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Background To analyze the anatomical partitioning of the retroperitoneal space and determine the distribution patterns of primary retroperitoneal neurogenic tumors (PRNTs). Methods Retrospectively analyze the clinical data and CT data of 401 patients with single-injection PRNT confirmed by surgical pathology, including 103 cases of neuroendocrine origin, 148 cases of neuroectodermal origin, and 150 cases of peripheral nerve origin. The retroperitoneal space was divided into the anterior pararenal space, posterior pararenal space, perirenal space, and great vessel space. The anatomical location of each PRNT was determined using axial CT images, and distribution patterns of PRNTs with different pathological properties were analyzed. Results There were statistically significant differences in the distribution of benign and malignant PRNTs ( p  < 0.01), PRNTs from different tissues ( p  < 0.01), and PRNTs of different pathological types ( p  < 0.01) in different anatomical divisions. The occurrence of PRNTs varied significantly by age group, with statistically significant differences across pathological types ( p  < 0.01). Conclusion The anatomical distribution of PRNT could provide an effective imaging basis for the diagnosis and differential diagnosis of PRNT, aiding in more accurate clinical assessment and treatment planning.

Extraskeletal osteosarcoma (ESOS) is an extremely rare malignancy with poor prognosis, accounting for 2–4% of all osteogenic sarcomas. The purpose of this study was to examine the oncological outcomes of this disease related to surgical treatment and/or combined adjuvant therapies and to analyze the associated prognostic factors in ESOS. From January 1990 to June 2016, 22 patients with primary ESOS were analyzed in this retrospective study. Overall survival (OS) and progression-free survival (PFS) rates were calculated by Kaplan-Meier methods and compared with log-rank test. 22 patients were diagnosed with ESOS, 19 showed localized diseases and 3 presented with metastatic lesions. The median age at diagnosis was 55.5 years. Surgery resection was performed for all patients, 18 of whom received adjuvant chemotherapy. The median follow-up time was 48.5 months. There were 10 cases of recurrence and 9 patients developed new metastases. The 5-year OS rate for all patients was 58%. For localized cohort, the 5-year OS rate was 62%, and the 3-year PFS rate was 31% with a median PFS of 16 months. Univariate analysis of related prognosis factors showed that larger size of tumor (>5.5 cm) and higher histologic grade emerged as significant factors associated with worse OS. The addition of combination chemotherapy has no effect found on OS or PFS in this study. In summary, for patients who presented with ESOS, larger tumor size and higher histologic grade indicate a lower OS rate. The combination chemotherapy does not improve the OS or PFS.

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.

Background Surgery is the principal treatment for retroperitoneal sarcoma. These tumors typically involve or abut multiple organs and therefore require multivisceral resections. Despite the complexity of such operations, a standardized approach has not been described. As a result, referral centers often see patients who have undergone suboptimal surgery, with gross disease left behind. This is one of the causes of the dismal prognosis of this disease. Methods These consensus statements came out from E-Surge, an educational symposium with live sarcoma surgery, performed by European and North American expert sarcoma surgeons illustrating an optimal technique to an international audience, held in 2010 and 2011. The content was then shared among members of the local subcommittee of European Organization for Research and Treatment of Cancer (EORTC)–Soft Tissue and Bone Sarcoma Group (STBSG). Results An attempt to describe a reproducible and standardized approach to these tumors is illustrated. A detailed description of the different procedures according to the variety of different presentations is made. Conclusions The approach described herein should be used as the reference standard in clinical practice and serve to perform quality check of local treatment in future trials.

Catecholamine-producing tumors of childhood include neuroblastic tumors, phaeochromocytoma, and paraganglioma (PPGL). PPGL and neuroblastic tumors can arise in similar anatomical locations and clinical presentations can overlap, resulting in diagnostic challenges. Distinguishing between these tumor types is critical as management and long-term surveillance strategies differ depending on the diagnosis. Herein we describe 2 clinical cases and illustrate key considerations in the diagnostic workup of a neuroblastoma vs PPGL for patients presenting with adrenal, pelvic, and retroperitoneal masses in childhood.

Pelvic masses frequently originate from the pelvic cavity and are often associated with uterine, ovarian, or intestinal disorders. This report describes the case of a patient with a pelvic mass diagnosed as a retroperitoneal dermoid cyst at our hospital. We analyzed this case and conducted a literature review, to mitigate the risk of misdiagnosis and enhance the treatment of retroperitoneal masses.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas originating from peripheral nerves or nerve sheath cells. They are often associated with neurofibromatosis type 1 (NF1) and have a poor prognosis due to high rates of recurrence and metastasis. We report a case of a 30-year-old female presenting with a retroperitoneal MPNST accompanied by metastases to the right pelvic wall and left rectus femoris muscle. The patient exhibited numerous café au lait spots densely distributed across the head, neck, trunk, and extremities, consistent with clinical features of NF1. Due to the limitations of gynecological ultrasonography, the mass was initially considered to originate from the adnexal region. Surgical interventions included exploratory laparotomy, partial colectomy, partial rectal resection, retroperitoneal neoplasms resection, and colostomy; however, only palliative resection was possible due to the tumor’s extensive invasion and inability to achieve clear margins. Histopathological examination, supported by immunohistochemical findings, confirmed the diagnosis of MPNST. Given the palliative nature of surgery and the short postoperative follow-up, the clinical outcome remains uncertain. This case highlights the diagnostic challenges of retroperitoneal MPNST, particularly in patients with NF1, and underscores the importance of early recognition and multidisciplinary management when curative resection is not achievable.

Background Extraskeletal 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 presentation A 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. Conclusion Extraskeletal 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.

Objective To explore the clinical features of inflammatory myofibroblastic tumors (IMT) of the retroperitoneal and genitourinary system. Methods We conducted a retrospective analysis of the clinical data of 23 patients with pathologically confirmed retroperitoneal and urogenital inflammatory myofibroblastic tumours who were admitted to the First Affiliated Hospital of Xiamen University between January 2010 and December 2024. Results There were 10 male patients and 13 female patients, with an average age of (42.1 ± 4.1) years. The tumors were located in the retroperitoneum in 8 cases, in the bladder in 5 cases, in the kidney in 5 cases, in the spermatic cord in 2 cases, in the testis in 1 case, in the epididymis in 1 case, and in the prostate in 1 case. All 23 patients were diagnosed with space-occupying lesions before surgery.Among the 23 cases, 19 underwent surgical treatment and 4 received conservative treatment.Seven patients received postoperative adjuvant treatment. Among them, 4 patients received non-steroidal anti-inflammatory drug treatment, 3 patients had no recurrence and are currently alive, and 1 patient had recurrence and died; 1 patient received treatment with epirubicin and cisplatin, had no recurrence and is currently alive; 1 patient was treated with ifosfamide combined with epirubicin and pirarubicin, had no recurrence and is currently alive; 1 patient was treated with azathioprine and crizotinib, had no recurrence and is currently alive. As of the last follow-up, 2 patients had died, and the rest were all alive. Conclusion IMT can originate from any part of the genitourinary system, with pain being the most common symptom. Its clinical management is similar to that of malignant tumours of the genitourinary system, and complete surgical resection is the standard treatment strategy for all genitourinary IMT.

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.