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Ganglioneuroblastoma in a 3-year-old female. A Axial contrast-enhanced CT abdomen shows a large, round, well-defined cystic appearing mass on the right-sided retroperitoneum (asterisk). B Photograph of the gross specimen reveals a large ovoid rubbery thinly encapsulated mass (518 g). Cut surface shows tan, multilobulated firm appearance with foci of hemorrhage and calcification. C Photomicrograph (200×; H–E stain) demonstrates ganglion cells (arrow), which are cells with abundant cytoplasm, rounded contour, and large nuclei. Neuroblasts, which are the dark purple cells with scant cytoplasm (arrowhead), and neuropil, which is the fine, fibrillary light pink-staining material (asterisk)
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The retroperitoneum is a large space where primary and metastatic tumors grow silently before clinical signs appear. Neoplastic retroperitoneal diseases may be solid or cystic, primary or secondary and range from benign to aggressive in behavior. Retroperitoneal neoplasms are notable for their widely disparate histologies. The solid primary retrope...
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... IVCS is associated with a wide spectrum of malignancies. Imaging data usually enables radiologists and oncologists to narrow the differential diagnoses and to define staging strategies [59][60][61][62][63][64][65][66]. Nevertheless, histology and molecular genetics are important for an exact tumor diagnosis and often determine therapeutic decisions and over-all prognosis. ...
Inferior vena cava syndrome (IVCS) is caused by agenesis, compression, invasion, or thrombosis of the IVC, or may be associated with Budd-Chiari syndrome. Its incidence and prevalence are unknown. Benign IVCS is separated from malignant IVCS. Both cover a wide clinical spectrum reaching from asymptomatic to highly symptomatic cases correlated to the underlying cause, the acuity, the extent of the venous obstruction, and the recruitment and development of venous collateral circuits. Imaging is necessary to determine the underlying cause of IVCS and to guide clinical decisions. Interventional therapy has changed the therapeutic approach in symptomatic patients. This article provides an overview over IVCS and focuses on interventional therapeutic methods and results.
... [1] Although the posterior peritoneum is the second most common site for extragonadal germ cell tumors, these tumors account for only 5% of primary retroperitoneal tumors. [2] Primary retroperitoneal germ cell tumors almost exclusively affect men. Herein, we report a case of primary retroperitoneal germ cell tumor in a woman along with detailed clinical imaging and literature review. ...
... This is because 30% to 50% of testicular gonadal tumors can give rise to retroperitoneal metastasis, accounting for the majority of retroperitoneal germ cell tumors. [2] However, unlike previous cases, the present case was a female. Moreover, because there was no primary lesion in the pelvic cavity, our case could be excluded from metastatic tumor cell tumors. ...
... [11] Primary retroperitoneal seminoma can be shown as an isolated retroperitoneal mass on CT, measuring 1 to 30 cm, with uniform density, regular or irregular margins, and clear boundaries. [1,2,5,9,11] Tumors can easily surround and further invade blood vessels (eg, vena cava, abdominal aorta, iliac vessels) and invade the ureter. [5,9,11] Through contrast-enhanced scanning, primary retroperitoneal seminoma can be characterized based on mild-to-moderate uniformity or uneven enhancement, [5,9,11] with segregated or punctate calcification in lesions. ...
Rationale:
Primary retroperitoneal germ cell tumors are uncommon and especially rare in female patients. However, this type should be included in the differential diagnosis of retroperitoneal tumors that may metastasize from the gonads and be a primary tumor.
Patient concerns:
An abdominal mass was detected in a 38-year-old woman during physical examination, which was accompanied by left renal obstructive hydrops. She was admitted to our institution for further investigation. The patient had no obvious clinical symptoms, and the levels of serum tumor markers did not signifificantly increase. Abdominal noncontrast enhanced and contrast-enhanced computed tomography revealed a retroperitoneal neoplasm that invaded the left ureter, thereby causing left hydronephrosis.
Diagnoses:
Imaging examination characterized the tumor as malignant based on its invasion in the left ureter. Histopathology and immunohistochemistry confifirmed the resected tumor as a dysgerminoma. The primary gonad-derived germ cell tumor was not found in the pelvis; therefore, the patient was fifinally diagnosed with primary retroperitoneal germ cell tumor.
Interventions:
Preoperative examination was completed, and the retroperitoneal mass was resected.
Outcomes:
During the short-term follow-up, no tumor recurrence was detected.
Lessons:
Primary retroperitoneal seminoma should be included in the differential diagnosis of primary retroperitoneal tumors in female patients. The primary retroperitoneal seminoma/anaplastic tumor has an obvious occupying effect and can easily invade the surrounding structures. However, surgical resection of such tumors is an optional treatment strategy.
