Primary Retroperitoneal Tumor Research Articles

Mixed reality in primary retroperitoneal tumour surgery: Evaluation of preoperative and intraoperative application value.

To evaluate the feasibility and application value of mixed reality technology (MR) in Primary retroperitoneal tumour (PRT) surgery. From 276 patients who underwent PRT resection at the First Affiliated Hospital of Xi'an Jiaotong University, we screened 46 patients who underwent MR-assisted retroperitoneal tumour resection and 46 patients who underwent tumour resection without MR assistance. The intraoperative and postoperative recovery of the patients in both groups were compared, and the reliability and validity of the application of MR were further examined using the Likert scale. There was a significant difference in the mean intraoperative bleeding volume between the two groups, but it was reduced in the MR group. The resultsof the Likert scale showed higher scores in the MR group than non-MR group. MR can be used to assist PRT resection and has great potential to improve the rate of complete retroperitoneal tumour resection.

Patients with Primary Malignant Retroperitoneal Tumor: Surgical Treatment Outcomes

Introduction . Determining the most optimal tactics for the diagnosis and treatment of primary malignant retroperitoneal tumors is highly relevant today. This can be attributed to a number of reasons, including: lack of versatile approach to the treatment of primary malignant retroperitoneal tumors, lack of effective ways to prevent the disease progression, small number of publications on this issue. All this motivates oncologists engaged in the treatment of patients with this pathology to accumulate and analyze the results of treatment of each patient. Materials and methods. The study was based on data analysis of 32 patients with primary retroperitoneal tumors, 26 of which were treated at the Yaroslavl Clinical Oncological Hospital from 2016 to 2021 with the diagnosis of primary malignant retroperitoneal tumors. Results and discussion. 13 patients out of 26 (50%) had T4N0M0. Stage 3B was detected in 73.08% of patients. Histologically verified: liposarcoma — 19.23%, leiomyosarcoma and myxoid liposarcoma — 15.38% each, the remaining formations comprised 3.8% each and consisted to different types of sarcomas from mesodermal rudiments. 22 patients (84.62%) had high-grade tumors, 4 patients (15.38%) had low-grade tumors. 22 patients underwent radical surgical treatment. Isolated resection of the tumor was performed in 11 patients (50%), and 11 operations (50%) were accompanied by combined or concurrent tumor resection. No postoperative complications were reported. Histologically confirmed tumor invasion was detected in 50% of operated patients. The most frequently involved organ was the kidney. Conclusion . The obtained data indicate high resectability rates for tumors of this localization. Analysis of the excised tumor volume revealed direct correlation with the degree of tumor malignancy. High rates of one-, three- and five-year survival of patients testify to the effectiveness of radical surgical resection both in case of isolated resection and combined or concurrent resection. However, frequent recurrences and, therefore, repeated surgical interventions lead to a regressive reduction in survival.

Primary retroperitoneal mucinous cystic tumour of borderline malignancy mimicking kidney duplicate: cases report and literature review

BackgroundPrimary retroperitoneal mucinous cystic tumours with borderline malignancy (PRMC-BM) are rare and difficult to diagnose preoperatively. We are the first to report two cases of PRMC-BM which mimic a duplex kidney and evaluate the outcomes of different surgical procedures.Case presentationWe describe two cases of retroperitoneal cystic tumours. Both were diagnosed with duplex kidney with hydronephrosis on computed tomography scan. The first patient underwent robot-assisted laparoscopic surgery and was found to have a retroperitoneal cystic tumour. The other patient underwent an ultrasound-guided puncture before surgery and was diagnosed with retroperitoneal lymphangioma. Retroperitoneal cystectomy was performed using an open transperitoneal procedure. The final pathologic diagnosis in both cases implies PRMC-BM. The open surgical approach was associated with a shorter operation time, less intraoperative blood loss, and protected cyst wall integrity by comparing the different surgical approaches. During follow-up, the patient in the first case had tumour recurrence six months post-surgery, and the other patient was healthy without recurrence or metastasis 12 months post-surgery.ConclusionsPrimary retroperitoneal mucinous cystic tumours with borderline malignancy can be enclosed within the kidney and misdiagnosed as other cystic diseases of the urinary system. Thus, an open surgical approach may be more suitable for this type of tumour.

