Nelson-Salassa Syndrome Progressing to Pituitary Carcinoma: A Case Report and Review of the Literature

Nelson-Salassa Syndrome (NSS) is a rare sequela of bilateral adrenalectomy as a treatment for persistent hypercortisolism in refractory Cushing disease (CD). Radiographic NSS has been observed in half of CD patients after adrenalectomy, yet often follows a mild course and rarely requires treatment. We present the case of a 44-year-old male with a history of CD who underwent primary treatments including transsphenoidal resection, radiotherapy, and bilateral adrenalectomy. He subsequently presented with acute vision loss and progressive somnolence. MRI revealed marked enlargement of an invasive sellar and suprasellar lesion exerting significant mass effect on the chiasm, and multiple new enhancing bony lesions. The patient was taken for emergent transsphenoidal resection and calvarial biopsy; visual function was restored postoperatively, and pathologic analysis confirmed pituitary carcinoma. While NSS typically follows an indolent course, pituitary carcinoma is a highly morbid metastatic disease, and has been theorized to occur at a higher frequency in the NSS population. We review all published cases of NSS to pituitary carcinoma progression, which further underscores the highly aggressive nature and considerable mortality of this patient cohort. Although mild, asymptomatic NSS is more commonly observed, symptomatic patients or those with rapid growth after adrenalectomy, should be targeted for routine close clinical follow-up and serial radiographic surveillance.