Pseudoadrenal Mass: Unusual Presentation of Bronchogenic Cyst By Sidney
J. Swanson
III, Steven J. Skoog, Washington,
0 Isolated abdominal bronchogenic cysts are extremely rare. We report the fourth such case in an asymptomatic 4-year-old girl who initially presented for evaluation of urinary tract infection and new-onset urinary incontinence. Ultrasound, computed tomography, and magnetic resonance imaging findings were consistent with an adrenal mass. At exploration, the patient was found to have a mass in the area of the gastroesophageal junction and a normal left adrenal gland. The final pathology confirmed the diagnosis of a bronchogenic cyst. Abdominal bronchogenic cysts, although rare, should be considered in the diagnosis of a retroperitoneal mass. Copyright $1 1991 by W. B. Saunders Company INDEX
WORDS:
Bronchogenic
cyst,
abdominal.
RONCHOGENIC cysts are rare abnormalities of the ventral foregut. The majority of these lesions occur above the diaphragm, with a small number of “dumbbell” or transdiaphragmatic bronchogenic cysts reported. There have been only three reported cases of isolated abdominal bronchogenic cysts and we report the fourth occurrence in an asymptomatic 4-year-old girl.
B
CASE
REPORT
A 4-year-old black girl presented with a history of diurnal enuresis after 18 months of continence. She previously had two afebrile bacterial urinary tract infections. Physical examination showed a normal, healthy young girl who was tall for her age. Radiological assessment of the urinary tract was performed because of the history of recurrent urinary infections. A voiding cystourethrogram was normal with no evidence of vesicoureteral reflux. Renal sonography demonstrated a left upper pole simple renal cyst and a solid left adrenal mass. A computed tomography (CT) scan of the abdomen confirmed the left renal cyst and a 2- to 3.cm soft tissue mass of 30 Hounsfield units (HU) in the left adrenal bed (Fig 1). A magnetic resonance imaging study showed the suspected adrenal mass with a long T2 and a neutral Tl signal (Fig 2). Serum electrolytes, dihydroepiandrosterone, cortisol, 17ketosteroid, 17-hydroxysteroids, pregnanetriol, androstenedione, catecholamine, vanillylmandelic acid, and metanephrines were normal. The patient underwent abdominal exploration, which showed a normal left adrenal. However, the mass was noted near the gastroesophageal junction and was mobilized until it was connected only by a fibrous stalk that passed posterior to the esophageal hiatus. The stalk was suture ligated and transected and the mass was removed. The patient’s postoperative course was uncomplicated.
Pathological Findings The lesion measured 2.1 cm, was multicystic, and contained a white mucoid substance. Microscopically, the cyst was lined with pseudostratified ciliated columnar epithelium. The wall was comprised of smooth muscle, cartilage plates, and peripheral nerve JournalofPediatricSurge~
Vol26,No
12 (December),1991:pp
1401-1403
Victor DC
Garcia, and Richard
C. Wahl
branches. Bronchial mucous glands were observed opening into the lumen. The pathological diagnosis was bronchogenic cyst without evidence of teratoma (Fig 3). DISCUSSION
Between the 4th and 6th weeks of embryologic development, the primitive foregut separates to form the ventral trachea and dorsal esophagus. Incomplete division results in tracheoesophageal fistula. Abnormalities in the further development of the dorsal foregut may result in esophageal lesions such as atresia or duplication. Abnormal budding of the ventral foregut (future tracheobronchial tree) in the ensuing development of the lungs results in the anomalies of bronchogenic cyst, pulmonary sequestration, and tracheal lobe. Pulmonary sequestrations are markedly abnormal collections of nonfunctioning pulmonary tissue without connection to the bronchial tree. Extrathoracic locations have been described. Tracheal lobes arise from normal bronchi that develop proximal to the carina. They are usually present with obstructive respiratory symptoms. Surgical excision is the treatment of choice. An embryologic explanation for the occurrence of abdominal bronchogenic cyst was outlined by Sumiyoshi et al.’ Early in embryonic life, the abdominal and thoracic cavities are connected by the pericardioperitoneal canal. With the closure of the pleuroperitoneal membranes, which forms the future diaphragm, abnormal lung buds could be pinched off and trapped in the abdomen. Bronchogenic cysts are rare cystic malformations with walls similar to those of normal bronchi and with respiratory epithelial lining. They usually have no functional connection with the bronchial tree but often are attached by a fibrous cord to the bronchus. The majority are intrathoracic and intralobar. Cutaneous, intrapericardiac, and intradiaphragmatic locaFrom the Departments of Pediatric Urology and Pediatric Surgery Walter Reed Army Medical Center, and the Pediattic Pathology Section, Armed Forces Institute of Pathology, Washington, DC. The opinions and assertions contained herein are the private views of the authors and are not to be construed as o@ial or reflecting the views of the Department of the Army or the Department of Defense. Address reprint requests to Steven J. Skoog, MD, Chie$ Pediatric Urology Service, Walter Reed Amy Medical Center, Washington, DC 20307-5001. Copyright o 1991 by WB. Saunders Company 0022-3468191/2612-0015$03.0010 1401
1402
SWANSON
Fig 1. CT scan of the mass measuring 30 HU.
