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Chrisanthi Marakaki Third Department of Pediatrics, Attikon University Hospital, Rimini 1 Haidari, Athens, 12462Greece

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Anna Papadopoulou Third Department of Pediatrics, Attikon University Hospital, Rimini 1 Haidari, Athens, 12462Greece

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Olga Karapanou
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Dimitrios T Papadimitriou Third Department of Pediatrics, Attikon University Hospital, Rimini 1 Haidari, Athens, 12462Greece

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Kleanthis Kleanthous Third Department of Pediatrics, Attikon University Hospital, Rimini 1 Haidari, Athens, 12462Greece

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Anastasios Papadimitriou Third Department of Pediatrics, Attikon University Hospital, Rimini 1 Haidari, Athens, 12462Greece

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identified mutations, clinical and biochemical spectrum is variable (2) (3) . Despite the fact that CYP11B1 deficiency is the second more common form of CAH, no patient with this disorder has been reported from Greece. Herein, we describe a Greek patient

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Nikitas S Skarakis Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece

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Irene Papadimitriou Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece

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Labrini Papanastasiou Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece

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Sofia Pappa Department of Pathology, ‘G. Gennimatas’ General Hospital, Athens, Greece

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Anastasia Dimitriadi Department of Pathology, ‘G. Gennimatas’ General Hospital, Athens, Greece

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Ioannis Glykas Department of Urology, General Hospital of Athens ‘G Gennimatas’, Athens, Greece

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Konstantinos Ntoumas Department of Urology, General Hospital of Athens ‘G Gennimatas’, Athens, Greece

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Penelope Lampropoulou Department of Radiology, General Hospital of Athens ‘G Gennimatas’, Athens, Greece

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Theodora Kounadi Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece

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reported in Greece. Case presentation A 33-year-old Greek man was referred to the Endocrinology Unit of a tertiary care hospital for the diagnostic evaluation and treatment of hypertension despite receiving antihypertensive drugs. His medical

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S Livadas Endocrine Unit, Metropolitan Hospital, Athens, Greece

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I Androulakis Endocrine Unit, Metropolitan Hospital, Athens, Greece

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N Angelopoulos Endocrine Unit, Metropolitan Hospital, Athens, Greece

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A Lytras Endocrine Unit, Metropolitan Hospital, Athens, Greece

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F Papagiannopoulos Novo-Nordisk, Athens, Greece

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G Kassi Endocrine Unit, Alexandra Hospital, Athens, Greece

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, liraglutide was not available at the dose of 3 mg in Greece and available studies in PCOS have used the 1.8 regimen. No other drug was prescribed to the studied subjects. None of the patients used metformin 3 months prior to treatment. Informed consent was

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Evangelos Karvounis Department of Endocrine Surgery, ‘Euroclinic’ Hospital, Athens, Greece

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Ioannis Zoupas Department of Endocrine Surgery, ‘Euroclinic’ Hospital, Athens, Greece

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Dimitra Bantouna Private Practice, Patras, Greece

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Rodis D Paparodis Private Practice, Patras, Greece
Center for Diabetes and Endocrine Research, University of Toledo College of Medicine and Life Sciences, Toledo, Ohio, USA

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Roxani Efthymiadou PET-CT Department, Hygeia Hospital, Athens, Greece

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Christina Ioakimidou Department of Pathology

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Christos Panopoulos Department of Medical Oncology, ‘Euroclinic’ Hospital, Athens, Greece

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thyroid metastases, thus making the diagnostic approach an extremely challenging task. Case presentation A 78-year-old man presented to the emergency room (ER) of a rural Greek hospital complaining of a lump on the side of his neck. His medical

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Michail Katsamakas Surgical Oncology Department, Thessaloniki, Greece

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Eleni Tzitzili Surgical Oncology Department, Thessaloniki, Greece

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Maria Boudina Endocrinology Department, Thessaloniki, Greece

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Anastasia Kiziridou Pathology Department, Thessaloniki, Greece

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Rosalia Valeri Cytology Department, Theageneio Cancer Hospital, Thessaloniki, Greece

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Georgios Zafeiriou Surgical Oncology Department, Thessaloniki, Greece

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Alexandra Chrisoulidou Endocrinology Department, Thessaloniki, Greece

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Summary

We present two cases of thyroid sarcoidosis that were misdiagnosed as thyroid cancer. In the first patient, fine needle aspiration cytology (FNAc) of a suspicious thyroid nodule indicated the presence of papillary thyroid cancer, and the patient underwent thyroid surgery. However, histopathology identified a sarcoid granuloma, without any sign of malignancy. The second patient had a history of papillary microcarcinoma with suspicious lymph nodes diagnosed years after the initial diagnosis and was referred for assessment of cervical lymphadenopathy. Fine needle aspiration cytology (FNAc) of the suspicious lymph nodes erroneously indicated metastasis from thyroid cancer, and lateral modified lymph node dissection was performed, based on FNAc and ultrasonographic features. Histopathology excluded malignancy and identified non-caseating granulomas. Sarcoidosis of the thyroid may have a clinical presentation similar to well-differentiated thyroid carcinoma and, although rare, should be considered in the differential diagnosis, especially when other signs of the disease are already present. In these cases, FNAc provided a false diagnosis of papillary thyroid carcinoma and lymph node metastases that led to unnecessary surgery.

