When Symptomatic Treatment Becomes Antitumor Treatment for Vipoma: Opportunity for Frail Elderly Adults

To the Editor: An 82-year-old woman was admitted to the emergency department because of a fall with head injury and loss of consciousness. Her hospital record revealed a history of untreated urinary incontinence, dyslipidemia, and hypertension. During the previous 6 weeks, she had had untreated profuse diarrhea (>10 watery stools per day), a 10-kg loss of weight, and severe asthenia. On examination, she showed evidence of delirium with fluctuating level of consciousness and disorientation, ophthalmic zoster lesions, and severe dehydration. Laboratory tests showed hypokalemia 1.5 mmol/L (normal range 3.5–5.0 mmol/L), hypercalcemia 3.2 mmol/L (normal range 2.2–2.6 mmol/L), and acute renal failure. Her electrocardiogram showed inverted T waves and ST depression. Fluid and electrolyte replacement urgently performed under cardiac monitoring led to partial improvement of her condition. Thoracoabdominal computed tomography was performed to investigate the profuse diarrhea and showed a 7-cm hypervascularized tumor in the tail of the pancreas, with nodes of peritoneal carcinomatosis and lesions in the liver and lungs, and a bilateral pulmonary embolism. Emergency physicians consulted the mobile geriatric team, and she was admitted to the geriatric ward, because it was best suited to provide the comprehensive care required. Anticoagulant therapy was initiated, and in consultation with gastroenterologists, short-acting octreotide (somatostatin analogue) 100 μg twice a day for symptomatic diarrhea was initiated, leading to dramatic improvement. To implement the best care plan, comprehensive geriatric assessment was performed 3 weeks after admission.1, 2 Of the 10 standard domains assessed, six were impaired: mild cognitive impairment, mobility and balance disturbance, activity of daily living impairment, malnutrition, and social isolation. It was decided to investigate further her pancreatic tumor to determine the best treatment. Computed tomography–guided needle biopsy of the pancreatic mass diagnosed a well-differentiated neuroendocrine tumor. Chromogranin A was 143 ng/mL (normal <85 ng/mL) and vasoactive intestinal peptide (VIP) was 157 ng/L (normal 23–63 ng/L). Indium-111-octreotide scintigraphy showed an isolated strong uptake in the tail of the pancreas. A potential diagnosis of metastatic VIPoma was reached. The institutional tumor board chose palliative care for this frail 82-year-old woman and decided to switch to a long-acting somatostatin analogue for its antisecretory and antitumor effects. She died 5 months later, before progression of the tumor could be assessed. VIPoma is a rare functional neuroendocrine tumor of the pancreas (pNET) that secretes VIP. Although VIPoma is rare (estimated incidence 0.05–0.2/1,000,000 individuals), the incidence of NET is rising and could be more common in elderly adults.3-5 The typical clinical features of VIPoma result from excessive secretion of VIP and include large-volume secretory diarrhea, hypokalemia, and dehydration.3 Somatostatin analogues, which inhibit the release of neuroendocrine hormones, control hormone-related symptoms in up to 70% to 90% of symptomatic pNETs.6 Forty percent to 95% of VIPomas are metastatic at presentation, and the symptoms related to excessive secretion of VIP are more likely to shorten life expectancy than tumor growth.7 Thus, management of these tumors in frail elderly adults requires collaboration between endocrinologists and geriatricians to weigh the pros and cons of each therapeutic option. Two new therapeutic options are worth mentioning. First, two targeted therapies everolimus and sunitinib, have recently been approved for treatment of progressive metastatic pNET, although no evidence of efficacy in older adults is available.8 Second, somatostatin analogues, which have an excellent tolerance profile, have been shown to have an antitumor effect: in retrospective studies, stabilizing up to 50% of 60% of NET, and in two randomized controlled trials of octreotide long-acting release in metastatic midgut NET and nonfunctioning gastroenteropancreatic NET.6, 9, 10 Somatostatin analogues are thus recommended for antiproliferative purposes in nonresectable, slowly progressive, low proliferative NET.6, 8 Even though there is no evidence of efficacy in older adults, somatostatin analogues have been used in elderly adults for their antisecretory effects and could be a nonaggressive alternative treatment for frail elderly adults. In conclusion, this case illustrates that multidisciplinary collaboration enabled the choice of adequate treatment for this frail elderly woman. Moreover, a well-known symptomatic treatment has been proven to have an antitumor effect, increasing the spectrum of treatment options in this population. Conflict of Interest: All the authors declare no conflict of interest. Author Contributions: All the authors met the ICMJE criteria to be considered as authors. Sponsor's Role: No sponsor.

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