Clinicopathological differences between classical schwannomas and cellular schwannomas in the retroperitoneum.

This study aimed to compare clinical and pathological features of retroperitoneal classical schwannomas and cellular schwannomas. A total of 64 cases of retroperitoneal classical schwannoma and 48 cases of cellular schwannoma were studied. Histopathological analysis was performed using hematoxylin and eosin staining and immunohistochemistry. Retroperitoneal cellular schwannomas exhibited 100% (48/48) and 75% (36/48) positive expression for glial fibrillary acidic protein (GFAP) and cytokeratins (CK), respectively. Classical schwannomas showed rates of 6.25% (4/64) and 15.63% (10/64), respectively (P < .05). In classic schwannomas, 85.9% (55/64) showed a reticular pattern of positive anti-CD34 staining around tumor margins and subcapsular areas vs 52.1% (25/48) in cellular schwannomas (P < .05). Cellular schwannomas exhibited more mitotic figures than classical schwannoma (P < .05). The recurrence rate of cellular schwannomas was 10.42% (5/48), while that of classical schwannomas was 1.56% (1/64) (P < .05). Retroperitoneal cellular schwannomas commonly express GFAP and CK compared to classical schwannomas, suggesting that cellular schwannoma may originate from unmyelinated Schwann cells, while classical schwannoma may originate from myelinated Schwann cells. Anti-CD34 staining patterns may be used to distinguish between the 2 types. Retroperitoneal cellular schwannomas also show higher mitotic activity and are more prone to recurrence.

On the Origin of Abdominal Venous Leiomyosarcomas: The Role of the Sex-Hormone Drainage Pathways.

Background: We hypothesized that abdominal venous leiomyosarcoma (AV-LMS) disproportionately originates in veins of the sex-hormone drainage pathway (SHDP). Our purpose was to classify the anatomical origin of AV-LMS in a large cohort using imaging and explore prognostic implications. Methods: A retrospective review of imaging of all patients presenting with abdominal non-uterine LMS at a single tertiary oncology center was performed. Inclusion criteria were a biopsy-proven LMS of non-uterine abdominal/pelvic origin with pretreatment enhanced computed tomography (CT)/magnetic resonance imaging (MRI). Patients with uterine LMS or prior radiation were excluded. LMS site of origin was assigned by one expert radiologist and indeterminate sites were reviewed with a second external expert radiologist. Locations of inferior vena cava (IVC) tumors were subclassified based on a modification of prior literature. SHDP was defined as originating from ovarian/testicular vein, distal left renal vein, adrenal vein or mid-IVC (IIA). Results: One hundred fifty-five (155) patients were included (92/152 (61%) female) with distant metastases found at presentation in 23/155 (14.8%). Most common organs of origins were veins (84/152, 55.3%), gastrointestinal (24, 15.8%), genital (11, 7.2%) and paratesticular/spermatic cord (11, 7.2%). For venous LMS, the adrenal (both sexes), mid-IVC (IVC IIA, females) and ovarian veins had the highest relative predilection for abdominal non-uterine LMS. Eighty-four (84/152, 55.3%) of tumors were SHDP. On multivariable analysis, both size and SHDP were significant predictors of distant metastases at presentation (P = 0.01), while sex, age, organ system/site and grade were not. Conclusions: For both sexes, tumors arising from SHDP constitute the majority of AV-LMS and may impart a significantly lower risk of metastatic disease at presentation. Among veins, the adrenal veins had the highest predilection for LMS.

Duplicated and dilated ovarian vein with coexistence of persistent mesonephric artery.

During standard cadaveric dissection we encountered multiple vascular variations in the retroperitoneum: duplicated and dilated left ovarian vein with the coexistence of a persistent right mesonephric artery.

Primary retroperitoneal tumor: mucinous cystoadenocarcinoma.

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free during the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and optimal therapeutic strategies require further investigation.

Los cistoadenocarcinomas mucinosos primarios retroperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años sometida a tratamiento quirúrgico. La paciente tenía antecedentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisciplinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

Laparoscopic resection for retroperitoneum ganglioneuroma with Supine hypotension syndrome.

