The challenge to differentiate between sarcoma or adrenal carcinoma—an observational study

Abstract

Background Adrenal sarcomas are rare malignant tumors with structural and clinical similarities to sarcomatoid adrenocortical carcinoma. Preoperative diagnosis of tumors of the adrenal gland can be challenging and often misleading thus detaining patients from appropriate oncological strategies. Objective This analysis of a case series evaluated the predictive capability of the primary clinical diagnosis in case of malignancies of the adrenal gland. Methods Thirty two patients were treated from 2009 to 2015 at our clinic and analyzed retrospectively. All patients had computed tomography and/or magnet resonance imaging and a primary histopathological examination at our institution after surgery. Ten questionable cases were surveyed by a reference pathologist. Results Twelve out of 32 diagnoses had to be revised (37.5%). Only 15 out of 24 tumors primarily classified as adrenocortical carcinoma were finally described as primary adrenal cancer. We found two leiomyosarcomas, one liposarcoma, one sarcomatoid adrenocortical carcinoma, and one epitheloid angiosarcoma among 12 misleading diagnoses. Other tumors turned out to be metastases of lung, hepatocellular, and neuroendocrine tumors. Larger tumors were significantly more often correctly diagnosed compared to smaller tumors. Four patients of the group of revised diagnoses died whereas all patients with confirmed diagnoses survived during the follow-up. Conclusion Preoperative assessment of tumors of the adrenal gland is still challenging. In case of wrong primary diagnosis, the prognosis could be impaired due to inadequate surgical procedures or insufficient preoperative oncological treatment.

Primary Adrenal Epithelioid Angiosarcoma: A Case Report

“…Due to their propensity for a wide spectrum of morphological presentation and sarcomatoid differentiation, the correct diagnosis of these lesions can be easily misinterpreted. 14,15 Immunohistochemical stains may be useful, with Melan-A and SF1 expected to be positive. Clinical symptoms such as Cushing syndrome or Diagnoses of adrenal angiosarcoma are often made late in the disease course, and beyond surgical resection to negative margins, there is no standard treatment approach.…”

Histopathologic Features of Adrenal Cortical Carcinoma

“…1). [5][6][7][8][9][10][11][12] Focusing on adrenal cortical tumors (ACTs), the primary diagnostic concern is the accurate and precocious assessment of the malignant potential of the lesion to guide subsequent therapeutic management and follow-up. 13 Unfortunately, ACC can present clinical, laboratory, and imaging features overlapping with other primary or secondary malignant lesions and with benign conditions.…”