Solitary neuroendocrine carcinoma of the heart: a case report

Abstract

Background Cardiac tumours are of rare incidence and usually occur in the form of secondary tumours. Most metastatic tumours are melanomas, sarcomas, lung, and haematological malignancies. Neuroendocrine carcinomas (NECs) of the heart are extremely unusual. This case report demonstrates a solitary high-grade NEC of the heart with an individual therapy strategy and follow-up. Case summary A 50-year-old gentleman presented with a 2 days history of recurrent episodes of chest pain. Echocardiography, computed tomography, and magnetic resonance imaging revealed tumorous lesions of the ventricles and aortic valve with large circular pericardial effusion. Histopathology results of the biopsy revealed a poorly differentiated small cell tumour of the neuroendocrine type. Despite further investigations with multiple imaging modalities and laboratory, no primary was found. Chemotherapy was initiated but size progression of the tumour was detected. As no other tumorous lesions were detected and resection was not possible because of the tumour complexity, decision on heart transplantation was made. However, due to the necessary immunosuppression after the heart transplantation, multiple metastasis where discovered in the course of treatment. Discussion The presence of a NEC in the heart without evidence of any other metastasis or evidence of primary tumour in other organs is clinically unique. For this individual case, heart transplantation was the therapy of choice due to tumour progression under chemotherapy and lacking possibility of resection, as no other suspect lesion was found other than the ones found in the heart. However, the risk of exacerbation of undiscovered micrometastases under necessary immunosuppression following the heart transplantation should be considered.

Cardiac Metastases in Patients with Neuroendocrine Tumours: Clinical Features, Therapy Outcomes, and Prognostic Implications

“…Small bowel NET made up 84% ( n = 21) of all patients with CM in our series, and over 90% patients ( n = 23) had concomitant liver metastases. All CM were derived from well-differentiated NET in our series as well as in other reported cases (Table 5), with G1 and G2 NET being the most predominant categories, but a very rare case of poorly differentiated NEN CM was also reported previously [20]. The majority of NET ( n = 20, 80%) presented with carcinoid syndrome around the time of CM diagnosis, with elevated CgA and 5-HIAA levels, which is consistent with previous reported cases [21].…”

“…Even though management guidelines for the CM in NET have been published by ENETS [6], comprehensive description and experience are still lacking. Most literature studies regarding CM in NET over the past 10 years are small case series reports [7,8,[18][19][20][21][22][23][24][25][26][27][28][29][30][31][32] (Table 5) that Compared to other cancer types, the incidence of CM in NET was relatively lower [9,[33][34][35]. Although no malignant tumours are known preferentially to involve the heart, some do involve the heart more often than others.…”

A primary neuroendocrine tumor of the left ventricle presenting with diarrhea—an unusual experience and literature review

“…Treatment for high-grade neuroendocrine carcinoma usually consists of surgical resection and platinum-based [12]. Literature review indicated that alternative therapies (chemotherapy and radiation therapy) were considered when there was no possibility of resection; however, the prognosis was still poor [6,7,9] due to remote metastasis. On the other hand, owing to the necessity of inducing immunosuppression, heart transplantation should be avoided in patients with systemic malignant tumor [6].…”

“…Literature review indicated that alternative therapies (chemotherapy and radiation therapy) were considered when there was no possibility of resection; however, the prognosis was still poor [6,7,9] due to remote metastasis. On the other hand, owing to the necessity of inducing immunosuppression, heart transplantation should be avoided in patients with systemic malignant tumor [6]. The tumor, which was histologically well-differentiated, was completely removed in the present case and the patient showed good outcome.…”

Neuroendocrine carcinoma of the heart base in a dog: A case report

“…The liver, gallbladder, kidneys, gastrointestinal tract, nasal cavities, apocrine glands of the anal sac, mammary glands and skin were reported to be the place of occurrence of neuroendocrine carcinomas in dogs (Sako et al 2005;Birettoni et al 2008;Joiner et al 2010;Ogawa et al 2011;Nakahira et al 2015;Morgan et al 2019;Sozmen et al 2020). Case reports, which describe the occurrence of neuroen-docrine carcinomas of the heart, exist in human literature (Guajardo-Salinas et al 2013;Wißt et al 2018). To the best of our knowledge, no studies nor case reports have been published about a neuroendocrine carcinoma of the heart in a dog, unless these tumours originated from paraganglion cells of the aortic body.…”