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Case Report Pseudoglandular Myxoid Adrenocortical Adenoma with Positive Epithelial Markers: a Case Report and Review of the Literature

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Case Report Pseudoglandular Myxoid Adrenocortical Adenoma with Positive Epithelial Markers: a Case Report and Review of the Literature Int J Clin Exp Pathol 2020;13(5):1090-1093 www.ijcep.com /ISSN:1936-2625/IJCEP0109355 Case Report Pseudoglandular myxoid adrenocortical adenoma with positive epithelial markers: a case report and review of the literature Ming-He Bai, Li-Yun Liu, Meng Zhao, Qian Wu, Xiao-Juan Zheng, Ji-Xian Wang, Zhi-Yong Zhang Department of Pathology, Tangshan Gongren Hospital, Tangshan, China Received February 17, 2020; Accepted March 27, 2020; Epub May 1, 2020; Published May 15, 2020 Abstract: Myxoid adrenal cortical adenoma with a pseudoglandular structure is a special histological variant and is extremely rare. We report about a 32-year-old Chinese woman with a right adrenal mass during a routine physical examination. The cut surface of the mass had a vague nodular, which gross appearance was pale, yellowish, and semitransparent. Histologically, the region is mostly characterised by pseudoglandular pattern with myxoid stroma. They are filled with clear cells or eosinophilic cells, as well as semitransparent regions, in which anastomosing small eosinophilic cells arranged in pseudoglandular, cord-like, or wreath-shaped structure float in the mucous pool. Im- munohistochemical staining shows Melan-A, vimentin, and CD56 were positive and CK (AE1/AE3) were nucleus- side staining. A small number of tumor cells were positive for alpha-inhibin and synaptophysin, ki-67 labeling index was 3%. EMA, chromogranin A, WT-1, and P63 were negative. This report aimed to emphasize pseudoglandular patterns with mucus secretion which could occur in adenomas of the adrenal cortex, nucleus-side positive for CK is remarkable. However, this type may have malignant potential, so regular follow-up is needed. Keywords: Myxoid adrenal cortical adenoma, pseudoglandular pattern, CK, nucleus-side Case presentation scope was performed under general anes- thesia. Then, the specimen was sent for patho- A 32-year-old Chinese woman discovered a logical examination. Shortly afterwards, she painless mass in her right adrenal gland in a recovered quickly. routine physical examination in Nov., 2018. There were no additional abnormal findings, Pathological examination such as obesity and hypertension, and neither biochemical nor physical signs of endocrine Grossly, the surgical specimen of the adrenal pathology. By viewing the results of the con- gland was measured about 5×4×3 cm. The trast-enhanced test, an irregular mass of soft- mass was a vague nodule which was 4.5×3.5×3 tissue’s higher density that approximately 4.0 cm in size and its cut surface partly showed cm×3.5 cm in size was found in the right faint yellow, semitransparent in myxoid areas, adrenal gland. After the injection of the con- as well as partly golden yellow. trast medium, the lesions showed obvious homogeneous enhancement. The MRI of the Histologically, the tumour was enveloped by right adrenal area displayed a slightly lower T1 fibrous capsule. It was characterised by pseu- nonuniformity and a slightly higher T2 nonuni- doglandular structure in myxoid background. formity. By the contrast, the opposite phase The tumor cells were arranged in irregularly didn’t exist significant reduction of contrast sig- anastomosing cords-like, nest patterns, and nal. The boundary was clear, and the maximum pseudoglandular architecture with occasional diameter was about 4.1 cm. It was clear that microcystic dilatations floating in pools of myx- the local right adrenal tissue’s development oid material. The shapes of these cells were could be seen. After the patient’s admission, small and round, oval, or polygonal. In some right adrenal mass resection by the laparo- areas, most of the tumor cells had a moderate Pseudoglandular myxoid adrenocortical adenoma with positive epithelial markers Tumor cells were also posi- tive for Vimentin (Figure 2B), Melan-A (Figure 2C) and CD- 56, focal positive for inhi- bin-α, and synaptophysin (Fi- gure 2D). EMA, chromogranin A, WT-1, P63 were negative. Discussion Figure 1. Tumor cells are arranged in irregularly anastomosing cords, nest patterns and pseudoglandular architecture with occasional microcysts float- Myxoid adrenal cortical ade- ing in pools of myxoid material. Cells are small and round, oval, or polygo- nomas are very rare. How- nal. Cytoplasm contains granular and weakly eosinophilic material. Regular ever, pseudoglandular arran- nuclei with dispersed chromatin had visible nucleoli but lacking atypia and gements in tumors of the pleomorphism (A); The tumor extends to the periadrenal adipose tissue (B) (hematoxylin and eosin). adrenal cortex have rarely be- en mentioned. Papotti et al. reported 14 adrenocortical