Abdominal cystic lymphangioma

A 22-year-old woman presented with a severe episode of right lower quadrant abdominal pain. Her medical history included alcoholism and depression as well as endometriosis, a condition for which she had been taking oral contraceptive pills and had undergone a previous diagnostic laparoscopy. A computerized tomography scan of the abdomen and pelvis was performed to rule out appendicitis, and a 4-cm cystic mass was noted at the left suprarenal area adjacent to the left renal vein (Figure 1, arrows). Magnetic resonance imaging confirmed the finding of a left adrenal cyst. The patient was scheduled for surgery. Intraoperatively, a 3.8-cm cystic lesion from the left adrenal gland, closely associated with the tail of the pancreas, was identified; the pancreas, liver, ovaries, and appendix were normal. The cyst was inadvertently opened during resection, and a clear, straw-colored fluid was evacuated. It was resected with a portion of the left adrenal gland. Macroscopically, the ruptured cyst measured approximately 3.5 × 3.0 × 1.0 cm. A cut section showed that this was a thin-walled cyst with a smooth lining surrounded by a rim of yellow-orange adrenal tissue. Histologie sections showed irregularly shaped cystic spaces mantled by flattened endothelial cells, surrounded by normal-appearing adrenal tissue (Figure 2). The cells lining the spaces displayed no atypia and were strongly immunoreactive for CD31 (Figure 3). The diagnosis of cystic adrenal lymphangioma was rendered. Postoperatively, the patient recovered without complications and was discharged home on postoperative day 4.

Adrenal lymphangioma is a rare and benign lesion, most often found incidentally during abdominal imaging studies or abdominal surgery or at autopsy. Its incidence has been reported in the literature as approximately 0.06%.1 However, it may be seen more frequently with improvements in imaging techniques, thus placing this entity in the group of increasingly seen "incidentalomas." It can occur at all ages and has a peak incidence from the third to sixth decades of life.2 Lymphangiomas typically occur in children, more frequently in boys, and are usually extrapelvic, being almost always localized to the mesentery of the small intestine, omentum, mesocolon, or retroperitoneum.3 Adrenal cysts are usually asymptomatic; if symptoms do occur, they are usually related to mass effect and include pain, a gastrointestinal disturbance, or a palpable mass. Laboratory findings are nonspecific and are usually not helpful as a diagnostic tool. On ultrasound, the diagnosis of cystic lymphangioma is suggested by the presence of a well-marginated, anechoic lesion, typically located at the suprarenal area, which can show shadows or echoes when calcifications are present. On computerized tomography, cystic lymphangiomas are identified as hypodense, nonenhancing lesions in the adrenal gland. On magnetic resonance imaging, uncomplicated adrenal cysts are low in signal intensity on Tl-weighted images and high on T2-weighted images. Complicated adrenal cysts will be high in signal intensity on both Tl- and T2-weighted images. Magnetic resonance imaging in the coronal and sagittal plane is particularly helpful for determining the location of a large cystic mass in the region of the adrenal gland. Surgical resection is indicated for large and complicated cysts to confirm the diagnosis or relieve symptoms.2

References

1. Milsten MS, Minielly |A, VanSchoyck P, Forrest JB. Abdominal pain secondary to a lymphangiomatous cyst of the adrenal: case report and review of literature. ; OkIa State Med Assoc. 1994)87:225-227.

2. Khoda J, Hertzanu Y, Sebbag C, Lantsberg L, Barky Y. Adrenal cysts: diagnosis and therapeutic approach, lnt Surg. 1993;78:239-242.

3. Enzinger FM, Weiss SW. Tumors of lymph vessels. In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors. 3rd ed. St Louis, Mo: The CV Mosby Co; 1995:679-698.

Monica Garcia, MD; Louis B. Louis IV, MD; Stephen Vernon, MD

Accepted for publication February 16, 2004.

From the Departments of Pathology (Drs Garcia and Vernon) and Surgery (Dr Louis), University of Miami School of Medicine, Miami, FIa.

The authors have no relevant financial interest in the products or companies described in this article.

Reprints: Stephen Vernon, MD, Department of Pathology, Jackson Memorial Hospital, 1611 NW 12th Ave, Holtz Center, Room 2042, Miami, FL 33136 (e-mail: verndoc50@aol.com).

Copyright College of American Pathologists Jun 2004
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