Imaging Pearls ❯ Small Bowel ❯ Benign Tumors
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- “Castleman disease (CD) is a group of rare and complex lymphoproliferative disorders that can manifest in two general forms: unicentric CD (UCD) and multicentric CD (MCD). These two forms differ in clinical manifestation, imaging appearances, treatment options, and prognosis. UCD typically manifests as a solitary enlarging mass that is discovered incidentally or after development of compression-type symptoms. MCD usually manifests acutely with systemic symptoms including fever and weight loss. As a whole, CD involves lymph nodes throughout the chest, neck, abdomen, pelvis, and axilla and can have a wide variety of imaging appearances.”
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021 - “Most commonly, lymph nodes or masses in UCD occur in the chest, classically with well-defined borders, hyperenhancement, and possible characteristic patterns of calcification and/or feeding vessels. Lymph nodes affected by MCD, while also hyperenhancing, tend to involve multiple nodal chains and manifest alongside anasarca or hepatosplenomegaly. The polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) subtype of MCD may demonstrate lytic or sclerotic osseous lesions in addition to features typical of MCD. Since a diagnosis of CD based solely on imaging findings is often not possible, pathologic confirmation with core needle biopsy and/or surgical excision is necessary. Nevertheless, imaging plays a crucial role in supporting the diagnosis of CD, guiding appropriate regions for biopsy, and excluding other potential causes or mimics of disease.”
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021 - - Castleman disease (CD), also known as angiofollicular lymph node hyperplasia, is a complex lymphoproliferative disorder broadly divided into two forms:unicentric CD (UCD) and multicentric CD (MCD). The disease likely encompasses at least four different entities with overlapping histopathologic features but distinct causes, clinical manifestations, radiologic features, treatment approaches, and outcomes.
- Most commonly, UCD manifests as a solitary enlarging mass, which can be discovered incidentally at imaging performed for other reasons, be palpated at physical examination, or be discovered owing to compression-type symptoms on adjacent structures.
- CD most commonly manifests as unicentric disease with various sites of involvement. The chest is the most common site (30%–70%), followed by the neck (10%–40%), abdomen and pelvis (12%–39%), and axilla (4%–5%).
- Multiple modalities may demonstrate prominent feeding vessels to the lymph node or mass, which can affect the surgical approach.
- Lymphoma is a common mimic that is often a diagnostic consideration for presumed CD in different parts of the body. Lymphoma can be confused with CD in the mediastinum, MCD, or nonspecific masses in other parts of the body.
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021 - “Lymph nodes in MCD tend to be hypervascular, similar to those in UCD, although some reports suggest that nodal masses associated with HHV8-MCD may not show substantial enhancement. MCD in the chest typically manifests as cervical, hilar, mediastinal, or axillary lymphadenopathy, which affects multiple nodal chains. Pleural or pericardial effusions are common. Lung involvement may appear as centrilobular nodules and nodular opacities and less frequently as ground-glass attenuation, airspace consolidation, and bronchiectasis. Although unusual, when direct pleural involvement occurs, it can manifest as a well-defined pleural-based mass with or without associated pleural effusion.”
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021 - “Lymphoma is a common mimic that is often a diagnostic consideration for presumed CD in different parts of the body .Lymphoma can be confused with CD in the mediastinum, MCD, or nonspecific masses in other parts of the body .At imaging, increasing adenopathy, ascites, or new splenomegaly may indicate a diagnosis of lymphoma in the setting of known CD . Nodal masses with higher SUVmax are alsoconcerning for either non-Hodgkin lymphoma (NHL) or Hodgkin lymphoma, as the median SUV of lymph nodes involved by CD tends to be slightly lower.”
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021 - “In the absence of complications or development of lymphoma, UCD typically has no significant effect on life expectancy. Available data on outcomes and prognosis in MCD are more limited. The clinical course of patients with MCD is variable: some patients may present with indolent and slowly progressive disease, others may experience a relapsing-remitting course, and still others may present with fulminant disease that can be fatal within the course of weeks. In one study, 35% of patients diagnosed with iMCD were reported to have died within 5 years of diagnosis and 60% were reported to have died within 10 years. Patients also have a threefold increased prevalence of malignancy.”
