|Year : 2022 | Volume
| Issue : 2 | Page : 74-76
Primary intimal sarcoma with chondrosarcoma differentiation of the pulmonary artery
Tzer-Ming Chuang1, Hui-Hua Hsiao2, Kun-Bow Tsai3
1 Division of Hematology and Oncology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
2 Division of Hematology and Oncology, Department of Internal Medicine, Kaohsiung Medical University Hospital; Department of Internal Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
3 Department of Internal Medicine, Faculty of Medicine, College of Medicine; Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
|Date of Submission||13-Oct-2021|
|Date of Decision||19-Nov-2021|
|Date of Acceptance||23-Nov-2021|
|Date of Web Publication||01-Jun-2022|
Dr. Tzer-Ming Chuang
Division of Hematology and Oncology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, No. 100, Tzyou 1st Road, Kaohsiung 807
Source of Support: None, Conflict of Interest: None
We report a rare case and our experience of successfully treating intimal sarcoma (IS) with chondrosarcoma differentiation arising in the pulmonary artery. A 36-year-old man presented with a thrombosis-like mass in the pulmonary trunk. Anticoagulant therapy was initiated, but his clinical condition worsened despite adequate anticoagulation. Positron-emission tomography/computed tomography (CT) revealed a neoplastic lesion. The patient underwent endarterectomy and tumor resection. Microscopically, marked cartilaginous differentiation of the cancer cells admixed with pleomorphic, spindle, and round cells was noted. They were immunoreactive for S-100 protein and isocitrate dehydrogenase 1, focal and weak for desmin and murine double minute-2, but negative for ERG, CD34, and myogenin. A diagnosis of IS with chondrosarcoma differentiation was made. The patient received six cycles of adjuvant chemotherapy, and a follow-up chest CT did not show evidence of recurrence.
Keywords: Chondrosarcoma, intimal sarcoma, pulmonary artery neoplasm
|How to cite this article:|
Chuang TM, Hsiao HH, Tsai KB. Primary intimal sarcoma with chondrosarcoma differentiation of the pulmonary artery. J Cancer Res Pract 2022;9:74-6
|How to cite this URL:|
Chuang TM, Hsiao HH, Tsai KB. Primary intimal sarcoma with chondrosarcoma differentiation of the pulmonary artery. J Cancer Res Pract [serial online] 2022 [cited 2022 Jun 30];9:74-6. Available from: https://www.ejcrp.org/text.asp?2022/9/2/74/346415
| Introduction|| |
Intimal sarcomas (ISs) are rare malignant mesenchymal tumors arising from the tunica intima of large blood vessels of the systemic and pulmonary circulation. About two-third of all cases occur in the pulmonary arteries, and about 80% of these cases occur in the pulmonary trunk. IS presents with various histological subtypes of differentiation, including fibrosarcoma, leiomyosarcoma, anaplastic sarcoma, osteosarcoma, and chondrosarcoma. These patients are frequently misdiagnosed with pulmonary embolic disease and inappropriately treated with anticoagulation or thrombolysis for a period of time. Even though the prognosis of most patients is poor due to aggressive tumor growth and progressive heart failure, a recent large observational analysis suggested that a rapid and accurate diagnosis followed by appropriate treatment can improve outcomes. We report a case of IS with chondrosarcoma differentiation which was successfully treated. Ethical approval for this study was obtained from our hospital, and we informed both the patient and his family regarding this study and obtained their written informed consent (KMUHIRB-E (I)-20210214).
| Case Report|| |
A 36-year-old man was admitted to our hospital with a chief complaint of exertional dyspnea and chest tightness for 2 months. A physical examination revealed a systolic murmur. An ultrasound examination revealed moderate tricuspid regurgitation and possible pulmonary hypertension. Contrast-enhanced chest computed tomography (CT) showed a filling defect with fat content within the pulmonary trunk and right lower pulmonary arteries without any calcification [Figure 1]a. The differential diagnosis included pulmonary thromboembolism and a primary pulmonary artery tumor. The patient initially received anticoagulation for suspected pulmonary embolism; however, his clinical condition worsened despite adequate anticoagulant therapy. Therefore, a fluorodeoxyglucose-positron-emission tomography/CT (FDG-PET/CT) scan was ordered, and a mass with FDG accumulation in the pulmonary trunk was found with a maximum standard uptake value of 10.2. No other mass with increased FDG uptake was detected [Figure 1]b. Under the possible diagnosis of a neoplastic lesion, median sternotomy with the institution of cardiopulmonary bypass and endarterectomy of the pulmonary artery was performed, and the tumor was completely removed from the vessel lumen [Figure 1]c.
|Figure 1: (a) Contrast-enhanced chest computed tomography showed a filling defect with fat content within the pulmonary trunk and right lower pulmonary arteries (red arrow). (b) A fluorodeoxyglucose-positron-emission tomography/computed tomography scan was ordered, and a mass with fluorodeoxyglucose accumulation in the pulmonary trunk was found. (c) Gross picture of the tumor during endarterectomy of the pulmonary artery. (d) Grayish-tan soft tissue fragments of the tumor|
Click here to view
Gross pathology showed ten grayish-tan soft tissue fragments [Figure 1]d. Microscopically, marked cartilaginous differentiation of the cancer cells admixed with pleomorphic, spindle, and round cells was noted. They were immunoreactive for S-100 protein and isocitrate dehydrogenase 1, focal and weak for desmin and murine double minute, but negative for ERG, CD34, and myogenin [Figure 2]. The features were consistent with high-grade intimal chondrosarcoma. He received six cycles of adjuvant chemotherapy with doxorubicin and ifosfamide and a follow-up contrast-enhanced chest CT scan 3 months later showed no evidence of tumor recurrence. The patient has currently been followed up for 1 year at an outpatient clinic without any known complications.
