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Introduction: Extrarenal angiomyolipomas are extremely rare tumors, with only isolated reports in the literature. Their diagnosis is challenging as these lesions are often misdiagnosed as more common retroperitoneal tumors. This report presents a case of a retroperitoneal extrarenal angiomyolipoma, emphasizing its clinical, imaging, and histopathological features to facilitate accurate diagnosis and avoid errors. Case Presentation: A 58-year-old male with a history of benign prostatic hyperplasia presented with progressive abdominal distension and weight gain. Imaging studies revealed a giant retroperitoneal mass displacing the left kidney and abdominal aorta. A core needle biopsy initially suggested a well-differentiated liposarcoma T4N0M0, leading to neoadjuvant chemotherapy with doxorubicin and ifosfamide. Surgical resection of a 20 × 30 × 25-cm mass was performed. Histopathological and immunohistochemical analysis of the specimen confirmed the diagnosis of retroperitoneal extrarenal angiomyolipoma. Chemotherapy was discontinued, and the patient remains stable under follow-up. Discussion and Conclusion: Retroperitoneal extrarenal angiomyolipomas are rare, presenting significant diagnostic challenges due to their resemblance to other retroperitoneal neoplasms such as liposarcomas. This case highlights the importance of comprehensive imaging, histopathological examination, and immunohistochemical studies for accurate diagnosis. Increased awareness of this entity can help clinicians avoid unnecessary treatments and ensure appropriate management of similar cases. [ABSTRACT FROM AUTHOR]

Schwannomas are benign, generally indolent tumors of neural crest origin and comprise the most common histologic tumor of peripheral nerves. Schwannomas are a rare histology for retroperitoneal tumors and very rare histologic findings for tumors of the adrenal gland with fewer than 50 cases in the reported literature. Here we present a case report of a non-hormonally functional but metabolically active adrenal tumor with indeterminate imaging characteristics with final pathology showing a 6.1 cm adrenal schwannoma as well as historical institutional pathology review revealing two additional cases.

A jejunal intussusception as a presentation of retroperitoneal liposarcoma (RLPS) is a rare occurrence. The majority of RLPS are presented as an abdominal mass, however, having a jejunal obstruction is an interesting case. The aim is to describe the management of jejunal intussusception secondary to atypical lipomatous tumours with concurrent RLPS. A 61-year-old lady presented with a sudden onset of intestinal obstruction with 1 month of constitutional symptoms and an enlarging right lumbar mass. Computed tomography showed a small bowel intussusception with diffuse peritoneal and retroperitoneal lipomatosis. Emergency exploratory laparotomy, segmental bowel resection, and partial excision of intraperitoneal mesenteric lipoma were performed. A stage En-bloc resection of the RLPS and right nephrectomy was done later. However, she refused for subsequent surgery. A complete resection is the gold standard in managing RLPS. In this report, the management is rendered not to the standard as the patient first presented with intestinal obstruction requiring emergency reduction with a piecemeal resection. A stage surgery was required to determine a promising prognosis, but the patient refused such surgery. A small bowel intussusception in adults is rare but is mostly caused by a tumor or neoplasm. Early recognition of the complexity of the case should be preempted and referred to the tertiary team for further definitive surgery. Patient exhaustion from the subsequent surgery might hamper the only management available for the case.

Abstract Background Benign nerve sheath tumors presenting as solitary retroperitoneal masses (RBNSTs) pose a complex diagnostic challenge for multidisciplinary teams regarding differential diagnosis, staging, and treatment planning. This article reviews the role played by different imaging techniques in assessing RBNSTs and elucidates their typical pathological features with a particular emphasis on the correlation between imaging and histological findings. Furthermore, some examples of retroperitoneal tumors that merit consideration in the process of differential diagnosis based on cross-sectional investigations (CSIs) are reported. The correlation between tissue architecture and appearance on imaging can help increase the accuracy of differential diagnosis with other retroperitoneal neoplasms at CSIs. Critical relevance statement This educational review critically examines the correlation between imaging and histological features in solitary retroperitoneal benign nerve sheath tumors, offering valuable insights for improving the accuracy of differential diagnosis in clinical radiology. Key Points RBNSTs are challenging to diagnose because they lack specific radiological features. Differential diagnosis of RBNSTs from other retroperitoneal neoplasms on imaging is complex. Surgical removal of RBNSTs is recommended for an accurate diagnosis. Graphical Abstract

Background: Benign nerve sheath tumors presenting as solitary retroperitoneal masses (RBNSTs) pose a complex diagnostic challenge for multidisciplinary teams regarding differential diagnosis, staging, and treatment planning. This article reviews the role played by different imaging techniques in assessing RBNSTs and elucidates their typical pathological features with a particular emphasis on the correlation between imaging and histological findings. Furthermore, some examples of retroperitoneal tumors that merit consideration in the process of differential diagnosis based on cross-sectional investigations (CSIs) are reported. The correlation between tissue architecture and appearance on imaging can help increase the accuracy of differential diagnosis with other retroperitoneal neoplasms at CSIs. Critical relevance statement: This educational review critically examines the correlation between imaging and histological features in solitary retroperitoneal benign nerve sheath tumors, offering valuable insights for improving the accuracy of differential diagnosis in clinical radiology. Key Points: RBNSTs are challenging to diagnose because they lack specific radiological features. Differential diagnosis of RBNSTs from other retroperitoneal neoplasms on imaging is complex. Surgical removal of RBNSTs is recommended for an accurate diagnosis. [ABSTRACT FROM AUTHOR]