BACKGROUND: Supine hypotension syndrome (SHS) has been reported to occur due to compression by a giant tumor such as ovarian tumor. We herein report a case of retroperitoneal ganglioneuroma with SHS treated with laparoscopic resection. CASE PRESENTATION: The patient was an 11-year-old male with right-sided abdominal pain. He had a pale complexion and tachycardia while falling asleep. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a giant mass lesion (60 × 35 mm) with compression of the inferior vena cava (IVC) and duodenum ventrally and the right kidney caudally. The IVC was flattened by mass compression. Abdominal ultrasonography (US) revealed narrowing of the IVC due to the mass and accelerated blood flow after IVC stenosis in the supine and left lateral recumbent position. His pale complexion and tachycardia while falling asleep was thought to be due to decreased venous return caused by the tumor compressing the IVC, resulting hypotension. 123I-MIBG scintigraphy revealed no abnormal findings. Tumor markers were normal. He was diagnosed with SHS due to a right adrenal gland tumor. The tumor compressed the IVC from the dorsal side, and hemostasis was expected to be difficult during bleeding. Therefore, a guidewire was inserted from the right femoral vein into the IVC for emergency balloon insertion during bleeding. A laparoscopic tumor resection was performed. A histopathological examination confirmed the diagnosis of primary retroperitoneal ganglioneuroma. CONCLUSIONS: The treatment of symptomatic retroperitoneal tumors requires a multidisciplinary approach.

Primary retroperitoneal müllerian adenocarcinoma: a case report.

A 45-year-old woman presented with right hip pain for a month. Imaging results revealed that the left peritoneal mass was accompanied by metastases of the right sciatic branch, lung, and retroperitoneal lymph nodes. A biopsy of the left peritoneal mass was performed. The pathological morphology demonstrated clear cell adenocarcinoma. Immunohistochemical staining revealed a positive expression of keratin7 and PAX8 and a negative expression of keratin20, GCDFP-15, ER, PR, WT1, CDX2, villin, TTF-1, napsin-A, vimentin, calretinin, and GATA3. Finally, the diagnosis of primary retroperitoneal müllerian adenocarcinoma (PRMA) was confirmed. PRMA is a very rare type of primary retroperitoneal tumor. PRMA should be considered for the retroperitoneal mass.

Retroperitoneum revisited: a review of radiological literature and updated concept of retroperitoneal fascial anatomy with imaging features and correlating anatomy.

PURPOSE: Spread of disease in the retroperitoneum is dictated by the complex anatomy of retroperitoneal fasciae and is still incompletely understood. Conflicting reports have led to insufficient and incorrect anatomical concepts in radiological literature. METHODS: This review will discuss previous concepts prevalent in radiological literature and their shortcomings will be highlighted. New insights from recent anatomical and embryological research, together with imaging examples, will be used to clarify patterns of disease spread in the retroperitoneum that remain unexplained by these concepts. RESULTS: The fusion fascia and the renal fascia in particular give rise to planes and spaces that act as vectors for spread of disease in the retroperitoneum. Some of these planes and structures, such as the caudal extension of the renal fascia, have previously not been described in radiological literature. CONCLUSION: New insights, including the various fasciae, potential spaces and planes, are incorporated into an updated combined retroperitoneal fascial concept.

Optimizing surgical strategies for retroperitoneal liposarcoma: a comprehensive evaluation of standardized aggressive surgical policies.