Replantation of solitary kidney in a patient with giant primary retroperitoneal sarcoma

Background. Surgery for non-organ malignant retroperitoneal tumors poses a serious challenge. Long-term asymptomatic course of the disease leads to a delayed diagnosis, when tumors can reach massive size with potential involvement of adjacent organs and critical structures. In most cases, nephrectomy is often required during resection of retroperitoneal sarcomas. Recently, there has been growing interest in nephronsaving surgery for large retroperitoneal tumors.Description of the clinical case. A 70-year-old patient was admitted to Irkutsk Regional Cancer Center with right-sided giant primary retroperitoneal tumor and grade 1 hydronephrosis of solitary right kidney. The patient underwent extended resection of primary retroperitoneal tumor with right-sided hemicolectomy and nephradrenalectomy. Extracorporeal resection of the solitary kidney was then performed. After obtaining negative kidney smears (imprints), the kidney was replanted in the pool of right iliac vessels. No complications were observed in the postoperative period. Histopathology examination revealed was welldifferentiated liposarcoma. On follow-up examination 38 months after surgery, there were no signs of local tumor recurrence, and renal function was satisfactory.Conclusion. Extracorporeal resection of the kidney with heterotopic replantation is an effective nephron-saving method in surgery for primary retroperitoneal sarcomas. The patient’s solitary kidney with primary retroperitoneal sarcoma is an absolute indication for kidney’s saving, including the use of transplant technologies.

Resecting liver segments VII–VIII without reconstructing the right hepatic vein

Aim. To improve outcomes of treatment for neoplasms in segments VII-VIII of the liver adjacent to the main stem of the right hepatic vein and/or to its forming large branches.Materials and methods. In the period of 2016–2022, the resection of segments VII and/or VIII of the liver and the right hepatic vein, as well as its forming branches without reconstruction was performed in 24 patients. Surgical indications were liver metastases of colorectal cancer in 15 cases, hepatocellular cancer (cholangiocarcinoma) in six cases, angiomyolipoma in one case, uterine cancer metastases in one case and primary retroperitoneal tumor metastases in one case. Minor liver resections were additionally performed in 11 cases. Results. There were no intraoperative signs of impaired venous drainage. After surgery bile in the drains was detected in three patients, and a biliary fistula was formed in one case. No other complications were identified. No fatalities were registered after surgery. The late postoperative period revealed no specific complications related to a disturbance of venous drainage through the right hepatic vein.Conclusion. Existing and emerging collaterals can provide adequate venous drainage to the middle hepatic vein, short hepatic veins after resection of liver segments VII and VIII with resection of the right hepatic vein stem and its forming branches without reconstruction, even if the lower right hepatic vein is missing, and without complete obstruction of the right hepatic vein before surgery.

A CASE REPORT OF RETROPERITONEAL TERATOMA IN A 11 MONTH OLD INFANT:

The incidence of primary retroperitoneal teratoma in infancy is rare but in children it is seen in 3-4% of overall germ cell tumors and 1-11% of primary retroperitoneal neoplasms . Most often they are asymptomatic and diagnosed incidentally and presents clinically only after attaining large size in the form of abdominal distension, abdominal mass with or without compressive symptoms. It is basically diagnosed using the help of radiological investigations like ultrasonography and contrast-enhanced computed tomography(CECT) of abdomen and nal diagnosis is conrmed after a thorough histopathological examination of the specimen. The denitive treatment includes complete surgical removal of teratoma. Here we are reporting a case of primary retroperitoneal teratoma in a 11month old male infant who presented with right sided abdominal mass and treated by complete surgical resection. Histopathological examination suggested the diagnosis of mature cystic teratoma.

