Large retroperitoneal lymphangioma.

Abnormal cystic lymphangioma is a rare, benign tumour of lymphatic origin that often presents with subtle clinical symptoms. A man in his 20s sought medical attention for a progressively tender mass located in the right lower quadrant. Following ultrasound and CT imaging, surgical pathology confirmed the presence of abnormal cystic lymphangioma. This case is noteworthy because of the patient's young age and the retroperitoneal location. An exploratory laparotomy was performed, mitigating the risk of recurrence. In the future, if imaging identifies a cystic mass, consideration of abnormal cystic lymphangioma within the differential diagnosis is imperative. Despite its predominantly subtle symptoms and non-malignant nature, the mass effect by the abnormal cystic lymphangioma can compromise surrounding structures. Overall, clinicians confronted with a cystic mass should adopt a comprehensive diagnostic approach, encompassing abnormal cystic lymphangioma in the differential diagnosis due to its unknown and complex nature.

Diagnosis and treatment of malignant retroperitoneal mesothelioma: A case report.

RATIONALE: Malignant peritoneal mesothelioma (MPM) is a rare clinical disease. Although there are several reports describing intraperitoneal mesothelioma of the lung, liver, and intestine, retroperitoneal mesothelioma is, to our knowledge, very rare and rarely reported. In recent years, our best clinical protocols for the treatment and diagnosis of retroperitoneal mesothelioma have not been proven and the diagnosis and treatment are challenging. PATIENT CONCERNS: A 37-year-old Chinese woman complained of bilateral low back pain for a month, with obvious symptoms of low back pain on the left side. To treat low back pain, retroperitoneal masses were found during physical examination. The patient consulted a urological specialist for further treatment. DIAGNOSIS: After the operation, pathological biopsy confirmed retroperitoneal epithelioid diffuse mesothelioma. INTERVENTIONS: After exclusion of surgical contraindications, the patient underwent laparoscopic retroperitoneal lesion resection under tracheal intubation and general anesthesia, and the operation was successful. OUTCOMES: On the tenth day after surgery, the patient vital signs were stable, and he was discharged. LESSONS: Patients with malignant peritoneal mesothelioma may have no typical clinical symptoms, and the diagnosis is based on pathological and immunohistochemical examination. In selected patients, surgical cell reduction and intraoperative intraperitoneal heat chemotherapy have become the first choice of treatment, which can achieve ideal therapeutic effects and prolong survival.

A retrospective clinical analysis of 11 cases of PEComa from different sites.

PURPOSE: The objective of this paper is to offer a thorough examination of the clinical presentations, etiology, and treatment strategies associated with perivascular epithelioid cell tumors (PEComas). METHODS: This retrospective study examined the comprehensive archival data of PEComa cases diagnosed at Beijing Hospital from 2015 to 2023. The pathology slides of all patients were thoroughly reassessed by two experienced pathologists. A thorough retrospective analysis was undertaken, incorporating clinicopathological data including gender, age at diagnosis, initial clinical manifestations, signs, disease onset site, tumor markers, imaging findings, therapeutic modalities, pathological features, immunohistochemical profiles, treatment responses, and prognostic indicators. Patients were evaluated for disease severity according to established pathological classification criteria and were followed up until the designated analysis cut-off date. In instances where patients were unable to be monitored on-site, they were contacted via telephone for postoperative follow-up inquiries. RESULTS: This study included 11 patients with ages ranging from 17 to 66 years old, presenting with the disease in multiple anatomical sites, including the retroperitoneum (2/11), liver (4/11), kidney (4/11), lung (1/11), and broad ligament of the uterus (1/11). Most patients presented with non-specific clinical symptoms and were subsequently diagnosed with space-occupying lesions upon physical examination. The tumor demonstrated progressive growth and enlargement, which could result in compression of neighboring organs. Preoperative imaging alone is insufficient for a definitive diagnosis of PEComa, but MRI can provide an initial evaluation of the tumor's potential malignancy. Molecular marker testing specific to PEComa, such as HMB-45 (90.0%), SMA (81.8%), Melan-A (90.9%), vimentin (90.9%), and Desmin (36.3%), was conducted on all patients. No adjuvant therapies were administered postoperatively. Upon analysis, no instances of relapse at the primary site or the development of new tumors at other sites were observed. Regular imaging reviews of three patients with malignant PEComa post-surgery showed no evidence of recurrence. CONCLUSIONS: The clinical presentation, tumor biomarkers, and imaging characteristics of PEComa lack specificity, necessitating dependence on pathology and immunohistochemistry for precise diagnosis. The mainstay of treatment consists of surgical resection, with patients typically experiencing a favorable prognosis.

