Retroperitoneal Tumor Research Articles

Da Vinci robot-assisted retroperitoneal tumor resection in 105 patients: a single-center experience.

The Da Vinci Surgical System (DVSS) has the advantages of minimal invasion, rapid recovery, safety, and reliability. Although the DVSS has been widely used in various abdominal surgeries, descriptions of its use in robot-assisted retroperitoneal tumor resection (RRTR) are limited to case reports; large-sample systematic studies are lacking. The present study was performed to analyze the data of RRTR in our center, summarize our experience, and provide a reference for other retroperitoneal tumor centers. We retrospectively analyzed the clinical data of 105 patients who underwent RRTR at the Affiliated Hospital of Qingdao University from January 2015 to December 2022. Logistic univariate and multivariate analyses were performed to identify independent risk factors affecting RRTR. A receiver operating characteristic curve was used to find the cut-off value, which was then included in the logistic multivariate analysis for verification. Among the 105 patients, 87 successfully underwent RRTR (DVSS group) and 18 underwent conversion to open surgery (conversion group). There was no significant difference in sex, age, body mass index, history of abdominal surgery, or tumor location between the two groups (P > 0.05). The maximum tumor diameter [odds ratio (OR), 1.041; 95% confidence interval (CI), 1.015-1.067; P = 0.002] and pathological property (OR, 8.646; 95% CI, 2.370-31.544; P = 0.001) were independent risk factors for conversion to open surgery. Further analysis confirmed that the success rate of RRTR was higher for tumors with a maximum diameter of ≤64 mm and benign tumors. Based on our experience and statistical results, we believe that retroperitoneal tumors that meet the following criteria have a higher success rate of DVSS resection: maximum tumor diameter of ≤64 mm, benign tumors, the tumor has relatively clear boundary, no obvious invasion of surrounding tissues and organs, and no need for combined organ resection. RRTR is safe and effective in the treatment of RPT, and the clinical prognosis is similar to that of open surgery. The success rate of RRTR in patients with appropriate surgical indications for this procedure is higher.

Discordance of retroperitoneal and thoracic histologic findings in patients with metastatic germ cell tumors at postchemotherapy residual tumor resection

Introduction and objectivesPostchemotherapy residual tumor resection (PC-RTR) is an important part of the multimodal treatment for patients with metastatic germ cell tumors. Simultaneous retroperitoneal and thoracic metastases often require consecutive surgical procedures. This study analyzes the histologic findings after abdominal and thoracic surgery in order to tailor the sequence and intensity of surgery.Patients and methodsFrom a total of 671 PC-RTRs from 2008 to 2021 we analyzed 50 patients with stage III non-seminomatous germ cell tumor (NSGCT) who had undergone both retroperitoneal and thoracic postchemotherapy residual tumor resection after first-line and salvage chemotherapy.ResultsAll patients included had stage III NSGCT. 39 and 11 patients received first-line and salvage chemotherapy, respectively. 45 (90%) patients received retroperitoneal resection first, followed by thoracic surgery. Three patients (6%) underwent thoracic surgery before retroperitoneal surgery and two patients (4%) underwent simultaneous surgery. Overall, the histology of retroperitoneal and thoracic specimens was discordant in 23% of cases. After first-line chemotherapy, of fourteen patients with necrosis in retroperitoneal histology, four patients had vital carcinoma in lung histology. In patients with teratoma in the retroperitoneum, the thoracic findings were concordant in most cases (78%). When teratomatous elements were also present in the orchiectomy specimen, concordance was 100%. After salvage chemotherapy, the discordance rate was 55%.ConclusionThe data presented in this study underline that retroperitoneal residual masses with necrosis cannot reliably predict histologic findings of thoracic specimens. Patients with teratoma in the retroperitoneum have a high likelihood of teratoma in the thoracic specimen.

