Neoplastic pericarditis as the initial manifestation of a papillary thyroid carcinoma Full Terms & Conditions of access and use can be found at https://www.tandfonline.com/action/journalInformation?journalCode=iups20 Upsala Journal of Medical Sciences ISSN: 0300-9734 (Print) 2000-1967 (Online) Journal homepage: https://www.tandfonline.com/loi/iups20 Neoplastic pericarditis as the initial manifestation of a papillary thyroid carcinoma Nikolaos Tsoukalas, Ioannis D. Kostakis, Stamatina Demiri, Georgios Koumakis, Vasileios Barbounis, Kalypso Barbati & Anna Efremidis To cite this article: Nikolaos Tsoukalas, Ioannis D. Kostakis, Stamatina Demiri, Georgios Koumakis, Vasileios Barbounis, Kalypso Barbati & Anna Efremidis (2013) Neoplastic pericarditis as the initial manifestation of a papillary thyroid carcinoma, Upsala Journal of Medical Sciences, 118:3, 196-198, DOI: 10.3109/03009734.2013.801541 To link to this article: https://doi.org/10.3109/03009734.2013.801541 © Informa Healthcare Published online: 23 May 2013. 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KOSTAKIS2, STAMATINA DEMIRI3, GEORGIOS KOUMAKIS3, VASILEIOS BARBOUNIS3, KALYPSO BARBATI4 & ANNA EFREMIDIS3 1Department of Medical Oncology, ‘401’ General Military Hospital, Athens, Greece, 2Second Department of Propedeutic Surgery, ‘Laiko’ General Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece, 3Second Department of Medical Oncology, ‘Agios Savvas’ Anticancer Hospital, Athens, Greece, and 4Department of Pathology, Hellenic Red Cross Hospital ‘Korgialeneio-Benakeio’, Athens, Greece Abstract Neoplastic pericarditis represents approximately 5%–7% of the cases with acute pericarditis and is rarely the initial manifestation of malignancy. The most common cause is lung cancer, followed by breast cancer, lymphomas, leukemia, and esophageal cancer. Neoplastic pericardial disease is extremely rare in thyroid cancer, especially as the first manifestation. Here, we present a papillary thyroid carcinoma that was manifested with pericarditis and cardiac tamponade in a 49-year-old female. Key words: Cardiac tamponade, neoplastic pericarditis, pericardial effusion, thyroid cancer Introduction Neoplastic pericarditis represents approximately 5%–7% of the cases with acute pericarditis (1-4). Theoretically, any malignant tumor may cause pericarditis/pericardial effusion (1,2) through direct extension or metastasis via lymphatic or blood vessels into the pericardium (3,5). However, the most common malignancy causing pericarditis/pericardial effusion is lung cancer, followed by breast cancer, lymphomas, leukemia, and esoph- ageal cancer. Primary neoplasms of the pericardium, such as mesothelioma, are very rare (1-3,5). Only few cases of neoplastic pericarditis in patients with thyroid cancer have been reported in the literature. Here, we present a case of papillary thyroid carcinoma that was manifested with pericarditis and cardiac tamponade. Case report A 49-year-old female, who was a heavy smoker, but with negligible previous medical history, was admitted to a hospital due to stridor, dyspnea, cough, and hoarseness. Physical examination revealed no patho- logical signs, blood tests were normal, and the patient was treated with corticosteroids without a definite diagnosis. Ten days later, she developed epigastric pain that radiated to the left scapula and the left supraclavicular fossa. She was admitted to a hospital, where an echocardiogram and a chest CT scan revealed the presence of pericardial effusion (Figure 1). During her hospitalization, she developed cardiac tamponade, for which she urgently underwent pericardiocentesis. Blind biopsies were taken from the pericardium and the upper lobe of the left lung, which showed malignant cells forming papillae and invading lymphatic and blood vessels (Figure 2A). However, it was not possible to identify the primary origin of the neoplasm, despite the thorough investigation with abdominal CT scan and technetium-99m-MDP bone scan. In addition, blood tests were normal, apart from the values of CEA (20.6 ng/mL (normal value: <4.7 ng/mL)) and CA-125 (341.4 IU/mL (normal Correspondence: Nikolaos Tsoukalas, MD, MSc, Medical Oncologist, Department of Medical Oncology, ‘401’ General Military Hospital, 10-12 Gennimata N. Street, 11524 Ampelokipi, Athens, Greece. Fax: +30 2107494095. E-mail: tsoukn@yahoo.gr (Received 16 March 2013; accepted 29 April 2013) ISSN 0300-9734 print/ISSN 2000-1967 online � 2013 Informa Healthcare DOI: 10.3109/03009734.2013.801541 http://informahealthcare.com/journal/ups mailto:tsoukn@yahoo.gr value: <34 IU/mL)). The patient received one cycle of chemotherapy with carboplatin 5AUC, docetaxel 60 