Search

A 76-year-old woman with a history of radiation therapy for vaginal cancer was referred to our hospital because of fever and hepatobiliary dysfunction. Computed tomography showed stenosis of the lower bile duct and edema-like changes in the duodenum from the descending to transverse parts. Endoscopic biliary stenting was performed according to the rendezvous method. Squamous cell carcinoma, similar to vaginal cancer, was found on pathological examination of the duodenum. We accordingly diagnosed obstructive jaundice and duodenal stenosis caused by vaginal cancer and retroperitoneal metastasis. To the best of our knowledge, other such cases have not been reported.

The main objective is to establish the overall survival and disease-free survival profiles regarding the patients with retroperitoneal liposarcoma, making a comparison based on the well-differentiated and dedifferentiated histological subtypes. The secondary objectives are to descriptively analyze the clinical characteristics of said patients and to identify and analyze other independent variables that might modify these survival profiles significantly.An observational and analytical study was performed using a retrospective historical cohort that was followed prospectively. The inclusion criteria consisted of: the procedure of liposarcoma located in the retroperitoneum, the well-differentiated and dedifferentiated histological subtypes, between January of 2002 and May of 2019. As a result, 32 patients took part in this study's sample. Kaplan-Meier estimator was used to summarise the results and log-rank test was used in the comparative analysis.The overall survival at 5 years was around 59%. No differences were found between the patients with a well-differentiated subtype compared to the dedifferentiated ones (p = 0.834). The disease-free survival at 2 years was 59% regarding the well-differentiated and 26% regarding the dedifferentiated, with these differences being statistically significant (p = 0.005). None of the other studied variables modified these survival profiles significantly.Dedifferentiated retroperitoneal liposarcomas show less disease-free survival than well-differentiated liposarcomas. However, regarding overall survival no differences can be claimed.

Retroperitoneal soft-tissue sarcoma is a very rare neoplasm, the most frequent histological subtype is liposarcoma with up to 45% of all cases. Unspecific clinical presentation, late diagnosis and high local recurrence rate represent important problems in clinical practice. We present the case of an adult patient with an unusual large liposarcoma of the retroperitoneum analyzing diagnostic workup, surgical approach and therapeutic strategies.A 68-years old female was admitted with weight gain (+12 kg) and increasing abdominal girth. Computed tomography scan imaging showed a retroperitoneal tumor with 40 cm maximum diameter. Biopsy revealed a myxoid liposarcoma. The interdisciplinary curative surgical treatment included preoperative ureteral splinting, en-bloc tumorexstirpation, ileocecal resection, right ureteral resection and vascular reconstruction of the Arteria iliaca communis. The postoperative course was uneventful. After sixteen months the patient developed multifocal local recurrence requiring extensive surgical resection of tumor and retroperitoneal fat (Figs. 3, 4). However, thirteen months later the tumor reappeared and the patient was assigned to palliative chemotherapy. The patient is still alive with stable tumor disease.The removal of a huge retroperitoneal sarcoma is a significant challenge for the surgeon. Accurate planning, interdisciplinary treatment options, and radical surgery are essential. However, the recurrence risk is exceptionally high because of the enormous tumor dimensions and the big tumor surface, multimodal therapeutic approaches may improve the outcome in these patients.Liposarcoma, Retroperitoneum, Surgery.Il sarcoma retroperitoneale è un tumore molto raro, il tipo istologico più frequente (45 %) è il liposarcoma. Per l’assenza di sintomi specifici e per la diagnosi spesso tardiva si tratta frequentemente di lesioni piuttosto voluminose, in grado di raggiungere dimensioni notevoli occupando gran parte dell’addome e dislocando organi e strutture circostanti. La terapia standard è l’asportazione chirurgica completa del tumore, che determina significativamente la sopravvivenza a lungo termine. Il rischio oncologico più importante consiste nello sviluppo di una recidiva locale. Progettazione accurata (con o senza biopsia preoperatoria) e resezione radicale, preferibilmente una resezione dell’intero compartimento retroperitoneale coinvolto, rappresentano gli elementi centrali dal punto di vista del chirurgo. Nel caso clinico proposto, viene descritto il decorso di una paziente con un grande liposarcoma retroperitoneale con un diametro di 40 cm trattato con resezione multiviscerale interdisciplinare. In base a questo caso analizziamo e discutiamo l’iter diagnostico e terapeutico di sarcomi retroperitoneali giganteschi.

