BrifishJournolof Urology (1990). 65,57&575
8 1990 British Journal of Urology
Adrenal Cysts: Diagnosis and Management A. S. SROUJIEH, G. R. FARAH, M. J. HADDAD and M. M. ABU-KHALAF Department of Surgery, University of Jordan and the Surgical Services of Jordan University Hospital, Amman, Jordan
Summary-We present 7 patients (5 adult females and 2 neonate males) with adrenal cysts. The cysts included 1 hydatid, 1 lymphatic and 5 pseudocysts. Three cysts were diagnosed preoperatively and all were resected surgically. The existence of true epithelial cysts of the adrenal gland is doubted by many authors; the present series includes most types of adrenal cyst.
Adrenal cysts are a rare occurrence. Greiselius reported the first case in 1670. Scattered case reports appeared until 1959, when Abeshouse etal. reviewed 155 cases from the literature, including 3 of their own. The total number of cases reviewed by Foster (1966) rose to 220, including 1 of his own. With the advent of computed tomography (CT) and ultrasound (US) scans (Copeland, 1983), more cysts are being discovered incidentally. We describe 7 types of adrenal cyst treated surgically at Jordan University Hospital between 1977 and 1989.
Patients and Methods The records of 27 patients who underwent adrenalectomy between June 1977 and June 1989 were reviewed. Adrenal cyst was the indication for adrenalectomy in 7 patients (Table). They included 2 neonate males (6 and 12 days old) and 5 adult females aged between 40 and 78 years. Three cysts were discovered incidentally: 2 neonates were found to have right upper abdominal masses during investigation for sepsis and jaundice which proved to be physiological, and in a 60-year-old female the cyst was found during US studies for gallbladder disease; a 6-day-old neonate had a history of difficult vaginal delivery. Four patients were symptomatic and had upper abdominal and loin pain. One had had a left nephrectomy for calculi several years earlier and another had undergone previous surgery for retroperitoneal hydatid disease with a Accepted for publication 11 October 1989
discharging sinus in the left iliac fossa (LIF). An abdominal mass was felt in a 78-year-old female and hepatomegaly was detected in a 40-year-old female. Laboratory tests showed leukocytosis in a patient with recurrent infected hydatid disease and an erythrocyte sedimentation rate (ESR) above 100 mm in the 78-year-old female, who proved later to have a malignant cyst. Serum electrolytes were normal and hormonal studies, requested in 3 patients, were also normal, Casoni’s test and echinococcus antibody titres were evaluated in 3 patients and were positive in 1. Radiological studies showed peripheral curvilinear calcification (Fig. 1) in a 44-year-old female at the site of previous nephrectomy. Two patients had stippled calcification in the right hypochondrium ; a neonate also had a non-functioning right kidney and a 78-year-old had, in addition, distorted upper calices in the right kidney and absence of neovascularisation on renal arteriography (Fig. 2 ) . US and CT scans were done in 5 patients and all showed cystic retroperitoneal swellings. A pre-operative diagnosis of adrenal cyst was made in 3 cases: (1) in a patient with recurrent hydatid disease the tract of a discharging sinus from the LIF extended upwards behind the left kidney to a left adrenal cyst (Fig. 3 ) ; (2) in a neonate, new hyperdense areas were seen on a repeat ultrasound which rightly suggested a haemorrhagic cyst (Fig. 4); ( 3 ) a 60-year-old female was found to have a right adrenal cyst during US studies of the gallbladder. In the other 4 patients
570
571
ADRENAL CYSTS:DIAGNOSIS AND MANAGEMENT
Table Adrenal Cysts Year Age
Sex
Presentation
Imaging studies
Final diagnosis
1977 44years
F
Abdominal and loin pain L. nephrectomy
Adrenal pseudocyst
1980 12days
M
Sepsis. Abdominal mass
1983 78years
F
Abdominal and loin pain. Abdominal mass. ESR > 100
1988 40years
F
1988 6days
M
1989 50years
F
1989 60years
F
Abdominal and loin pain. Hepatomegaly Physiologicaljaundice. Abdominal mass Discharging sinus LIF. Previous surgery for hydatid cyst Gallbladder disease
Retroperitoneal cyst with curvilinear calcification. ? Hydatid cyst Stippled calcification. Non-functioning R. kidney. ? Neuroblastoma Stippled calcification. Distorted upper calices. No neovascularisation. ? Renal cyst US and CT: ? liver hydatid cyst
Adrenal pseudocyst due to infarction Cystic cortical carcinoma
Adrenal pseudocyst
US and CT: adrenal mass. Repeat US : haemorrhagic cyst CT: adrenal cyst with communicating tract to LIF
Haemorrhagic adrenal cyst Hydatid cyst
US: gall stones and adrenal cyst
Lymphatic cyst
the diagnosis was made at operation. Two cysts were diagnosed pre-operatively as hydatid cysts. The 78-year-old patient was thought to have a renal cyst due to distorted upper calices; she had mixed echoes on US, stippled calcification and a high ESR, yet the malignant nature of the cyst was missed due to absence of neovascularisation on the arteriogram. The fourth patient, a 12-day-old infant, was diagnosed as having a neuroblastoma. All patients underwent surgery through abdominal incisions. Adrenalectomy was achieved easily
Fig. I X-ray abdomen showing peripheral curvilinear calcification in left adrenal cyst.