BackgroundGanglioneuromas are a benign tumor originating from neural crest cells. As one of the neuroblastic tumors, ganglioneuromas are most common in children, with a mean age at presentation of 7 years. Ganglioneuromas are typically singular in nature, but rarely can present with lymph node involvement and distant metastasis. We present a rare case of a retroperitoneal ganglioneuroma found in a human immunodeficiency virus positive adult, which was complicated by lymph node involvement. This case is notable not only in regard to the age of the patient, but also because of his human immunodeficiency virus positive status and the extension of tumor to lymph nodes.Case presentationA 27-year-old Latino male with history of human immunodeficiency virus presented with a 6-month history of left upper quadrant and epigastric abdominal pain with associated nausea and vomiting. The patient had a computed tomography scan showing a retroperitoneal mass encasing the aorta, celiac axis, and superior mesenteric artery. Core needle biopsy revealed ganglioneuroma. Owing to obstructive symptoms, resection of the mass along with partial gastric resection, partial pancreatic resection, and splenectomy was performed by a multidisciplinary group of surgeons. Pathology results revealed metastatic spread of ganglioneuroma to surrounding lymph nodes.ConclusionsGanglioneuromas are most common in children and very rarely occur in adults. However, it is still important to consider this entity in the differential for patients presenting with suspicious symptoms. While rare, it is essential to consider metastasis in this generally benign disease.

Objective: Rare disease. Background: Neurilemmomas are rare tumors derived from the Schwann cells that comprise the peripheral nerve sheaths. They have a slow growth and rarely display malignancy. Early diagnosis is rare, and the treatment consists by surgical resection. Although robotic-assisted surgery is commonly used for treating retroperitoneal diseases, there are few reports of resection of retroperitoneal and pelvic schwannoma through robotic-assisted surgery. In the present study, we reported a case of complete excision of a benign retroperitoneal schwannoma of the obturator nerve by robotic-assisted surgery. Case Report: A 51-year-old woman was referred by her gynecologist for left pelvic discomfort of a 3-month duration. The physical examination was normal, but a computerized tomography scan of the abdomen and pelvis showed an expansive pelvic lesion in the topography of the left iliac vessels, a hypodense contrast enhancement measuring 4.6Ã--3.4 cm. Magnetic resonance imaging showed an extraperitoneal lesion located medially and inferiorly to the left external iliac vessels, with a size of 4.9Ã--3.7 cm, and of probable neural etiology. Surgical resection of the tumor was recommended because of the diagnostic hypothesis of obturator nerve schwannoma. Conclusions: This case showed that retroperitoneal neurilemmomas are difficult to diagnose owing to a lack of specific symptoms, and the best treatment is complete tumor resection. The use of robotic techniques gives greater dexterity to the surgeon, since it provides high-definition 3-dimensional vision, which can make the removal of retroperitoneal tumors susceptible to minimally invasive resection in a safe and effective way. [ABSTRACT FROM AUTHOR]

Objectives: Retroperitoneal tumor resection commonly disturbs major vessels; therefore, surgical teams can recruit vascular surgeons to prevent injuries and improve the prognosis of oncologic patients. The objective of the present study is to establish long-term survival after retroperitoneal tumor resection surgery with an emphasis on the potential impact of preventing or repairing major vessel injuries when tumors are adjacent to the aorta or vena cava. Methods: Retrospective case series including all cases of surgical removal of retroperitoneal tumors between 2007 and 2020 in a highly specialized hospital in Mexico City. Long-term survival was defined as 5 years after surgical intervention. Descriptive statistics, group-comparison tests, and regression analysis were performed using Stata 16. Results: From a total of 70 cases, vascular injury occurred in 30 (42.8%) and the vascular surgeon intervened in 19 (27.1%) of them, 4 (21%) were performed by a vascular surgeon with planned intervention, and in 9 (47.3%) cases the vascular surgeon was called to join the surgery due to emergency. Intraoperative bleeding was 2-fold greater in the group with an emergent participation of vascular surgery in contrast with the planned intervention group (4, 235 mL vs 2, 035 mL, p = 0.04). The regression model revealed a significant association between the intervention of a vascular surgeon and long-term survival (OR 59.3, p = 0.03) after adjusting for sociodemographic and characteristics of oncologic nature. Conclusions: Planned intervention of vascular surgeons in retroperitoneal tumor resection may have a positive impact not only in trans-operatory period, but also on long-term survival. [ABSTRACT FROM AUTHOR]

Malignancies with an extended encasement or infiltration of the aorta were previously considered inoperable. This series demonstrates replacement and subsequent resection of the thoracoabdominal aorta and its large branches as an adjunct to curative radical retroperitoneal and spinal tumor resection. Five consecutive patients were enrolled between 2016 and 2020, suffering from cancer of unknown primary, pleomorphic carcinoma, chordoma, rhabdoid sarcoma, and endometrial cancer metastasis. Wide surgical resection was the only curative option for these patients. For vascular replacement, extracorporeal membrane oxygenation (ECMO) was used as a partial left-heart bypass. The early technical success rate was 100% for vascular procedures and all patients underwent complete radical tumour resection with negative margins. All patients required surgical revision (liquor leak, n = 2; hematoma, n = 3; bypass revision, n = 1; bleeding, n = 1; biliary leak, n = 1). During follow-up (average 47 months, range 22–70) primary patency rates of aortic reconstructions and arterial bypasses were 100%; no patient suffered from recurrent malignant disease. Thoracoabdominal aortic replacement with rerouting of visceral and renal vessels is feasible in oncologic patients. In highly selected young patients, major vascular surgery can push the limits of oncologic surgery further, allowing a curative approach even in extensive retroperitoneal and spinal malignancies.