Learning points

  • Sarcoidosis may share clinical and ultrasonographic features with papillary thyroid carcinoma.

  • Fine needle aspiration cytology is helpful in the diagnosis of both conditions; however, the overlapping cytological characteristics may lead to erroneous diagnosis.

  • The present cases illustrate the importance of cytological identification of these difficult cases. Every piece of information provided by the clinician is essential to the cytologist.

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Paraskevi Kazakou Endocrine Unit, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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George Simeakis Endocrine Unit, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Maria Alevizaki Endocrine Unit, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Katerina Saltiki Endocrine Unit, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Summary

Medullary thyroid carcinoma (MTC) has a varying clinical course; distant metastases are frequently present even at diagnosis. We present two MTC cases with unusual metastatic sites. Two female patients are presented with slow progressive MTC. The first case developed distant metastases 23 years after diagnosis and underwent locoregional therapies. At the same time a breast mass developed representing MTC metastasis. Treatment with vandetanib led to long-term disease stabilization. The second patient is presented with metastases in the pancreas 13 years after diagnosis. Shortly, a painful mass developed in the mandible and metastasis of MTC was diagnosed. Disease progression was recorded 20 months after the initiation of local and systemic therapy. Such cases have only rarely been reported in the literature and highlight the need for prompt recognition of unexpected MTC metastases.

Learning points

  • Unusual sites of metastasis may appear in patients with medullary thyroid carcinoma (MTC) years after the initial diagnosis.

  • Although rare, unexpected MTC metastases highlight the need for prompt recognition and appropriate treatment.

  • Local recurrences accompanied by inappropriately low calcitonin levels should prompt further investigation for possible distant metastatic disease.

  • Systemic treatment with tyrosine kinase inhibitors may be effective even in patients with unusual metastases from MTC.

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Kanella Kantreva Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Stavroula A Paschou Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Katerina Stefanaki Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Kanella Pappa Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Paraskevi Kazakou Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Dionysios Vrachnis Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Evangelia Kavoura Pathology Department, IASO Hospital, Athens, Greece

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Kitty Pavlakis Pathology Department, IASO Hospital, Athens, Greece

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Eirini Giovannopoulou Department of Gynecological Oncology, IASO Hospital, Athens, Greece

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Konstantinos Lathouras Department of Gynecological Oncology, IASO Hospital, Athens, Greece

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Maria Alevizaki Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Katerina Saltiki Endocrine Unit and Diabetes Center, Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Summary

Struma ovarii is an ovarian teratoma that comprises 2–5% of all ovarian teratomas. Malignant transformation of struma ovarii occurs in less than 5% of all cases, and metastatic disease is even rarer. We report two cases initially diagnosed with benign struma ovarii that presented malignant transformation, specifically highly differentiated follicular carcinoma of the ovary (HDFCO), some years after the first diagnosis. Case 1 concerns a 37-year-old female featuring HDFCO of the right ovary with multiple metastatic foci, who was diagnosed with benign struma ovarii 14 years ago. Case 2 concerns a 26-year-old female diagnosed with HDFCO of the left ovary. This patient was initially diagnosed with benign struma ovarii 6 years ago that recurred 4 years after the diagnosis. Both patients were treated with surgery, adjunctive total thyroidectomy, and radioactive iodine (131I) therapy.

Learning points

  • Malignant transformation of struma ovarii is very rare (<5%).

  • Diagnosis of HDFCO without extra ovarian dissemination is difficult due to the resemblance of its histological appearance with normal thyroid tissue.

  • There is no consensus on the postoperative treatment of malignant struma ovarii (MSO). Clinical and histological features of MSO should be assessed for the postoperative treatment decisions.

  • TSH suppression and thyroglobulin level measurements are necessary for patient follow-up.

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Maria Pikilidou Hypertension Excellence Center, First Department of Internal Medicine
Second Department of Internal Medicine, Papanikolaou General Hospital, Thessaloniki, Greece

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Maria Yavropoulou Division of Endocrinology and Metabolism, AHEPA University Hospital, Thessaloniki, Greece

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Marios Katsounaros Second Department of Internal Medicine, Papanikolaou General Hospital, Thessaloniki, Greece

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Summary

We report a case of a female with hemihypertrophy, who developed five recurrences of pheochromocytomas until the age of 35. Timely follow-up of the patient's blood pressure assisted in early diagnosis and treatment of recurrent tumors.