in 4 cases. Additional nephrectomy was done in a neonate with an adherent non-functioning right kidney; splenectomy was indicated during excision of a hydatid cyst of the left adrenal gland. Excision of an adrenal cyst, proved to be malignant at operation, was followed by right nephrectomy and excision of a segment of inferior vena cava with reimplantation of the left renal vein into a dacron graft.
Results Pathologically, all cysts proved to be adrenal; 5 were on the right and 2 on the left. They included a hydatid cyst, a lymphatic cyst and 5 pseudocysts. In the latter group were a cyst due to degeneration in an adrenal cortical carcinoma, 2 cysts thought to be due to previous trauma following left nephrectomy in a 40-year-old female and after difficult delivery in a neonate; the fourth pseudocyst developed in an infarcted adrenal which was accompanied by evidence of infarction in the adjacent kidney, and the cause of the fifth cyst was not known. One neonate died soon after surgery from uncontrolled sepsis. The 78-year-old female with a malignant cyst developed deep venous thrombosis and repeated wound infection and died from sepsis 6 months after surgery. The other 5 patients recovered well and are symptom-free 3 months to 12 years post-operatively.
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BRITISH JOURNAL OF UROLOGY
Fig. 2 (A) Right renal arteriograrn with absent neovascularisation above kidney. Note scattered calcification. ( B ) IVU showing right kidney pushed downwards with distorted upper calices.
Discussion Adrenal cysts are rare and their incidence in autopsy studies ranges from 0.064% (Wahl, 1951) to 0.18% (Hodges and Ellis, 1958). Copeland (1983) found 5 cysts in 51 patients with adrenal masses discovered incidentally on CT scan. Adrenal cysts occur at all ages, from birth to 80 years, with a peak in the third to sixth decades (Abeshouse et al., 1959; Esquivel and Grabstald, 1965;Tehrani et a[., 1965; Foster, 1966; Cerneyet af., 1970). The female to male ratio is 2 : l in some reports (Abeshouse el al., 1959; Cerney et af., 1970), inconclusive or equal inothers(Foster, 1966; Mikailand Kirshman, 1968). The present series included 2 neonate males and 5 adult females and their ages ranged from 6 days to 78 years. Adrenal cysts are usually asymptomatic and many are discovered at autopsy (Wahl, 1951; Hodgeset a/., 1958; Abeshouse et a/., 1959; Foster, 1966). They seldom cause adrenal hyk -function in infants (Moore and Cermak, 1950), iushing’s syndrome (Brindley and Chisholm, 1951) or phaeochromocytoma (Ellis et al., 1952; Cheema et af., 1981). A correct pre-operative diagnosis was made in 3.2 to 7.2% of patients (Abeshouse et al., 1959;
Foster, 1966). Symptoms related to adrenal cysts include vague upper abdominal or loin pain, gastrointestinal upsets and abdominal mass. Acute abdomen and/or a tender mass occur occasionally when intracystic haemorrhage, rupture or infection occur (Stewart and Lester, 1962; Foster, 1966; Pasciak and Cook, 1988). The cyst may fill the abdomen to such an extent that it is diagnosed as an ovarian cyst (Foster, 1966). Three ofour patients were correctly diagnosed pre-operatively and 4 were diagnosed at surgery. Adrenal cysts were symptomatic in 4 of our adult patients who had abdominal and loin pain with acute exacerbations in a patient with a discharging sinus from an infected hydatid cyst. An abdominal mass was palpated in the 78-year-old female and incidentally in the 2 neonates, who were initially thought to have a neuroblastoma. A soft tissue swelling with curvilinear calcification is seen occasionally on plain abdominal films (Levison, 1933; Palubinska et al., 1959; Gordon et al., 1960); it denotes a cystic lesion rather than a neoplasm when the calcification is stippled (Foster, 1966; Cerney et al., 1970), as in our elderly patient, although such calcification is present in organised haematoma (Cheema et al., 1981 ; Copeland. 1983)
573
ADRENAL CYSTS:DIAGNOSIS A N D MANAGEMENT
Fig. 3 CT scan showing hydatid cyst in left adrenal gland (A) extending behind the left kidney (B) forming a tract (C and D)
or infarct, as in one of our neonate patients. IVU usually showsdownward displacement and rotation of the related kidney, which occasionally has distorted upper calices (Foster, 1966; Cerney et al., 1970). Barium studies of the gastrointestinal tract may show displacement of stomach and colon
anteriorly, downwards, and to the other side due to a large cyst (Abeshouse et al., 1959; Esquivel and Grabstald, 1965; Foster, 1966; Cerney et al., 1970). Neovascularisation on angiography suggests malignancy, although it is sometimes absent (Copeland, 1983). The absence of neovascularisation and the
Fig. 4 (A) US showing echogenic adrenal mass above right kidney (M). (B) US showing development of new hyperdense areas ( + ) in the right adrenal gland reported as new haemorrhages.
