A 77-year-old woman with heart failure was admitted to our hospital. Computed tomography (CT) of the chest revealed an anterior mediastinal tumor. CT-guided biopsy revealed a malignant nonepithelial tumor of unknown origin. She was not treated with chemotherapy or radiotherapy because of her poor clinical condition. She died 33 days after admission. Following autopsy, we confirmed that the mediastinal tumor had infiltrated the large blood vessels. After final histological examination, undifferentiated pleomorphic sarcoma was diagnosed. Primary mediastinal sarcomas are very rare; clinicians should be aware of their possibility because some cases may progress rapidly as evidenced in this case

Arao, Tsuyoshi

Igarashi, Akira

Inoue, Sumito

Nakano, Hiroshi

Sato, Kento

Sato, Masamichi

Watanabe, Masafumi

Yamauchi, Keiko

English

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EXCLI Journal 2020;19:1161-1165 – ISSN 1611-2156 Received: July 22, 2020, accepted: August 03, 2020, published: August 17, 2020   1161 Case report: UNDIFFERENTIATED PLEOMORPHIC SARCOMA IN THE  ANTERIOR MEDIASTINUM WITH A RAPIDLY PROGRESSIVE COURSE: A CASE REPORT  Masamichi Sato1, Sumito Inoue1,*, Tsuyoshi Arao2, Akira Igarashi1, Keiko Yamauchi1,  Kento Sato1, Hiroshi Nakano1, Masafumi Watanabe1  1 Department of Cardiology, Pulmonology, and Nephrology, Yamagata University Faculty of Medicine, Yamagata, Japan 2 Internal Medicine, Shiseido General Hospital, Yamagata, Japan  * Corresponding author: Sumito Inoue, Department of Cardiology, Pulmonology, and Nephrology, Yamagata University Faculty of Medicine, 2-2-2 Iida-Nishi, Yamagata  990-9585, Japan, Tel: +81-23-628-5302; Fax: +81-23-628-5305,  E-mail: sinoue@med.id.yamagata-u.ac.jp  http://dx.doi.org/10.17179/excli2020-2694  This is an Open Access article distributed under the terms of the Creative Commons Attribution License  (http://creativecommons.org/licenses/by/4.0/).  ABSTRACT A 77-year-old woman with heart failure was admitted to our hospital. Computed tomography (CT) of the chest revealed an anterior mediastinal tumor. CT-guided biopsy revealed a malignant nonepithelial tumor of unknown origin. She was not treated with chemotherapy or radiotherapy because of her poor clinical condition. She died 33 days after admission. Following autopsy, we confirmed that the mediastinal tumor had infiltrated the large blood vessels. After final histological examination, undifferentiated pleomorphic sarcoma was diagnosed. Primary me-diastinal sarcomas are very rare; clinicians should be aware of their possibility because some cases may progress rapidly as evidenced in this case.  Keywords: Mediastinal tumor, undifferentiated pleomorphic sarcoma, computed tomography-guided biopsy    INTRODUCTION There are various histological types of mediastinal tumors; however, sarcoma is rarely seen in this location. Pleomorphic sar-coma is a soft tissue malignancy comprising several types of histologically undifferenti-ated atypical cells. Herein, we present an au-topsy case of pleomorphic sarcoma that de-veloped rapidly in the anterior mediastinum.  CASE REPORT The patient was a 77-year-old woman who presented with a 1-month history of shortness of breath on exertion and palpita-tions. Her medical history was notable for di-abetes, hypertension, hyperlipidemia, and subarachnoid hemorrhage. Her body mass in-dex was 34.7 kg/m2. She was afebrile with blood pressure 109/71 mmHg, heart rate 92 beats/min, and SpO2 96% on room air. Her physical examination was notable for bilateral lower extremity edema. No heart murmur nor abnormal breath sounds were auscultated. The conjunctiva, abdomen, and skin were normal. Neurological abnormalities were nor observed. Peripheral blood counts, including white blood cell fraction, were within normal limits. The lactate dehydrogenase (LDH) EXCLI Journal 2020;19:1161-1165 – ISSN 1611-2156 Received: July 22, 2020, accepted: August 03, 2020, published: August 17, 2020   1162 level was elevated (613 IU/L), and laboratory findings were consistent with mild general-ized inflammation (C-reactive protein level was 1.45 mg/dL, and B-type natriuretic pep-tide level was 133.4 pg/mL). Human chori-onic gonadotropin, alpha fetoprotein, carci-noembryonic antigen, squamous cell carci-noma antigen, and anti-acetylcholine receptor antibody levels were within the normal range. Chest radiography (Figure 1) showed a cardi-othoracic ratio of 66 % with cardiac enlarge-ment and bilateral pleural effusions. Lung le-sions were not clearly visible. She was subse-quently admitted to the hospital with a diag-nosis of heart failure.  