ELSEVIER
Original contribution
Cystic lesions of the adrenal gland: our experience over the last 20 years
Christopher Sebastiano MD, Xiangrong Zhao MD, PhD*,1, Fang-Ming Deng MD, PHD, Kasturi Das MD2
Department of Pathology, New York University Langone Medical Center, New York, NY 10016, USA
Received 2 November 2012; revised 6 February 2013; accepted 8 February 2013
Keywords:
Adrenal cyst; Pseudocyst; Endothelial cyst; Epithelial cyst; Radiopathologic correlation
Summary Cystic lesions of the adrenal gland are uncommon, often presenting with nonspecific clinical and radiologic findings, and are thus underrecognized. They are occasionally associated with malignant neoplasms, which can greatly mimic benign lesions and carry detrimental clinical consequences if misdiagnosed. Here we present our 20-year experience (1992-2012) with these lesions at an academic medical center. Among more than 4500 adrenal gland specimens, 31 cases of adrenal lesions with a predominant cystic component were identified in 30 patients with an age range of 34 to 86 years (median, 55.5 years) and a male/female ratio of 13:17. Macroscopic descriptions, available histologic and immu- nostain slides, and available radiologic records were reviewed for all included cases. Radiologic studies and gross examination correlated well, and hemorrhage (26 cases; 84%) and encapsulation (25 cases; 81%) appeared to be nonspecific radiologic/gross features shared across histologic subtypes. Microscopic review identified 12 cases (39%) of pseudocysts, 2 cases (6%) of endothelium-derived cysts, and 17 cases (55%) of epithelium-derived cysts. Among these 31 cystic adrenal lesions, 2 cases (6%) were malignant neoplasms (1 epithelioid angiosarcoma, 1 adrenocortical carcinoma). Radiologic impression and histo- pathologic diagnosis were concordant in 11 (73%) of the 15 cases for which radiologic records were available. This study represents the second largest case series to date on cystic adrenal lesions and presents a comprehensive review on their demographic, clinical, radiologic, and gross and microscopic pathologic features, as well as their differential diagnoses. Published by Elsevier Inc.
1. Introduction
Cystic lesions of the adrenal gland are uncommon. The first case of adrenal cyst was reported in 1670 [1], and the second case in 1837 [1]. To date, there are only a few case series on cystic adrenal lesions, with generally small numbers
of cases [2-9]. These include 8 cases by Medeiros et al [2], 12 cases by Bellantone et al [3], 15 cases by Erbil et al [4], 25 cases by Chien et al [5], and 28 cases by Major et al [6]. The largest case series included 41 cases identified at a single institute (1973-1998) [7]. The true incidence of adrenal cysts may be higher because small lesions are often clinically silent and therefore likely to be underestimated in studies based on medically explored cases. The currently well-accepted classification criteria, first proposed in 1961 [8] and then fine tuned in 1966 [9], subtype adrenal cysts as pseudocysts, endothelial cysts, epithelial cysts, and parasitic cysts.
Cystic lesions of the adrenal gland often present with nonspecific clinical and radiologic findings and are thus
# Disclosure: The authors report no potential conflicts of interest.
* Corresponding author. E-mail address: xiangrong.zhao@nih.gov (X. Zhao).
1 Current address: Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD.
2 Current address: Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison, WI.
underrecognized. They are occasionally associated with malignant neoplasms, which can greatly mimic benign lesions and, if misdiagnosed, carry detrimental clinical con- sequences. A review is therefore desired on their demo- graphic, clinical, radiologic, and gross and microscopic pathologic features, as well as their differential diagnoses. Here we present our 20-year experience (1992-2012) with these lesions at an academic medical center.
2. Materials and methods
An extensive database search of all surgical specimens evaluated in the Department of Pathology was conducted for cystic adrenal lesions identified from 1992 to 2012 at New York University Langone Medical Center. Approval from the institutional review board was obtained. Our search criteria included the keyword “cystic” in the diagnosis and/or macroscopic description of all partial and radical adrenalec- tomies for any reason, adrenal gland resections as a part of more comprehensive procedures, and adrenal glands dis- sected at autopsies. Adrenal masses exhibiting only a minor cystic component were excluded.
