Adenomatoid tumour (AT) is a rare, benign neoplasm of mesothelial origin, which

usually occurs in the genital tract of both sexes. Occasionally these tumours are found

in extra genital locations such as heart, pancreas, skin, pleura, omentum, lymph

nodes, retroperitoneum, intestinal mesentery and adrenal gland. Histologi cally ATs

show a mixture of solid and cystic patterns usually with focal presence of signet-ring

like cells and scattered lymphoid infiltration. The most important thing about these

tumours is not to misdiagnose them as primary malignant or metastatic neoplasms.

We present a case of an adrenal AT in a 29-year-old asymptomatic male. The tumour

was an incidental finding during abdominal CT-scan for an unrelated condition.

We also present a review of the literature concerning adrenal gland AT and give

possible differential diagnosis

Hubalewska-Dydejczyk, Alicja

Kostecka-Matyja, Marta

Okoń, Krzysztof

Szczepański, Wojciech

Szpor, Joanna

Tomaszewska, Romana

Ulatowska-Białas, Magdalena

Polish Journal of Pathology

English

Adenomatoid tumour (AT) is a rare, benign neoplasm of mesothelial origin, which
usually occurs in the genital tract of both sexes. Occasionally these tumours are found
in extra genital locations such as heart, pancreas, skin, pleura, omentum, lymph
nodes, retroperitoneum, intestinal mesentery and adrenal gland. Histologi cally ATs
show a mixture of solid and cystic patterns usually with focal presence of signet-ring
like cells and scattered lymphoid infiltration. The most important thing about these
tumours is not to misdiagnose them as primary malignant or metastatic neoplasms.
We present a case of an adrenal AT in a 29-year-old asymptomatic male. The tumour
was an incidental finding during abdominal CT-scan for an unrelated condition.
We also present a review of the literature concerning adrenal gland AT and give
possible differential diagnosis

