Successful treatment of metastatic adrenocortical carcinoma in the spine: A case report and literature review

Abstract

Rationale: Adrenocortical carcinoma is a rare aggressive type of cancer whose prognosis is poor, particularly for metastatic entities. Metastatic adrenocortical carcinoma in the spine is a rare disease with no standard curative managements yet. The objective of this study is to report a very rare case of spinal metastases of adrenocortical carcinoma successfully managed by combination of cement augmentation, radiotherapy together with adjuvant programmed cell death 1 (PD-1) therapy. The management of these unique cases has yet to be well-documented.

Patient concerns: A 42-year-old woman presented with a 3-month history of continuous and progressive back pain. The patient, who had been diagnosed of right pheochromocytoma, received surgical treatment of right adrenalectomy 14 months ago in another hospital, followed by no further treatment.

Diagnosis: Magnetic resonance imaging of spine showed vertebral pathological fracture of L1, spinal cord compression secondary to the epidural component of the L1 mass, with increased metastatic marrow infiltration of the right L1 vertebral body, which presented as a solid tumor. Postoperative pathology confirmed the diagnosis of spinal metastases of adrenocortical carcinoma.

Interventions: The patient underwent cement augmentation via a posterior approach, radiotherapy, radiofrequency ablation of psoas major muscle occupying lesions, right chest wall, liver and kidney recess together with adjuvant PD-1 therapy.

Outcomes: The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 6-month and 1-year follow-up visit. There were no complications associated with the operation during the follow-up period.

Lessons: Combined efforts of specialists from orthopedics, urology, interventional radiology, radiotherapy, pathology, endocrinology, and medical oncology led to the successful diagnosis and management of this patient. Metastatic adrenocortical carcinoma of the spine, although rare, should be part of the differential diagnosis when the patient has a history of adrenal carcinoma and presents with back pain, myelopathy, or radiculopathy. We recommend the posterior approach for total excision of the spinal metastatic adrenocortical carcinoma when the tumor has caused neurological deficits. Osteoplasty by cement augmentation, radiotherapy, and targeted PD-1 therapy may also be good choices for treatment.

Figure 1

(A and B) Preoperative x-rays revealing vertebral fracture of L1 with high suspicion of metastatic spinal tumors.

Figure 2

(A–L) Preoperative sagittal magnetic resonance imaging scan revealing the density of soft tissue, obvious bony destruction in the L1, and spinal cord compression caused by metastatic adrenocortical carcinoma, with increased metastatic marrow infiltration of the vertebrae and paravertebral regions.

Figure 3

(A–F) Preoperative transverse magnetic resonance imaging images showing the metastatic adrenocortical carcinoma in L1 and spinal cord compression caused by metastatic adrenocortical carcinoma.

Figure 4

Positron emission tomography-computed tomography revealed malignant tumors of the L1 with paravertebral involvement.

Figure 5

(A and B) Posteroanterior (PA) and lateral x-ray image of the lumbar spine obtained postoperatively.

Figure 6

Pathologic histology of paraspinal tumors in the right psoas major. (A and B) Microphotography showing characteristic nests of tumor cells separated by vascular septa (Zellballen) with cells showing significant nuclear pleomorphism with prominent nucleoli (H&E, original magnification 100× and 200×). (C) Synaptophysin immunostaining shows strong, diffuse cytoplasmic staining in the tumor cells. (D) Melan-A immunostaining shows focally positive staining in the tumor cells. (E and F) EMA and α-inhibin immunostaining shows negative staining in the tumor cells. (G) Ki-67 immunostaining shows 30% Ki-67 positive cells. Ki-67 staining is localized in the tumor nuclei.

Figure 7

Pathologic histology of spinal metastatic adrenocortical carcinoma. (A and B) Microphotography showing characteristic nests of tumor cells separated by vascular septa (Zellballen) with cells showing significant nuclear pleomorphism with prominent nucleoli (H&E, original magnification 100× and 200×). (C) Synaptophysin immunostaining shows strong, diffuse cytoplasmic staining in the tumor cells. (D) Melan-A immunostaining shows focally positive staining in the tumor cells. (E–G) Chromogranin A, S100, and α-inhibin immunostaining shows negative staining in the tumor cells.