BACKGROUND Our study aimed to compare the comprehensive complication index (CCI) to the conventional Clavien-Dindo classification (CDC) in patients undergoing surgery for primary retroperitoneal sarcoma (RPS). METHODS Data were collected for patients who underwent surgery from 2008 to 2019 at a tertiary institution. The length of hospital stay (LOS) was used as a surrogate marker for clinical outcomes, and associations with CDC and CCI were assessed. RESULTS Data were available for 191 patients, with the highest CDC Grade of I, II, III, and IV in 18.3%, 41.9%, 17.8%, and 4.2%, respectively; the 30-day postoperative mortality (CDC Grade V) was 1.6% (N = 3). Whilst both classification systems were significantly correlated with LOS, this association was significantly stronger for CCI (Spearman's ρ: 0.768 vs. 0.648, p

Background Neuroblastoma (NB) is the most common extracranial solid tumor in children. It is known for high heterogeneity and concealed onset. In recent years, the mechanism of its occurrence and development has been gradually revealed. The purpose of this study is to summarize the clinical characteristics of children with NB and abnormal chromosome 10, and to investigate the relationship between the number and structure of chromosome 10 abnormalities and NB prognosis. Methods Chromosome G-banding was used at the time of diagnosis to evaluate the genetics of chromosomes in patients with NB and track their clinical characteristics and prognosis. All participants were diagnosed with NB in the Medical Oncology Department of the Beijing Children’s Hospital from May 2015 to December 2018 and were followed up with for at least 1 year. Results Of all 150 patients with bone marrow metastases, 42 were clearly diagnosed with chromosomal abnormalities. Thirteen patients showed abnormalities in chromosome 10, and chromosome 10 was the most commonly missing chromosome. These 13 patients had higher LDH and lower OS and EFS than children with chromosomal abnormalities who did not have an abnormality in chromosome 10. Eight patients had both MYCN amplification and 1p36 deletion. Two patients had optic nerve damage and no vision, and one patient had left supraorbital metastases 5 months after treatment. Conclusions The results indicated that chromosome 10 might be a new prognostic marker for NB. MYCN amplification and 1p36 deletion may be related to chromosome 10 abnormalities in NB. Additionally, NB patients with abnormal chromosome 10 were prone to orbital metastases.

The quality of the initial management of sarcomas is fundamental because it conditions the patient's quality of life and his overall survival. Radiotherapy should be discussed in a multidisciplinary consultation meeting within the framework of the Netsarc+network. The place of radiotherapy in patients with soft tissue or bone sarcoma depends on the histology and tumour location, knowing that it is most often associated with surgery which remains the main treatment. It is part of the standard treatment for grade II and III deep limb sarcomas of 5cm or greater in size and Ewing's sarcomas. In these indications, conformal radiotherapy with modulation of intensity is used routinely, in combination with IGRT. In other locations, such as retroperitoneal sarcomas or uterine sarcomas, radiotherapy is not a standard of care and must be discussed according to the prognostic criteria related to the patient, the tumour, and the previously received treatments. New techniques, such as proton therapy, hadron therapy (carbon ions) are techniques particularly suited to bone sarcomas considered to be radioresistant. However, large prospective trials are lacking in these rare indications, explaining the lack of recommendations of a high level of evidence.

Soft tissue sarcomas (STS) are rare, aggressive, and heterogenous tumors, comprising approximately 1% of adult cancers with over 50 different subtypes. The mainstay of treatment for retroperitoneal sarcomas (RPS) includes surgical resection. The addition of radiation therapy (RT), either preoperatively or postoperatively, has been used to potentially decrease the risk of local recurrence. The recently published results from STRASS (EORTC-STBSG 62092-22092), which randomized patients to receive or not receive preoperative radiation, indicate no abdominal recurrence-free survival benefit (primary endpoint) nor overall survival benefit to date from the addition of preoperative RT prior to surgical resection in patients with RPS. Keeping in mind caveats of subgroup analyses, the data show a significant reduction in local recurrence with radiation therapy in resected patients and non-significant trends toward improved abdominal recurrence-free survival in all patients and improved local control and abdominal recurrence-free survival in patients with liposarcoma and low-grade sarcoma. Given the high rate of local failure with surgery alone, it is possible that higher RT dose and/or selective RT dose painting may improve outcomes. Prior to treatment, the authors encourage multidisciplinary review and discussion of management options at a sarcoma center for patients with RPS. Selective use of RT may be considered for patients at high risk of local recurrence.