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021 - “CD is a complex and heterogeneous group of lymphoproliferative disorders that can manifest with varying clinical, imaging, and histopathologic features. UCD typically manifests as an incidental solitary enlarging mass, whereas MCD manifests acutely with systemic manifestations. A multidisciplinary approach to CD including clinical information, laboratory evaluation, and imaging workup is necessary to select appropriate regions for biopsy, exclude potential mimics, differentiate UCD from MCD, arrive at an accurate diagnosis, and ultimately manage CD. Imaging can be used to monitor response to treatment and detect development of any complications such as lymphoma. Surgical excision is typically curative for UCD, while MCD requires systemic therapy with a more guarded prognosis.”
Imaging of Castleman Disease
Marika A. Pitot et al.
RadioGraphics 2023; 43(8):e22021
- “The most common DTs are sporadic and extraabdominal ones (extremities, head-neck, and chest wall/ breast). The typical clinical presentation is a slow growing painless or minimally symptomatic soft tissue mass. Approximately 30% of patients have tumors related to familial adenomatous polyposis (FAP), and in these patients, intra-abdominal location is the most common type and can be also multifocal. Intra-abdominal DTs’ clinical presentation can be typically as a slow-growing mass but can have also acute presentation such as intestinal ischemia or obstruction.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103 - Desmoid Tumors: Facts
- 30% of familial adenomatous polyposis (FAP) patients have desmoid-type fibromatosis (also called Gardner syndrome); 7.5–
- 16% of patients with fibromatosis have FAP.
- Mean age: 36–42 years.
- Female predominance from puberty to age 40; younger and older patients have M:F = ~ 1:1.
- Male predominance in FAP of 3:1. - Extra-Abdominal Desmoid Tumors: Facts
The most common locations of extra-abdominal DTs are as follows:
- 10% head and neck
- 33% shoulder and upper extremity
- 17% chest wall or back
- 30% gluteal region and lower extremity
- DTs are multiple in 15% of cases - “Intra-abdominal DTs’ incidence significantly differs between sporadic and FAP-related cases: only 5% of sporadic DTs are intra-abdominal ones, whereas 80% of cases of FAP-related DTs are intra-abdominal (especially mesenteric ones) . Abdominal DTs can be divided into pelvic or mesenteric lesions.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103 - “Mesenteric DT is the most common primary tumor of the mesentery . The clinical presentations range from a painless palpable abdominal mass to bowel obstruction or perforation and chronic hydronephrosis due to ureteral infiltration. Mesentery is the most common DT location in the Gardner syndrome.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103 - “CT is considered as the first level imaging technique for a prompt diagnosis of intra-abdominal DT and their possible acute complications especially in the Emergency Department. CT scans show mesenteric DT often appearing as soft tissue mass with ill-defined margins and radiating spicules extending into the adjacent mesenteric fat (“whorled appearance” ), but it can also appear as a well-demarcated lesion.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103 - “DTs are usually visualized as a large mass (> 15 cm), isodense to the muscle. Uncommonly, it can entrap ureters or encase small bowel loops, leading to intestinal perforation or obstruction. Otherwise, in patients with FAP, lesions are smaller and multiple.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103 - DTs are uncommon, locally aggressive tumors with a high risk of recurrence. There are several therapeutic options, including “wait-and-see policy,” surgery, radiotherapy, chemotherapy, and hormonal and molecular targeted drugs. A multidisciplinary approach for a “tailored therapy” is usually needed: in this clinical scenario, radiologists play a crucial role to make a correct diagnosis and to guide the proper management, depending on the location, imaging features, and clinical presentation of these kinds of masses.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103 - Mesenteric Desmoid Tumor: Differential Diagnosis
- Lymphoma
- GIST tumor
- Carcinoid tumor
- Sclerosing mesenteritis
- "Desmoid tumors are rare, locally aggressive, nonencapsulated masses resulting from a benign proliferation of fibrous tissue. Abdominal desmoids can occur sporadically and develop anywhere in the abdomen, including the musculature of the abdominal wall, the retroperitoneum, and the pelvis. However, desmoids forming in the mesentery are especially common in patients with familial adenomatous polyposis (Gardner syndrome), occurring in 9%–18% of cases."
Mesenteric Neoplasms: CT Appearances of Primary and Secondary Tumors and Differential Diagnosis
Sheth S, Horton KM, Garland MR, Fishman EK
RadioGraphics Mar 2003, Vol. 23, No. 2:457–473
- “A lymphangioma is a benign proliferation of lymph vessels, producing fluid filled cysts that result from a blockage of the lymphatic system. ae incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge
Oguzhan Güven Gümüştaş et al.
Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “The incidence of abdominal lymphangiomas is unknown: however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Although retroperitoneal lymphangiomas may sometimes be asymptomatic, they usually present as a palpable abdominal mass and are easily confused with other retroperitoneal cystic tumors including those arising from the liver, kidney and pancreas.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge
Oguzhan Güven Gümüştaş et al.
Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “Neurofibromatosis type 1 (NF1) is a neurocutaneous syndrome that occurs in one in 3000 live births (42). It is autosomal dominant, with 100% penetrance, and it is characterized by a mutation in chromosome 17q11.2 that codes for a protein known as neurofibromin, a guanosine triphosphatase–activating protein for the group of p21ras (Ras) proteins that is expressed in the brain, kidneys, spleen, and thymus.”
Abdominal Manifestations of Neurologic Disorders
Anil S. Bhavsar et al.
RadioGraphics 2013; 33:135–153 - “The most common abdominal manifestation of NF1 is a plexiform neurofibroma that arises in the retroperitoneum. Malignant transformation is indicated by intense contrast enhancement. Gastrointestinal involvement is seen in 10%–25% of patients. Those with NF1 also have an increased risk for gastrointestinal stromal tumors, mesenchymal neoplasms that arise from the Cajal cells of the myenteric plexus, and angiomyolipomas.”
Abdominal Manifestations of Neurologic Disorders
Anil S. Bhavsar et al.
RadioGraphics 2013; 33:135–153 - “In NF1, gastrointestinal stromal tumors are frequently multiple and located within the small bowel, and there is an increased risk for development of carcinoid tumors near the ampulla of Vater. Pheochromocytomas are also more common in patients with NF1 than in the general population, occurring in approximately 5% of normotensive patients with NF1 and as many as 50% of hypertensive patients with NF1. Information regarding the effect of NF1 on mortality is limited, although life expectancy appears to be shortened (59 years for those with NF1 versus 74 years for those without the condition), according to data obtained in 1997.”
Abdominal Manifestations of Neurologic Disorders
Anil S. Bhavsar et al.
RadioGraphics 2013; 33:135–153 - “Mesenteric involvement in NF1 has been described and should not be confused with hereditary intestinal neurofibromatosis, a rare but clinically distinctive genetic disease in which involvement is limited to the small bowel and colon. Intestinal involvement in NF1 rarely occurs in isolation and is more typically preceded and accompanied by peripheral manifestations of the disease. Abnormal neuronal tissue may involve the entire gastrointestinal tract but is more typically seen in the small bowel and colon.”
Neurofibromatosis Type 1: A Diagnostic Mimicker at CT
Brian J. Fortman, Brian S. Kuszyk, Bruce A. Urban, Elliot K. Fishman
RadioGraphics 2001; 21:601–612 - “More commonly, when abdominal involvement occurs in NF1, tumors tend to arise in the retroperitoneal, mesenteric, and paraspinal regions. Focal involvement of individual organs is rare but does occur. Plexiform tumors with mesenteric infiltration may cause vascular narrowing and compromise blood flow. Affected patients typically present with secondary hypertension or occasionally with intestinal ischemia and abdominal pain. Stenosis of the mesenteric vasculature in NF1 may also be seen as a result of primary mesodermal abnormalities of the vessel wall.”
Neurofibromatosis Type 1: A Diagnostic Mimicker at CT
Brian J. Fortman, Brian S. Kuszyk, Bruce A. Urban, Elliot K. Fishman
RadioGraphics 2001; 21:601–612 - “At CT, multiple well-defined neurofibromas arising from nerves traversing the mesentery or retroperitoneum may be quite extensive and difficult to distinguish from adenopathy such as that seen with lymphoma. Often, these masses may have very low attenuation and mimic other disease processes that cause low-attenuation adenopathy (eg, tuberculosis, Whipple disease, mycobacterium avium complex, metastatic disease from tumors such as seminoma).”
Neurofibromatosis Type 1: A Diagnostic Mimicker at CT
Brian J. Fortman, Brian S. Kuszyk, Bruce A. Urban, Elliot K. Fishman
RadioGraphics 2001; 21:601–612
- “Lymphangioma is a rare, benign congenital malformation of the lymphatic system that usually affects the neck and head in children. Intra-abdominal lymphangioma accounts for less than 5 percent of all cases of lymphangioma. The clinical presentation of intra-abdominal lymphangioma can vary from asymptomatic to nausea, vomiting, and abdominal pain. The diagnosis of intra-abdominal lymphangioma is based on imaging modalities and histopathological examination. The definitive treatment is surgical resection.”
