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Lymphangioma (Mesenteric Cyst) bb42a128-c819-4368-a085-232b6db3434c
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c1df94ab-4a9f-44c4-add7-1f174fb9ac45 Siva P. Raman, MD
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Gastrointestinal 554a62de-23db-4b78-9d4c-102d1586b91e 18 03/23/25 Lymphangioma (Mesenteric Cyst) Gastrointestinal, Diagnosis, Peritoneum, Mesentery, and Abdominal Wall, Benign Neoplasms, Lymphangioma (Mesenteric Cyst) Lymphangioma (Mesenteric Cyst) | STATdx Lymphangioma (Mesenteric Cyst) DX true
Gastrointestinal
Diagnosis
Peritoneum, Mesentery, and Abdominal Wall
Benign Neoplasms
Lymphangioma (Mesenteric Cyst)

title: "Lymphangioma (Mesenteric Cyst)" docid: "bb42a128-c819-4368-a085-232b6db3434c" authors:

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  • "Gastrointestinal"
  • "Diagnosis"
  • "Peritoneum, Mesentery, and Abdominal Wall"
  • "Benign Neoplasms"
  • "Lymphangioma (Mesenteric Cyst)"

KEY FACTS

  • Terminology

    • Generic descriptive term for variety of different benign, congenital, cystic masses that can arise in mesentery or retroperitoneum
  • Imaging

    • Lymphangiomas of abdomen are rare (7% of all lymphangiomas) and can arise from or involve virtually any structure
    • Circumscribed, cystic mass that typically demonstrates water density (near 0 HU) or chylous density (< -20 HU) - Can vary in size from very small lesions to massive cysts occupying much of abdomen - No enhancement, mural nodularity, or solid component - Can be multiloculated (± septations) withfeatheryappearance and may demonstrate subtle peripheral or septal calcifications - Soft lesions without mass effect that are easily indented by surrounding structures - Usually hypointense on T1WI MR and hyperintense on T2WI but can demonstrate more complex signal characteristic in setting of prior hemorrhage
  • Clinical Issues

    • Symptoms are uncommon in adults, but can rarely cause issues as result of mass effect, superinfection, or internal hemorrhage
    • In vast majority of cases, these are incidental imaging findings that can be followed with serial imaging to establish stability
    • In those rare cases where lesions are symptomatic, surgery is treatment of choice
  • Diagnostic Checklist

    • Differentiate from other primary cystic lesions or cystic neoplasms arising from adjacent visceral organs (such as exophytic renal cysts or hepatic cysts), as these are much more common than abdominal lymphangiomas

TERMINOLOGY

  • Synonyms

    • Lymphoepithelial cyst, cystic lymphangioma, mesenteric cyst, lymphatic malformation
  • Definitions

    • Generic descriptive term used for benign, congenital, cystic mass arising in mesentery or retroperitoneum that encompasses variety of different histologic entities - Many different cyst types are included under this term, including true cystic lymphangioma, chylolymphatic mesenteric cyst, enteric duplication cyst, peritoneal simple mesothelial cyst, etc. - Exact histologic cyst type can usually not be ascertained on imaging, and accordingly, generic term mesenteric cyst is usually utilized

IMAGING

  • General Features

    • Best diagnostic clue

      - Cystic mass (without mass effect on adjacent structures) in mesentery or retroperitoneum that does not clearly arise from any adjacent visceral organ
      
    • Location

      - Majority of lymphangiomas arise in head, neck, or axillae
      - Lymphangiomas of abdomen are rare (7% of all lymphangiomas)
              - Can involve multiple compartments of peritoneum or retroperitoneum
              - Can arise from or involve virtually any structure
              - Abdominal lymphangiomas most often arise in retroperitoneum in adults (> 50%)
      - Lymphangiomatosis: Widespread lymphangiomas (usually liver, spleen, mediastinum, lungs, mesentery)
              - Usually presents in infants and young children
      