Patient: Female, 29-year-old Final Diagnosis: Ganglioneuroma Symptoms: Asymptomatic • incidental finding Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: Ganglioneuromas (GNs) are benign neuroblastic tumors. These extra-cranial solid tumors are common in childhood but unusual in adults. Patients with GNs typically do not have any symptoms and the tumors usually are incidental findings. However, if a GN is large enough to compress adjacent organs, complications can occur. Furthermore, even in patients who have incomplete resection of a GN, long-term survival rates are high. After a GN is seen on imaging, the diagnosis usually is made with a biopsy and treatment is with surgery alone. Case Report: A 29-year-old woman was referred to General Surgery from the Gynecology Clinic for an incidental finding of an abdominal mass on routine ultrasound for secondary infertility and admitted for an investigation. Abdominal and pelvic computed tomography (CT) and magnetic resonance imaging showed a retroperitoneal mass that measured 6.318×22 cm arising from the paravertebral region with intraspinal extension. The mass was displacing the patient’s thoracic aorta, abdominal inferior vena cava, and ureters. A CT-guided biopsy revealed a GN. Debulking surgery was performed and a small amount of residual tumor was left in the paravertebral nerve roots. The patient recovered well with no complications. The diagnosis of GN was confirmed with pathology, which was reviewed by the Tumor Board; the Board agreed that only follow-up in the Surgery Clinic was needed. During the patient’s last visit, 10 months after surgery, a follow-up CT scan showed that the residual tumor was stable. Conclusions: GNs are benign abdominal and retroperitoneal tumors that are typically asymptomatic and detected incidentally. Surgical resection is the treatment of choice and even when it is incomplete, the prognosis for patients is excellent.

Background Primary retroperitoneal serous adenocarcinoma (PRSA) is a rare malignant disease. Given the rarity of the disease, the imaging features of PRSA are unclear. Contrast-enhanced ultrasound (CEUS) also plays an important role in the evaluation of the differential diagnosis of retroperitoneal lesions. Case presentation We report the case of a 62-year-old woman of with increased CA125 levels for 1 year who was referred to our hospital. After conducting contrast-enhanced computed tomography and magnetic resonance imaging, the mass was misdiagnosed as a chocolate cyst. After transvaginal ultrasound (TUS) combined with CEUS, cystadenocarcinoma was considered as the initial diagnosis. Pathology results confirmed PRSA as the final diagnosis. Conclusions CEUS features of PRSA are reported for the first time based on this case, potentially aiding in the differential diagnosis of this rare entity before surgery.

Purpose of review The aim is to bring latest evidence of the role of perioperative chemotherapy in localized soft-tissue sarcomas (STS) of limbs and to review the risk classification systems of retroperitoneal and extremity STS. Recent findings High-risk subset of localized STS of limbs and trunk-wall, defined with classic prognostic factors, consistently obtained 5-year overall survival ranging from 69 to 76% in randomized patients treated with full-dose of anthracycline and ifosfamide. Validated nomograms accurately predict, on individual basis, the risk of death and recurrence in localized STS of retroperitoneum and limbs, enabling a better selection of high-risk patients (usually those with death risk of ≥40%) that potentially could benefit with perioperative systemic treatment. Nomograms have virtually converted a negative large perioperative trial into a positive, favouring chemotherapy arm in the high-risk selection. Summary Perioperative three cycles of full-dose of anthracycline and ifosfamide should be proposed on an individual basis, in reference sarcoma centres, to high-risk localized STS of limbs or trunk-wall in certain histologies.