BACKGROUND: Retroperitoneal liposarcoma (RLPS) constitutes the majority of retroperitoneal sarcomas. While surgical resection remains the sole curative approach, determining the optimal surgical strategy for RLPS remains elusive. This study addresses the ongoing debate surrounding the optimal surgical strategy for RLPS. METHODS: We recruited 77 patients with RLPS who underwent aggressive surgical policies. Patients were categorized into three surgical subtypes: suprapancreatic RLPS, pancreatic RLPS, and subpancreatic RLPS. Our standardized surgical strategy involved resecting macroscopically uninvolved adjacent organs according to surgical subtypes. We collected clinical, pathological and prognostic data for analyses. RESULTS: The median follow-up was 45.5 months. Overall survival (OS) and recurrence-free survival (RFS) were significantly correlated with multifocal RLPS, pathological subtype, recurrent RLPS and histological grade (P for OS = 0.011, 0.004, 0.010, and < 0.001, P for RFS = 0.004, 0.001, < 0.001, and < 0.001, respectively). The 5-Year Estimate OS of well-differentiated liposarcoma (WDLPS), G1 RLPS, de novo RLPS and unifocal RLPS were 100%, 89.4%, 75.3% and 69.1%, respectively. The distant metastasis rate was 1.4%. The morbidity rates (≥ grade III) for suprapancreatic, pancreatic, and subpancreatic RLPS were 26.7%, 15.6%, and 13.3%, respectively. The perioperative mortality rate is 2.6%. CONCLUSIONS: Standardized aggressive surgical policies demonstrated prognostic benefits for RLPS, particularly for G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS. This approach effectively balanced considerations of adequate exposure, surgical safety, and thorough removal of all fat tissue. G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS could be potential indications for aggressive surgical policies.

A Rare Case of Retroperitoneal Tumefactive Fibroinflammatory Lesion Related to IgG4-Sclerosing Disease.

We present a unique case of a retroperitoneal tumefactive fibroinflammatory lesion related to IgG4-sclerosing disease; it is a rare manifestation of the IgG4-related disease, which usually causes diffuse fibrosis when located in the retroperitoneum, rather than mass-like lesions. A 49-year-old man presented to the emergency department complaining of abdominal pain and vomiting. Subsequent testing with abdominal ultrasound, CT, and MRI revealed a large retroperitoneal mass of unknown origin, heterogenous, with a concentric circles pattern best visualized in MRI. The lesion was resected, and the histological and immunohistochemical studies revealed an IgG4-related tumefactive fibroinflammatory lesion of the retroperitoneum.

Torsed retroperitoneal leiomyomas: A case report and review of literature.

BACKGROUND: Retroperitoneal leiomyomas (RLs) are rare benign tumours that can occur in the pelvic and/or abdominal parietal retroperitoneum. Once torsion occurs, it causes acute abdominal pain and can even lead to serious consequences such as gangrene, peritonitis, haemoperitoneum and shock if not identified and treated promptly. Therefore, a better understanding of the characteristics of RL torsion is needed. Here, we present a case of acute pedicle torsion of an RL in the posterior peritoneum followed by a literature review. CASE SUMMARY: Herein, we report the case of a 42-year-old woman with RL torsion. The patient visited our hospital complaining of lower abdominal pain for 6 d. Pelvic examination revealed a tender mass superior to the uterus. Pelvic magnetic resonance imaging (MRI) revealed an anterior uterine mass, multiple uterine fibroids and slight pelvic effusion. MRI suggested the possibility of a subserosal myoma of the anterior uterine wall with degeneration. Intraoperative exploration revealed a 10 cm pedunculated mass arising from the posterior peritoneum, with the pedicle torsed two times. Pathological examination confirmed a torsed RL. CONCLUSION: In the case of a pelvic mass complicated with acute abdomen, the possibility of torsion should be considered.

Hydatid cyst at unusual locations: Report of three cases.

INTRODUCTION: Hydatidosis is an anthropozoonosis due to the development in humans of the larval form of Echinococcus granulosus and is endemic in many countries of the Mediterranean region such as Morocco. CASES PRESENTATION: We report three cases of hydatid cyst at unusual locations such as the peritoneum, and the retroperitoneum. DISCUSSION: Hydatid disease usually involves the liver (75 %), the lungs (15.4 %), and the spleen (5.1 %). Almost any anatomic location can be the host site of the parasitic cysts. CONCLUSION: Multiple locations of hydatid cyst often pose a problem of differential diagnosis. Surgery is the mainstay of treatment.