    • Size

      - Few mm to 40 cm in diameter
      
  • CT Findings

    • Circumscribed, cystic mass with variable density - Typically water density (near 0 HU) or chylous (< -20 HU) with lesions rarely demonstrating hemorrhagic contents - No internal enhancement, solid component, or mural nodularity
    • Can be multiloculated (± septations) with feathery appearance - ± fine calcifications along cyst wall
    • Soft lesions without mass effect that are indented by surrounding structures (e.g., mesenteric vessels or bowel)
  • Ultrasonographic Findings

    • Fluid-filled cystic structure with thin internal septa - ± internal echoes due to debris, hemorrhage, or infection - May demonstrate multiloculated appearance with multiple internal septations and cystic spaces
  • MR Findings

    • Simple or multiloculated cyst, which is usually hypointense on T1WI and hyperintense on T2WI - Can be T1 hyperintense due to internal fat/chyle - Septations and internal complexity may be more apparent on T2 MR compared to CECT - Internal signal characteristics can appear more complex on T1WI and T2WI in setting of prior hemorrhage

DIFFERENTIAL DIAGNOSIS

PATHOLOGY

  • General Features

    • Etiology

      - True lymphangiomas result from failure of normal embryologic development with lymphatic tissue not communicating with rest of lymphatic system
      
  • Gross Pathologic & Surgical Features

    • Thin walled and multiseptated with serous, serosanguineous, hemorrhagic, or chylous fluid contents
  • Microscopic Features

    • Cuboidal or columnar cells lining cyst ± smooth muscle, lymphatics, and blood vessels within walls

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Usually asymptomatic (particularly in adults) and incidental findings on imaging
      - Rare symptoms (abdominal distention, pain) due to size (particularly in neonates) or superinfection
      - Complications due to mass effect (such as bowel obstruction, volvulus, urinary tract obstruction, etc.) are unusual due to "soft" nature of these lesions
      
  • Demographics

    • Epidemiology

      - Can occur at any age but 75% discovered < 5 years of age
              - 90% continue to grow until 2 years of age
      - M > F
      - Rare (1/40,000 persons)
      
  • Treatment

    • Asymptomatic lesions do not require any intervention or treatment and can be followed with repeat imaging
    • Utilization of aspiration and sclerosing agents for symptomatic lesions is controversial as treatments almost always ineffective and relapse rates approach 100%
    • Open or laparoscopic surgery preferred approach if lesion is symptomatic or there is concern for malignancy - Given that these are benign lesions, attempt is usually made at surgery to spare adjacent organs and structures (such as bowel) - Good prognosis after surgery with low rates of recurrence (0-13.6%) - Recurrence more common with incomplete resections

DIAGNOSTIC CHECKLIST

  • Consider

    • Differentiate from other primary cystic lesions or tumors of visceral organs (such as exophytic renal or hepatic cysts)

