Jodi Widner, Chief Surgical Resident, Department of Surgical Education, Exempla St. Joseph Hospital, Denver, CO; Margaret Schrieber, Attending Surgeon, Colorado Permanente Medical Group, Denver, CO; Nita Ellis, Attending Surgeon, Colorado Permanente Medical Group, Denver, CO, Assistant Clinical Professor, University of Colorado Health Science Center, Denver, CO
Although there are guidelines for laparoscopic resection of a solid adrenal mass, a large cystic adrenal mass presents unique challenges, as described by Widner and colleagues. A very large upper abdominal mass may be renal, adrenal, pancreatic, or originate from some other source. Once the presence of the mass is established by computed tomography (CT) scanning, aspiration with fluid analysis and a post-aspiration CT scan can provide information on the origin of the cyst. Before aspiration, an adrenal origin should be suspected and the cyst evaluated for a functional adrenal tumor, especially in a patient with hypertension. The interval between aspiration and laparoscopic resection should be guided by post-aspiration CT scans and performed before there is significant reaccumulation of cystic fluid. Alpha blockade is required for solid and cystic adrenal masses that raise suspicion of pheochromocytoma.
Thomas Gadacz, MD Series Guest Editor, Professor and Chairman, Department of Surgery, Medical College of Georgia, Augusta, GA
Jodi Widner, MD Chief Surgical Resident
Department of Surgical Education
Exempla St. Joseph Hospital
Margaret Schrieber, MD
Colorado Permanente Medical Group
Nita Ellis, MD
Colorado Permanente Medical Group
Assistant Clinical Professor
University of Colorado Health Science Center
Adrenal cystic masses are rare, pathologically variable, and may be true cysts or pseudocysts.1 They may also be associated with pheo?chromocytoma or cancer.2,3 Adrenal mass?es are identified incidentally or by symptoms produced by the mass effect. Pre?operative evaluation of these cysts can be difficult due to the wide spectrum of presentations.2,4 We report a case of laparoscopic resection of a very large mesothelial cystic adrenal mass.
A 57-year-o?ld woman went to her primary care physician because of a 2-month history of vague abdominal discomfort and early satiety. She reported no recent headaches, palpitations, fa?tigue, or weight loss. Her medical history included hypertension, which was controlled with two medications. A recent colonoscopy revealed diverticulosis, but her abdominal pain did not improve with medical treatment. She presented to our institution 2 months after the colon?os?copy, and a physical examination re?vealed a nontender, left upper quadrant mass on palpation. A subsequent computed tomography (CT) scan showed a 15 x 10 x 11-cm cystic lesion in the left upper quadrant displacing the spleen anteriorly and compressing the stomach (Figure 1). The cyst's origin could not be identified but it appeared to be a large renal cyst. Ultra?sound-guided needle as?piration of the cyst yielded 1,100/mL of straw-colored fluid, but there was incomplete resolution of the cyst. The aspirated fluid did not contain abnormal cells, elevated levels of creatinine, vanillylmandelic acid (VMA), cortisol, or other me?tabolites. Repeat CT scanning 6 weeks later revealed the cyst was adrenal in origin and that approximately 50% of the fluid had reaccumulated.
The patient was taken to the operating room for laparoscopic resection of the cyst and possible left adrenalectomy. On induction she became profoundly hypertensive to 250/130 mm Hg and the procedure was aborted. She was kept overnight for observation and recovered uneventfully. Further workup for possible pheochromocytoma was performed in?cluding serum cortisol and electrolytes, plasma and urine metanephrines, VMA, and catecholamines, all of which were normal. Alpha blockade was started.
The patient returned 10 days later for a laparoscopic left adrenalectomy and cyst excision. The patient tolerated in?duction and there were no significant variations in her hemodynamics through?out the procedure. Three 12-mm ports were placed from midline to midaxillary line along the left subcostal border, and the left adrenal gland was resected in continuity with the large cyst (Figure 2). The cyst wall was separated from the left diaphragm, left lobe of the liver, left kidney, spleen, and anterior pancreas. The cyst appeared to originate from the superior lateral portion of the adrenal gland. No drains were placed. The patient was discharged to home on postoperative day 2 tolerating a regular diet, and she resumed her regular antihypertensive medications. Alpha blockade was withdrawn immediately after surgery, and no untoward hemodynamic events were noted. At 2-month follow-up, there were no recurrent abdominal symptoms, but intermittently labile blood pressures re?quired multiple medication changes. No further testing has been undertaken.
Pathology revealed a large mesothelial cyst and normal appearing adrenal tissue. The entire cyst wall was examined grossly, and representative sections of the adrenal gland were serially sectioned. No evidence of pheochromocytoma was found (Figure 3).