Clinical Outcomes and Safety of Transcatheter Arterial Embolization in Patients with Traumatic or Spontaneous Psoas and Retroperitoneal Hemorrhage.

Background: We aimed to assess the effectiveness and safety of transcatheter arterial embolization (TAE) in the management of spontaneous or traumatic psoas and/or retroperitoneal hemorrhage. Methods: This single-center retrospective study enrolled 36 patients who underwent TAE for the treatment of psoas and/or retroperitoneal hemorrhage between May 2016 and February 2024. Results: The patients' mean age was 61.3 years. The spontaneous group (SG, 47.1%) showed higher rates of anticoagulation therapy use compared with the trauma group (TG, 15.8%) (p = 0.042). The TG (94.7%) demonstrated higher survival rates compared with the SG (64.7%; p = 0.023). Clinical failure was significantly associated with the liver cirrhosis (p = 0.001), prothrombin time (p = 0.004), and international normalized ratio (p = 0.007) in SG and pRBC transfusion (p = 0.008) in TG. Liver cirrhosis (OR (95% CI): 55.055 (2.439-1242.650), p = 0.012) was the only identified independent risk factor for primary clinical failure in the multivariate logistic regression analysis. Conclusions: TAE was a safe and effective treatment for psoas and/or retroperitoneal hemorrhage, regardless of the cause of bleeding. However, liver cirrhosis or the need for massive transfusion due to hemorrhage increased the risk of clinical failure and mortality, necessitating aggressive monitoring and treatment.

Maximizing Success: An Overview of Optimizing the Ovarian Tissue Transplantation Site.

Ovarian tissue cryopreservation and transplantation (OTCT) has emerged in recent years as a potential method for reversing abnormal endocrine and reproductive functions, particularly in patients receiving gonadotoxic cancer treatments having longer survival rates. From its first rodent experiments to human trials, OTCT has evolved tremendously, opening new windows for further utilization. Since then, significant progress has been achieved in terms of techniques used for surgical removal of the tissue, optimal fragment size, freezing and thawing procedures, and appropriate surgical sites for the subsequent reimplementation of the graft. In addition, various approaches have been proposed to decrease the risk of ischemic injury, which is the leading cause of significant follicle loss during neo-angiogenesis. This review aims to discuss the pros and cons of ovarian and retroperitoneal transplantation sites, highlighting the justifications for the viability and efficacy of different transplantation sites as well as the potential advantages and drawbacks of retroperitoneal or preperitoneal area.

Retroperitoneal leiomyosarcoma mimicking an ovarian tumor diagnosed using a negative ovarian pedicle sign.

Retroperitoneal leiomyosarcoma (RPLMS) is rare and usually presents as a large abdominal mass with poor clinical symptoms. Radiological findings of an RPLMS arising in the pelvis of a woman resemble those of adnexal tumors. Herein, we present a case of RPLMS mimicking an adnexal tumor which was differentiated from having an ovarian origin as the right ovarian vein was passing through the tumor but there was no direct vascular connection with the tumor. Therefore, it is important to identify the ovarian vein to distinguish between these tumors.

Imaging Diagnosis of Retroperitoneal Müllerian Duct-derived Cyst Complicated by Diaphragmatic Hernia: A Case Report.

BACKGROUND: This case report describes a case of Müllerian duct cyst that occurred in a male retroperitoneum. The cyst lesion is rare and complicated with diaphragmatic hernia. Müllerian duct-derived cyst is a rare developmental disorder that is more common in male pelvic tissues and rare in the retroperitoneum. We investigated the important role of computerized tomography (CT) and magnetic resonance imaging (MRI) in preoperative diagnosis and disease prediction of this condition. CASE PRESENTATION: A 25-year-old male was found to have an abnormal occupying lesion in the left diaphragm in imaging examinations, usually healthy with no obvious clinical symptoms. X-ray examination showed a circular, high-density shadow near the left diaphragm. CT scan showed a soft tissue density shadow resembling a tumor in the left adrenal area, irregularly protruding into the chest cavity, with uneven density. MRI examination showed an irregular elongated T1 and T2 signal shadow in the left adrenal area. T2 fat suppression showed high signal intensity with unrestricted diffusion. Robotic-assisted laparoscopic surgery showed left retroperitoneal tumor resection. The patient recovered well postoperatively and had no recurrence after discharge follow-up. CONCLUSION: The preclinical symptoms of retroperitoneal Müllerian cysts complicated by diaphragmatic hernia in young men are difficult to distinguish, and it is difficult to diagnose other similar cysts with imaging. The method of combined CT and MRI diagnosis guides the endoscopic robot-assisted minimally invasive surgery for excision of cysts to achieve accurate diagnosis and treatment of such diseases.

