Adrenal Lymphangioma Clinically Mimicking a Cystic Pheochromocytoma: a Case Report and Literature Review
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Adrenal cystic lymphangiomas are rare benign lesions , 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 , 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 . 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.
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 . 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 . 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 . 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 . 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 . Adrenal cystic lymphangiomas are asymptomatic and rarely do they present labile hypertension . 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 . 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 . Surgical resection represents the definitive treatment if the cystic lymphangioma is symptomatic . 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.
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Conflict of Interest
The authors declare that they have no conflict of interest.
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