Functional Analysis of Injectable Substance Treatment on Surgically Injured Rabbit Vocal Folds.

OBJECTIVES: The objective of this study was to evaluate the efficacy of immediate injection treatments of dexamethasone, hyaluronic acid (HA)/gelatin (Ge) hydrogel and glycol-chitosan solution on the phonatory function of rabbit larynges at 42 days after surgical injury of the vocal folds, piloting a novel ex vivo phonatory functional analysis protocol. METHODS: A modified microflap procedure was performed on the left vocal fold of 12 rabbits to induce an acute injury. Animals were randomized into one of four treatment groups with 0.1 mL injections of dexamethasone, HA/Ge hydrogel, glycol-chitosan or saline as control. The left mid vocal fold lamina propria was injected immediately following injury. The right vocal fold served as an uninjured control. Larynges were harvested at Day 42 after injection, then were subjected to airflow-bench evaluation. Acoustic, aerodynamic and laryngeal high-speed videoendoscopy (HSV) analyses were performed. HSV segments of the vibrating vocal folds were rated by three expert laryngologists. Six parameters related to vocal fold vibratory characteristics were evaluated on a Likert scale. RESULTS: The fundamental frequency, one possible surrogate of vocal fold stiffness and scarring, was lower in the dexamethasone and HA/Ge hydrogel treatment groups compared to that of the saline control (411.52±11.63 Hz). The lowest fundamental frequency value was observed in the dexamethasone group (348.79±14.99 Hz). Expert visual ratings of the HSV segments indicated an overall positive outcome in the dexamethasone treatment group, though the impacts were below statistical significance. CONCLUSION: Dexamethasone injections might be used as an adjunctive option for iatrogenic vocal fold scarring. An increased sample size, histological correlate, and experimental method improvements will be needed to confirm this finding. Results suggested a promising use of HSV and acoustic analysis techniques to identify and monitor post-surgical vocal fold repair and scarring, providing a useful tool for future studies of vocal fold scar treatments.

Pediatric Vocal Fold Paresis and Paralysis: A Narrative Review.

Importance: Vocal fold paralysis (VFP) results from the disruption of neural motor outputs to laryngeal muscles. Children with VFP manifest various degrees of difficulties in phonation, breathing, and swallowing. Although the etiologic characteristics and symptoms of VFP are well established in adults, corresponding clinical profiles are notably different in children. Clinical management of VFP is particularly challenging in children because their larynges are still actively developing and the recovery of disrupted laryngeal nerves is often unpredictable. This review discusses the neurologic conditions and diagnostic and treatment considerations in pediatric VFP. Observations: Injury to the peripheral laryngeal nerves and certain central nervous system diseases, such as Arnold-Chiari malformation type II, can result in VFP in infants and children. The incidence of unilateral vs bilateral VFP is variable across pediatric studies. Most reported VFP cases are associated with injury of the recurrent laryngeal nerve. Laryngeal electromyography requires needle insertion that must be performed under anesthesia with special care in the pediatric setting. Neither normative values nor standardized procedures of laryngeal electromyography are currently established for the pediatric population. Laryngeal reinnervation, endoscopic arytenoid abduction lateropexy, and laryngeal pacing are plausible treatment options for pediatric VFP. Despite these new advances in the field, no corresponding efficacy data are available for clinicians to discern which type of patients would be the best candidates for these procedures. Conclusions and Relevance: The neuroanatomy and neurophysiology of VFP remain more elusive for the pediatric population than for adults. Basic and clinical research is warranted to fully comprehend the complexity of this laryngeal movement disorder and to better inform and standardize clinical practice.

Characterizing Vocal Fold Injury Recovery in a Rabbit Model With Three-Dimensional Virtual Histology.