The Value of MRI and CT in the Diagnosis of Retroperitoneal Tumours

Objective: This study aimed to investigate the effectiveness and value of MRI and CT in the diagnosis of retroperitoneal tumours. Methods: 60 patients with retroperitoneal tumours admitted to our hospital between July 2022 and March 2023 were selected as the study subjects. All of them received MRI and CT examinations. The detection of the two examination methods was compared and analyzed using the pathological findings as the standard. Results: The detection rate of MRI (58/60, 96.67%) was significantly higher than that of CT (50/60, 83.33%), and the difference was significant (P = 0.015 < 0.05). Conclusion: Both MRI and CT have important application values in the diagnosis of retroperitoneal tumours. MRI has advantages in observing soft tissue structures, nerve tissues, etc., and can provide more detailed anatomical structure information, which can help differentiate the retroperitoneal tumours and locate them accurately. CT, on the other hand, has unique advantages in observing the skeletal structure and the density of certain tumours, etc. It can quickly obtain comprehensive imaging information, which helps to determine the extent and invasion of the tumour.

Extraskeletal osteosarcoma infiltrating pancreas, spleen, gastric, and left kidney: a case report

BackgroundExtraskeletal osteosarcoma is an extremely rare malignancy that accounts for 1% of soft tissue sarcoma and 4.3% of all osteosarcoma. Extraskeletal osteosarcoma can develop in a patient between the ages of 48 and 60 years. The incidence of extraskeletal osteosarcoma is slightly higher in male patients than in females.Case presentationA 50-year-old Caucasian male patient presented with a 6-month history of intermittent lower-left back pain that limits his activity. Prior ultrasonography and abdominal computed tomography scan showed a diagnosis of kidney stone and tumor in the lower-left abdomen. The computed tomography urography with contrast revealed a mass suspected as a left retroperitoneal malignant tumor. Hence, the tumor was resected through laparotomy and the patient continued with histopathological and immunohistochemistry examination with the result of extraskeletal osteosarcoma.ConclusionExtraskeletal osteosarcoma presents diagnostic challenges requiring multimodal examination, including histological and immunohistochemistry analyses. This case underscores the aggressive nature and poor prognosis despite undergoing the current suggested treatment.

Laparoscopic Removal of a Large Extraorgan Retroperitoneal Cyst

Introduction. Extraorgan cysts of the retroperitoneal space (ECRS) remain a challenge in terms of determining therapeutic and diagnostic tactics. In most cases, ECRS are asymptomatic, which explains their detection as an incidental finding during examinations or surgical interventions in the abdominal cavity and/or retroperitoneal space. The main method for treatment of true and formed false ECRS consists in surgery with both laparotomy and laparoscopic accesses. Materials and methods. Clinical case: patient M., female, 39 years old, addressed to one of the clinics in Ufa with complaints of periodic lower left abdominal pain. Based on the results of the examinations, a diagnosis was made of a large tumor of the left ovary, complicated by pain. Concomitant diagnosis: multinodular uterine myoma. Surgical treatment — conservative myomectomy — with laparotomy access was performed due to the large size of the neoplasm. Intraoperatively, in the left iliac region, a volumetric liquid formation measuring 20×15 cm, not associated with the ovary, was discovered retroperitoneally. After further examination, the patient was referred for surgical treatment for retroperitoneal tumor in the City Clinical Hospital No 21. Results and discussion. The surgery was performed laparoscopically. The duration of the surgical intervention accounted for 39 minutes. The postoperative period was reported to be uneventful. Drains were removed on the second day. The patient was discharged from hospital on the fifth day after surgery. Histological examination of the cyst wall revealed no epithelial lining; the wall consisted of fibrous tissue. In this clinical case, according to the results of histological examination, the structure of the cyst wall corresponds to the secondary type or false cysts due to no epithelial lining. Conclusion. In cases of extraorgan retroperitoneal cysts, laparoscopic excision is considered to be a safe and effective method with less postoperative pain and a shorter recovery period compared to open interventions.

