A Case of Malignant Metastatic Pheochromocytoma After Eight Years of Primary Diagnosis

Abstract

We report a case of a 66-year-old female who presented to the hospital with abdominal discomfort for 3 months. Work-up revealed a 2.4 cm mass in the right adrenal gland. A laparoscopic resection of the adrenal mass was performed and the histopathology was consistent with a pheochromocytoma. Patient was under active surveillance for 8 years, until she developed local recurrence in the right adrenal bed. A right adrenal bed resection and right nephrectomy were performed. Although the tumor margins were positive, none of the sampled lymph nodes (0/6) were positive for metastasis. Patient refused any adjuvant therapy, and was discharged on surveillance from the hospital. A year later, patient was found to have metastatic disease involving her spine, iliac bones, bilateral hips and right dome of the diaphragm. Patient was offered a metaiodobenzylguanidine scan, and positive subsequent treatment with radioactive iodine was discussed with her. However, she denied any further intervention and was made hospice.

Keywords: MIBG; Paragangliomas; Pheochromocytomas; Sunitinib.

Figure 1

Right adrenal bed resection compatible with recurrent malignant pheochromocytomas. (A) × 100: microscopically, the tumor cells are arranged in well defined nests (“Zellballen”) bound by delicate fibrovascular stroma; (B) × 200: higher power shows amphophilic cytoplasm with a somewhat fibrillary cytoplasm.

Figure 2

Magnetic resonance imaging of the abdomen showing metastasis to right hip.

Figure 3

Magnetic resonance imaging of the abdomen showing (A) empty right adrenal and renal fossa and (B, C) metastasis seen in the vertebrae.

Figure 4

(A) Multilevel-multifocal osseous metastases particularly within thoracolumbar spine with slight epidural extension at T-7 and mild epidural disease at T-10 without cord compression. (B) Right retrocrural/paraspinal metastasis is seen at T-12 and L-1 level measuring about 1.5 cm.

Figure 5

Multiple predominantly osseous metastasis with metastasis in the right iliac bone, increased conspicuity of several soft tissue nodules in the right adrenalectomy bed consistent with recurrent neoplasm, a superior structure measuring 3.4 × 2.6 cm not well defined from adjacent unopacified liver is seen, the right diaphragmatic crus and the unopacified IVC and an adjacent posterior nodule measuring 3 × 1.6 cm is noted.