Kidney autotransplantation: a method for treating ureteral lesions in urological and oncological practice

The first successful kidney autotransplantation was performed in 1902. The technique has undergone several changes since then. The indications and surgical technique are presented in this literature review. Kidney autotransplantation is the treatment of choice for preserving renal function. Three clinical observations on the use of kidney autotransplantation in urological and oncological practice are described: a patient after iatrogenic ureteral injury and two patients with primary retroperitoneal tumor. Literature analysis and clinical observations from urological and oncological practice show that kidney autotransplantation could be safely used for strictly selected indications.

A Nine Years Histopathological Retrospective Study of Retroperitoneal Masses

Introduction: Retroperitoneum is potentially large space that allows both primary and metastatic tumours to grow silently before clinical signs and symptoms appear. However, retroperitoneal masses due to their unhinhibited growth and with no facial boundaries they are often attain large size before the development of symptoms and the establishment of a diagnosis. There are a vast number of neoplasms occurring in this region. Understanding the pathology and lesions is important, for early diagnosis and for treatment of these neoplasms. Aim: To study the overall prevalence of primary and secondary retroperitoneal neoplasms and the varied histomorphological spectrum of retroperitoneal masses. Also to categorise the lesions as benign and malignant tumors of retroperitoneum. Materials and Methods: This retrospective study was conducted in the Department of Pathology, Karnataka Institute of Medical Sciences Hubballi, Karnataka, India from 2011 to 2019. A total of 39,757 speimens were recieved to histopathology section during this period. Among them, 53 (0.13%) were retroperitoneal tumours. Haematoxylin and Eosin stained slides were thoroughly analysed and categorised into non neoplastic, benign and malignant tumours. Further, based on the finer microscopic features, the accurate subtype of the tumour type was made. The descriptive statistics like mean, range and percentage are used. Results: Total 53 cases of retroperitoneal tumours were studied of which 10 (18.9%) cases were primary retroperitoneal masses and 43 (81.1%) cases were secondary retroperitoneal masses. Mean age was 40.7 years with male to female ratio 1.5:1. Malignant lesions were more common accounting to 50.9% cases, followed by benign 22.64% cases, the rest were intermediate grade and inflammatory. Kidney lesions were most common secondary retroperitoneal masses, accounting for 41.5% cases, followed by pancreas 22.64% cases, adrenals 13.20% cases and Lymphnodes 3.7% cases. Primary retroperitoneal masses which accounted for 18.9% cases were, categorised histomorphologically as spindle cell tumours, adipocytic, small round cell tumours, cartilaginous origin and cystic lesions. Conclusion: Diagnosis of retroperitoneal tumours is challenging due to their varied histomorphology and histopathology forms the gold standard for diagnosis.

A Brief Study on Retroperitoneal Paraganglioma

Paragangliomas are tumors of the autonomic nervous system, located extra-adrenally, presenting inside the base of the head, chest and abdomen. They can appear as a primary retroperitoneal neoplasm when viewed inside the abdominal cavity and might resemble as vascular malformations or any other conditions same as retroperitoneal organs particularly such as pancreas, kidney. These are relatively uncommon endocrine tumors that originate from specific tissue of the paraganglion. They synthesise, store and secrete catecholamines, which can contribute to headache, sweating, palpitation and hypertension. These may be mistaken for GISTs with no histological diagnosis and symptoms of catecholamine overload (non functional). We are reporting a case of 42 yrs male with clinical features of slow backache radiating to both lower legs and with the presence of tingling sensation. Corresponding contrast enhanced CT scan shows a para – aortic mass.

LEIOMYOSARCOMA MIMICKING AN ADRENAL GLAND TUMOR

Leiomyosarcoma is a malignant smooth muscle tumor. It is the second most common primary retroperitoneal tumor behind liposarcoma. The retroperitoneal léiomyosarcoma represents 11% of all retroperitoneal malignant tumors. The age of discovery is between 40 and 60 years.