SEOM-GG clinical guidelines for the management of germ-cell testicular cancer (2023)

Testicular germ cell tumors are the most common tumors in adolescent and young men. They are curable malignancies that should be treated with curative intent, minimizing acute and long-term side effects. Inguinal orchiectomy is the main diagnostic procedure, and is also curative for most localized tumors, while patients with unfavorable risk factors for recurrence, or those who are unable or unwilling to undergo close follow-up, may require adjuvant treatment. Patients with persistent markers after orchiectomy or advanced disease at diagnosis should be staged and classified according to the IGCCCG prognostic classification. BEP is the most recommended chemotherapy, but other schedules such as EP or VIP may be used to avoid bleomycin in some patients. Efforts should be made to avoid unnecessary delays and dose reductions wherever possible. Insufficient marker decline after each cycle is associated with poor prognosis. Management of residual masses after chemotherapy differs between patients with seminoma and non-seminoma tumors. Patients at high risk of relapse, those with refractory tumors, or those who relapse after chemotherapy should be managed by multidisciplinary teams in experienced centers. Salvage treatment for these patients includes conventional-dose chemotherapy (TIP) and/or high-dose chemotherapy, although the best regimen and strategy for each subgroup of patients is not yet well established. In late recurrences, early complete surgical resection should be performed when feasible. Given the high cure rate of TGCT, oncologists should work with patients to prevent and identify potential long-term side effects of the treatment. The above recommendations also apply to extragonadal retroperitoneal and mediastinal tumors.

Immediate and long-term outcomes of surgical treatment in patients with retroperitoneal and abdominal desmoid fibromatosis: A retrospective study

Background. The main method of treatment of desmoid fibromatosis is surgical, especially in patients with symptomatic disease or in cases of progression during the Look and Stay period. Due to the rarity of the disease, different localization, unpredictability of the clinical course, the lack of generally accepted clear criteria for choosing a treatment method and/or a sequence of treatment methods, the determination of prognostic criteria for the course of the disease is of great scientific and practical interest. Aim. To study the immediate and long-term outcomes of surgical treatment in patients with retroperitoneal and abdominal desmoid fibromatosis. Materials and methods. The study analyzed the data of 121 patients with histologically verified retroperitoneal and abdominal desmoid fibromatosis who underwent surgical treatment at the Blokhin National Medical Research Center of Oncology from 1999 to 2022. Results. In 89% of cases, desmoid tumors are resectable; however, resections of adjacent organs are often required to remove the tumor mass completely. The frequency of combined interventions in the abdominal and retroperitoneal groups was 7.0 and 60.4%, respectively. Tumor cells along the edge of the incision are identified in 15.8% of patients, including 10% of patients with macroscopically detectable residual tumors. Surgical treatment of patients with desmoid tumors is associated with an acceptable complication rate and provides high rates of overall and relapse-free survival. Risk factors for disease-free survival of operated patients are retroperitoneal localization, multicentric tumor growth, and R2 category. Conclusion. The treatment of patients with retroperitoneal and abdominal desmoid tumors should be carried out in specialized clinics with sufficient experience in performing surgical interventions, including combined ones. The treatment approach in patients with desmoid tumors should be selected by a multidisciplinary team based on personalized oncological and functional prognoses in accordance with the prognostic risk groups.

Retroperitoneal tumors: a case series and literature review

Abstract Retroperitoneal tumors present significant diagnostic challenges. These tumors can either originate from retroperitoneal tissues or invade the retroperitoneum secondarily. While occurrence of benign lesions is observed, malignant tumors are more prevalent. The clinical presentation varies greatly depending on the tumor size, extent, and involvement of the surrounding tissues, making diagnosis difficult. Given the heterogeneous composition of the tumors, establishing a definitive diagnosis is challenging. Histopathology remains the gold standard technique for studying and analyzing these tumors, with immunohistochemistry being an important tool for confirming the diagnosis. Thus, we describe our experience with a series of four cases of rare retroperitoneal tumors, all of which were surgically resected and subjected to histopathological and immunohistochemical analyses.

