Tracheal invasion and perforation from advanced primary thyroid lymphoma: A case report and literature review

Tracheal invasion and perforation from advanced primary thyroid lymphoma: A case report and literature review

AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI N E AN D SUR G E RY 3 4 ( 2 0 13 ) 55 9–5 6 2 Available online at www.scien...

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AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI N E AN D SUR G E RY 3 4 ( 2 0 13 ) 55 9–5 6 2

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Tracheal invasion and perforation from advanced primary thyroid lymphoma: A case report and literature review☆ Cynthia Chen, MD a , Kathleen M. Tibbetts, MD b,⁎, Andrew B. Tassler, MD b , Bradley A. Schiff, MD b a b

Central Carolina Ear Nose and Throat, Sanford, NC, USA Department of Otorhinolaryngology-Head and Neck Surgery, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx NY, USA

ARTI CLE I NFO

A BS TRACT

Article history:

Objective: We report a case of an elderly female with primary diffuse large B-cell thyroid

Received 1 April 2013

lymphoma causing an extensive tracheal defect that was managed expectantly with good results. Method: Case report Results: This is the only known reported case of a patient with tracheal invasion and perforation caused by primary thyroid lymphoma who has subsequently survived. Conclusion: Due to the rarity of invasive primary thyroid lymphoma there is currently no standard surgical management of the airway. We propose that expectant management with temporary airway protection is an alternative to invasive procedures such as tracheotomy or tracheal stent placement, even in the scenario of serious airway defects. © 2013 Elsevier Inc. All rights reserved.

1.

Introduction

Rapidly expanding thyroid lesions are concerning for anaplastic thyroid carcinoma, but primary thyroid lymphoma (PTL) must also be considered in the differential diagnosis. Primary thyroid lymphoma is not thought to have the propensity for invasion of the adjacent airway as is seen with anaplastic and high grade thyroid carcinomas. We present a case of a large tracheal defect resulting from either mass effect compression of the anterior tracheal wall, or direct local extension and invasion of the primary tumor, and review the management of the patient and her outcome.

2.

Case report

An 80 year old female presented in the office with a one month history of diffuse enlargement of the thyroid gland and a left-

sided neck mass. An FNA was performed that was suggestive of lymphoma and a subsequent excisional biopsy showed diffuse large B cell lymphoma. While awaiting treatment, the patient presented to the emergency room with acutely worsening hoarseness and shortness of breath. Flexible fiberoptic laryngoscopy showed mobile true vocal folds. Her shortness of breath worsened and she was intubated with a 5.0 endotracheal tube as attempts to pass larger tubes through the subglottis were unsuccessful. CT imaging of the neck showed diffuse heterogeneous enlargement of the thyroid gland with significant airway compression (Fig. 1). No direct invasion or defect of the trachea was noted at this time. The patient remained intubated and was started on a modified R-CVP (Rituximab, Cyclophosphamide with 25% dose reduction, Vincristine, and solumedrol) chemotherapy regimen. By the end of the first cycle of chemotherapy, there was a significant reduction in the size and firmness of the patient’s neck mass. The decision



Presented as a poster at the AAO-HNSF Annual meeting September 9–12, 2012. ⁎ Corresponding author. Department of Otorhinolaryngology-Head and Neck Surgery, Montefiore Medical Center, Albert Einstein College of Medicine, 3400 Bainbridge Avenue, 3rd Floor, Bronx NY, 10467, USA. Tel.: +1 718 920 4646. E-mail address: [email protected] (K.M. Tibbetts). 0196-0709/$ – see front matter © 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjoto.2013.04.004

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Fig. 1 – Pre-treatment CT scan showing the large anterior neck mass surrounding the trachea.

