Rare Tracheal Tumors and Lesions Initially Diagnosed as Isolated Differentiated Thyroid Cancers

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1 THYROID Volume 23, Number 1, 2013 ª Mary Ann Liebert, Inc. DOI: /thy THYROID SURGERY Rare Tracheal Tumors and Lesions Initially Diagnosed as Isolated Differentiated Thyroid Cancers Sophie Scherl, 1 Eran E. Alon, 2 William E. Karle, 1,3 Jason B. Clain, 1 Azita Khorsandi, 4 and Mark L. Urken 5 Background: Thyroid carcinoma with tracheal invasion is uncommon; however, this is significantly more prevalent than primary tracheal tumors. Rare tracheal tumors at the level of the thyroid can be misinterpreted as invasive thyroid cancer upon initial diagnosis. We present a series of tumors within the tracheal wall that were initially misdiagnosed as isolated, but aggressive, thyroid cancer, and later diagnosed to be different histopathologic entities. Methods: The series consisted of four women and five men, all but two age 60 or older, who were initially diagnosed with tracheal invasion from differentiated thyroid carcinoma (DTC). Eight had obstructive airway and one experienced gagging and choking sensations. Preoperatively, the patients underwent fineneedle aspiration (FNA) and imaging studies. A complete resection of the involved airway in combination with the thyroid gland was performed in all patients. Results: In this series of patients, the final diagnosis was tracheal stenosis, recurrent laryngeal nerve schwannoma, papillary thyroid carcinoma (PTC) with benign intratracheal thyroid tissue, adenoid cystic carcinoma, and squamous cell carcinoma, each in one patient. Two patients had a tracheal chondrosarcoma, and two patients had collision tumors (PTC with laryngeal squamous cell carcinoma). All patients were misunderstood preoperatively as having isolated DTC with aggressive involvement of the trachea. An accurate diagnosis in these cases was difficult due to misleading FNA readings, thought due to the FNA needle passing through the thyroid before reaching the trachea or a tumor that abuts both structures on imaging. Primary tracheal tumors and a nontumorous lesion, as well as benign thyroidal masses, mimicked invasive thyroid carcinoma in this preoperative setting. Conclusions: Various entities other than thyroid cancer can masquerade as invasive thyroid cancer. In patients with an FNA showing thyroid tissue or suggesting PTC, but also have obstructive or other airway, physician awareness is needed to consider the distinct possibility of a primary tracheal lesion. Obtaining the correct preoperative diagnosis is essential for accurate surgical planning for patients with tracheal tumors. Introduction Imaging studies play an important role in the diagnosis, staging, and surveillance of thyroid cancer. Mapping the extent of locoregional disease plays a pivotal role in surgical planning and adjuvant therapy. Thyroid carcinoma has the potential to spread into the trachea due to its location and proximity. Tracheal invasion is a poor prognostic indicator and increases the potential for significant such as dyspnea and hemoptysis as well as patient death, often secondary to hemorrhage or asphyxiation (1). Apart from its significant impact on prognosis, tracheal involvement also alters surgical planning, which must include some degree of tracheal resection to achieve oncologic clearance around the disease (2). Commonly ultrasonography, magnetic resonance imaging (MRI), or computed tomography is used to diagnose and evaluate the presence of tracheal invasion; however, not all abnormal findings necessarily reflect invasive thyroid carcinoma. A fine-needle aspiration (FNA) biopsy is frequently Portions of this article were presented at the 19th Annual Meeting and Clinical Congress of the American Association of Clinical Endocrinologists, Boston, Massachusetts, April 21 25, Thyroid Head and Neck Cancer Foundation, New York, New York. 2 Department of Otolaryngology Head and Neck Surgery, Chaim Sheba Medical Center, Tel Hashomer, Israel. 3 Albert Einstein College of Medicine, New York, New York. Departments of 4 Radiology and 5 Otolaryngology Head and Neck Surgery, Beth Israel Medical Center, New York, New York. 79

