Floating left innominate vein neoplastic thrombus: A rare case of mediastinal extension of follicular thyroid carcinoma

Abstract

Objective

We report a case of advanced follicular thyroid cancer with innominate vein involvement. To our knowledge, this seems to be the first case treated in emergency surgery, reported in literature.

Method

A 59-year-old woman with a five-year history of a large and mainly right-sided cervical mass presented with dyspnea, unilateral arm swelling, facial flushing, and venous congestion. An emergency computed tomography scan revealed a thyroid mass extending into the upper mediastinum with displacement and compression of the right jugular vein and carotid artery and apparent adherence to the superior vena cava and left innominate vein.

Results

An emergency total thyroidectomy was performed by means of a sternotomy. The lower portion of the retrosternal goiter projected directly into the left innominate vein, with tumor floating in its lumen. Removal of the neoplastic thrombus was performed, through an incision in the vein, en bloc with the thyroid mass. Both goiter and thrombus were completely replaced by follicular carcinoma.

Conclusions

Accurate preoperative assessment through contrast-enhanced computed tomography is strongly suggested in the presence of enlarged thyroid gland extending into the mediastinum whenever angioinvasion is suspected. This could prevent blinded maneuvers such as digital externalization of the thoracic component of the gland, which can be fatal in cases of cervico-mediastinal goiter extending into great cervical or mediastinal veins.

Keywords

Thyroid carcinoma follicular carcinoma endocrine surgery neoplastic thrombus cancer

Introduction

Macroscopic angioinvasion from follicular thyroid carcinoma is rare and usually leads to a poor outcome.¹ Thompson was the first to describe a successful thrombectomy of an extended tumor thrombus in the mediastinal great veins from follicular thyroid carcinoma.

We report a case of follicular thyroid carcinoma with extensive mediastinal invasion into the left brachiocephalic vein, treated in emergency setting.

Case report

A 59-year-old woman with a five-year history of a large and mainly right-sided cervical mass was referred to our Academic Institution with worsening dyspnea, unilateral arm swelling, facial flushing, and venous congestion. An emergency contrast-enhanced computed tomography (CT) scan revealed a grossly enlarged thyroid mass extending into the upper mediastinum with complete obstruction of the tracheal lumen, displacement and compression of the right jugular vein and carotid artery and apparent adherence to the superior vena cava and left innominate vein (Figure 1).

Figure 1.

Contrast-enhanced CT scan: a grossly enlarged thyroid mass extending into the upper mediastinum with complete obstruction of the tracheal lumen, displacement and compression of the right jugular vein and carotid artery and apparent adherence to the superior vena cava and left innominate vein.

An emergency total thyroidectomy and a prophylactic lymph node dissection of the central compartment were performed by means of a sternotomy, preserving the parathyroid glands and the recurrent laryngeal nerves on both sides, also using 3 × magnification loupe. The tumor mass extended directly into the left innominate vein (Figure 2), with a thrombus arising from the lower portion of the retrosternal goiter, projecting into the vein and floating in its lumen (Figure 3). The vein was clamped at both proximal and distal sites, and an elliptical excision was performed on the anterior wall. The extended tumor thrombus was successfully removed en bloc with the thyroid mass achieving complete primary tumor resection. The left brachiocephalic vein was repaired with 5-0 polypropylene suture. The post-operative course was uneventful and the patient was discharged without complications.

Figure 2.

The tumor mass extended directly into the left innominate vein.

Figure 3.

The tumor thrombus projected into the left innominate vein, floating in its lumen.

A histological examination later revealed that both the goiter and thrombus were completely replaced by follicular carcinoma. The patient received 131I radioisotope and external radiation beam therapy (ERBT) and died five years later due to the progression of the metastatic disease.

Discussion

Vascular invasion is more frequently associated with follicular thyroid carcinoma. Even though the microscopic vascular invasion is well recognized in follicular thyroid cancer, the macroscopic one is a less recognized cause of morbidity and mortality.²

The management of these patients is challenging as they typically present with an advanced and rapidly progressive disease.

