Rhabdoid adrenocortical carcinoma with brain metastasis: a case report

Abstract

Introduction: Adrenocortical carcinoma (ACC) is a rare disease with the incidence about 0,02-0,2% from all case of malignancies. The number of malignancies cases reported annually being about 0,7-2 cases per million people. ACC can be functional or nonfunctional tumors, being diagnosed incidentally because of loco-regional symptoms or distant metastases. The overall 5-year survival rate after diagnosis was 15% to 47% and there are no significant differences in survival based on patient age, gender or tumor functional status.Case Report: A 47 years old male came into emergency room with nauseous, vomiting, headache and weakness for 4 weeks. From the physical examination, there were slight hypertension and tenderness on left hypochondric region. Abdominal ultrasound showed a solid mass with central necrotic on upper left abdominal cavity. Then, contrast abdominal CT revealed a well defined heterogeneous solid mass, contrast enhancement with necrotic area that attached on the upper pole of left kidney suspicious on left adrenal organ. He was performed left radical adrenalectomy as initial treatment to determine the histopathology result and tumor staging. After surgery complains of his headache remained, thus head CT was performed and it concluded an intracranial metastatic process at right parietal region, left temporal brain parenchymal and cerebellum which pressing ventricle IV. Steroid and neurotrophic treatment were given by neurologic division. This patient was also consulted to neurology, hemato-oncology, neurosurgery and radiotherapy for further treatment.Conclusion: ACC is a progressive disease with a poor prognosis because of its initial presentation with advance disease (tumor burden or distant metastases), high incidence of local recurrence, and systemic therapy still controversial. The patient has to do a complete physical examination, imaging and laboratory work-up. Multimodality treatment with a multidisciplinary approach is needed to manage the patient for optimal outcome.