Malignant Immature Teratoma of the Larynx and Trachea with Functional Osteocutaneous Free Flap Reconstruction

Photos Literature Review References Malignant Immature Teratoma of the Larynx and Trachea with Functional Osteocutaneous Free Flap Reconstruction Samer Al-Khudari1 MD, Saurabh Sharma2 MD PhD, Robert Stapp3 DO, Michael J. Simoff4 MD, Tamer A. Ghanem1 MD PhD 1Department of Otolaryngology-Head & Neck Surgery Henry Ford Health System, Detroit, Michigan 2Wayne State University College of Medicine, Detroit, Michigan 3Department of Pathology, Henry Ford Health System, Detroit, Michigan 4Department of Pulmonary Medicine, Henry Ford Health System , Detroit Michigan Objective: To present a case of malignant immature teratoma of the larynx and trachea managed by non-circumferential cricotracheal resection and reconstruction with an osteocutaneous radial forearm free flap. Study Design: Case report and literature review. Methods: Literature review of tracheal teratoma in adults and methods for partial cricotracheal reconstruction with discussion of a recent representative case within our tertiary health care system. Results: We present the case of a 27 year old male who presented with new onset stridor found to have a 4 cm mass obstructing his subglottis and trachea diagnosed as a malignant immature teratoma. This tumor was locally aggressive with extension into the trachea, subglottis, thyroid gland, and recurrent laryngeal nerve. He subsequently underwent non-circumferential cricotracheal resection including 6cm of trachea, central neck dissection, hemithyroidectomy, recurrent laryngeal nerve sacrifice, and reconstruction with an osteocutaneous radial forearm flap. Postoperatively the patient has been able to cap his tracheostomy and have near normal quality speech. Conclusions: Cervical teratomas are rarely encountered in adults. This represents the first reported case of a malignant immature teratoma with involvement of the larynx and trachea. We also present a novel technique at cricotracheal reconstruction using an osteocutaneous radial forearm free flap avoiding a laryngectomy. 1. Kuhel WI, Yagoda M, Peterson P. Benign cervical teratoma in the adult: report of a rare case with dense fibrosis involving adjacent vital structures. Otolaryngol Head Neck Surg 1996 Jul;115(1):152‐5. 2. Jordan RB, Gauderer MW. Cervical teratomas: an analysis. Literature review and proposed classification. J PediatrSurg 1988 Jun;23(6):583‐91. 3. Kim E, Bae TS, Kwon Y, et al. Primary malignant teratoma with a primitive neuroectodermal tumor component in thyroid gland: a case report. J Korean Med Sci 2007 Jun;22(3):568‐71. 4. Als C, Laeng H, Cerny T, et al. Primary cervical malignant teratoma with a rib metastasis in an adult: five‐year survival after surgery and chemotherapy. A case report with a review of the literature. Ann Oncol 1998 Sep;9(9):1015‐22. 5. Djalilian HR, Linzie B, Maisel RH. Malignant teratoma of the thyroid: review of literature and report of a case. Am J Otolaryngol 2000 Mar;21(2):112‐5. 6. Fujiwara T, Nishino K, Numajiri T. Tracheal reconstruction with a prefabricated and double‐folded radial forearm free flap. J PlastReconstrAesthetSurg 2009 Jun;62(6):790‐4. 7. Teng MS, Malkin BD, Urken ML. Prefabricated composite free flaps for tracheal reconstruction: a new technique. Ann OtolRhinolLaryngol 2005 Nov;114(11):822‐6. 8. Yu P, Clayman GL, Walsh GL. Human tracheal reconstruction with a composite radial forearm free flap and prosthesis. Ann ThoracSurg 2006 Feb;81(2):714‐6. Teratomas are germ cell tumors composed of multiple cell types derived from all three germ layers (endoderm, ectoderm and mesoderm). These tumors may be poorly differentiated in the immature variant and well differentiated in the mature type.1 Immature teratomas are more likely malignant and mature teratomas are generally benign. The first reported case of a cervical teratoma was in 1854 by Hess. Since then, about 300 cases of cervical teratomas have been reported in the literature. A majority of which are found in infants and are commonly benign. It has been proposed that teratomas arise from the embryonic thyroid anlage despite frequent lack of clear association with the gland at excision or autopsy, or absence of thyroid tissue within the excised specimen.2 In our patient, the tumor was found to be directly involving the thyroid parenchyma which supports this theory. 43 cases of cervical teratomas in adults with 32 malignant and 11 benign cases have been reported. Malignant cervical teratoma is an extremely aggressive tumor with a poor prognosis. Complete surgical excision followed by extensive chemotherapy is recommended for most patients with malignant teratoma.3 With surgery alone median survival has been reported to be 8 to 22 months.3, 4 Patients receiving adjuvant chemotherapy seems to increase five year clinical outcome.4, 5 Chemotherapy regimens usually consist of bleomycin, cisplatin and etoposide (BEP). Currently surgical excision followed by chemotherapy is the recommended treatment for malignant teratomas. 