OR 5.19, 95 CI 4.49-7.79), and rhabdomyosarcoma (OR 2.75, 95 CI 1.32-5.72) histology were found to be predictors of palliative therapy use. CONCLUSIONS: This study sheds light on the evolving landscape of palliative therapy use for patients with advanced RPS in the United States from 2004 to 2020. The observed gradual increase in the use of palliative therapy underscores the growing recognition of its importance in managing the unique challenges associated with this complex disease. Despite this positive trend, the persistently low overall rates highlight the need for further efforts to enhance awareness and accessibility of palliative therapy for this patient population.

Revisiting abdominal wall "morbidity" of the extensile anterolateral approach to the thoracolumbar spine.

PURPOSE: To assess patients' perceptions of their abdominal wall following extensile anterolateral approaches to the thoracolumbar spine for adult spinal deformity (ASD) using validated questionnaires. METHODS: Adults who underwent anterior-posterior thoracolumbar spinal operations to the pelvis for ASD in which the anterior fusion was performed through an extensile anterolateral approach were reviewed. Three questionnaires were administered at least 1 year following surgery and included The Abdominal Core Health Quality Collaborative Survey (AHS-QC), The Patient Scar Assessment Scale (PSAS), and The Anterior Abdominal Incision Questionnaire (AAIQ). RESULTS: Fifty-one patients (80.4% female, median age 65 years) were included. Average follow-up was 2.8 ± 1.7 years. Average number of anterior fusion levels was 3.5 ± 1.4. Patients achieved high satisfaction rates from surgery (74.5%). AAIQ responses included postoperative pain (33.3%), bulging (41.7%), and limitations in daily activities (18.8%) with only 15.7% experienced moderate-severe pain related to their incisions and only 6.3% seeking treatment for their scars. Post-operatively, 63.2% had a neutral or improved self-image of their torso and trunk, while only 10.2% stating it was much worse. Patients' overall opinion of their scar compared to their normal skin was very positive [average 2.75 ± 2.93 (10 = worst possible scar)]. Favorable scores were also reported for color difference, stiffness, change in thickness, and irregularity in their abdominal scar compared to normal skin. CONCLUSIONS: Following extensile anterolateral approaches to the thoracolumbar spine for ASD, the majority of patients reported mild pain, mild functional limitations, good cosmesis, and high satisfaction rates with their anterior incisions based on validated questionnaires.

Synchronous retroperitoneal liposarcoma and gastric adenocarcinoma: A rare case report.

Multiple primary malignancies can occur as either synchronous or metachronous tumors, particularly in adults. Retroperitoneal liposarcoma is a rare malignancy arising from adipose tissue, while gastric adenocarcinoma is one of the most common neoplasms worldwide. The simultaneous occurrence of these 2 malignancies is extremely rare, with only 1 case reported worldwide. Here, we present the case of a 52-year-old man diagnosed with both retroperitoneal liposarcoma and gastric adenocarcinoma, marking the second reported case in the world.

Rare giant retroperitoneal melanotic schwannoma: a case report and literature review.

Background: Melanotic schwannoma (MS), a rare variant of peripheral nerve sheath tumor, is especially infrequent when originating from the peritoneum. Its definitive diagnosis relies on postoperative histopathological examination and immunohistochemical analysis, while preoperative diagnosis is difficult. Case presentation: In the present study, we reported a rare case of giant MS in the retroperitoneum, which was previously misdiagnosed before surgery. However, intraoperative exploration revealed it was retroperitoneal tumor. The tumor had invaded the abdominal aorta and bilateral common illiac artery walls. A surgical resection was subsequently executed, and postoperative histopathological examination confirmed it as a MS. Conclusion: The incidence of peritoneal MS is extremely rare, and surgical resection remains the preferred treatment modality. Given the absence of established guidelines for postoperative adjuvant therapy, long-term follow-up becomes imperative to accumulate valuable clinical expertise.

Single-incision Retroperitoneal Laparoscopic Resection of Adrenal Tumors in Children.

BACKGROUND: We describe our experience with single-incision retroperitoneal laparoscopic (SIRL) for resection of adrenal tumors in pediatric patients and discuss the technique's clinical value. METHODS: We retrospectively analyzed clinical data of 27 pediatric patients who underwent SIRL between January 2020 and September 2023. Patients with tumors >5 cm in size and those requiring vascular skeletonization surgery or extensive lymph node dissection were excluded. Demographic, perioperative, and prognostic data were collected, and computed tomography (CT) and magnetic resonance imaging were used for preoperative tumor assessment. RESULTS: Of 27 patients, 16 were male and 11 were female; mean age 54 ± 45 months and mean body mass index 17.2 ± 3.6 kg/m2. Mean tumor length, width, and height were 4.1 ± 1.8 cm, 3.3 ± 2.1 cm, and 2.9 ± 1.7 cm, respectively. One patient experienced a diaphragmatic tear, three patients incurred peritoneal damage, and one patient developed postoperative renal artery injury, leading to thrombosis and renal atrophy. No surgery was converted to open surgery, and no intraoperative or postoperative blood transfusions were required. Operative time, blood loss, and postoperative dietary recovery time were satisfactory. No local recurrence or distant metastases were detected during the 6-48 months of follow-up involving outpatient and telephone assessments. CONCLUSIONS: Application of SIRL in pediatric patients with adrenal tumors achieved favorable clinical outcomes with an effective, minimally invasive surgical option for treating children with adrenal tumors. This technique demands a high level of surgical expertise, specialized instruments and experienced surgeons. Our findings indicate that SIRL is safe and provides significant postoperative benefits in pediatric patients. LEVEL OF EVIDENCE: Level IV.

New insights into predictors of autoimmune pancreatitis relapse after steroid therapy.

OBJECTIVES: While autoimmune pancreatitis (AIP) responds well to steroid therapy, the high relapse rate in type 1 AIP remains a critical problem. The present study examined predictors of relapse of type 1 AIP following steroid therapy. MATERIALS AND METHODS: Nine factors potentially predictive of relapse were analyzed in 81 AIP patients receiving steroid therapy with follow-up ≥ 12 months. The rate of serum IgG4 decrease following steroid therapy was calculated by dividing the difference between serum IgG4 values before and at two months after the start of steroid by the IgG4 value before steroid. RESULTS: A relapse occurred in 11 patients (13.5%) during a median of 38 months. Multivariate analysis revealed that the presence of IgG4-related retroperitoneal fibrosis (HR: 5.59; 95% CI: 1.42-22.0; p = 0.014) and the low rate of serum IgG4 decrease after steroid therapy (HR: 0.048; 95% CI: 0.005-0.46; p = 0.008) were significant, independent predictors of AIP relapse. The cut-off value based on receiver operating characteristic curve data for the rate of serum IgG4 decrease before and at two months after steroid therapy distinguishing patients with and without a relapse was 0.65. Using this cut-off value, the area under the curve, sensitivity, and specificity were found to be 0.63, 0.73, and 0.60, respectively. CONCLUSION: The low rate of serum IgG4 decrease after the start of steroid therapy and the presence of IgG4-related retroperitoneal fibrosis were predictive of type 1 AIP relapse. Cautious, gradual tapering of steroid dosage and longer maintenance therapy are recommended for patients with these factors.