Complete en bloc surgical resection offers the best opportunity for the cure of primary retroperitoneal sarcomas (RPS). The potential for disease recurrence, in the form of both loco-regional recurrence and distant metastases, underpins the rationale for postoperative surveillance. There is a paucity of high-quality evidence underpinning follow-up for RPS patients, and most practice guidelines draw from expert opinion and evidence from soft tissue sarcomas of the extremities. The available observational retrospective data analysis has failed to demonstrate that high-intensity radiological surveillance improves the overall survival in patients. The lack of a robust evidence base has given rise to variations in approaches to post-operative surveillance strategies adopted by specialist centres managing RPS across the world. More high-quality prospective research is needed and planned to more clearly support surveillance approaches that balance oncologic outcomes, patient-centric care, and health service value. Risk stratification tools exist and are available for use in routine practice. Their use will likely support more individualised post-operative surveillance moving forward. Surveillance will likely be underpinned by serial radiological imaging for the medium term. However, developments in genomics offer hope for biomarkers such as ctDNA to impact patient care positively in the future and further support individualised patient care pathways.

When obstructive lesions from the uterus or ovaries are suspected, patients with hydronephrosis are usually referred to a gynecologist. Here, a case of suspected endometriosis-related hydroureteronephrosis is reported. A 43-year-old woman with hydronephrosis was found to have a left distal periureteral tumor on the computerized tomography scan. Before the operation, the hydroureteronephrosis was suspected caused by the obstruction of ureter, related with ureteral endometriosis; however, the postoperative pathology revealed the diagnosis of retroperitoneal well-differentiated liposarcoma. When female patients have hydronephrosis, gynecologic causes should be considered. Both benign and malignant causes are needed to include when making differential diagnosis. Therefore, robot-assisted surgery is a feasible option because of its lower morbidity rate and more precise dissection of soft tissue than laparotomy in both benign and malignant retroperitoneal tumors.

Liposarcoma is the most common primary retroperitoneal sarcoma in adults. We report the case of an 86-year-old male who presented to the emergency department with frequent falls and unexplained weight loss that was found to have a cystic retroperitoneal dedifferentiated liposarcoma. Initial computed tomography revealed a large heterogeneous complex cystic hypoenhancing lesion in the left retroperitoneum. Subsequent magnetic resonance imaging demonstrates a multilocular cystic mass with microscopic lipid content, diffusion restriction, and enhancing nodular soft-tissue components. Histologic examination of the tissue sample following biopsy is consistent with cystic retroperitoneal dedifferentiated liposarcoma. Further management was not pursued due to the patient’s advanced age and frailty. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Spontaneous pancreatic and peripancreatic hemorrhage (SPH) is a rare subtype of spontaneous retroperitoneal hemorrhage. With diverse clinical manifestations and no specific presentations, early diagnosis of SPH becomes challenging. Patient-specific underlying causes and vital signs guide the SPH treatment approach. Case Reports: Case 1: A 39-year-old man reported unexplained hypogastralgia at the emergency department (ED). An abdominal MRI revealed a mixed hematoma and cystic lesions between the pancreatic head and descending duodenum, attributed to ruptured mucinous cystic neoplasms. Extensive hematoceles were identified around the liver and abdominal pelvis on an enhanced CT scan. After undergoing fasting, rehydration, proton pump inhibitor and somatostatin intravenous injections, and peritoneal puncture, his condition improved. He was discharged nine days post-admission. Case 2: A 44-year-old man arrived at the ED with back pain and right upper quadrant pain. Enhanced CT indicated peritoneal fluid and a hematoma between the pancreatic head and descending duodenum. He initially received conservative treatment. However, on the eighth day, he reported recurrent abdominal pain. Follow-up CT showed an enlarged hematoma and gastric content accumulation. The patient was fasted and put on parenteral nutrition, and by the 37th day of hospitalization, he had fully recovered and was discharged. Both patients, having stable hemodynamics, fully recovered following conservative management, with no surgical intervention required. Conclusions: Given its varied clinical presentations, SPH can easily be misdiagnosed. However, successful conservative management can lead to full recovery, as demonstrated in these case reports. [ABSTRACT FROM AUTHOR]