574 distortion in the upper calices of the right kidney led us to believe that our elderly patient had a renal cyst. US and CT scans are useful diagnostic tools and many adrenal masses are discovered incidentally (Copeland, 1983; Wood etal., 1987). These studies were positive in 5 of our patients. There were mixed echoes in the 78-year-old female which, together with the high ESR, should have alerted us to the possibility of malignancy. US was also very useful when it was repeated and showed the development of new hyperdense areas in the adrenal gland leading to the correct diagnosis of a haemorrhagic cyst in the 6-day-old neonate. Complete blood counts may show low haemoglobin and haematocrit levels if there is heavy intracystic bleeding (Pasciak and Cook, 1988), leukocytosis in infected cysts (Foster, 1966), as in our infected hydatid cyst, eosinophilia in parasitic cysts and a high ESR in malignant cysts, as in our elderly patient. Electrolytes and hormonal studies are usually normal except on the rare occasion where hypoadrenocorticism is present (Moore and Cermak, 1950), or pseudocysts in functioning tumours are the cause (Brindley and Chisholm, 1951 ; Ellis et al., 1952). Foster (1 966) divided adrenal cysts into 4 groups : (1) parasitic (echinococcal) cysts (7%); (2) epithelial cysts (973, including true glandular, embryonal cysts and cystic adenomas; (3) endothelial cysts (4573, including lymphatic cysts, lymphangiomas and haemangiomas ; (4) pseudocysts (39%) due to infarction or haemorrhage in the adrenal gland, or cystic degeneration in benign or malignant adrenal neoplasms. The existence of true epithelial cysts is doubted by many authors (Foster, 1966). Thus this study, although small, presents most types of adrenal cyst. Hydatid disease is endemic in Jordan (El-Muhtaseb, 1984) and our case represents a single occurrence of hydatid cyst in the adrenal gland in 250 (0.4%) hydatid cysts in various organs treated at this hospital. The incidence is therefore similar to the 0.5% reported by Barnett (1943). All cysts in our patients were unilateral (4 on the right side and 2 on the left). However, adrenal cysts are seldom bilateral (Levison, 1933; Moore and Cermak, 1950) and they have no predilection for either side (Abeshouse et a f . , 1959; Foster, 1966), except perhaps in adrenal haemorrhage in neonates, which is more common on the right side, possibly because of the susceptibility of the adrenal gland to compression between the liver and spine due to birth trauma (Kuhn et al., 1971); this may have occurred in 1 of our neonates who had a difficult delivery.
BRITISH JOURNAL OF UROLOGY
The treatment of adrenal cysts (Abeshouse et al., 1959; Foster, 1966) depends on the symptoms and size of the cyst, the occurrence of complications and the underlying pathology. Under the guidance of CT and US scans, asymptomatic non-functioning cysts can now be aspirated percutaneously (Copeland, 1983; Wood etal., 1987). Indications for surgery include large and complicated cysts, parasitic, functioning and malignant cysts (Abeshouse et al., 1959; Foster, 1966). Malignant cysts are rare (Schmieden and Peiper, 1950; Brindley and Chisholm, 1951 ; Plaut, 1962) and may be distinguished by a high ESR, aspiration cytology, neovascularisation on arteriography (Copeland, 1983) and the presence of mixed echoes on ultrasound. Excision of adrenal cysts in patients who do not have a phaeochromocytoma is reported to bring the blood pressure back to normal and may be taken into consideration (Foster, 1966; Cheema, et al., 1981). Surgical excision can usually be achieved through an abdominal or a loin incision (Foster, 1966) and occasionally by a thoraco-abdominal approach (Pasciak and Cook, 1988). All of our patients were treated surgically through abdominal or flank incisions. In 4 cases the cysts were easily excised. Nephrectomy was required in 2 patients, splenectomy in 1 and excision of a segment of inferior vena cava in a third because of adhesions or direct invasion. Five patients are alive and well and 2 died of sepsis. Adrenal cysts are seen occasionally by the surgeon and urologist. With the advent of better imaging techniques such as US and CT scans and magnetic resonance imaging, the diagnosis of adrenal cysts is possible pre-operatively and this was evident in our last 3 patients (Table). The distinction between malignant and benign adrenal cysts can still be difficult and underlying carcinoma should be suspected in patients with a high ESR, mixed echoes on ultrasound, stippled calcification on plain radiography and neovascularisation on arteriography. It should be emphasised that the last 2 radiological signs may be misleading and it is hoped that aspiration cytology will differentiate more accurately between benign and malignant cysts.
