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    Abstract
   Introduction
   Case Report
   Discussion
    References
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Table of Contents
CASE REPORT  
Year : 2013  |  Volume : 16  |  Issue : 4  |  Page : 293-295
Primary malignant fibrous histiocytoma involving the left pulmonary vein presenting as a left atrial tumor


1 Department of Anesthesiology and Pain Medicine, Medical Superspecialty Hospital, Kolkata, West Bengal, India
2 Department of Cardiology, Medical Superspecialty Hospital, Kolkata, West Bengal, India
3 Department of Cardiothoracic and Vascular Surgery, Medical Superspecialty Hospital, Kolkata, West Bengal, India

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Date of Submission05-Apr-2013
Date of Acceptance29-Jul-2013
Date of Web Publication1-Oct-2013
 

   Abstract 

A 35-year-old woman presented with 4 months history of progressively increasing intermittent dyspnea and hemoptysis. Transthoracic echocardiography revealed a loculated mass in the left atrium (LA). A provisional diagnosis of LA myxoma was made. Intraoperatively the tumor was found extending into and closely adherent to the left pulmonary vein and could not be completely cleared off from the pulmonary venous wall. The histopathological examination of the tumor revealed it to be a myxoid malignant fibrous histiocytoma.

Keywords: Atrial tumor; Malignant fibrous histiocytoma; Transesophageal echocardiography

How to cite this article:
Bandyopadhyay S, Banerjee S, Paul A, Das RK. Primary malignant fibrous histiocytoma involving the left pulmonary vein presenting as a left atrial tumor. Ann Card Anaesth 2013;16:293-5

How to cite this URL:
Bandyopadhyay S, Banerjee S, Paul A, Das RK. Primary malignant fibrous histiocytoma involving the left pulmonary vein presenting as a left atrial tumor. Ann Card Anaesth [serial online] 2013 [cited 2019 Dec 15];16:293-5. Available from: http://www.annals.in/text.asp?2013/16/4/293/119184



   Introduction Top


The incidence of primary cardiac tumors varies between 0.002% and 0.3%. [1],[2] Nearly 75% primary cardiac tumors are benign and the majority are myxomas. Primary malignant cardiac tumors are extremely rare and bear a poor prognosis because of local recurrence and distant metastasis. In general, a variant of sarcoma is the most common lesion among malignant primary cardiac tumors. Malignant fibrous histiocytoma is a rare sarcoma, which constitutes <3% of primary cardiac tumors. [1],[2] Even rarer is the origin of this tumor from the pulmonary vein and its growth into the left atrium (LA) mimicking a myxoma. Very few reports mention a primary malignant fibrous histiocytoma involving the pulmonary vein. [3] Unlike a myxoma, malignant fibrous histiocytoma is far more aggressive and has a much higher incidence of local infiltration, recurrence and distant metastasis and hence carries a very poor prognosis.


   Case Report Top


A 35-year-old woman presented with 4 months history of progressively increasing, intermittent dyspnea associated with episodes of hemoptysis. Apart from bleeding piles no other comorbidity was evident from the patient's clinical history. Transthoracic echocardiogram (TTE) showed a 5.8 cm × 3.7 cm well circumscribed loculated mass arising from the postero-lateral surface of the LA, raising the suspicion of a LA myxoma. The mass was mobile and at times, it was partially prolapsing into the left ventricular (LV) cavity. The mitral valve apparatus was not involved and the anterior as well as posterior mitral leaflets showed normal movement. Continuous wave Doppler (CWD) across the valve showed a peak gradient of 7 mmHg and a mean gradient of 5 mmHg, signifying mild obstruction across the valve. Other than a small rim of pericardial effusion and mild pulmonary hypertension, the echocardiographic study was normal and revealed good biventricular function. The chest X-ray showed minimal bilateral costophrenic angle obliteration suggestive of mild bilateral pleural effusion. The rest of the clinical work-up was within the normal limits. The routine pre-operative hemogram showed microcytic hypochromic anemia with hemoglobin 9.0 g/dl. Patient was scheduled for excision of the LA tumor. Patient underwent an uneventful induction of anesthesia using fentanyl, midazolam and sevoflurane. Intraoperative transesophageal echocardiography (TEE) revealed the LA tumor to be loculated with a clear stalk like attachment arising from the left upper pulmonary vein, which was not evident on the pre-operative TTE [Figure 1]a and b raising the suspicion that the tumor might not be a myxoma. On cardiopulmonary bypass (CPB), the LA was opened posterior and parallel to the interatrial groove and the LA mass was removed with sharp dissection. The peduncle of the tumor extended into the left pulmonary vein as suspected during TEE examination. The lesion was adherent closely to the pulmonary venous wall. The mass was removed piecemeal; however, complete clearance of the tumor from the pulmonary venous wall could not be achieved. Thereafter, the LA was closed, heart de-aired, cross-clamp released and the patient was rewarmed and weaned-off CPB with only minimal inotropic support. After completion of the surgical procedure, patient was shifted to the intensive care unit and electively ventilated overnight and extubated next morning. The gross appearance of the cut surface of the tumor was greyish with congested/hemorrhagic areas [Figure 2]. The histopathology revealed the the tumor tumor to be a myxoid malignant fibrous histiocytoma (pleomorphic sarcoma). Microscopic examination of the tumor showed a pleomorphic appearance composed of oval cells arranged in vague storiform pattern intermixed with myxoid areas and tumor giant cells [Figure 3]. The patient had an uneventful post-operative recovery and was referred to the medical oncology department. However, the patient refused any further treatment. Subsequently, patient reported 4 months later with dyspnea, with echocardiographic recurrence of the tumor in the LA; she refused further surgical interventions and died 2 months later. Institutional review board permitted publication of this case report.
Figure 1:

