IMAGES IN RADIOLOGY Robert G. Stern, MD, Section Editor
The Hypermetabolic Mushroom: Superior Vena Cava Syndrome Benjamin Coiffard, MD,a Xavier Elharrar, MD,a Thomas Vandemoortele, MD,a,c Sophie Laroumagne, MD,a,b Hervé Dutau, MD,a Philippe Astoul, MD, PhDa,b a
Department of Thoracic Oncology, Pleural Diseases, and Interventional Pulmonology, Hôpital Nord and bAix-Marseille University, Marseille, France and the cDepartment of Pulmonology, Centre Hospitalier de l’Université de Montréal, Hôpital Notre-Dame, Montreal, Quebec, Canada.
PRESENTATION A 40-year-old man, who had had a successful heart transplant, later developed a very rare and unrelated cardiac problem. He presented with progressive symptoms of cough along with cyanosis and swelling of the face and arms. On examination, he had edema of the face and upper torso and telangiectasias on his chest (Figure 1A). His jugular veins were nonpulsatile and distended. The patient had a history of heavy smoking.
ASSESSMENT Cardiac ultrasound revealed what appeared to be a thrombus in the right atrium. A contrast-enhanced computed tomography (CT) scan of the chest demonstrated multiple venous collaterals (Figure 1B). More striking was a mediastinal mass measuring 5.1 in (130 mm) at its largest diameter; this protruded through the superior vena cava and entered the right atrium (Figures 1B and 1C). The mass, including the portion that extended into the vena cava, was mushroomshaped and hypermetabolic on 18F-fluorodeoxyglucose positron emission tomography (Figure 1D).There were no other lesions.
DIAGNOSIS The patient presented with typical superior vena cava syndrome, marked by cyanosis and edema of the face, neck, arms, Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and contributed to writing the manuscript. Requests for reprints should be addressed to Benjamin Coiffard, MD, Department of Thoracic Oncology, Pleural Diseases, and Interventional Pulmonology, Hôpital Nord, Aix-Marseille University, Assistance PubliqueHôpitaux de Marseille (AP-HM), Chemin des Bourrely, 13915, Marseille Cedex 20, France. E-mail address: [email protected] 0002-9343/$ -see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjmed.2014.01.019
and upper chest. In addition, he had jugular turgor due to increased venous pressure engendered by obstruction of the superior vena cava. He had no headache or dyspnea, common symptoms thought to be secondary to cerebral and laryngeal edema. These were likely prevented by progressive blockage that spurred formation of collateral circulation. Malignancy is the most common cause of this syndrome. Lung cancer, particularly masses located in the upper lobe of the right lung, can impede blood flow through the superior vena cava. The obstruction, which may or may not include a tumor-related thrombus within the vessel, can be the result of tumor invasion or external pressure by the mass or by nearby enlarged lymph nodes. Lymphoma, thymoma, thyroid, and other expansive processes in the mediastinum also can lead to superior vena cava syndrome, as can blood clots caused by invasive medical procedures, such as implantation of a pacemaker wire or placement of an intravenous central catheter. Rarely, infectious causes, including syphilis, tuberculosis, histoplasmosis, and actinomycosis, can trigger the instigating obstruction. Similarly, granulomatous diseases like sarcoidosis can occasionally produce superior vena cava syndrome. Our patient underwent bronchoscopy with transbronchial needle aspiration. Subsequent biopsies were positive for small cell lung cancer. Superior vena cava obstruction is present at diagnosis in 10% of patients with small cell lung cancer and 1.7% of patients with non-small cell lung cancer.1
MANAGEMENT First, any patient with superior vena cava syndrome should be maintained in a sitting position to reduce hydrostatic pressure in the upper body.2 While corticosteroids and diuretics are frequently administered to reduce symptoms, their effectiveness has not been demonstrated; thus, use should be limited because these agents can cause many side
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The American Journal of Medicine, Vol 127, No 5, May 2014
Figure 1 (A) Superficial veins were apparent on the patient’s chest (red arrows). (B) Computed tomography revealed a mediastinal mass (blue arrows), as well as multiple venous collaterals (red arrowheads). (C) The mass protruded through the superior vena cava and entered the right atrium (green arrow). (D) 18F-fluorodeoxyglucose positron emission tomography showed that the mass, including the portion extending into the vena cava, was hypermetabolic (purple arrow, maximum standardized uptake value ¼ 10).