References Abeshouse, G . A., Goldstein, R. B. and Abeshouse, B. S . (1959). Adrenal cysts: review of literature and report of three cases. J. Urol., 81.71 1-719. Barnett, L. (1943). Hydatid cysts: their location in variousorgans and tissues of the body. Ausr. N.Z. J . Surg., 12,240445. Brindley, G. V., Jr. and Chisholm, J. T. (195 1). Cystic turnours of
515
ADRENAL CYSTS:DIAGNOSIS AND MANAGEMENT the adrenal gland associated with Cushing’s syndrome. Texas J. Med., 47,234-236. Cerney, J. C., Warhawsky, A., Hall, J. er d (1970). The preoperative diagnosis of adrenal cysts. J. Urol.. 104, 787789.
Cheema, P., Cartagena, R. and Staubitz, W. (1981). Adrenal cysts: diagnosis and treatment. J. Urol., 126,396-399. Copeland, P. M. (1983). The incidentally discovered adrenal mass. Ann. Intern. Med., 98, 940-945. Ellis, F. H., Dave, C. J. and Clagett, 0. T. (1952). Cysts of the adrenal glands. Ann. Surg.. 136,217-219. El-Muhtaseb, H. H. (1984). Biliary complications of hepatic hydatid disease. J . R . Coil. Surg. Edinb., 29, 29G300. Esquivel, E. and Grabstald, H. (1965). Giant adrenal cyst. J. Urol..94,635-638. Foster, D. G. (1966). Adrenal cysts. Review of literature and report of case. Arch. Surg., 92, 131-143. Gordon, S. K., Friedell, G. H., Jankleson, 0. M. et d (1960). Cystic hamartoma of the adrenal gland: a case report. Boston Med. Quart., 11, 84-85. Greiselius (1670). Cited by Doran, A. H . G . (1908). Cystic tumour of suprarenal gland, successfully removed by operation. Br. Med. J., 1, 1558-1559. Hodges, F. V. and Ellis, F. R. (1958). Cystic lesions of the adrenal gland. Arch. Pathol.. 66,53-58. Kuhn, J., Jewett, T. and Munschauer, R. (1971). The clinical and radiographic features of massive neonatal adrenal hemorrhage. Radiology, 99,647-650. Levison, P. H. (1933). A case of bilateral adrenal cysts. Endocrinology..17,372-376. Mikail, M. and Kirshman, A. (1968). Adrenal cysts: report of 3 cases and review of the literature. J . Urol..99, 361-365. Moore, F. P. and Cermak, E. G. (1950). Adrenal cyst and adrenal insufficiency in an infant with fatal complication. J . Pediutr., 36.91-95.
Palubmska, A. J., Cristensen, W. R., Harrison, J. H. et d.(1 959). Calcified adrenal cysts. Am. J . Roentgenol., 82,853-855. Pasciak, R. M. and Cook, W. A. (1988). Massive retroperitoneal hemorrhage owing to a ruptured adrenal cyst. J . Urol., 139, 98- 100.
PIaut, A. (1962). Locally invasive lymphangioma of adrenal gland. Cancer, 15, 1165-1 166. Schmieden,N. and Peiper, H. Cited in Moore, F. P. and Cermak, E. G . (1950). Adrenal cyst and adrenal insufficiency in an infant with fatal complication. J . Pediatr.. 36.91, Stewart, J. 0.R. and Lester, J. G. (1962). Ruptured adrenal cyst. Postgrad. Med. J.,38, 709-7 I 1. Tehrani, M., Wolgin, W. and Niedwich, A. (1965). Adrenal cyst: report of 2 cases. J. Urol.. 93,427-429. Wahl,H. R. (1951). Adrenalcysts. Am. J . Pathol., 27, 758-761. Wood, D. E., Delbridge, L. and Reeve, T. S. (1987). Surgery for adrenal tumours: is operation for small incidental tumour appropriate? Aust. N.Z. J . Surg.. 51,739-741.