Hibernomas are extremely rare, benign tumors of brown fat origin with no specific symptoms. Surgery is the only treatment option, and because a definitive preoperative diagnosis is often not obtained, open surgery is usually chosen. In this case, we performed laparoscopic surgery on a 33‐year‐old woman with retroperitoneal hibernoma. As in most cases, a definitive diagnosis had not been preoperatively made; therefore, we laparoscopically removed the retroperitoneal tumor of unknown pathology as a diagnostic treatment. We chose laparoscopic surgery because of the magnifying effect of the laparoscope and to minimize scarring. The surgery was uneventful, with a procedure time of 280 minutes and a blood loss of 20 mL. The postoperative course was uneventful with no complications or recurrence. We conclude that laparoscopic surgery may be a viable option for hibernomas. [ABSTRACT FROM AUTHOR]

Simple Summary: The diagnosis of retroperitoneal sarcoma may be challenging for the radiologist. Current guidelines report postsurgical margin as the strongest predictive factor for disease-specific survival and recurrence, as well as histologic subtype and grade. The role of the radiologist is indeed important in RPS diagnosis, management, and follow-up, as the ability to promptly recognize local progression, invasion of nearby structures, and complications has a direct impact on patients' survival. A practical guide is provided to radiologists with an overview of the current knowledge regarding cross-sectional CT/MRI imaging features of patients with retroperitoneal sarcomas, presenting tips and tricks to improve imaging diagnosis of RPS. Primary retroperitoneal sarcomas (RPS) represent around 10–16% of all sarcomas, with liposarcomas and leiomyosarcomas being the most common subtypes. RPS have some peculiar characteristics, imaging appearances, worse prognosis, and complications compared to other locations of sarcoma. Commonly, RPS primarily present as large masses, progressively encasing adjacent structures, causing mass effect, and complications. RPS diagnosis is often challenging, and these tumors may be overlooked; however, failure to recognize RPS characteristics leads to a worse prognosis for the patients. Surgery is the only recognized curative treatment, but the anatomical constraints of the retroperitoneum limit the ability to achieve wide resection margins; therefore, these tumors have a high rate of recurrence, and require long-term follow-up. The radiologist has an important role in the diagnosis of RPS, the definition of their extent, and their follow-up. Specific knowledge of the main imaging findings is required to reach an early diagnosis, and, ultimately, to guarantee the best patient management. This article provides an overview of the current knowledge regarding cross-sectional imaging features of patients with retroperitoneal sarcomas, presenting tips and tricks to improve imaging diagnosis of RPS. [ABSTRACT FROM AUTHOR]

Objective: To explore the characteristics of clinical cases misdiagnosed as gynecological tumors and to improve the knowledge and diagnostic ability of pelvic non-genital system tumors. Methods: A retrospective analysis of the case data of 58 patients admitted between August 2009 and March 2022 in the Beijing Obstetrics and Gynecology Hospital of Capital Medical University who were misdiagnosed as gynecological tumors, all of which were confirmed to be non -genital tumors by intraoperative exploration and pathology, including 24 cases of retroperitoneal tumors, 15 cases of appendiceal tumors and 19 cases of intestinal or mesenteric tumors. The clinical features, imaging manifestations, surgical methods and pathological diagnosis were analyzed. Results: Twenty -four patients with retroperitoneal tumors were preoperatively misdiagnosed as adnexal masses in 18 cases (75.0%), as uterine fibroids or broad ligament fibroids in 5 cases (20.8%), and as inflammatory pelvic masses in 1 case (4.2%). The clinical manifestations were abdominal pain or lower abdominal cramping in 6 cases (25.0%), frequent urination in 2 cases (8.3%), dyspareunia with sacrococcygeal discomfort in 1 case (4.2%), and no obvious symptoms in 15 cases (62.5%). Ultrasound or pelvic magnetic resonance imaging (MRI) did not suggest the possibility of retroperitoneal masses, and only one patient had pelvic and abdominal CT enhancement that did not exclude the possibility of retroperitoneal masses. In 15 patients with appendiceal tumors, all patients were misdiagnosed as adnexal tumors before surgery. The clinical manifestations were abdominal pain or lower abdominal distension in 6 cases (40.0%), and no obvious symptoms in 9 cases (60.0%). None of the patients had ultrasound suggesting a possible source of the appendiceal mass, and 3 patients had CT or MRI suggesting possible tumors of appendicular origin. 19 patients with intestinal or mesenteric tumors were misdiagnosed as having an adnexal mass in 18 cases (94.7%), a case (5.3%) was misdiagnosed as subserous myoma. The clinical manifestations were abdominal pain or lower abdominal distension in 8 cases (42.1%), and no obvious symptoms in 11 cases (57.9%). A patient had ultrasound suggesting that mesenteric cysts were not excluded, and neither CT nor MRI suggested the possibility of intestinal or mesenteric masses. Conclusions: Retroperitoneal tumors, appendiceal tumors and intestinal or mesenteric tumors are mostly suggested as pelvic masses by preoperative imaging, most of them have no specific clinical symptoms, and it is difficult to differentiate them from gynecological tumors. The gynecologists should pay attention to the above tumors, and carefully indentify patients with adnexal tumors to avoid misdiagnosis. [ABSTRACT FROM AUTHOR]