A Rare Case of Bilateral Retroperitoneal Ovaries: A Case Report and Review of the Literature

Introduction: Retroperitoneal gynecologic tumors and cysts are rare. They are often asymptomatic or present similarly to their intraperitoneal counterpart with vague abdominal discomfort and distention. Our literature review revealed that many retroperitoneal cysts are found intraoperatively due to their benign predominance consistent with their ultrasound findings. The rarity of retroperitoneal gynecologic tumors and cysts creates uncertainty regarding the best practice management for general gynecologists caught off guard by their location when found intraoperatively. Rarer still are retroperitoneal ovaries. There are few cases of retroperitoneal ovary displacement in the literature, most often associated with female neonatal incarcerated inguinal hernia repair surgery. We found one case report involving an intraoperatively found retroperitoneal ovarian cystadenofibroma in a patient with a hernia repair performed in infancy. To our knowledge, there are no bilateral retroperitoneal ovaries described in the literature. Objective: To report a rare case of bilateral retroperitoneal ovaries in an 11 year old female. Methods: Case report. Results: We present an 11-year-old female with a past medical history of Dandy-Walker malformation and hydrocephalus status post ventriculoperitoneal (VP) shunt with six laparoscopic VP shunt revisions who endorsed hirsutism and irregular, heavy, and painful menstrual periods. Her family history is significant for polycystic ovarian syndrome (PCOS), endometriosis, and Lynch syndrome. Physical exam revealed mild abdominal distention and lower abdominal tenderness. She had an elevated estradiol, luteinizing hormone (LH), and total and free testosterone. Normally, an elevated estradiol causes a negative feedback response that results in a low LH. Due to these abnormal results, a pelvic ultrasound was ordered to rule out a potential estrogen secreting ovarian tumor. Ultrasound revealed a large anechoic structure in the left adnexa not definitively separated from the ovary measuring 13.0 x 7.7 x 10.7 cm. Radiologic differential diagnosis included large simple cyst, hemorrhagic cyst, and serous neoplasm. A referral to an adolescent gynecologist was made where the patient presented five months later with 25-pound weight gain, constipation, and a hard belly. Repeat ultrasound was consistent with a left ovarian simple cyst with an increased size measuring 16.78 x 12.58 x 8.66 cm as well as a new finding consistent with a right ovarian simple cyst measuring 4.96 x 4.48 x 3.61 cm. Due to her history of severe abdominal adhesions secondary to multiple laparoscopic VP shunt revisions, likely endometriosis, and large ovarian cyst, she was referred to a gynecologic oncologist where she was consented for robotic assisted bilateral ovarian cystectomy and lysis of adhesions. In the operating room, extensive pelvic adhesions were lysed to reveal bilateral retroperitoneal ovaries. The right cyst was drained of clear fluid, revealing no internal complexity, and was completely excised. Persistent pelvic adhesions and cyst size prolonged the time required to excise the left ovarian cyst wall. Once open, papillary excrescences were seen inside the cyst raising concern for a borderline ovarian tumor. The decision was made to remove the cyst wall in its entirety regardless of increased risk of ovarian function compromise. Restoration of normal anatomy was not attainable, and her bilateral ovaries remained embedded in the side walls and superior to the iliac vessels. Pathology revealed a benign 8 cm right paratubal cyst and a benign 18 cm left serous cystadenoma with papillary excrescences. Conclusions: Pelvic anatomy can be greatly distorted secondary to prior abdominal surgeries or certain pathologies including endometriosis and congenital abnormalities. Ultrasound cannot easily discriminate between intraperitoneal and retroperitoneal ovarian cysts but is often the only preoperative imaging obtained prior to cystectomy. Thus, many retroperitoneal cysts are found intraoperatively resulting in diagnostic and therapeutic challenges. Therefore, general gynecologists must be aware of risk factors associated with abnormal pelvic anatomy and prepared to request intraoperative consultation if needed to ensure optimized patient care and safety.