Jagiellonian Univeristy Repository

97POL J PATHOL 2010; 2: 97–102ADENOMATOID TUMOUR OF THE ADRENAL GLAND: A CASE REPORT AND LITERATURE REVIEWMAGDALENA BIAŁAS1, WOJCIECH SZCZEPAŃSKI1, JOANNA SZPOR1, KRZYSZTOF OKOŃ1, MARTA KOSTECKA-MATYJA2, ALICJA HUBALEWSKA-DYDEJCZYK2, ROMANA TOMASZEWSKA11Chair and Department of Pathomorphology, Jagiellonian University Medical College, Kraków 2Chair and Department of Endocrinology, Jagiellonian University Medical College, Kraków Adenomatoid tumour (AT) is a rare, benign neoplasm of mesothelial origin, whichusually occurs in the genital tract of both sexes. Occasionally these tumours are foundin extra genital locations such as heart, pancreas, skin, pleura, omentum, lymphnodes, retroperitoneum, intestinal mesentery and adrenal gland. Histologi cally ATsshow a mixture of solid and cystic patterns usually with focal presence of signet-ringlike cells and scattered lymphoid infiltration. The most important thing about thesetumours is not to misdiagnose them as primary malignant or metastatic neoplasms.We present a case of an adrenal AT in a 29-year-old asymptomatic male. The tumourwas an incidental finding during abdominal CT-scan for an unrelated condition. We also present a review of the literature concerning adrenal gland AT and givepossible differential diagnosis.Key words: adenomatoid tumour, adrenal gland, immunophenotype.IntroductionAdenomatoid tumour (AT) is a benign neoplasmof mesothelial origin [1-3]. The usual place of itsappearance is the male and female genital tract, mostoften epididymis in men and fallopian tube in women[4, 5]. Extragenital ATs have occasionally beendescribed in heart [6], pancreas [7], skin [8], pleura[9], omentum [10], lymph nodes [11], retroperi-toneum [12], intestinal mesentery [13], periadrenalsoft tissue [14] and adrenal gland [15-31]. We report the clinicopathological and immuno-histochemical findings of an incidentally discoveredAT of the right adrenal gland in a 29-year-old man.Material and methodsThe resected adrenal gland with tumour was fixedin a 10% buffered formaldehyde solution. Represen-tative sections of the tumour were embedded inparaffin. Tissue samples were cut into approximately4 µm thick sections by microtome, placed on glassslides and stained with haematoxylin end eosin forhistopathological examination. Additional sectionswere made for immunohistochemical analysis (fordetails see Table I).Proliferative activity of the tumour was deter-mined using immunohistochemical staining fornuclear protein MIB-1 (Ki-67). The proliferativeactivity rate (MIB-index) was calculated as a per-centage of nuclei positive for MIB-1 in five areas of100 cells each.Clinical data were obtained from the patient’s filesin the Endocrinology and Surgery Departments.ResultsClinical and macroscopic findingsA 29-year-old male was admitted to the hospitalwith the diagnosis of inguinal hernia. The pre-opera-tive imaging procedures revealed a well circum-scribed, mostly solid, 4 µm tumour of the right adre-nal gland. The right adrenal gland with the tumourwas removed.The material sent for the histological examinationweighted 34 g and consisted of fatty tissue and98enlarged adrenal gland. On cross-sections a mass 4 cm in the biggest dimension arising from not-widened 3.5 cm long adrenal gland was seen (Fig. 1).The tumour was solid and well circumscribed. Its cutsurface was smooth, greyish-white and partly yellowwithout haemorrhage or necrosis.Histological findingsThe histological examination revealed an unen-capsulated tumour consisting of a mixture of differ-ent patterns: gland-like spaces of various size, irreg-ular outline and focal cystic dilation and solid nestsof cells set in a fibrous stroma (Fig. 2). The tumourstructures were seen between nests of residualadrenocortical cells (Fig. 3). A discontinuous rim ofcompressed adrenal cortex was focally presentaround the tumour. Cells lining these irregular chan-nels and tubular structures were mostly flattenedresembling endothelial cells (Fig. 2). Some of themwere plump with epithelioid features and abundantcytoplasm. Small groups of cells with the appearanceof so-called signet-ring cells with round, intracyto-plasmic vacuoles were also seen (Fig. 3). Nuclei werevesicular, occasionally with small nucleolus. None ofthe cells exhibited atypia or pleomorphism. Mitoticfigures and necrosis were absent. Small lymphoidaggregates and foci of lipomatous metaplasia werefocally present within the tumour (Fig. 4). The adre-nal medulla had typical histological structure with-out evidence of the tumour cells. Mucicarmin staindid not reveal any presence of mucin within signet-ring like cells or in tumour channels. An immuno-histochemical examination showed that the tumourcells exhibited strong expression of cytokeratinFig. 1. Cut surface of the adrenal gland showing a solid,well circumscribed tumour with smooth, greyish-whitesurfaceFig. 