|Year : 2014 | Volume
| Issue : 1 | Page : 29-31
Metastatic follicular carcinoma of the thyroid with tumor thrombus extending on to the superior vena cava
Arvind Krishnamurthy1, Vijayalakshmi Ramshankar2
1 Department of Surgical Oncology, Cancer Institute (Women's Indian Association), Adyar, Chennai, India
2 Department of Preventive Oncology, Cancer Institute (Women's Indian Association), Adyar, Chennai, India
|Date of Web Publication||2-Jan-2014|
Department of Surgical Oncology, Cancer Institute (WIA), 36, Sardar Patel Rd, Adyar, Chennai - 600 020
Source of Support: None, Conflict of Interest: None
Microscopic vascular invasion is well-recognized phenomenon in thyroid cancer especially in the follicular and poorly differentiated histological variants; however, massive tumor invasion of the great veins is very rare. Management of these patients is technically challenging and is further complicated due to lack of evidence-based guidelines. Complete resection whenever possible is recommended by most authors as was successfully done in our patient. With the best available evidence, a multimodality therapeutic approach comprising surgery, radioiodine, and external beam radiotherapy may give the best result for patients in whom thyroid cancer is occluding the great veins. We share our experience of thyroid cancer patient with a massive tumor thrombus involving the great veins of the neck and medistinum.
Keywords: Follicular carcinoma thyroid, superior vena cava, tumor thrombus, vascular invasion
|How to cite this article:|
Krishnamurthy A, Ramshankar V. Metastatic follicular carcinoma of the thyroid with tumor thrombus extending on to the superior vena cava. Thyroid Res Pract 2014;11:29-31
|How to cite this URL:|
Krishnamurthy A, Ramshankar V. Metastatic follicular carcinoma of the thyroid with tumor thrombus extending on to the superior vena cava. Thyroid Res Pract [serial online] 2014 [cited 2020 Jun 1];11:29-31. Available from: http://www.thetrp.net/text.asp?2014/11/1/29/124193
| Introduction|| |
Microscopic vascular invasion is well-recognized phenomenon in thyroid cancer especially in the follicular and poorly differentiated histological variants; however, massive tumor invasion of the great veins is rare, only about 38 cases have been documented in the literature. Management of these patients is technically challenging as a majority of them present with advanced and rapidly progressive disease. We share our experience of thyroid cancer patient with a massive tumor thrombus Involving the great veins of the neck and medistinum.
| Case Report|| |
A 55-year-old lady underwent a subtotal thyroidectomy for a presumed multinodular goiter at an outside center. Due to extensive extrathyroidal spread to the surrounding structures including the right internal jugular vein, the tumor was deemed unresectable and complete clearance was not achieved by surgery, and the histopathology of the partly resected tumor suggested a diagnosis of follicular thyroid carcinoma. She was referred to our center for further management with a postoperative computed tomography (CT) scan which demonstrated the residual tumor involving the right lobe of thyroid along with a tumor thrombus with a positive rim sign of size 9 × 3 × 3 cm in the right internal jugular vein extending down the right brachiocephalic vein and superior vena cava (SVC). [Figure 1] and [Figure 2]a and b. The scan also showed moderate-sized right cervical lymph nodes involving levels II-IV, the largest 2 × 2 cm. She was taken up for a curative resection. A median sternotomy was initially done and vascular control of all the major great vessels was obtained. Completion thyroidectomy with thrombectomy was performed by means of a phlebotomy in the right brachiocephalic vein [Figure 3]a and b. A complete primary tumor resection was achieved by sacrificing only the right internal jugular vein and with the addition of a radical neck dissection. The postoperative histopathology confirmed the residual follicular thyroid carcinoma with a tumor thrombus in the right internal jugular vein and also in 10 out of the 21 cervical lymph nodes with perinodal extention [Figure 4]a-d. The postoperative Iodine-131 scan revealed 0.5% neck uptake and the whole body scan was negative. She received 40 grey adjuvant external beam radiation therapy (EBRT) and remnant radioiodine ablation. (80 mill curies of Iodine-131). While on a regular follow-up (after 2 years) with suppressive doses of eltroxin, she was detected to have a rising thyroglobulin and on further evaluation was found to have non iodine avid multiple parenchymal pulmonary metastases. After extensive counseling, the patient wished and continues to be on best supportive care.