Learning points

  • Recurrent benign pheochromocytomas should raise suspicion of a genetic syndrome.

  • A pheochromocytoma at a young age has a high propensity to recur and strict follow-up is mandatory.

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Marinos C Makris First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece
First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Konstantinos C Koumarelas First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Apostolos S Mitrousias First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Giannos G Psathas First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Athanasios Mantzioros First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Stratigoula P Sakellariou First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Panagiota Ntailiani First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Evripides Yettimis First Surgical Department of General Hospital of Athens ‘Georgios Gennimatas’, Alpha Institute of Biomedical Sciences (AIBS), Department of Pathology, Mesogeion 154, Athens 15669, Greece

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Summary

Until now, less than ten cases of extra-adrenal chromaffin cell tumors have been reported to be localized to the spermatic cord area. All published studies report benign tumors with a diameter <2–3 cm and no invasion of the testis. In this article, we present one case of a giant malignant paraganglioma in the testis of a patient who had initially been operated for a giant mass in the scrotum. The mass developed in approximately 4 months. This is the first study reporting the following findings: i) paraganglioma was found exclusively in the testis, invading the testicle and not the spermatic cord, ii) it was malignant with lung metastasis, and iii) its size was 17.5 cm×10 cm×9.5 cm. We present the first – giant – malignant paraganglioma. Moreover, it is the first case report of a paraganglioma in the testis.

Learning points

  • This is the first study reporting the following findings:

  • Paraganglioma found exclusively in the testis, invading the testicle and not the spermatic cord.

  • It is malignant with lung metastasis.

  • It is of the size 17.5 cm×10 cm×9.5 cm.

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Maria P Yavropoulou Division of Endocrinology and Metabolism, 1st Department of Internal Medicine, AHEPA University Hospital, Thessaloniki, Greece

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Christos Poulios Department of Pathology, Faculty of Medicine, Aristotle University of Thessaloniki, Greece

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Christoforos Foroulis Department of Thoracic Surgery, AHEPA University Hospital, Thessaloniki, Greece

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Symeon Tournis Laboratory of Research of Musculoskeletal System ‘Th. Garofalidis’, KAT Hospital University of Athens, Greece

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Prodromos Hytiroglou Department of Pathology, Faculty of Medicine, Aristotle University of Thessaloniki, Greece

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Kalliopi Kotsa Division of Endocrinology and Metabolism, 1st Department of Internal Medicine, AHEPA University Hospital, Thessaloniki, Greece

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Isaak Kessisoglou 3rd Department of Surgery, AHEPA University Hospital, Thessaloniki, Greece

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Pantelis Zebekakis Division of Endocrinology and Metabolism, 1st Department of Internal Medicine, AHEPA University Hospital, Thessaloniki, Greece

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Summary

Tumor-induced osteomalacia (TIO) is a rare form of hypophosphatemia usually caused by phosphaturic mesenchymal tumors (PMTs); the biologic behavior of PMTs is under investigation. Herein we present a case of TIO with a protracted course over 12 years leading to a fatal outcome. A 39-year-old man presented with weakness in 2004 and was found to have decreased serum phosphorus, phosphaturia and low levels of 1,25-dihydroxyvitamin D3. Four years later he developed a painful left calf mass. The lesion was resected, but recurred causing extreme pain and dysfunction. Radiological examination showed a large cluster of soft tissue tumors affecting all the muscle compartments of the calf and a smaller lesion inside the metaphysis of the tibia. Above-knee amputation was performed. Histological examination of all lesions showed a cellular spindle cell neoplasm with variously sized vessels, wide vessel-like spaces and scattered deposits of calcified extracellular material. The tumor infiltrated skeletal muscles, subcutaneous fat and the proximal end of the fibula. The tibial lesion had identical histology. Three years after the amputation the patient presented with cough and dyspnea. Radiological examination, followed by an open biopsy, showed that there were multiple metastatic nodules of PMTs in both lungs. Shortly after the diagnosis the patient died. This case illustrates that even benign cases of PMTs may lead to a fatal outcome and the classification of PMTs into benign and malignant should be reassessed in order to correspond to its biological behavior.

Learning points:

  • PMTs, aside from having locally aggressive behavior, may metastasize and cause death

  • PMTs may behave aggressively despite ‘benign’ histological findings

  • Accurate diagnosis of tumor-induced osteomalacia and patient management require a multidisciplinary approach

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