What is the association of preoperative biopsy with recurrence and survival in retroperitoneal sarcoma? A systematic review by the Australia and New Zealand Sarcoma Association clinical practice guidelines working party.

Preoperative biopsy for retroperitoneal sarcoma (RPS) enables appropriate multidisciplinary treatment planning. A systematic review of literature from 1990 to June 2022 was conducted using the population, intervention, comparison and outcome model to evaluate the local recurrence and overall survival of preoperative biopsy compared to those that had not. Of 3192 studies screened, five retrospective cohort studies were identified. Three reported on biopsy needle tract seeding, with only one study reporting biopsy site recurrence of 2 %. Two found no significant difference in local recurrence and one found higher 5-year local recurrence rates in those who had not been biopsied. Three studies reported overall survival, including one with propensity matching, did not show a difference in overall survival. In conclusion, preoperative core needle biopsy of RPS is not associated with increased local recurrence or adverse survival outcomes.

New Sarculator Prognostic Nomograms for Patients With Primary Retroperitoneal Sarcoma: Case Volume Does Matter.

OBJECTIVE: To update the current Sarculator retroperitoneal sarcoma (RPS) prognostic nomograms considering the improvement in patient prognosis and the case volume effect. BACKGROUND: Survival of patients with primary RPS has been increasing over time, and the volume-outcome relationship has been well recognized. Nevertheless, the specific impact on prognostic nomograms is unknown. METHODS: All consecutive adult patients with primary localized RPS treated at 8 European and North American sarcoma reference centers between 2010 and 2017 were included. Patients were divided into 2 groups: high-volume centers (HVC, ≥13 cases/year) and low-volume centers (LVC, <13 cases/year). Primary end points were overall survival (OS) and disease-free survival (DFS). Multivariable analyses for OS and DFS were performed. The nomograms were updated by recalibration. Nomograms performance was assessed in terms of discrimination (Harrell C index) and calibration (calibration plot). RESULTS: The HVC and LVC groups comprised 857 and 244 patients, respectively. The median annual primary RPS case volume (interquartile range) was 24.0 in HVC (15.0-41.3) and 9.0 in LVC (1.8-10.3). Five-year OS was 71.4% (95% CI: 68.3%-74.7%) in the HVC cohort and 63.3% (56.8%-70.5%) in the LVC cohort ( P =0.012). Case volume was associated with both OS (LVC vs. HVC hazard ratio 1.40, 95% CI: 1.08-1.82, P =0.011) and DFS (hazard ratio 1.93, 95% CI: 1.57-2.37, P <0.001) at multivariable analyses. When applied to the study cohorts, the Sarculator nomograms showed good discrimination (Harrell C index between 0.68 and 0.73). The recalibrated nomograms showed good calibration in the HVC group, whereas the original nomograms showed good calibration in the LVC group. CONCLUSIONS: New nomograms for patients with primary RPS treated with surgery at high-volume versus low-volume sarcoma reference centers are available in the Sarculator app.

[Diagnosis and surgical treatment of a patient with Castleman disease]. / Diagnostika i khirurgicheskoe lechenie patsienta s bolezn'yu Kastlemana: klinicheskoe nablyudenie.