Intra-abdominal lymphangioma: A case report.
Shayesteh S, Salimian KJ, Fouladi DF, Blanco A, Fishman EK, Kawamoto
S Radiol Case Rep. 2020 Nov 11;16(1):123-127. - "Lymphangiomas are uncommon, noncancerous lymphatic malformations composed of thin-walled cysts. While lymphangiomas are most often found in the neck, head, and axillary regions, in fewer than 5 percent of lymphangiomas can present intra-abdominally, including the mesentery, greater omentum, and rarely in the retroperitoneum. They can occur at any age but predominantly appear in pediatric populations with a great prognosis.”
Intra-abdominal lymphangioma: A case report.
Shayesteh S, Salimian KJ, Fouladi DF, Blanco A, Fishman EK, Kawamoto S
Radiol Case Rep. 2020 Nov 11;16(1):123-127. - ” Lymphangioma is characterized as an uncommon, benign fluid-filled cystic anomaly originating in lymphatic vessels with an excellent prognosis that often appears in the head and neck with high prevalence in children and young adults with equal occurrence between males and females. Intra-abdominal lymphangioma is one of the very rare intra-abdominal tumors that accounts for only 5% of all lymphangiomas. Although the exact etiology is not clear, infection, lymphatic obstruction, and surgery could be risk factors for the development of this anomaly of the lymphatic system. They are classified into different subtypes based on microsporocyte characteristics. Capillary lymphangiomas are made of small, thin-walled lymphatic vessels located in the skin. The cavernous type consists of dilated lymphatic channels of various sizes that keep their connection with normal lymphatic vessels.”
Intra-abdominal lymphangioma: A case report.
Shayesteh S, Salimian KJ, Fouladi DF, Blanco A, Fishman EK, Kawamoto S
Radiol Case Rep. 2020 Nov 11;16(1):123-127. - “Cystic mesenteric lymphangioma is a benign peritoneal tumor. It is a spontaneous malformation composed by lymphatics vessels and lymphoid tissue. It is the most common cystic mesenteric tumor. It frequently appears in children, in 60% of cases before one year old and is rare in adult people . It localizes frequently in neck, facial, axillar and thoracic regions in 95%. The abdominal intra-peritoneal localization is out of ordinary. The diagnosis is difficult in preoperative because of polymorphous clinical symptomatology. The imagery helps for the diagnosis.”
Cystic mesenteric lymphangioma: A case report,
Ousmane Thiam et al.
International Journal of Surgery Case Reports, Volume 61,2019,Pages 318-321 - "Cystic lymphangioma is a benign and rare tumor of lymphatics vessels. It is localized in subcutaneous tissue of face and neck (60%), limb (20%), thoracic (10%) and axillar regions. The abdominal (2–10%), and thoracic (5%) localizations are rare. In abdomen, the lesions are preferentially localized in mesentery and the epiploon, and also found in the liver, spleen, pancreas, kidney, adrenal gland, large intestine and duodenum. In less than 10% of cases, lesions are diffuse with a peritoneal cystic lymphangiomatosis, similar to peritoneal carcinosis.”
Cystic mesenteric lymphangioma: A case report,
Ousmane Thiam et al.
International Journal of Surgery Case Reports, Volume 61,2019,Pages 318-321 - "Cystic lymphangioma is a rare evolutive malformation benign tumor, requiring a complete surgical excision to minimize any recidivism. Aggressive surgery with extensive resection of adjacent organs to the cystic lymphangioma is prohibited because of its benign character. The positive diagnosis is histological. In case of surgical abstention, the patient should undergo a regular screening with ultrasound and a medical treatment. Persistent symptoms under medical treatment indicate a surgery. The per cutaneous treatment is reserved for cases requiring an extensive resection.”
Cystic mesenteric lymphangioma: A case report,
Ousmane Thiam et al.
International Journal of Surgery Case Reports, Volume 61,2019,Pages 318-321
- “Adenomas are benign tumors that arise from glandular epithelium. Adenomatous polyp and villous adenoma are terms related to the growth pattern and morphology of adenomas.They are characterized histologically as tubular, villous, or tubulo-villous. Adenomas are most commonly found in the duodenum, particularly in the vicinity of the ampulla of Vater, where they tend to be of the villous subtype.They are least common in the ileum, where they are usually of the tubular subtype.”