A Rare Case of Bilateral Retroperitoneal Ovaries: A Case Report and Review of the Literature

Introduction: Retroperitoneal gynecologic tumors and cysts are rare. They are often asymptomatic or present similarly to their intraperitoneal counterpart with vague abdominal discomfort and distention. Our literature review revealed that many retroperitoneal cysts are found intraoperatively due to their benign predominance consistent with their ultrasound findings. The rarity of retroperitoneal gynecologic tumors and cysts creates uncertainty regarding the best practice management for general gynecologists caught off guard by their location when found intraoperatively. Rarer still are retroperitoneal ovaries. There are few cases of retroperitoneal ovary displacement in the literature, most often associated with female neonatal incarcerated inguinal hernia repair surgery. We found one case report involving an intraoperatively found retroperitoneal ovarian cystadenofibroma in a patient with a hernia repair performed in infancy. To our knowledge, there are no bilateral retroperitoneal ovaries described in the literature. Objective: To report a rare case of bilateral retroperitoneal ovaries in an 11 year old female. Methods: Case report. Results: We present an 11-year-old female with a past medical history of Dandy-Walker malformation and hydrocephalus status post ventriculoperitoneal (VP) shunt with six laparoscopic VP shunt revisions who endorsed hirsutism and irregular, heavy, and painful menstrual periods. Her family history is significant for polycystic ovarian syndrome (PCOS), endometriosis, and Lynch syndrome. Physical exam revealed mild abdominal distention and lower abdominal tenderness. She had an elevated estradiol, luteinizing hormone (LH), and total and free testosterone. Normally, an elevated estradiol causes a negative feedback response that results in a low LH. Due to these abnormal results, a pelvic ultrasound was ordered to rule out a potential estrogen secreting ovarian tumor. Ultrasound revealed a large anechoic structure in the left adnexa not definitively separated from the ovary measuring 13.0 x 7.7 x 10.7 cm. Radiologic differential diagnosis included large simple cyst, hemorrhagic cyst, and serous neoplasm. A referral to an adolescent gynecologist was made where the patient presented five months later with 25-pound weight gain, constipation, and a hard belly. Repeat ultrasound was consistent with a left ovarian simple cyst with an increased size measuring 16.78 x 12.58 x 8.66 cm as well as a new finding consistent with a right ovarian simple cyst measuring 4.96 x 4.48 x 3.61 cm. Due to her history of severe abdominal adhesions secondary to multiple laparoscopic VP shunt revisions, likely endometriosis, and large ovarian cyst, she was referred to a gynecologic oncologist where she was consented for robotic assisted bilateral ovarian cystectomy and lysis of adhesions. In the operating room, extensive pelvic adhesions were lysed to reveal bilateral retroperitoneal ovaries. The right cyst was drained of clear fluid, revealing no internal complexity, and was completely excised. Persistent pelvic adhesions and cyst size prolonged the time required to excise the left ovarian cyst wall. Once open, papillary excrescences were seen inside the cyst raising concern for a borderline ovarian tumor. The decision was made to remove the cyst wall in its entirety regardless of increased risk of ovarian function compromise. Restoration of normal anatomy was not attainable, and her bilateral ovaries remained embedded in the side walls and superior to the iliac vessels. Pathology revealed a benign 8 cm right paratubal cyst and a benign 18 cm left serous cystadenoma with papillary excrescences. Conclusions: Pelvic anatomy can be greatly distorted secondary to prior abdominal surgeries or certain pathologies including endometriosis and congenital abnormalities. Ultrasound cannot easily discriminate between intraperitoneal and retroperitoneal ovarian cysts but is often the only preoperative imaging obtained prior to cystectomy. Thus, many retroperitoneal cysts are found intraoperatively resulting in diagnostic and therapeutic challenges. Therefore, general gynecologists must be aware of risk factors associated with abnormal pelvic anatomy and prepared to request intraoperative consultation if needed to ensure optimized patient care and safety.