was made to extubate the patient but the patient quickly developed respiratory distress requiring reintubation, and a second cycle of chemotherapy was initiated. During the course of the second cycle of chemotherapy, the patient developed increased airway pressure and ventilation became more difficult. Fiberoptic examination through the endotracheal tube revealed tissue obstructing the distal end of the tube. The endotracheal tube was withdrawn slightly, revealing a patent tracheal lumen with an intraluminal irregularity or mass identified in the more distal trachea. Peak pressures and oxygen saturation improved with repositioning of the endotracheal tube. A CT neck was performed (Fig. 2) that showed a large tracheal defect at the level of the thoracic inlet and the endotracheal tube extending 10 mm into the defect. There was pneumomediastinum, a small left anterior pneumothorax, and extensive paratracheal air. The patient was taken to the operating room and a rigid bronchoscopy revealed a large defect in the anterior tracheal wall between the third and 11th tracheal rings (Fig. 3) connecting to a large defect in the soft tissue of the anterior neck where the lymphoma had initially

Fig. 2 – Computed tomography scan showing tracheal perforation due to PTL. The endotracheal tube is seen just posterior to tracheal defect, and there is extensive pneumomediastinum and paratracheal air.

Fig. 3 – Anterior tracheal wall defect seen on rigid bronchoscopy.

presented. The endotracheal tube was passed through the defect and into the normal trachea distal to the defect, secured in place, and the patient was returned to the ICU. Sedation was titrated to prevent agitation and manipulation of the endotracheal tube was avoided. The patient was successfully extubated 3 days later. The patient’s respiratory status remained stable and chemotherapy was continued through the end of the second cycle. The tracheal defect was managed expectantly and no reconstruction was performed. The patient continued to improve clinically and was ultimately discharged from the hospital on hospital day 44. Upon follow up in our clinic, she was without respiratory complaints but was now noted to have decreased adduction of the vocal cords but an otherwise patent upper airway. She was brought back to the operating room one month later for follow-up bronchoscopic evaluation of her airway, which revealed short segment tracheal stenosis caused by fibrotic tissue at the site of the perforation (Fig. 4). The stenotic area was dilated with a 15mm vascular angiocatheter balloon after CO2 laser division of scar tissue. The anterior tracheal wall defect was seen to be mucosalized. The patient tolerated this procedure well and her respiratory status remained stable post-operatively.

Fig. 4 – Short segment tracheal stenosis at the site of perforation. This was asymptomatic and managed with CO2 laser division and balloon dilation.

AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI N E AN D SUR G E RY 3 4 ( 2 0 13 ) 55 9–5 6 2

3.

Discussion

Primary thyroid lymphoma is a rare malignant lesion of the thyroid gland accounting for less than 5% of thyroid cancers [1]. The majority of the lymphomas are of B-cell origin [1,2]. This malignancy tends to occur in elderly females and is associated with a background of Hashimoto’s thyroiditis in up to 80% of cases. The risk of PTL in patients with Hashimoto’s is at least 60 times that of patients without thyroiditis [6]. Diagnosis requires incisional biopsy in the majority of cases, but core needle biopsy can be considered as well for histopathologic diagnosis. Fine needle aspiration biopsy (FNAB) is recommended as a first-line tool as its efficacy has increased due to improved techniques in immunohistochemistry [11]. In the past, PTL and anaplastic carcinoma were difficult to differentiate histologically, but this is no longer the case with the use of flow cytometry and immunohistological staining techniques. It is important to differentiate between two particular cell types of lymphoma: diffuse large B cell lymphoma and mucosa-associated lymphoid tissue (MALT) lymphoma, as MALT lymphomas tend to have a better prognosis and can be treated less aggressively. A 2009 SEER review of 1408 patients with PTL found that certain factors such as age greater than 80 years, stage II–IV disease, no radiation or surgery, and large B-cell or follicular histology conferred a worse prognosis. Eighty eight percent of patients in the database had stage I–II disease and the median survival of all patients was 9.3 years [8]. In the past, surgical intervention has been shown to have no effect on survival in patients with PTL, although it is often important for obtaining tissue for diagnosis and possible palliation for large obstructing lesions. [3] Surgery is now recommended in some studies as primary therapy for MALT lymphomas that are localized to the thyroid (stage IE). Treatment of B cell lymphoma is multimodality (chemotherapy and external beam radiation). Surgical debulking has been shown to have no benefit on patients with stage IE and IIE disease, and is therefore not recommended [7]. PTL can often be difficult to differentiate clinically from anaplastic carcinoma, as both present with a rapidly expanding, firm, anterior neck mass. Similar to anaplastic carcinoma, PTL is often locally invasive, with almost 60% of cases involving tumor already extending outside the gland at presentation [4]. Invasion of the adjacent trachea has been reported only sporadically in the literature, with poor outcomes in patients with these advanced tumors [4,5,9]. In one report [4], a patient with T cell PTL who received cisplatin, doxorubicin, and methylprednisolone developed dyspnea and subcutaneous emphysema requiring endotracheal intubation on day 2 of chemotherapy. The patient underwent bronchoscopy and CT imaging, which showed tracheal perforation and necrotic tumor occluding the airway. The cartilaginous anterior trachea was necrotic from the cricoid cartilage to 4 cm above the carina. The patient expired not long after the airway was stabilized with tracheotomy. Extensive necrosis of the entire cartilaginous trachea was the cause of death [10]. Due to the rarity of the disease, it is difficult to make recommendations on the role of surgery in palliation of patients with PTL and airway involvement. Sippel et al. described a series of 27 patients with PTL of B cell NHL subtype