Figure 2 (A) A chest x-ray showed that the size of the mediastinal mass was greatly diminished. (B) Computed tomography disclosed the residual right paratracheal mass (blue arrow). (C) With treatment, the tumor no longer invaded the right atrium (green arrow).
Coiffard et al
Superior Vena Cava Syndrome
effects.3-5 Endovascular stenting is effective in rapid superior vena cava recanalization, regardless of the etiology of obstruction, with resolution rates exceeding 90% in 1-7 days.1,6 Thoracic radiotherapy and/or chemotherapy can be used to improve symptoms of tumor-induced superior vena cava syndrome—in this regard, the modalities are equally effective, whether used alone or together.1 Chemotherapy and radiation also are the mainstays of treatment for small cell lung cancer. Due to high mortality rates, surgery, such as vein grafting or tumor resection, is not recommended for patients with lung cancer that is further complicated by superior vena cava syndrome.7 Our patient, despite his condition on presentation, had good clinical tolerance. He had no respiratory or neurological signs and a grade of 0 on the Eastern Cooperative Oncology Group scale; that is, he was capable of performing his daily tasks without restriction. We chose to administer radiochemotherapy without stenting. He received chemotherapy with etoposide and carboplatin, followed by mediastinal external radiotherapy, a regimen that produced a dramatic positive response (Figure 2A).8-10 On the last CT scan, obtained 11 months after diagnosis, only a persistent paratracheal mass remained (Figure 2B). This measured 1.4 in (35 mm) at its largest diameter. The tumor segment that invaded the right atrium had completely disappeared (Figure 2C). At present, the patient is clinically fine, but he remains under close surveillance.
397
References 1. Rowell NP, Gleeson FV. Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic review. Clin Oncol (R Coll Radiol). 2002;14: 338-351. 2. Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007;356: 1862-1869. 3. Schraufnagel DE, Hill R, Leech JA, Pare JA. Superior vena caval obstruction. Is it a medical emergency? Am J Med. 1981;70:1169-1174. 4. Wilson P, Bezjak A, Asch M, et al. The difficulties of a randomized study in superior vena caval obstruction. J Thorac Oncol. 2007;2: 514-519. 5. Ostler PJ, Clarke DP, Watkinson AF, Gaze MN. Superior vena cava obstruction: a modern management strategy. Clin Oncol (R Coll Radiol). 1997;9:83-89. 6. Kalra M, Gloviczki P, Andrews JC, et al. Open surgical and endovascular treatment of superior vena cava syndrome caused by nonmalignant disease. J Vasc Surg. 2003;38:215-223. 7. Bacha EA, Chapelier AR, Macchiarini P, Fadel E, Dartevelle PG. Surgery for invasive primary mediastinal tumors. Ann Thorac Surg. 1998;66:234-239. 8. Planchard D, Le Péchoux C. Small cell lung cancer: new clinical recommendations and current status of biomarker assessment. Eur J Cancer. 2011;47(Suppl 3):S272-S283. 9. Sørensen M, Pijls-Johannesma M, Felip E; ESMO Guidelines Working Group. Small-cell-lung cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment, and follow up. Ann Oncol. 2010;21(suppl 5): vl20-vl25. 10. Simon GR, Turrisi A; American College of Chest Physicians. Management of small cell lung cancer: ACCP evidence-based clinical practice guidelines (2nd ed). Chest. 2007;132(3 suppl):324S-339S.
