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A novel case of left atrial and right lung mass turned out to be unconventional metastasis of uterine leiomyosarcoma with a review of literature


1 Department of Pathology, All India Institute of Medical Sciences (AIIMS), Rajkot, Gujarat, India
2 Department of Pathology, Government Medical College, Haldwani, Uttarakhand, India
3 Department of Surgical Oncology, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India
4 Department of Radiodiagnosis, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India

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Date of Submission09-Jun-2021
Date of Decision23-Jun-2021
Date of Acceptance09-Jul-2021
Date of Web Publication26-May-2022
 

   Abstract 


Uterine leiomyosarcoma (ULMS) is a rare malignancy arising from the myometrial smooth muscle wall, and cardiac metastases are extremely rare. Metastasis to the heart is a very unusual finding, and atrial metastasis is even rarer. Here, we report a case of a 45 year old woman who presented with dyspnea and pleural effusion and had a significant history of hysterectomy done for ULMS. Magnetic resonance imaging revealed a left atrial mass, which was resected and revealed to be a metastasis of leiomyosarcoma on histopathology and immunohistochemistry. Metastatic ULMS may rarely present as a left atrial mass with acute clinical presentation. Detailed clinical history and accurate diagnosis are vital for further management.

Keywords: Left atrial mass, metastasis, right lung mass, uterine leiomyosarcomas


How to cite this URL:
Patel T, Aswal P, Jakhetiya A, Meena V, Pandey A. A novel case of left atrial and right lung mass turned out to be unconventional metastasis of uterine leiomyosarcoma with a review of literature. Indian J Pathol Microbiol [Epub ahead of print] [cited 2022 Sep 28]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345885





   Introduction Top


Uterine leiomyosarcoma (ULMS) is a rare entity and accounts for 1% to 2% of malignancies in postmenopausal women.[1] It is a highly aggressive tumor with high predilection for metastasis to the lung, liver, and less frequently to the bone and brain. Symptomatic cardiac metastasis is exceptionally rare. Cardiac metastasis may progress to complications such as heart failure and shock, if not diagnosed early in ULMS patients.[2] Very few cases of ULMS with atrial metastasis have been reported in the literature. Here, we report the case of a 45-year-old woman with a history of ULMS diagnosed 7 years back who presented with left atrial metastasis leading to pericardial and pleural effusion.


   Case Report Top


A 45-year-old female presented to the emergency department with complaints of progressive shortness of breath associated with progressive edema of both lower extremities. The patient was dyspneic with a respiratory rate of 23, SpO2 92%, and a blood pressure of 170/110 mmHg. There was no history of chest pain, nausea, diaphoresis, dizziness, fever, or chills associated with her shortness of breath. There was no associated cough, abdominal pain, diarrhea, or emesis. The patient gave a history of hysterectomy 7 years before the current presentation. At that time, she was evaluated for a large mass in the uterus, and a hysterectomy was performed. The specimen sample was sent to a tertiary-center pathology department, and histopathology revealed the uterine mass to be a low-grade leiomyosarcoma.

On physical examination, significant distant heart sounds, mild abdominal distention, and bilateral lower limb edema were noticed. Echocardiography showed large hemodynamically significant pericardial effusion with both right ventricular and right atrial collapse. A computed tomography (CT) scan showed a large pericardial effusion, with a small right pleural effusion, and anterior cardiac wall density. Positron emission tomography–computed tomography (PET-CT) scan showed significant uptake in the left atrial mass and right lung mass [Figure 1].
Figure 1: (a) Sagittal reformatted contrast-enhanced CT chest image shows a large hypodense mass lesion in the left atrium (black arrow). (b) Coronal reformatted PET-CT image shows a large mass lesion in the left atrium with significant fluorodeoxyglucose (FDG) uptake (black arrow) and metastatic lesion in the right lung with FDG uptake (white arrow). (c) Gross specimen shows a well-encapsulated lesion with a gray-white cut surface. (d) Microscopy shows spindle to pleomorphic hyperchromatic nuclei with atypical mitosis (black arrow) (hematoxylin and eosin, 40×)

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The early clinical diagnosis was left atrial myxoma. The patient underwent cardiac surgery for resection of the atrial mass. Operative findings revealed a large tumor with a broad-based attachment to the medial wall of the left atrium. Grossly, the atrial mass measured about 7.5 × 6.8 × 3.6 cm and was smooth and nodular in appearance with gray-white shiny areas on the cut surface. Histopathology sections of the atrial mass showed a lesion composed of spindle cells arranged in fascicles showing moderate to marked nuclear pleomorphism. Areas of tumor necrosis and atypical mitoses were present with hot areas showing >10 mitoses/10 high-power fields. Focal myxoid areas were also seen. Immunohistochemistry was performed, and the lesional cells were revealed to be Desmin and smooth muscle actin (SMA) positive and Ki-67 showing high index, consistent with high-grade leiomyosarcoma [Figure 1] and [Figure 2]. The patient was referred to the medical oncology and radiotherapy department for further appropriate chemotherapy and radiotherapy and was subsequently discharged.
Figure 2: (a) Section shows fascicles of tumor cells having spindle hyperchromatic nuclei with indistinct eosinophilic cytoplasm (hematoxylin and eosin, 10×). (b) Tumor cells show focal positive for SMA. (c) Strong positivity for Desmin. (d) Ki-67 showing high proliferative index, about 20% to 25%

