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ABSTRACT: Objective: To report the role of liver transplantation in the management of refractory carcinoid syndrome. Methods: We describe the clinical course, biochemical, radiographic, and histopathologic features of a patient with severe refractory carcinoid syndrome due to a metastatic well-differentiated enterochromaffin cell neuroendocrine tumor of the jejunum. We also review and summarize the literature on liver transplantation in this setting. Results: An 18-year-old patient presented with a 3-year history of progressive palpitations, flushing, abdominal pain, and diarrhea. Initial imaging studies demonstrated multiple cystic liver lesions; biochemistry confirmed elevated 24-hour urinary 5-hydroxy-indolacetic acid (5-HIAA) levels. She was managed with somatostatin analogues followed by a partial small-bowel resection. Five sessions of liver embolization and escalating somatostatin analogues failed to control her symptoms or normalize urinary 5-HIAA levels. Cross-sectional imaging detected disease only in the liver. Due to progressively worsening symptoms, liver transplantation was offered as a therapeutic option at age 21 years. One year following the procedure, her condition improved clinically and biochemically with minimal disease outside the liver. Conclusion: Liver transplantation represents a potential therapeutic option in selected patients with refractory carcinoid syndrome.

ABSTRACT: Objective: To report a rare case of malignant ovarian steroid cell tumor with androgen excess.Methods: We describe the clinical presentation and management of a malignant ovarian steroid tumor with severe hyperandrogenism in a postmenopausal woman.Results: An 83-year-old patient had a 6-year history of features of androgen excess. Biochemistry confirmed elevated testosterone and dehydroepiandrosterone sulfate; investigations identified a 4.5-cm adnexal mass that was resected, and the histopathology diagnosis was a malignant steroid cell tumor. Eight months later, disease recurrence prompted a second surgical intervention, and a 3.5-cm mass adherent to the right pelvic sidewall was removed. The patient was not able to tolerate mitotane due to severe side effects. Eighteen months later, the disease relapsed with recurrence of clinical and biochemical features of androgen excess.Conclusion: This case illustrates the challenges of diagnosis and management of malignant ovarian steroid cell tumors.Abbreviations: CT computed tomography; DHEAS dehydroepiandrosterone sulfate; GnRH gonadotropin-releasing hormone; MRI magnetic resonance imaging; SCT steroid cell tumor