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Keiko Ohkuwa Department of Surgery, Ito Hospital, Tokyo, Japan

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Kiminori Sugino Department of Surgery, Ito Hospital, Tokyo, Japan

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Ryohei Katoh Department of Pathology, Ito Hospital, Tokyo, Japan

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Mitsuji Nagahama Department of Surgery, Ito Hospital, Tokyo, Japan

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Wataru Kitagawa Department of Surgery, Ito Hospital, Tokyo, Japan

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Kenichi Matsuzu Department of Surgery, Ito Hospital, Tokyo, Japan

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Akifumi Suzuki Department of Surgery, Ito Hospital, Tokyo, Japan

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Chisato Tomoda Department of Surgery, Ito Hospital, Tokyo, Japan

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Kiyomi Hames Department of Surgery, Ito Hospital, Tokyo, Japan

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Junko Akaishi Department of Surgery, Ito Hospital, Tokyo, Japan

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Chie Masaki Department of Surgery, Ito Hospital, Tokyo, Japan

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Kana Yoshioka Department of Surgery, Ito Hospital, Tokyo, Japan

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Koichi Ito Department of Surgery, Ito Hospital, Tokyo, Japan

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Introduction Parathyroid carcinoma (PC) is a rare endocrine tumor that accounts for <0.5–5% of patients with primary hyperparathyroidism ( 1 , 2 , 3 , 4 ). Complete surgical resection is the only curative treatment for PC. To prevent local

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Luigia Cinque Medical Genetics, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Angelo Sparaneo Laboratory of Oncology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Antonio S Salcuni Endocrinology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Danilo de Martino Thoracic Surgery, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Claudia Battista Endocrinology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Francesco Logoluso Department of Emergency and Organ Transplantation, Unit of Endocrinology, University Medical School of Bari ‘Aldo Moro’, Bari, Italy

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Orazio Palumbo Medical Genetics, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Roberto Cocchi Maxillofacial Surgery, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Evaristo Maiello Oncoematology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Paolo Graziano Pathology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Geoffrey N Hendy Departments of Medicine, Physiology and Human Genetics, McGill University and Metabolic Disorders and Complications, McGill University Health Centre Research Institute, Montreal, Quebec, Canada

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David E C Cole Departments of Laboratory Medicine and Pathobiology, Medicine and Genetics, University of Toronto, Toronto, Ontario, Canada

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Alfredo Scillitani Endocrinology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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Vito Guarnieri Medical Genetics, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (FG), Italy

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inactivating mutations of the MEN1 gene, encoding menin, an intracellular protein that interacts with transcription factors involved in cell cycle regulation and proliferation ( 8 ). Fifteen well-documented cases of parathyroid carcinoma (PC) and one atypical

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Sara Lomelino Pinheiro Serviço de Endocrinologia, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

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Ana Saramago Unidade de Investigação em Patobiologia Molecular, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

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Branca Maria Cavaco Unidade de Investigação em Patobiologia Molecular, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

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Carmo Martins Unidade de Investigação em Patobiologia Molecular, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

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Valeriano Leite Serviço de Endocrinologia e Unidade de Investigação em Patobiologia Molecular, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

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Tiago Nunes da Silva Serviço de Endocrinologia e Unidade de Investigação em Patobiologia Molecular, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

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Introduction Parathyroid carcinoma (PC) is a rare endocrine malignancy, accounting for less than 1% of all primary hyperparathyroidism (PHPT) cases ( 1 , 2 ). PC may occur sporadically or as part of genetic syndromes. The most frequent

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Qian Wang Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Jiacheng Wang Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Yunhui Xin Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Ziyang He Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Xiang Zhou Department of Pathology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Xing Liu Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Teng Zhao Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Lihan He Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Hong Shen Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Mulan Jin Department of Pathology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Bojun Wei Department of Thyroid and Neck Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

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Introduction Parathyroid carcinoma (PC) is an extremely rare disease, accounting for 0.005% of all malignancies ( 1 ) and representing approximately 1–5% of primary hyperparathyroidism cases ( 2 , 3 ). It is worth noting that the best

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Felix Haglund Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Gustaf Rosin Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Inga-Lena Nilsson Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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C Christofer Juhlin Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Ylva Pernow Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Sophie Norenstedt Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Andrii Dinets Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Catharina Larsson Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Johan Hartman Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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Anders Höög Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden
Department of Oncology–Pathology, Cancer Centre Karolinska, Department of Biosciences and Nutrition, Department of Molecular Medicine and Surgery, Department of Surgery #4, Karolinska Institutet, Stockholm, Sweden

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with an increased risk of cardiovascular and cerebrovascular diseases as well as cancer (1, 2, 3) . Parathyroid carcinomas are very rare (<1% of parathyroid tumours), but today's oncological treatment regimens have only a limited effect (4