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   Discussion Top


ULMS is a rare malignant mesenchymal neoplasm arising from smooth muscle cells of the myometrium. It constitutes 1% to 2% of malignant uterine tumors; however, highly aggressive potential often leads to unforeseen clinical course.[1] The most common sites for metastasis include the lung, peritoneum, bones, and liver. ULMS commonly metastasize through the hematogenous route. Survival is primarily dependent on the metastasis, with a 5-year survival rate of 91.2% and 41.2% for nonmetastatic and metastatic ULMS, respectively.[3] A 5-year disease-specific survival of ULMS is determined by factors such as age, race, pathological stage, mitotic index, and lymphovascular invasion.[4]

The first case was published by Rosenblatt and Featherston[5] in 1960. This case series detected six cases as primary ULMS out of the total 2,000 cases of metastatic cardiac tumors studied.[5] In our case, the patient presented with cardiac metastasis after 7 years of hysterectomy, whereas another study showed cardiac metastasis after 16 years of primary resection.[6] Cardiac metastasis may also be diagnosed on autopsy with significant history of uterine mass.[7] ULMS spreads to the heart and lung via the hematogenous route, and it may present with an evidence of vascular spread in the form of multiple intravenous tumor foci.[8] Suggestive cardiac symptoms along with the utility of echocardiography and PET-CT scan are helpful to rule out metastasis.[9],[10] On imaging, metastasis may show variable anatomic distribution in the heart, which may be extensive involving multiple chambers and blood vessels,[11] single chamber involvement,[12] tricuspid valve infiltration,[13] or interventricular septal involvement.[14] Cardiac metastasis of ULMS may show variable clinical presentation as reported by Barış et al.[15] and Karass et al.,[16] where the patients presented with acute coronary syndrome and hypertrophic obstructive cardiomyopathy, respectively. Histology of the tumor reveals spindle cells with eosinophilic cytoplasm and marked nuclear atypia. Frequent mitosis and necrosis is often present. Immunohistochemistry (IHC) shows positivity for smooth muscle actin and Desmin.[13],[17]

Surgical resection is the preferred treatment for the intracardiac spread of ULMS. In cases where surgery is not possible or in advanced cases, additional treatment with chemotherapy and/or radiotherapy is proposed. Reconstructive surgery might be performed in case of a cardiac wall defect.[18],[19]

In recent literature, very few cases have been reported.[20],[21] Taking into consideration the metastatic potential of ULMS, this case has been presented to show the rare metastasis to the heart. In our case, the patient had an atrial mass and the differential diagnosis included were primary cardiac tumors such as atrial myxoma, intracardiac clot, or any metastasis. Echocardiography is the easiest and most widely available method to assess the cardiac chambers. Preoperative thoracic imaging (CT scan or radiography) is also suggested during the initial diagnostic workup. Pathological diagnosis with IHC confirmation assists in excluding other mesenchymal tumors [Table 1].[10],[13],[20]
Table 1: Literature search of cardiac metastasis from uterine leiomyosarcoma (ULMS)

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   Conclusion Top


We presented this case to indicate the aggressiveness of ULMS and its potential for heart metastasis, albeit very rare. Thus, the clinicians treating patients must be aware of the propensity of these tumors for metastasis, sometimes to unexpected sites. In patients showing cardiac symptoms with a past history of leiomyosarcoma, we must recognize the utility of echocardiography and CT scan for diagnosis along with histopathological and IHC confirmation with cognizance of the need to carry out surgical ablation for better survival chance.

Consent for publication

Complete anonymity of the study participant has been maintained and consent obtained for publication.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ricci S, Stone RL, Fader AN. Uterine leiomyosarcoma: Epidemiology, contemporary treatment strategies and the impact of uterine morcellation. Gynecol Oncol 2017;145:208-16.  Back to cited text no. 1
    
2.
Bartosch C, Afonso M, PiresLuís AS, Galaghar A, Guimarães M, Antunes L, et al. Distant metastases in uterine leiomyosarcomas: The wide variety of body sites and time intervals to metastatic relapse. Int J Gynecol Pathol 2017;36:31-41.  Back to cited text no. 2
    
3.
Lamm W, Natter C, Schur S, Köstler WJ, Reinthaller A, Krainer M, et al. Distinctive outcome in patients with non-uterine and uterine leiomyosarcoma. BMC Cancer 2014;14:981.  Back to cited text no. 3
    