OBJECTIVES/HYPOTHESIS: In animal studies of vocal fold scarring and treatment, imaging-based evaluation is most often conducted by tissue slicing and histological staining. Given variation in anatomy, injury type, severity, and sacrifice timepoints, planar histological sections provide limited spatiotemporal details of tissue repair. Three-dimensional (3D) virtual histology may provide additional contextual spatial information, enhancing objective interpretation. The study's aim was to evaluate the suitability of magnetic resonance imaging (MRI), microscale computed tomography (CT), and nonlinear laser-scanning microscopy (NM) as virtual histology approaches for rabbit studies of vocal fold scarring. METHODS: A unilateral injury was created using microcup forceps in the left vocal fold of three New Zealand White rabbits. Animals were sacrificed at 3, 10, and 39 days postinjury. ex vivo imaging of excised larynges was performed with MRI, CT, and NM modalities. RESULTS: The MRI modality allowed visualization of injury location and morphological internal features with 100-µm spatial resolution. The CT modality provided a view of the injury defect surface with 12-µm spatial resolution. The NM modality with optical clearing resolved second-harmonic generation signal of collagen fibers and two-photon autofluorescence in vocal fold lamina propria, muscle, and surrounding cartilage structures at submicrometer spatial scales. CONCLUSIONS: Features of vocal fold injury and wound healing were observed with MRI, CT, and NM. The MRI and CT modalities provided contextual spatial information and dissection guidance, whereas NM resolved extracellular matrix structure. The results serve as a proof of concept to motivate incorporation of 3D virtual histology techniques in future vocal fold injury animal studies. LEVEL OF EVIDENCE: NA Laryngoscope, 131:1578-1587, 2021.

Laryngotracheal Reconstruction in the Pediatric Burn Patient: Surgical Techniques and Decision Making.

The management of laryngotracheal stenosis (LTS) in the pediatric burn patient is complex and requires a multidisciplinary approach. The mainstay of treatment for LTS is laryngotracheal reconstruction (LTR), however, limited reports of burn-specific LTR techniques exist. Here, we provide insight into the initial airway evaluation, surgical decision making, anesthetic challenges, and incision modifications based on our experience in treating patients with this pathology. The initial airway evaluation can be complicated by microstomia, trismus, and neck contractures-the authors recommend treatment of these complications prior to initial airway evaluation to optimize safety. The surgical decision making regarding pursuing single-stage LTR, double-stage LTR, and 1.5-stage LTR can be challenging-the authors recommend 1.5-stage LTR when possible due to the extra safety of rescue tracheostomy and the decreased risk of granuloma, which is especially important in pro-inflammatory burn physiology. Anesthetic challenges include obtaining intravenous access, securing the airway, and intravenous induction-the authors recommend peripherally inserted central catheter when appropriate, utilizing information from the initial airway evaluation to secure the airway, and avoidance of succinylcholine upon induction. Neck and chest incisions are often within the TBSA covered by the burn injury-the authors recommend modifying typical incisions to cover unaffected skin whenever possible in order to limit infection and prevent wound healing complications. Pediatric LTR in the burn patient is challenging, but can be safe when the surgeon is thoughtful in their decision making.

Lessons learned to aid the successful outcome of pediatric recurrent laryngeal nerve reinnervation.

While Ansa to recurrent laryngeal nerve reinnervation is gaining popularity in the treatment of unilateral vocal fold immobility, little has been reported on commonly encountered surgical challenges with this procedure. Here, we present a cohort of 21 pediatric patients who underwent this procedure with a full description of techniques used to overcome common challenges with this procedure. We report vocal and swallowing outcomes for these patients, with an overall success rate of 19/21 (90.5%) patients, with success defined as improvement of a half-consistency or resolution of vocal issues.

Rhinologic manifestations of Burkitt Lymphoma in a pediatric population: Case series and systematic review.

INTRODUCTION: Burkitt lymphoma (BL), an aggressive form of B-cell non-Hodgkin's lymphoma, arising from the nose and paranasal sinuses is relatively rare. It can present with various symptoms leading to potential misdiagnosis and delayed treatment. BL is fatal if left untreated, while early identification and treatment can improve prognosis. OBJECTIVES: 1) To review clinical presentations and sites of involvement of six cases of pediatric BL with rhinologic manifestations and compare these with the current literature. 2) To raise awareness on the variety of presentations of BL in this particular anatomic location. METHODS: A series of six cases of pediatric (0-18 years) BL with rhinologic manifestations is presented. Age, sex, ethnicity, symptoms, imaging, staging, treatment and outcome were recorded. A systematic review of literature was also conducted using PRISMA guidelines. The search strategy used keywords related to rhinologic manifestations of BL (nasal cavity, nasopharynx, paranasal sinus etc.; Burkitt etc.) and included studies published in English and French describing patients 0-18 years of age. RESULTS: 42 patients were included (six from case series and 36 from current literature). Most common presenting symptoms were: nasal obstruction (29%), facial swelling (24%), headache (21%) and proptosis (19%). Most frequent sites of presentation were: nasopharynx (40%), maxilla (40%) and sphenoid (33%). More than half (60%) had systemic involvement, of which the most common locations were: kidney (19%), pancreas (17%) and liver (17%). Mortality from BL in children from this study population was correlated with a longer duration of symptoms prior to presentation, as well as a misdiagnosis preceding the final diagnosis of BL. CONCLUSIONS: This study brings understanding to the numerous presentations of the same aggressive disease, promotes high clinical suspicion when evaluating common otolaryngologic symptoms and can guide healthcare providers in diagnosing pediatric BL with rhinologic manifestations.