2. Adrenal AT composed of mixture of patterns:fenestrated channels of varied size and irregular outlines,and solid nests of cells (HE, 10×)Table I. Primary antibodies used for immunohistochemical staining ANTIBODY PRETREATMENT DILUTION/ DETECTION MANUFACTURERINCUBATION TIME METHODAE1/AE3 citrate buffer 1/50 (30’) LAB VISION DakoCK5/6 EDTA 1/50 (30’) EN VISION DakoCK7 citrate buffer 1/50 (30’) EN VISION DakoCK20 citrate buffer 1/50 (30’) EN VISION Dakocalretinin citrate buffer 1/100 (60’) EN VISION NovocastraD2-40 citrate buffer ready to use (30’) EN VISION PolgenCD31 EDTA 1/20 (60’) EN VISION DakoCD34 citrate buffer 1/50 (30’) LAB VISION Dakofactor VIII citrate buffer 1/50 (60’) EN VISION DakoKi-67 citrate buffer 1/50 (night) LAB VISION DakoMAGDALENA BIAŁAS, WOJCIECH SZCZEPAŃSKI, JOANNA SZPOR, ET AL.99ADENOMATOID TUMOUR OF THE ADRENAL GLANDAE1/AE3, CK7, calretinin (Fig. 5), D2-40 (Fig. 5)and vimentin (Fig. 5). Focal expression of cytoker-atin CK5/CK6 was observed. Tumour cells did notexpress any vascular markers like: CD31, CD34 andFactor VIII or epithelial marker CK20. Proliferatingactivity measured by expression of proliferativemarker Ki-67 (MIB-index) did not exceed 1% ofcells.DiscussionAT is a rare finding usually present in the genitaltract. Adrenal gland AT is extremely rare: we couldonly find 27 cases reported previously in the litera-ture [15-31]. Only four authors reported more thanone case of AT [16-18, 28] with the biggest seriesconsisting of 5 cases [18]. Characteristics of the 28 adenomatoid tumours reported previouslytogether with our case are summarized in Table II. ATs occurred in young adults mostly in the 3rd-4th decades of life. The average age of patients in allreported cases was 40, ranging from 24 to 64 years.Only 6 patients were older than 50. Most of the patients with the adrenal gland ATwere men. Only one tumour was reported ina woman [16]. A possible explanation for this dis-proportion can be connected with the differences inembryological development of the gonads in bothFig. 5. Adrenal AT immunohistochemistry: A) calretinin, B) D2-40, C) VimentinFig. 3. Tumour structures intermingled with residualadrenal cortical tissue (HE, 10×)Fig. 4. Focal lymphoid aggregates and foci of lipomatousmetaplasia within AT (HE, 10×)A B C100sexes, especially different role of mesonephric ductsin males and females [20] together with the hypoth-esis that ATs arise from inclusions of pluripotentialmesenchyme cells associated with the Mullerian tract[1, 17-19]. The mesonephric duct in men slowlytransforms into a duct of epididymis and remains inthat form. The mesonephric duct in women regress-es early during the embryogenesis [20]. The possi-bility of penetration of pluripotential mesenchymecells from the developing mesonephric duct to dif-ferent locations (e.g. adrenal glands) in women ismuch less probable than in men.Adrenal ATs were almost equally distributedbetween the right and left adrenal glands (14 : 13), inone case, data were not available. Almost all caseswere discovered incidentally during radiographicexamination [15-19, 21-29, 31], surgery for an unre-lated reason [18] or during autopsy [16, 18, 20, 30].ATs demonstrated a great degree of size variation:the smallest described examples were about 1 cm indiameter [16-19], the biggest one had about 15 cm[15]. 21 reported ATs were smaller than 5 cm andall were asymptomatic [16-20, 22-25, 28-31] as wellas two the biggest tumours [15, 27]. Only onepatient had symptoms which could be connected toadrenal mass [17]. The patient with central andright flank abdominal pain had a coexisting big adre-nal cyst (11 cm in diameter) with only small AT1.2 cm in diameter in the cyst wall. The symptomswere more probably connected with the big cysticmass in the abdomen than with the small solid areaof AT in the cyst wall [17]. Table II. Clinicopathological features of adenomatoid tumours of the adrenal glandPATIENT AGE SEX LATE- DIAM- CLINICAL FINDINGS AND SYMPTOMS CITA-NO. RALITY ETER TION1. 26 M R 15 IRF (suspicion of a giant echinococcus cyst of the liver) [15]2. 49 M R 1.3 IFA [16]3. 57 M L 3.8 IFA [16]4. 50 F R 0.5 IFA [16]5. 40 M L 6.0 IRF (found on CT scan during sarcoma staging) [16]6. 46 M R 1.2 CT (symptoms: central and right flank abdominal pain) [17]7. 33 M L 1.7 IRF (CT during workup for hypertension) [17]8. 33 M R 4.2 IRF (CT) [17]9. 37 M L 3.1 IFS [18]10. 31 M R 3.2 IRF [18]11. 31 M – 3.5 IRF (investigation in the course of hypertension) [18]12. 64 M L 1.2 IFA [18]13. 44 M L 3.2 IRF (investigation in the course of hypertension) [18]14. 24 M L 1.1 IRF (during investigation for Cushing syndrome) [19]15. 30 M L 3.0 IFA [20]16. 54 M L 6.5 IRF (investigation in the course of pneumonia) [21]17. 31 M R 4.0 IRF [22]18. 42 M L 2.5 IRF [23]19. 54 M R 3.6 IRF [24]20. 51 M R 3.0 IRF (investigation in the course of hypertension) [25]21. 28 M R 9.0 IRF (investigation in the course of acute cholecystitis) [26]22 42 M L 14.5 IRF (during evaluation of renal colic) [27]23. 47 M R 7.0 IRF (CT performed for diverticulitis) [28]24. 52 M R 5.5 IRF (investigation in the course of hypertension) [28]25. 44 M L 3.5 IRF (investigation in the course of hypertension) [29]26. 34 M R 3.0 IFA [30]27. 33 M L 1.7 IRF (investigation in the course of hypertension) [31]28. 