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| Available online at www.sciencedirect.com | Annals of |
| SCIENCE d DIRECT® Annals of Diagnostic Pathology 9 (2005) 11-15 | DIAGNOSTIC PATHOLOGY |
Adenomatoid tumor of the adrenal gland: a clinicopathologic study of 3 cases
Karuna Garg, MDª, Peng Lee, MDb, J.Y. Rob, Zhenhong Qu, MD, PhDª, Patricia Troncoso, MDº, Alberto G. Ayala, MDa,b,*
ªDepartment of Pathology, Health Science Center, University of Texas, Houston, TX 77030-4009, USA
bDepartment of Pathology, M.D. Anderson Cancer Center, University of Texas, Houston, TX 77030-4009, USA
Abstract
Adenomatoid tumors are relatively uncommon benign neoplasms of mesothelial origin, usually occurring in the male and female genital tracts. Rare extragenital adenomatoid tumors have been identified in the adrenal glands, heart, mesentery, pleura, and lymph nodes. In the adrenal gland, adenomatoid tumors may pose a diagnostic challenge. The differential diagnosis includes adrenocortical carcinoma and metastatic carcinoma, especially signet ring cell carcinoma. Because of its glandular pattern, an adenomatoid tumor may be confused with an adenocarcinoma. We present 3 cases of adrenal adenomatoid tumors, including one with a concurrent large hemorrhagic vascular adrenal cyst. The adenomatoid tumors were unilateral, appeared solid and white, and varied from 1.7 to 4.2 cm in diameter. They occurred in 3 male patients aged 33, 33, and 46 years. One patient presented with abdominal pain due to the presence of a concurrent large adrenal cyst. The tumor was an incidental radiological finding in another case and was discovered during the course of a workup for hypertension in the third case. The light microscopic appearances were consistent with those of typical adenomatoid tumors. Immunohistochemical stains for calretinin and cytokeratin 5/6 were positive, confirming the tumors’ mesothelial origin. Ultrastructural studies performed in 2 cases revealed microvilli and desmosomes. Follow-up showed no evidence of recurrence or metastasis. In our experience, the key to the diagnosis of this rare benign tumor is to consider adenomatoid tumor in the differential diagnosis of any glandular tumor occurring in the adrenal gland. C 2005 Published by Elsevier Inc.
Index words: Adenomatoid tumor; Adrenal gland
1. Introduction
Adenomatoid tumors are benign neoplasms that are most commonly found in the male and female genital tracts [1-4]. These tumors are characterized by anastomosing tubules lined by epithelioid and flattened cells. The mesothelial origin of adenomatoid tumors has been well established by immunohistochemical and ultrastructural studies [1,3,4]. Extragenital adenomatoid tumors are rare and have been described in the pancreas [5], heart [6], pleura [7], uterus [8], omentum [9], suprarenal location [10], lymph node [11], and adrenal glands [12-18]. In extragenital locations such as
the adrenal gland, adenomatoid tumors may pose a diagnostic challenge, with a wide range of differential diagnoses [12-18]. We describe 3 patients with adenomatoid tumors involving the adrenal glands. One case is the first reported case of concurrent adenomatoid tumor and vascular cyst of an adrenal gland.
2. Case reports
2.1. Case 1
A 46-year-old man was admitted to one of our institutions because of a long standing history of central abdominal and right flank pain. Ultrasonography and computed tomography (CT) performed at another hospital were suggestive of a renal or hepatic cyst. The preoperative differential diagnosis was an intraabdominal cyst of hepatic,
* Corresponding author. Department of Pathology, M.D. Anderson Cancer Center, University of Texas, Houston, Texas 77030-4009. Tel .: +1 713 792 3151; fax: +1 713 792 4049.
E-mail address: aayala@mdanderson.org (A.G. Ayala).
METRIC 1|
renal, or adrenal origin. A laparotomy was performed and a 12-cm cyst was found adjacent to the venacava, with right adrenal tissue partially involved by the cyst. The cyst did not appear to involve the liver or retroperitoneal structures such as the kidney.
2.2. Case 2
A 33-year-old man was found to have a left adrenal mass during a workup for hypertension. The patient had developed hypertensive intracerebral hemorrhage in the right basal ganglia and had undergone right frontotemporal craniotomy with hematoma evacuation 2 months earlier. Computed tomography scan revealed a small mass in the left adrenal gland. A left adrenalectomy was then performed.
2.3. Case 3
A 33-year-old man was incidentally found to have a right adrenal mass. Magnetic resonance imaging and CT revealed a 3-cm mass without invasion into the surrounding tissue. The levels of serum metanephrines and urinary catechol- amines and metanephrines were within normal limits. The clinical differential diagnosis included primary cortical adenoma and carcinoma. A fine needle aspiration was performed and the differential diagnosis was expanded to pheochromocytoma and clear cell neoplasm in view of the presence of atypical clear cells. Right adrenalectomy was performed shortly thereafter. Follow-up CT scan 1 year later showed no recurrent disease.
2.4. Pathology
The specimen from case 1 consisted of an 11.0-cm multilocular cyst with a mean wall thickness of 1.2 cm (see
Fig. 1). The inner lining of the cyst was hemorrhagic, with areas of calcification. No large blood vessel was associated with the cyst. A rim of compressed adrenal gland was present in the cyst wall. The adenomatoid tumor was not grossly visible. Intraoperative frozen sections were interpreted as an adrenal cyst, either a cystic neoplasm or a pseudocyst.
