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.

Metastatic testicular seminoma presenting as acute lower limb ischaemia.

We describe a unique presentation of acute lower limb ischaemia due to metastatic seminoma in a middle-aged man with a large retroperitoneal mass. The patient underwent vascular bypass surgery of the right lower limb, completed chemotherapy, and had a right scrotal orchiectomy. The patient had pre-existing vascular risk factors including peripheral vascular disease and smoking. To our knowledge this is the first published case in the literature that has described a large retroperitoneal seminoma compressing the abdominal aorta resulting in acute lower limb ischaemia.

Emergent Management of Growing Teratoma Syndrome: A Case Presentation.

Growing teratoma syndrome (GTS) represents a rare yet significant complication following treatment for non-seminomatous germ cell tumors (NSGCT), characterized by the growth of mature teratoma elements despite prior chemotherapy. We present the case of a 30-year-old male who, following orchidectomy for NSGCT and subsequent chemotherapy, developed acute abdominal pain and pulmonary metastasis. Despite normal serum tumor markers, imaging revealed a large retroperitoneal mass encasing significant vessels. Surgical excision led to symptom resolution. This case underscores the diagnostic challenges GTS poses, the importance of imaging in diagnosis, and the efficacy of prompt surgical intervention in achieving favorable outcomes.

Primary Right Adrenal Teratoma in a Postmenopausal Female: a Case Report and Review of Literature.

Non-seminomatous germ cell tumors with structural components from all three cellular lineages are called teratomas. We report a rare case of a primary right adrenal teratoma in a postmenopausal female, presenting with abdominal pain. Ultrasound revealed a complex cystic shadow in the perihepatic region superior to the upper pole of the right kidney, which was suggestive of a complex supra-renal space-sequence-occupying lesion. Computerized tomography revealed a large-sized mature single multilobulated complex cystic lesion in the right hypochondrial lumbar region arising from the right adrenal gland. A right-sided transperitoneal adrenalectomy was performed. The resected mass (18 × 13 × 10 cm) was well encapsulated. Cut surfaces showed cystic mass filled with necrotic gray-white pultaceous material, along with a solid white gelatinous area. Microscopic sections showed a tumor which displayed differentiation along various tissue lineages. After immunohistochemical examinations, the tumor was confirmed to be a mature cystic teratoma. Mature teratomas show a good prognosis, and surgical resection and follow-up remain the standard approach.

Resection of primary left renal vein leiomyosarcoma with renal preservation following an unusual clinical presentation.

An elderly man was referred to vascular surgery on incidental discovery of a left retroperitoneal mass ultimately found to be of left renal vein (LRV) origin. He initially presented with recurring lower back pain. CT of the abdomen/pelvis showed a 6.0×5.5 cm lobulated retroperitoneal mass anterior to the infrarenal aorta. Resection of the mass necessitated a multidisciplinary team consisting of medical oncologists, radiation oncologists, urologists and vascular surgeons. In efforts to obtain an R0 margin, en-bloc resection of the LRV from its confluence with the inferior vena cava (IVC) was necessary. A primary repair of the IVC was performed with preservation of the left kidney. The patient's back pain has since resolved after the surgery. A literature search found IVC reconstructions to be safe and effective in the removal of vascular leiomyosarcomas.

Pathology results of endoscopic ultrasound-guided tissue acquisition in retroperitoneal masses: a multicenter study.