Spontaneous retroperitoneal hematoma treated with transarterial embolization: a systematic review and metanalysis.

PURPOSE: The purpose of this systematic review and meta-analysis was to evaluate the safety, technical, and clinical effectiveness of percutaneous Transarterial Embolization (TAE) in treating spontaneous retroperitoneal hematomas as well as assess treatment outcomes in patients who underwent target or empirical embolization. MATERIALS AND METHODS: Through the PubMed, Embase, and Google Scholar databases, an extensive search was performed in the fields of spontaneous retroperitoneal hematomas treated with transcatheter arterial embolization. We collected pooled data on 141 patients from 6 separate articles selected according to the inclusion and exclusion criteria. RESULTS: Technical success rate was 100% in all six studies, for both targeted and empirical embolization. The clinical success rate varied from 56.3 to 89.5%. The total number of complications related to the embolization procedure was 10 events out of 116 procedures analyzed. Empirical or empirical embolization was performed in three studies, where the source of active bleeding was not evident during DSA. A meta-analysis compared the rebleeding rates between targeted and empirical embolization groups. The odds ratio from pooled data from the three assessed studies (72 patients) showed no significant difference in rebleeding rates after empirical TAE compared with targeted TAE. CONCLUSIONS: TAE is a safe, effective, and potentially life-saving procedure for the treatment of life-threatening spontaneous retroperitoneal hematomas. Empirical and targeted TAE procedures demonstrate a relatively low risk of complications, compared to the high technical and relatively high clinical success rates.

Clinicopathological and molecular genetic analysis of 13 cases of primary retroperitoneal Ewing sarcoma.

Retroperitoneal Ewing sarcomas (RES) are very rare and mostly described in case reports. The purpose of this study was to retrospectively analyze the clinicopathology, molecular characteristics, biological behavior, and therapeutic information of 13 cases of primary RES with immunohistochemical staining, fluorescence in situ hybridization, RT-PCR and NGS sequencing detection techniques. The thirteen patients included eight males and five females with a mean age of 34 years. Morphologically, the tumors were comprised of small round or epithelial-like cells with vacuolated cytoplasm (6/13,46 %) arranged in diffuse, nested (8/13,62 %) and perivascular (7/13,54 %) patterns. Unusual morphologic patterns, such as meningioma-like swirling structures and sieve-like structures were relatively novel findings. Immunohistochemical studies showed CD99 (12/13; 92 %), CD56 (11/13; 85 %), NKX2.2 (9/13; 69 %), PAX7 (10/11;91 %) and CD117(6/9;67 %) to be positive.12 cases (92 %) demonstrated EWSR1 rearrangement and 3 cases displayed EWSR1::FLI1 fusion by FISH. ERCC4 splice-site variant, a novel pathogenic variant, was discovered for the first time via RNA sequencing. With a median follow-up duration of 14 months (6 to 79 months), 8/13 (62 %) patients died, while 5/13(38 %) survived. Three cases recurred, and five patients developed metastasis to the liver (2 cases), lung (2 cases) and bone (1 case). RES is an aggressive, high-grade tumor, prone to multiple recurrences and metastases, with distinctive morphologic, immunohistochemical, and molecular genetic features. ERCC4 splicing mutation, which is a novel pathogenic variant discovered for the first time, with possible significance for understanding the disease, as well as the development of targeted drugs.