mg/m2, and bevacizumab 5 mg/kg for a neoplasm of unknown primary origin, which improved her symptoms. Afterwards, she was admitted to our department for further investigation and treatment. The physical examination revealed a palpable nodule of the right lobe of the thyroid gland, and the ultrasound dem- onstrated diffuse heterogeneity of the same lobe. Nevertheless, the ultrasound failed to reveal any spe- cific suspicious area, and the serum concentration of thyroglobulin was normal (17.7 ng/mL (normal value: <78 ng/mL)). The cytological examination after a blind fine-needle aspiration biopsy of the right lobe of the thyroid gland revealed the presence of a papillary thyroid carcinoma. Subsequently, a cervical MRI was performed, demonstrating extensive lymph- adenopathy. The patient underwent total thyroidec- tomy and bilateral lymph node dissection. The histological examination verified the presence of a stage IVC (T3N1bM1) papillary carcinoma with high-grade (grade III) malignancy and sporadic ana- plastic changes, lymphovascular and perineural inva- sion, and metastatic infiltration of the cervical lymph nodes (Figure 2B). A reexamination of the pericardial fluid from the pericardiocentesis revealed the expres- sion of thyroglobulin by the malignant cells. An I131 scan was also performed, but without demonstrating abnormal I131 uptake. The patient’s treatment was continued with the use of the same chemotherapy regimen for a total of six cycles without any major adverse events and with excellent tolerance. After the completion of chemotherapy, a FDG- PET scan was performed with negative results. Afterwards, the follow-up was based on physical examination, blood tests, and imaging examinations. Unfortunately, the disease relapsed six months later with a very aggressive clinical course due to lung metastases and cardiac tamponade. It was not possi- ble to administer any anticancer treatment at that time, and she passed away due to respiratory and cardiac failure. Discussion As mentioned previously, approximately 5%–7% of pericardial effusions have a malignant origin (1-4). Most patients with neoplastic pericardial disease have an already diagnosed malignancy (3). Acute pericar- ditis or pericardial effusion is the initial manifestation of malignancy in about 4% of the cases with pericar- dial disease (1,3), but this percentage rises to approx- imately 20% in large, symptomatic effusions without obvious origin (1,5), as in our case. Risk factors for malignant etiology are cardiac tamponade at presen- tation, as in our case, recurrent or incessant pericar- ditis, no response to non-steroidal anti-inflammatory drugs, and a history of malignancy (1-3). Cytological examination of pericardial fluid or pericardial biopsy is required for a diagnosis to be made (1-3,5). However, pericardial effusion is detected in only A B Figure 2. Histological images with malignant cells from the papillary thyroid carcinoma. A: Biopsy of the pericardium (hematoxylin and eosin stain, 100�); B: Histological examination of the thyroid gland (hematoxylin and eosin stain, 200�). Figure 1. Chest CT scan in which the pericardial effusion is revealed. Thyroid cancer presented with neoplastic pericarditis 197 12%–25% of patients with metastasis to the pericar- dium, but cardiac tamponade, which developed in our patient, is relatively rare among them (5). Neoplastic pericardial disease is extremely rare in thyroid cancer, especially as the initial manifestation. Several cases have been reported in the literature (6-16). Neoplastic pericarditis (6,9), massive pericar- dial effusion (13), or cardiac tamponade (7,8,10,12,16) have been reported as the first manifestation of a previously occult thyroid cancer. On the other hand, malignant pericardial effusion (14,15) or cardiac tam- ponade (11) have also been diagnosed in patients with already diagnosed thyroid cancer. Most cases have pertained to papillary carcinoma (7,9-11,14-16), as in our patient, whereas a follicular (13) and a mucin- producing carcinoma (8) have also been described as causes of neoplastic pericardial disease. Conclusions Neoplastic pericardial disease is most commonly caused by lung cancer, followed by breast cancer, lymphomas, leukemia, and esophageal cancer (1-3,5), and is rarely the first manifestation of malig- nancy (1,3). However, thyroid cancer, and especially papillary carcinoma, should be included in the dif- ferential diagnosis of pericarditis/pericardial effusion. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. References 1. Imazio M, Brucato A, Derosa FG, Lestuzzi C, Bombana E, Scipione F, et al. 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