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Endocrine Abstracts (2021) 73 EP126 | DOI: 10.1530/endoabs.73.EP126

1Clinic for Endocrinology, Diabetes and Diseases of Metabolism, University Clinical Center of Serbia, Department for Neuroendocrine Tumors and Hereditary Cancer Syndromes, Belgrade, Serbia; 2Institute of Oncology and Radiology of Serbia, Belgrade, Serbia, Belgrade, Serbia; 3Department for Radiology and Magnetic Resonance Imaging, University Clinical Centre of Serbia, Belgrade, Serbia, Belgrade, Serbia; 4Department for Pathohistology, University Clinical Centre of Serbia, Belgrade, Serbia, Belgrade, Serbia


Introduction

Merkel cell carcinoma-MCC) is a rare, aggressive, neuroendocrine skin tumor with a poor prognosis. Chemotherapy (CT) and/or local/locoregional radiotherapy (LRRT) are first-line MCC therapies, but disseminated/locally advanced MCCs often become resistant to this treatment. The use of programmed cell death receptor1/programmed cell death ligand 1 (PD-1/PD-L1) monoclonal antibodies is a new type of therapy for MCC. While PD-1 monoclonal antibody (pembrolizumab) is registered for the treatment of various types of aggressive tumors, PD-L1 monoclonal antibody (avelumab) is registered exclusively for the treatment of locally advanced/metastatic MCC. Significant efficacy of these drugs has been shown by prolonged stabilization or partial regression (PR) of the disease in 30–60% of patients with MCC.

The aim

A case report of a patient with locally advanced MCC who developed tumor hyperprogression during immunotherapy.

Case presentation

A 47-year-old patient was diagnosed with MCC (Ki67 index 97%) in the subcutaneous tissue of the left pectoral and axillary region, size 9×10×10 cm. The tumor was inoperable, and the diagnosis was made by biopsy. Treatment started with 4 cycles of HT (etoposide–platinum), followed by LRRT and 2 more cycles of HT, and the effect of therapy was PR (tumor reduction by 90%). One month later, the disease progressed – several confluent tumors with a total size of 5×2×2 cm were diagnosed outside the field of LRRT (left hemitorax and hemiabdominal region). Pembrolizumab was administered in 3 doses, after which the disease progressed: multiple confluent tumors in the left hemitorax and hemiabdomen, size 11×7×9 cm. Retreatment with the same HT was performed in 3 cycles, which was complicated by significant myelotoxicity (pancytopenia grade III–IV) and sepsis. Because of this adverse event, further application of HT is abandoned, although PR of tumors has occurred (three tumors, size: 4×3 cm, 4×4 cm and 2×1.5 cm). After 3 doses of avelumab, a rapid tumor growth was registered (one tumor size 9×7 cm-increase by 120% in comparison to pre-avelumab size and second tumor size 12×8 cm-increase by 90%). An emergency LRRT was performed in order to stop the further tumor growth, which threatened to exulcerate. Three months later, the patient died.

Conclusion

Unlike pseudoprogression, which occurs due to immune cell infiltration of the tumor and is followed with tumor shrinkage (relatively common during immunotherapy), tumor hyperprogression during immunotherapy is a rare phenomenon and has not been described in patients with MCC.

Volume 73

European Congress of Endocrinology 2021

Online
22 May 2021 - 26 May 2021

European Society of Endocrinology 

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