Karoline Nowillo, MD. February 1, 2008

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Case Presentation Karoline Nowillo, MD SUNY Downstate t February 1, 2008

Case Presentation Chief complaint enlarging goiter x 8 months History of present illness shortness of breath, heaviness in chest Physical exam large rubbery symmetric goiter, no dominant nodules, no palpable nodes

Work-up Imaging Chest XR CT Neck CT Chest Consultation Cardiothoracic Surgery

Neck CT

Thyroid mass Azygos vein SVC

Work-up CT Neck & Chest large goiter, extension of left thyroid lobe into mediastinum measuring 5.2 x6.5cm in superior compartment, coursing to posterior compartment, tracheal compression and deviation to the right, compression of major bronchi Cardiothoracic Surgery available in OR

Intra-op: Cervical Approach Collar incision Total thyroidectomy Unable to mobilize entire left lobe Combined approach

Intra-op Views left thyroid lobe trachea right thyroid lobe

Intra-op: Thoracic Approach Right posterior lateral thoracotomy Division of azygos vein Resection of thyroid goiter in posterior mediastinum Thoracic and cervical incisions closed

Intra-op Views superior posterior anterior azygos vein thyroid mass

superior posterior anterior thyroid mass esophagus ligated azygos vein tract of azygos vein inferior

Specimen: Nodular Goiter right thyroid lobe left thyroid lobe left thyroid lobe thoracic component

Post-op Discharged on POD 4 2 weeks post-op: General surgery started on synthroid, complained hoarse voice 4 weeks post-op: Cardiothoracic surgery thoracotomy yp paresthesias, incisions healed

Post-op 8 weeks post-op: ENT laryngoscopy- left true vocal cord paresis v. paralysis consistent with left recurrent laryngeal nerve injury referred for possible vocal cord augmentation

Anterior Mediastinal Masses Diagnosis & Treatment Karoline Nowillo, MD SUNY Downstate t February 1, 2008

Components of Anterior Mediastinum Thymus Lymph nodes Ascending aorta Transverse aorta Great vessels Areolar tissue

Diagnosis

Disorders of Anterior Mediastinum Thymoma Lymphoma Teratoma Thyroid disorder Stem cell tumor Parathyroid disorder Lipoma

Differential Diagnosis: i Anterior Mediastinal Masses Neoplastic thyroid, thymus, teratoma, germ cell, lymphoma, ectopic parathyroid Infectious acute/subacute mediastinitis Vascular aneurysm of aortic arch, superior vena cava, inominate vein, persistent left superior vena cava

ANTEROSUPERIOR MEDIASTINUM (n=287) Type of Tumor or Cyst Percentage Thymic neoplasms 33 Lymphomas 19 Germ cell tumors 17 Benign 9 Malignant 8 Carcinoma 11 Cysts 8 Mesenchymal 4 Endocrine 6 Other 2

Preoperative Evaluation Provisional diagnosis based on clinical evaluation Diagnostic imagingi CXR PA & lateral CT Scan MRI Scintiscan (possible substernal goiter) Fluoroscopy, barium swallow Bronchoscopy, esophagoscopy

Thymoma Most common neoplasm of anterior mediastinum Incidence 0.15 per 100,000 Uncommon before fourth decade of life Associated with para- neoplastic syndromes

Thymoma Measure autoantibodies to acetylcysteine Tx: Resection once diagnosed Local recurrence once capsule violated Chemotherapy or radiation for invasion, local metastasis t or if inoperable

Thymic Carcinoma & Carcinoid Rare, invasive epithelial malignancies Tx: complete resection can be curative, chemotherapy and radiation when unresectable

Thymolipoma & Thymic Cysts Lipoma is slow growing tumor seen on CT & MRI Cysts are rare tumors, may be congenital or acquired Tx: for both is surgical excision

Primary Mediastinal Lymphoma Persons younger than 40 years old Presence of group B symptoms Palpable remote adenopathy Elevated serum LDH Dx: biopsy via Chamberlain procedure, mediastinoscopy or VATS Tx: chemotherapy, radiation, BMT for patients with lymphoblastic lymphoma

Germ Cell Tumors Second or third decade of life (> 90%) produce tumor markers, including hcg and AFP

Germ Cell Tumors Classification benign teratoma seminoma embryonal tumors yolk sac tumors choriocarcinomas embryonal carcinomas teratocarcinomas

Germ Cell Tumors: Benign Teratoma Benign teratomas are well-encapsulated From at least 2 of 3 primitive germ cell layers Tx: surgery, not biopsy Adjunctive chemotherapy if not complete surgical resection

Germ Cell Tumors: Seminoma Men ages 20-40 years Tx: uniquely sensitive to radiation therapy Debate as to role of chemotherapy and surgical resection, but residual masses should probably be resected Over 90% 5-year survival is typical for seminomatous cancer

Germ Cell Tumors: Nonseminomatous Often symptomatic and malignant More common (>90%) in men Need to look for primary gonadal malignancy Dx:hCG, AFP tumor markers; percutaneous biopsy first when suspected Tx: Primary chemotherapy or radiotherapy; surgery for residual tumor only after all elevated tumor markers have normalized. Over 50% 5-year survival is achievable

Mediastinal Goiter Incidence 1 15% in patients undergoing thyroidectomy t On CT scan, see continuity of cervical and mediastinal components of the thyroid Scintigraphy can be diagnostic if functional Tx: nearly all can be removed through h cervical incision

Mediastinal Parathyroid Adenoma A common location of ectopic parathyroid tumor Tumors are round, encapsulated, small Dx: MRI or nuclear sestamibi scans Tx: surgical resection

Treatment Techniques

Minimally invasive FNA Core needle biopsy Excisional biopsy

Mediastinoscopy

Mediastinoscopy Daniel TM, Jones DR. CTSNET.ORG:2004

Chamberlain procedure Anterior parasternal mediastinoscopy Incision over second costal cartilage Resect in a subperichondrial plane Blunt dissection Wedge resection

Transcervical approach Elevate the sternum Exposure of anterior mediastinum Alternative to anterior mediastinotomy Can convert to sternotomy if exposure is needed

Median Sternotomy Standard approach for primary invasive masses of the anterior mediastinum Optimum exposure Thoracotomy Exposure to all three compartments

Video-Assisted Thoracic Surgery Has been applied for diagnostic biopsy Helpful to evaluate other compartments Intercostal incisions more painful

Summary Clinical evaluation will usually give you the diagnosis. If after initial work-up, the mass appears to be benign, proceed with surgery. Otherwise, proceed with diagnostic biopsy by any variety of ways to access the mediastinum.

References 1. Duwe, BV, et al. Tumors of the Mediastinum. Chest. Vol 123(4): 2893. October 2005. 2. Kucharczuk, JC. Disorders of the Mediastinum and Hilum. ACP Medicine. 2008. 3. Shrager, JB and Patel, V. Mediastinal Procedures. ACS Surgery: Principles & Practice. 2008. 4. Theodore, PR and Jablons, D. Thoracic Wall, Pleura, Mediastinum & Lung. Current Surgical Diagnosis and Treatment 12 th edition. 2006.