Castleman disease (CD) is a benign lymphoproliferative disease. Small prevalence and diverse clinical course of disease makes it difficult to standardize diagnostics and treatment. Currently, the number of CD patients has increased with improvement in the quality of examination. Therefore, differential diagnosis of this disease is important. We present a young patient with CD and retroperitoneal non-organ neoplasm. Despite a thorough preoperative examination, the final diagnosis was established only after histological examination of surgical specimen. We discuss the diagnosis and surgical treatment of a patient with unicentric type of CD.

Retroperitoneal cystic lymphangioma in a cirrhotic patient: A case report and review of the literature.

Retroperitoneal cystic lymphangiomas (RCL) are rare benign tumors of the lymphatic system. They account for less than 1% of all lymphangiomas. Surgical resection is the recommended treatment option; however, obtaining a pre-operative diagnosis is often difficult and, in most cases, the final diagnosis is only possible following histological assessment of the surgical specimen. This report describes a case of RCL in a 58-year-old female cirrhotic patient who presented to our center with dull aching abdominal pain and distension. To our knowledge, this is the first case of a RCL in a cirrhotic patient reported in the literature.

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma.

With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as 'ultra-rare' sarcomas, with an incidence of ≤1-5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing's sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes.

Secreting retroperitoneal latero-aortic paraganglioma revealed by acute abdominal pain: a case report.

We report the case of a retroperitoneal paraganglioma in a 35-year-old man discovered by abdominal pain. The abdomino-pelvic computed tomography (CT) showed a retro-peritoneal latero-aortic mass compatible with a paraganglioma, confirmed by the 24-hour urinary metanephrines and normetanephrines test. After an adequate pharmacological preparation, we decide to perform a laparoscopic resection of the paraganglioma. The surgery went without any complication. Blood pressure and urinary catecholamines were normal after the surgery. Our observation presents the particularity of the mode of discovery which is atypical, as well as the particularity of the therapeutic management, which is the laparoscopic resection of the mass.

Recurrent metastatic retroperitoneal dedifferentiated liposarcoma: a case report and literature review.

BACKGROUND: Retroperitoneal liposarcoma (RPLPS), a rare tumor, is often treated using surgical procedures as the first choice for treatment. However, there is no consensus on the scope of surgical resection. In addition, the treatment outcomes of conventional radiotherapy and chemotherapy have not been satisfactory, particularly for specific types of LPS, such as dedifferentiated LPS. In this case study, we present a brief review of other cases of RPLPS, highlighting the selection of surgical scope for RPLPS and related adjuvant treatment for advanced RPLPS. CASE PRESENTATION: A case study is reported regarding an extremely rare recurrent and metastatic retroperitoneal dedifferentiated LPS. The primary RPLPS tumor, with a diameter of 20 cm and a weight of 2.5 kg, occupied the whole left abdomen and adhered to the left kidney. A surgical tumor resection combined with a left nephrectomy is performed. During the 6th -month postoperative follow-up examination, we observed the local recurrence of the tumor in the operation area, in addition to multiple metastatic tumors in both lungs. Further, the prescribed 3-month targeted treatment with anlotinib significantly reduced the size of the metastatic pulmonary tumors. However, the recurrent retroperitoneal tumors showed no significant change in size. Eventually, we observed no substantial evidence of tumor progression, with the patient's condition under control. CONCLUSION: The case demonstrated that the postoperative recurrence of widespread RPLPS required R0 resection to cure the disease, considering targeted therapy for advanced RPLPS control.

[Retroperitoneal Dedifferentiated Liposarcoma with No Recurrence after 5 Years of Postoperative Radiation Therapy --A Case Report-].