Small Bowel Neoplasms: A Pictorial Review
Jasti R, Carucci LR
RadioGraphics 2020;40:1020-1038 - "Important polyposis syndromes that involve the small bowel and are reviewed in this article include familial adenomatous polyposis and Peutz-Jeghers syndrome. Polyposis syndromes are a group of disorders characterized by multiple polyps affecting part or all of the gastrointestinal tract. Lesions in these conditions are usually diagnosed relatively early, in a screening setting as compared with small bowel lesions overall. Also, patients tend to have multiple small bowel lesions and may present because of symptoms due to intermittent intussusception.”
Small Bowel Neoplasms: A Pictorial Review
Jasti R, Carucci LR
RadioGraphics 2020;40:1020-1038
- “Mesenteric cysts are rare, generally benign intra-abdominal lesions with an incidence ranging from 1 in 105,000 to 1 in 250,000 among admitted surgical patients. Mesenteric cysts have a wide range of presentation in terms of size, clinical presentation, etiology, radiological features, and pathological characteristics. In fact, the average size ranges from 2 to 35 cm and, thus, patients present with nonspecific complaints of abdominal pain, distension, or an abdominal mass.”
Giant mesenteric cyst: Successful management in low-resource setting
Antunes M et al
Int J Surg Case Reports 2020;70:185-187. - "Although several classifications have been proposed, the most widely recognized includes 4 groups based on clinical and etiological features: 1) lymphatic, 2) mesothelial, 3) enteric, 4) urogenital, 5) mature cystic teratoma and 6) non pancreatic pseudocysts. In general, simple lymphatic and mesothelial cysts are usually asymptomatic, while lymphangiomas and benign cystic mesotheliomas can be aggressive and invasive. Finally, the only malignant is the malignant cystic mesothelioma.”
Giant mesenteric cyst: Successful management in low-resource setting
Antunes M et al
Int J Surg Case Reports 2020;70:185-187. - "Mesenteric cysts are frequently symptomatic and localized in the mesentery but can appear also in adults and middle aged women mainly in retroperitoneal space. The clinical presentation is influenced by the size of the cyst and the age of patients. During childhood, it can simulate an appendicitis, while in adults is often asymptomatic . The symptoms are generally a-specific and include pain, nausea and vomiting, constipation or diarrhea. Moreover, an abdominal palpable mass is present in up to 61% of the patients. Usually, the preoperative diagnosis is done by ultrasound in order to diagnosis.”
Giant mesenteric cyst: Successful management in low-resource setting
Antunes M et al
Int J Surg Case Reports 2020;70:185-187. - “Mesenteric cysts are rare, benign intra-abdominal cystic lesions of the gastrointestinal mesentery, with an incidence of 1/100000 in adults and 1/20000 in children, and a female to male ratio of 2:1, which commonly present in the second decade of life. Lesions are most commonly located in the small bowel (66%) and large bowel (33%), with the ascending colon being most frequently affected, and rarely found in the descending colon, sigmoid colon and rectum. They only occasionally extend to the retroperitoneum. The size of a cyst can vary from a few millimetres to 30cm in diameter, containing up to 2500 mL of fluid.”
Conservative approach to the acute management of a large mesenteric cyst
Billy C Leung et al.
World J Clin Cases. 2017 Sep 16; 5(9): 360–363. - "Mesenteric cysts are often asymptomatic and discovered as an incidental finding from radiological investigations, but may present with acute or chronic abdominal pain (55%-81%), palpable mass (44%-61%), distension (17%-61%), nausea and vomiting (45%), constipation (27%) and diarrhoea (6%). In severe cases, it can lead to bowel obstruction, obstructive uropathy, volvulus, and peritonitis usually from a haemorrhagic or infective cyst. Surgical excision of the mesenteric cyst, with or without resection of any neighouring visceral organs is the common management approach in the acute setting, with marsupialisation reserved for cases that may require wide resection of adjacent organs, but the recurrence rate is high. Drainage is no longer advised due to infection and recurrence risks.”
Conservative approach to the acute management of a large mesenteric cyst
Billy C Leung et al.
World J Clin Cases. 2017 Sep 16; 5(9): 360–363.