The Hypermetabolic Mushroom: Superior Vena Cava Syndrome Benjamin Coiffard, MD,a Xavier Elharrar, MD,a Thomas Vandemoortele, MD,a,c Sophie Laroumagne, MD,a,b Hervé Dutau, MD,a Philippe Astoul, MD, PhDa,b a
Department of Thoracic Oncology, Pleural Diseases, and Interventional Pulmonology, Hôpital Nord and bAix-Marseille University, Marseille, France and the cDepartment of Pulmonology, Centre Hospitalier de l’Université de Montréal, Hôpital Notre-Dame, Montreal, Quebec, Canada.
PRESENTATION A 40-year-old man, who had had a successful heart transplant, later developed a very rare and unrelated cardiac problem. He presented with progressive symptoms of cough along with cyanosis and swelling of the face and arms. On examination, he had edema of the face and upper torso and telangiectasias on his chest (Figure 1A). His jugular veins were nonpulsatile and distended. The patient had a history of heavy smoking.
ASSESSMENT Cardiac ultrasound revealed what appeared to be a thrombus in the right atrium. A contrast-enhanced computed tomography (CT) scan of the chest demonstrated multiple venous collaterals (Figure 1B). More striking was a mediastinal mass measuring 5.1 in (130 mm) at its largest diameter; this protruded through the superior vena cava and entered the right atrium (Figures 1B and 1C). The mass, including the portion that extended into the vena cava, was mushroomshaped and hypermetabolic on 18F-fluorodeoxyglucose positron emission tomography (Figure 1D).There were no other lesions.
DIAGNOSIS The patient presented with typical superior vena cava syndrome, marked by cyanosis and edema of the face, neck, arms, Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and contributed to writing the manuscript. Requests for reprints should be addressed to Benjamin Coiffard, MD, Department of Thoracic Oncology, Pleural Diseases, and Interventional Pulmonology, Hôpital Nord, Aix-Marseille University, Assistance PubliqueHôpitaux de Marseille (AP-HM), Chemin des Bourrely, 13915, Marseille Cedex 20, France. E-mail address: [email protected] 0002-9343/$ -see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjmed.2014.01.019
and upper chest. In addition, he had jugular turgor due to increased venous pressure engendered by obstruction of the superior vena cava. He had no headache or dyspnea, common symptoms thought to be secondary to cerebral and laryngeal edema. These were likely prevented by progressive blockage that spurred formation of collateral circulation. Malignancy is the most common cause of this syndrome. Lung cancer, particularly masses located in the upper lobe of the right lung, can impede blood flow through the superior vena cava. The obstruction, which may or may not include a tumor-related thrombus within the vessel, can be the result of tumor invasion or external pressure by the mass or by nearby enlarged lymph nodes. Lymphoma, thymoma, thyroid, and other expansive processes in the mediastinum also can lead to superior vena cava syndrome, as can blood clots caused by invasive medical procedures, such as implantation of a pacemaker wire or placement of an intravenous central catheter. Rarely, infectious causes, including syphilis, tuberculosis, histoplasmosis, and actinomycosis, can trigger the instigating obstruction. Similarly, granulomatous diseases like sarcoidosis can occasionally produce superior vena cava syndrome. Our patient underwent bronchoscopy with transbronchial needle aspiration. Subsequent biopsies were positive for small cell lung cancer. Superior vena cava obstruction is present at diagnosis in 10% of patients with small cell lung cancer and 1.7% of patients with non-small cell lung cancer.1
MANAGEMENT First, any patient with superior vena cava syndrome should be maintained in a sitting position to reduce hydrostatic pressure in the upper body.2 While corticosteroids and diuretics are frequently administered to reduce symptoms, their effectiveness has not been demonstrated; thus, use should be limited because these agents can cause many side
396
The American Journal of Medicine, Vol 127, No 5, May 2014
Figure 1 (A) Superficial veins were apparent on the patient’s chest (red arrows). (B) Computed tomography revealed a mediastinal mass (blue arrows), as well as multiple venous collaterals (red arrowheads). (C) The mass protruded through the superior vena cava and entered the right atrium (green arrow). (D) 18F-fluorodeoxyglucose positron emission tomography showed that the mass, including the portion extending into the vena cava, was hypermetabolic (purple arrow, maximum standardized uptake value ¼ 10).