|Figure 2: Marked cartilaginous differentiation of the cancer cells admixed with pleomorphic, spindle, and round cells (a and b). They were immunoreactive for S-100 protein (c) and isocitrate dehydrogenase 1 (d), focal and weak for desmin (e) and murine double minute-2 (f)|
Click here to view
| Discussion|| |
IS with chondrosarcoma differentiation of pulmonary artery is a rare disease, with only a few case reports reported., In the present case, the tumor showed features of sarcoma and involved only the pulmonary artery, findings consistent with a diagnosis of IS. The mean age at diagnosis is 48 years for pulmonary tumors and 62 years for aortic tumors, and the sex distribution is almost equal. In a review of 180 primary sarcomas of large arteries, 61% were classified as ISs, and of these, 26% were differentiated. Differentiated IS presents with a spectrum of histopathological morphologies with variable immunophenotypes. Commonly reported types include angiosarcoma, leiomyosarcoma, myxofibrosarcoma, epithelioid hemangioendothelioma, and osteosarcoma.
A correct diagnosis of IS of the pulmonary artery is challenging and is often misdiagnosed as pulmonary thromboembolism due to its rarity and insidious growth characteristics. The pulmonary artery lesion in our case showed resistance to anticoagulant therapy, leading to the suspicion of neoplasia on FDG-PET/CT and prompt surgery for the tumor. The mean survival in untreated patients is approximately 1.5 months, and more than half of the patients receiving surgical resection died within the 1st year. Furthermore, the presence of distant metastasis has been associated with a more than twofold increase in mortality during follow-up.
Regarding treatment for IS of the pulmonary artery, patients who undergo an attempt at curative resection have been reported to have a longer overall survival (OS) compared to those who undergo incomplete resection (median OS of 36.5 vs. 11 months). Thus, the standard approach is an early aggressive surgery aiming for complete surgical resection with clear margins. As for medical management, due to its rarity, there is no clear consensus concerning the role of systemic therapy. A chemotherapeutic regimen combining doxorubicin and ifosfamide has been demonstrated to be effective regardless of the histological subtype of these tumors. Other agents such as carboplatin, epirubicin, cyclophosphamide, gemcitabine, dacarbazine, etoposide, and vinorelbine have shown a positive effect in some cases. Neoadjuvant chemotherapy is usually recommended to improve the outcomes and facilitate surgery of locally advanced soft tissue sarcoma patients who are not amenable to optimal surgery with clear margins. This treatment approach has led to surgery in initially inoperable patients with a positive impact on survival. On the other hand, the available data are not clear concerning the benefits of adjuvant chemotherapy for IS of the pulmonary artery, even though many case reports have demonstrated successful results in favor of chemotherapy in the postoperative setting. Adjuvant therapy in the study by Wong et al. was administered to 25% of their patients, with a trend toward improved survival compared to surgery alone (24 vs. 8 months, P = 0.3417). Further studies are warranted.
| Conclusion|| |
We present a very rare case of IS with chondrosarcoma differentiation arising in the pulmonary artery. Although a diagnosis is often challenging, early detection and histological confirmation of the tumor followed by complete surgery and multidisciplinary collaboration between clinicians, radiologists, and pathologists are fundamental to achieve the best long-term outcomes.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bode-Lesniewka B, Debiec-Rychter M. Tumours of uncertain differentiation, intimal sarcoma. In: Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. WHO Classification of Tumours of Soft Tissue and Bone. 4th
ed. Lyon: IRAC; 2013. p. 232-3.
Cox JE, Chiles C, Aquino SL, Savage P, Oaks T. Pulmonary artery sarcomas: A review of clinical and radiologic features. J Comput Assist Tomogr 1997;21:750-5.
Bandyopadhyay D, Panchabhai TS, Bajaj NS, Patil PD, Bunte MC. Primary pulmonary artery sarcoma: A close associate of pulmonary embolism-20-year observational analysis. J Thorac Dis 2016;8:2592-601.
Onagi H, Horimoto Y, Arai T, Terukina H, Asai T, Arakawa A, et al.
Primary intimal sarcoma of the pulmonary artery in a 25-year-old woman with dyspnea and palpitation: A case report. Case Rep Oncol 2021;14:318-24.
Miyai K, Takeo H, Matsukuma S. Intimal sarcoma of the pulmonary trunk showing broad intimal extension and focal chondrosarcomatous differentiation: An autopsy case. Cardiovasc Pathol 2017;31:5-8.
Sebenik M, Ricci A Jr., DiPasquale B, Mody K, Pytel P, Jee KJ, et al.
Undifferentiated intimal sarcoma of large systemic blood vessels: Report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 2005;29:1184-93.
Mussot S, Ghigna MR, Mercier O, Fabre D, Fadel E, Le Cesne A, et al.
Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg 2013;43:787-93.
Blackmon SH, Rice DC, Correa AM, Mehran R, Putnam JB, Smythe WR, et al.
Management of primary pulmonary artery sarcomas. Ann Thorac Surg 2009;87:977-84.
Assi T, Kattan J, Rassy E, Moussa T, Nassereddine H, Honore C, et al.
A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery. Crit Rev Oncol Hematol 2020;147:102889.
Casali PG, Abecassis N, Aro HT, Bauer S, Biagini R, Bielack S, et al.
Soft tissue and visceral sarcomas: ESMO-EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2018;29 Suppl 4:v51-67.
Wong HH, Gounaris I, McCormack A, Berman M, Davidson D, Horan G, et al.
Presentation and management of pulmonary artery sarcoma. Clin Sarcoma Res 2015;5:3.
[Figure 1], [Figure 2]