ЛАПАРОСКОПИЧЕСКОЕ УДАЛЕНИЕ КИСТЫ ЗАБРЮШИННОГО ПРОСТРАНСТВА С РЕЗЕКЦИЕЙ И ПЛАСТИКОЙ ДИАФРАГМЫ

Background. Due to some challenges in diagnosis and surgical decision-making of retroperitoneal tumor-like masses, their treatment is regarded as a current issue in surgery. Objective. To demonstrate surgical treatment outcomes of a patient with a retroperitoneal tumor. Material and methods. The article presents our own clinical observation of a surgical removal of a tumor-like retroperitoneal tissue mass on the right with diaphragm resection as well as drainage of the pleural and abdominal cavities. Results. Based on laboratory and instrumental studies, the patient was clinically diagnosed with Chronic calculous cholecystitis as well as Parasitic cyst of the right liver lobe. A tumor-like posterolateral wall mass, about 5 cm in diameter, located retroperitoneally and associated with the right dome of the diaphragm was visualized intraoperatively. Laparoscopic removal of the mass within healthy tissues with resection and plastic of the right diaphragm dome was performed. The patient was discharged in a satisfactory condition, without complaints, for further outpatient follow-up by a surgeon at his place of residence. There were no repeated clinic visits or readmissions. Conclusions. The clinical observation indicating difficulty in diagnosis of retroperitoneal tumors in the preoperative period, it should be taken into consideration when planning surgery. When choosing a surgical treatment method, priority should be given to modern minimally invasive interventions, even in case of adjacent organs involvement.

Phase I/II study of TROP2 CAR engineered IL15-transduced cord blood-derived NK cells delivered intraperitoneally for the management of platinum resistant ovarian cancer, mesonephric-like adenocarcinoma, and pancreatic cancer.

TPS5626 Background: Novel treatments are needed for patients with ovarian, pancreatic, and mesonephric-like adenocarcinoma (MLA) that has recurred or progressed after initial treatment, as current standard of care therapies in these settings result in low response rates. Chimeric antigen receptor-transduced natural killer (CAR-NK) cells have emerged as a therapeutic alternative to CAR-T cells, with decreased toxicity and enhanced feasibility as an “off-the-shelf” therapy. TROP2, or trophoblast cell-surface antigen 2, is a transmembrane calcium-signal transducer that is overexpressed in many cancer types and associated with poor clinical outcomes. Recent studies have demonstrated TROP2 as a promising target for adoptive cell therapy in breast and gastric cancers. An intraperitoneal route of adoptive cell therapy delivery may provide superior disease control and decreased toxicity in these target cancers with predisposition for intra-abdominal/peritoneal involvement. Pre-clinical studies have established the efficacy and safety of TROP2 CAR NK cells in ovarian and pancreatic cancers. Methods: This is a phase I/II study of the intraperitoneal delivery of TROP2-CAR/IL-15 transduced cord blood (CB)-NK cells for recurrent/progressive ovarian cancer, pancreatic cancer, and MLA (NCT05922930). Subjects must have disease present in the peritoneal cavity or retroperitoneal lymph nodes and tumors must demonstrate at least 1+ TROP2 expression by immunohistochemistry. The primary endpoints are toxicity rates and defining the recommended phase II dose (RP2D). Key secondary endpoints include treatment efficacy, circulating tumor DNA levels, serial characterization of the immune microenvironment, and patient-reported outcomes. There are four possible dose levels ranging from 8.0x106 to 4.0x109 cells. We will enroll patients from the three disease cohorts using the Bayesian optimal interval (BOIN) design to find the maximum tolerated dose and RP2D, with a maximum sample size of 21. Once the R2PD is chosen, we will enroll up to 10 patients within each disease cohort to further evaluate safety and efficacy. Subjects enrolled will be admitted to the hospital, receive lymphodepleting chemotherapy (fludarabine 30 mg/m2 and cyclophosphamide 300mg/m2) on days -5 to -3, and will receive a single dose of cryopreserved and thawed intraperitoneal TROP2-CAR/IL15-transduced CB-NK cells on day 0. The dose-limiting toxicity window will be from day 0 until day 28. Severity of adverse events will be graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (NCI CTCAE) version 5. The first patient on this trial was treated in January 2024. Clinical trial information: NCT0592293 .

Development of an enhanced recovery after surgery program for pediatric solid tumors.