- “Small bowel lipomas commonly occur in the ileum, especially in the terminal ileum. They usually appear as well-defined, intraluminal masses with lipid attenuation (− 100 to − 50 HU) without apparent enhancement. While most small lipomas (less than 1 cm) are asymptomatic, patients with larger tumors may present with various degrees of abdominal pain, gastrointestinal bleeding, or palpable abdominal masses. Some of the patients have abdominal pain resulting from small bowel or ileocolic intussusception due to lipoma as a leading cause.”
Tumors of the jejunum and ileum: a pattern‐based imaging approach on CT
Kim SW et al.
Abdominal Radiology (2019) 44:2337–2345 
Tumors of the jejunum and ileum: a pattern‐based imaging approach on CT
Kim SW et al.
Abdominal Radiology (2019) 44:2337–2345
- “Small bowel lipomas commonly occur in the ileum, especially in the terminal ileum [10]. They usually appear as well-defined, intraluminal masses with lipid attenuation (− 100 to − 50 HU) without apparent enhancement. While most small lipomas (less than 1 cm) are asymptomatic, patients with larger tumors may present with various degrees of abdominal pain, gastrointestinal bleeding, or palpable abdominal masses. Some of the patients have abdominal pain resulting from small bowel or ileocolic intussusception due to lipoma as a leading cause.”
Tumors of the jejunum and ileum: a pattern‐based imaging approach on CT
Kim SW et al.
Abdominal Radiology (2019) 44:2337–2345
- “A lymphangioma is a benign proliferation of lymph vessels, producing fluid filled cysts that result from a blockage of the lymphatic system. ae incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge Oguzhan Güven Gümüştaş et al. Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “The incidence of abdominal lymphangiomas is unknown: however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Although retroperitoneal lymphangiomas may sometimes be asymptomatic, they usually present as a palpable abdominal mass and are easily confused with other retroperitoneal cystic tumors including those arising from the liver, kidney and pancreas.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge Oguzhan Güven Gümüştaş et al. Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “Retroperitoneal lymphangiomas manifest with clinical symptoms of abdominal pain, fever, fatigue, weight loss, and hematuria, due to their size and occasionally might be complicated by intracystic hemorrhage, cyst rupture, volvulus or infection. Differentiating cystic lymphangiomas from other cystic growths by imaging studies alone is often inconclusive and surgery is most frequently required for definitive diagnosis and to ameliorate the symptoms.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge Oguzhan Güven Gümüştaş et al. Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “The lymphatic system is derived during the third or fourth fetal month from 2 paired and unpaired endothelial channels proliferate centrifugally from these sacs, which are located in the neck, mesenteric root, and bifurcation of the femoral and sciatic veins . A lymphangioma is a benign proliferation of lymphatic tissue believed to originate from the early sequestration of lymphatic vessels that fail to establish connections with normal draining lymphatics. Lymphangiomas are therefore considered a congenital rather than an acquired tumor.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge Oguzhan Güven Gümüştaş et al. Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “Intravenous contrast-enhanced CT may show enhancement of the cyst wall and septa. The fluid component is typically homogeneous with low attenuation values. Occasionally, negative attenuation values occur in the presence of chyle. Calcification may occur but is uncommon .If hemorrhage occurs, the intracystic attenuation values may simulate a solid tumor mass or abscess. ae mass may traverse adjacent retroperitoneal anatomical compartments, displacing organs and vessels . They can compress and infiltrate vital structures or present with complications like intracystic hemorrhage, cyst
rupture, volvulus, or infection.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge Oguzhan Güven Gümüştaş et al. Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053 - “Diagnostic important point for cystic lymphangioma: an elongated shape and a crossing from one retroperitoneal compartment to an adjacent one. Also at CT, cystic lymphangioma typically appears as a large, thin-walled, multiseptate cystic mass. A teratoma with a large cystic component can resemble a lymphangioma.”
Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge Oguzhan Güven Gümüştaş et al. Case Reports in Pediatrics Volume 2013 (2013), Article ID 292053
- “Ectopic pancreas, also known as heterotopic pancreatic tissue, is defined as aberrant pancreatic tissue with no ductal or vascular continuity to the main pancreatic gland. Most patients are asymptomatic and this disorder is typically found incidentally. Ectopic pancreas is normally located in the submucosal layer of the stomach, duodenum, and/or jejunum and may easily be misconstrued as a submucosal tumor, such as a gastrointestinal stromal tumor (GIST).”
Imaging Spectrum of Non-neoplastic Duodenal Diseases Sitthipong S et al. Clinical Imaging (in press)
- “Ganglioneuroma is a rare tumor which should nevertheless be included in differential diagnosis of retroperitoneal masses when presenting as a well-delimited tumor with possible tendency to surround or displace major blood vessels, low density on non-enhanced CT, and delayed progressive enhancement on CT and MRI.”
