Adrenal lymphangiomas, also known as cystic adrenal lymphangiomas, are rare, benign cystic adrenal lesions.
Adrenal lymphangiomas are extremely rare; prevalence is estimated at 0.06% 8. They can occur at any age, with a peak incidence between the 3rd and 6th decades of life. According to one series, there may be a slight right-sided and female predilection 3.
They usually remain asymptomatic throughout life and are almost always incidentally detected. Rarely, patients may have abdominal pain 3.
They supposedly occur as a result of a developmental abnormality of lymphatic channels 4.
Their appearance is that of a lymphangioma occurring anywhere else in the body, i.e. a multilocular cyst.
- D2-40 cytoplasmic staining tends to be positive (for lining of lymphatic vessels) 6,8
- AE1/AE3 staining tends to be negative 6
- typically seen as cystic lobulated lesions of fluid attenuation with thin enhancing walls 7-8
- tend to present as relatively large lesions with an average size around 3 cm 6
- internal septations may be present, and when present, enhance after contrast administration
- may have calcifications, which tend to present in one of two patterns: thick curvilinear or scattered punctate, and can involve the septation(s) 7
Treatment and prognosis
They are benign lesions, although if they become symptomatic, surgical resection is warranted. Unnecessary surgery, on the other hand, can be the ultimate consequence of unfamiliarity with these rare lesions.
The differential diagnosis for adrenal lymphangiomas is that of adrenal cystic tumors, most of which are rare in themselves, and includes:
- 1. Ates LE, Kapran Y, Erbil Y et-al. Cystic lymphangioma of the right adrenal gland. Pathol Oncol. Res. 2006;11 (4): 242-4. Pubmed citation
- 2. Longo JM, Jafri SZ, Bis KB. Adrenal lymphangioma: a case report. Clin Imaging. 2001;24 (2): 104-6. Pubmed citation
- 3. Ellis CL, Banerjee P, Carney E et-al. Adrenal lymphangioma: clinicopathologic and immunohistochemical characteristics of a rare lesion. Hum. Pathol. 2011;42 (7): 1013-8. doi:10.1016/j.humpath.2010.10.023 - Pubmed citation
- 4. Secil M, Demir O, Yorukoglu K. MRI of adrenal lymphangioma: a case report. Quant Imaging Med Surg. 2014;3 (6): 347-8. doi:10.3978/j.issn.2223-4292.2013.12.07 - Free text at pubmed - Pubmed citation
- 5. Bettaïeb I, Mekni A, Bédioui H et-al. Huge cystic lymphangioma of the adrenal gland. A case report and review of the literature. Pathologica. 2007;99 (1): 19-21. Pubmed citation
- 6. Zhao M, Gu Q, Li C et-al. Cystic lymphangioma of adrenal gland: a clinicopathological study of 3 cases and review of literature. Int J Clin Exp Pathol.7 (8): 5051-6. Free text at pubmed - Pubmed citation
- 7. Rowe SP, Bishop JA, Prescott JD, Salvatori R, Fishman EK. CT Appearance of Adrenal Cystic Lymphangioma: Radiologic-Pathologic Correlation. AJR. American journal of roentgenology. 206 (1): 81-5. doi:10.2214/AJR.15.14786 - Pubmed
- 8. Lattin GE, Sturgill ED, Tujo CA, Marko J, Sanchez-Maldonado KW, Craig WD, Lack EE. From the radiologic pathology archives: Adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation. Radiographics : a review publication of the Radiological Society of North America, Inc. 34 (3): 805-29. doi:10.1148/rg.343130127 - Pubmed