|Figure 1: Axial computed tomography scan demonstrating the residual tumor involving the right lobe of thyroid along with a tumor thrombus|
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|Figure 2: Tumor thrombus occupying the right internal jugular vein, brachiocephalic vein, and extending on to the superior venacava, (a) Axial view also demonstrating a positive rim sign (a thin rim of contrast medium surrounding the tumor thrombus), (b) Coronal view|
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|Figure 3: (a) Intraoperative clinical picture showing the tumor thrombus being milked out of the superior vena cava from an opening in the right brachiocepahlic vein, (b) The right internal jugular vein clamped and ligated post the thrombectomy|
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|Figure 4: (a-d) H and E: Section showing blood vessel wall completely filled and infiltrated with tumor (metastatic follicular carcinoma thyroid) The cells are seen as closely packed follicles. The cells are large, round to polygonal with moderate cytoplasm, and large vesicular nuclei with prominent nucleoli. Some cells have bizarre giant nuclei, increased mitosis is seen|
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| Discussion|| |
Obstruction of venous return in the mediastinum and neck, either by extrinsic compression or by tumor invasion of the venous wall and thrombosis is caused by a malignant process in up to 90% of cases, most commonly lung cancer. In less than 1% of the cases, massive invasion into the great veins or compression of the SVC is attributed to a thyroid cancer. 
The clinical presentation of a tumor thrombus in great cervical veins generally varies from asymptomatic presentation to florid SVC syndrome depending on the degree of obstruction. The circulation is well-compensated by collaterals in patients with long-standing venous obstruction and surgery is generally well-tolerated in these groups of patients as was seen in our patient.
Various imaging modalities have been described to aid in an accurate preoperative diagnosis of a tumor thrombus. Ultrasound of the neck is a common imaging modality in the routine evaluation of a thyroid lesion to characterize the nature and location of abnormal thyroid and extrathyroid masses. High frequency Doppler ultrasound, in a case series was found to be a useful noninvasive method to detect tumor extensions to great veins of the neck. 
Imaging studies in the form of magnetic resonance imaging, CT, and positron emission tomography-CT scanning can help differentiate external compression from intraluminal tumor and in delineating the location, extent, and nature of the obstruction or thrombosis.  Demonstration of the cause of the thrombus by Iodine-131 scan is possible because tumor tissue in the vein will generally be iodine avid but simple thrombus will not. 
Management of these patients is technically challenging and is further complicated due to lack of evidence-based guidelines. Complete resection whenever possible is recommended by most authors. A tumor thrombus can sometimes be removed by a thrombectomy alone without the need for simultaneous resection of the great veins , as was successfully done in our patient. Some authors have suggested that a positive ring sign, a thin rim of contrast medium surrounding the tumor thrombus on enhanced CT examination, indicates the feasibility of successful tumor thrombectomy.  This sign may be useful to make a decision regarding the surgical strategy. In cases of fixed adhesion and invasion of the tumor to the intraluminal wall of the great veins, resection of the involved vein along with reconstruction with autologous or artificial grafts is recommended.  During the performance of a thrombectomy, care needs to be taken to isolate and get vascular control of all the great veins in order to prevent tumor embolization.
Surgery should be complemented with adjuvant radioiodine in iodine-avid tumors as this may reduce the risk of recurrence.  The value of EBRT in the management of thyroid cancer remains controversial because published data are conflicting. There is good evidence that EBRT improves local control in patients with gross macroscopic residual disease following surgery. 
Surgical intervention in the presence of SVC syndrome, however, remains controversial because of the treatment dilemma between perioperative morbidity and mortality with aggressive surgery and the poor prognosis with palliative therapy. Without surgery, death follows within a couple of months from tumor embolism or obstruction of the right atrium. , Endovascular therapy as a palliative therapy can be considered if surgery is not feasible. In conclusion, tumor invasion of great veins and forming thrombus is a rare phenomenon in thyroid carcinomas, with the best available evidence, a multimodality therapeutic approach comprising surgery, radioiodine, and EBRT may give the best results.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]