Imaging of retroperitoneal ganglioneuroma Otal P et al. May 2001, Volume 11, Issue 6, pp 940-945 - “Various types of neurogenic tumors can affect the abdomen. These tumors can be classified as being of ganglion cell origin (ganglioneuromas, ganglioneuroblastomas, neuroblastomas), paraganglionic system origin (pheochromocytomas, paragangliomas), or nerve sheath origin (neurilemmomas, neurofibromas, neurofibromatosis, malignant nerve sheath tumors).”
Neurogenic Tumors in the Abdomen: Tumor Types and Imaging Characteristics Sung Eun Rha et al. RadioGraphics2003;23:29–43 - “Ganglioneuromas are rare, benign neurogenic tumors that arise from sympathetic ganglia. The tumors are composed of mature Schwann cells, ganglion cells, and nerve fibers. Ganglioneuromas may arise anywhere along the paravertebral sympathetic plexus and occasionally from the adrenal medulla. The retroperitoneum (32%–52% of cases) and posterior mediastinum (39%– 43%) are the two most common locations for a ganglioneuroma, followed by the cervical region (8%– 9%).”
Neurogenic Tumors in the Abdomen: Tumor Types and Imaging Characteristics Sung Eun Rha et al. RadioGraphics2003;23:29–43 - “Ganglioneuromas are often asymptomatic even if they are large. Otherwise, abdominal pain or the palpation of an abdominal mass is the most common clinical feature. Hormonally active forms have been reported, and the secretion of catecholamines, vasoactive intestinal polypeptides, or androgenic hormones explains such symptoms as hypertension, diarrhea, and virilization.”
Neurogenic Tumors in the Abdomen: Tumor Types and Imaging Characteristics Sung Eun Rha et al. RadioGraphics2003;23:29–43 - “At unenhanced CT, the tumors appear homogeneous, with an attenuation less than that of muscle. At CT, they contain calcification in about 20% of cases. The calcifications are discrete and punctate rather than amorphous and coarse as in neuroblastomas . Varying degrees of contrast material enhancement in
ganglioneuroma have been reported, from slight to moderate to marked.”
Neurogenic Tumors in the Abdomen: Tumor Types and Imaging Characteristics Sung Eun Rha et al. RadioGraphics2003;23:29–43 - “Ichikawa et al reported an interesting contrast enhancement pattern in ganglioneuroma that consisted of delayed heterogeneous uptake . These enhancement features are explained by the presence of an abundance of myxoid matrices in the tumors, resulting in delayed progressive accumulation of contrast material in the extracellular space.”
Neurogenic Tumors in the Abdomen: Tumor Types and Imaging Characteristics Sung Eun Rha et al. RadioGraphics2003;23:29–43
- “Tumors of small intestine account for only 1%-2% of all gastrointestinal tumors, and benign tumors account for approximately 30% of all small intestinal tumors. Gastrointestinal stromal tumors are the most common symptomatic benign tumors of the small bowel, while the lipomas are rare benign tumors, representing 2.6% of non-malignant tumors of the intestinal tract.”
Small intestinal lipomas: Diagnostic value of multi-slice CT enterography Song-Hua Fang et al. World J Gastroenterol. 2010 Jun 7; 16(21): 2677–2681. - “Small bowel lipomas tend to occur in the elderly, and the sixth to seventh decades of life are considered to be the most risky period. Jejunum and ileum, especially the terminal ileum, are common locations. Eleven of the 15 lesions arose from jejunoileum and the mean age of the patients was 63.2 years in this study, which was consistent with those previously reported. Patients may be completely asymptomatic with small lipomas (< 1 cm), which are usually detected incidentally. Larger tumors may produce symptoms .”
Small intestinal lipomas: Diagnostic value of multi-slice CT enterography Song-Hua Fang et al. World J Gastroenterol. 2010 Jun 7; 16(21): 2677–2681. - “Approximately 25% of patients with benign intestinal neoplasm have alimentary tract hemorrhage. Small bowel lipomas usually cause lower gastrointestinal hemorrhage, and lipomas arising from duodenum may cause upper gastrointestinal hemorrhage and symptoms like peptic ulcer. Some of the bleeding is intermittent, some is continuous in small amount or only presents as microscopically positive fecal occult blood.”