Until today, only 33 cases have been documented in the literature (Table 1),^2–20 and the prognosis in most of these has generally been unsatisfactory, ranging from 12 days to five and half years. Thrombectomy should be seen as the best surgical procedure when an intraluminal extension of the disease is evident.¹

Table 1.

Reported cases of thyroid carcinoma with tumor thrombus in the mediastinum great veins.

First author (ref.)	Year	Age (years)/sex	Histology	Involvement	SCV syn^a	Metastasis^b	Surgical management	RI	Prognosis
Thompson³	1978	67/F	FTC	Rt JV to RA	Present	No	Tx >thrombectomy^c	Yes	AWD (36 m)
Perez⁴	1984	48/F	FTC	Lt BCV to SVC	No	No	Thrombectomy >Tx	Yes	Alive (4 m)
Niederle⁵	1990	57/M	FTC	Lt JV to RA	Present	No	Tx >thrombectomy	No	DOD (13 m)
Niederle⁵	1990	79/F	FTC	Rt JV to RA	Present	Lung (bone)	Thrombectomy	Yes	AWD (50 m)
Niederle⁵	1990	53/F	FTC	Lt JV to RA	Present	(Bone, brain)	Tx >reconstruction	Yes	DOD (8 m)
Patel⁶	1997	79/F	PTC	Lt JV to SVC, azygos v.	Present	No	Thrombectomy	No	D^d (12 d)
Lalak⁷	1997	68/F	FTC	JV	No	n.d.	Thrombectomy,	No	AWD (9 m)
Onaran⁸	1998	48/F	PTC	JV	No	n.d.	Segmental resection JV	No	AWD (37 m)
Onaran⁸	1998	68/F	Hürthle	JV	No	n.d.	Segmental resection JV	No	AWD (over 36 m)
Onaran⁸	1998	48/M	Hürthle	JV to SVC	No	n.d.	No surgery (Biopsy)	Yes	D (12 m)
Wiseman⁹	2000	84/M	DTC	Rt JV to SVC and ext. JV	No	n.d.	Biopsy	Yes	D (12 m)
Mishra¹⁰	2001	30/F	Por PTC	JV	No	No	Thrombectomy	No	n.d.
Mishra¹⁰	2001	32/F	PTC	BV, JV	No	No	Reconstruction	Yes	AWD(5 y)
Mishra¹⁰	2001	36/F	Por PTC	JV	No	No	Thrombectomy	Yes	AWD(2 y 6 m)
Mishra¹⁰	2001	36/F	Por	JV	No	No	Thrombectomy	No	D (4 d)
Mishra¹⁰	2001	60/M	Por	JV	No	No	Reconstruction	No	D(1 d)
Koike¹¹	2002	26/F	Por PTC	(LN>) Lt BCV to SVC	No	n.d.	Thrombectomy	No	Alive (8 m)
Hasegawa¹²	2002	78/F	PTC	Rt JV to RA	No	(Lung)	Reconstruction	No	DOD^e (36 d)
Motohashi¹³	2005	64/F	Por PTC	Lt BCV to SVC	Present	No	Reconstruction	Yes	Alive (24 m)
Sugimoto¹⁴	2006	61/M	Por PTC	BCV, SVC, RA	Yes	Reconstruction	No	D (12 d)
Taib¹⁵	2007	66/F	FTC	Rt JV to RA	No	(Lung)	Tx >thrombectomy	Yes	AWD (18 m)
Taib¹⁵	2007	62/F	FTC	Rt JV to RA	Present	(Lung)	Thrombectomy	Yes	AWD (18 m)
Taib¹⁵	2007	45/F	FTC	Rt JV to SVC	No	Lung	Thrombectomy	No	DOD^f (3 wk)
Tripathi¹⁶	2008	48/F	FTC	(LN>) BCV to SVC	No	No	Tx >n.d.	n.d.	n.d.
Yamagami¹⁷	2008	74/M	Por PTC	Lt JV to RA	No	No	Thrombectomy	No	Alive (7 m)
Hyer²	2008	81/F	FTC	Rt JV to SVC	No	(Brain)	Tx >EBRT	Yes	DOD (66 m)
Hyer²	2008	59/F	FTC	JV	No	No	Tx >EBRT	Yes	DOD (23 m)
Hyer²	2008	61/F	Hürthle	JV	No	No	Tx >EBRT	DOD (28 m)
Hyer²	2008	43/F	Por PTC	JV	No	(Lung, bone)	Tx >EBRT	Yes	AWD (53 m)
Hyer²	2008	70/F	PTC	SVC	Yes	No	Tx >EBRT	Yes	DOD (26 m)
Wada¹⁸	2009	64/M	FTC	Lt JV to SVC	Present	No	Reconstruction	Yes	Alive (8 m)
Marcy¹⁹	2009	81/F	Por FTC	JV to SVC^g	Present	No	Thrombectomy >EBRT	Yes	DOD (50 m)
Onoda²⁰	2012	70/F	FTC	Rt JV to SVC	No	Lung	Thrombectomy	Yes	AWD (12 m)