2 80 SCHERL ET AL. used to evaluate the presence of thyroid cancer in patients with abnormal physical or radiologic findings. This diagnostic test has a specificity as high as 90.1%, yielding a small, but significant, number of false-positive results (3). When diagnosing a potential invasive thyroid carcinoma, it is important to keep in mind the wide differential of tracheal tumors. However, to date, there is very little literature on primary tracheal lesions presenting as thyroid carcinoma to aid the surgeon in this diagnosis. Thirteen cases have been previously reported, with the largest two studies containing only four patients (4 9). Herein, we report nine cases that were originally diagnosed as being highly suspicious for thyroid carcinoma with tracheal invasion, but were later redefined based on final histopathology or FNA. To our knowledge,thisisthelargestcaseseriesofpatientspresentingwith signs and suspicious for thyroid cancer with tracheal invasion, which were later diagnosed as rare tracheal tumors, a tracheal lesion, collision tumors, or benign thyroid rests. Sex/age Pt 1 F/60s Mild obstructive AW Pt 2 M/50s Obstructive AW and paralyzed vocal fold Pt 3 M/70s Obstructive AW Pt 4 a M/70s Obstructive AW, neck mass, hoarseness, dysphagia, paralyzed vocal fold Pt 5 a F/20s Gagging and choking sensation Pt 6 M/80s Obstructive AW, neck mass, hoarseness, hypothyroidism, paralyzed vocal fold Pt 7 M/80s Obstructive AW Pt 8 F/60s Obstructive AW Pt 9 F/70s Obstructive AW Table 1. Case Series Summary Methods A retrospective chart review was performed for nine patients who presented to our senior surgeon with an initial diagnosis of metastatic thyroid cancer with tracheal invasion. Hospital records were reviewed, and data were collected regarding each patient s initial presentation, FNA, imaging studies, and final pathology report. The FNA and/or final pathology for all of these patients showed rare tracheal tumors, benign rests of thyroid tissue, or tracheal stenosis. This research was approved by the Beth Israel Medical Center Institutional Review Board (IRB ). It adheres to all legal requirements of this institution and State. Results Nine patients were retrospectively identified who had preoperative findings suggestive of an invasive thyroid cancer of the trachea, but were later found to have a different diagnosis. This cohort included five men and four women, two of which were previously reported in the literature (19, 20). One patient was in her 20s, while the rest were in their sixth through ninth decades of life. Of the nine patients, eight initially presented with obstructive airway, while one complained of dysphagia. Three of these patients had a paralyzed vocal fold, and two had palpable neck masses. The two patients with collision tumors both presented with hoarseness. Eight of the nine patients underwent an FNA, of which seven were interpreted as either papillary thyroid carcinoma (PTC) or highly suspicious for a thyroid neoplasm, while the last was Presenting signs and Fine needle aspiration Operation Final diagnosis Suspicious for thyroid neoplasm Suspicious for highgrade thyroid ca Tx and segmental tracheal resection Partial tracheal resection and thyroid lobectomy Tracheal stenosis Recurrent laryngeal nerve schwannoma Suspicious for highgrade thyroid ca Partial laryngectomy and thyroid lobectomy Thyroid cartilage chondrosarcoma PTC Tx, TL, ND Collision tumor: PTC and laryngeal SCCA PTC Total tx, partial tracheal and cricoid resection PTC with benign intratracheal thyroid tissue PTC Tx and TL Collision tumor: PTC and laryngeal SCCA Not available Adenoid cystic carcinoma High-grade thyroid ca with squamous differentiation Segmental tracheal resection and left thyroid lobectomy Segmental tracheal resection and thyroid lobectomy Total tx, TL, bilateral ND, tracheal resection, and TEP Tracheal chondrosarcoma Adenoid cystic carcinoma Poorly differentiatedstage IVa SCCA Figures 1, 2, 3, and 4 refer to patients 1, 2, 6, and 7, respectively. a Patients number 4 and 5 in Table 1 were previously reported in the literature (19,20). AW, airway; ca, cancer; F, female; M, male; ND, neck dissection; Pt, patient; PTC, papillary thyroid carcinoma; SCCA, squamous cell carcinoma; TEP, tracheoesophageal puncture; TL, total laryngectomy; Tx, thyroidectomy.