Current trends in practices in the treatment of pediatric unilateral vocal fold immobility: A survey on injections, thyroplasty and nerve reinnervation.

INTRODUCTION: The objective of this study was to poll pediatric otolaryngologists with a special interest in pediatric laryngology on their experience with injection medialization laryngoplasty (IML), thyroplasty, and reinnervation procedures in order to reflect on changing clinical practices. METHOD: A 10-items questionnaire was designed to inquire about current management practices in the treatment of symptomatic UVFI. The 59 members of an ASPO pediatric laryngology working group received the anonymous online survey. A 47% (28/59) response rate was obtained over a 2 weeks period. RESULTS: Carboxymethylcellulose gel (Prolaryn) is the most popular injectable material used in the setting of IMLs (preferred by 46%; 13 respondents). Most clinicians perform IMLs in the operating room. However, 14% (4/28) of respondents currently perform office-based injections on older patients. When faced with the case of a young child with longstanding iatrogenic VFP, most respondents (41%; 11/27) would perform an IML alone as a first step for management. 37% (10/27) of respondents would inject and perform a laryngeal EMG while 22% (6/27) would offer reinnervation and concomitant IML. When faced with the case of a teenager with long-standing VFP, the polled physicians' opinions were divided: 37% (10/27) favored reinnervation and concomitant IML, while 26% (7/27) would proceed to an IML only. The remaining 37% (10/27) would first assess for vocal atrophy through LEMG or visual inspection. CONCLUSIONS: Two significant shifts in practice seem to have occurred. First, ansa-RLN reinnervation procedures are now being considered as a first line treatment for a subset of patients in whom only IML was considered in the past. In fact, more than 20% of respondents did consider this route of management. Second, there is an increased use of in-office augmentations for older pediatric patients.

A retrospective cohort study: do patients with graves' disease need to be euthyroid prior to surgery?

BACKGROUND: The 2016 American Thyroid Association guidelines indicate that patients with Graves' disease who undergo a thyroidectomy should be rendered euthyroid through the use of antithyroid drugs (ATD) prior to surgery to avoid complications such as a thyroid storm. At times, the use of ATDs can have limited efficacy and therefore some patients will inevitably remain biochemically hyperthyroid at the time of surgery. The aim of this study is to assess if hyperthyroid patients undergoing a thyroidectomy are at an increased risk of developing a thyroid storm in comparison to euthyroid patients. Furthermore, this study seeks to establish a correlation between thyroid storm identified by the levels of thyroid hormones (T3 and T4) and the level of thyroid stimulating hormone (TSH). METHODS: A retrospective cohort study was conducted at two Canadian centers, one in Montreal and the other in Nova Scotia. Sixty-seven patients undergoing thyroidectomy for Graves' disease from January 2006 to December 2016 were evaluated. RESULTS: The study comprised 67 participants with a mean age of 46 years (range16-78 years). A total of 78% of patients were on methimazole, 34% on beta-blockers, 27% on potassium iodine solution, 10% on propylthiouracil and 7% on steroids. At the time of surgery 21% were in an overt hyperthyroid state and 33% were in a subclinical hyperthyroid state. The average TSH level of 0.03 mIUL/L (range 0.01-0.23 mIUL/L). Sixteen percent of patients had a TSH level less than 0.01 mIUL/L. The average free T4 level was 29.58 pmol/L (range 11.5-95.2 pmol/L). The average total T3 level was 11.52 nmol/L (range 4.5-29.1 nmol/L) and free T3 level was 6.35 pmol/L (range 6.1-6.6 pmol/L). No patient developed thyroid storm. CONCLUSIONS: In our study, biochemically hyperthyroid patients undergoing thyroidectomy did not develop thyroid storm. Additional studies with larger sample sizes are needed to better understand the risk of thyroid storm in hyperthyroid patients.

The first Canadian experience with the Afirma® gene expression classifier test.