Figure 1: Chest radiography showed a cardiothoracic ratio of 66 % with cardiac enlargement and bilat-eral pleural effusions, which were greater on the right. Lung lesions were not clearly visible.   EXCLI Journal 2020;19:1161-1165 – ISSN 1611-2156 Received: July 22, 2020, accepted: August 03, 2020, published: August 17, 2020   1163 Electrocardiography revealed a heart rate of 90 beats/min, normal sinus rhythm, and mild ST depression in leads V3–V6. Trans-thoracic cardiac ultrasonography revealed a mass, which extended into the side of the right atrium, around the ascending aorta. There was a hypoechoic area within the mass, suggesting presence of fluid. Contrast-enhanced chest Computed tomography (CT) scan (Figure 2) confirmed the ultrasound findings and showed mass effect on the superior vena cava, which was significantly narrowed. The inside of the tumor could not be clearly visualized on contrast-enhanced imaging; neither fatty nor calcified components were observed. A CT-guided needle biopsy of the medi-astinal tumor revealed a malignant nonepithe-lial tumor of unknown origin. Because the pa-tient presented with respiratory failure in poor clinical condition, she received palliative care without any active treatment. Her health grad-ually deteriorated, and she died 33 days after admission. Post-mortem pathological exami-nation was performed with the consent of her family; a mediastinal tumor containing a large amount of nonserous fluid that invaded the heart and large blood vessels was macroscop-ically found. Microscopic examination of he-matoxylin and eosin-stained slides of the tu-mor specimen showed no pattern or any spe-cific tissue architecture at low magnification (Figure 3A), indicating an undifferentiated malignant tumor. In addition, highly de-formed nuclei were found on high magnifica-tion (Figure 3B). Immunostaining showed positive results for vimentin (Figure 3C) and negative results for CD5, CD31, CD34, CD45, CD68, CD138, CK-CAM5.2, CK-AE1/1, AE3, EMA, calretinin, desmin, α-SMA, MA-HHF, S100, D2-40, HMB45, EMA, and MelanA. Thus, the condition was diagnosed as undifferentiated pleomorphic sarcoma.  Figure 2: Contrast-enhanced chest computed tomography scan confirmed the ultrasound findings and showed mass effect on the superior vena cava, which was significantly narrowed. The inside of the tumor could not be clearly visualized on contrast-enhanced imaging; neither fatty nor calcified compo-nents were observed.EXCLI Journal 2020;19:1161-1165 – ISSN 1611-2156 Received: July 22, 2020, accepted: August 03, 2020, published: August 17, 2020   1164    Figure 3: Microscopic examination of hematoxy-lin and eosin-stained slides of the tumor specimen showed no pattern or any specific tissue architec-ture at low magnification (A), indicating an undif-ferentiated malignant tumor. In addition, highly de-formed nuclei were found on high magnification (B). Immunostaining was positive for vimentin (C).   DISCUSSION Various tumors originate in the mediasti-num. In Japan, the incidence of thymoma is highest (39.8 %), followed by congenital cysts (20.4 %), and neurogenic tumors (10.7 %). Thymus cancer accounts for 5.8 %, and germ cell tumors account for 5.1 % (Ma-suda et al., 2015). Undifferentiated pleo-morphic sarcoma belongs to a group of histo-logically undifferentiated sarcomas contain-ing spindle shaped, circular, polygonal, and several atypical giant cells. Undifferentiated pleomorphic sarcoma, previously known as malignant fibrous histiocytoma (MFH), is the most common soft tissue sarcoma according to the World Health Organization (WHO) di-agnostic criteria for soft tissue sarcoma, which was revised in 2013. Undifferentiated pleomorphic sarcoma is now classified as an independent histotype (Fletcher et al., 2013). According to previous studies, MFH often de-velops in the extremities, retroperitoneum, and abdominal cavity but rarely in the chest (Shibuya et al., 1991; Weiss et al., 1978). Mu-rakawa et al. have summarized 35 mediastinal cases of MFH (Murakawa et al., 2001). Thereafter, several other cases of undifferen-tiated pleomorphic sarcoma have been re-ported (Nakamura et al., 2019). Sarcoma in the mediastinum, particularly undifferenti-ated pleomorphic sarcoma, is rare. There is no standard therapy for the treat-ment of undifferentiated pleomorphic sar-coma other than surgery. A previous study re-ported the case of