Formalin-fixed, paraffin-embedded tissue blocks from 31 qualified cases were on file. All selected cases were documented macroscopically through photographs and pathology and/or surgical reports, as well as by microscopic review. Computed topography (CT) and magnetic reso- nance imaging (MRI) studies were reviewed for all cases up to and including 2002; records for prior cases were not readily available. Hematoxylin and eosin-stained sections and immunohistochemical stains were available and reviewed for 17 cases, which were within the 10-year sur- gical pathology slide-retention period per policy of the College of American Pathologists; original diagnoses were verified for all of these cases.
3. Results
Search of databases containing more than a half million surgical pathology and autopsy cases (1992-2012), including more than 4500 adrenal gland specimens microscopically examined, identified a total of 31 cases in 30 patients of adrenal lesions exhibiting a significant cystic component. The demographic, radiologic, gross, and microscopic find- ings of these 31 cases are summarized in the Table. The patient population included 13 men and 17 women. Age at diagnosis ranged from 34 to 86 years, with a mean age of 57.8 years and a median of 55.5 years.
In our case series, 12 cases (12/31; 39%) were pseu- docysts without any inner wall lining or associated neoplastic tissue; 2 (2/31; 6%) were endothelium derived, including one benign endothelial cyst and one cystic epi- thelioid angiosarcoma; and 17 (17/31; 55%) were epithe- lium/neuroectoderm-derived cystic neoplasms. Of these
17 epithelium/neuroectoderm-derived cystic neoplasms, 1 (1/17; 6%) was malignant (adrenocortical carcinoma) and the other 16 (16/17; 94%) were benign pheochromocyto- mas, 2 of which were bilateral lesions in the same patient. Overall, 2 cases (2/31; 6.4%) were malignant neoplasms (1 epithelioid angiosarcoma, 1 adrenocortical carcinoma); the remaining 29 (29/31; 93.6%) were benign lesions.
The 12 adrenal pseudocysts (Fig. A and B) ranged from 0.9 to 17.0 cm in greatest dimensions and demonstrated typical histomorphology; all were unilocular and encapsu- lated, devoid of inner wall lining. Three of the 5 (60%) cases available for microscopic review exhibited calcification within their fibrous capsules. The cyst contents were pre- dominantly proteinaceous materials and blood clots. All cases showed areas of organization with prominent, irre- gular, dilated, thin-walled vascular channels. Necrotic neo- plastic cells or nuclear debris were not present, nor was any solid component identified. The adjacent adrenal tissue exhibited no significant abnormality.
In the subgroup of endothelium-derived cysts, the single benign endothelial cyst exhibited a central 2.8-cm cystic cavity with no solid mass lesion, lined by flattened, bland endothelial cells, and partially filled with fresh blood without necrosis or calcification.
The single case of primary adrenal angiosarcoma (Fig. C- E) exhibited a 13.7-cm well-circumscribed, encapsulated cyst. Grossly, no distinct adrenal gland was discernible, and the cut surface was predominantly hemorrhagic. The cyst wall was thick up to 0.5 cm and focally calcified. The peri- phery of the cyst exhibited focal spongy, dark red areas. The initial impression on radiologic studies and macroscopic examination was a pseudocyst with extensive hemorrhage, or an organizing hematoma. Microscopic examination dem- onstrated thick fibrous capsule with residual uninvolved adrenal tissue compressed to the periphery. The cyst contents consisted of predominantly organizing blood clot; dilated vascular-type channels were identified, many of which were devoid of any lining, whereas a few were more complex, with an interconnecting sinusoidal pattern and lined by large atypical cells. Necrosis was present. Subsequent extensive sectioning from the peripheral spongy areas revealed solid sheets and nests of large, highly pleomorphic epithelioid cells with abundant amphophilic cytoplasm, irregular vesicular nuclei, and prominent central nucleoli. These cells stained uniformly, strongly positive with the endothelial cell markers CD31 and CD34 and were completely negative for inhibin, melan-A, and synaptophysin. Only rare mitotic figures (<2/50 high-power fields) were present; however, the proli- feration index indicated by Ki-67 expression level was more than 90% in these large epithelioid cells.