Enhanced recovery after surgery (ERAS) is an evidence-based, multi-modal approach to decrease surgical stress, expedite recovery, and improve postoperative outcomes. ERAS is increasingly being utilized in pediatric surgery. Its applicability to pediatric patients undergoing abdominal tumor resections remains unknown. A group of key stakeholders adopted ERAS principles and developed a protocol suitable for the variable complexity of pediatric abdominal solid tumor resections. A multi-center, prospective, propensity-matched case control study was then developed to evaluate the feasibility of the protocol. A pilot-phase was utilized prior to enrollment of all patients older than one month of age undergoing any abdominal, retroperitoneal, or pelvic tumor resections. The primary outcome was 90-day complications per patient. Additional secondary outcomes included: ERAS protocol adherence, length of stay, time to administration of adjuvant chemotherapy, readmissions, reoperations, emergency room visits, pain scores, opioid usage, and differences in Quality of Recovery 9 scores. Institutional review board approval was obtained at all participating centers. Informed consent was obtained from each participating patient. The results of this study will be presented at pertinent society meetings and published in peer-reviewed journals. We expect the results will inform peri-operative care for pediatric surgical oncology patients and provide guidance on initiation of ERAS programs. We anticipate this study will take four years to meet accrual targets and complete follow-up. NCT04344899.

Inflammatory myofibroblastic tumor of the liver after adrenal neuroblastoma surgery: a case report

A boy aged 55 months was diagnosed with stage IV Neuroblastoma (NB) of the right adrenal gland 2 years ago. Preoperative chemotherapy was given and he was then treated with retroperitoneal tumor resection and lymph node dissection. After surgery, the children were transferred to the Hemato-Oncology Department for chemotherapy according to the high-risk group NB, with outpatient follow-up every 6 months. In the second postoperative year, abdominal computed tomography (CT) scan revealed a rounded hypodense area in the upper part of the right posterior lobe of the liver, with marked inhomogeneous enhancement in the venous phase after enhancement, which was surgically resected, and postoperative pathology confirmed inflammatory myofibroblastic tumor (IMT) of liver. The patient was not given any special treatment after surgery. In this study, whole transcriptome sequencing was performed on the postoperative specimen of adrenal NB and the specimen of IMT of liver. This unusual case emphasizes the need for close monitoring of second tumor development in NB survivors even in the absence of known predisposing factors.

Primary Retroperitoneal Tumors: A Report of 08 Cases at the General Surgery Department of The Mohamed VI University Hospital in Marrakech

Primary retroperitoneal tumors are a rare and heterogeneous group of neoplasms arising within the retroperitoneal space, excluding the kidneys, urinary tract, adrenal glands, major blood vessels, and adjacent viscera. Treatment involves surgical resection, often requiring sacrifices of adjacent organs, with adjuvant chemotherapy considered in some cases. Histologically, mesodermal tumors were most common, with myxoid liposarcomas being predominant. Long-term outcomes vary, with some cases showing favorable evolution but others experiencing recurrence or mortality post-surgery. This study aims to provide insights into the clinical presentation, diagnostic approach, treatment modalities, and long-term outcomes of primary retroperitoneal tumors based on a retrospective analysis of cases treated at the general surgery department of the Mohamed VI University Hospital in Marrakech. By elucidating the characteristics and management of these tumors, this study seeks to contribute to the understanding of primary retroperitoneal tumors and guide future diagnostic and therapeutic strategies to improve patient outcomes.

Low-grade malignant peripheral nerve sheath tumour presenting as retroperitoneal spindle cell neoplasm.

Retroperitoneal spindle cell neoplasms are diagnostically challenging. Malignant peripheral nerve sheath tumours (MPNSTs) can sometimes present as sporadic primary retroperitoneal tumours. MPNSTs are usually high-grade and highly aggressive tumours and are associated with a poor prognosis. Low-grade MPNSTs are very rarely described. This current case report describes a case of sporadic primary low-grade MPNST presenting as retroperitoneal spindle cell neoplasm. The diagnosis, imaging and immunohistopathological findings, as well as its successful surgical management, are presented.