The Authors A. S. Sroujieh, FRCS, Associate Professor, University of
Jordan; Consultant General and Endocrine Surgeon, Jordan University Hospital. G . R. Farah, FRCS, Associate Professor, University of Jordan; Consultant General and Oncological Surgeon, Jordan University Hospital. M. J. Haddad, FRCS, Assistant Professor, University of Jordan; Consultant Paediatric Surgeon, Jordan University Hospital. M. M. Abu-Khalaf, FACS, Professor, University of Jordan; Consultant General and Gastroenterological Surgeon, Jordan University Hospital. Requests for reprints to: A. S. Sroujieh, P.0. Box 910232, Jabal Webdeh, Amman, Jordan.
8 1990 British Journal of Urology
Adrenal Cysts: Diagnosis and Management A. S. SROUJIEH, G. R. FARAH, M. J. HADDAD and M. M. ABU-KHALAF Department of Surgery, University of Jordan and the Surgical Services of Jordan University Hospital, Amman, Jordan
Summary-We present 7 patients (5 adult females and 2 neonate males) with adrenal cysts. The cysts included 1 hydatid, 1 lymphatic and 5 pseudocysts. Three cysts were diagnosed preoperatively and all were resected surgically. The existence of true epithelial cysts of the adrenal gland is doubted by many authors; the present series includes most types of adrenal cyst.
Adrenal cysts are a rare occurrence. Greiselius reported the first case in 1670. Scattered case reports appeared until 1959, when Abeshouse etal. reviewed 155 cases from the literature, including 3 of their own. The total number of cases reviewed by Foster (1966) rose to 220, including 1 of his own. With the advent of computed tomography (CT) and ultrasound (US) scans (Copeland, 1983), more cysts are being discovered incidentally. We describe 7 types of adrenal cyst treated surgically at Jordan University Hospital between 1977 and 1989.
Patients and Methods The records of 27 patients who underwent adrenalectomy between June 1977 and June 1989 were reviewed. Adrenal cyst was the indication for adrenalectomy in 7 patients (Table). They included 2 neonate males (6 and 12 days old) and 5 adult females aged between 40 and 78 years. Three cysts were discovered incidentally: 2 neonates were found to have right upper abdominal masses during investigation for sepsis and jaundice which proved to be physiological, and in a 60-year-old female the cyst was found during US studies for gallbladder disease; a 6-day-old neonate had a history of difficult vaginal delivery. Four patients were symptomatic and had upper abdominal and loin pain. One had had a left nephrectomy for calculi several years earlier and another had undergone previous surgery for retroperitoneal hydatid disease with a Accepted for publication 11 October 1989
discharging sinus in the left iliac fossa (LIF). An abdominal mass was felt in a 78-year-old female and hepatomegaly was detected in a 40-year-old female. Laboratory tests showed leukocytosis in a patient with recurrent infected hydatid disease and an erythrocyte sedimentation rate (ESR) above 100 mm in the 78-year-old female, who proved later to have a malignant cyst. Serum electrolytes were normal and hormonal studies, requested in 3 patients, were also normal, Casoni’s test and echinococcus antibody titres were evaluated in 3 patients and were positive in 1. Radiological studies showed peripheral curvilinear calcification (Fig. 1) in a 44-year-old female at the site of previous nephrectomy. Two patients had stippled calcification in the right hypochondrium ; a neonate also had a non-functioning right kidney and a 78-year-old had, in addition, distorted upper calices in the right kidney and absence of neovascularisation on renal arteriography (Fig. 2 ) . US and CT scans were done in 5 patients and all showed cystic retroperitoneal swellings. A pre-operative diagnosis of adrenal cyst was made in 3 cases: (1) in a patient with recurrent hydatid disease the tract of a discharging sinus from the LIF extended upwards behind the left kidney to a left adrenal cyst (Fig. 3 ) ; (2) in a neonate, new hyperdense areas were seen on a repeat ultrasound which rightly suggested a haemorrhagic cyst (Fig. 4); ( 3 ) a 60-year-old female was found to have a right adrenal cyst during US studies of the gallbladder. In the other 4 patients
570
571
ADRENAL CYSTS:DIAGNOSIS AND MANAGEMENT
Table Adrenal Cysts Year Age
Sex
Presentation
Imaging studies
Final diagnosis
1977 44years
F
Abdominal and loin pain L. nephrectomy
Adrenal pseudocyst
1980 12days
M
Sepsis. Abdominal mass
1983 78years
F
Abdominal and loin pain. Abdominal mass. ESR > 100
1988 40years
F
1988 6days
M
1989 50years
F
1989 60years
F
Abdominal and loin pain. Hepatomegaly Physiologicaljaundice. Abdominal mass Discharging sinus LIF. Previous surgery for hydatid cyst Gallbladder disease
Retroperitoneal cyst with curvilinear calcification. ? Hydatid cyst Stippled calcification. Non-functioning R. kidney. ? Neuroblastoma Stippled calcification. Distorted upper calices. No neovascularisation. ? Renal cyst US and CT: ? liver hydatid cyst
Adrenal pseudocyst due to infarction Cystic cortical carcinoma
Adrenal pseudocyst
US and CT: adrenal mass. Repeat US : haemorrhagic cyst CT: adrenal cyst with communicating tract to LIF
Haemorrhagic adrenal cyst Hydatid cyst
US: gall stones and adrenal cyst
Lymphatic cyst
the diagnosis was made at operation. Two cysts were diagnosed pre-operatively as hydatid cysts. The 78-year-old patient was thought to have a renal cyst due to distorted upper calices; she had mixed echoes on US, stippled calcification and a high ESR, yet the malignant nature of the cyst was missed due to absence of neovascularisation on the arteriogram. The fourth patient, a 12-day-old infant, was diagnosed as having a neuroblastoma. All patients underwent surgery through abdominal incisions. Adrenalectomy was achieved easily
Fig. I X-ray abdomen showing peripheral curvilinear calcification in left adrenal cyst.