In the subgroup of epithelium/neuroectoderm-derived cysts, the single case of cystic adrenocortical carcinoma was almost entirely necrotic, with benign adrenal tissue com- pressed to the periphery. The residual viable neoplastic cells, although scant, were significantly atypical. No calcifications were seen.
| Age (y) | Sex | Final diagnosis | Additional histopathologic findings | Size | (cm) | C/M/D | Hemorrhagic | Encapsulated | CT/MRI impression |
|---|---|---|---|---|---|---|---|---|---|
| 52 | F | Pseudocyst | 6.8 | C | Y | Y | Cyst | ||
| 61 | M | Pseudocyst | 7.8 | C | Y | Y | Cyst | ||
| 56 | F | Pseudocyst | 2.0 | C | Y | Y | Adenoma | ||
| 53 | F | Pseudocyst | 17.0 | C | N | Y | Cyst | ||
| 52 | M | Pseudocyst | 0.9 | C | Y | Y | Pheo vs hemangioma | ||
| 55 | F | Pseudocyst | 7.0 | C | Y | Y | ACC vs Pheo | ||
| 53 | F | Pseudocyst | Ipsilateral renal oncocytoma | 4.5 | C | N | Y | NA | |
| 46 | M | Pseudocyst | 7.0 | C | N | Y | Cyst | ||
| 80 | M | Pseudocyst | 7.0 | C | Y | Y | NA | ||
| 34 | F | Pseudocyst | 10.0 | C | N | Y | NA | ||
| 36 | M | Pseudocyst | 5.0 | C | Y | Y | NA | ||
| 86 | F | Pseudocyst | 8.0 | C | Y | Y | NA | ||
| 74 | F | Endothelial cyst | Cortical hyperplasia | 9.3 | C | Y | N | Pseudocyst | |
| 62 | F | Epithelioid | Extensive hemorrhage and necrosis | 13.7 | C | Y | Y | ACC | |
| angiosarcoma | |||||||||
| 59 | F | ACC | Extensive necrosis | 4.0 | C | N | N | NA | |
| 76 | F | Pheo | Focal capsular invasion | 16.6 | D | Y | Y | Pheo | |
| 49 | F | Pheo | Cytologic atypia | 2.5 | M | Y | Y | Pheo | |
| 71 | M | Pheo | Focal capsular invasion | 8.0 | C | Y | Y | ACC vs pheo | |
| 52 | M | Pheo (right) | Focal extraadrenal extension | 2.3 | M | Y | N | ACC vs pheo vs | |
| Pheo (left) | 3.7 | M | Y | N | metastasis | ||||
| 68 | M | Pheo | Focal necrosis | 8.5 | C | Y | N | Pheo | |
| 63 | M | Pheo | Central necrosis | 4.8 | M | Y | N | NA | |
| 68 | F | Pheo | 4.5 | D | Y | Y | NA | ||
| 53 | F | Pheo | 5.0 | C | Y | Y | NA | ||
| 53 | M | Pheo | 7.0 | M | Y | Y | NA | ||
| 46 | M | Pheo | Multinodular | 2.5 | D | Y | Y | NA | |
| 77 | M | Pheo | Adrenal cortical invasion | 4.2 | M | Y | Y | NA | |
| 43 | M | Pheo | Focal capsular invasion | 6.0 | C | Y | Y | NA | |
| 56 | F | Pheo | 5.2 | M | Y | Y | NA | ||
| 46 | F | Pheo | Focal capsular invasion | 5.0 | M | Y | Y | NA | |
| 53 | F | Pheo | Focal capsular invasion | 12.0 | C | Y | Y | NA |
Abbreviations: C/M/D, predominantly cystic/mixed solid and cystic components/significant cystic degeneration; F, female; Y, yes; M, male; N, no; Pheo, pheochromocytoma; ACC, adrenocortical carcinoma; NA, not available.
The 16 cases of benign cystic pheochromocytoma (Fig. F-H) ranged from 2.5 to 16.0 cm in greatest dim- ensions and consisted of nested proliferations of granular, basophilic or amphophilic cells with variably sized nuclei and prominent nucleoli. All of these 16 cases exhibited adjacent compressed, benign adrenal tissue. Fourteen lesions (14/16; 88%) were encapsulated, and 2 lesions (2/16; 12%) had neoplastic tissue focally in confluence with adjacent benign adrenal tissue. Five cases (5/16; 31%) demonstrated focal capsular invasion, and 1 (1/16; 6%) exhibited extra- adrenal extension into the surrounding adipose tissue. Of note, all of the identified cyst capsules were thin, ranging from 0.1 to 0.3 cm in thickness.
Radiologic records were available for review for cases up to and including 2002. Overall, radiologic studies and gross examination correlated well: 20 (20/31; 64%) of these lesions were almost entirely cystic, 8 (8/31; 26%) exhibited accom- panying solid components, and the remaining 3 (3/31; 10%) had significant cystic degeneration. In addition, hemorrhage
(26 cases; 84%) and encapsulation (25 cases; 81%) appeared to be nonspecific radiologic/gross features shared across histo- logic subtypes. Radiologic impression and histopathologic diagnosis were concordant in 11 (73%) of the 15 cases for which radiologic records were available; no discordant cases changed status from benign to malignant or vice versa. This study, from a single institute, greatly expands the database of cystic adrenal lesions and represents the second largest case series on these lesions ever reported worldwide.