Figure 2 (A) A chest x-ray showed that the size of the mediastinal mass was greatly diminished. (B) Computed tomography disclosed the residual right paratracheal mass (blue arrow). (C) With treatment, the tumor no longer invaded the right atrium (green arrow).
Coiffard et al
Superior Vena Cava Syndrome
effects.3-5 Endovascular stenting is effective in rapid superior vena cava recanalization, regardless of the etiology of obstruction, with resolution rates exceeding 90% in 1-7 days.1,6 Thoracic radiotherapy and/or chemotherapy can be used to improve symptoms of tumor-induced superior vena cava syndrome—in this regard, the modalities are equally effective, whether used alone or together.1 Chemotherapy and radiation also are the mainstays of treatment for small cell lung cancer. Due to high mortality rates, surgery, such as vein grafting or tumor resection, is not recommended for patients with lung cancer that is further complicated by superior vena cava syndrome.7 Our patient, despite his condition on presentation, had good clinical tolerance. He had no respiratory or neurological signs and a grade of 0 on the Eastern Cooperative Oncology Group scale; that is, he was capable of performing his daily tasks without restriction. We chose to administer radiochemotherapy without stenting. He received chemotherapy with etoposide and carboplatin, followed by mediastinal external radiotherapy, a regimen that produced a dramatic positive response (Figure 2A).8-10 On the last CT scan, obtained 11 months after diagnosis, only a persistent paratracheal mass remained (Figure 2B). This measured 1.4 in (35 mm) at its largest diameter. The tumor segment that invaded the right atrium had completely disappeared (Figure 2C). At present, the patient is clinically fine, but he remains under close surveillance.
397
References 1. Rowell NP, Gleeson FV. Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic review. Clin Oncol (R Coll Radiol). 2002;14: 338-351. 2. Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007;356: 1862-1869. 3. Schraufnagel DE, Hill R, Leech JA, Pare JA. Superior vena caval obstruction. Is it a medical emergency? Am J Med. 1981;70:1169-1174. 4. Wilson P, Bezjak A, Asch M, et al. The difficulties of a randomized study in superior vena caval obstruction. J Thorac Oncol. 2007;2: 514-519. 5. Ostler PJ, Clarke DP, Watkinson AF, Gaze MN. Superior vena cava obstruction: a modern management strategy. Clin Oncol (R Coll Radiol). 1997;9:83-89. 6. Kalra M, Gloviczki P, Andrews JC, et al. Open surgical and endovascular treatment of superior vena cava syndrome caused by nonmalignant disease. J Vasc Surg. 2003;38:215-223. 7. Bacha EA, Chapelier AR, Macchiarini P, Fadel E, Dartevelle PG. Surgery for invasive primary mediastinal tumors. Ann Thorac Surg. 1998;66:234-239. 8. Planchard D, Le Péchoux C. Small cell lung cancer: new clinical recommendations and current status of biomarker assessment. Eur J Cancer. 2011;47(Suppl 3):S272-S283. 9. Sørensen M, Pijls-Johannesma M, Felip E; ESMO Guidelines Working Group. Small-cell-lung cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment, and follow up. Ann Oncol. 2010;21(suppl 5): vl20-vl25. 10. Simon GR, Turrisi A; American College of Chest Physicians. Management of small cell lung cancer: ACCP evidence-based clinical practice guidelines (2nd ed). Chest. 2007;132(3 suppl):324S-339S.