Benign retroperitoneal tumors (BRT) represent a rare group of heterogeneous diseases. The literature lacks high-quality evidence about the optimal management of BRT, and most of the information available takes the form of case reports or case series. The aim of this review is to provide an overview of current management strategies for adult patients with BRT. A literature search using PubMed indexed articles was conducted and BRT were classified into five different biological subgroups: 1) lipomatous tumors, 2) smooth muscle tumors, 3) peripheral nerve sheath tumors, 4) myofibroblastic tumors, and 5) others. Tumors that are primarily pelvic in origin were excluded. Despite the significant heterogeneity of the disease, several generic considerations have emerged and can be applied to the management of BRT. Specifically, the risk of misdiagnosing a BRT with another pathology such as retroperitoneal sarcoma is notable. When encountered, suspected BRT should therefore be referred to a specialized sarcoma center. Multidisciplinary tumor boards, present at these centers, have a pivotal role in managing BRT. The decision of whether to offer surgery, nonsurgical treatment or a "watch-and-wait" approach should be made after multidisciplinary discussion, depending on tumor histology. Moving forward, collaborative research efforts dedicated to BRT remain crucial in gathering evidence and knowledge to further optimize patient care. [ABSTRACT FROM AUTHOR]

Introduction Extramammary myofibroblastomas are extremely rare. Case presentation The patient was an 88‐year‐old male. He presented for evaluation of frequent urination and a “pushing up” sensation from the groin during defecation. Thorough physical and radiographic examinations revealed a retroperitoneal tumor on the right side of the rectum. The pathologic examination of the biopsy tissue showed that the tumor was unlikely to be malignant. Nevertheless, the patient was symptomatic and thus underwent a laparoscopic tumor resection through a transperitoneal approach. The tumor was circumscribed with a solid capsule. Based on the pathologic findings, which included immunostaining, the tumor was diagnosed as a myofibroblastoma. There was no evidence of a recurrence 6 months postoperatively. Conclusion We present this case with the clinical course and surgical findings, and discuss the possibility of establishing a preoperative pathologic diagnosis of a myofibroblastoma.

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Copyright of Revista de Gastroenterología del Perú is the property of Sociedad de Gastroenterologia del Peru and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Malignancies with an extended encasement or infiltration of the aorta were previously considered inoperable. This series demonstrates replacement and subsequent resection of the thoracoabdominal aorta and its large branches as an adjunct to curative radical retroperitoneal and spinal tumor resection. Five consecutive patients were enrolled between 2016 and 2020, suffering from cancer of unknown primary, pleomorphic carcinoma, chordoma, rhabdoid sarcoma, and endometrial cancer metastasis. Wide surgical resection was the only curative option for these patients. For vascular replacement, extracorporeal membrane oxygenation (ECMO) was used as a partial left-heart bypass. The early technical success rate was 100% for vascular procedures and all patients underwent complete radical tumour resection with negative margins. All patients required surgical revision (liquor leak, n = 2; hematoma, n = 3; bypass revision, n = 1; bleeding, n = 1; biliary leak, n = 1). During follow-up (average 47 months, range 22–70) primary patency rates of aortic reconstructions and arterial bypasses were 100%; no patient suffered from recurrent malignant disease. Thoracoabdominal aortic replacement with rerouting of visceral and renal vessels is feasible in oncologic patients. In highly selected young patients, major vascular surgery can push the limits of oncologic surgery further, allowing a curative approach even in extensive retroperitoneal and spinal malignancies. [ABSTRACT FROM AUTHOR]

Complete en bloc surgical resection offers the best opportunity for the cure of primary retroperitoneal sarcomas (RPS). The potential for disease recurrence, in the form of both loco-regional recurrence and distant metastases, underpins the rationale for postoperative surveillance. There is a paucity of high-quality evidence underpinning follow-up for RPS patients, and most practice guidelines draw from expert opinion and evidence from soft tissue sarcomas of the extremities. The available observational retrospective data analysis has failed to demonstrate that high-intensity radiological surveillance improves the overall survival in patients. The lack of a robust evidence base has given rise to variations in approaches to post-operative surveillance strategies adopted by specialist centres managing RPS across the world. More high-quality prospective research is needed and planned to more clearly support surveillance approaches that balance oncologic outcomes, patient-centric care, and health service value. Risk stratification tools exist and are available for use in routine practice. Their use will likely support more individualised post-operative surveillance moving forward. Surveillance will likely be underpinned by serial radiological imaging for the medium term. However, developments in genomics offer hope for biomarkers such as ctDNA to impact patient care positively in the future and further support individualised patient care pathways. [ABSTRACT FROM AUTHOR]

Synovial Sarcoma (SS) is a rare soft-tissue malignancy. Only about 15% of SS originates from the retroperitoneum. Retroperitoneal SS (RSS) is usually diagnosed incidentally due to the anatomy of the retroperitoneum. The most common complaints of patients are abdominal and low back pain. Other common symptoms of RSS are palpable abdominal mass, weight loss, and anemia. In this study, we will describe a 29-year-old white Asian man with a diagnosis of RSS after radical nephrectomy. He was admitted to the Urology Department of Shohada-e Tajrish hospital, Tehran, Iran in March 2019. The distinguishing feature of this case is the size of the mass, which has never been reported so much for retroperitoneal synovial sarcoma. Radiologic imaging showed a huge retroperitoneal mass originating from the kidney. Based on the pathologic features and immunohistochemistry (IHC) study, the diagnosis was consistent with synovial sarcoma. Accurate diagnosis of RSS is usually based on the pathological findings. Therefore, in case of doubt, a biopsy can be employed. Surgical resection of the tumor and lymph nodes dissection is the main and most important part of the treatment. Aggressive resection with free margin is recommended. The role of adjuvant and neoadjuvant chemotherapy in RSS is not certain to date, but it is recommended according to the patient’s condition.