4. Discussion
4.1. Adrenal cysts consist of a heterogeneous group of uncommon entities
Adrenal cysts, when growing large in size, may present with mass effect displacing adjacent abdominal structures, resulting in gastrointestinal symptoms, abdominal and/or
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flank pain, or palpable mass. The first adrenal cyst in literature was described by Viennese anatomist Greiselius in 1670, as the cause of death due to rupture of an adrenal cyst of greater than 4 kg filled with fluid and blood clots [1]. Since then, more than 600 cystic adrenal lesions had been reported in medical literature during the past ~350 years. Overall, these lesions remain uncommon, with incidence in autopsy studies ranging from 0.064% to 0.18% [7,10].
Adrenal cysts can be associated with a variety of disease entities including polycystic renal disease [11], Beckwith- Wiedemann syndrome [12], Klippel-Trenaunay-Weber syndrome [13], abdominal neuroblastomas [14], aortic aneu- rysm [15], shewannomas [16], and, rarely, with pregnancy [17]. Metastases from a variety of anatomic sites, particu- larly the breast, have been reported to form cystic masses in the adrenal gland and therefore need to be considered as differential diagnosis of a cystic adrenal mass when clinically indicated [18].
Nowadays, cystic lesions of the adrenal gland are more often discovered incidentally by radiologic studies or sur- gery for other reasons, or at the time of autopsy. In a review of the literature of 613 cases of adrenal cysts compiled from both historical and modern radiology eras, 34% of those cases presented incidentally [19]. The increasing use of advanced cross-sectional imaging studies has resulted in an increasing frequency with which nonsymptomatic cystic adrenal lesions are found. Of note, lack of symptoms or nonfunctionality does not necessarily indicate a benign nature of a cystic adrenal lesion. On the other hand, malig- nancy is infrequently associated with cystic adrenal lesions, with 7% of those 613 cases analyzed in the review men- tioned previously [19] being malignant or potentially malig- nant. Our case series showed similar rate of malignancy, with 2 cases (2/31; 6.4%) diagnosed as malignant neo- plasms, including 1 epithelioid angiosarcoma and 1 adreno- cortical carcinoma.
Cystic adrenal lesions, in general, exhibit a broad histo- morphologic spectrum. Classification of these lesions was first attempted in 1906 by Terrier and Lecène [20], who categorized them into hemorrhagic cysts, endothelial cysts, congenital retention cysts, cystic adenomas, and parasitic
cysts. In 1959, Abershause et al [21] reclassified adrenal cysts based on 155 compiled cases. Further modifications on classification were proposed, and the one proposed by Barron and Emanuel [8] in 1961 has been widely accepted: adrenal cysts include 4 subtypes as pseudocysts, endothelial cysts, epithelial cysts, and parasitic cysts. In 1966, Foster [9] fine tuned the classification criteria and evaluated the frequencies in 220 compiled cases: pseudocysts (39%), endothelial cysts (45%), epithelial cysts (9%), and parasitic cysts (7%).
4.2. The relative frequencies of histologic subtypes vary among studies per case compositions and categorization criteria
Although pseudocysts represent the most frequently identified adrenal cysts in surgical series [7,21], endothelial cysts are more common in autopsy series where they account for up to 45% of adrenal cysts. The overall frequency of endothelial cysts among adrenal cysts in surgical series approximates 9% [7,21]. Both endothelial cysts and pseu- docysts are considered variants of adrenal vascular cysts, based on immunohistochemical and ultrastructural evidence. The nonvascular adrenal cysts include epithelial cysts and parasitic cysts. Epithelial cysts have 2 subtypes: true glan- dular and embryonal. In some case series including ours, epithelium-derived cystic adrenal neoplasms were placed into this category [7,21]. Functioning cysts originating from the adrenal cortex and/or medulla have been described [7,22,23]. Parasitic cysts are usually discovered incidentally at autopsy and represent approximately 7% of adrenal cysts [7,21]. Overall, most reported adrenal cysts are unilateral without side predominance, whereas bilaterality has been reported with a prevalence of 8% to 15% [24]. In our case series, 2 (6.4%) of 31 cases represented bilateral lesions from the same patient.
