Primary adrenal gland epithelioid sarcoma: A case report and literature review

Abstract

Epithelioid sarcoma is a malignant mesenchymal neoplasm with morphologic and immunophenotypic epithelioid differentiation, which rarely arises in solid organs. We report a case of primary epithelioid sarcoma in the adrenal gland of a 31‐year‐old female. The patient initially presented with nausea and rectal bleeding, and subsequent imaging studies revealed a 4.4 cm left adrenal gland mass and left retroperitoneal lymphadenopathy. Clinical and radiological studies did not reveal tumor elsewhere in the patient. Histologic features were those of epithelioid sarcoma, proximal type with cohesive clusters of epithelioid tumor cells harboring frequent mitoses, and areas of necrosis. Immunohistochemical stains showed strong, diffuse expression of epithelial markers (pancytokeratin), and CD34 and Fli‐1. Partial and focal positive staining of CK7 was also noted. Nuclear expression of SMARCB1 (INI‐1) protein was lost. ERG was negative in this case. We believe that this is the second‐case report of a primary adrenal gland epithelioid sarcoma. Fli‐1 positivity was seen in our case, and ERG was negative as shown in some recent publications regarding epithelioid sarcomas.

Abnormal Adrenal Mass Presents as Proximal Epithelioid Sarcoma

“…ESs are very rare neoplasms that tend to have an aggressive clinical course and tend to recur [ 1 – 4 ]. The size of ES neoplasms can get quite large with a median diameter of 4 cm, and they are typically painless [ 1 – 4 ]. In this case, the ES neoplasm presenting as an adrenal mass was an incidental finding and was quite large as well—measuring 6.5 × 5.1 × 5.0 cm.…”

“…Epithelioid sarcoma (ES) is a malignant mesenchymal neoplasm that shows some level of epithelial differentiation [ 1 – 4 ]. It is a very rare sarcoma that accounts for less than one percent of all soft tissue sarcomas [ 1 , 2 ].…”

“…It is a very rare sarcoma that accounts for less than one percent of all soft tissue sarcomas [ 1 , 2 ]. There are two subtypes: classic and proximal [ 1 – 4 ]. The classic subtype presents more commonly in adolescents and young adults in distal extremities, whereas the proximal subtype tends to present in older adults in proximal soft tissue [ 1 – 4 ].…”

“…There are two subtypes: classic and proximal [ 1 – 4 ]. The classic subtype presents more commonly in adolescents and young adults in distal extremities, whereas the proximal subtype tends to present in older adults in proximal soft tissue [ 1 – 4 ]. The cytomorphology of the proximal type tends to be higher grade than the classic type, and some of its malignant cells have a rhabdoid phenotype [ 1 – 4 ].…”

“…This is the third reported ES presenting as an adrenal mass. The first reported primary adrenal ES was in 2017 by Alikhan et al [ 3 ] and the second was in 2019 by Huang et al [ 4 ].…”