Risk factors influencing prognosis of patients undergoing primary retroperitoneal tumor surgery

Risk factors influencing prognosis of patients undergoing primary retroperitoneal tumor surgery

Laparoscopic resection of giant retroperitoneal mature cystic teratoma with diaphragm invasion in a 3-month-old girl

Abstract Primary retroperitoneal teratomas are rare, representing only 1%–11% of primary retroperitoneal neoplasms. They typically present as an asymptomatic abdominal mass but can grow to enormous size. The majority of these are benign mature and immature teratomas. We found a large mass in the left abdomen of the child. According to examinations (CT, Ultrasound, Tumor markers, etc.), it was considered to be benign, which was treated by laparoscopic surgery. However, CT revealed that part of the diaphragm was invaded, increasing the difficulty of our operation and intraoperatively confirmed the invasion of the left diaphragm. A frozen section and final histopathologic examination also revealed mature cystic teratoma. This case study highlights the importance of pre-operative imaging and tumor markers. Under the premise of ensuring the safety of the child, laparoscopic procedures have resulted in decreased tissue trauma and accelerated postoperative recovery.

Outcome of patients with primary retroperitoneal solitary fibrous sarcoma.

To describe the clinicopathological features of primary retroperitoneal solitary fibrous tumor (RSFT) and define the prognostic factors. The comprehensive data of 35 primary RSFT patients who got curative surgery at a tertiary cancer center from April 2004 to October 2018 were retrospectively analyzed. Male patients outnumbered female patients (19 vs. 16), with the age ranging from 19 to 73years (median, 51years). 7 (20%) patients had tumors located in special parts, including three in kidney, one in renal pelvis, one in bladder, one in prostate, and one in mesentery. Tumor sizes ranged from 2.5 to 25cm (median, 9cm). Microscopic negative margin was reached in 33 (94.3%) cases. 13 (37.1%) were classified as atypical/malignant, while 22 (62.9%) were benign. Concomitant organ excision was performed on 11 (31.4%) patients, with kidney (n = 5) being the most frequent organ. Multifocality was only found in 4 (11.4%) cases. The majority of the patients (31, 88.6%) did not get adjuvant treatment. The median follow-up time was 46months (range 4-153months). The 5-year DSS rate and DFS rate were 100% and 63.6%, respectively. In univariate analysis, tumor size ≥ 10cm (P = 0.002) and atypical/malignant pathology (P = 0.024) were associated with decreased DFS. Multivariate analysis revealed that tumor size was the only independent prognostic factor for DFS (HR 6.03, 95% CI 1.18-30.77, P = 0.031). RSFT is uncommon, slow-growing, and recrudescent tumors. Large tumor size and malignant pathology are associated with decreased DFS. Tumor size ≥ 10cm independently predicts shortened DFS.

Primary retroperitoneal yolk sac tumor:CT findings and literature review

内胚窦瘤是少见的恶性生殖细胞源性肿瘤，腹膜后原发者罕见，与其他腹膜后肿瘤鉴别困难。笔者报道一例原发性腹膜后内胚窦瘤CT表现，并复习国内外文献报道中有影像诊断描述的32例。腹膜后内胚窦瘤的影像特征包括密度不均匀、出现浸润与转移、纤维血管间隔征、罕见钙化等，诊断还需依靠病理结果。

88. A Large Primary Retroperitoneal Mucinous Tumor in Pregnancy: A Case Report

Primary retroperitoneal mucinous tumors are rare with only about 50 cases reported in the literature. Of these only 13 cases were primary retroperitoneal mucinous cystic tumors of borderline malignancy. There is no unanimous consensus on the origin of these tumors and due to its extreme rarity, its optimal management and prognosis are uncertain.

Non-functional retroperitoneal paraganglioma: A case report

Paragangliomas are extra-adrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. When presenting within the abdominal cavity, they may arise as a primary retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys.[1] They synthesize, store, and secrete catecholamines because of which they may present with headache, sweating, palpitation, and symptoms of hypertension (functional).[2] In the absence of histological diagnosis and symptoms of catecholamine excess (non-functional), these may be mistaken for GISTs.[3] We are reporting a case of a 36-year-old female who was clinically diagnosed as GIST, underwent excision, and postoperative histopathological examination was found to be paraganglioma.

An Unusual Case of Primary Retroperitoneal Germ Cell Tumour in a Young Man.