Congenital adrenal hyperplasia (CAH) is an inherited disorder of adrenal steroidogenesis often diagnosed in infancy. Gynecologists may encounter adult patients with CAH due to the clinical effects of increased androgens, e.g. hirsutism, clitoromegaly, oligomenorrhea, or, rarely, pelvic masses. This case report reviews the association of para-ovarian adrenal rest tumors with CAH, and the role of gynecologists in their evaluation and treatment. A 23-year-old woman with CAH (21-hydroxyase deficiency) untreated for the past 5 years presented with a pelvic mass and elevated serum testosterone (1433 ng/dL) and plasma ACTH (1117 pg/mL). Intraoperative findings revealed multiple retroperitoneal masses. Final pathology demonstrated adrenal rest tissue. Para-ovarian and ovarian adrenal rest tumors may present as a rare gynecologic manifestation in patients with untreated CAH.

INTRODUCTION: Retroperitoneal sarcoma (RPS) is a rare disease accounting for 0.1%-0.2% of all malignancies. Management of RPS is complex and requires multidisciplinary, tailored treatment strategies at all stages, but especially in the context of metastatic or multifocal recurrent disease. Due to the rarity and heterogeneity of this family of diseases, the literature to guide management is limited. METHODS: The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG) is an international collaboration of sarcoma experts from all disciplines convened in an effort to overcome these limitations. The TARPSWG has compiled the available evidence surrounding metastatic and multifocally recurrent RPS along with expert opinion in an iterative process to generate a consensus document regarding the complex management of this disease. The objective of this document is to guide sarcoma specialists from all disciplines in the diagnosis and treatment of multifocal recurrent or metastatic RPS. RESULTS: All aspects of patient assessment, diagnostic processes, local and systemic treatments, and palliation are reviewed in this document, and consensus recommendations provided accordingly. Recommendations were guided by available evidence, in conjunction with expert opinion where evidence was lacking. CONCLUSIONS: This consensus document combines the available literature regarding the management of multifocally recurrent or metastastic RPS with the practical expertise of high-volume sarcoma centers from multiple countries. It is designed as a tool for decision making in the complex multidisciplinary management of this condition and is expected to standardize management across centers, thereby ensuring that patients receive the highest quality care.

Spindle cell tumors exhibit a relatively low occurrence rate and can manifest in various locations within the human body, including soft tissues and bones. The process of making a diagnosis is supported by conducting pathological and immunohistochemical tests. A 50-year-old female patient visited the hospital with abdominal pain that lasted about a week. Magnetic resonance imaging of the pelvis showed that this mass was independent and was not a lymph node mass, but a retroperitoneal sarcoma type mass. As part of the treatment, the mass was surgically excised, and a supracervical hysterectomy was carried out. The tumor was wrapped in a grayish-white capsule and showed a lobulating pattern. Retroperitoneal spindle cell tumors, particularly those occurring in abdominal soft tissues, are infrequently observed. Histopathological diagnosis is done in stages, and when cases are ambiguous, immunohistochemistry can provide valuable guidance in the right direction. [ABSTRACT FROM AUTHOR]

Introduction: Adrenal gland tumors are unusual and are usually found incidentally by imaging studies. Within this group, myelolipomas are one of the rarest tumors, considered 2% of adrenal tumors. Clinical case: We present a 60-year-old female patient with a history of sporadically colicky pain at the level of the right hypochondrium. Abdominal ultrasound revealed cholelithiasis and a mass suggestive of a left adrenal adenoma. Abdominal tomography confirmed a giant adrenal tumor and gallstones. An adrenalectomy and conventional cholecystectomy were performed without complications. The histopathological diagnosis showed an adrenal myelolipoma and chronic cholecystitis. Discussion: Adrenal myelolipoma is infrequent, the etiology is unknown, it is usually asymptomatic and its finding is incidental, they are usually unilateral, smaller than 4cm and the incidence increases with age. Conclusions: When myelolipomas reach dimensions greater than 10cm, conventional adrenalectomy [ABSTRACT FROM AUTHOR]

Inferior vena cava leiomyosarcoma (IVCL) is a rare malignant mesenchymal tumor. Surgical treatment is a challenge because it must combine free surgical margins with vascular reconstruction, using prosthetic or autologous grafts, primary suture, or simple ligation without vein reconstruction. The ligation option is possible thanks to the slow growth of the tumor, allowing collateral venous circulation to develop. We present a case of an IVCL treated with radical resection without vascular reconstruction. The patient was a 48-year-old female with abdominal pain in the right upper quadrant, asthenia, and postprandial dyspeptic symptoms. Abdominal tomography revealed a mass with an expansive formation located in the infrahepatic segment of the inferior vena cava and reduced vessel lumen. During surgery, vein clamping did not provoke hemodynamic repercussions, suggesting sufficient collateral circulation formation. It was decided to perform a radical resection of the entire portion of the retrohepatic vena cava and ligate the vena cava without vascular reconstruction. The patient recovered without complications. [ABSTRACT FROM AUTHOR]