A 49-year-old woman presented to our hospital with right lower back pain and epigastric pressure. A computed tomographic scan showed a 12×7×20 cm retroperitoneal mass comprising fatty components and contrast areas around the right kidney. Based on the results, a right retroperitoneal liposarcoma was suspected. Thus, right retroperitoneal tumor resection combined with right kidney resection was performed. Instances of tumor adhesion were found in the ascending colon, duodenum, and the iliopsoas muscle, which could be dissected ; therefore, combined resection of the intestinal tract was not performed. The resected tumor was found to be mixed with dedifferentiated and well-differentiated components and was diagnosed as dedifferentiated liposarcoma. Due to the presence of positive margins, the patient received 50 Gy in 25 fractions of radiation therapy to the right side of the retroperitoneum as postoperative adjuvant therapy. During the irradiation period, vomiting and anorexia were observed as adverse events. Five years have passed since the surgery, and no local recurrence or late complications due to radiation have been observed. Although dedifferentiated liposarcoma is a highly malignant histological type with a very high local recurrence rate, no adjuvant therapy has been established. Some reports have suggested that postoperative radiation therapy for retroperitoneal sarcoma is effective in terms of survival and local control. However, there are no reports of prospective clinical trials, and the evidence is expected to widen in the near future.

Retroperitoneal hibernoma resected via laparoscopic surgery.

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.

Current management of benign retroperitoneal tumors.

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.

Clinical characteristics and prognosis of cystic degeneration in retroperitoneal schwannoma: A retrospective study of 79 patients.

BACKGROUND AND PURPOSE: Diagnosis of retroperitoneal schwannoma (RS), especially cystic RS, is frequently missed or delayed owing to its rarity, location, nonspecific symptoms, and similarities with other tumors on various imaging modalities. This study aimed to determine associations between clinical, radiological, and histopathologic features and outcome. MATERIALS AND METHODS: Seventy-nine patients with pathologically confirmed RS who underwent tumor resection between June 2010 and June 2020 were retrospectively reviewed and analyzed. Patients were stratified into three groups according to degree of tumoral cystic degeneration. RESULTS: Cystic degeneration was significantly associated with multiple foci (p = 0.025), calcification (p = 0.012), and hemorrhage (p = 0.000), but not size (p = 0.08), high Ki-67 (p = 0.094), malignancy (p = 0.115; prevalence of cystic degeneration in the benign and malignant groups were 53.9% vs 100%), rough margin (p = 0.162), or irregular shape (p = 0.369). Malignant RS was significantly associated with multiple lymph nodes enlargement (p = 0.034). Tumor size, margins, shape, or/and multiplicity did not significantly differ between benign and malignant tumors. No recurrence occurred in patients with benign RS (mean follow-up, 45 months). All malignant tumors recurred; mean time to recurrence was 11.4 months (mean follow-up, 33 months). CONCLUSION: Since RS is misdiagnosed mostly as malignancy and diagnosis is often delayed, a suspicion is necessary for diagnosis when atypical features are present. In RS, cystic degeneration was not associated with tumor size, Ki-67, or malignancy; however, it was significantly associated with multiple foci, calcification, and hemorrhage. Cystic degeneration and related factors are useful for the diagnosis of RS. Malignant RS should be considered when a mass involves multiple lymph nodes. Margins, morphology, and size are not associated with malignancy. Pathological tumor type, tumor location, and adjacent anatomic structures are associated with outcome.

Preoperative Neutrophil-to-Lymphocyte Ratio and a New Inflammatory Biomarkers Prognostic Index for Primary Retroperitoneal Sarcomas: Retrospective Monocentric Study.