in 4 cases. Additional nephrectomy was done in a neonate with an adherent non-functioning right kidney; splenectomy was indicated during excision of a hydatid cyst of the left adrenal gland. Excision of an adrenal cyst, proved to be malignant at operation, was followed by right nephrectomy and excision of a segment of inferior vena cava with reimplantation of the left renal vein into a dacron graft.
Results Pathologically, all cysts proved to be adrenal; 5 were on the right and 2 on the left. They included a hydatid cyst, a lymphatic cyst and 5 pseudocysts. In the latter group were a cyst due to degeneration in an adrenal cortical carcinoma, 2 cysts thought to be due to previous trauma following left nephrectomy in a 40-year-old female and after difficult delivery in a neonate; the fourth pseudocyst developed in an infarcted adrenal which was accompanied by evidence of infarction in the adjacent kidney, and the cause of the fifth cyst was not known. One neonate died soon after surgery from uncontrolled sepsis. The 78-year-old female with a malignant cyst developed deep venous thrombosis and repeated wound infection and died from sepsis 6 months after surgery. The other 5 patients recovered well and are symptom-free 3 months to 12 years post-operatively.
572
BRITISH JOURNAL OF UROLOGY
Fig. 2 (A) Right renal arteriograrn with absent neovascularisation above kidney. Note scattered calcification. ( B ) IVU showing right kidney pushed downwards with distorted upper calices.
Discussion Adrenal cysts are rare and their incidence in autopsy studies ranges from 0.064% (Wahl, 1951) to 0.18% (Hodges and Ellis, 1958). Copeland (1983) found 5 cysts in 51 patients with adrenal masses discovered incidentally on CT scan. Adrenal cysts occur at all ages, from birth to 80 years, with a peak in the third to sixth decades (Abeshouse et al., 1959; Esquivel and Grabstald, 1965;Tehrani et a[., 1965; Foster, 1966; Cerneyet af., 1970). The female to male ratio is 2 : l in some reports (Abeshouse el al., 1959; Cerney et af., 1970), inconclusive or equal inothers(Foster, 1966; Mikailand Kirshman, 1968). The present series included 2 neonate males and 5 adult females and their ages ranged from 6 days to 78 years. Adrenal cysts are usually asymptomatic and many are discovered at autopsy (Wahl, 1951; Hodgeset a/., 1958; Abeshouse et a/., 1959; Foster, 1966). They seldom cause adrenal hyk -function in infants (Moore and Cermak, 1950), iushing’s syndrome (Brindley and Chisholm, 1951) or phaeochromocytoma (Ellis et al., 1952; Cheema et af., 1981). A correct pre-operative diagnosis was made in 3.2 to 7.2% of patients (Abeshouse et al., 1959;
Foster, 1966). Symptoms related to adrenal cysts include vague upper abdominal or loin pain, gastrointestinal upsets and abdominal mass. Acute abdomen and/or a tender mass occur occasionally when intracystic haemorrhage, rupture or infection occur (Stewart and Lester, 1962; Foster, 1966; Pasciak and Cook, 1988). The cyst may fill the abdomen to such an extent that it is diagnosed as an ovarian cyst (Foster, 1966). Three ofour patients were correctly diagnosed pre-operatively and 4 were diagnosed at surgery. Adrenal cysts were symptomatic in 4 of our adult patients who had abdominal and loin pain with acute exacerbations in a patient with a discharging sinus from an infected hydatid cyst. An abdominal mass was palpated in the 78-year-old female and incidentally in the 2 neonates, who were initially thought to have a neuroblastoma. A soft tissue swelling with curvilinear calcification is seen occasionally on plain abdominal films (Levison, 1933; Palubinska et al., 1959; Gordon et al., 1960); it denotes a cystic lesion rather than a neoplasm when the calcification is stippled (Foster, 1966; Cerney et al., 1970), as in our elderly patient, although such calcification is present in organised haematoma (Cheema et al., 1981 ; Copeland. 1983)
573
ADRENAL CYSTS:DIAGNOSIS A N D MANAGEMENT
Fig. 3 CT scan showing hydatid cyst in left adrenal gland (A) extending behind the left kidney (B) forming a tract (C and D)
or infarct, as in one of our neonate patients. IVU usually showsdownward displacement and rotation of the related kidney, which occasionally has distorted upper calices (Foster, 1966; Cerney et al., 1970). Barium studies of the gastrointestinal tract may show displacement of stomach and colon
anteriorly, downwards, and to the other side due to a large cyst (Abeshouse et al., 1959; Esquivel and Grabstald, 1965; Foster, 1966; Cerney et al., 1970). Neovascularisation on angiography suggests malignancy, although it is sometimes absent (Copeland, 1983). The absence of neovascularisation and the
Fig. 4 (A) US showing echogenic adrenal mass above right kidney (M). (B) US showing development of new hyperdense areas ( + ) in the right adrenal gland reported as new haemorrhages.
