SN Comprehensive Clinical Medicine

, Volume 1, Issue 2, pp 93–95 | Cite as

Adrenal Lymphangioma Clinically Mimicking a Cystic Pheochromocytoma: a Case Report and Literature Review

  • Silvia TaccognaEmail author
  • Enrico Papini
  • Paola Alexandra Mascioli
  • Tatiana Di Cesare
  • Andrea Liverani
Part of the following topical collections:
  1. Topical Collection on Surgery


Adrenal cystic lymphangiomas are rare benign lesions [1], which are usually asymptomatic, and may be incidentally discovered at imaging work-up, at abdominal surgery or at autopsy [2, 3]. They are quite infrequent in adult subjects, 0.06–0.18% of the general population [3], and appear as thin-walled, fluid density lesions. At CT scan, they usually appear as hypodense, nonenhancing lesions with imaging features that may not fully discriminate lymphangiomas from malignant or hyperfunctioning lesions such as adrenal carcinoma or pheochromocytomas [4]. So, their surgical removal may be necessary to rule out their types of adrenal neoplasms [5, 6, 7].

We report the case of a patient with a right adrenal cystic lymphangioma clinically mimicking a pheochromocytoma and the review of the radiological and pathological features of the tumor.

Case Presentation

A 34-year-old male was admitted to the Endocrinology Department of our hospital because of a severe clinical condition characterized by recurrent episodes of headache, palpitation, and hypertension. Physical findings, including heart and lung examination, were unremarkable. Laboratory tests were normal. During the work-up for his hypertension, an abdominal CT scan was performed, which showed a previously unrecognized mass 6.6 × 3.3 × 4.8 cm in the right adrenal gland. Cystic degeneration was present with nonvascularized walls (Fig. 1).
Fig. 1

The cross-sectional CT image

On the basis of the clinical suspicion of pheochromocytoma, an iodine-123 (123I)-labeled metaiodobenzylguanidine (MIBG) scintigraphy was performed as a complementary examination. The test was substantially negative. The determination of urinary metanephrine provided border-line results (360 mg/24 h with high-performance liquid chromatography [HPLC] method). Serum ACTH, cortisol, and aldosterone were within normal limits. Due to the ambiguous clinical picture and the persistent symptoms, the patient underwent a laparoscopic right adrenalectomy. The postoperative course was uneventful. Two months after surgery, mean blood pressure levels returned to normal. The surgical specimen showed an undamaged adrenal gland, 7.5 × 5.2 × 1.0 cm, occupied by a well-circumscribed multicystic mass measuring 6 × 4 × 1 cm. The numerous cavities of the lesion contained watery fluid (Fig. 2).
Fig. 2

The gross photograph of the resected specimen with cystic spaces, note the residual adrenal tissue (arrow)

These small cystic spaces were characterize by thin walls and were lined by flat, bland endothelial cells. The fibrous septs between the cystic spaces showed foci of normal adrenal tissue (Fig. 3a). On immunohistochemical stain, the endothelial cells showed a strong immunoreactivity for CD31, factor VIII, CD34 and D2-40 (Fig. 3b), and absent staining for cytokeratin and calretinin.
Fig. 3

a Multiple dilated cystic spaces filled with pale pink proteinaceous amorphous, homogeneous fluid. The wall is made of fibrous tissue with adrenal cortex cellular groups (H&E × 40). b D2-40 antibody immunostain (× 40) showing positive staining of cystic lining endothelial cells (high-magnification inset)

The conclusive histopathological diagnosis was adrenal cystic lymphangioma.


This article reports a rare case of symptomatic cystic lymphangioma originating from the left adrenal gland in a mal patient successfully treated by complete surgical resection. The majority of intra-abdominal cystic lymphangiomas is located in the mesentery, in contrast to adrenal location that is very rare [2]. Adrenal cysts are uncommon, with a reported incidence of 0.06–0.18% in autopsy series [6, 7]; cystic masses account for only about 4–22% of all adrenal incidentalomas. 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 [4]. The differential diagnosis of adrenal cysts includes primary or metastatic adrenal tumors, benign or malignant cystic neoplasms, hemorrhage secondary to trauma, bleeding disorder, burns, shock or toxemia, and infections [2, 8]. Cystic degeneration of pheochromocytomas is not common [6, 9]. Patients with cystic pheochromocytoma are often asymptomatic and yield negative plasma and urine biochemical analysis. In a majority of cases were female, half had no symptoms, and half had normal biochemical analysis [10]. Microscopic aspect undivided four categories: parasitic, epithelial, pseudocysts, and endothelial in origin [8, 11]. Parasitic cysts are the least common, epithelial cysts consist of true glandular cysts and embryonal cysts, including cystic adenomas. Adrenal pseudocysts are cystic lesions which arise within the adrenal gland. They are surrounded by a fibrous wall devoid of an epithelial lining [11]. The first case of adrenal cystic lymphangioma had been reported in 1965; recently, approximately 69 immunohistochemically cases have been reported in the literature [10, 11, 12, 13]. Lymphangiomas are benign cystic vascular lesions, most frequently discovered in childhood. They are most commonly located in the neck, axillary region, and mediastinum (95%).The remaining 5% are found in the abdominal cavity [1]. Adrenal cystic lymphangiomas are asymptomatic and rarely do they present labile hypertension [14]. It is issued that mass effect of the tumor was the cause of hypertension; therefore, the blood pressure returned to normal after removal of the adrenal mass. Pre-operative diagnosis of retroperitoneal lymphangioma, in general, is challenging and rare. Laboratory findings are nonspecific and are usually not helpful as a diagnostic tool. On computerized tomography, cystic lymphangiomas are identified as hypodense, nonenhancing lesions in the adrenal gland [15]. Current treatment recommendations for adrenal cyst lymphangioma are that small asymptomatic nonfunctioning lesions can simply be observed, whereas large cysts (> 5 cm in diameter), cyst with high density on unenhanced CT (> 10 HU) or delayed washout of the contrast medium, significant growth of the cyst on serial imaging, functional cysts, symptomatic cysts, and cysts which can cause complications such as hemorrhage, rupture, or infection should be excised [11]. Surgical resection represents the definitive treatment if the cystic lymphangioma is symptomatic [4]. Histologically, adrenal lymphangiomas are characterized by multiloculated cystic cavities. The cysts are lined by a single layer of flattened endothelial cells. Immunohistochemically, cells with an endothelial lining mostly stain positively for CD31, factor VIII, CD34 (endothelial specific markers), and D2-40 (lymphatic marker). The management strategy is influenced by three factors: (a) functional status of the cyst, (b) the possibility of incidental malignancy, and (c) potential complications such as hemorrhage or infection [6, 16].


Cystic lymphangioma of the adrenal gland is a rare pathology that should be included in the differential diagnosis of cystic lesions of the adrenal glands. If the patient is symptomatic, definitive treatment is surgery. So, definite diagnosis relies on histologic and immunohistochemical examination.


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.


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© Springer Nature Switzerland AG 2018

Authors and Affiliations

  1. 1.Department of PathologyRegina Apostolorum HospitalAlbano Laziale (RM)Italy
  2. 2.Department of Endocrinology and MetabolismRegina Apostolorum HospitalAlbano Laziale (RM)Italy
  3. 3.Department of General SurgeryRegina Apostolorum HospitalAlbano Laziale (RM)Italy

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