PURPOSE: Inflammatory biomarkers and neutrophil-to-lymphocyte ratio (NLR) are associated with prognosis in several tumors. Data on sarcomas are limited, and insufficient on retroperitoneal sarcoma (RPS). EXPERIMENTAL DESIGN: Patients with primary RPS operated between 2002 and 2016 were included. Hemoglobin, monocytes, NLR, platelet-to-lymphocyte ratio (PLR) were retrieved and analyzed both individually and combined into a prognostic index (IBPI). Correlation with clinicopathologic variables was studied, as well as postoperative morbidity according to NLR and IBPI risk categories. The association between overall survival (OS) and biomarkers and, in addition, the 7-year Sarculator-predicted OS probability (pOS) was analyzed using univariable and multivariable Cox models. RESULTS: 423/463 patients had complete data. The median follow-up was 84 months. The median NLR was 3.3 (IQR, 2.4-4.7), with significant variation across histologies. NLR was the only biomarker that independently predicted OS (HR, 1.2; 95% CI, 1.03-1.40; P = 0.02). The IBPI showed good discrimination for subgroups at different OS (log-rank test P < 0.0001). The Cox model for pOS alone showed a 7-year index of prediction accuracy of 26.9, which increased to 29.5 when IBPI was added to pOS as a complementary prognostic tool. IBPI was also associated with the risk of serious infectious postoperative complications (P = 0.0094; noninfectious complications, P = 0.6463). CONCLUSIONS: NLR was an independent prognostic factor for OS in RPS. When combined into a prognostic index with hemoglobin, monocytes, and PLR, it serves as a readily available prognostic tool addressing tumor-related inflammation and helps in classifying RPS risk in addition to the Sarculator nomogram.

Pelvic soft tissue sarcomas.

Pelvic soft tissue sarcomas (PSTS) are a rare, heterogeneous group of tumors. They have been usually analyzed with retroperitoneal sarcomas (RPS), but actually have key differences. Due to their unique anatomic location, symptomatic presentation of PSTS may be more common than RPS. Adequate imaging approach is paramount for guiding differential diagnosis, while preoperative biopsy is mandatory, especially when preoperative treatment may be considered as initial approach. The most frequent histologic subtype is leiomyosarcoma, which is different as expected in the retroperitoneum where liposarcoma is the commonest histology. Also solitary fibrous tumor is commonly diagnosed in the pelvis. Surgical approach for PSTS differs from that for RPS mainly due to anatomic relations. Similarly, in the lack of definite evidence from specific trials about neoadjuvant and adjuvant treatments, the anatomic constraints to obtain wide margins in the pelvis as well as the expected functional outcome in case of organ resections should be factored into decision for individualized treatment offer. Vascular and genitourinary involvement are frequent, as well as herniation through pelvic foramina. For these reasons a multidisciplinary surgical team should always be considered. Early referral of these patients to high-volume centers is critical and may impact on survival, given that optimal initial resection is a major predictor of curative treatment. International consensus on PSTS treatment is advocated, similarly to the recent efforts realized for RPS.

New developments in the pathology and molecular biology of retroperitoneal sarcomas.

Retroperitoneal sarcomas (RPS) refer to a heterogeneous group of malignancies of mesenchymal origin developing from retroperitoneal tissues and vessels. The most frequent RPS are well differentiated/dedifferentiated liposarcomas and leiomyosarcomas, but other rare histological subtypes can be observed. Over the last decade, significant advances have been made in the pathological and molecular characterization of sarcomas. These advances have led to major changes in their diagnostic management as well as in the development of new therapeutic strategies based on tumor biology and microenvironment. This review describes the current knowledge and recent findings in the pathology and molecular biology of the most frequent RPS subtypes.

Retroperitoneal liposarcoma and craniosynostosis: possible genomic relationship, case report, and literature review.

Based on a case report, this review explores the genomic landscape for patients with liposarcomas and possible relationships with gene mutations related to craniosynostosis. We describe the case of a 40-year-old man, known for a surgical correction of craniosynostosis before the age of 1 year, who underwent a radical resection of a voluminous retroperitoneal liposarcoma; histopathological analysis revealed a low-grade well-differentiated, mostly sclerosing, liposarcoma. A genetic analysis searching for mutations in blood DNA was performed and did not detect any specific mutation. A literature review was also conducted. Several tumors related to syndromic and non-syndromic craniosynostosis are mentioned in the literature; no specific link with retroperitoneal liposarcoma is established but the FGFR3 mutation is detected in dedifferentiated liposarcomas. To date, no case has been reported in the literature demonstrating a genetic relationship between craniosynostosis and low-grade differentiated retroperitoneal liposarcoma. We conclude that further studies for gene complex mutations should be conducted to show a possible genetic relationship between retroperitoneal liposarcoma and craniosynostosis.