3 RARE TRACHEAL TUMORS 81 FIG. 1. (a) Axial computed tomography (CT) with contrast. Left thyroid mass neoplasm with circumferential tracheal mucosa thickening. (b) Intraoperative gross photo of the benign thyroid mass (corresponds with patient 1 from Table 1). interpreted as an adenoid cystic carcinoma. Cross-sectional imaging studies were performed on all patients, which revealed an intraluminal tracheal soft tissue mass adjacent to the thyroid gland. The initial interpretation of the radiographic images included invasive thyroid cancer. All patients were later found to have a different histologic diagnosis than what had been presumed preoperatively. The final diagnoses in this series (Table 1) were tracheal stenosis (n = 1) with unknown etiology, recurrent laryngeal nerve schwannoma (n = 1), thyroid cartilage chondrosarcoma (n = 1), tracheal chondrosarcoma (n = 1), collision tumor PTC and laryngeal squamous cell carcinoma (n = 2), PTC with benign intratracheal thyroid tissue (n = 1), adenoid cystic carcinoma (n = 1), and squamous cell carcinoma (n = 1). Discussion The correct distinction of invasive thyroid cancer from other pathological entities is crucial for appropriate preoperative and operative surgical planning. The prognosis for survival is excellent among patients with benign processes such as a tracheal thyroid rest, tracheal stenosis, and a recurrent laryngeal nerve schwannoma, as compared to those with thyroid carcinoma invading the trachea. Nevertheless, these patients with benign intratracheal masses would have still required a partial resection of the trachea due to obstructive airway with the exception of patient 5. This patient presented with of gagging and choking, and would have undergone a thyroidectomy for PTC, without a tracheal resection, if the correct diagnosis had been identified before surgery. Tracheal resections were necessary for the remainder of the patients in this study. Adult tracheal tumors are exceedingly rare. A review of tracheal tumors revealed that 80% 90% are malignant (10). The reported incidence of primary tracheal tumors is two cases per million, per year (11). The most common primary malignant tumors are squamous cell and adenoid cystic carcinoma (12); however, a variety of other benign and malignant neoplasms have been reported (Table 2). The incidence of thyroid carcinoma is 110 per 1 million per year (13), with as low as 1% of patients having tracheal involvement (1). Therefore, secondary invasion of the trachea by invasive thyroid carcinoma is significantly more prevalent than primary tracheal tumors (14). This can lead clinicians astray in the initial findings of patients presenting with tracheal masses. In cases in which there is a mass compressing or invading the trachea, patients often present with similar obstructive airway regardless of the etiology of the mass. Physical findings that raise the suspicion for significant laryngotracheal involvement include hoarseness and signs of airway narrowing. When there is only tracheal wall involvement without compromise of the lumen or the recurrent laryngeal nerve, invasion may be diagnosed preoperatively with visualization using bronchoscopy or through imaging. Bronchoscopic findings suggestive of tracheal involvement include localized mucosal redness, telangiectasia, mucosal elevation, mucosal edema, or mucosal erosion (15). Ultrasonography may also be used, and in one report by Tomoda FIG. 2. (a) Axial CT with contrast. Left tracheal mass with suspicious extension into the trachea and esophagus. (b) Intraoperative photo of the mass (corresponds with patient 2 from Table 1).