The discrepancies in the relative frequencies of histologic subtypes among various studies may reflect the difference in their case compositions, that is, cystic adrenal lesions iden- tified at autopsy versus in surgically excised specimens. The
Fig. Cystic lesions of the adrenal gland. A and B, Adrenal pseudocyst. A, CT with contrast of the abdomen and pelvis was conducted for a 52-year-old woman presenting with abdominal pain. A complex cystic left upper quadrant mass was detected with anterior displacement of the pancreas, favoring retroperitoneal origin; radiologic impression was “calcified adrenal cyst.” B, Subsequent pancreatospleno- adrenalectomy revealed a 6.8-cm adrenal pseudocyst with thick capsule containing focal calcifications. C-E, Cystic adrenal epithelioid angiosarcoma. C, In a 62-year-old woman presenting with lower back pain and mild anemia, abdominal CT showed a well-circumscribed, cystic right adrenal mass with extensive central hemorrhage and necrosis, suspected for adrenocortical carcinoma. D, The subsequent adrenalectomy specimen contained a 13.7-cm cyst exhibiting a variegated cut surface with spongy, dark red tissue at the periphery; no viable cyst lining or distinct adrenal gland was grossly identifiable. E, Histomorphologic review demonstrated cyst content as predominantly organizing blood clots, with compressed, uninvolved adrenal gland at the periphery (black arrowheads); several minute foci of residual neoplastic tissue (green arrowheads) were identified. These neoplastic cells were strongly positive for CD31, in contrast to the negatively stained adjacent benign adrenal tissue (E [inset], original magnification ×400). F-H, Cystic pheochromocytoma. F, In a 52-year- old man presenting with abdominal mass lesion, CT identified bilateral adrenal masses with central areas of low attenuation (left, 3.7 cm; right, 2.3 cm), suspected for cystic pheochromocytomas. Subsequent adrenalectomies (G) identified adrenal cysts, confirmed the radiologic impression of pheochromocytoma histologically (H) and immunohistochemically (data not shown). B, E, and H, Hematoxylin and eosin (H&E), original magnifications ×100 (H inset, H&E ×400).
autopsy-based statistics more likely represent a true prevalence in general population, whereas surgical excision is considered a potential confounding factor and may be disproportionally associated with clinically significant le- sions. The largest case series to date reviewed 41 cystic adrenal lesions surgically excised at the Mayo Clinic (1973- 1998) [7]: 66% were symptomatic, typically with abdominal pain or gastrointestinal manifestations, and the remaining 34% were incidental. Among those 41 cases, 78% were pseudocysts including those associated with neoplasms, 20% endothelial, and 2% epithelial.
It may worth noting that it is still under debate whether cystic neoplasms with an adrenal cortex or medulla derivation should be categorized as epithelium-derived cystic adrenal lesions or categorized solely based on the cyst inner wall linings [7,21]. In some recent studies, cystic adrenal neoplasms were placed into the subgroup of pseudocysts because their cyst inner walls were devoid of any lining on examined sections; those cystic neoplasms included adrenocortical carcinomas, adenomas, pheochro- mocytomas, and neuroblastomas and other rarer entities [5,7]. Other authors have doubted the origin of pseudocysts from degenerated adrenal neoplasms and have suggested that at least most of the adrenal pseudocysts arise from endothelium-lined vascular lesions. Such controversy may contribute to the discrepancies in the statistics on histologic subtypes. In our study, adrenocortical carcinoma and pheochromocytoma were considered epithelium/neuroecto- derm-derived neoplasms. As a result, in our case series that included both autopsy and surgical pathology specimens, 39% were pseudocysts, 6% were endothelium derived, and 55% were epithelium/neuroectoderm derived.
4.3. Adrenal pseudocyst and endothelial cyst are considered 2 variants of adrenal vascular cysts
Pseudocysts of adrenal gland have been considered to result from hemorrhage within an adrenal neoplasm or hemorrhage into normal adrenal parenchyma due to trau- matic, toxic, or infectious process [9]. Anomalous vascular channels have been identified within and surrounding most adrenal pseudocysts [25,26]. Some of these vascular structures exhibit lymphatic differentiation under electron microscope, further confirmed by positive D2-40 immu- nostaining [5,24].