BACKGROUND: Thyroid nodules are common and often benign, although prove to be malignant upon surgical pathology in 5-15% of cases. When assessed with ultrasound-guided fine-needle aspiration (USFNA), 15-30% of the nodules yield an indeterminate result. The Afirma® gene expression classifier (AGEC) was developed to improve management of indeterminate thyroid nodules (ITNs) by classifying them as "benign" or "suspicious." Objectives were (1) to assess the performance of the AGEC in two Canadian academic medical centres (2), to search for inter-institutional variation and (3) to compare AGEC performance in Canadian versus American institutions. METHODS: We undertook a retrospective cohort study of patients with indeterminate cytopathology (Bethesda Class III or IV) as per USFNA who underwent AGEC testing. We reviewed patient demographics, cytopathological results, AGEC data and, if the patient underwent surgery, results from their final pathology. RESULTS: In total, we included 172 patients with Bethesda Class III or IV thyroid nodules underwent AGEC testing, 109 in Montreal, Quebec and 63 in St. John's, Newfoundland, in this study. Among the nodules sent for testing, 55% (60/109) in Montreal and 46% (29/63) in St. John's returned as "benign." None of these patients underwent surgery. On the other hand, 45% (49/109) nodules in Montreal and 54% (34/63) in St. John's were found to be "suspicious," for a total of 83 specimens. Seventy seven of these patients underwent surgery. Both in Montreal and St. John's, the final pathology yielded malignant thyroid disease in approximately 50% of the specimens categorized as "suspicious." Since 2013, no patient diagnosed with a benign nodule as per AGEC testing was found to harbor a malignant thyroid nodule on follow-up. CONCLUSIONS: Molecular analysis is increasingly used in the management of indeterminate thyroid nodules. This study highlights the experience of two Canadian centres with AGEC testing. We found inter-institutional variability in the rate of nodules returning as "benign," however we found similar rates of confirmed malignancy in nodules returning as "suspicious." According the literature, results for AGEC testing in two Canadian institutions align with results reported in American centres.

Error Detection-Based Model to Assess Educational Outcomes in Crisis Resource Management Training: A Pilot Study.

Otolaryngology-head and neck surgery (OTL-HNS) residents face a variety of difficult, high-stress situations, which may occur early in their training. Since these events occur infrequently, simulation-based learning has become an important part of residents' training and is already well established in fields such as anesthesia and emergency medicine. In the domain of OTL-HNS, it is gradually gaining in popularity. Crisis Resource Management (CRM), a program adapted from the aviation industry, aims to improve outcomes of crisis situations by attempting to mitigate human errors. Some examples of CRM principles include cultivating situational awareness; promoting proper use of available resources; and improving rapid decision making, particularly in high-acuity, low-frequency clinical situations. Our pilot project sought to integrate CRM principles into an airway simulation course for OTL-HNS residents, but most important, it evaluated whether learning objectives were met, through use of a novel error identification model.

Medical Management for the Treatment of Nontuberculous Mycobacteria Infection of the Parotid Gland: Avoiding Surgery May Be Possible.

Infection with nontuberculous mycobacteria (NTM) is uncommon in the head and neck; therefore there is no clear consensus on treating these infections. Our objective was to report our experience with a unique case of NTM infection of the parotid in an immunocompetent patient, in order to determine appropriate management through our experience with this pathology. A 57-year-old man, known for numerous comorbid diseases, presented to our institution complaining of right parotid swelling and pain. A computed tomography (CT) of the neck showed a multiloculated collection in the inferior portion of the right parotid gland, compatible with abscess formation. This abscess was drained by interventional radiology (IR) but required repeat drainage twice due to lack of initial improvement. He was treated with several antibiotics as culture results initially indicated Gram-positive bacilli and then Mycobacterium species, with final identification by a reference laboratory as Mycobacterium abscessus. Imipenem was initiated with amikacin and clarithromycin. His infection clinically and radiologically resolved after 5 months of antibiotherapy. In our case, the patient improved following intravenous antibiotic therapy. Our experience demonstrates that appropriate antibiotherapy can lead to resolution of Mycobacterium abscessus infection in the parotid without the risks associated with surgical intervention.

The McGill Thyroid Nodule Score's (MTNS+) role in the investigation of thyroid nodules with benign ultrasound guided fine needle aspiration biopsies: a retrospective review.