4.
Tirumani SH, Deaver P, Shinagare AB, Tirumani H, Hornick JL, George S, et al. Metastatic pattern of uterine leiomyosarcoma: Retrospective analysis of the predictors and outcome in 113 patients. J Gynecology Oncol 2014;25:306-12.  Back to cited text no. 4
    
5.
Rosenblatt WH, Featherston WP. Metastatic leiomyosarcoma of the heart. Am J Cardiol 1960;6:672-5.  Back to cited text no. 5
    
6.
Moreno-Cabral RJ, Fukuyama O, Powell WS, Gold S, McNamara JJ. Surgical resection of giant metastatic leiomyosarcoma of the heart. J Thorac Cardiovasc Surg 1987;94:447-9.  Back to cited text no. 6
    
7.
Cordioli E, Pizzi C, Bugiardini R. Left ventricular metastasis from uterine leiomyosarcoma. Cardiologia 1999;44:1001-3.  Back to cited text no. 7
    
8.
Moorjani N, Kuo J, Ashley S, Hughes G. Intravenous uterine leiomyosarcomatosis with intracardial extension. J Card Surg 2005;20:382-5.  Back to cited text no. 8
    
9.
Moreno Antón F, Casado Herraez A, Puente Vázquez J, Gómez Díaz R, Aragoncillo P, Díaz-Rubio García E, et al. Cardiac metastasis from uterine leiomyosarcoma. Clin Transl Oncol 2006;8:375-8.  Back to cited text no. 9
    
10.
Dencker M, Valind S, Stagmo M. Right ventricular metastasis of leiomyosarcoma. Cardiovasc Ultrasound 2009;7:20.  Back to cited text no. 10
    
11.
Calleja AM, Wellnitz CV, Alharthi MS, Khandheria BK, Chaliki HP. Extensive cardiac metastases secondary to uterine leiomyosarcoma. J Am Soc Echocardiogr 2009;22:1419.  Back to cited text no. 11
    
12.
Nguyen SK, Wong F. Right atrial metastasis of uterine leiomyosarcoma causing obstructive shock. Curr Oncol 2012;19:292-4.  Back to cited text no. 12
    
13.
Marak CP, Ponea AM, Alappan N, Shaheen S, Guddati AK. Uterine leiomyosarcoma manifesting as a tricuspid valve mass. Case Rep Oncol 2013;6:119-26.  Back to cited text no. 13
    
14.
Tunio MA, Al-Asiri M, Fareed MM. Uterine leiomyosarcoma metastasizing to the heart. J Coll Physicians Surg Pak 2014;24(Suppl 1):S20-1.  Back to cited text no. 14
    
15.
Barış VÖ, Özcan ÖU, Gerede DM, Vurgun VK. Case images: Giant left ventricular metastasis of uterine leiomyosarcoma mimicking acute coronary syndrome. Turk Kardiyol Dern Ars 2015;43:496.  Back to cited text no. 15
    
16.
Karass M, Mondal P, Alkayem M, Ojo A, Aronow WS, Puccio C. A rare presentation of acute heart failure secondary to aggressive uterine leiomyosarcoma metastatic to the myocardium initially diagnosed as hypertrophic obstructive cardiomyopathy. Ann Transl Med 2016;4:374.  Back to cited text no. 16
    
17.
Zmaimita H, Samlali H, Rachdi A, Taleb A, Bouchbika Z, Benchekroune N. Cardiac metastasis of uterine leiomyosarcoma: Case report and literature review. Oncol Cancer Case Rep 2017;3:123.  Back to cited text no. 17
    
18.
Artioli G, Borgato L, Calamelli S, Azzarello G. Unusual cardiac metastasis of uterine leiomyosarcoma: Case report and literature review. Tumori 2016;11:102.  Back to cited text no. 18
    
19.
Katayama K, Takahashi S, Sueda T. Rupture of a metastatic uterine leiomyosarcoma invading the right atrium causing cardiac tamponade. Ann Thorac Surg 2017;104:e83.  Back to cited text no. 19
    
20.
Ahoubim J, Sarcar B, Abdeen Y. Cardiac metastasis of a uterine leiomyosarcoma. J Nat Sc Biol Med 2019;10:217-9.  Back to cited text no. 20
  [Full text]  
21.
Maebayashi A, Nagaishi M, Nakajima T, Hata M, Xiaoyan T, Kawana K. Successful surgical treatment of cardiac metastasis from uterine leiomyosarcoma: A case report and literature review. J Obstet Gynaecol Res 2020;46:795-800.  Back to cited text no. 21
    

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Correspondence Address:
Tarang Patel,
5,Vaibhav Bungalows, Ghuma, PO Bopal, Ahmedabad-380058, Gujarat
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_577_21



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