Sarala Ravindran¹*, Raja Norazah Raja Alam Shah² and Pathmanathan Rajadurai³

¹Pantai Premier Pathology Sdn Bhd, Kuala Lumpur, Malaysia
²Hospital Sultanah Aminah, Johor Bahru, Malaysia
³Jeffrey Cheah School of Medicine & Health Sciences, Monash University Malaysia Level 2 Building 3, Jalan Lagoon Selatan, 47500 Bandar Sunway, Selangor Darul Ehsan, Malaysia

*Corresponding Author: Sarala Ravindran, Pantai Premier Pathology Sdn Bhd, Kuala Lumpur, Malaysia.

Received:April 06, 2021; Published: June 24, 2021

Citation: Sarala Ravindran., et al. “Primary Osteosarcoma of Pulmonary Artery - A Case Report and Review of Literature". Acta Scientific Microbiology 5.7 (2021): 117-120.

Abstract

  Primary sarcomas of pulmonary artery are extremely rare, highly malignant tumours and primary osteosarcoma of pulmonary artery is even rarer. Most of the cases are often misdiagnosed as pulmonary thromboembolism and anticoagulated to no effect. We present a case of 38-year-old male initially treated for infection and pulmonary thromboembolism but did not respond to antibiotics and anticoagulative measures. Chest radiograph revealed multiple lung nodules. Excision of right main pulmonary artery mass and upper lobe lung nodules was performed and was proven to be an osteogenic sarcoma of primary pulmonary artery on histopathological examination.

Keywords: Osteosarcoma; Pulmonary Artery; Sarcoma

Introduction

  Primary tumours of the pulmonary arteries are rare and most of the reported cases are sarcomas [9]. Primary osteogenic sarcoma of pulmonary artery is extremely rare. Clinical presentation is of pulmonary thromboembolism refractory to anticoagulation and this frequently leads to a delayed diagnosis [1]. A case of primary osteosarcoma of pulmonary artery with predominant chondroblastic component in this case report is presented.

Case Report

  A 38-year-old gentleman presented with fever, cough, chest pain and dyspnea on effort for 2 months, unresponsive to treatment and sought medical advice from various hospitals. He was initially treated for infection with antibiotics. On subsequent examination, multiple nodules were noted on chest radiograph and pulmonary thromboembolism was suspected on chest computed tomography. The patient was anticoagulated to no effect and possibility of neoplasm was suggested. Right main pulmonary artery mass and upper lobe nodules were excised and submitted for histopathological examination. An initial diagnosis of primary intimal artery sarcoma with metastasis to the lung was made. Subsequently, the tissue was referred to this centre for second opinion.

  The tumour was composed of malignant spindle shaped cells forming osteoid and chondroid elements. Herring bone and storiform pattern of spindle cell tumour were also observed. The tumour cells showed hyperchromatic or vesicular nuclei with prominent nucleoli. Mitoses were seen. Immunohistochemical studies showed heterogeneous positivity for osteocalcin. A final diagnosis of primary pulmonary osteosarcoma of pulmonary artery with predominant chondroblastic component and metastases to the lung was made.

Discussion

  The first case of primary sarcoma of the pulmonary artery was reported from an autopsy by Mandelstamm in 1923 [2]. Ramp., et al. reported that approximately 60% of cases of primary pulmonary artery sarcoma were diagnosed at autopsy [3]. More than two hundred cases are reported in the literature but many are underestimated through misdiagnosis as pulmonary embolism, supported by clinical and radiological findings [4-6]. Clinical symptoms of pulmonary artery sarcomas are nonspecific such as dyspnoea, chest pain and cough [1]. Clinical features that can aid in the differentiating the tumours from pulmonary embolism include fever, elevated erythrocyte sedimentation rate, anaemia, weight loss, absence of procoagulant state and particularly lack of history of deep vein thrombosis [7,9]. Most cases are initially being treated for thromboembolism before a suspicion of tumour is made [8]. Due to recent advances in imaging technology, appropriate use and interpretation of different imaging studies can significantly facilitate accurate diagnosis [1,4,9]. Blackmon., et al. proposed a staging system for primary pulmonary artery sarcomas to determine optimal therapy for management of these patients [7].