Introduction and importance The solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin, with a reported incidence of 2.8 cases per 100,000 tumors and with distinctive histopathological and immunohistochemical characteristics. It was initially described as a pleural lesion and subsequently, it was found in different organs and tissues. The abdominoinguinal incision described by Karakousis allows a safe and radical approach for lower quadrants abdominopelvic tumors. Case presentation A 47-year-old man was referred to us with a 5-months history of lower backache radiating to the left lower limb. MRI and CT revealed a retroperitoneal mass of 10 cm extending to left iliac vessels. The initial diagnosis corresponded to a sarcomatous retroperitoneal tumor. It was decided to perform an abdominal exploration using Karakousis's approach for surgical resection. The immunohistochemistry and histopathological study revealed neoplasia compatible with a SFT. It was categorized as low risk for developing metastasis and death from disease, according to the new malignancy criteria. Currently, the patient is asymptomatic and disease-free at 19 months after surgery. Clinical discussion Most patients with SFTs present symptoms derived from the tumor growth and the compression on adjacent structures with clinical manifestations that are frequently insidious and precede the tumor discovery. The diagnosis is based on histopathological studies. Nonetheless, when they present an extrathoracic location, they represent a diagnostic challenge, due to their variable histological characteristics. Conclusion Presacral SFT is a rare entity, with a scant incidence reported regarding this location and long-term treatment. Surgical resection is needed as the immediate treatment., Highlights • A solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin. • SFT requires immediate treatment with surgical resection. • The Karakousis abdominoinguinal incision allows a safe and radical approach. • The presacral SFT with extension to iliac vessels benefits from the Karakouis approach.

Little level 1 evidence exists to guide multimodality treatment in retroperitoneal soft tissue sarcoma (RPS), which may lead to significant variation in therapeutic approaches. This analysis aimed to describe national RPS treatment trends and explore potential variability among low-/high-volume hospitals (LVH/HVHs).In total, 5992 patients who underwent resection for primary RPS were retrospectively identified in the National Cancer Database (2004-2017). Time trend analyses examined rates of multivisceral resection (MVR), radiation, and chemotherapy use. LVHs were defined as those carrying out fewer than ten resections per year (N = 5433), whereas HVHs were defined as those carrying out ten or more (N = 559). Descriptive statistics and logistic regression models compared trends between groups.MVR was more frequent at HVHs (63.7% versus 43.5%, p0.001). Use of radiation varied widely by hospital volume. HVHs more frequently employed preoperative radiation as compared with LVHs (14.7% versus 8.1%, p0.001). Throughout the study period, LVHs increased utilization of preoperative radiation (2.6% to 12.0%, p0.001) whereas rates at HVHs remained stable. Overall, LVHs utilized postoperative radiation significantly more frequently as compared with HVHs (14.7% versus 2.7%, respectively, p0.001). Postoperative radiation at LVHs remained stable until 2013 and sharply declined thereafter (16.7% to 6.9%, p0.001). Rates of postoperative radiation use at HVHs remained lower than those at LVHs at all time points.Strategies for resection and radiation use at LVHs have trended towards those of HVHs. Current national implementation of preoperative radiation, MVR, and chemotherapy remains heterogeneous. These findings inform future trial design and support standardization of care.

Lymphangiomas are classified as lymphatic malformations, which are more common in children and rare in adults. It frequently occurs in the cervical and axillary regions and uncommonly in the retroperitoneum. A 39-year-old woman presented to our department for the investigation for a 55 mm asymptomatic mass in the right anterior adrenal cavity. Abdominal ultrasound showed a tumor containing cysts in the right anterior adrenal cavity. Contrast-enhanced computed tomography showed that the tumor was poorly contrasted and ill-defined. Magnetic resonance imaging suggested that the tumor contained a small amount of fat. The tumor tended to grow, and the possibility of malignant diseases such as liposarcoma could not be excluded. Therefore, surgical resection was performed. Since intraoperative findings showed that the tumor tightly invaded to the duodenum and pancreatic head, a pancreaticoduodenectomy was selected. The entire tumor was removed without exposing the tumor. Macroscopic findings indicated that the specimen was 55 mm in size, indistinctly demarcated, yellow–white in color, and polycystic. Histologically, lymphovascular proliferation was observed with infiltration of the pancreatic head and the duodenal muscle layer. The diagnosis of lymphangioma was finally made. There was no recurrence 2 years after surgery.

