Anterior Mediastinal Masses: The 4 T s

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May 2001 Anterior Mediastinal Masses: The 4 T s Rachel Van Sambeek, Harvard Medical School, Year III

1 Mediastinal Compartments 3 arbitrary divisions that do not correlate with anatomic planes: Anterior sternum to line anterior to heart and great vessels thymus, fat, lymph nodes Middle heart, pericardium, ascending and transverse aorta, brachiocephalic vessels, SVC, IVC, main PAs and PVs, trachea, bronchi, lymph nodes Posterior from posterior border of heart and trachea to anterior surface of thoracic spine and ribs descending aorta, esophagus, azygous vein, autonomic ganglia and nerves, thoracic duct, lymph nodes, fat From Strollo DC, et al. Chest 1997; 112: 512.

Evaluation of mediastinal mass by location 2 From Ronson RS, et al. Surgical Clinics of North America 2000; 80(1): 160.

3 Differential Diagnosis of Anterior Mediastinal Masses 1. Thymus a. Thymoma b. Thymic Cyst c. Thymic Hyperplasia d. Thymolipoma e. Thymic Carcinoma f. Thymic Carcinoid 2. Teratoma & other germ cell tumors 3. Thyroid (intrathoracic goiter) 4. Terrible Lymphoma (can be middle or posterior mediastinal) **These are often difficult to differentiate radiographically!** (the presence of fat or fluid or the use of nuclear scanning can help in some situations)

4 Ability to discriminate different pathologies based on radiography Two independent radiologists were shown CXRs and CTs from 128 patients with anterior mediastinal masses and asked to list their top 3 differential diagnoses and their level of confidence in those diagnoses. Results were compared with histologic dxs. CXR CT CT interpretation most often correct for benign germ cell tumors, thymolipoma, and omental hernias. #1 dx correct 36% 48% Correct in top 3 dxs 59% 72% Highly confident 9% 34% Correct when high confidence 58% 80% From Ahn, JM, et al. J Thorac Imaging 1996 Fall; 11(4)265-71.

5 Normal Mediastinal Anatomy at T4 level Note: Thymic expansion rarely causes tracheal or esophageal displacement because of its soft density and very anterior position. From Ronson RS, et al. Surgical Clinics of North America 2000; 80(1): 162.

6 Characteristic Thymic Features on CXR Sail sign Thymic wave Thymic notch Loss of Retrosternal Clear Space From Day DL and E Gedgaudas. The Radiologic Clinics of North America 1984; 22(3): 520-1. From BIDMC teaching files

7 Normal Thymus in a 10-year-old boy Sail sign Retrosternal opacity From BIDMC Teaching files

8 Thymoma Most common anterior mediastinal primary tumor; 20% of adult mediastinal neoplasms Presentation between ages 30-50 (most patients are >40 years old) 50% are asymptomatic Symptoms secondary to compression: chest pain, cough, dyspnea, SVC syndrome (obstructed SVC head and neck venous congestion, facial edema) Parathymic syndromes (approx. 40% of patients) Myasthenia Gravis: seen in 30-50% of thymoma patients; 8-20% of MG patients have a thymoma (although 90% have some sort of thymic abnormality); may develop post thymectomy Pure Red Cell Aplasia: seen in 5% of thymoma patients, but 50% of patients with red cell aplasia have a thymoma Others: hypogammaglobulinemia (10%), endocrine disorders, connective tissue disorders Usually arise in the midline and extend unilaterally Grading: invasive vs. non-invasive (histologically identical) Staging I: within intact capsule II: extension through capsule into surrounding fat, pleura, or pericardium III: intrathoracic metastasis (including pleural seeding) IV: extrathoracic metastasis

9 Thymoma in a 10-year-old boy Grossly widened mediastinum subclavicularly Note presence of thymic wave and thymic notch From BIDMC Teaching files

10 Thymoma in patient with MG Smooth, lobulated mass Preservation of aortic knob indicates that mass is not encompassing the aortic arch Lateral confirms anterior location From BIDMC Teaching files

11 Calcifications in a Thymoma Nonspecific finding as thymomas, teratomas, germ cell tumors, and carcinomas can all calcify Note also the lobulated contour of the mass From BIDMC Teaching files

12 Thymic Cyst: Looking for fluid Anterior Mass is indeterminate on T1 3% of anterior mediastinal masses Mass is hyperintense on T2 Mass on CT, with attenuation similar to that of water (10 HU) Merine D, et al. CT and MRI of the thorax. New York: Churchill Livingstone, 1990: 73.