abdomen
showing
a 2 x 3 cm suprarenal
tions have been described.“4 Presentation may include cough, pneumonia, dyspnea, and frank respiratory distress. Treatment is surgical excision. Abdominal location of a bronchogenic cyst is unusual. Amendola et al’ reported the 10th case of transdiaphragmatic (“dumbbell”) bronchogenic cyst in a lZyear-old boy with dyspneic episodes. Only the thoracic lesion was diagnosed prior to operation. At the time of operation it was noted that the cyst communicated with an abdominal component. There are only three previously described cases of isolated abdominal bronchogenic cyst. A 59-year-old man had a symptomatic 6-cm cyst near the gastro-
Fig 2. abdomen
TZ-weighted coronal showing a suprarenal
ET AL
magnetic resonance mess consistent with
image of the adrenal lesion.
Fig 3. Micrograph of pathological specimen demonstrating pseudostratified columnar epithelium, bronchial glands, smooth muscle, and cartilage consistent with the diagnosis of bronchogenic cyst.
esophageal junction causing epigastric pain, nausea, and vomiting.’ Miller et al6 described an infant with abdominal bronchogenic cyst. The child had multiple anomalies to include a duplicated stomach. Murley and Lenz’ discovered a third case in an asymptomatic 17-year-old boy. The present case is a classic bronchogenic cyst by pathological diagnosis but is extremely unusual as to its location. It was asymptomatic but may have increased in size and caused symptoms of esophageal obstruction due to its location. Based on the preoperative evaluation, the differential diagnosis was that of an adrenal mass. In children, this includes neuroblastoma, sympathoblastoma, pheochromocytoma, adenoma, adrenocortical carcinoma, and rare mesenchyma1 tumors such as fibromas, lipomas, hemangiomas, and myelolipomas. Buddingtor? described a bronchogenic cyst interpreted as an adrenal mass in 1957. The preoperative diagnosis of an adrenal mass was suggested by the intravenous pyelogram that demonstrated deviation
ABDOMINAL
BRONCHOGENIC
CYST
of the renal axis. However, a transdiaphragmatic bronchogenic cyst was found at surgery. Radiological assessment of an adrenal mass in the child includes CT scanning and magnetic resonance imaging. The CT number of 30 HU observed in the present patient was consistent with four cases of bronchogenic cyst described by Mendelson et ak9 who found HU ranging from 30 to 100. This range of density coefficients is more consistent with solid lesions and supported the diagnosis of adrenal mass. The magnetic resonance imaging finding of a long T2 relaxation time is consistent with the observation of Brasch et al”’ in a patient with a thoracic bronchogenie cyst. Long T2 relaxation times are also commonly observed with most adrenal malignancies. Recent studies by Reinig et al” have indicated that magnetic resonance imaging can accurately distinguish adrenal adenomas from adenocarcinoma, pheochromocytomas, and adrenal metastases by comparing the T2 signal intensity with that of the liver.
1403
Pheochromocytoma and malignant adrenal masses could also be generally differentiated from each other. Simple cysts normally have long Tl and long T2 relaxation times (signal intensity equal to or greater than skeletal muscle on Tl and brighter than fat on T2). Bronchogenic cysts, due to their higher protein content, will have shorter Tl relaxation times (greater signal intensity), as seen in this patient. We have reviewed the literature regarding abdominal bronchogenic cysts and report the fourth such case. This was the first patient in whom magnetic resonance imaging was available in the diagnostic evaluation. However, in this instance it was not helpful in determining the diagnosis. Instead, this case demonstrates the uncertainty of present diagnostic techniques. Abdominal bronchogenic cyst, although rare, should be considered in the differential diagnosis of a retroperitoneal mass.