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Figure 2: The greyish yellow congested/hemorrhagic cut surface of the tumor

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Figure 3: The histological appearance of the tumor revealed a pleomorphic appearance composed of oval cells arranged in vague storiform pattern

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   Discussion Top


The first reported case of cardiac malignant fibrous histiocytoma was published in 1978 by Shah et al., [4] Okamoto et al., [5] reported a case of malignant fibrous histiocytoma arising from the LA and reviewed 46 other cases previously reported. Analysis of the 47 cases revealed that the age of presentation widely varied and ranged between 14 and 77 years. Malignant cardiac tumors are associated with a poor long-term survival despite surgery, radiotherapy and/or chemotherapy. [3] A few individual reports document complete recovery after successful removal of tumor with [6],[7] or without [3] follow-up chemotherapy or radiotherapy. However, majority of the reported cases showed rapid progression, in terms of recurrence, local infiltration and distant metastasis, with clinical deterioration in a short time and eventual death. [8] Complete surgical resection helps ameliorate symptoms and obtain histologic information. Complete surgical excision is the only treatment that has been shown to prolong survival. [9] Adriamycin [9] and doxorubicin [10],[11] has shown a survival benefit over others in some reports.

The anesthetic management of a patient with a large LA mass may be challenging. Detailed history and clinical examination of the patient help identify patients at risk. Pre-operative evidence of heart failure, pulmonary hypertension and evidence of LV inflow obstruction should be sought for and when identified should be treated adequately prior to surgery. CWD help reveal grades of mitral inflow obstruction. [12] A mean pressure gradient of 6-10 mmHg indicates moderate obstruction and a gradient of more than 10 mmHg, severe obstruction. The basic principles for managing such patients are similar to those of mitral stenosis and include a carefully planned, balanced anesthetic approach using opiates, benzodiazepines and volatile anesthetic agents. The choice of muscle relaxants for intubation should take heart rate into consideration.

 
   References Top

1.Urías-Báez R, de la Cerda-Belmont G, Cuevas-Domínguez A, Careaga-Reyna G. Intracardiac malignant fibrous histiocytoma. A case report. Cir Cir 2012;80:182-5.  Back to cited text no. 1
    
2.Shanmugam G. Primary cardiac sarcoma. Eur J Cardiothorac Surg 2006;29:925-32.  Back to cited text no. 2
    
3.Toda R, Yotsumoto G, Masuda H, Sakata R, Umekita Y. Surgical treatment of malignant fibrous histiocytoma in the left atrium and pulmonary veins: Report of a case. Surg Today 2002;32:270-3.  Back to cited text no. 3
    
4.Shah AA, Churg A, Sbarbaro JA, Sheppard JM, Lamberti J. Malignant fibrous histiocytoma of the heart presenting as an atrial myxoma. Cancer 1978;42:2466-71.  Back to cited text no. 4
    
5.Okamoto K, Kato S, Katsuki S, Wada Y, Toyozumi Y, Morimatsu M, et al. Malignant fibrous histiocytoma of the heart: Case report and review of 46 cases in the literature. Intern Med 2001;40:1222-6.  Back to cited text no. 5
    
6.Eckstein R, Gössner W, Rienmüller R. Primary malignant fibrous histiocytoma of the left atrium. Surgical and chemotherapeutic management. Br Heart J 1984;52:354-7.  Back to cited text no. 6
    
7.Balaceanu A, Mateescu D, Diaconu C, Sarsan A. Primary malignant fibrous histiocytoma of the right ventricle: Case report and review of the literature. J Ultrasound Med 2010;29:655-8.  Back to cited text no. 7
    
8.Dorobantu M, Fruntelata A, Constantinescu D, Racoveanu I, Ardeleanu C, Tatu-Chitoiu G, et al. Primary left heart malignant fibrous histiocytoma. Eur J Echocardiogr 2005;6:225-7.  Back to cited text no. 8
    
9.Sato M, Suenaga E, Senaha S, Furutachi A. Primary malignant fibrous histiocytoma of the heart. Gen Thorac Cardiovasc Surg 2007;55:29-31.  Back to cited text no. 9
    
10.Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906-10.  Back to cited text no. 10
    
11.Llombart-Cussac A, Pivot X, Contesso G, Rhor-Alvarado A, Delord JP, Spielmann M, et al. Adjuvant chemotherapy for primary cardiac sarcomas: The IGR experience. Br J Cancer 1998;78:1624-8.  Back to cited text no. 11
    
12.Abernathy JH 3 rd , Locke AB, Shernan SK. Dynamic left ventricular inflow obstruction associated with a left atrial myxoma. Anesth Analg 2006;103:1406-7.  Back to cited text no. 12
    

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Correspondence Address:
Saikat Bandyopadhyay
D 401, Mayfair Greens, 449 S N Ghosh Avenue, Kolkata - 700 103, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.119184

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