Adrenal cysts are rare with an incidence of 0.06% to 0.18% in autopsy specimens.4 In 1999, Neri and Nance reviewed the 615 cases of adrenal cysts then reported in the literature, which included the first reported case in 1670 by Griselius.3 With the widespread use of abdominal CT scanning, adrenal cysts and other incidental adrenal masses are found more frequently. Approximately 5.7% of adrenal masses identified by CT scanning are cystic.5 Adrenal cysts are more common in women, with a fe?male-to-male ratio of 2:1, and typically occur in the fourth and fifth decades of life.6 They may be associated with other diseases, such as polycystic kidney disease, Beckwith-Wiedemann syndrome, and Klippel-Trenaunay-Weber syndrome.7
Eighty-three percent of adrenal cysts are symptomatic. The most common symptoms are related to mass effect, which generally results in abdominal pain.7 Approximately one third of adrenal cysts are discovered incidentally, and only 57% of adrenal cysts are correctly diagnosed at the initial evaluation.3 Conducting a thorough physical examination and obtaining a detailed patient history, with directed questions regarding possible functional tumors such as aldo-steronomas, pheochromocytomas, and steroid-producing cortical adenomas, are crucial measures for making the diagnosis in these patients.
Diagnosis?Abdominal CT scanning is necessary to determine the spatial relationships of adrenal cysts as well as heterogeneity or layering within the cyst representing hemorrhage. As in our pa?tient's case, decompression of a nonfunctional cyst may provide further assistance in determining the source of the cyst. Urinary and serum chemical screen?ing for pheochromocytoma should be performed before any manipulation of a suspected adrenal cyst. Fluid aspirated from the cyst should be examined for malignant cells and measured for catecholamines, metanephrines, and VMA. Cytology in malignant adrenal cyst fluid is positive in 85% of cases. Benign adrenal cyst fluid shows few, if any, cells.8
Evaluation of cystic adrenal masses for pheochromocytoma includes ab?domin??al CT scanning, 24-hour urinary me?tane?phrines, VMA, catecholamines, 17-hydro?xycorticosteroids, aldosterone, and serum renin, potassium, and cortisol. Cystic pheochromocytomas are typically asymptomatic and may show normal or borderline urinary and serum catecholamines, which creates a diagnostic challenge. Klinger and colleagues suggest obtaining a second 24-hour urine sample in patients who had an initial negative chemical screening.2 They also ad?vocate chemical screening for all patients with adrenal cysts before any manipulation, including aspiration.
?Adrenal cysts are divided into four categories: parasitic, epithelial, endothelial, and pseudocysts.6 Parasitic cysts are the least common (2%) and may be secondary to or infection.2,3 Only 0.5% of those infected with de?velop cystic disease of the adrenals.3 Epithelial cysts consist of true glandular cysts and embryonal cysts, including cystic adenomas. These me?sothelial-lined cysts represent only 6% of all adrenal cysts and are characterized by keratin immunoreactivity within the cyst wall.2,7,9,10
Endothelial cysts are either angiomatous or lymphangiomatous and contain reddish or milky fluid, respectively.7 Endothelial cysts and pseudocysts are each reported in various studies as the most common cystic lesions, but in Neri and Nance's review of 615 patients, only 24% had endothelial cysts while 56% had pseudocysts.7 Pseudocysts are probably related to a traumatic hematoma or infection with subsequent degeneration of the endothelial components. Can?aliculi within the wall of the pseudocyst may also indicate a primary vascular cause.3 Pseudocysts may contain adrenal tissue in the cyst wall or ectopic tissue such as thyroid or intracystic fat.
Cystic neoplasms?Adrenal neoplasms (adenomas and cancer) are more commonly associated with endothelial cysts or pseudocysts, while pheochromocytomas have been identified more commonly with epithelial cysts.3 Ap?prox?i?mately 7% of adrenal cysts are associated with malignant or potentially malignant neoplasms. Complete pathologic sampling of the cyst is necessary to rule out a focal cancer. Hemorrhage or cystic degeneration within an adrenal mass must be differentiated from coagulative tumor necrosis. Pathologic features of eosinophilic tumor cytoplasm, vascular invasion, foci of necrosis, and mitotic figures are associated with malignancy.7 Patients with a history of cancer, especially breast or lung cancer, can have cystic metastasis to the adrenal glands.7
Treatment of adrenal cysts?Small, uncomplicated cysts (those smaller than 4 cm with walls less than 3 mm thick and low attenuation fluid without hemorrhage or septations) may be observed.2 If these lesions are stable in size, asymptomatic, and nonfunctional, observation with serial CT scanning over a 2-year period is sufficient.3,11 Some have recommended aspiration with or without sclerosis as a form of treatment for large, simple adrenal cysts, but this treatment has a significant recurrence rate.3,11 Mar?supialization or enucleation of the cyst cavity may also be an option to preserve the adrenal gland and can be performed laparoscopically.
Laparoscopic resection of adrenal lesions has become more common compared with open operations. Small studies have demonstrated adrenal cysts can be safely treated laparoscopically.8 Large cysts may be treated with marsupialization, cystectomy, or partial adrenalectomy via the laparoscope, avoiding complete adrenalectomy.4,8 Pheochromo?cytomas can also be removed safely by laparoscopic techniques without any difference in complications, blood loss, or operative time.12
Cystic adrenal lesions are rare but require a thorough diagnostic evaluation. Large cysts in the upper abdomen should be suspected of being adrenal in origin, and the patient should be evaluated for a functional adrenal tumor. Large cysts may require evacuation before laparoscopic resection. Any abnormality in the cyst wall should be extensively sampled for small pheochromocytoma and malignancy.
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