Background The prognostic significance of histological organ involvement by retroperitoneal sarcoma subtype is unknown. The present study aimed to describe organ involvement across the subtypes, and the implications for survival. Methods Patients undergoing surgery for primary retroperitoneal sarcoma at the Queen Elizabeth Hospital, Birmingham from April 2005 to September 2018 were identified retrospectively. Histological organ involvement was classed as pushing, infiltrative or neither. Univariable and multivariable Cox regression models were produced to analyse the association between histological organ involvement and both overall (OS) and recurrence-free (RFS) survival for the cohort as a whole, and by histological subtype. Results Data were available for a total of 197 patients, of whom 171 (86.8 per cent) had at least one organ resected. Infiltrative organ behaviour was seen in 37 patients (18.8 per cent), and pushing behaviour in 67 (34.0 per cent). For the cohort as a whole, infiltration (hazard ratio (HR) 4.32, 95 per cent c.i. 2.35 to 7.93; P < 0.001), but not pushing (HR 1.62, 0.90 to 2.92; P = 0.106), was associated with significantly shorter OS, in comparisons with a group with neither of these behaviours. However, this effect was found to differ significantly by histological subtype (P = 0.009). For patients with dedifferentiated liposarcoma, there was no significant association between tumour behaviour and either OS (P = 0.508) or RFS (P = 0.313). However, in leiomyosarcoma, infiltrative behaviour was associated with shorter OS (P = 0.002), and both infiltrative (P < 0.001) and pushing (P = 0.010) behaviours were associated with shorter RFS, compared with tumours with neither behaviour. Multivariable analyses of both OS and RFS returned similar results. Conclusion The prognostic implications of organ involvement in retroperitoneal sarcoma vary by histological subtype., The prognostic significance of histological organ involvement by retroperitoneal sarcoma subtype is unknown. The present study described organ involvement across the subtypes, and the implications for survival. The prognostic implications of organ involvement in retroperitoneal sarcoma were found to vary by histological subtype.

Background A novel Palladium-103 low-dose rate (LDR) brachytherapy device was developed to provide dose-escalation to the tumor bed after resection while shielding adjacent tissues. This multicenter report describes the initial experience with this device in patients with retroperitoneal sarcoma (RPS). Materials and methods Patients with recurrent RPS, prior radiotherapy, and/or concern for positive margins were considered. An LDR brachytherapy dose of 20-60 Gy was administered, corresponding to biologically effective dose values of 15-53 Gy and equivalent dose values of 12-43 Gy. Results Six patients underwent implantation at four institutions. Of these, five had recurrent disease in the retroperitoneum or pelvic sidewall, one had untreated locally advanced leiomyosarcoma, two had prior external beam radiation therapy at the time of initial diagnosis, and four received neoadjuvant external beam radiation therapy plus brachytherapy. The device was easily implanted and conformed to the treatment area. Median follow-up was 16 mo; radiation was delivered to the at-risk margin with minimal irradiation of adjacent structures. No local recurrences at the site of implantation, device migration, or radiation-related toxicities were observed. Conclusions The novel LDR directional brachytherapy device successfully delivered a targeted dose escalation to treat RPS high-risk margins. Lack of radiation-related toxicity demonstrates its safety.

Rationale: Renal-occupying lesions positive for urine fluorescence in situ hybridization (FISH) are usually considered urothelial carcinomas. Here, we describe 2 cases of renal metastases with chromosome duplications in urine exfoliated cells. Patient symptoms: Patient 1, a 56-year-old male with a history of esophageal cancer, was admitted to our hospital on May 2017 after presenting with right back pain with microscopic hematuria for 1 month. Magnetic resonance imaging (MRI) showed right renal space-occupying lesions (5.4 cm × 4.6 cm) and multiple enlarged lymph nodes in the right renal hilum and retroperitoneum. The cystoscopy results were negative, and FISH analysis of urine exfoliated cells was positive, indicative of chromosome 3, 7, and 17 amplifications. Patient 2 was a 50-year-old male who was admitted to our hospital on May 2019 with no obvious cause of abdominal pain and abdominal distension (lasting for 7 days), with a serum creatinine level of 844 μmol/L. Patient 2 had no hematuria or fever, and MRI showed left renal inferior and medial space-occupying lesions, and multiple mesenteric nodules at the junction of the left adrenal gland, retroperitoneum, abdomen, and pelvis, which were partially fused. The tumor lesions were approximately 3.1 cm × 2.3 cm in size. The urine FISH results were positive, indicating chromosome 3, 7, and 17 amplifications. Diagnoses: Both patients were diagnosed with renal tumors with unknown pathology. Interventions: Patient 1 underwent laparoscopic resection of the kidney and ureter, and sleeve cystectomy. The postoperative pathological diagnosis was metastatic keratinized squamous cell carcinoma, with squamous cell carcinoma in the right hilar lymph node. Histological FISH of the primary esophageal cancer and renal metastases were consistent with the urine FISH test results. Patient 2 underwent a biopsy of the left renal inferior and retroperitoneal areas, and was diagnosed with diffuse large B-cell lymphoma. Outcomes: Patient 1 survived 6 months after urological surgery. After treating patient 2 with the R-CHOP regimen and kinase inhibitors, his renal function recovered significantly and the mass become undetectable. Lessons: Our results imply that FISH-positive renal occupying lesions should be considered as potential renal metastases with chromosome aberrations when making a differential diagnosis.

