Retroperitoneal Foregut Duplication Cyst Presenting As an Adrenal Mass

Written By: Sam Savage

Published Date: March 23, 2007 Last Edited: March 23, 2007

By Terry, N Elizabeth; Senkowski, Christopher K; Check, William; Brower, Steven T

A 75 year-old woman presented to the authors’ institution with abdominal pain and early satiety. An adrenal mass was found on CT scanning. Laparoscopic adrenalectomy was performed, and the patient was found to have a retroperitoneal bronchogenic cyst adherent to the adrenal gland. The workup of an adrenal mass is discussed as well as the pathophysiology of bronchogenic cysts.

THE DISCOVERY OF incidental adrenal masses has increased secondary to increased utilization of advanced imaging techniques. Many adrenal masses are found by imaging conducted for other reasons, and autopsy studies show more than a 2 per cent prevalence of clinically unapparent adrenal tumors.1 The workup of incidental adrenal masses can reveal functioning or nonfunctioning lesions, carcinomas, or other benign lesions. When identified, a diagnostic workup is performed to determine hormonal functionality and malignancy. The classification of the majority of masses includes adenomas, pheochromocytomas, myelolipomas, ganglioneuromas, adrenal cysts, hematomas, adrenal cortical carcinomas, metastases, and other more cryptic entities.1 All functioning tumors must be removed. As for nonfunctioning masses, a recent National Institutes of Health consensus panel recommends a more stratified approach.

Seldom does a surgeon come across an embryologic mass identified as a foregut duplication cyst. These are more commonly identified with pediatric mediastinal tumors. Congenital cysts are rarely encountered in the pediatric population and even more rarely discovered in adulthood. They can occur above or, rarely, below the diaphragm. The pathology of these cysts reveals tissue that is pericardial, bronchogenic, or enteric, with a small percentage that is histologically unclassifiable. Bronchogenic and enteric cysts are often referred to as foregut duplication cysts because they originate from aberrant portions of the ventral and dorsal foregut respectively.2-4

We present the case of an elderly woman who was discovered to have a foregut duplication cyst in the retroperitoneum, after workup for an adrenal incidentaloma.

Case Report

A 75-year-old woman presented to the emergency room for abdominal pain and was admitted by the internal medicine department. She reported a 25 kg weight loss with early satiety, decreased appetite, and chronic shortness of breath. She had no changes in her bowel habits. Her past medical history was significant for dilated cardiomyopathy with congestive heart failure and previous myocardial infarction, hypertension, gout, and atrial fibrillation. Past surgical history was significant for hysterectomy and excision of a benign oral tumor. Her medications included coumadin, Lopressor, allopurinol, digoxin, Vasotec, Lipitor, Premarin, and Lasix. The patient was a nonsmoker and denied alcohol use. Family history was unknown. Her review of systems was significant for the previously mentioned weight loss, anorexia, abdominal pain, and palpitations. She reported no headaches, flushing, nausea, or diarrhea. On physical examination, the patient was a thin, cachectic female. Her blood pressure was 136/90 mm Hg and her heart rate was 69 beats per minute (bpm). She was awake, alert, oriented, and neurovascularly intact. She had a 3/6 systolic murmur with no arrhythmia. Auscultation of her lungs revealed bibasilar crackles. Her abdomen was soft and flat with no palpable masses. Her extremities showed no cyanosis or edema.

The patient underwent CT and she was found to have a 5-cm left adrenal mass (Fig. 1). The mass was not demonstrated with washout on delayed images, and Hounsfield units measured 100-atypical for a benign adenoma. No adenopathy was appreciated. Functional studies of the mass were negative, to include 24-hour urine cortisol, urine and serum epinephrine and norepinephrine levels, and serum potassium level. She was referred to the surgery service for further evaluation. MRI was planned to assess for metastatic disease, but the patient refused the study secondary to claustrophobia. Preoperative colonoscopy and esophagogastroduodenoscopy revealed no other lesions to explain her 50pound weight loss.

FIG. 1. CT scan revealing a 5-cm left adrenal mass.

Laparoscopic adrenalectomy was performed on the patient’s third hospital day. The adrenal gland itself appeared normal; however, there was a 5-cm fleshy, soft, cystic mass that was adherent to the lateral-most portion of the gland (Fig. 2). En bloc resection of the adrenal gland and mass was undertaken.