... Органите на ретроперитонеума могат да бъдат предимно ретроперитонеални (включително тези на надбъбречните жлези, бъбреците, уретерите, аортата и долната вена кава, както и част от ректума и гръдния хранопровод) и вторично ретроперитонеални (тези, които са мигрирали към ретроперитонеума по време на своето развитие -главата, шията и панкреасното тяло, от втората до четвъртата част на дванадесетопръстника, възходящо и низходящо двоеточие). Ретроперитонеалните тумори включват голямо разнообразие от неоплазми с различна хистология, чийто произход може да бъде от органите, които го съставят, от съседни структури, независими от органите, и метастатични [1]. ...
Аbstract: Primary retroperitoneal masses, which originate in the retroperitoneum as well as outside the retroperitoneal organs, are uncommon and can be divided primarily into the solid and cystic masses, which can be further subdivided into neoplastic and non-neoplastic masses. A reasonable approach is to consider the masses as predominantly solid or cystic and to subdivide these into neoplastic and non-neoplastic masses. Because the treatment options vary, it is useful to be able to differentiate these masses by using imaging criteria. Although the differential diagnosis of retroperitoneal masses can be narrowed down to a certain extent on the basis of imaging characteristics, patterns of involvement, and demographics, there is still a considerable overlap of imaging findings for these masses, and histologic examination is often required for definitive diagnosis. Ultrasound(US), Computed tomography (CT) and magnetic resonance (MR) imaging play an important role in characterization and in the assessment of the extent of the disease and involvement of adjacent and distant structures. Familiarity with the US, CT and MR imaging features of various retroperitoneal masses will facilitate accurate diagnosis and staging for aggressive lesions. The purposes of this review are: 1. To present the imaging findings that are suspicious of tumors of retroperitoneal origin containing solid, liquid and fat components. 2. To illustrate the most common imaging characteristics of these focal lesions of US, CT and MRI studies for obtaining adequate diagnosis.
... O lipossarcoma bem diferenciado é o subtipo mais comum, contém tecido adiposo maduro e se caracteriza por massa infiltrativa em relação às estruturas adjacentes (3,4,14,18) . Entre as características de imagem que favorecem esse diagnóstico, em detrimento de uma lesão benigna, está o tamanho da lesão superior a 10 cm e a presença de septos espessos (> 0,2 cm) e de focos de realce nodular (3,19,20) . ...
... Entre as lesões sólidas que podem conter calcificações estão os tumores neurogênicos, os lipossarcomas desdiferenciados, o teratoma e o sarcoma pleomórfico indiferenciado (5,18) . ...
... Lipossarcoma desdiferenciado -Apresenta calcificações em cerca de 30% dos casos, e quando essas calcificações estão associadas a nódulos com realce heterogêneo, sugerem áreas de desdiferenciação tumoral e pior prognóstico (2,18,31,32) (Figura 3). Na investigação dos lipossarcomas, a 18 F-FDG PET/CT pode auxiliar na avaliação do grau tumoral, assim como no estadiamento e no seguimento pós-cirúrgico, por meio da captação dos componentes sólidos da lesão, sobretudo nos subtipos desdiferenciado e pleomórfico (18,22,32) . Alguns estudos apontam que o grau tumoral se correlaciona com o metabolismo glicolítico (22) . ...
Primary retroperitoneal masses constitute a heterogeneous group of uncommon lesions and represent a challenge due to overlapping imaging findings. Most are malignant lesions. Although they are more prevalent in adults, they can occur at any age. Such lesions are classified as primary when they do not originate from a specific retroperitoneal organ and are divided, according to the image findings, into two major groups: solid and cystic. The clinical findings are nonspecific and vary depending on the location of the lesion in relation to adjacent structures, as well as on its behavior. The main imaging methods used for staging and surgical planning, as well as for selecting the biopsy site and guiding the biopsy procedure, are computed tomography and magnetic resonance imaging. In most cases, the treatment is challenging, because of the size of the lesions, vascular involvement, or involvement of adjacent organs. In this article, we present a review of the retroperitoneal anatomy and a practical approach to the main imaging features to be evaluated, with a view to the differential diagnosis, which can guide the clinical management.
... Neurogenic tumors account for 10% to 20% of retroperitoneal neoplasm and are classified as ganglion cell origin (ganglioneuromas), paraganglionic system origin (paragangliomas), or nerve sheath origin (neurilemmomas/schwannomas, neurofibromas, and malignant nerve sheath tumors). 27,28 Ganglioneuromas, neurofibromas, and schwannomas are mainly myxoid stroma tumors because paragangliomas are classified as cystic/ necrotic tumors. 2,12 Overall, neurogenic tumors tend to show a fusiform shape, increased T2-weighted signal intensity, and highly intense contrast enhancement. ...