29 M R 4.0 IRF our caseM – male, F – female, R – right adrenal gland, L – left adrenal gland, IRF – incidental radiographic finding IFA – incidental finding during autopsy, IFS – incidental finding during surgery for unrelated reasonsMAGDALENA BIAŁAS, WOJCIECH SZCZEPAŃSKI, JOANNA SZPOR, ET AL.101ATs were round, usually well circumscribed, grayor greyish-white with light-yellow areas. In some ofthem irregular cystic spaces had been macroscopical-ly visible [16, 21, 23, 27].The immunohistochemical profile was consistentwith the one described in ATs in usual locations(genital tract) and was typical for the tumuor ofmesothelial origin: calretinin +, CK5/6+, CK7+,vimentin+, D2-40+, CD31–, CD34–. Diagnosing difficulties of AT may arise when thistumour occurs in sites different from its usual loca-tion which is the urogenital tract. The adrenal ATtumour can mimic all other entities occurring in thisplace like: adenomas, carcinomas, pheochromocy-tomas, myelolipomas, benign cystic lesions andmetastases from distant locations. It may also be eas-ily misdiagnosed as vascular neoplasm: lymphan-gioma or angiosarcoma. Differential diagnosisincludes all these entities.Numerous irregular cystic spaces lined with flat-tened cells may suggest a vascular neoplasm, espe-cially lymphangioma. However, the cells are positivefor CK and they lack reactivity for the usual vascularmarkers (CD31, CD34, factor VIII), which excludesvascular tumours. It is important that ATs may showreactivity for D2-40, a marker which is known to bepositive in endothelia of lymphatic vessels. At least intwo cases reported in the literature ATs were prima-rily diagnosed as lymphangiomas [18].Histologically all ATs presented a local pseudo-glandular pattern often with an infiltrative appear-ance at the periphery which could lead to it beingconfused with adenocarcinoma. In case number 1described by Isotalo [18] AT was incorrectly diag-nosed as adenocarcinoma (the diagnosis from a frozensection). The final diagnosis from permanent sectionswas correct. Adenocarcinomas of the adrenal glandshow striking nuclear pleomorphism, the mitotic fig-ures are easily found and the cells are cytokeratinpositive but they do not show co-expression of cyto -keratin and calretinin (nuclear staining). The MIB-index in any reported AT was not higher than 3%(the highest one reported by Isotalo [18]). MIB-index in malignant primary and metastatic adrenaltumours is usually much higher.Mucin stain may be helpful in excluding the neo-plastic nature of signet-like looking cells. Such cellsin AT are mucin negative. Signet-ring cell carcinoma(usually metastatic from a stomach) shows nuclearpleomorphism and cells contain a lot of acid mucin:dark blue droplets in Alcian-PAS. Cystic adenomaswhich are considered in differential diagnosis do notexpress CK and D2-40. Excluding cases of ATsfound incidentally during autopsy, the local resectionof the tumour was curative for all patients. No localrecurrences or metastatic spread of AT have everbeen reported. In summary, ATs in extra genital sites like adre-nal gland are very uncommon. Nonetheless, suchdiagnostic possibility should always be considered inorder to avoid misdiagnosing them as primary ormetastatic malignant tumours.References1. 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Adenomatoid tumorof the adrenal gland: case report with immunohistochemicalstudy. Patol Int 2005; 55: 665-669.ADENOMATOID TUMOUR OF THE ADRENAL GLAND10221. Glatz K, Wegmann W. Correspondence: papillary adenoma-toid tumour of the adrenal gland. Histopathology 2000; 37:376-382.22. Schadde E, Meissner M, Kroetz M, et al. Adenomatoidertumor der nebenniere – eine seltene differenzialdiagnose desnebennierentumors. Chirurg 2003; 3: 248-252.23. Song-Qing F, Yi J, Duo L, et al. Adenomatoid tumor of theleft adrenal gland with concurrent left nephrolithiasis and leftkidney cyst. Pathology 2005; 37: 398-400.24. Varkarakis IM, Mufarrij P, Studeman KD, et al. Adenoma-toid tumor of the adrenal gland. Urology 2005; 65: 175.25. Chung-Park M, Yang JT, McHenry C, et al. Adenomatoidtumor of the adrenal gland with micronodular adrenal corti-cal hyperplasia. Hum Pathol 2003; 34: 818-821.26. Gasque CR, Marti-Bonmati L, Dosda R, et al. MR imaging ofa case of adenomatoid tumor of the adrenal gland – casereport. Eur Radiol 1999; 9: 552-554.27. Denicol NT, Lemos FR, Koff WJ. Adenomatoid tumor ofsupra-renal gland – case report. Internat Braz J Urol 2004;30: 313-315.28. Timonera ER, Paiva ME, Lopes JM, et al. Composite adeno-matoid tumor and myelolipoma of adrenal gland: report of 2 cases. Arch Pathol Lab Med 2008; 132: 265-268.29. Simpson PR. Adenomatoid tumor of the adrenal gland. ArchPathol Lab Med 1990; 114: 725-727.30. Angeles-Angeles A, Reyes E, Munoz-Fernandez L, et al. Ade-nomatoid tumor of the right adrenal gland in patient withAIDS. Endocr Pathol 1997; 8: 59-64.31. Mi-Jung K, Jae YR. A 33-year-old man with an incidentallyfound left adrenal mass during workup for hypertension –pathologic quiz case. Arch Pathol Lab Med 2003; 127: 1633-1634.Address for correspondenceMagdalena BiałasChair and Department of PathomorphologyJagiellonian University Medical Collegeul. Grzegórzecka 1631-531 Krakówe-mail: mbialas7@gmail.comfax +48 12 411 97 25MAGDALENA BIAŁAS, WOJCIECH SZCZEPAŃSKI, JOANNA SZPOR, ET AL.

Adenomatoid tumour of the adrenal gland : a case report and literature review

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Adenomatoid tumour of the adrenal gland : a case report and literature review

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