Postoperatively the patient was admitted to the intensive care unit. Her course was uneventful save for a mild ileus, which resolved by postoperative day 4. She had no cardiac complications. She was discharged home on postoperative day 5 in stable condition.

Gross pathology revealed a fluid-filled cystic mass. Microscopic evaluation showed a benign adrenal gland without evidence of nodularity, neoplasia, or true hyperplasia. Surrounding adipose tissues contained large numbers of blood vessels, peripheral nerve fibers, and a true cyst lined by tall, columnar, ciliated cells consistent with an upper respiratory tract-type epithelium with an underlying wall containing bundles of smooth muscle (Fig. 3). Rare, small epitheliallined glandular inclusions extended into the muscle off of the true single cyst lining. The findings were consistent with a benign retroperitoneal bronchogenic foregut duplication cyst. Direct connection to the adrenal gland was not identified.

FIG. 2. Intraoperative photo of the adrenal gland and mass. A, bronchogenic cyst; B, left adrenal gland; C, left adrenal vein divided; D, vein to the bronchogenic cyst; E, splenic artery.

FIG. 3. Microscopic view of the mass demonstrating tall, columnar, ciliated cells with an underlying wall of smooth muscle.

Discussion

Clinically inapparent adrenal masses are challenging entities for surgeons and other physicians. In 2002, the NIH published a consensus on the management of incidentalomas, including diagnostic workup, surgical indications, and follow-up protocols. Diagnostic evaluation consists of laboratory and imaging data, and is performed to determine the hormonal and functional activity of the mass and to determine the presence of malignancy (Fig. 4).

The results of the diagnostic testing determine the need for surgery. In patients with functional tumors, adrenalectomy is the treatment of choice.1 In functional tumors without clinical symptoms, some argue that treatment options become more difficult. The risk for hypertensive crisis warrants adrenalectomy with “silent” pheochromocytomas. Other more subtle chemical abnormalities, such as insulin resistance, could lead to long-term physical derangements. The current recommendations, therefore, are for excision of any functional tumor.1

In patients with nonfunctioning tumors, distinguishing between benign and malignant tumors dictates treatment strategies. Variables to consider include the size of the lesion, its imaging characteristics, and its growth rate. More than 60 per cent of adrenalomas less than 4 cm are benign adenomas, and approximately 25 per cent of masses larger than 6 cm are found to be adrenocortical carcinomas.1 Radiographically, adrenocortical carcinomas are inhomogeneous masses with irregular margins that enhance after administration of intravenous contrast. On MRI, they often demonstrate increased signal intensity with T2-weighted images. On chemical-shift MRI, the signal does not “drop out” on opposed-phase imaging.5 The general recommendation is to excise lesions that are larger than 6 cm or have radiographie characteristics suggestive of malignancy.1 Lesions that are smaller than 4 cm and appear to be defined as low risk by imaging criteria are unlikely to have malignant potential and are generally not resected, especially in patients older than 50 years of age.6 Follow-up recommendations vary, but the transformation rate of small (

FIG. 4. Recommended workup for an incidental adrenal mass.

The first laparoscopic adrenalectomy was described in 1992, and since then it has become the standard of care for benign adrenal masses in many institutions.7 Laparoscopic techniques use smaller incisions with less tissue trauma, resulting in decreased morbidity and mortality.8-11 When performed by skilled surgeons, laparoscopic adrenalectomy has shown to decrease hospital stay, postoperative complications, and costs to the hospital and patient.8, 12, 13 Faster returnto-work times that have been demonstrated with other laparoscopic cases also holds true for laparoscopic adrenalectomy.8

Fewer than 20 cases of retroperitoneal bronchogenic cysts have been reported in the English literature, and less than five originally presented as adrenal masses. Because these retroperitoneal cysts are so rare and are not actually part of the adrenal gland itself, they are rarely included in the discussion of adrenal incidentalomas. Bronchog\enic cysts are usually found above the diaphragm and are developmental abnormalities of the primitive foregut.

Bronchogenic cysts arise from an abnormal budding of the tracheobronchial anlage of the primitive foregut during the third to seventh weeks of development. When attachment to the primitive foregut persists, the cyst remains associated with the tracheobronchial tree or the esophagus. If complete separation occurs, the cyst may occur in other unusual locations, presumably by migration.14, 15

Retroperitoneal locations are extremely rare for bronchogenic cysts. Those retroperitoneal cysts that have been described were located in the region of the left adrenal gland or the superior body of the pancreas. Most are very small and asymptomatic, and symptomatic cysts are usually very large-often 7 to 10 cm in size. One patient has even been described with pheochromocytoma-like symptoms from compression of the adrenal gland.16 Our patient presented with the vague complaints of abdominal pain and early satiety. She had no endocrine symptoms with a tumor approximately 5 cm in diameter.