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References

Selected References

  1. Al-Khafaji RA et al: Mesenteric cystic lymphangioma, an acute presentation in a 9-year-old child. Radiol Case Rep. 19(6):2371-5, 2024
  2. Hoang VT et al: Review of diagnosis, differential diagnosis, and management of retroperitoneal lymphangioma. Jpn J Radiol. 41(3):283-301, 2023
  3. Maghrebi H et al: Intra-abdominal cystic lymphangioma in adults: a case series of 32 patients and literature review. Ann Med Surg (Lond). 81:104460, 2022
  4. Yacoub JH et al: Approach to cystic lesions in the abdomen and pelvis, with radiologic-pathologic correlation. Radiographics. 41(5):1368-86, 2021
  5. Raufaste Tistet M et al: Imaging features, complications and differential diagnoses of abdominal cystic lymphangiomas. Abdom Radiol (NY).45(11):3589-607, 2020
  6. Gümüştaş OG et al: Retroperitoneal cystic lymphangioma: a diagnostic and surgical challenge. Case Rep Pediatr. 2013:292053, 2013
  7. Kwag E et al: CT features of generalized lymphangiomatosis in adult patients. Clin Imaging. 37(4):723-7, 2013
  8. Rajiah P et al: Imaging of uncommon retroperitoneal masses. Radiographics. 31(4):949-76, 2011
  9. Hachisuga M et al: Prenatal diagnosis of a retroperitoneal lymphangioma: a case and review. Fetal Diagn Ther. 24(3):177-81, 2008
  10. Rani DV et al: Unusual presentation of a retroperitoneal lymphangioma. Indian J Pediatr. 73(7):617-8, 2006
  11. Chan IYF et al: Retroperitoneal lymphangioma in an adult. J HK Coll Radiol 6:94-96, 2003
  12. de Perrot M et al: Mesenteric cysts. Toward less confusion? Dig Surg. 17(4):323-8, 2000
  13. Stoupis C et al: Bubbles in the belly: imaging of cystic mesenteric or omental masses. Radiographics. 14(4):729-37, 1994
  14. Dyer R et al: Cystic retroperitoneal lymphangioma: CT, ultrasound, and MR findings. Pediatr Radiol 23:305-306, 1993
  15. Ros PR et al: Mesenteric and omental cysts: histologic classification with imaging correlation. Radiology. 164(2):327-32, 1987
  16. Vanek VW et al: Retroperitoneal, mesenteric, and omental cysts. Arch Surg. 119(7):838-42, 1984

Images

Selected Images

Axial CECT demonstrates a cystic mass  in the left retroperitoneum. The lesion abuts the pancreatic tail and left colon without appreciable mass effect. Note the presence of a coarse calcification    along the margin of the lesion. Axial CECT demonstrates a cystic mass in the left retroperitoneum. The lesion abuts the pancreatic tail and left colon without appreciable mass effect. Note the presence of a coarse calcification along the margin of the lesion.

Axial CECT demonstrates a cystic mass  in the left retroperitoneum. The lesion abuts the pancreatic tail and left colon without appreciable mass effect. Note the presence of a coarse calcification    along the margin of the lesion. Axial CECT demonstrates a cystic mass in the left retroperitoneum. The lesion abuts the pancreatic tail and left colon without appreciable mass effect. Note the presence of a coarse calcification along the margin of the lesion.

Coronal CECT in the same patient nicely demonstrates the multiloculated, feathery morphology of the lesion . This appearance is quite common with lymphangiomas, which frequently appear to have multiple internal discrete components or locules. Coronal CECT in the same patient nicely demonstrates the multiloculated, feathery morphology of the lesion . This appearance is quite common with lymphangiomas, which frequently appear to have multiple internal discrete components or locules.

Axial NECT demonstrates a large, cystic lesion  in the right hemiabdomen immediately adjacent to the gallbladder , representing a lymphangioma. Notice the simple appearance of this lesion without any appreciable internal complexity. Axial NECT demonstrates a large, cystic lesion in the right hemiabdomen immediately adjacent to the gallbladder , representing a lymphangioma. Notice the simple appearance of this lesion without any appreciable internal complexity.

Coronal CECT demonstrates a retroperitoneal lymphangioma    abutting the duodenal sweep.  This lesion had been stable over many exams and was deemed to be benign. Coronal CECT demonstrates a retroperitoneal lymphangioma abutting the duodenal sweep. This lesion had been stable over many exams and was deemed to be benign.

Coronal volume-rendered CECT  demonstrates a large, simple-appearing lymphangioma  in the left hemiabdomen abutting adjacent loops of small bowel without any appreciable internal complexity or enhancement. Coronal volume-rendered CECT demonstrates a large, simple-appearing lymphangioma in the left hemiabdomen abutting adjacent loops of small bowel without any appreciable internal complexity or enhancement.

Axial T1 C+ MR in the same patient demonstrates the absence of any significant enhancement, mural nodularity, or solid component within the cystic lesion . The presence of any of these features should argue against the diagnosis of a lymphangioma. Axial T1 C+ MR in the same patient demonstrates the absence of any significant enhancement, mural nodularity, or solid component within the cystic lesion . The presence of any of these features should argue against the diagnosis of a lymphangioma.