tumors with variably abun- dant myxoid component. My- xoid changes are identified most frequently in malignant or borderline adrenocortical tumors [1]. Adrenal cortical adenoma is a benign neoplas- tic proliferation of adrenal cor- tical tissue. Most adrenal adenomas occur on one side and clinical symptoms are diverse. The tumors can be classified into two groups: functional and non-functional tumor according to the sec- retion situation of endocrine hormone. Excessive cortisol Figure 2. Immunohistochemistry of pseudoglandular myxoid adrenocortical secretion could cause Cu- adenoma including perinuclear positivity for cytokeratin (AE1/AE3) (A), vi- shing syndrome. Excessive mentin (B), and melan-A (C). Focal positivity for synaptophysin (D). secretion of aldosterone can cause primary aldosteronism amount of clear vacuolated cytoplasm and con- or Conn syndrome [2]. Non-functional adrenal tained tiny granular and weakly eosinophilic adenomas show no clinical symptoms. Myxoid material. They had regular nuclei with dispersed adrenocortical adenomas are usually filled chromatin. Part of the nuclei had visible nucle- with extracellular myxoid materials, which is oli but lacked atypia. In the myxoid area the very necessary to differentiate from metasta- tumor cells neither had dysplasia nor cell tic mucinous adenocarcinoma in the adrenal pleomorphism (Figure 1A). Mitotic figures and gland. Under an existing related study, it se- necrosis were not observed. The tumor infi- emed relatively straightforward to distinguish ltrated into the periadrenal adipose tissue myxoid adrenal cortical tumor from other ad- areas (Figure 1B). Residual adrenal cortex was renocortical ones by the histopathology and found surrounding the tumor. No necrosis or immunohistochemical findings. Surgical resec- signs of invasive growth were observed. tion of the mass is the primary way. Immunohistochemical findings Here, we have described a case of pseudoglan- dular myxoid adenoma of the adrenal gland. Tumor cells were positive for CK (AE1/AE3) and Gelatinous areas can be seen grossly, which apparently nucleus-side staining (Figure 2A). exactly indicates an extensive myxoid transfor- 1091 Int J Clin Exp Pathol 2020;13(5):1090-1093 Pseudoglandular myxoid adrenocortical adenoma with positive epithelial markers mation. As the occurrence of myxoid changes comprising 25% or less of the tumor, a diffuse is limited to the focal areas in some cases, architecture, microscopic necrosis, as well the situation mentioned is not the rule [3]. as invasion of venous, and sinusoidal and cap- Microscopically, the patterns of these neo- sular structures. Malignant adrenal cortical plasm cells take on irregular clusters, nests, neoplasms tend to be larger and heavier than trabeculae, anastomosing cords, or rare pseu- its benign counterparts. doglands [4]. Pseudoglandular areas contain mucin type material that exists mainly in ex- The differential diagnosis of myxoid adren- tracellular matrix not in cytoplasm and show ocortical neoplasms also includes metastatic Alcian blue and PAS staining. Myxoid material and primary tumors with myxoid changes, such might represent a degenerative process or a as chordoma, chondrosarcoma, liposarcoma, product of stromal fibroblasts and tumor cells myxoid leiomyosarcoma, and malignant fibrous [5]. The tumor cells have a moderate amount histiocytoma [8]. Chordomas may be the most of eosinophilic and hyaline cytoplasm. The ma- difficult to recognize from myxoid adrenocorti- terial has never been found in the cytoplasm cal tumors. It has much more sparse cells than of neoplasm cells, so the origin of myxoid mate- myxoid adrenocortical tumors. Keratin is strong rial is unknown. positive staining in chordomas and abundant smooth endoplasmic reticulum [9]. Liposar- Immunohistochemical expressions have re- comas have distinct adipocytes and S-100 vealed adrenal cortex markers are positive, protein is positive. Myxoid leiomyosarcomas namely inhibin α, Melan-A, and vimentin. consist of spindle and epithelial cells, which Synaptophysin and CD56 can also be positive. usually express muscle markers such as des- Synaptophysin often express in adrenal me- min and smooth muscle actin. When pseudo dulla but in this case it expressd focally in glandular areas are prominent, a well-differen- adrenal cortex tumour. CKpan is negative or tiated metastatic adenocarcinoma is to be focal weak positive. EMA, S-100, and CgA are taken into account first, since pulmonary, negative [6]. Immunohistochemical staining breast, and other rarely glandular carcinomas of neoplastic cells floating the myxoid compo- may metastasize to the adrenal gland. Unlike nent has confirmed an adrenal cortical origin. the nuclei of mucinous adenocarcinoma, nuclei Vimentin is expressed primarily in cells of of myxoid adrenocortical adenoma are usually mesenchymal origin. In addition it is also located in the central portion of the cytoplasm
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