Background: Malignant etiologies are found in 70-80% of symptomatic retroperitoneal masses. Histology is required for diagnosis and treatment. Information about endoscopic ultrasound (EUS)-guided tissue acquisition (EUS-GTA) is scant for retroperitoneal masses. This study aimed to assess the pathology results of EUS-GTA for diagnosing retroperitoneal masses. Methods: This retrospective, multicenter study involved patients from 5 care centers. All patients with retroperitoneal masses who underwent EUS evaluation were enrolled. We recorded demographic and clinical characteristics, location and size of the mass, type of needle (FNA/FNB), and complications related to the procedure. Results: A total of 43 patients were included. The median age was 50.5 (range: 23-83) years, and 22 (51.2%) were female. The initial symptom was abdominal pain in 23 (52.3%) cases and weight loss in 11 (25%). Initial imaging was by computed tomography in 33 (75%) patients. Diagnosis with EUS-GTA was reached in 67.5% (29/43) cases. The most frequent histological diagnosis was carcinoma, in 25.5% (11/43). A malignant etiology was found in 31 (72%): 20 were primary tumors from the retroperitoneum, and 11 were metastases. In patients with metastasis, surgery was avoided and medical treatment was indicated. No adverse events were reported. Conclusion: EUS and EUS-GTA can frequently provide accurate tissue diagnosis and significantly impact the subsequent management.

Extralobar pulmonary sequestration: A case report and literature review.

Pulmonary sequestration is a congenital malformation of lung development in which part of the lung tissue is separated from the normal lung during the embryonic phase and develops separately and receives blood supply from an aberrant systemic artery forming a nonrespiratory mass. In brief, early in embryonic development, certain tissues that should have atrophied and been gradually absorbed are left behind due to impairment of the atrophy process and form anomalous branches of the aorta, which pull parts of the lung tissue, isolating them from normal lung tissue and bronchi, and thus forming separate lung tissue. According to the relationship of the mass to the pleural covering, pulmonary sequestration can be divided into two types, intralobar pulmonary sequestration (ILS) and extralobar pulmonary sequestration (ELS), of which approximately 75% of cases are ILS, but ELS is less common. Symptoms are not obvious in either type, making diagnosis and differential diagnosis more difficult. Here we report a 33-year-old patient with only insignificant abdominal distension who was eventually diagnosed with retroperitoneal ELS.

A case report of multimodal ultrasound imaging in the diagnosis of giant retroperitoneal ganglioneuroma.

Retroperitoneal ganglioneuroma is a rare benign tumor that is challenging in terms of clinical diagnosis. Computed tomography and magnetic resonance imaging are usually performed for diagnosis rather than convenient and inexpensive ultrasonography. Here, we present the case of a 21-year-old female patient who was diagnosed by multimodal ultrasound imaging and whose diagnosis was confirmed by ultrasound-guided core needle biopsy before surgery. We hope that this rare case will help clinicians and radiologists realize the advantages of multimodal ultrasound imaging in the diagnosis of retropeitoneal solid tumors, and reduce misdiagnosis.

Reporting a single case of cystic echinococcosis in retroperitoneal mass of uncertain origin.

Echinococcal disease (hydatid disease (HD) is an endemic parasitosis caused by Echinococcus granulosus in the larval stage, and it is typically due to the production of unilocular cystic lesions, usually involving the liver for the majority of patients and the lungs in 25%, but also any other organs can be potentially involved in developing echinococcal disease. We report a case of extrahepatic, retroperitoneal echinococcal disease, caused by Echinococcus granulosus. The patient underwent a surgical removal of the abdominal mass, revealed by abdominal ultrasound and computerized tomography scanning, and in the founded clinical and radiological suspicion of echinococcal disease, multiple bioptical samples were sent for microbiological analysis and albendazole therapy was started; Echinococcus granulosus protoscolices were found on the bioptical sample, and the diagnosis was successfully confirmed. According to the current parasitology literature on echinococcal disease, extrahepatic localization, although rare, can be found, and it should be considered in the differential diagnosis of an abdominal mass when epidemiological risk factors and anamnestic data are present, regardless of the usual site of the disease.

Primary Retroperitoneal Serous Cyst Adenoma: A Case Report and Literature Review.