13 Lipoma: Looking for fat Mass obscuring right heart border, simulating cardiomegaly MR shows hyperintense mass (indicating high fat content) and demonstrates close application of mass to cardiac structures Reddy SCB, et al. American Heart Journal 1996; 132(4): 895.

14 Teratoma (60-70% mediastinal germ cell tumors) Germ cell tumors account for 15% adult anterior mediastinal masses; 24% in children Well-circumscribed mass in lower anterior mediastinum From BIDMC teaching files May contain calcifications visible on CXR or CT, even well-formed teeth or bone Strollo DC, et al. Chest 1997; 112(2): 517.

15 Thyroid: Intrathoracic Goiter 10% mediastinal masses Superior mass that extends above the clavicles Nuclear scan with radioactive iodine may show expanded area of uptake From BIDMC Teaching files

16 The Patient: KL CC: A 56-year old man with fever, cough, and fatigue for about 1 month, presents 4/21/01 with dizziness and weakness ER: Hct of 22.7 (1/02 baseline: 40); CXR notes unusual R heart border contour, but report doubts any significance to this finding In hospital, hematologic evaluation was obtained given inappropriately low reticulocyte count (3.5%)

17 The Consult: Hem/Onc Fellow Bone marrow biopsy showed only deficiency of erythroid precursors. Viral serologies were negative. Suggested thymoma in differential of red cell aplasia in the absence of bone marrow pathology. Recommended chest CT evaluation

18 The Chest X-ray Largely unremarkable BUT, contour of right heart border is unusual Hilar vessels and right bronchus can be seen through mass, implying anterior mediastinal location From PACS, BIDMC

19 The Chest X-ray: Lateral Confirms anterior location Opacification of retrosternal clear space From PACS, BIDMC

20 The Chest CT-Level of the Mitral & Tricupsid Valves Note density applied to anteriolateral surface of the heart Pericardium can be identified as dark line outlined by mass and pericardial fat Pericardium is intact at this level From PACS, BIDMC

21 The Chest CT- Higher Level At this level, the smooth line of pericardium is disrupted, indicating invasion by the mass near right atrial appendage. Findings were confirmed on MRI. From PACS, BIDMC

22 Comparison of Levels on CT From PACS, BIDMC

23 The Surgery Complete thymectomy was performed two weeks later. Involvement of pericardium was noted at surgery, but gross tissue planes were still identifiable Histologically, tumor cells were identified to within 2-3 cell layers from the pericardial specimen margin. Pathologic analysis of the surgical specimen was somewhat controversial. There was some disagreement as to the etiology of the tumor: primary thymoma vs. metastatic lymphoma. CT imaging of head, abdomen, and pelvis did not reveal any other tumor sites. Pure red cell aplasia supports the diagnosis of thymoma.

24 References Ahn JM, Lee KS, Goo JM, Song KS, Kim SJ, Im JG. Predicting the histology of anterior mediastinal masses: comparison of chest radiography and CT. Journal of Thoracic Imaging 1996; 11(4): 265-71. Cohn WE. Anterior mediastinal mass lesions. UpToDate, Inc., 2001. Day DL and E Gedgaudas. The thymus. The Radiologic Clinics of North America 1984; 22(3): 519-37. Reddy SCB, Taneja K, Kothari SS, Goel P, Sharma S, Rajani M, Wasir HS. Mediastinal lipoma mimicking dextrocardia and cardiac enlargement: Diagnosis by magnetic resonance imaging. American Heart Journal 1996; 132(4): 894-6. Ronson RS, Duarte I, Miller JI. Embryology and surgical anatomy of the mediastinum with clinical implications. Surgical Clinics of North America 2000; 80(1): 157-69. Rosado de Christenson ML, Galobardes J, Moran CA. Thymoma: radiologic-pathologic correlation. RadioGraphics 1992; 12(1): 151-68. Strollo DC, Rosado de Christenson ML, Jett JR. Primary mediastinal tumors, part 1: tumors of the anterior mediastinum. Chest 1997; 112(2): 511-22. Merine D, Pessar ML, Zerhouni EA, Fishman EK, Soulen RL. CT and MRI assessment of the mediastinum. CT and MRI of the thorax. New York: Churchill Livingstone, 1990: 67-91.

25 Acknowledgements Dr. James Busch Larry Barbaras and CaraLyn D amour, Webmasters