1, 1 January 2014 | Journal of Histology and Histopathology, Vol. Ganglioneuroma occasionally secretes hormones such as catecholamines, vasoactive intestinal peptides, or androgenic hormones (30). Lymphangioleiomyomatosis is a subtype of perivascular epithelioid cell tumor. Tumors with degenerative changes (ancient schwannomas) have hemorrhage, cystic changes, calcification, and hyalinization. Gastrointestinal and ovarian cancers are the most common etiologies. Table 3 Imaging Features Suggestive of a Specific Diagnosis for Retroperitoneal Masses. The CT attenuation at diagnosis is very homogeneous in most cases with minimal to no enhancement. Radionculide imaging performed after administration of m-iodobenzylguanidine (MIBG) shows high uptake in paragangliomas and is a sensitive technique for localizing these lesions (28). A cyst in an obese woman who is receiving hormonal therapy for irregular menstruation is likely to be a müllerian cyst. These tumors are believed to arise from aberrant primordial germ cell rests that are due to (a) faulty migration of germ cells from the yolk sac or endoderm to the urogenital ridge or (b) germ cells distributed physiologically to liver, bone marrow, and brain (36). Viewer. Fibrosis has low signal intensity on T2-weighted MR images, with minimal enhancement. Teratoma.—Teratoma is a germ cell tumor that originates from pluripotent germ cells that have been interrupted in their normal migration to the genital ridges. 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. Axial CT image shows a large heterogeneous mass with areas of curvilinear calcification (arrow) in the retroperitoneum. CT and MR imaging appearances are nonspecific and demonstrate a large, infiltrating, and heterogeneously enhancing soft-tissue mass with areas of necrosis and hemorrhage and with invasion of adjacent organs (Fig 5). The first step when diagnosing peritoneal or mesenteric masses is to separate them into cystic and solid. Rarely, paraganglioma can manifest with acute abdomen caused by retroperitoneal hemorrhage. Myxoid liposarcoma is seen in a younger population. The cyst is asymptomatic or produces pressure symptoms such as constipation or lower abdominal pain. 45, No. The imaging findings are nonspecific. Unlike peritoneal metastases, there are no tumor nodules. Miscellaneous Uncommon Neoplasms.—Chordoma is a tumor that arises from notochordal remnants and is seen most commonly in the sacrococcygeal region as a heterogeneously enhancing soft-tissue mass, with bone destruction of the sacrum and involvement of adjacent organs. 13, No. 12, American Journal of Roentgenology, Vol. 1, Journal of the Korean Society of Radiology, Vol. Knowing as much as possible about your enemy precedes successful battle and learning about the disease process precedes successful management 4. Retroperitoneal fibrosis radiology Ali Jiwani. Lymphangioleiomyoma is a retroperitoneal cystic mass that is produced because of lymphatic obstruction by proliferation of smooth muscle cells and the resultant dilated abdominal lymphatic vessels. 1, 29 October 2014 | Current Radiology Reports, Vol. 6, 18 September 2017 | Journal of Feline Medicine and Surgery Open Reports, Vol. Dr. Hinal Bhagat Assistant Prof., GMC Surat Radioimaging in Retroperitoneal Masses. Soft tissue masses with “cyst-like” appearance on MR imaging: distinction of benign and malignant lesions. CT shows a complex cystic mass, with areas of fat and calcification. It usually comes back and when it does, it comes back as a more aggressive tumor. 1, Journal of Clinical Ultrasound, Vol. Hodgkin lymphoma has a bimodal age distribution, occurring in patients in their 20s and 60s, and manifests with limited disease, often involving the mediastinum and spleen. The histopathologic findings from biopsy showed that this mass was a primary retroperitoneal embryonal carcinoma.Figure 17Download as PowerPointOpen in Image The hormonally active ovaries secrete fluid that becomes loculated in the pelvis. Malignant degeneration is more common with neurofibroma than with schwannoma, particularly in those patients who have neurofibromatosis (28).Figure 12 Neurofibroma in a 37-year-old man. Viewer. Figure 7 Angiosarcoma in a 61-year-old woman. Teratoma can be benign or malignant, and benign teratoma can be either mature or immature. The differential diagnosis includes benign thrombus, angiosarcoma, and a tumor extending to the IVC from adjacent organs. Primary retroperitoneal neoplasms are a rare but diverse group of benign and malignant tumors that arise within the retroperitoneal space but outside the major organs in this space. Table 2 Classification of Cystic Retroperitoneal Masses. Teratoma is more common in females, with a bimodal age distribution (<6 months and early adulthood). These images look quite similar to images of a pseudomyxoma peritonei which was discussed before. 19, No. Lymphangioma has enhancing septa. Tumors involving the superior segment of the IVC (above the hepatic veins) may manifest with Budd-Chiari syndrome, those of the middle segment (between the hepatic and renal veins) manifest with right upper quadrant pain or tenderness or the nephrotic syndrome, and tumors of the inferior segment (below the renal veins) manifest with pain and lower extremity edema (13). The aorta and IVC can be anteriorly displaced, producing the “floating aorta” or “CT angiogram” sign (Fig 20). Myxoid malignant fibrous histiocytoma, leiomyosarcoma, and other sarcomas are moderately vascular; and lymphoma, low-grade liposarcoma, and benign tumors are generally hypovascular, without appreciable contrast enhancement. The signs and symptoms are variable. For this journal-based CME activity, the authors (P.R., R.S., W.H.B. Angiomyolipoma.