Session: MON 37-82-Pheochromocytoma & Paraganglioma
Poster Board MON-49
Malignant pheochromocytoma (MPC) is a neuroendocrine tumor that originates from chromaffin cells and it has been shown to secrete a variety of bioactive neuropeptides and cytokines other than catecholamines (CA), resulting in unusual clinical manifestations such as high-fever and cytokine storms. While surgical debulking of primary tumor as well as removal of metastases in combination with neoadjuvant therapy including radiometabolic and/or chemotherapeutic treatments is recommended, the complexity of the clinical manifestations due to cytokine production or CA crisis upon a tumor collapse could be critical for MPC management. Here we describe a young case of IL-6-producing MPC who presented severe fever, pain and weight loss with bone marrow metastases and the multimodal preoperative treatment by intravenous infusion of both phentolamine and octreotide was useful for the safe management of CVD therapy and subsequent surgical debulking of primary tumor.
The patient, a 39-year-old woman without any disease in past history, exhibited the walking difficulty because of severe waist and back pain, and then was admitted to our hospital. She showed a high-spiky fever (39.5°C) with normal blood pressure (122/68 mmHg). Laboratory data displayed an elevated C-reactive protein (23.3 mg/dl), IL-6 (198 pg/mL) and anemia (Hb 5.6 mg/dl), possibly due to chronic disorders. CT scan showed the large left adrenal tumor with a size of 7.0×10cm and bilateral pleural effusion. The urinary normetanephrine (UNMN) as well as blood noradrenaline level was elevated (approximately more than 10-fold to normal range) and consistently, MIBG scintigraphy and FDG-PET scan showed abnormal accumulations in the left adrenal tumor and multiple-bone lesions. Bone marrow biopsy revealed the extensive replacement of marrow by chromogranin-positive tumor cells stained with IL-6, diagnosed as IL-6 producing MPC with bone marrow metastases. To improve her general condition and hormone/IL-6-mediated symptoms, she was treated with continuous intravenous infusion of phentolamin, an alpha-adrenergic receptor blocker, and octreotide, increasing the doses of them until her symptoms and inflammatory markers were controlled. Indeed she recovered very well after 2 months treatment, and eventually fifth cycles of CVD therapy could be performed without any problems, resulting in tumor regression and significant decrease of UNMN. Furthermore, primary tumor was surgically removed and immunohistological and in vitro analysis using by tumor primary culture demonstrated that SSTR2 and IL-6 were expressed and they were downregulated by phentolamin and octreotide.
Thus, we report a case of IL-6-producing MPC who had a good response to multimodal therapy with intravenous administration of phentolamine and octreotide, leading to the safe chemotherapy and surgical debulking.
Nothing to Disclose: TT, NH, HK, TY, AS, AN, SH, IS, HN, SS, KS, IT, KY, TT
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