Coronal CECT demonstrates a large congenital cyst/lymphangioma    in the right hemiabdomen in a patient being imaged for a primary pancreatic lesion (not shown). These lesions are frequently incidental findings on exams performed for other reasons. Coronal CECT demonstrates a large congenital cyst/lymphangioma in the right hemiabdomen in a patient being imaged for a primary pancreatic lesion (not shown). These lesions are frequently incidental findings on exams performed for other reasons.

Axial CECT shows a thin-walled mass    with water density in this patient with cystic lymphangioma. Notice the manner in which this cystic mass confirms to the shape of nearby structures in the left upper quadrant without any appreciable mass effect. Axial CECT shows a thin-walled mass with water density in this patient with cystic lymphangioma. Notice the manner in which this cystic mass confirms to the shape of nearby structures in the left upper quadrant without any appreciable mass effect.

Coronal CECT demonstrates a cystic lymphangioma  wrapped around the margins of the right colon  but without any associated mass effect. Coronal CECT demonstrates a cystic lymphangioma wrapped around the margins of the right colon but without any associated mass effect.

Coronal NECT demonstrates the classic appearance of an incidental lymphangioma  . Notice the feathery, multiloculated appearance of the lesion with multiple small internal compartments. Coronal NECT demonstrates the classic appearance of an incidental lymphangioma . Notice the feathery, multiloculated appearance of the lesion with multiple small internal compartments.

Coronal volume-rendered CECT demonstrates a multiloculated, cystic lesion  in the left retroperitoneum. The lesion envelops multiple arteries and veins , which do not appear deviated or narrowed. Coronal volume-rendered CECT demonstrates a multiloculated, cystic lesion in the left retroperitoneum. The lesion envelops multiple arteries and veins , which do not appear deviated or narrowed.

Axial CECT shows a complex mesenteric cyst/lymphangioma in the mesentery, which surrounds a small bowel segment . The mass is of near water density, and has small foci of calcification in its septations and peripheral walls . Axial CECT shows a complex mesenteric cyst/lymphangioma in the mesentery, which surrounds a small bowel segment . The mass is of near water density, and has small foci of calcification in its septations and peripheral walls .

Axial CECT demonstrates a large mesenteric cystic mass  with a thin wall, multiple septations , and no soft tissue mass component. The mass is very soft, as shown by blood vessels  extending freely through the mass. Axial CECT demonstrates a large mesenteric cystic mass with a thin wall, multiple septations , and no soft tissue mass component. The mass is very soft, as shown by blood vessels extending freely through the mass.

Axial T2 MR in the same patient nicely demonstrates that the mass  has numerous internal septations    and conforms to the shape of surrounding structures without any significant mass effect, typical of a lymphangioma. Axial T2 MR in the same patient nicely demonstrates that the mass has numerous internal septations and conforms to the shape of surrounding structures without any significant mass effect, typical of a lymphangioma.

Axial CECT demonstrates a cystic mass  that fills much of the pelvis and lower abdomen. Note the presence of calcifications  both within septations and peripheral walls. The mass was resected and proved to be a lymphangioma. Axial CECT demonstrates a cystic mass that fills much of the pelvis and lower abdomen. Note the presence of calcifications both within septations and peripheral walls. The mass was resected and proved to be a lymphangioma.

Axial CECT demonstrates a water density mass  with no discernible wall. This lesion demonstrates multiple small internal compartments and loculations, a typical appearance for a lymphangioma. Axial CECT demonstrates a water density mass with no discernible wall. This lesion demonstrates multiple small internal compartments and loculations, a typical appearance for a lymphangioma.

Additional Images

Axial CECT shows cystic lymphangioma as a thin-walled, water-density mesenteric mass with scattered calcifications in septa. Axial CECT shows cystic lymphangioma as a thin-walled, water-density mesenteric mass with scattered calcifications in septa.

Coronal T2WI MR shows large, multiloculated cystic lymphangioma with water intensity. Coronal T2WI MR shows large, multiloculated cystic lymphangioma with water intensity.