A HUGE ADRENAL INCIDENTALOMA – CYSTIC LYMPHANGIOMA，A CASE
PRESENTATION
1Y-H
Lin, 1J-L Lin, 2D-Y Chen, 1Division of Endocrinology and Metabolism, Department of
Internal Medicine, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C. 2Department of
Pathology, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.
Abstract
A 37 year-old female admitted to our hospital due to fever for evaluating origin of
infection. A huge right cystic adrenal incidentaloma ( 9 x 10.5 cm) was found by ultrasound
and computed tomography of abdomen containing subtle internal septal structures with
spots of calcifications. Under functional screen test with favoring a nonfunctional tumor,
surgical resection and histologic finding were compatible with cystic lymphangioma of the
adrenal gland. We present this case for a clinic practice for adrenal incidentalomas and also a
rare huge and benign lesion happen in adrenal glands.
Key words: adrenal incidentaloma, cystic lymphangioma
Case report
A 37 year-old female, housewife, underlying only with Meniere’s disease, presented to our
hospital due to fever for 3 days and admitted for evaluation origin of infection. She denied
symptoms and sign of respiratory tract infection, urinary tract infection, nor diarrhea and
headache, neck stiffness, but mild right upper abdominal dull pain was told. Abdominal echo
and computerized tomography scan of abdomen was performed to rule out intra-abdominal
lesions, and a huge cystic adrenal mass ( 9 x 10.5 cm) in right side retropertoneum, with local
invasion to adjacent posterior aspect of liver (Figure 1). Empiric antibiotics was prescribed
and fever was subsided. Meanwhile, endocrinologists were consulted to evaluate this cystic
adrenal incidentaloma. The screen tests for functional tumors for this non-hypertensional
patient with normal serum sodium and potassium level were reported with negative finding
(Figure 2). She was discharge successfully after finishing seven-day course of antibiotics with
no evidence of blood cultures. Furthermore, she re-admitted to receive the scheduled
surgical excision for right cystic mass in the next hospitalized time, showing multiple dilated
cystic spaces were filled with clear, straw-colored fluid (Figure 3). The histological section
revealed multicystic architecture with dilated spaces lined by flattened simple lining cells,
lacking cytologic atypia (Figure 4), but had positive D2-40 immunohistochemical cytoplasmic
staining and no reactivity in synaptophysin staining (Figure 5), which the front stands for
lymphatic nature and the latter excludes neuroendocrine component. These are diagnostic
for the cystic lymphangioma. The patient recovered well after the operation and is under
regular follow-up in the outpatient service now.
Disccusion
Adrenal lymphangioma is a rare and benign lesion, most often found incidentally by
abdominal imaging studies or abdominal surgery or at autopsy. Its incidence has been
reported in the literature as approximately 0.06% 1. It can occur at all ages, with the peak
incidence between the third and sixth decades and female, right-sided predominance in
current series2, as similar in our patient but was incidentally found for initial infection survey.
Functional screening study for any adrenal incidentaloma should be approached before
surgical intervention. Usually surgery is indicated for large and complicated cysts, parasitic
cysts and functioning and malignant cysts3. Our patient is a simple case of non-functional
tumor, so that further surgical resection is soon indicated and then was discovered as benign
lymphangioma. Surgery alone(complete resection) is the treatment of choice with no
adjunctive therapy needed. There is no case of recurrence or metastasis has been described
in recent review of literature 4.
Reference
1. Milsten MS, Minielly JA, VanSchoyck P, Forrest JB. Abdominal pain secondary to a
lymphangiomatous cyst of the adrenal: case report and review of literature. J Okla State
Med Assoc. 1994;87:225–227.
2. Carla L. Ellis MD, MS. Adrenal lymphangioma: clinicopathologic and
immunohistochemical characteristics of a rare lesion. Human Pathology.
2011;42:1013-1018.
3. J.M.Longo, S.Z. Jafri. Adrenal lymphangioma: a case repot. Journal of Clinical Imaging.
2000;24:104-106.
4. Michele Bisceglia, MD. Cystic lymphangioma-like adenomatoid tumor of the adrenal
gland: Case presentation and Review of the literature. Adv Anat Pathol.2009;16:424-432.
Figure 1 (Abdominal computerized tomography scan)
Figure 2 (Function screening)
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Figure 3 (Gross photograph)
Figure 4 (Hematoxylin and eosin x 100)
Figure 5 (right, D2-40; left, synaptophysin staining)