Myxoid changes rarely occur in adrenocortical adenomas and carcinomas. Only eight benign tumours with such features have been described thus far, five of which also had a prominent pseudoglandular component. We report an additional pseudoglandular myxoid adenoma of the adrenal gland detected in a 58-year-old male patient who developed mild hypertension. At surgery, a 4-cm mass was resected and found to contain cords and tubules of polygonal cells in a myxoid background. Limited areas of classical adrenocortical adenoma were detected in less than 20% of the tumour area. Lack of atypias and absence of mucin markers, together with an immunophenotype consistent with adrenal tumours (focal cytokeratin, vimentin, synaptophysin and alpha-inhibin immunoreactivities), led to a diagnosis of primary adrenocortical adenoma with an extensive pseudoglandular myxoid pattern. However, the differential diagnosis from metastatic well-differentiated adenocarcinomas, chordomas and retroperitoneal myxoid mesenchymal tumours (e.g. liposarcoma) may be difficult in the absence of a complete clinical history and a reliable immunoprofile. We strongly recommend staining of any myxoid or glandular tumour of the adrenal gland for alpha-inhibin and synaptophysin (probably the currently best characterised markers of adrenocortical origin) before considering alternative (probably more common) diagnoses of metastatic adenocarcinoma or retroperitoneal tumours localised to the adrenal gland.
Myxoid adrenocortical adenoma with a pseudoglandular pattern
PAPOTTI, Mauro Giulio;PORPIGLIA, Francesco;TERZOLO, Massimo;CRACCO, CECILIA MARIA;
2004-01-01
Abstract
Myxoid changes rarely occur in adrenocortical adenomas and carcinomas. Only eight benign tumours with such features have been described thus far, five of which also had a prominent pseudoglandular component. We report an additional pseudoglandular myxoid adenoma of the adrenal gland detected in a 58-year-old male patient who developed mild hypertension. At surgery, a 4-cm mass was resected and found to contain cords and tubules of polygonal cells in a myxoid background. Limited areas of classical adrenocortical adenoma were detected in less than 20% of the tumour area. Lack of atypias and absence of mucin markers, together with an immunophenotype consistent with adrenal tumours (focal cytokeratin, vimentin, synaptophysin and alpha-inhibin immunoreactivities), led to a diagnosis of primary adrenocortical adenoma with an extensive pseudoglandular myxoid pattern. However, the differential diagnosis from metastatic well-differentiated adenocarcinomas, chordomas and retroperitoneal myxoid mesenchymal tumours (e.g. liposarcoma) may be difficult in the absence of a complete clinical history and a reliable immunoprofile. We strongly recommend staining of any myxoid or glandular tumour of the adrenal gland for alpha-inhibin and synaptophysin (probably the currently best characterised markers of adrenocortical origin) before considering alternative (probably more common) diagnoses of metastatic adenocarcinoma or retroperitoneal tumours localised to the adrenal gland.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.