Adrenal endothelial cysts, on the other hand, have been suggested to originate from a preexisting vascular malfor- mation or postobstruction ectatic lymphatic channels [21,27]. Endothelial cysts include 3 subtypes: angiomatous, lymphangiomatous, and hamartomatous, all demonstrating a recognizable endothelial lining. Among these 3 subtypes, lymphangiomatous endothelial cyst is the most frequent, demonstrated by positive D2-40 immunostaining.
Grossly, both variants of adrenal vascular cysts are usually well circumscribed and encapsulated, as observed in our case series. Their reported sizes range from 1.4 to 33 cm
[26]; in our case series, the size range was 0.9 to 17.0 cm in greatest dimension. Pseudocysts and endothelial cysts share certain histopathologic features including fibrous capsule, hemorrhage, calcification, proliferating thin-walled vessels, and lymphoid aggregates around the cysts. In addition, each displays its own distinctive features such as adipose metaplasia and entrapped benign adrenal cortical cells in pseudocysts, in contrast to the endothelial lining, smooth muscle wall, and atheroma-like plaques in endothelial cysts.
4.4. Radiologic features of cystic adrenal lesions may help with differential diagnosis
Abdominal imaging studies for cystic lesions of the adrenal gland may show a solid or cystic suprarenal mass. Ultrasonography, CT, and MRI studies have been very effective in recognizing cystic lesions; however, radiologic findings are usually inadequate for the definitive subtyping of a cystic adrenal lesion [26] or to distinguish benign from malignant entities [7]. When suspicious, further investiga- tions, that is, biopsies or surgery, are usually performed to rule out malignancy. Adrenal pseudocysts, in particular, may exhibit a range of appearances on CT, as suprarenal cystic, mixed, or predominantly solid masses. Pseudocysts are usually of low density, sometimes with thick walls, internal septations, and/or calcifications. On MRI, adrenal pseudo- cysts usually exhibit intermediate to high density in T1 wedge images (as opposed to other subtypes of adrenal cysts) and marked light up in T2 wedge images [5]. Calcifications of pseudocysts are usually located along the cyst wall or septum, whereas central calcifications and solid components have been observed in adrenal pseudocysts, which mimic true adrenal neoplasms [4,28]. In comparison, endothelial cysts tend to be more multilocular and more often exhibit septal calcifications. They also have more hemorrhagic contents than do other subtypes of adrenal cysts [4]. In our case series, radiologic studies and gross examination cor- related well in detecting the presence, and often also eval- uating the extent, of the cystic components. This observation further supports the value of radiologic studies as initial workups for these lesions.
On imaging studies, the differential diagnoses of cystic adrenal lesions include cysts of spleen and pancreas, empyema of the gallbladder, and abdominal aneurysms. Because of the heterogeneous etiologies, overlapping clinical symptoms, and often nonspecific radiologic findings of cystic adrenal lesions, it is often difficult to render a definitive preoperative diagnosis [5]. A definitive diagnosis, therefore, often relies on extensive tissue sampling and thorough microscopic examination.
4.5. Management and treatment
A consolidated consensus has not been reached on the management and treatment of cystic adrenal lesions, in part,
because of the low incidence of these lesions, as well as the difficulty in establishing definitive preoperative diagnosis. Some authors prefer more conservative approaches of sur- veillance, especially in small, asymptomatic lesions, given their usual benign clinical course. Other authors advocate surgery for all adrenal cysts, especially for those arising in and/or closely associated with malignant neoplasms, those larger than 5 cm because of risk of hemorrhage or other secondary complications, and those with an unclear radiologic impression. Certain other findings (eg, metastasis and Beckwith-Wiedemann syndrome) or associated anom- alies (eg, superior vena cava syndrome) should also prompt surgical intervention. Possible management procedures in- clude percutaneous needle aspiration and sclerotherapy, sur- gical resection, and cyst unroofing [29].
In summary, cystic lesions of the adrenal gland are un- common, often nonsymptomatic or with nonspecific clinical findings, easily underrecognized, occasionally associated with malignant neoplasms, and with significant clinical con- sequences, especially if misdiagnosed. Radiologic studies are helpful as initial workups and, in general, correlate well with macroscopic examinations. A definitive diagnosis, how- ever, is often inaccessible preoperatively and relies on exten- sive tissue sampling and thorough microscopic examination.
Acknowledgment
We thank Dr Christopher Fletcher at Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, for providing expert consult review on the case of primary adrenal epithelioid angiosarcoma; faculty, residents, and laboratory staff at New York University Langone Medical Center for insightful contributions and technical support; and editor and anonymous reviewers for advice on manu- script revisions.
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