Primary retroperitoneal serous cyst adenomas (PRSCs) are extremely rare thin-walled cystic lesions whose pathogenesis is not well understood. Clinical presentation varies depending on the lesion's size and location, i.e., larger lesions compress adjacent organs, giving the impression of malignancy. Although advances in imaging techniques enable to identify various characteristics of retroperitoneal cystic lesions, there are no pathognomonic signs to confirm the diagnosis. The exact diagnosis is based on the histology after complete surgical excision. An open surgical approach is considered the traditional method of complete resection; however, laparoscopic techniques have increasingly been employed. Diagnostic aspiration is discouraged due to the potential risk of seeding if the lesion is malignant. We present the case of a 51-year-old woman who underwent complete excision of a large right retroperitoneal cyst, histologically confirmed as PRSC with a review of the background and management options of this phenomenon.

Clinical application of real-time PET/CT guided targeted retroperitoneal masses biopsy in diagnosing malignant tumors.

OBJECTIVE: To explore the feasibility, safety, and clinical application value based on the fusion image of 18 F-FDG PET/CT, for guiding retroperitoneal puncture biopsy technology and to determine the diagnosis of retroperitoneal masses in diagnosing malignant tumors. METHODS: From March 2019 to January 2023, 42 patients underwent 18 F-FDG PET/CT imaging and were found to have retroperitoneal lesions that required definite diagnosis; 22 were male, 20 were female, and the average age was(59.17 ± 13.23) years. According to the fused 18 F-FDG PET/CT tomographic image, the target point with the highest metabolic activity, the safest, and expected maximum sample size was selected. CT scans were acquired with the same machine and fused with 18 F-FDG PET, guiding the puncture biopsy needle to approach the expected target zone, enabling timely delivery of pathological and immunohistochemical examination of the biopsy. Success rate, total examination time, biopsy operation time, complications, CT radiation dose, pathological, and immunohistochemical results were recorded. RESULTS: All 42 patients were sampled successfully with the successful rate being 100%. The site of sampling of 42 patients accurately targeted the highest metabolic activity, the safest, and the expected maximum sample size. All 42 patients received clear diagnosis (25 cases of malignant tumors and cases of 17 benign tissues). 15 cases of patients had a change in clinical diagnosis, accounting for 35.7% of all patients, and affecting subsequent treatment plans. The average total examination time for patients was (41.3 ± 7.3) minutes, and the biopsy operation time was (29.1 ± 8.7) minutes. The effective radiation dose generated by the entire examination generated by CT guidance was (2.0 ± 0.5) mSv; no severe complications occurred in the patients. CONCLUSION: Real-time-guided retroperitoneal puncture biopsy based on 18 F-FDG PET/CT fusion image is safe, accurate, and feasible, and can provide patients of retroperitoneal mass with clear pathological diagnosis and immunohistochemical evaluation.

New-Onset Paralysis following Biopsy of a Retroperitoneal Mass with Intraspinal Extension in a Pediatric Patient.

Image-guided percutaneous biopsies are routine, safe procedures and complications are infrequent and usually directly related to the biopsy itself. This report describes a biopsy of a retroperitoneal mass with extension into the spinal canal, following which the patient developed paralysis unrelated to the biopsy itself but secondary to spinal cord ischemia during the procedure. Multiple factors contributed to the ischemia, including prone positioning, compression of spinal vasculature by the mass, low arterial pressures, and an extended duration of anesthesia. While the patient eventually recovered neurologic function, it is an important reminder to consider individual patient factors that may complicate typically routine procedures. In masses with intraspinal extension, patient positioning is critical to prevent positional ischemia, and maintaining elevated mean arterial pressures is crucial for ensuring adequate spinal perfusion throughout the procedure.

Giant renal angiomyolipoma: A case report.

