SFEBES2021 Poster Presentations Neuroendocrinology and Pituitary (47 abstracts)
Three cases of metastatic spinal cord compression secondary to malignant pheochromocytoma and paraganglioma
Amber Khan , Ashutosh Kapoor , Prakash Manoharan , Amarjot Chander , Thomas Westwood , David Woolf , Jan Hoong Ho & Safwaan Adam
The Christie NHS Foundation Trust, Manchester, United Kingdom
Approximately 15-20% of phaeochromocytomas and paragangliomas (PPGL) are metastatic. Metastatic spinal cord compression (MSCC) has been reported infrequently. We present three cases of MSCC secondary to metastatic PPGLs. MSCC can occur in patients with PPGLs and should be considered in patients with either spinal symptoms or spinal metastases on imaging. Our series has shown radiotherapy followed by therapeutic MIBG to be effective in treating spinal metastases. Each case should be discussed with a multidisciplinary team comprising a spinal surgeon, nuclear medicine physician, radiologist, endocrinologist and medical and clinical oncologists. Furthermore, dexamethasone can precipitate PPGL crises so enhanced vigilance for this is required in patients with MSCC.
| Case 1 | Case 2 | Case 3 | |
| Patient characteristics | 57, male, SDHB, metastatic paraganglioma, referred for consideration of metiodobenzylguanidine (MIBG) treatment (progressive disease). | 63, female, no genetic mutation, diagnosis following initial presentation with COVID-19 infection and incidental adrenal mass. | 62, male, no genetic mutation, metastatic phaeochromocytoma treated with left nephrectomy and adrenalectomy, liver metastatectomy, and lung ablation. Referred for second opinion (progressive disease). |
| Spinal symptoms | Back ache. | None | None |
| Plasma metanephrines (<510.0 pmol/l) | 318.7 | 1051.0 | 202.0 |
| Plasma normetanephrines (<1180.0 pmol/l) | 1327.0 | >30000.0 | 18026.0 |
| Plasma 3-methoxytyramine (<180.0 pmol/l) | <75.0 | 12540.0 | 311.6 |
| Imaging | Right-sided L3 paraspinal mass with impending cord compression. MIBG and Ga-68 DotaPET avid. Widespread skeletal metastases and enlarged retroperitoneal mass. | Right adrenal phaechromocytoma, with left adrenal, spinal and pelvic metastases on MIBG. Grade 1B spinal cord compression at L2/3. | Multiple lung and liver lesions, and T3 metastasis on MIBG. Multiple spinal lesions with Grade 3 cord compression at T3 on MR spine. |
| Treatment and progress | Radiotherapy 20 Gy in 5# to L3 and MIBG therapy. Partial response. Normal mobility. | Radiotherapy 20 Gy in 5# to L2/3 and MIBG therapy (2 cycles, developed pancytopaenia). | Single fraction radiotherapy and MIBG therapy. Improved appearance of T3 and skeletal metastases. |
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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