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who received palliative thyroidectomy (56% lobectomy, 44% subtotal/total thyroidectomy). The most common symptoms were impending airway obstruction in 40% of patients, dyspnea/stridor in 30%, and 30% with dysphagia/pain. Four patients received tracheotomy at the time of surgery because of tracheal invasion, edema, or concern of airway patency. A fifth tracheotomy was performed for inability to maintain the airway postoperatively. One patient died of myocardial infarction postoperative day 10, and three patients died of their disease within 3 years. Overall, the median survival rate was 10 years, similar to that of those patients who undergo chemoradiation. Decannulation rate was not discussed. The authors conclude that there is a subset of patients who could benefit from palliative surgery, such as those with severe compression or invasion of surrounding neck structures [9]. Other interventions such as endotracheal stenting have been suggested as possible palliative procedures for patients with advanced PTL involving the airway. In a series of 30 patients in Belgium who received tracheal stents via flexible bronchoscopy for both benign and malignant thyroid disease, the authors reported a 100% rate of relief of dyspnea immediately, and 88% rate long-term. In several of the patients, invasive tumor was debulked endoluminally with Nd-YAG laser prior to stenting. They had an “acceptable” rate of complications, including granulation formation, stent migration, and stent obstruction. There was one death in this series that was not caused by thyroid disease. The authors suggested that interventional bronchoscopy procedures were a safe option for patients who were not surgical candidates. It is not clear whether our patient’s respiratory distress was due to decreased vocal cord mobility, tracheal invasion or tracheal compression. In consultation with the Oncology service, we elected to proceed with one cycle of chemotherapy after initial airway stabilization. She responded with rapid and dramatic reduction in tumor size, resulting in a large defect of the anterior tracheal wall and anterior neck secondary to tumor necrosis. The defect in the tracheal cartilage presumably arose either from direct invasion by tumor or from compression and destruction of the surrounding tissues and blood supply. We elected to attempt extubation with a known tracheal perforation thinking that either performing a tracheotomy or placing a stent might have negative long term effects on a tenuous airway potentially resulting in long term airway complications. The extent of perforation was fully investigated prior to this decision with both serial CT imaging and rigid bronchoscopic evaluation. The plan was to follow the patient clinically and to repeat bronchoscopy regardless of the clinical status. The patient did well without any intervention, although she later exhibited scar formation in the perforated area of trachea with mild, asymptomatic stenosis that was easily managed with dilation.

4.

Conclusion

Prior to this report, there is no mention in the literature of a patient who survived tracheal perforation in the setting of PTL without aggressive surgical intervention. We propose that if the surgeon is fully aware of the extent of the tracheal defect and the patient lacks other major comorbidities and is responding favorably to multimodality treatment, expectant

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management of the airway is an option in the treatment of invasive PTL with tracheal perforation. REFERENCES

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