Retroperitoneal diseases are frequently a great clinical challenge because of their nonspecific presentation. Diseases may be asymptomatic with indolent biological behavior, and often show a large mass at initial imaging evaluation. Magnetic resonance (MR) imaging is a powerful diagnostic technique for assessment of detailed disease location and for characterization of tissue components and vascularization of lesions, with many advantages compared with computed tomography and ultrasonography. This article presents the MR imaging features of common retroperitoneal pathologic processes.
... Evidence of degeneration, which includes cysts, subacute haemorrhage and microscopic fat, was common for retroperitoneal extra-adrenal paragangliomas and schwannomas, and all tended to be observed [28]. Takatera et al. [19] reported that 66% retroperitoneal schwannomas showed cystic degeneration, while schwannomas demonstrated an exceptional risk of degeneration [11,29]. In this study, more than 90% of retroperitoneal schwannomas showed the same feature, which was only found in 62.5% of retroperitoneal extra-adrenal paragangliomas, highlighting that fact that this feature is helpful in the differential diagnosis. ...
Background:
To determine whether MRI feature analysis can differentiate benign retroperitoneal extra-adrenal paragangliomas and schwannomas.
Methods:
The MRI features of 50 patients with confirmed benign retroperitoneal extra-adrenal paragangliomas and schwannomas were retrospectively reviewed by two radiologists blinded to the histopathologic diagnosis. These features were compared between two types of tumours by use of the Mann-Whitney test and binary logistic regression. The patients' clinical characteristics were reviewed.
Results:
Analysis of MRI images from 50 patients revealed no significant differences in the quantitative MRI features of lesion size, ratio of diameter and apparent diffusion coefficient. There were significant differences in the qualitative MRI features of location, necrosis, cysts and degree of tumour enhancement for two readers, with no significant differences in the other qualitative MRI features between these tumours. The combination of necrosis with degree of tumour enhancement during the arterial phase increased the probability that a retroperitoneal mass would represent retroperitoneal extra-adrenal paraganglioma as opposed to schwannoma.
Conclusion:
We have presented the largest series of MRI features of both benign retroperitoneal extra-adrenal paragangliomas and schwannomas. Some MRI features assist in the differentiation between these tumours, with imaging features consisting of necrosis and avid enhancement during the arterial phase, suggestive of retroperitoneal extra-adrenal paragangliomas.
Retroperitoneal non-organ-originated malignancies are rare pediatric tumors with challenging diagnosis and treatment. The present study aimed to analyze the clinicopathological characteristics, treatment, and prognosis of retroperitoneal non-organ-originated malignancies. In the study, we included the pathological diagnosis of pediatric retroperitoneal non-organ-originated malignant tumors between 2000 to 2019 through the updated Surveillance, Epidemiology, and End Results database. We use the Kaplan–Meier survival curve to calculate the overall survival (OS) and cancer-specific survival (CSS). The risk of all-cause death and disease-specific death were analyzed using Cox proportional hazard regression model and Fine-and-Grey competitive hazard model, respectively. In the study, a total of 443 pediatric retroperitoneal non-organ-originated malignancies were included. Of them, only 22.3% of patients had no metastatic disease, 42.9% had distant metastasis and 34.8% had locally advanced diseases. The primary pathological tumor was neuroblastoma followed by germ cell tumor. The overall 10-year OS and CSS were 70.7% and 73.1%, respectively, and the 10-year OS and CSS of metastatic diseases were 54.4% and 56.6%, respectively. Older children, worse tumor stage at diagnosis, incomplete resection, and prolonged time from diagnosis to treatment were significantly associated with worse survival outcomes. Radiotherapy and chemotherapy did not significantly improve the prognosis of patients without complete tumor resection. The study indicated that most pediatric retroperitoneal non-organ-originated malignancies diagnosed with metastatic diseases have plagued treatment. Radiotherapy and chemotherapy are the main treatment methods for children unable to undergo complete surgical treatment. However, these treatments do not reach the same therapeutic effect as complete tumor resection after early diagnosis. Hence, early diagnosis and surgery for complete tumor resection are of utmost importance.
Imaging techniques represent clinical decision-making tools. Abdominopelvic imaging is moving from the pure anatomical imaging techniques to functional-molecular imaging or imaging-based analysis of tumor heterogeneity and complexity. The introduction of advanced imaging techniques, such as diffusion-weighted imaging (DWI), may improve our assessment of diagnosis, prognosis, planning therapy, and tumor response evaluation. This chapter will review recent developments in DWI and the evolving role of this imaging technique in the assessment of renal, adrenal, and retroperitoneal lesions.
1Alcalá de Henares. Madrid/ES, 2Salamanca/ES