INTRODUCTION: Renal angiomyolipoma (AML), which is a rare solid kidney tumor with benign characteristics, also known as a renal hamartoma, can exhibit various clinical symptoms and severe consequences may arise if the lesion becomes large. PRESENTATION OF THE CASE: A 58-year-old woman was admitted to a hospital, with general fatigue, abdominal swelling, and epigastric fullness. Upon examination, a large mass was palpated, which occupied almost the entire right abdomen. The abdominal computed tomography scan revealed a large right renal mass measuring 22 × 18 × 8 cm, which was exophytic and heterogeneous with a large fat component and an enhancing solid part. The tumor was successfully excised through a generous right subcostal incision with left-side extension. The total weight of the resected specimen was 2500 g, which appears to be the largest angiomyolipoma ever resected in Ethiopia. DISCUSSION: Renal AML, a benign tumor derived from mesenchymal components, is sometimes referred to as a "hamartoma" due to its variable makeup. The most common complaints of patients with renal AML are lower back pain, hematuria, and physical finding of hypotension (shock), though patients with giant AML, as in this case, may also experience gastrointestinal symptoms due to the mass' compression. CONCLUSION: Although treatment options requiring contemporary medical technologies and skilled manpower are difficult to offer in set ups of resource-limited countries, such as the one we reported from, giant renal angiomyolipoma can be treated safely with open nephrectomy.

Primary Retroperitoneal Mucinous Cystadenocarcinoma: A Case Report.

Mucinous neoplasms are commonly seen in the ovaries and pancreas. Their occurrence in the retroperitoneum is uncommon. We present a case of a retroperitoneal mucinous cystadenocarcinoma in a 54-year-old female who presented with right flank pain. Imaging demonstrated an 8.6 × 7.9 cm mass at the anterior surface of the lower pole of the right kidney, suspicious for renal cell carcinoma. Serum tumor markers carbohydrate antigen 19-9 (CA 19-9) and cancer embryonic antigen (CEA) were within normal limits, and cancer antigen 125 (CA 125) was elevated. Surgical resection of the mass was performed. Intraoperatively, the mass was noted to lie in the retroperitoneum, unattached to the kidney. On gross examination, a 10.0 × 7.0 × 7.0 cm unilocular cystic structure with red-brown mucoid material was present. The inner lining was mostly smooth with areas of excrescences, covering less than 5% of the surface area. Microscopic examination showed cystic areas lined by mucinous epithelium with an underlying ovarian-type stroma. Solid areas showed features of a borderline papillary mucinous tumor with invasive carcinoma. A diagnosis of mucinous cystadenocarcinoma was made. Their occurrence in the retroperitoneum is unusual. Although rare, this entity should always be considered in the differential diagnosis of retroperitoneal cystic lesions.

Retroperitoneal lymphatic malformations: A case report.

Retroperitoneal lymphatic malformation is a rare benign vascular malformation, of various locations, which can reveal itself at any age in its abdominal localizations. The retroperitoneal localization of this malformation is extremely rare. The clinical symptomatology is polymorphic depending on the lesion volume and the presence or absence of complications. The diagnosis is evoked by the liquid character of the retroperitoneal mass on ultrasound, CT scan and abdomino-pelvic MRI, brought during surgery and confirmed by the histological study of the surgical specimen. The treatment of choice is complete surgical removal of the mass.

Imaging of Ganglioneuroma: A Literature Review and a Rare Case of Cystic Presentation in an Adolescent Girl.

Retroperitoneal ganglioneuroma is a rare neuroectodermal tumor with a benign nature. We performed a literature review among 338 studies. We included 9 studies, whose patients underwent CT and/or MRI to characterize a retroperitoneal mass, which was confirmed to be a ganglioneuroma by histologic exam. The most common features of ganglioneuroma are considered to be a solid nature, oval/lobulated shape, and regular margins. The ganglioneuroma shows a progressive late enhancement on CT. On MRI it appears as a hypointense mass in T1W images and with a heterogeneous high-intensity in T2W. The MRI-"whorled sign" is described in the reviewed studies in about 80% of patients. The MRI characterization of a primitive retroperitoneal cystic mass should not exclude a cystic evolution from solid masses, and in the case of paravertebral location, the differential diagnosis algorithm should include the hypothesis of ganglioneuroma. In our case, the MRI features could have oriented towards a neurogenic nature, however, the predominantly cystic-fluid aspect and the considerable longitudinal non-invasive extension between retroperitoneal structures, misled us to a lymphatic malformation. In the literature, it is reported that the cystic presentation can be due to a degeneration of a well-known solid form while maintaining a benign character: the distinguishing malignity character is the revelation of immature cells on histological examination.