Introduction: Testicular cancers represent about 5% of all urological malignancies and 1-1.5% of all male neoplasms. Most of the testicular cancers are localized (68%) at diagnosis. Bulky masses in the scrotum are rare. We present a rare case of bulky testicular cancer with retroperitoneal spread through the inguinal canal. Case report: A 44-year-old man came to the emergency department referring weakness and the presence of a scrotal mass. At physical examination, a voluminous mass was found, with necrotic phenomena within the scrotum. Abdomen was tense and sore. Abdominal CT scan revealed a bulky testicular mass spreading to the retroperitoneal space through the inguinal canal with node enlargement. Patient underwent orchiectomy with excision of infiltrated scrotum skin. Histologic diagnosis confirmed a typical form seminoma. The patient was then treated with a cisplatin-based chemotherapy, with a partial response. The patient recently relapsed and he is being treated with a new line of chemotherapy and subsequent surgery with or without radiotherapy. Conclusions: We described a rare presentation of testicular cancer. This case highlights the importance of a multidisciplinary approach to rare testis tumour presentation and early diagnosis for testicular cancers.

Ratioanle: Interdigitating dendritic cell sarcoma (IDCS) is a rare sarcoma that originates from interdigitating dendritic cells in lymphoid tissue, the imaging characteristics of which are poorly defined. Pathological examination can identify the tumor, but reports on the imaging characteristics of IDCS are limited. Patient concerns: Here, we report a case of IDCS in a 48-year-old female involving the retroperitoneal area. The patient had a lumbar mass on her right lower back for 4 years, and which started increasing in size 1 year before. Diagnoses: An irregular soft tissue mass (10.1cm × 8.5 cm in size) in the right lower back of retroperitoneum was detected by CT examination with unclear borders, uneven density, and necrosis. The solid components of the mass were significantly enhanced on postcontrast imaging. The soft tissue was irregular and uneven. Cystic solid masses were observed on MRI examination in the right retroperitoneum, lateral abdominal wall, waist, and back. Necrosis, hemorrhage, and cystic transformation were observed inside the lesion. The cyst wall, separation, and wall nodules were significantly enhanced on the postcontrast image. No distant metastasis was observed. Postoperative pathology confirmed the diagnosis of IDCS. Interventions: The patient underwent surgical resection. The resected margin was positive, and the patient received adjuvant radiotherapy 2 months after the surgery. Outcomes: Twelve months after radiotherapy, the patient's chest CT showed multiple metastases in both lungs. The patient was started on combination chemotherapy of doxorubicin and ifosfamide, and the follow-up is still ongoing. Lessons: Imaging provides a unique advantage to determine the extent of the IDCS, the invasion of adjacent tissues, and the presence or absence of distant metastases.

Extrauterine leiomyoma occasionally occurs in rare locations with unusual growth patterns, especially pelvic retroperitoneal leiomyoma, which brings great challenges for surgeons to make a diagnosis. It is essential to distinguish benign from malignant retroperitoneal neoplasms according to the imaging manifestations. Laparotomy and laparoscopy are the common options for pelvic retroperitoneal neoplasms, while they may cause side effects during operation such as secondary damage. Appropriate surgical techniques should be adopted to ensure the complete excision of neoplasms meanwhile preserve the urination, defecation, and sexual function.A 30-year-old woman was referred to our hospital because of dull pain in the perianal region for 1 month. Laboratory results including tumor markers were all within normal limits. The digital rectal examination revealed a huge and tough mass with smooth mucosa protruding into the rectal cavity from the rear area of rectum.Imaging examinations were performed. Contrasted computed tomography (CT) of pelvis showed an enhanced retroperitoneal solid mass in the space between sacrum and rectum, and very close to the levator ani muscle. The mass was about 11.0*8.0 cm in size. Computerized tomography angiography (CTA) showed the distal branches of bilateral internal iliac artery went into the mass. Endoscopic ultrasonography (US) showed the mass compressed the rectum, as well as a clear boundary to the rectal wall. A histopathologic examination confirmed the mass was a pelvic retroperitoneal leiomyoma.The patient underwent an operative resection with da Vinci Si surgical system after routine preoperative preparation. Anorectal motility was weekly monitored postoperation. No additional adjuvant therapy was performed.The patient could walk after 1 day and defecate normally on the third day after operation. She was discharged on the seventh postoperative day. No adverse events including pelvic floor hernia or defecation dysfunction occurred in the follow-up period. At 4 weeks follow-up, the patient was pain-free and recovered well.Although imaging examinations were crucial for retroperitoneal neoplasms, histopathological examination remains the "gold standard" for making a definite diagnosis. This case highlights the possibility of retroperitoneal leiomyoma occurring in a woman of reproductive age and the advantages of robotic surgical system in pelvic retroperitoneal surgeries.