REFERENCES 1. Sumiyoshi K, Shimizu S, Enjoji M, et al: Bronchogenic cyst in 6. Miller RF, Graub M, Pashuck ET: Bronchogenic cysts (case the abdomen. Virchows Arch Path01 Anat 408:93-98, 1985 VIII). Am J Roentgen01 Rad Ther Nucl Med 70:771-785, 1953 7. Murley GD, Lenz TR: Bronchogenic cyst, intraabdominal. 2. Aaron BL: Intradiaphragmatic cyst: A rare entity. J Thorac Rocky Mtn Med J 76:243-244,1979 Cardiovasc Surg 49:531-534, 1965 8. Buddington TW: Intradiaphragmatic cyst, ninth reported 3. Deenadyalu RP, Tuure D, Dewall RA, et al: Intrapericardial case. N Engl J Med 257:613-615,1957 teratoma and bronchogenic cyst. Review of literature and report of 9. Mendelson DS, Rose JS, Efremidis SC, et al: Bronchogenic successful surgery in infant with intrapericardial teratoma. J cysts with high CT numbers. AJR Am J Roentgen01 140:463-46.5, Thorac Cardiovasc Surg 67:945-952,1974 1983 4. Fraga S, Helwig EB, Rosen SH: Bronchogenic cyst of the skin 10. Brasch RC, Gooding CA, Lallemand DP, et al: Magnetic and subcutaneous tissue. Am J Clin Pathol56:230-238, 1971 resonance imaging of the thorax in childhood. Radiology 150:4635. Amendola MA, Shirazi KK, Brooks J, et al: Transdiaphrag467,1984 maic bronchopulmonary foregut anomaly: “Dumbbell” broncho11. Reinig JW, Doppman JL, Dwyer AJ, et al: Adrenal masses genie cyst. AJR Am J Roentgen01 138:1165-1167, 1982 differentiated by MR. Radiology 158:81-84, 1986
J. Swanson
III, Steven J. Skoog, Washington,
0 Isolated abdominal bronchogenic cysts are extremely rare. We report the fourth such case in an asymptomatic 4-year-old girl who initially presented for evaluation of urinary tract infection and new-onset urinary incontinence. Ultrasound, computed tomography, and magnetic resonance imaging findings were consistent with an adrenal mass. At exploration, the patient was found to have a mass in the area of the gastroesophageal junction and a normal left adrenal gland. The final pathology confirmed the diagnosis of a bronchogenic cyst. Abdominal bronchogenic cysts, although rare, should be considered in the diagnosis of a retroperitoneal mass. Copyright $1 1991 by W. B. Saunders Company INDEX
WORDS:
Bronchogenic
cyst,
abdominal.
RONCHOGENIC cysts are rare abnormalities of the ventral foregut. The majority of these lesions occur above the diaphragm, with a small number of “dumbbell” or transdiaphragmatic bronchogenic cysts reported. There have been only three reported cases of isolated abdominal bronchogenic cysts and we report the fourth occurrence in an asymptomatic 4-year-old girl.
B
CASE
REPORT
A 4-year-old black girl presented with a history of diurnal enuresis after 18 months of continence. She previously had two afebrile bacterial urinary tract infections. Physical examination showed a normal, healthy young girl who was tall for her age. Radiological assessment of the urinary tract was performed because of the history of recurrent urinary infections. A voiding cystourethrogram was normal with no evidence of vesicoureteral reflux. Renal sonography demonstrated a left upper pole simple renal cyst and a solid left adrenal mass. A computed tomography (CT) scan of the abdomen confirmed the left renal cyst and a 2- to 3.cm soft tissue mass of 30 Hounsfield units (HU) in the left adrenal bed (Fig 1). A magnetic resonance imaging study showed the suspected adrenal mass with a long T2 and a neutral Tl signal (Fig 2). Serum electrolytes, dihydroepiandrosterone, cortisol, 17ketosteroid, 17-hydroxysteroids, pregnanetriol, androstenedione, catecholamine, vanillylmandelic acid, and metanephrines were normal. The patient underwent abdominal exploration, which showed a normal left adrenal. However, the mass was noted near the gastroesophageal junction and was mobilized until it was connected only by a fibrous stalk that passed posterior to the esophageal hiatus. The stalk was suture ligated and transected and the mass was removed. The patient’s postoperative course was uncomplicated.