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Boju Pan Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Anqi Wang Clinical Biobank, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Junyi Pang Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Yuhan Zhang Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Ming Cui Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Jian Sun Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Zhiyong Liang Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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) assay to examine and evaluate PD-L1 expression in these parathyroid tumors. Materials and methods Tumor samples and patient information In this analysis, we included 26 parathyroid carcinoma samples, including two primary foci without

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Sara Storvall Department of Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Helena Leijon Department of Pathology and Huslab, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Eeva Ryhänen Department of Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Johanna Louhimo Department of Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Caj Haglund Department of Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Camilla Schalin-Jäntti Department of Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Johanna Arola Department of Pathology and Huslab, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Introduction Primary hyperparathyroidism (PHPT) is a common endocrine disease usually caused by a parathyroid adenoma (in 85% of cases) or by glandular hyperplasia (15% of cases). Parathyroid carcinoma is only rarely the underlying cause (PC

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Elizaveta Mamedova Department of Neuroendocrinology and Bone Diseases, Endocrinology Research Center, Moscow, Russian Federation

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Natalya Mokrysheva Department of Parathyroid Diseases, Endocrinology Research Center, Moscow, Russian Federation

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Evgeny Vasilyev Department and Laboratory of Inherited Endocrine Disorders, Endocrinology Research Center, Moscow, Russian Federation

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Vasily Petrov Department and Laboratory of Inherited Endocrine Disorders, Endocrinology Research Center, Moscow, Russian Federation

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Ekaterina Pigarova Department of Neuroendocrinology and Bone Diseases, Endocrinology Research Center, Moscow, Russian Federation

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Sergey Kuznetsov Department of Surgery, Endocrinology Research Center, Moscow, Russian Federation

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Nikolay Kuznetsov Department of Surgery, Endocrinology Research Center, Moscow, Russian Federation

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Liudmila Rozhinskaya Department of Neuroendocrinology and Bone Diseases, Endocrinology Research Center, Moscow, Russian Federation

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Galina Melnichenko I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation
Institute of Clinical Endocrinology, Endocrinology Research Center, Moscow, Russian Federation

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Ivan Dedov Endocrinology Research Center, Moscow, Russian Federation

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Anatoly Tiulpakov Department and Laboratory of Inherited Endocrine Disorders, Endocrinology Research Center, Moscow, Russian Federation

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typical onset in early adulthood, but it can occur at any age, and in about 15% of cases is caused by parathyroid carcinoma ( 4 , 7 ). PHPT is rarely if ever the first manifestation of MEN2A ( 5 ). The typical features of PHPT in MEN4 have not been

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Maria Mizamtsidi Department of Endocrinology, Diabetes and Metabolism, Hellenic Red Cross Hospital, Athens, Greece

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Constantinos Nastos Second Department of Surgery, Endocrine Surgery Unit, Aretaieion University Hospital, National and Kapodistrian University of Athens, Athens, Greece

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George Mastorakos Unit of Endocrinology, Diabetes and Metabolism, Aretaieion University Hospital, National and Kapodistrian University of Athens, Athens, Greece

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Roberto Dina Department of Pathology, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

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Ioannis Vassiliou Second Department of Surgery, Endocrine Surgery Unit, Aretaieion University Hospital, National and Kapodistrian University of Athens, Athens, Greece

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Maria Gazouli Department of Basic Medical Sciences, Laboratory of Biology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Fausto Palazzo Department of Thyroid and Endocrine Surgery, Imperial College London, London, UK

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glands (10%), double adenomas (2–5%) or rarely parathyroid carcinomas (<1%) ( 1 , 2 ). The wide spectrum of disease presentation is very important as different management strategies are in demand. There are data implicating specific genetic pathways in

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Filomena Cetani Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Chiara Banti Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Elena Pardi Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Simona Borsari Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Paolo Viacava Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Paolo Miccoli Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Liborio Torregrossa Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Fulvio Basolo Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Maria Rosa Pelizzo Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Massimo Rugge Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Gianmaria Pennelli Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Guido Gasparri Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Mauro Papotti Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Marco Volante Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Edda Vignali Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Federica Saponaro Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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Claudio Marcocci Department of Clinical and Experimental Medicine, Section of Pathology, Department of Surgical, Surgery Unit, Medicine, General Surgery 3 and Esophageal Surgery, Clinical and Biological Sciences, Department of Oncology

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histological diagnosis of parathyroid carcinoma (PC) is currently restricted to lesions showing unequivocal extra-parathyroidal growth, as evidenced by perineural invasion, full thickness capsular invasion with growth into adjacent tissues, extratumoral

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