Retrorectal tumors, although rare, pose diagnostic and treatment challenges due to their nonspecific symptoms and complex anatomical location. This single-center case series reports short-term outcomes of laparoscopic transabdominal resection as a surgical approach for large retrorectal tumors. Between 2017 and 2020, five patients underwent this procedure. The median patient age was 53.2 years (range, 34-60 years), and the median operating time was 130 minutes (range, 95-205 minutes). All tumors were located in the retrorectal space. The median tumor size was 5.8 × 4.3 cm (range, 3.5-7.5 cm). Biopsy results included epidermoid cysts, tailgut cyst, lipoma, and keratinous cyst. The median hospital stay was 7.8 days (range, 5-11 days), and the median follow-up duration was 78.0 days (range, 14-219 days). One patient developed a postoperative surgical site infection. Overall, laparoscopic transabdominal resection appears to be a minimally invasive and effective treatment option for retrorectal tumors.

Introduction: Echinococcosis or hydatidosis is a parasitic zoonosis endemic in Chile that poses an important public health concern. It mainly affects the liver and lungs; however, it may involve diverse areas, with the isolated pelvic location being exceptional and difficult to manage. Case presentation. A 41-year-old female consulted the emergency department of a hospital in Chile due to lumbosacral pain and was diagnosed with hydatidosis. The patient received surgical treatment and medication, but the disease recurred twice: the first time, a year after the first intervention, and the second time, two months after the second. Therefore, she required a multidisciplinary approach that included long-term antibiotic therapy and a radical approach to the lesion, achieving an adequate control of the disease. Conclusion: Pelvic echinococcosis is difficult to diagnose due to its low frequency. This disease should be considered in the event of any cystic lesion in endemic areas. A multidisciplinary management reduces possible complications and recurrence.

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Objective To explore the efficacy and safety of robotic-assisted laparoscopy for children with retroperitoneal schwannoma. Methods A hospitalized 15-year-old girl with retroperitoneal schwannoma in October 2020 was selected and the relevant clinical data were retrospectively reviewed. Results Intermittent low back pain was a major symptom. Imaging examination revealed a right retroperitoneal mass with a distinct boundary and no obvious tumor infiltration in adjacent organs. Hematological tests showed no abnormalities. After complete preoperative preparation, robot-assisted laparoscopic complete resection of right retroperitoneal tumor was performed. Tumor size was around 10 cm X 6 cm X 5 cm and capsule remained intact. During Sh operation, intraoperative blood loss was around 450 ml without blood transfusion. Postoperative pathological examination confirmed the diagnosis of classical schwannoma. Discharge occurred at Day 3 post-operation. During a follow-up period of 17 months, there was no back pain, other discomforts or tumor recurrence. Conclusion Retroperitoneal schwannoma is rare in children. Robot-assisted laparoscopy can be safely and effectively applied to children's large retroperitoneal tumors. [ABSTRACT FROM AUTHOR]

Purpose To evaluate the safety and efficacy of ultrasound-guided percutaneous core needle biopsy (USPCB) of pancreatic and peripancreatic lesions adjacent to critical vessels. Materials and Methods Data were collected retrospectively from 162 patients who underwent USPCB of the pancreas (n = 98), the peripancreatic area adjacent to the portal vein, the paraaortic area adjacent to pancreatic uncinate (n = 34), and lesions on the third duodenal portion (n = 30) during a 10-year period. An automated biopsy gun with an 18-gauge needle was used for biopsies under US guidance. The USPCB results were compared with those of the final follow- up imaging performed postoperatively. The diagnostic accuracy and major complication rate of the USPCB were calculated. Multiple factors were evaluated for the prediction of successful biopsies using univariate and multivariate analyses. Results The histopathologic diagnosis from USPCB was correct in 149 (92%) patients. The major complication rate was 3%. Four cases of mesenteric hematomas and one intramural hematoma of the duodenum occurred during the study period. The following factors were significantly associated with successful biopsies: a transmesenteric biopsy route rather than a transgastric or transenteric route; good visualization of targets; and evaluation of the entire US pathway. In addition, the number of biopsies required was less when the biopsy was successful. Conclusion USPCB demonstrated high diagnostic accuracy and a low complication rate for the histopathologic diagnosis of pancreatic and peripancreatic lesions adjacent to critical vessels.

BackgroundThe modified frailty index (mFI) is an important method to risk-stratify surgical patients and has been validated for general surgery and selected surgical subspecialties. However, there are currently no data assessing the efficacy of the mFI to predict acute morbidity and mortality in patients undergoing surgery for retroperitoneal sarcoma.MethodsUsing the American College of Surgeons' National Surgical Quality Improvement Program from 2007 to 2012, we performed a retrospective analysis of patients with a diagnosis of primary malignant retroperitoneal neoplasm who underwent surgical resection. The mFI was calculated according to standard published methods. Univariate and multivariate statistical analyses including χ2 and logistic regression were used to identify predictors of 30-d overall morbidity, 30-d severe morbidity (Clavien III/IV), and 30-d mortality.ResultsWe identified 846 patients with the diagnosis of primary malignant retroperitoneal neoplasm who underwent surgical resection. The distribution mFI scores was 0 (48.5%) or 1 (36.3%), with only 4.5% of patients presenting with a score ≥3. Rates of 30-d overall morbidity, serious morbidity, and mortality were 22.6%, 12.9%, and 1.2%, respectively. Only selected mFI scores were associated with serious morbidity and overall morbidity on multivariate analysis (P  0.05).ConclusionsOur data demonstrate that the majority of patients undergoing retroperitoneal sarcoma resections have few, if any, comorbidities. The mFI was a limited predictor of overall and serious complications and was not a significant predictor of mortality. Better discriminators of preoperative risk stratification may be needed for this patient population.

