Adrenocortical carcinoma with skeletal metastases in a postmenopausal woman

Abstract

Adrenocortical cancer is a very rare tumor with a poor prognosis. About half of them are hormone-secreting tumors. In most cases, hormonal investigations reveal an excess secretion of steroids, mostly cortisol and androgens. A 54-year-old lady presented with history of pain in left shoulder and leg for 6 months and features of virilization. CT-guided fine-needle aspiration cytology of an abdominal mass revealed the presence of a carcinoma of the left adrenal cortex. A whole-body radionuclide bone scan revealed increased uptake in the left clavicle and left femur. The patient has received palliative radiotherapy for the skeletal lesions and 3 cycles of palliative chemotherapy at present.

Uncommon Metastasizing Site of Adrenocortical Carcinoma

“…ACC is an uncommon malignant epithelial tumor of adrenal cortical cells with female predilection and commonly arises in the left adrenal gland [1,2,4]. The ACC can be functional with clinical manifestations of excess hormone secretion, and hypercortisolism is the most common presentation in these cases [4][5][6]. However, non-functional ACC is presented with non-specific symptoms and affects males more than females [6].…”

“…The ACC can be functional with clinical manifestations of excess hormone secretion, and hypercortisolism is the most common presentation in these cases [4][5][6]. However, non-functional ACC is presented with non-specific symptoms and affects males more than females [6]. Approximately 25%-35% of patients present with metastatic disease at the time of presentation [2,6].…”

“…The lung, liver, and regional lymph nodes are the common metastatic sites while bone involvement mainly in the pelvis is infrequent [1][2][3][4][5]. Patients with BM may present with bone pain, pathological fractures, hypercalcemia, and spinal cord and nerve root compressions [1,6]. In the present case, the patient was a 35year old who presented with ill-defined swelling over the right mandibular region with a history of left radical nephrectomy without any clinical symptoms of increased hormone secretion.…”

“…Surgical resection is the cornerstone of the management in ACC. If both the metastatic and primary lesions are to be removed entirely, then surgical resection may be considered at the metastatic site [6]. The chemotherapy drug, such as mitotane, plays a role in incompletely removed or metastatic ACC by impeding the steroid biosynthesis and damaging adrenocortical cells [5,11].…”

“…The chemotherapy drug, such as mitotane, plays a role in incompletely removed or metastatic ACC by impeding the steroid biosynthesis and damaging adrenocortical cells [5,11]. Mitotane can be used either in combination with other cytotoxic drugs or alone, with response rates ranging from 12% to 54% [6,11]. The tyrosine kinase inhibitors and rapamycin signaling components may also show some agreement in the management of ACC; however, their role is still uncertain [3,11].…”