Retroperitoneal and pelvis sarcomas are uncommon tumors for which complete surgical resection is the mainstay of treatment. However, achieving complete gross resection with microscopically negative margins is challenging, and local recurrence rates can be high. Patients often succumb to uncontrolled local disease. Radiation therapy offers a potential means for sterilizing microscopic residual disease, although its use continues to be controversial. Chemotherapy alone or in combination with radiation continues to be investigated as an adjunct to surgery, along with immunotherapy and targeted therapies. In this review, we discuss the current management of retroperitoneal and pelvis sarcomas, focusing on studies of surgery and radiation therapy to maximize local control.

It would be best to consider the pathogenesis of retroperitoneal EP to identify high-risk patients, manage them better, and reduce the delay in diagnosing this rare but more frequent condition. Laparoscopy Is a feasible, safe, and more accurate approach to retroperitoneal EP when entrusted to the expert hands of gynecologists experienced in oncology or deep endometriosis.

A 70-year-old man complaining of pain in his right leg presented to the Department of Orthopedics in our hospital. X-ray findings revealed calcifications around the left kidney. He was referred to our department for further examination. Computed tomography revealed a tumor 3 cm in diameter with calcifications and an obscure border that was located on the caudal side of the pancreas, anterior to the left iliopsoas muscle and at the left side of the aorta. Magnetic resonance imaging showed that the tumor had comparatively low intensity in diffusion-weighted images and the cell density was not high. The contrast of the tumor by enhanced computed tomography was weak, and we had difficulty judging whether the tumor was benign or malignant. Each tumor marker, immunity factor, and hormone-like catecholamine were within the normal range. We considered the retroperitoneal tumor with calcifications as Castleman disease or tumor of nerve origin. It is believed that most retroperitoneal tumors are malignant. We performed laparoscopic surgery to resect the retroperitoneal tumor. Histopathological diagnosis was a primary retroperitoneal venous malformation. Vascular malformation derived from the retroperitoneum is rare. Furthermore, very few cases of venous malformation in the retroperitoneum have been reported.

Background: There is currently no consensus on optimal management of patients with primary or recurrent non-resectable/residual retroperitoneal sarcomas (RPS). The objective of this study was to document the outcomes of patients with primary or recurrent non-resectable/residual RPS treated in our center with definitive radiotherapy (RT) and to perform a systematic review on the topic. Methods: A retrospective analysis of consecutive RPS patients treated in our center between 2000 and 2019 was performed. All consecutive patients who underwent definitive conformal RT with image guidance for primary or recurrent non-resectable or macroscopically residual RPS were included. Additionally, a systematic review compliant with the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses was performed. Results: The study enrolled 14 patients who met the aforementioned criteria. Data on clinicopathological characteristics, RT and response to treatment were assessed. RT allowed achieving prolonged local control of the disease, i.e. no local progression of the disease for more than 12 months after RT in 10 patients. Local control lasted more than 24 months in 6 cases, with minimal or no toxicity. A systemic review of 11 studies revealed concordance of our results with previous reports of primary or recurrent non-resectable/residual RPS. Conclusions: RT provided satisfactory local disease control with acceptable treatment tolerance in patients with primary or recurrent non-resectable/residual RPS and represents a valuable treatment modality in the selected group of patients. Additional RT modalities i.e. BT, particle therapy, MRI-guided RT, or GRID/Lattice RT may be introduced to improve local control and further minimize toxicity.