4 82 SCHERL ET AL. Table 2. Tracheal Tumors Benign neoplasms Malignant neoplasms FIG. 3. Axial CT of the neck with contrast shows a bulky lesion of the right lobe of the thyroid gland with destruction of the right one-half of the cricoid cartilage and associated paralaryngeal extension (corresponds with patient 6 from Table 1). et al., the sensitivity and specificity of ultrasonography for tracheal invasion were 91% and 93%, respectively (16). Wang et al. also found that tracheal invasion was evident on MRI if the images demonstrated soft tissue signal in the tracheal cartilage, an intraluminal mass, or a tumor abutted a circumference of 180 or more of the trachea (17). FIG. 4. Axial CT of the neck without contrast shows a bulk soft tissue mass with internal calcification involving the left lateral and anterior walls of the cervical trachea with both extra- and intraluminal components (corresponds with patient 7 from Table 1). Chondroblastoma Chondroma Fibroma Fibromatosis Fibrous histiocytoma Granular-cell tumor Glomus tumor Haemangioma Haemangioperycitoma Hamartoma Inflammatory pseudotumor Leiomyoma Lipoma Mucous-gland adenoma Myoepithelioma Oncocytoma Papilloma Papillomatosis Paraganglioma Peripheral nerve sheath tumor Pleomorphic adenoma Plexiformneurofibroma Pyogenic granuloma Schwannoma Adenocarcinoma Adenoid cystic carcinoma Carcinoid Chondrosarcoma Carcinosarcoma Invasive fibrous tumor Large cell carcinoma Leiomyosarcoma Lymphoepithelial carcinoma Lymphoma Malignant fibrous histiocytoma Malignant melanoma Mucinous adenocarcinoma Mucoepidermoid carcinoma Rhabdomyosarcoma Small cell carcinoma Spindle cell carcinoma Squamous cell carcinoma Secondary tracheal neoplasms Direct invasion Metastatic involvement Boldface indicates patient diagnosis on final pathology within this case series. Laryngotracheal invasion by a thyroid carcinoma is a negative prognostic indicator for overall survival as demonstrated by McCaffrey (18). Identifying laryngotracheal invasion and determining the extent of disease play a major role in the preoperative discussion with the patients and their surgical planning. It is generally accepted that luminal involvement of the trachea will require either window or complete circumferential resection with primary closure. When only partial thickness of the tracheal wall is involved, there is no consensus as to the most appropriate surgical approach, with a tracheal shave procedure representing a more conservative approach to surgical management. In the seven cases where FNAs were read as highly suspicious for thyroid carcinoma or as PTC, five were incorrectly diagnosed preoperatively. The two that did demonstrate PTC on final pathology were found to have collision tumors in conjunction with squamous cell carcinoma. Therefore, it is important to recognize that the cytopathological interpretation may be misguided if the path of the needle used to perform the FNA took a course through a portion of the thyroid gland, thereby including thyroid cells in the specimen. If the biopsy needle did pass though the thyroid gland to reach the mass, it should be reported with the sample, as thyroidal tissue present in the sample may confuse the diagnosis. Since the preoperative needle aspiration passed through thyroid tissue, the benefit of thyroglobulin assays is diminished and potentially misleading. In conclusion, while the majority of cases of tracheal invasion at the level of the thyroid appear to be aggressive thyroid cancer, it is important for the clinician to consider alternative diagnoses. We have presented a series of cases of patients with intraluminal neoplasms that mimicked invasive thyroid