The treatment of retroperitoneal bronchogenic cysts is surgical removal. Most of these masses are asymptomatic, but excision is recommended to establish diagnosis, alleviate any symptoms, and prevent complications such as infection or malignant transformation.17 Our patient underwent surgery not with a diagnosis of a bronchogenic cyst, but on the indication of an adrenal mass with abnormal imaging studies. More than 1 year later, she has completely recovered with improved weight gain and amelioration of her gastrointestinal symptoms.

REFERENCES

1. NIH state-of-the-science statement on management of the clinically inapparent adrenal mass (“incidentaloma”). NIH Consens State Sci Statements. 2002;19:1-25.

2. Silverman NA, Sabiston DCJ. Mediastinal masses. Surg Clin North Am 1980;60:757-77.

3. Salyer DC, Salyer WR, Eggleston JC. Benign developmental cysts of the mediastinum. Arch Pathol Lab Med 1997;101:136-9.

4. Norton JA, Bollinger RR, et al. Surgery: Basic Science and Clinical Evidence. New York: Springer, 2001.

5. Cameron JL. Current Surgical Therapy, 7th Ed. Philadelphia, PA: Mosby, 2001.

6. Saren ED, Prinz RA. Selection of patients with adrenal incidentalomas for operation. Surg Clin North Am 1995;75: 499-509.

7. Gagner M, Lacroix A, Boite E. Laparoscopic adrenalectomy in Cushing’s syndrome and pheochromocytoma. N Engl J Med 1992;327:1033.

8. Shell SR, Talamini MA, Udelsman R. Laparoscopic adrenalectomy for non-malignant disease: Improved safety, morbidity and cost- effectiveness. Surg Endosc 1998;13:30-40.

9. Takeda M, Go H, Imai T, et al. Experience with 17 cases of laparoscopic adrenalectomy: Use of ultrasonic aspirator and argon beam coagulator. J Urol 1994;152:902-5.

10. Go H, Takeda M, Imai T. Laparoscopic adrenalectomy for Cushing’s syndrome: Comparison with primary aldosteronism. Surgery (St. Louis) 1995;117:11-7.

11. Gagner M, Lacroix A, Boite E, et al. Laparoscopic adrenalectomy: The importance of a flank approach in the lateral decubitus position. Surg Endosc 1994;8:135-8.

12. Guazzoni G, Montorsi F, Bergamashi F, et al. Effectiveness and safety of laparoscopic adrenalectomy. J Urol 1994;152: 1375-8.

13. Rutherford JC, Stowasser M, Tunny TJ, et al. Laparoscopic adrenalectomy. World J Surg 1996;20:758-61.

14. Coselli MP, Ipolyi P, Bloss RS, et al. Bronchogenic cysts above and below the diaphragm: Report of eight cases. Am Thorac Surg 1987;44:491-4.

15. Sumiyoshi K, Simizu S, Enjoji M, et al. Bronchogenic cyst in the abdomen. Virchows Arch 1985;408:93-8.

16. Dogget RS, Carty SE, Clarke MR. Retroperitoneal bronchogenic cyst masquerading clinically and radiologically as a phaeochromocytoma. Virchows Arch 1997;431:73-6.

17. Sullivan SM, Okada S, Kudo M, et al. A retroperitoneal bronchogenic cyst with malignant change. Pathol Int 1999;49: 338- 41.

N. ELIZABETH TERRY, M.D.,* CHRISTOPHER K. SENKOWSKI, M.D.,* WILLIAM CHECK, M.D.,[dagger] STEVEN T. BROWER, M.D.*

From the Departments of * Surgical Education and [dagger] Pathology, Memorial Health University Medical

Center, Mercer University School of Medicine, Savannah Campus, Georgia

Address correspondence and reprint requests to Christopher K. Senkowski, M.D., Associate Professor of Surgery, Mercer University School of Medicine, 2nd Floor, Georgia Ear Institute, 4700 Waters Avenue, Savannah, GA 31404.

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