Bronchogenic cysts are congenital benign tumors resulting from abnormal budding of the primitive foregut. Usually presented on the posterior mediastinum, its presence on the retroperitoneum is extremely rare. We present an asymptomatic lady patient with a retroperitoneal cystic lesion that was submitted to endoscopic ultrasound–guided biopsies and intracystic fluid aspiration with histology excluding malignance despite intracystic fluid biochemical analysis that disclosed extremely high carbohydrate antigen 19–9. Definite diagnosis of bronchogenic cyst was only possible after complete surgical resection of the lesion. Furthermore, we discuss the use of this antigen as a tumor marker in this situation and its relevance to the preoperative diagnosis of such lesions. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Schwannomas are benign tumors and their appearance in the pelvic region is rare and poses a major diagnostic problem. They can be sporadic or associated with genetical syndromes. They have a slow growth rate and may be asymptomatic for many years. Symptoms are usually nonspecific and due to compression of adjacent structures. Abdominal imaging modalities may not be able to differentiate a benign schwannoma from a malignant retroperitoneal tumor. This report is of a case of a 68-year-old woman presenting with recurrent abdominal pain and a diagnosis of a presacral retroperitoneal benign schwannoma that mimicked an ovarian tumor on pelvic magnetic resonance imaging. Case Report: The patient had a history of a femoral hernia repair and recurrent lower abdominal pain. Pelvic imaging raised the suspicion of a primary ovarian tumor. The mass appeared to have clear cleavage planes with the surrounding structures, so the patient was proposed for an exploratory laparotomy. Prior to the surgery, an additional pelvic computed tomography (CT) was performed (10 months after the first one), which did not show progression of the disease. The histological examination result was compatible with a benign retroperitoneal schwannoma and not an ovarian tumor. Conclusions: This report highlights that the diagnosis of retroperitoneal and pelvic masses can be challenging. In women, a primary ovarian tumor should be excluded on imaging and the diagnosis of a benign tumor, such as schwannoma, must be confirmed by histopathology, either preoperatively or following tumor resection. [ABSTRACT FROM AUTHOR]

IntroductionRetroperitoneal soft tissue sarcomas (RPS) are rare tumors. Surgery is the mainstay of curative therapy, but local recurrence is common. No recommendations concerning the best management of recurring disease have been developed so far. Although every effort should be made to optimize the initial approach, recommendations to treat recurring RPS will be helpful to maximize disease control at recurrence.MethodsAn RPS transatlantic working group was established in 2013. The goals of the group were to share institutional experiences, build large multi-institutional case series, and develop consensus documents on the approach to this difficult disease. The outcome of this document applies to recurrent RPS that is nonvisceral in origin. Included are sarcomas of major veins, undifferentiated pleomorphic sarcoma of psoas, ureteric leiomyosarcoma (LMS). Excluded are desmoids-type fibromatosis, angiomyolipoma, gastrointestinal stromal tumors, sarcomas arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing sarcoma, alveolar/embryonal rhabdomyosarcoma, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma.ResultsRecurrent RPS management was evaluated from diagnosis to follow-up. It is a rare and complex malignancy that is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, but some patients may experience prolonged disease control also at recurrence, when the approach is optimized and follows the recommendations contained herein.ConclusionsInternational collaboration is critical for adding to the present knowledge. A transatlantic prospective registry has been established.

Synovial Sarcoma (SS) is a rare soft-tissue malignancy. Only about 15% of SS originates from the retroperitoneum. Retroperitoneal SS (RSS) is usually diagnosed incidentally due to the anatomy of the retroperitoneum. The most common complaints of patients are abdominal and low back pain. Other common symptoms of RSS are palpable abdominal mass, weight loss, and anemia. In this study, we will describe a 29-year-old white Asian man with a diagnosis of RSS after radical nephrectomy. He was admitted to the Urology Department of Shohada-e Tajrish hospital, Tehran, Iran in March 2019. The distinguishing feature of this case is the size of the mass, which has never been reported so much for retroperitoneal synovial sarcoma. Radiologic imaging showed a huge retroperitoneal mass originating from the kidney. Based on the pathologic features and immunohistochemistry (IHC) study, the diagnosis was consistent with synovial sarcoma. Accurate diagnosis of RSS is usually based on the pathological findings. Therefore, in case of doubt, a biopsy can be employed. Surgical resection of the tumor and lymph nodes dissection is the main and most important part of the treatment. Aggressive resection with free margin is recommended. The role of adjuvant and neoadjuvant chemotherapy in RSS is not certain to date, but it is recommended according to the patient's condition. [ABSTRACT FROM AUTHOR]