Recurrent Nested Stromal Epithelial Tumor of the Liver with Extrahepatic Metastasis: Case Report and Review of Literature

Abstract

Nested stromal epithelial tumor is a recently described primary neoplasm of the liver. This tumor is characterized by well-demarcated nests of spindle and epithelioid cells with occasional calcification and bone formation. An association between these tumors and Cushing syndrome has been described. Herein we report a case of a recurrent nested stromal epithelial tumor of the liver in a 17-year-old female with aggressive clinical behavior and an extrahepatic lymph node metastasis. Also, we provide the first detailed clinical, histologic, immunohistochemical, and cytogenetic comparison of the original and recurrent tumors. Initially, the patient presented with Cushingoid symptoms and epigastric pain, radiating to her back. A computed tomographic (CT) scan revealed a large lesion in the liver. After a partial hepatectomy, the Cushingoid features were resolved. A year later, a CT scan revealed multiple lesions within the liver, and positron emission tomographic/CT imaging showed a hypermetabolic lymph node. The patient underwent a cadaveric liver transplant. Histologically, both the original and recurrent tumors had similar characteristics, with different immunoreactivity, correlating with the absence of systemic hormonal symptoms. Electron microscopy of the original neoplasm revealed an abundance of rough cytoplasmic reticulum and mitochondria. No evidence of endocrine differentiation was found. Cytogenetics of the primary tumor was complex with an abnormal hypotriploid karyotype. Our data indicate that patients with nested stromal epithelial tumor of the liver must be carefully followed with imaging to detect hepatic recurrence and extrahepatic metastases.

Cushing's syndrome associated with a nested stromal epithelial tumor of the liver: hormonal, immunohistochemical, and molecular studies

“…Recently, rare cases of nonhepatocytic mixed nested stromal and epithelial tumors (NSET) of the liver were reported by two different groups, and their histopathologic and immunohistochemical features were extensively described (4,5). Of 22 cases reported in the literature, few were associated with Cushing's syndrome which clinical and biochemical characteristics were partially described (4,6). In the present report, we describe the occurrence of a NSET of the liver in a teenage girl who developed EAS originating from combined production of ACTH and corticotropinreleasing hormone (CRH) by the tumor.…”

“…Twenty-two cases were reported in the literature, eleven occurring during infancy (4, 5, 9, 10), seven in teenagers (4-6, 10), and only four cases in the adult (10,11). Four cases of liver NSET associated with Cushing's syndrome were reported in two children with ACTH-dependent hypercortisolism (4) and in two teenagers (6,10), but these cases were partially described. The prognosis of NSET remains unpredictable due to its scarcity.…”

“…The prognosis of NSET remains unpredictable due to its scarcity. Nevertheless, among the 22 cases reported in the literature, three have had local hepatic recurrence of the disease, one with lymph node metastasis, but none with disant metastasis (4,6,10).…”

Nested Stromal Epithelial Tumor of the Liver—Liver Transplantation and Follow-Up

“…There are only few reports worldwide reporting and defining the tumor [4,8]. One publication reports the tumor in a young man [9]. There are only few individual reports about the metastasizing behavior, and in those cases, there were mainly lymph node metastases [8].…”

Nested stromal-epithelial tumour of the liver: An unusual liver entity

“…Brodsky et al [15] reported experiences of recurrent NSETs of the liver with lymph node metastasis after partial hepatectomy. Hommann et al [16] reported a case of lung metastasis after liver transplantation for an unresectable NSET.…”