5 RARE TRACHEAL TUMORS 83 cancer, but ultimately proved to be caused by a different pathologic etiology. Considering a wide differential and obtaining a correct preoperative diagnosis should benefit the patient with a more precise, targeted resection of the tumor, as well as the tracheal component. This report is intended to provide the clinician with an awareness of the range of histopathologic entities that may be misinterpreted as invasive thyroid cancer. Disclosure Statement The authors declare that no competing financial interests exist. References 1. Czaja JM, McCaffrey TV 1997 The surgical management of laryngotracheal invasion by well-differentiated papillary thyroid carcinoma. Arch Otolaryngol Head Neck Surg 123: Gaissert HA, Honings J, Grillo HC, Donahue DM, Wain JC, Wright CD, Mathisen DJ 2007 Segmental laryngotracheal and tracheal resection for invasive thyroid carcinoma. Ann Thorac Surg 83: Raab SS, Vrbin CM, Grzybicki DM, Sudilovsky D, Balassanian R, Zarbo RJ, Meier FA 2006 Errors in thyroid gland fineneedle aspiration. Am J Clin Pathol 125: Na DG, Han MH, Kim KH, Chang KH, Yeon KM 1995 Primary adenoid cystic carcinoma of the cervical trachea mimicking thyroid tumor: CT evaluation. J Comput Assist Tomogr 19: Zirkin HJ, Tovi F 1984 Tracheal carcinoma presenting as a thyroid tumor. J Surg Oncol 26: Nuwal P, Dixit R, Singhal AK 2010 Primary adenoid cystic carcinoma of trachea presenting as midline neck swelling and mimicking thyroid tumor: a case report and review of literature. Lung India 27: Idowu MO, Reiter ER, Powers CN 2004 Adenoid cystic carcinoma. Am J Clin Pathol 121: Natarajan S, Greaves TS, Raza AS, Cobb CJ 2004 Fine needle aspiration of an adenoid cystic carcinoma of the larynx mimicking a thyroid mass. Diagn Cytopathol 30: Li W, Ellerbroek NA, Libshitz HI 1990 Primary malignant tumors of the trachea: a radiologic and clinical study. Cancer 66: Grillo HC 2004 Surgery of the Trachea and Bronchi. Pmph USA Ltd., Hamilton, Ontario. 11. Webb BD, Walsh GL, Roberts DB, Sturgis EM 2006 Primary tracheal malignant neoplasms: the University of Texas MD Anderson Cancer Center experience. J Am Coll Surg 202: Shadmehr MB, Farzanegan R, Graili P, Javaherzadeh M, Arab M, Pejhan S, Karam MB, Abbasidezfouli A 2011 Primary major airway tumors; management and results. Eur J Cardiothorac Surg 39: Ries L, Melbert D, Krapcho M, Stinchcomb D, Howlader N, Horner M, et al SEER Cancer Statistics Review, National Cancer Institute, Bethesda, MD, Schneider P, Schirren J, Muley T, Vogt-Moykopf I 2001 Primary tracheal tumors: experience with 14 resected patients. Eur J Cardiothorac Surg 20: Koike E, Yamashita H, Noguchi S, Yamashita H, Ohshima A, Watanabe S, Uchino S, Takatsu K, Nishii R 2001 Bronchoscopic diagnosis of thyroid cancer with laryngotracheal invasion. Arch Surg 136: Tomoda C, Uruno T, Takamura Y, Ito Y, Miya A, Kobayashi K, Matsuzuka F, Kuma K, Miyauchi A 2005 Ultrasonography as a method of screening for tracheal invasion by papillary thyroid cancer. Surg Today 35: Wang JC, Takashima S, Takayama F, Kawakami S, Saito A, Matsushita T, Matsuba H, Kobayashi S 2001 Tracheal invasion by thyroid carcinoma: prediction using MR imaging. AJR Am J Roentgenol 177: McCaffrey JC 2006 Aerodigestive tract invasion by welldifferentiated thyroid carcinoma: diagnosis, management, prognosis, and biology. Laryngoscope 116: Brandwein M, Som P, Urken M 1998 Benign intratracheal thyroid: a possible cause for preoperative overstaging. Arch Otolaryngol Head Neck Surg 124: Jacobson AS, Wenig BM, Urken ML 2008 Collision tumor of the thyroid and larynx: a patient with papillary thyroid carcinoma colliding with laryngeal squamous cell carcinoma. Thyroid 18: Address correspondence to: Mark L. Urken, MD, FACS Department of Otolaryngology Head and Neck Surgery Beth Israel Medical Center New York, NY murken@chpnet.org