Open versus robot-assisted retroperitoneal tumors resection involving inferior vena cava, abdominal aorta, and renal hilum: a comparative study

IntroductionSurgery is currently the only effective treatment for retroperitoneal tumors that do not involve any specific organ. The use of robots for removing both benign and malignant retroperitoneal tumors is considered safe and feasible. However, there is insufficient evidence to determine whether robotic retroperitoneal tumor resection (RMBRs) is superior to open retroperitoneal malignant resection (OMBRs). This study compares the short-term outcomes of robotic excision of benign and malignant retroperitoneal tumors with open excision of the same-sized tumors.MethodsThe study compared demographics and outcomes of patients who underwent robotic resection (n = 54) vs open resection (n = 54) of retroperitoneal tumors between March 2018 and December 2022. A 1:1 matching analysis was conducted to ensure a fair comparison.ResultsThe study found that RBMRs resulted in reduced operative time (OT), estimated blood loss (EBM), and postoperative hospital stay (PSH) when compared to OBMRs. Additionally, RBMRs reduced EBL, PHS, and OT for patients with malignant tumor involvement in major vessels. No significant differences were found in tumor size, blood transfusion rate, and morbidity rate between the RBMRs and OBMRs groups.ConclusionWhen comparing RMBRs to OMBRs, it was observed that RMBR was associated with lower (EBL), shorter postoperative hospital stays (PHS), and reduced operative time (OT) in a specific group of patients with both benign and malignant tumors.

Diagnosing liposarcoma on (peri)-renal mass biopsy: A clinicopathological study of 30 cases.

Classification of renal neoplasms on small tissue biopsies is in increasing demand, and maintaining broad differential diagnostic considerations in this setting is necessary. When evaluating a renal or perirenal tumour biopsy with sarcomatoid morphology, together with sarcomatoid renal cell carcinoma and sarcomatoid urothelial carcinoma as top diagnostic considerations, it is vital to additionally consider the possibility of well-differentiated and de-differentiated liposarcoma. This study reports a series of 30 biopsy samples from sites in or around the kidney collected from four institutions in which the correct diagnosis was either well-differentiated or de-differentiated liposarcoma. The majority (26 of 30, 87%) of lesions were accurately diagnosed on biopsy sampling, all of which incorporated testing for MDM2 by immunohistochemistry (IHC), fluorescence in-situ hybridisation (FISH) or a combination of the two as part of the diagnostic work-up. Tumour expression of MDM2 by IHC without confirmatory FISH analysis was sometimes (30%) sufficient to reach a diagnosis, but demonstration of MDM2 amplification by FISH was ascertained in the majority (57%) of biopsy samples. A diagnosis of de-differentiated liposarcoma was not definitively established until resection in four (13%) patients, as no MDM2 testing was performed on the corresponding pre-operative biopsies. When a retroperitoneal tumour is not clinically suspected, histological consideration of a liposarcoma diagnosis may be overlooked. Implementation of ancillary immunohistochemical and cytogenetic testing can ultimately lead to a definitive diagnosis in this potentially misleading anatomical location.

Retroperitoneal space tumour misinterpreted as a renal colic attack in a patient with urolithiasis, a suspected IgG4-related disease

Retroperitoneal space tumour misinterpreted as a renal colic attack in a patient with urolithiasis, a suspected IgG4-related disease

Advancements in Diagnosis and Multimodal Treatment Strategies for Retroperitoneal Tumors: A Comprehensive Review.

Retroperitoneal tumors (RPTs) encompass both benign and malignant entities, constituting ~0.1% to 0.2% of all malignant tumors, of which 70% to 80% manifest malignancy. Predominantly, retroperitoneal sarcomas (RPS) represent the most prevalent subtype among RPT. With over 70 histologic forms identified, liposarcomas and leiomyosarcomas emerge as the primary constituents of RPS. Accurate diagnosis of RPTs necessitates preoperative core-needle biopsy and comprehensive imaging assessment. The current staging protocol for RPS relies on the eighth edition of the American Joint Committee on Cancer/TNM classification. Surgical excision remains the established gold standard for treating RPS. Therapeutic approaches vary according to the underlying pathophysiology. Although chemotherapy and radiotherapy exhibit efficacy in managing metastatic and recurrent unresectable RPS, their role in primary RPS remains unresolved, necessitating further clinical trials for validation. Concurrently, ongoing research explores the potential of targeted therapies and immunotherapy. This literature review aims to provide a comprehensive overview of existing research, delineating diagnostic pathways and optimal therapeutic strategies for RPT.