Pathological Findings The lesion measured 2.1 cm, was multicystic, and contained a white mucoid substance. Microscopically, the cyst was lined with pseudostratified ciliated columnar epithelium. The wall was comprised of smooth muscle, cartilage plates, and peripheral nerve JournalofPediatricSurge~
Vol26,No
12 (December),1991:pp
1401-1403
Victor DC
Garcia, and Richard
C. Wahl
branches. Bronchial mucous glands were observed opening into the lumen. The pathological diagnosis was bronchogenic cyst without evidence of teratoma (Fig 3). DISCUSSION
Between the 4th and 6th weeks of embryologic development, the primitive foregut separates to form the ventral trachea and dorsal esophagus. Incomplete division results in tracheoesophageal fistula. Abnormalities in the further development of the dorsal foregut may result in esophageal lesions such as atresia or duplication. Abnormal budding of the ventral foregut (future tracheobronchial tree) in the ensuing development of the lungs results in the anomalies of bronchogenic cyst, pulmonary sequestration, and tracheal lobe. Pulmonary sequestrations are markedly abnormal collections of nonfunctioning pulmonary tissue without connection to the bronchial tree. Extrathoracic locations have been described. Tracheal lobes arise from normal bronchi that develop proximal to the carina. They are usually present with obstructive respiratory symptoms. Surgical excision is the treatment of choice. An embryologic explanation for the occurrence of abdominal bronchogenic cyst was outlined by Sumiyoshi et al.’ Early in embryonic life, the abdominal and thoracic cavities are connected by the pericardioperitoneal canal. With the closure of the pleuroperitoneal membranes, which forms the future diaphragm, abnormal lung buds could be pinched off and trapped in the abdomen. Bronchogenic cysts are rare cystic malformations with walls similar to those of normal bronchi and with respiratory epithelial lining. They usually have no functional connection with the bronchial tree but often are attached by a fibrous cord to the bronchus. The majority are intrathoracic and intralobar. Cutaneous, intrapericardiac, and intradiaphragmatic locaFrom the Departments of Pediatric Urology and Pediatric Surgery Walter Reed Army Medical Center, and the Pediattic Pathology Section, Armed Forces Institute of Pathology, Washington, DC. The opinions and assertions contained herein are the private views of the authors and are not to be construed as o@ial or reflecting the views of the Department of the Army or the Department of Defense. Address reprint requests to Steven J. Skoog, MD, Chie$ Pediatric Urology Service, Walter Reed Amy Medical Center, Washington, DC 20307-5001. Copyright o 1991 by WB. Saunders Company 0022-3468191/2612-0015$03.0010 1401
1402
SWANSON
Fig 1. CT scan of the mass measuring 30 HU.
abdomen
showing
a 2 x 3 cm suprarenal
tions have been described.“4 Presentation may include cough, pneumonia, dyspnea, and frank respiratory distress. Treatment is surgical excision. Abdominal location of a bronchogenic cyst is unusual. Amendola et al’ reported the 10th case of transdiaphragmatic (“dumbbell”) bronchogenic cyst in a lZyear-old boy with dyspneic episodes. Only the thoracic lesion was diagnosed prior to operation. At the time of operation it was noted that the cyst communicated with an abdominal component. There are only three previously described cases of isolated abdominal bronchogenic cyst. A 59-year-old man had a symptomatic 6-cm cyst near the gastro-
Fig 2. abdomen
TZ-weighted coronal showing a suprarenal
ET AL
magnetic resonance mess consistent with
image of the adrenal lesion.