a patient who underwent surgery for pleomorphic sarcoma in the medi-astinum and survived for > 1 year after sur-gery (Kitano et al., 2010). However, in an-other study, surgery was performed in a pa-tient with pleomorphic sarcoma, but there was recurrence with gastrointestinal metastasis 3 months postoperatively. The patient died 4 months later (Okuda et al., 2015). The effects of radiotherapy and chemotherapy are not de-fined (Nakamura et al., 2019). Some reports have shown that adriamycin and ifosfamide are effective against MFH (Nishida et al., 2011). In the present case, surgery was not in-dicated because her tumor had already in-vaded the pericardium at the time of admis-sion. We believed that chemotherapy was not a treatment option because the tumor had compressed the superior vena cava and the A B C EXCLI Journal 2020;19:1161-1165 – ISSN 1611-2156 Received: July 22, 2020, accepted: August 03, 2020, published: August 17, 2020   1165 patient presented with severe heart failure; the cardiotoxic effects of adriamycin were not likely to have been tolerated by the patient. Treatment in this type of patient is chal-lenging because tumor is often diagnosed at an advanced stage or after recurrence (Naka-mura et al., 2019; Okuda et al., 2015). Undif-ferentiated pleomorphic sarcomas may have a rapidly progressive course. Therefore, clini-cians should be cautious with the differential diagnosis and treatment of this disease. Be-cause of the revised version of the WHO di-agnostic criteria for soft tissue sarcoma in 2013, distinction between undifferentiated pleomorphic sarcoma and MFH has become challenging. Currently, the reports of medias-tinal undifferentiated pleomorphic sarcoma are limited. Thus, further collection and anal-ysis of such cases is required.  We experienced a case of undifferentiated pleomorphic sarcoma that developed in the anterior mediastinum and was diagnosed by postmortem pathological autopsy. We need to recognize that there is a case in which the dis-ease progresses rapidly as in this case and a diagnosis cannot be made. It is important to consider undifferentiated pleomorphic sar-coma as a differential diagnosis of anterior mediastinal tumor, and histological examina-tion for diagnosis should be actively per-formed and early treatment should be consid-ered.  Acknowledgments The authors would like to thank Enago (www.enago.jp) for the English language re-view. The authors would like to thank Mitsunori Yamakawa and Yuka Urano for the comments of pathological findings. Conflict of interest We have no financial relationship with the organization that sponsored the research.  REFERENCES Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F. WHO classification of tumours of soft tissue and bone. (WHO Classification of Tumours, 4th ed., Vol. 5, pp 236-8). Lyon: IARC Press, 2013. Kitano K, Konoeda C, Tanaka M. Multiple presenta-tion of undifferentiated pleomorphic sarcoma in the mediastinum. Kyobu Geka. 2010;63:1045-8. Masuda M, Kuwano H, Okumura M, Arai H, Endo S, Doki Y, et al. Thoracic and cardiovascular surgery in Japan during 2013: Annual report by The Japanese As-sociation for Thoracic Surgery. Gen Thorac Cardio-vasc Surg. 2015;63:670-701. Murakawa T, Nakajima J, Fukami T, Tanaka M, Takeuchi E, Takamoto S. Malignant fibrous histio-cytoma in the anterior mediastinum. Jpn J Thorac Car-diovasc Surg. 2001;49:722-7. Nakamura J, Inoue K, Akiyama S, Kondo H, Saeki K, Moritaka T. A case of mediastinal undifferentiated ple-omorphic sarcoma. Ann Jpn Respir Soc. 2019;8:2-5. Nishida K, Maekawa A, Yokoyama R. Successful treatment via chemotherapy for undifferentiated pleo-morphic sarcoma of back with pulmonary metastasis: A case report. Orthop Traumatol. 2011;60:661-3. Okuda K, Yano M, Moriyama S, Haneda H, Tatematsu T, Suzuki A, et al. A case of mediastinum undifferen-tiated high grade pleomorphic sarcoma. Int J Clin Exp Med. 2015;8:19566-70. Shibuya C, Komuta K, Maeda K, Igarashi T, Ayata M, Okamoto S. A case of malignant fibrous histiocytoma originated from posterior mediastinum. Jpn J Lung Cancer. 1991;31:1077-82. Weiss SW, Enzinger FM. Malignant fibrous histio-cytoma: an analysis of 200 cases. Cancer. 1978;41: 2250-66. 

Undifferentiated pleomorphic sarcoma in the anterior mediastinum with a rapidly progressive course

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Undifferentiated pleomorphic sarcoma in the anterior mediastinum with a rapidly progressive course

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