—Angiomyolipoma has varying amounts of blood vessels, smooth muscle cells, and adipose tissue. Lacy, linear, or amorphous areas of high signal intensity on T1-weighted images and intermediate signal intensity on T2-weighted images may be seen because of the intratumoral fat content. Xray film & film processing Rakesh Ca. A midline mass is more suggestive of a primary extragonadal germ cell tumor than of metastasis. Malignant retroperitoneal fibrosis is produced when small retroperitoneal neoplastic foci elicit a desmoplastic response, and malignant retroperitoneal fibrosis may be difficult to distinguish from nonmalignant retroperitoneal fibrosis. Viewer. Variations in the size of the mass have been reported with diet, time of the day, and gravitational factors (59). When ultrasound and CT were introduced direct imaging of retroperitoneal lesions became possible. Enlarged vessels coursing through the mass, aneurysms, and associated hemorrhage are features that differentiate an angiomyolipoma from a liposarcoma. Lipomatosis is more common in males (18:1), particularly African Americans, with a mean age of 48 years, and is not associated with obesity. Lastly we have to know which cystic masses are common and look for specific features of these masses. Retroperitoneal masses not arising from major solid organs are uncommon. Retroperitoneal fibrosis does not displace the aorta and IVC anteriorly, as lymphoma or metastatic nodes often do, but causes tethering of these structures to the underlying vertebrae. 3, Journal of the Korean Society of Radiology, Vol. Extramedullary hematopoiesis can be seen in any organ of mesenchymal origin, although it is more common in the liver, spleen, and lymph nodes (49). After reading this article and taking the test, the reader will be able to: Describe the various solid and cystic retroperitoneal masses. 36, No. Extragonadal germ cell tumor is often seen in or near the midline, especially between the T6 and S2 vertebrae. Note the surgical clip (curved arrow) anterior to the right psoas major.Figure 35Download as PowerPointOpen in Image Extramedullary hematopoiesis is characterized by abnormal deposits of hematopoietic tissue outside the bone marrow as a compensatory mechanism for reduced hematopoiesis by the bone marrow. Viewer. Amorphous punctate calcification may also be seen in the myxoid subtype. 2, 15 January 2015 | RadioGraphics, Vol. 3, Journal of Gastric Cancer, Vol. 6, Current Problems in Diagnostic Radiology, Vol. The well-differentiated subtype, the most common type of retroperitoneal liposarcoma (1), is a predominantly hypoattenuating lesion on CT images because of its fat content. Histopathologically, the tumor is encapsulated and multicystic, filled with serous fluid and protruding papillary nodules. 8, 3 December 2014 | Abdominal Imaging, Vol. Lee ES, Kim JH, Yu MH, Choi SY, Kang HJ, Park HJ, et al. Generally, ganglioneuroma is larger and has more calcification than a nerve sheath tumor. CT and MR imaging are valuable in the evaluation of retroperitoneal masses, particularly in staging and the assessment of vascular invasion. On the left another case of pseudomyxoma peritonei. J Oncol, Retroperitoneal masses: magnetic resonance imaging findings with pathologic correlation, Post-transplant lymphoproliferative disease in liver transplantation, CT features of abdominal plasma cell neoplasms, Abdominal manifestations of multiple myeloma: a retrospective radiologic overview, Neoplastic and nonneoplastic proliferative disorders of the perirenal space: cross-sectional imaging findings, Retroperitoneal fibrosis: a review of clinical features and imaging findings, Erdheim-Chester disease: clinical and radiologic characteristics of 59 cases, Typical and atypical presentations of extramedullary hemopoiesis, Retroperitoneal extramedullary hematopoiesis: sonographic, CT, and MR imaging appearance, Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review, CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients, Cystic angiomatosis: case report and review of the literature, Generalized lymphangiomatosis: radiologic findings in three pediatric patients, Lymphangioleiomyomatosis: radiologic-pathologic correlation, Delayed enhancement of pelvic lymphangiomyoma associated with lymphangioleiomyomatosis on MR imaging (2005: 9b), Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation, Primary retroperitoneal mucinous cystadenocarcinoma: a case report and review of the literature, A primary retroperitoneal serous cystadenocarcinoma with clinically aggressive behavior, Open in Image (b) In a 65-year-old woman, mixed pattern is shown in a dedifferentiated liposarcoma that contains fat (arrowhead) and a large enhancing soft-tissue component (arrow). Contrast enhancement is heterogeneous, with nonenhancing cystic components and enhancing solid components. Mesenteric cyst is a descriptive term for any cystic lesion within the mesentery. Report of a case and review of the literature, Multidetector CT and MR Imaging Findings of Renal Vein Leiomyosarcoma with Both Extraluminal and Intraluminal Components: A Case Report, Retroperitoneal sarcomas: A review of disease spectrum, radiological features, characterisation and management, A Comprehensive Review of the Retroperitoneal Anatomy, Neoplasms, and Pattern of Disease Spread. Floating aorta sign: Floating aorta sign: encasement of aorta by retroperitoneal mass. ABSTRACT Background-Retroperitoneal neoplasm, a part of abdominal cavity, is diverse group of benign and malignant neo-plasm. 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