Retroperitoneal teratomas (RPTs) are rare in infants. We report our experience of treating pediatric patients with RPTs over many years at a single institution, with the aim of developing a safe and secure operative strategy for RPTs in infants. We reviewed the medical records of patients who underwent treatment for RPTs in our institution between April, 1984 and December, 2017, to analyze their background and clinical data. The diagnosis of RPT was confirmed histologically in all patients. The subjects of this retrospective analysis were 14 pediatric patients (female, n = 11; male, n = 4), ranging in age from 6 days to 12 years, 11 (73.3%) of whom were under 1 year of age. Complete surgical resection was performed in all patients. The tumor ruptured during surgery in four (26.7%) patients and perioperative vessel injuries occurred in six (40.0%) patients, resulting in nephrectomy in one (6.7%). Three (20.0%) patients suffered unilateral renal dysfunction as a surgical complication. Only one patient received postoperative chemotherapy. All patients were free of disease at the time of writing. Perioperative complications are not uncommon during surgery for RPTs, despite their benign nature. Preoperative imaging evaluation is important and operative management may be challenging. Because of the favorable prognosis and the frequency of adverse events in surgery, partial resection or split excision is sometimes unavoidable. Meticulous follow-up for recurrence is required for such patients.

Retroperitoneal lipomas are extremely rare with few cases reported so far in the literature. They can reach different sizes and present with a variety of symptoms. The differential diagnosis is mainly with well-differentiated liposarcoma (WDLPS). We present a 34-year-old woman with a retroperitoneal lipoma herniating through the inguinal canal into the proximal thigh. The patient underwent complete oncological resection using a Karakousis’s abdominoinguinal incision. Retroperitoneal lipomas are a very rare condition and sometimes require resections technically challenging. MDM2 amplification is critical for its differential diagnosis with WDLPS.

When resecting small tumors or tumors with an irregular margin, a marking technique is conducted prior to the surgery. CT-guided marking techniques are common in pulmonary surgery, but it is rarely used in abdominal or urological surgery. We performed a marking technique for a small tumor that was undetectable by ultrasound using CT guidance prior to laparoscopic resection.A 63 year-old woman, two years after total hysterectomy for uterine stromal sarcoma, underwent combined right kidney resection and retroperitoneal tumor resection for a giant recurrence. Two months after the surgery, micro recurrence was observed in the vicinity of the right intestinal psoas muscle which upon follow up, the tumor size increased to 1 cm. Surgical resection of the small recurrent tumor was planned. Since it was difficult to detect by ultrasound, preoperative CT-guided marking was performed. Retroperitoneal laparoscopic resection was performed the following day. The histopathological diagnosis was endometrial stromal sarcoma.

A 59-year-old man visited our department because of cholecystectomy. Preoperative CT revealed a tumor shadow measuring 50 mm in front of the right iliopsoas muscle. MRI showed a low signal intensity on T1-weighted images and a slightly high signal intensity on the T2-weighted image. PET-CT showed accumulation of FDG(SUVmax 5.39)in the tumor but no other abnormal accumulations. We performed tumor resection for diagnostic purposes because malignancy could not be ruled out owing to the large size of the mass. Intraoperative findings showed a well-circumscribed margin of the tumor without invasion to other tissues. The retroperitoneum was incised circumferentially along the tumor under laparoscopic guidance, and the tumor was resected. Histopathological and immunostaining findings were consistent with leiomyosarcoma. In laparoscopic surgery, the surgical margin is observed in detail through the magnifying effect. Therefore, laparoscopic surgery can be a surgical option for tumors that may be completely excised based on preoperative findings.

We present a 46-year-old female patient who underwent resection of a retroperitoneal tumor, which was found by medical check-up. The tumor which was elastic hard and had good mobility displaced the duodenum to her abdominal wall. Since her right ovarian vein adhered to the tumor, we removed the tumor with the ligated vein. Pathological findings showed the tumor consisted of spindle-shaped cells with pleomorphic nucleus and it presented the fascicular growth pattern. Additional immunostaining showed positive for HHF35, h-caldesmon. Because the leiomyosarcoma connected with the smooth muscle of the right ovarian vein, we considered the vascular smooth muscle was the origin of the tumor. It is 2 years after the operation, there has been no local recurrence or metastasis.