A Rare Cause of Abdominal Pain: Erdheim-Chester Disease.

A 65-year-old man presented with hematuria, night sweats, nausea, intermittent nonbloody diarrhea, and abdominal pain. Computed tomography angiogram with enterography showed retroperitoneal fibrosis surrounding both kidneys and ureters without any evidence of vascular obstruction or hydronephrosis. Laparoscopic biopsy demonstrated fibroadipose tissue involved by a subtle histiocytic infiltrate in a background of marked fibrosis, scattered lymphocytes, and plasma cells. The histiocytes strongly expressed CD163, Factor XIIIa, and BRAF V600E. He was diagnosed with Erdheim-Chester disease, a rare histiocytic neoplasm uncommonly presenting with gastroenterological manifestations.

Diffuse gastric polyposis in a young patient with a giant retroperitoneal mass: A case report.

Familial adenomatous polyposis (FAP) is characterized by hundreds of colonic adenomatous polyps and extraintestinal manifestations beginning in adolescence and early adulthood. It is also one of the most common hereditary colorectal cancer syndromes. In this case study, a rare phenotype of FAP associated with diffuse gastric polyposis, colon oligo-polyposis, and a massive retroperitoneal mass is described. The results expand on the current body of knowledge of FAP and may represent a new phenotypic expression of FAP. Accurate evidence-based surveillance and management recommendations for this disease require further research and evaluation.

Extrauterine Retroperitoneal Leiomyomas: 3 Case Reports and Review of the Literature.

Leiomyomas are benign mesenchymal tumors which originate from smooth muscle cells. Extrauterine leiomyomas are rare and they may arise where smooth muscle cells are found. Their diagnosis is challenging due to their heterogeneous ways of presentation. Histological analysis may reveal areas of sarcomatous differentiation; therefore, complete resection of the entire tumor is the only curative treatment. There is no adjuvant therapy proved to increase overall survival. It is essential to develop a standardized protocol, detailing how to follow up these patients since it is not reported in the literature to date; however, it is advisable to follow them because the local recurrence rate is high if small implants remain. In this review, we present 3 cases of extrauterine leiomyomas diagnosed and treated in our hospital. The management was different in each case, highlighting the heterogeneity of this condition. According to the literature, there are no solid guidelines on their management. We compare our experience with the data available to date in order to support the existing knowledge and provide our expertise for future studies.

Traumatic Rupture of Cystic Left Retroperitoneal Mass: An Atypical Presentation of Diffuse Large B-Cell Lymphoma.

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma. Though the presentation is diverse, patients typically have a history of "B" symptoms and lymphadenopathy in areas such as the neck, mediastinum, or abdomen. However, a growing body of evidence suggests DLBCL can present as a cystic mass in diverse tissues. We present the case of a large cystic left retroperitoneal mass of unknown origin in a patient subsequently diagnosed with DLBCL. The diagnosis was obtained via percutaneous biopsy of the cystic mass in preparation for surgical excision. Upon diagnosis, surgical intervention was aborted, and the patient was started on chemotherapy treatment. However, four weeks into her treatment, she slipped and fell while in the bathroom and presented to the emergency department in shock with a computed tomography (CT) scan suggestive of splenic rupture. She underwent emergent splenectomy and resection of the cystic mass. She was discharged on postoperative day 7 and is currently continuing with outpatient chemotherapy. The presentation of DLBCL is notoriously diverse, however, this patient represents a unique presentation that adds to a growing body of literature suggesting DLBCL can present as a cystic mass. Pathological diagnosis should be obtained in all patients with cystic lesions of unknown origin before any surgical intervention to avoid unnecessary surgery and provide an optimal management plan.