Each of the surgical specimens from cases 2 and 3 disclosed a nodule that measured 1.7 and 4.2 cm, respectively. Both of these nodules were well circumscribed with a solid cut surface. One was gray-white (see Fig. 2) and the other one was yellowish.
2.5. Histopathologic examination
The adrenal cyst (case 1) was composed of a fibrous wall with an intramural well-demarcated tumor (adenomatoid
tumor). The cystic wall was made up of fibrous tissue with scattered chronic inflammatory cells and foci of calcification. The lining of the wall was largely denuded, except for several foci of flattened nondescript cells that resembled endothelial cells. Multiple aggregates of histiocytes and foreign body giant cells with cholesterol clefts were also present.
The adenomatoid tumors showed anastomosing glands and tubules that were lined by plump epithelioid cells with abundant eosinophilic cytoplasm to flat mesothelial-like cells (see Figs. 3 and 4). Another common finding was the presence of adipose tissue and lymphoid aggregates within the adenomatoid tumors (see Fig. 5). Many cells with a signet ringlike appearance were present, and this feature was seen in all 3 cases. Areas of mucin production were also present.
2.6. Immunohistochemistry
The adenomatoid tumor in the 3 cases showed strong immunoreactivity for calretinin (see Fig. 6) and focal weak or strong staining for cytokeratin 5/6 (in cases 1 and 2, respectively). Negative immunostaining was found for HMB45, CD34 (cases 2 and 3), and myeloperoxidase.
2.7. Electron microscopy
Ultrastructural study was done in the adenomatoid tumors of cases 1 and 2, and this study disclosed numerous long, bushy, and slender microvilli (see Fig. 7) that are typical of mesothelial-derived cells.
3. Discussion
Seventeen cases of adrenal adenomatoid tumors have previously been reported. In year 2003, Isotalo et al [17] described 5 cases and reviewed the literature regarding these uncommon lesions. The patients ranged in age from 24 to 65 years, and in most cases (16 of 17 cases), the tumor
was an incidental radiological or autopsy finding. Only one patient presented with gross hematuria, which was likely due to compression of the superior portion of the kidney by the suprarenal mass [10]. Although adrenal adenomatoid tumors have not been found to be associated with any specific disease, an adrenal adenomatoid tumor was incidentally found in a patient with AIDS and disseminated coccidioidomycosis [16], and we found one in a patient with a vascular cyst. Immunohistochemical and ultrastruc- tural studies of previous cases, as of our cases, support a mesothelial origin for adrenal adenomatoid tumors [1-4,14]. The adrenal gland is not lined by mesothelial cells, although a mesothelial cyst of the adrenal gland has been described [14]. However, no clear link has been established between mesothelial cysts and adrenal adenomatoid tumors. The origin of a mesothelial neoplasm in the retroperitoneum raises interesting considerations. Other tumors that are putatively derived from the submesothelial mesenchyme, such as solitary fibrous tumor, have been described in the adrenal gland [19,20]. Cystic mesotheliomas may also occur in the retroperitoneum. Another hypothesis for the occurrence of tumors of mesothelial origin in the adrenal glands is that they arise in the pluripotent mesenchyme associated with the Mullerian tract. Malignant tumors of Mullerian origin, for example, papillary serous carcinoma, have been described in the retroperitoneum [21].
Adrenal adenomatoid tumors may pose a diagnostic challenge and may be misdiagnosed as metastatic adeno- carcinoma, lymphangioma, or adrenal cortical tumor [13,15,17]. In particular, the characteristic presence of signet-ring-like cells may suggest an adenocarcinoma. Although only limited clinical follow-up data are available, it appears that such as their genital counterpart, adrenal adenomatoid tumors are benign. No local recurrence or metastatic disease has been reported. Adrenal cysts are an uncommon heterogeneous group of lesions first described by Griselius in 1670 [22]. They have conventionally been divided into 4 categories: epithelial, parasitic, endothelial vascular, and hemorrhagic vascular (pseudocystic) [23,24]. They occur predominantly in the fourth and fifth decades of life, with a 2:1 female-male ratio. The most common clinical presentations are abdominal pain, abdominal mass, hypertension, or a ruptured cyst presenting as acute abdomen. Various theories have been proposed concerning the pathogenesis, including an origin from cystic hamar- toma, hemorrhage into an adrenal gland, and degeneration of an adrenal neoplasm. The gross and microscopic findings in our case of adrenal cyst were consistent with those described in the literature [23,24]. The differential diagnosis of a cystic adrenal lesion includes a cystic or degenerating adrenal cortical neoplasm. However, the normal appearance of cortical cell nests, the lack of hormone production, and the presence of thrombotic material without any necrotic tumor cells should suggest the correct diagnosis. Surgical resection appears to be curative, and no recurrences have been reported.
In summary, both adrenal adenomatoid tumors and adrenal vascular cysts are rare lesions and may coexist as in one of our cases. The hemorrhagic cyst was of vascular origin and the adenomatoid tumor of mesothelial origin, as confirmed by immunohistochemical and ultra- structural studies. No histogenetic link is likely between the 2 tumors.
Acknowledgment
We would like to thank Dr Jonathan Melamed for his critical comments on the manuscript.
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