  Pathological classification of primary sarcoma of the pulmonary artery is based on the histological subtype and location of the tumour (luminal versus mural) [9,10,32]. Most sarcomas of the pulmonary artery arise from the intimal layer and can show wide variety of histological differentiation [9]. The variable differentiation could be due to origin of pulmonary artery sarcomas from the undifferentiated tissues of the bulbous cordis as suggested by Bleisch., et al. [11,12]. However, no significant correlation between histological type of the tumour and prognosis has been identified [1,7,13].

  Osteogenic sarcoma of the pulmonary artery is an extremely rare form of intravascular tumour. Osteosarcomas usually arise from the metaphyseal ends of long bones; however, extraosseous cases have been reported in soft tissues [19,20], retroperitoneum [21], lung [17], heart [14-16], uterus [18] and thyroid gland [22]. The wall of the pulmonary artery is the common site for osteosarcoma similar to that of sarcomas of pulmonary artery [23]. Histopathological examination confirms the diagnosis of osteosarcoma. The presence of chondroblastic component may contribute to better prognosis in some of these patients [17,29]. To the best of our knowledge, ten case reports of primary pulmonary artery osteosarcoma have been reported [13,23-31]. Pneumonectomy was done in three cases. Pulmonary embolectomy was done in two cases and one of them had pulmonary arteriotomy and extraction of tumour done. The rest of the cases were diagnosed at autopsy. Mc Connell reported the longest survival in his case for 51 months following recurrence at 9 months of initial diagnosis [24]. Cases reported by Madu., et al. and Kimura., et al. died 5 days and 7 months respectively following surgery [23,27]. The outcome of the case reported by Lyerly., et al. was unknown [28]. The patient was alive for 2 years in the case report published by Tsunezuka., et al. The recent case published by Forbes., et al. died three days after operation due to multiorgan failure [31]. The prognosis is generally poor and the recurrence rate is high. Despite complete surgical resection of the tumour, distant metastasis and recurrences can occur. Hence, a careful follow up is mandatory in these cases [29].

Conclusion

  Primary osteogenic sarcoma of pulmonary artery frequently masquerades as thromboembolic disease. Due to its rare occurrence and potential limitations of imaging to rule out a neoplasm, a high index of clinical suspicion should be borne in mind especially in cases unresponsive to anticoagulant therapy. Clinicians should be aware of this disease and has to consider in the differential diagnosis when investigating cases of pulmonary artery obstruction.