574 distortion in the upper calices of the right kidney led us to believe that our elderly patient had a renal cyst. US and CT scans are useful diagnostic tools and many adrenal masses are discovered incidentally (Copeland, 1983; Wood etal., 1987). These studies were positive in 5 of our patients. There were mixed echoes in the 78-year-old female which, together with the high ESR, should have alerted us to the possibility of malignancy. US was also very useful when it was repeated and showed the development of new hyperdense areas in the adrenal gland leading to the correct diagnosis of a haemorrhagic cyst in the 6-day-old neonate. Complete blood counts may show low haemoglobin and haematocrit levels if there is heavy intracystic bleeding (Pasciak and Cook, 1988), leukocytosis in infected cysts (Foster, 1966), as in our infected hydatid cyst, eosinophilia in parasitic cysts and a high ESR in malignant cysts, as in our elderly patient. Electrolytes and hormonal studies are usually normal except on the rare occasion where hypoadrenocorticism is present (Moore and Cermak, 1950), or pseudocysts in functioning tumours are the cause (Brindley and Chisholm, 1951 ; Ellis et al., 1952). Foster (1 966) divided adrenal cysts into 4 groups : (1) parasitic (echinococcal) cysts (7%); (2) epithelial cysts (973, including true glandular, embryonal cysts and cystic adenomas; (3) endothelial cysts (4573, including lymphatic cysts, lymphangiomas and haemangiomas ; (4) pseudocysts (39%) due to infarction or haemorrhage in the adrenal gland, or cystic degeneration in benign or malignant adrenal neoplasms. The existence of true epithelial cysts is doubted by many authors (Foster, 1966). Thus this study, although small, presents most types of adrenal cyst. Hydatid disease is endemic in Jordan (El-Muhtaseb, 1984) and our case represents a single occurrence of hydatid cyst in the adrenal gland in 250 (0.4%) hydatid cysts in various organs treated at this hospital. The incidence is therefore similar to the 0.5% reported by Barnett (1943). All cysts in our patients were unilateral (4 on the right side and 2 on the left). However, adrenal cysts are seldom bilateral (Levison, 1933; Moore and Cermak, 1950) and they have no predilection for either side (Abeshouse et a f . , 1959; Foster, 1966), except perhaps in adrenal haemorrhage in neonates, which is more common on the right side, possibly because of the susceptibility of the adrenal gland to compression between the liver and spine due to birth trauma (Kuhn et al., 1971); this may have occurred in 1 of our neonates who had a difficult delivery.