Small intestinal lipomas: Diagnostic value of multi-slice CT enterography Song-Hua Fang et al. World J Gastroenterol. 2010 Jun 7; 16(21): 2677–2681. - “Abdominal CT scans are considered to be the most definitive diagnostic measure of recognizing small bowel lipomas. With the fast volume scan technique of multiple spiral CT, reformation in any dimensions is feasible, and the quality of the reformatted images is almost as same as the axial ones, which ensures a good overall observation. The finding of lipid attenuation (-100 to -50 HU) is vital to the diagnosis of a lipoma.”
Small intestinal lipomas: Diagnostic value of multi-slice CT enterography Song-Hua Fang et al. World J Gastroenterol. 2010 Jun 7; 16(21): 2677–2681.
- “ Mesenteric fibromatosis, sclerosing mesenteritis, inflammatory pseudotumor, and extrapleural solitary fibrous tumor constitute a loosely associated group of benign fibrous tumors and tumorlike lesion of the mesentery.”
Benign Fibrous Tumors and Tumorlike Lesions of the Mesentery: Radiologic-Pathologic Correlation
Levy AF et al.
RadioGraphics 2006; 26:245-264 - Benign Fibrous Tumors and Tumorlike Lesions of the Mesentery: Facts
- Mesenteritis fibromatosis
- Sclerosing mesenteritis
- Inflammatory pseudotumor
- Extrapleural solitary fibrous tumor - Mesenteric Fibromatosis: Facts
- AKA intraabdominal fibromatosis or abdominal desmoid
- Tumors infiltrate but do not metastasize
- Small bowel mesentery is most common location
- Age range of 14-75 years (mean 41 years)
- 13% of cases associated with Gardner’s syndrome
- When associated with Gardners syndrome often after abdominal surgery - Mesenteric Fibromatosis: Presentation and Complications
- Abdominal pain or palpable mass
- GI bleeding
- Small bowel obstruction
- Fistula formation
- Bowel perforation - Mesenteric Fibromatosis: CT Findings
- Mesenteric mass which may be discrete or infiltrating
- Displacement of bowel
- Bowel obstruction may occur
- Variable enhancement pattern - Mesenteric Fibromatosis: Differential Diagnosis
- GIST tumor
- Lymphoma
- Metastatic disease
- Soft tissue sarcoma
- Duplication Cyst - Sclerosing Mesenteritis: Facts
- Idiopathic disorder with chronic, nospecific inflammation, fat necrosis and fibrosis
- Usually involves the small bowel mesentery but may involve the colonic mesocolon
- Synonyms include;
1. Mesenteric panniculitis
2. Retractile mesenteritis
3. Mesenteric lipodystrophy
4. Lipogranuloma of the mesentery - Sclerosing Mesenteritis: Facts
- Average age of presentation is 60 years
- More common in men by 2-1
- Abdominal pain is the most common presentation but weight loss, distension, and FUO are other presentations - Sclerosing Mesenteritis: CT Findings
- Retraction and shortening of small bowel mesentery
- Partial or complete SBO
- Well defined or ill-defined mesenteric mass with soft tissue or mixed fat and soft tissue attenuation
- May encase mesenteric vessels with preservation of fatty collar around the vessels (“fat ring sign”)
- Punctate or coarse calcifications may be seen - Sclerosing Mesenteritis: Differebtial Diagnosis
- Carcinoid tumor
- Metastatic disease to mesentery
- Lymphoma
- Soft tissue sarcomas
- Weber Christian disease - Inflammatory Pseudotumor: facts
- Benign, chronic inflammtory lesion of unclear etiology
- May be difficult to distinguish from a true neoplasm
- May occur in mesentery but also lung and orbit
- More common in the pediatric or young adult population
- Presentation may include fever, malaise, weight loss or abdominal pain - Inflammatory Pseudotumor: CT Findings
- Heterogeneous mass in mesentery
- May or may not have well defined margins
- Variable enhancement patterns with IV contrast
- Lipoma of the Small Bowel
- Benign submucosal tumor
- Duodenum is most common small bowel location
- Usually asymptomatic but can rarely present with hemorrhage or obstruction if large
- Usually smooth in appearance on CT
- No malignant potential - Lymphangioma
- Multiloculated large thin walled cystic mass
- Usually in mesentery when it involves the GI tract
- Can simulate a mesenteric cyst, duplicated cyst, cystic pancreatic mass or in theory an abscess