3 Reconstruction of cricotracheal defects presents a challenge due to the structural complexity and unique properties of the airway. The ideal reconstruction must not collapse during respiration, have some degree of mobility to allow for neck movements, and allow for clearance of airway secretions. For circumferential defects, generally end-to-end anastomosis is preferred and various laryngeal release and mediastinal release procedures can be performed. Reconstruction techniques involving foreign materials, cadaveric tissues, autogenous tissues and engineered tissue have been attempted, but no largely successful method for reconstruction has been established. In 2007 Fujiwara et. al. describe a two stage reconstruction using a radial forearm free flap implanted with costal cartilage used in 2 patients for 3.5 cm and 4.6 cm in non-circumferential tracheal defects6. Teng et al. used a similar two stage technique containing curved strips of costal cartilage to mimic tracheal rings implanted in a radial forearm flap to create a laryngotracheal anterior wall defect7. Yu et al. reported a single stage technique for reconstruction of a 6.5cm tracheal defect using a conduit composed of a radial forearm free flap with a combined PolyMax mesh and Hemashield vascular graft for rigid support8. Conclusion Case Report Our case is unique as it represents a novel single stage technique to reconstruct a non circumferential defect that involves the cricoid and trachea. We believe that this can be applied to large cricotracheal and isolated tracheal defects secondary to other causes and possibly contribute to advancement in stenosis surgery. This also represents the first reported case of a malignant immature teratoma in an adult to involve the larynx. History: 27 year old male who presented with 2 weeks of new onset stridor. He was found to have a 4 cm mass obstructing 90% his subglottis and trachea. A biopsy and intraluminal debulking was performed and a malignant immature teratoma was diagnosed. He underwent non circumferential right partial cricotracheal resection of the subglottis including 6cm of trachea, central neck dissection, hemithyroidectomy and recurrent laryngeal nerve sacrifice. His 50% of the right lateral cricoid and 75% of the anterolateral tracheal circumference was resected. Reconstruction: Tracheal reconstruction was performed in a single stage with an osteocutaneous radial forearm flap placed in a semi-rigid technique. The osseous portion of the free flap was positioned outside of the lumen and lateral tension sutures were used to provide rigidity and prevent lumen collapse. The superior aspect of the osseous bone was fixed with a “y” titanium plate secured to the thyroid cartilage with a suture instead of screws to allow passive motion. A second titanium plate was then rigidly fixed from the inferior aspect of the radius bone to the left cricoid which also allows passive motion since the radius bone is only passively attached at its superior aspect. An intraluminal silicone stent was used and was removed two weeks later. Post operative course: The stent was removed after 12 days and the lumen was patent and healthy. His tracheostomy was immediately downsized to a size 4 tracheostomy tube. Post operatively, the patient has a patent reconstructed function lumen with left vocal fold motion and is able to create good voice. He developed mild suprastomal stenosis at week 4 and is currently undergoing endoscopic laser excision. Despite this he has maintained a patent lumen with good voice 3 months post-operatively and tolerating a size 4 tracheostomy tube. We anticipate decannulation after successful excision of the mild scarring. Figure 1. Gross sections after formalin fixation (left). The mass involves both the larynx (single arrow head) and thyroid gland (double arrow heads). Histological images (right). Cartilage and epithelial lined ductules are present (a). Primitive neuroepithelium showing rosette formation (b) and frequent mitosis (c). Figure 2. Axial CTLarge intra-luminal mass involving thyroid gland and cricoid cartilage. Figure 3. Tumor prior to resection involving cricoid (arrow) and thyroid gland. Figure 4. Surgical defect after cricotracheal resection. Remnant tracheal rings (arrow). Figure 5. Superior aspect of radius bone prior to suture suspension to cricoid. Figure 6. Rigidly fixed inferior aspect of radius bone with stent placed intraluminally prior to mucosal closure. Figure 7. Image of patent lumen 1 month postoperatively via retroflexed flexible scope at stoma. Fascia of osteocutaneous flap covering right half of trachea and subglottis (arrow).