BRITISH JOURNAL OF UROLOGY
The treatment of adrenal cysts (Abeshouse et al., 1959; Foster, 1966) depends on the symptoms and size of the cyst, the occurrence of complications and the underlying pathology. Under the guidance of CT and US scans, asymptomatic non-functioning cysts can now be aspirated percutaneously (Copeland, 1983; Wood etal., 1987). Indications for surgery include large and complicated cysts, parasitic, functioning and malignant cysts (Abeshouse et al., 1959; Foster, 1966). Malignant cysts are rare (Schmieden and Peiper, 1950; Brindley and Chisholm, 1951 ; Plaut, 1962) and may be distinguished by a high ESR, aspiration cytology, neovascularisation on arteriography (Copeland, 1983) and the presence of mixed echoes on ultrasound. Excision of adrenal cysts in patients who do not have a phaeochromocytoma is reported to bring the blood pressure back to normal and may be taken into consideration (Foster, 1966; Cheema, et al., 1981). Surgical excision can usually be achieved through an abdominal or a loin incision (Foster, 1966) and occasionally by a thoraco-abdominal approach (Pasciak and Cook, 1988). All of our patients were treated surgically through abdominal or flank incisions. In 4 cases the cysts were easily excised. Nephrectomy was required in 2 patients, splenectomy in 1 and excision of a segment of inferior vena cava in a third because of adhesions or direct invasion. Five patients are alive and well and 2 died of sepsis. Adrenal cysts are seen occasionally by the surgeon and urologist. With the advent of better imaging techniques such as US and CT scans and magnetic resonance imaging, the diagnosis of adrenal cysts is possible pre-operatively and this was evident in our last 3 patients (Table). The distinction between malignant and benign adrenal cysts can still be difficult and underlying carcinoma should be suspected in patients with a high ESR, mixed echoes on ultrasound, stippled calcification on plain radiography and neovascularisation on arteriography. It should be emphasised that the last 2 radiological signs may be misleading and it is hoped that aspiration cytology will differentiate more accurately between benign and malignant cysts.
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Cheema, P., Cartagena, R. and Staubitz, W. (1981). Adrenal cysts: diagnosis and treatment. J. Urol., 126,396-399. Copeland, P. M. (1983). The incidentally discovered adrenal mass. Ann. Intern. Med., 98, 940-945. Ellis, F. H., Dave, C. J. and Clagett, 0. T. (1952). Cysts of the adrenal glands. Ann. Surg.. 136,217-219. El-Muhtaseb, H. H. (1984). Biliary complications of hepatic hydatid disease. J . R . Coil. Surg. Edinb., 29, 29G300. Esquivel, E. and Grabstald, H. (1965). Giant adrenal cyst. J. Urol..94,635-638. Foster, D. G. (1966). Adrenal cysts. Review of literature and report of case. Arch. Surg., 92, 131-143. Gordon, S. K., Friedell, G. H., Jankleson, 0. M. et d (1960). Cystic hamartoma of the adrenal gland: a case report. Boston Med. Quart., 11, 84-85. Greiselius (1670). Cited by Doran, A. H . G . (1908). Cystic tumour of suprarenal gland, successfully removed by operation. Br. Med. J., 1, 1558-1559. Hodges, F. V. and Ellis, F. R. (1958). Cystic lesions of the adrenal gland. Arch. Pathol.. 66,53-58. Kuhn, J., Jewett, T. and Munschauer, R. (1971). The clinical and radiographic features of massive neonatal adrenal hemorrhage. Radiology, 99,647-650. Levison, P. H. (1933). A case of bilateral adrenal cysts. Endocrinology..17,372-376. Mikail, M. and Kirshman, A. (1968). Adrenal cysts: report of 3 cases and review of the literature. J . Urol..99, 361-365. Moore, F. P. and Cermak, E. G. (1950). Adrenal cyst and adrenal insufficiency in an infant with fatal complication. J . Pediutr., 36.91-95.
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PIaut, A. (1962). Locally invasive lymphangioma of adrenal gland. Cancer, 15, 1165-1 166. Schmieden,N. and Peiper, H. Cited in Moore, F. P. and Cermak, E. G . (1950). Adrenal cyst and adrenal insufficiency in an infant with fatal complication. J . Pediatr.. 36.91, Stewart, J. 0.R. and Lester, J. G. (1962). Ruptured adrenal cyst. Postgrad. Med. J.,38, 709-7 I 1. Tehrani, M., Wolgin, W. and Niedwich, A. (1965). Adrenal cyst: report of 2 cases. J. Urol.. 93,427-429. Wahl,H. R. (1951). Adrenalcysts. Am. J . Pathol., 27, 758-761. Wood, D. E., Delbridge, L. and Reeve, T. S. (1987). Surgery for adrenal tumours: is operation for small incidental tumour appropriate? Aust. N.Z. J . Surg.. 51,739-741.
The Authors A. S. Sroujieh, FRCS, Associate Professor, University of
Jordan; Consultant General and Endocrine Surgeon, Jordan University Hospital. G . R. Farah, FRCS, Associate Professor, University of Jordan; Consultant General and Oncological Surgeon, Jordan University Hospital. M. J. Haddad, FRCS, Assistant Professor, University of Jordan; Consultant Paediatric Surgeon, Jordan University Hospital. M. M. Abu-Khalaf, FACS, Professor, University of Jordan; Consultant General and Gastroenterological Surgeon, Jordan University Hospital. Requests for reprints to: A. S. Sroujieh, P.0. Box 910232, Jabal Webdeh, Amman, Jordan.
