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Poorly Differentiated Neuroendocrine Carcinoma of Unknown Primary Site Presenting as Unexplained Hypoxemia: A Case Report and Literature Review FREE TO VIEW

Mohmmed Algamdi, MBBS
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Critical Care Western, University of Western Ontario, London Ontario, ON, Canada

Chest. 2015;148(4_MeetingAbstracts):525A. doi:10.1378/chest.2280686
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SESSION TITLE: Lung Cancer Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Patients with poorly differentiated neuroendocrine carcinoma (PDNEC) usually present with a locally advanced disease or a widespread metastasis.

CASE PRESENTATION: A 74-year-old female patient was found to have an incidental pelvic mass. Computed tomography (CT) scan showed small bilateral pulmonary nodules, liver lesions and a right-sided pelvic mass. Pelvic mass biopsy showed a metastatic adenocarcinoma with unknown primary site. After the second cycle of chemotherapy, the patient was admitted to our hospital with prerenal acute kidney injury secondary to decreased oral intake and diarrhea. During hospitalization, hypoxemia with partial response to O2 therapy was identified. Arterial blood gas showed a PaO2 of 51 mmHg, PaCO2 of 29 mmHg and alveolar-arterial gradient of 62 mmHg. Chest film, chest CT scan and ventilation-perfusion scan were unremarkable. Echocardiography revealed severe tricuspid regurgitation (TR) and thickening of the tricuspid valve (TV) leaflets (Fig 1) suggestive of carcinoid heart disease (CHD). Biochemical markers for carcinoid tumor were elevated and otreotide scintigraphy demonstrated liver, cardiac and pelvic mass uptake. Reexamination of the pathology slides revealed positive synaptophysin immunostain suggestive of a neuroendocrine differentiation (Fig 2) with a morphology indicative of a PDNEC. The patient passed away after a course of palliative therapy. She was not a candidate for systemic chemotherapy nor TV replacement due to progressive decline.

DISCUSSION: CHD in the setting of PDNEC is extremely rare. Right side heart failure and severe TR characterize severe CHD; both were present in our patient. Severe hypoxemia in the setting of CHD has been previously before. Blick et al1 and Mottram et al2 each described a patient with CHD in whom R-L shunt through a patent foramen ovale (PFO) was documented and successfully corrected by surgery. Hart et al3 reported a case of CHD with a R-L shunt due to a combined PFO and an intrapulmonary shunt.

CONCLUSIONS: PDNECs are rarely associated with secretory hormonal syndromes, however, symptoms of such could be the only clue to the diagnosis.

Reference #1: Blick DR, Zoghbi WA, Lawrie GM, Verani MS. Carcinoid heart disease presenting as a right to left shunt and congestive heart failure: successful surgical treatment. Am Heart J 1988; 115:201-203.

Reference #2: Mottram PM, McGaw DJ, Meredith IT, Peverill RE, Harper RW. Profound hypoxaemia corrected by PFO closure device in carcinoid heart disease. Eur J Echocardiogr. 2008 Jan;9(1):47-9.

DISCLOSURE: The following authors have nothing to disclose: Mohmmed Algamdi

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