Fig 3. Micrograph of pathological specimen demonstrating pseudostratified columnar epithelium, bronchial glands, smooth muscle, and cartilage consistent with the diagnosis of bronchogenic cyst.
esophageal junction causing epigastric pain, nausea, and vomiting.’ Miller et al6 described an infant with abdominal bronchogenic cyst. The child had multiple anomalies to include a duplicated stomach. Murley and Lenz’ discovered a third case in an asymptomatic 17-year-old boy. The present case is a classic bronchogenic cyst by pathological diagnosis but is extremely unusual as to its location. It was asymptomatic but may have increased in size and caused symptoms of esophageal obstruction due to its location. Based on the preoperative evaluation, the differential diagnosis was that of an adrenal mass. In children, this includes neuroblastoma, sympathoblastoma, pheochromocytoma, adenoma, adrenocortical carcinoma, and rare mesenchyma1 tumors such as fibromas, lipomas, hemangiomas, and myelolipomas. Buddingtor? described a bronchogenic cyst interpreted as an adrenal mass in 1957. The preoperative diagnosis of an adrenal mass was suggested by the intravenous pyelogram that demonstrated deviation
ABDOMINAL
BRONCHOGENIC
CYST
of the renal axis. However, a transdiaphragmatic bronchogenic cyst was found at surgery. Radiological assessment of an adrenal mass in the child includes CT scanning and magnetic resonance imaging. The CT number of 30 HU observed in the present patient was consistent with four cases of bronchogenic cyst described by Mendelson et ak9 who found HU ranging from 30 to 100. This range of density coefficients is more consistent with solid lesions and supported the diagnosis of adrenal mass. The magnetic resonance imaging finding of a long T2 relaxation time is consistent with the observation of Brasch et al”’ in a patient with a thoracic bronchogenie cyst. Long T2 relaxation times are also commonly observed with most adrenal malignancies. Recent studies by Reinig et al” have indicated that magnetic resonance imaging can accurately distinguish adrenal adenomas from adenocarcinoma, pheochromocytomas, and adrenal metastases by comparing the T2 signal intensity with that of the liver.
1403
Pheochromocytoma and malignant adrenal masses could also be generally differentiated from each other. Simple cysts normally have long Tl and long T2 relaxation times (signal intensity equal to or greater than skeletal muscle on Tl and brighter than fat on T2). Bronchogenic cysts, due to their higher protein content, will have shorter Tl relaxation times (greater signal intensity), as seen in this patient. We have reviewed the literature regarding abdominal bronchogenic cysts and report the fourth such case. This was the first patient in whom magnetic resonance imaging was available in the diagnostic evaluation. However, in this instance it was not helpful in determining the diagnosis. Instead, this case demonstrates the uncertainty of present diagnostic techniques. Abdominal bronchogenic cyst, although rare, should be considered in the differential diagnosis of a retroperitoneal mass.
REFERENCES 1. Sumiyoshi K, Shimizu S, Enjoji M, et al: Bronchogenic cyst in 6. Miller RF, Graub M, Pashuck ET: Bronchogenic cysts (case the abdomen. Virchows Arch Path01 Anat 408:93-98, 1985 VIII). Am J Roentgen01 Rad Ther Nucl Med 70:771-785, 1953 7. Murley GD, Lenz TR: Bronchogenic cyst, intraabdominal. 2. Aaron BL: Intradiaphragmatic cyst: A rare entity. J Thorac Rocky Mtn Med J 76:243-244,1979 Cardiovasc Surg 49:531-534, 1965 8. Buddington TW: Intradiaphragmatic cyst, ninth reported 3. Deenadyalu RP, Tuure D, Dewall RA, et al: Intrapericardial case. N Engl J Med 257:613-615,1957 teratoma and bronchogenic cyst. Review of literature and report of 9. Mendelson DS, Rose JS, Efremidis SC, et al: Bronchogenic successful surgery in infant with intrapericardial teratoma. J cysts with high CT numbers. AJR Am J Roentgen01 140:463-46.5, Thorac Cardiovasc Surg 67:945-952,1974 1983 4. Fraga S, Helwig EB, Rosen SH: Bronchogenic cyst of the skin 10. Brasch RC, Gooding CA, Lallemand DP, et al: Magnetic and subcutaneous tissue. Am J Clin Pathol56:230-238, 1971 resonance imaging of the thorax in childhood. Radiology 150:4635. Amendola MA, Shirazi KK, Brooks J, et al: Transdiaphrag467,1984 maic bronchopulmonary foregut anomaly: “Dumbbell” broncho11. Reinig JW, Doppman JL, Dwyer AJ, et al: Adrenal masses genie cyst. AJR Am J Roentgen01 138:1165-1167, 1982 differentiated by MR. Radiology 158:81-84, 1986