Some 2–5% of germ cell tumours are of extragonadal origin, with a retroperitoneal location being very rare. The majority of retroperitoneal germ cell tumours have metastasized from a testicular tumour. These tumours are diagnosed incidentally or symptomatically and nearly all present with high alpha-fetoprotein and lactate dehydrogenase levels.We describe the unusual case of a 31-year-old man with a yolk-sac, retroperitoneal germ cell tumour, with normal serum alpha-fetoprotein and lactate dehydrogenase levels, which has not previously been described. A testicular tumour was excluded by physical examination and additional tests. Our diagnosis was based on a high level of suspicion and histopathological results. As far as we know, this is the first case described with these characteristics.LEARNING POINTSExtragonadal germ cell tumours are rare but can have fatal consequences if undiagnosed.The usual laboratory markers of the disease were absent in our patient.A high level of suspicion is required for diagnosis and close follow-up is required.

Expression of MDM2 and p16 in angiomyolipoma

Angiomyolipoma (AML) arises primarily from the kidney but may grow into the retroperitoneal space mimicking a primary retroperitoneal tumor. Fine needle aspiration (FNA) and core needle biopsy of AML, particularly the fat-predominant variant, may be difficult to distinguish from retroperitoneal well-differentiated liposarcoma (WDLS) or lipoma. Commonly used immunomarkers, MDM2 and p16, have proven useful in diagnosing WDLS and dedifferentiated liposarcoma (DDLS), while HMB45 and Melan-A are melanocyte-related markers characteristically expressed in AML. In this study, we investigated the utility of MDM2 and p16 along with HMB45 and Melan-A immunohistochemical analysis in distinguishing AML from WDL/DDLS or lipoma. Immunohistochemically, AMLs demonstrated focal MDM2 expression (40% of cases) and focal/diffuse expression of p16 (60%). AMLs marked focally or diffusely with HMB45 (76% of cases) and Melan-A (96%). These latter two immunomarkers were not expressed in any of the WDLS/DDLSs or lipomas tested. WDLS/DDLSs showed focal/diffuse expression of MDM2 (91% of cases) and p16 (97%). While focal expression of MDM2 and p16 was observed in 14% and 67% of lipomas, respectively, no lipoma exhibited diffuse MDM2 positivity. In our hands, MDM2 expression by itself cannot exclude the diagnosis of AML or lipoma, and p16 alone is not helpful in separating AML and conventional lipoma from WDLS/DDLS. However, along with morphology, an immunohistochemical battery including HMB45, Melan-A, MDM2 and p16 are useful in distinguishing AML from WDLS/DDLS or lipoma. For equivocal cases, fluorescence in situ hybridization for MDM2 should be performed.

Retroperitoneal malignant peripheral nerve sheath tumor

Primary retroperitoneal neoplasms are a rare entity and account for 0.1%–0.2% of all malignancies. In addition, only 1% of malignant peripheral nerve sheath tumors (MPNSTs) occur in retroperitoneal region. Herein, we report a case of retroperitoneal peripheral nerve sheath tumor in a 50-year-old man who presented with right lower limb pain. On physical examination, an abdominal mass was palpable. Computed tomography of abdomen with contrast, showed a large, lobulated mass, in the right paravertebral region extending to the middle area of abdomen and pelvis. The patient underwent exploratory laparotomy and mass excision. Histopathological examination was consistent with “MPNSTs.” This case report emphasizes that, although rare, MPNSTs may arise from retroperitoneal region. They have varied clinical presentations, and combination of microscopic, radiological, and immunohistochemical analysis is needed for diagnosis of MPNSTs.

Primary retroperitoneal yolk sac tumor

Primary retroperitoneal yolk sac tumour is very rare. 3 year old with rapidly progressing abdominal mass. CT heterogeneously enhancing large lesion with liver metastasis. HPE showed features suggestive of YST. Elevated AFP noted. Patient improved with surgery followed by chemotherapy. Paediatric germ cell tumours are highly curable form of cancer, but their diversity makes it challenging for the clinicians.