FTC: follicular thyroid cancer, PTC: papillary thyroid cancer, DTC: differentiated thyroid cancer with positive thyroglobulin staining, Por: poorly differentiated, Rt: right, Lt: left, JV: internal jugular vein, SVC: superior vena cava, (LN>): invasion from a metastatic lymph node, n.d.: not described, Tx: thyroidectomy without thrombectomy, Thrombectomy: thrombectomy without vascular reconstruction, EBRT: external beam radiotherapy, RI: radioisotope therapy, d: days, wk: weeks, m: months, AWD: alive with disease, DOD: died of disease, D: died.

Clinically overt SVC syndrome at initial presentation.

Organs with metastatic disease at initial presentation, or (appeared after surgery).

Thyroidectomy as initial surgery, and thrombectomy as secondary surgery.

Died of deterioration of chronic pulmonary disease.

Died of respiratory failure caused by pulmonary dissemination of the tumor, and pneumonia.

Died of massive hemoptysis.

Laterally was not described.

Intrathoracic extension of thyroid tumors should raise suspicion of great vessels involvement and alert the surgeon to the possibility that a sternotomy or cardiopulmonary by-pass may be required.

Depending on the site and degree of the vascular obstruction, the patient may exhibit different clinical manifestations ranging from slight occlusion symptoms to superior vena cava syndrome. But, sometimes the evidence of a venous obstruction may not present itself until the patient undergoes imaging assessment or surgical procedures.²

Since the clinical presentation may be vague or misleading, appropriate preoperative vascular imaging is mandatory whenever angioinvasion is suspected in order to prevent potential lethal complications such as pulmonary embolism or intracranial/intracardiac propagation of the thrombus.¹⁹

An enhanced CT scan can be useful in the diagnosis of great vein thrombosis and the typical findings include: a smooth defect, external compression, a distended vein with an enhanced wall, low-attenuation intraluminal filling defects, and adjacent soft tissue swelling. Taib and Hisham¹⁵ described the importance of the “ring sign,” a rim of contrast around the tumor thrombus on an enhanced CT scan examination which is important in the decision making, indicating whether a thrombectomy is a feasible surgical procedure.

Venous involvement can also be assessed by magnetic resonance imaging and multidetector computed tomography with sagittal, coronal, and 3D vascular reconstructions, which may be helpful to define the localization, extent and nature of the mass, and distinguish between compression and invasion.¹⁹

Thrombectomy and/or resection with reconstruction are the operative procedure of choice. Vascular resection and reconstruction might be chosen in poorly differentiated and papillary thyroid cancers, whereas thrombectomy is often performed for follicular tumors, and is the most effective surgical treatment when it is feasible.¹

Conclusion

We report a case of advanced follicular thyroid cancer with innominate vein involvement. To our knowledge, this seems to be the first case treated in emergency surgery, reported in literature. Accurate preoperative assessment through contrast-enhanced computed tomography is strongly suggested in the presence of enlarged thyroid gland extending into the mediastinum whenever angioinvasion is suspected. This could prevent blinded maneuvers such as digital externalization of the thoracic component of the gland, which can be fatal in cases of cervico-mediastinal goiter extending into great cervical or mediastinal veins.²¹

Footnotes

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest

None declared.

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