Bibliography

1. Lei Huo., et al. “Pulmonary Artery Sarcoma A Clinicopathologic and Immunohistochemical Study of 12 Cases”. American Journal of Clinical Pathology 125 (2006): 419-424.
2. Mandelstamm M. “Uber primare Neubildungen des Herzen”. Virchows Archiv für pathologische Anatomie und Physiologie und für klinische Medizin 245 (1923): 43-54.
3. Ramp U., et al. “Sarcoma of the pulmonary artery: report of 2 cases and a review of literature”. Journal of Cancer Research and Clinical Oncology 118 (1992): 551-556.
4. Semin Chong., et al. “Pulmonary artery sarcoma mimicking pulmonary thromboembolism”. American Journal of Roentgenology 188 (2007): 1691-1693.
5. Sandhu A., et al. “Pulmonary artery sarcoma mimicking a pulmonary embolism”. Indian Journal of Cancer 45 (2008): 27-29.
6. Kim M Kerr. “Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension”. Nature Clinical Practice Cardiovascular Medicine 2 (2005): 108-122.
7. Shanda H Blackmon., et al. “Management of Primary Pulmonary Artery Sarcomas”. Annals of Thoracic Surgery 87 (2009): 977-984.
8. Simone Schleger., et al. “A Primary Pulmonary Artery Chondrosarcoma Manifesting as Acute Pulmonary Embolism”. Annals of Thoracic Surgery 94 (2012): 1731-1733.
9. A Coli., et al. “Pulmonary artery sarcoma: An insidious tumour still diagnosed too late. Analysis of the literature and report of a case”. Journal of Experimental and Clinical Cancer Research 26 (2007): 1.
10. Burke A., et al. “Tumours of great vessels. Atlas of tumour pathology”. Third series. Fascicle 16. Washington, D.C.: Armed Forces institute of Pathology (1996): 217-221.
11. Bleisch VR and Kraus FT. “Polypoid sarcoma of the pulmonary trunk: analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma”. Cancer 46 (1980): 314-324.
12. Johansson L and Carlén B. “Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations, and review of the literature”. Virchows Arch 424 (1994): 217-224.
13. Hirofumi Fujii., et al. “Primary pulmonary artery sarcoma”. Japanese Circulation Society 62 (1998): 379-381.
14. Sogabe O and Ohya T. “Right ventricular failure due to primary right ventricle osteosarcoma”. General Thoracic and Cardiovascular Surgery 1 (2007): 19-22.
15. Marvasti MA., et al. “Primary osteosarcoma of left atrium: complete surgical excision”. Annals of Thoracic Surgery4 (1985): 402-404.
16. Masakazu Yamagishi., et al. “Primary Cardiac Osteosarcoma in Right Ventricular Outflow Tract”. Circulation 101 (2000): 2220-2221.
17. Toru Kadowaki., et al. “Two cases of primary pulmonary osteosarcoma”. Internal Medicine 44 (2005): 632-637.
18. Cynthia Abraham., et al. “Primary osteosarcoma of the uterus with cardiac and pulmonary metastases”. Gynecologic Oncology Reports 11 (2015): 4-6.
19. Wils on H. “Extra skeletal ossifying tumors”. Annals of Surgery 113 (1941): 95-112.
20. Fine G and Stout AP. “Osteogenic sarcoma of the extra skeletal soft tissues”. Cancer 9 (1956): 1027-1043.
21. Ratnagiri R., et al. “Extra osseous osteosarcoma of the retroperitoneum: An unusual entity”. Journal of Cancer Research and Therapeutics 8 (2012): 424-426.
22. Ewa Zembala-Nożyńska and Dariusz Lange. “Primary osteosarcoma of the thyroid gland - a case report”. Contemporary Oncology (Pozn) 17.1 (2013): 97-99.
23. Kimura I., et al. “Primary osteogenic sarcoma of pulmonary artery”. Japan Journal of Medicine 29 (1990): 32-37.
24. McConnell TH. “Bony and cartilabinous tumors of the heart and great vessels”. Cancer 25 (1970): 611.
25. Murthy MSN., et al. “Primary intimal sarcoma of pulmonary valve and trunk with osteogenic sarcomatous elements”. Archives of Pathology and Laboratory Medicine 100 (1976): 649-651.
26. Shimizu M., et al. “An autopsy case of the osteosarcoma originating from the pulmonary artery”. Nichinan Kaishi 72 (1983): 1041.
27. Madu EC., et al. “Primary intimal sarcoma of the pulmonary trunk simulating pulmonary embolism”. American Heart Journal 125 (1993): 1790-1792.
28. Lyerly HK., et al. “Management of pulmonary sarcomas of the pulmonary artery and reperfusion intrabronchial hemorrhage”. Surgery, Gynecology and Obstetrics 163 (1986): 291-301.
29. Yoshio Tsunezuka., et al. “Primary Chondromatous Osteosarcoma of the Pulmonary Artery”. The Annals of Thoracic Surgery 77 (2004): 331-334.
30. Akaike K., et al. “Case of osteosarcoma of the pulmonary artery”. Nihon Kokyuki Gakkai Zasshi (Journal of the Japanese Respiratory Society) 48.12 (2010): 923-929.
31. Damon Forbes., et al. “Pulmonary Artery Chondroblastic Osteosarcoma Masquerading As a Refractory Pulmonary Embolus”. American Journal of Respiratory and Critical Care Medicine 191 (2015): A3613.
32. I Furest., et al. “Intimal Sarcoma of the Pulmonary Artery: A Rare Cause of Pulmonary Hypertension”. Archives of Bronconeumology 42 (2006): 148-150.

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