BACKGROUND: Ultrasound guided fine needle aspiration (USFNA) biopsies of thyroid nodules sometimes create a decision-making dilemma for surgeons as they may yield falsely benign results. The McGill Thyroid Nodule Score + (MTNS+) was developed to aid in clinical guidance regarding the management of patients with these USFNA results. The aim of this study was to assess the MTNS+ as a clinical tool in patients with benign preoperative thyroid nodule USFNAs and to analyze the relationship between nodule size and malignancy in these patients. METHODS: We conducted a retrospective chart review of 1312 patients who underwent thyroidectomies between 2010 and 2015 at the McGill University Teaching Hospitals. Patients with Bethesda II (benign) USFNA results, calculated MTNS+, and nodule size evaluated on ultrasound were included in the study. The false-negative rate was calculated, and MTNS+ and nodule size were each compared to final pathology results. Binary logistic regression was used for statistical analysis. RESULTS: Of the 1312 patients, 101 met the inclusion criteria and together had an average MTNS+ score of 6.83, which corresponds to a predicted malignancy rate between 25 and 33 %. Final pathology revealed malignancy in 16 (15.8 %) subjects. The average MTNS+ of patients with malignant nodules on surgical pathology was 8.25, while that of patients with benign nodules was 6.56. Patients with nodule size 1-1.9 cm (a) and 2-2.9 cm (b) each had an equal rate of malignancy of 2.97 % (n = 3), nodule size 3-3.9 cm (c) had a rate of 1.98 % (n = 2), and nodule size ≥4 cm (d) a rate of 7.92 % (n = 8). CONCLUSION: The rate of malignancy (15.8 %) is higher than expected when reviewing the risk of malignancy in nodules considered as Bethesda class 2. On the other hand, the rate is lower than the 25-33 % predicted by the MTNS+. We also found a higher malignancy rate for nodules above 4 cm in size, but size was a poor predictor of malignancy when used alone. Therefore, while the MTNS+ may be helpful at helping to identify USFNAs that are incorrectly classified as benign, the percentage risk of malignancy is lower than expected.

One-stage vs. two-stage BAHA implantation in a pediatric population.

OBJECTIVES: BAHA implantation surgery in a pediatric population is usually done in two-stage surgeries. This study aims to evaluate the safety and possible superiority of the one-stage over the two-stage BAHA implantation and which one would be the best standard of care for our pediatric patients. METHODS: A retrospective chart review of 55 patients operated in our tertiary care institutions between 2005 and 2010 was conducted. The actual tendency in our institutions, applied at the time of the study, is to perform a one-stage surgery for all operated patients (pediatric and adult), except for patients undergoing translabyrinthine surgeries for cerebellopontine tumor excision. These patients indeed had a two-stage insertion. 26 patients underwent one-stage surgery (group I) while 29 patients had a two-stage (group II) BAHA insertion. A period of 4 months was allowed for osseointegration before BAHA processor fitting. As for the safety assessment of the one-stage surgery, we compared both groups regarding the incidence and severity (minor, moderate and major) of encountered complications, as well as the operating time and follow-up. The operating time of the two-stage surgery includes the time of the first and of the second stage. RESULTS: The mean age at surgery was 8.5 years old for the group I and 50 years old for the group II patients. There was no difference in the incidence of minor (p=0.12), moderate (p=0.41) nor severe (p=0.68) complications between groups I and II. Two cases of traumatic extrusion were noted in the group I. Furthermore, the one-stage BAHA implantation requests a significantly lower operating time (mean: 54 [32-100] min) than the two-stage surgery (mean: 79 [63-148] min) (p=0.012). All pediatric cases of BAHA insertion were performed in a one day surgery. The mean postoperative follow-up was 114 and 96 weeks for groups I and II respectively (p=0.058). CONCLUSIONS: One-stage BAHA insertion surgery in the pediatric population is a reliable, safe and efficient therapeutic option that allows a good result in a significantly lower operating time compared to the two-stage insertion and is achieved in a one day surgery. It could therefore be considered as a standard of care for pediatric patients.

Are solitary thyroid nodules more likely to be malignant?

BACKGROUND: Traditional teaching demonstrated that solitary thyroid nodules were more likely to be malignant. Newer studies show that there is no clear answer regarding the influence of the number or the distribution of nodules on the risk of malignancy. OBJECTIVES: The purpose of this study was to establish whether patients undergoing thyroid surgery and presenting with a solitary thyroid nodule show a greater rate of malignancy when compared to those presenting with multiple nodules. The secondary goal was to evaluate the impact of the distribution of the nodules (multiple unilateral nodules versus bilateral nodules) on the rate of malignancy in this population. METHOD: Retrospective review of the medical records of the 656 patients who underwent thyroidectomy at one of the hospitals of the McGill University Thyroid Cancer Centre between July 2006 and April 2011 was conducted. RESULTS: There was no significant difference in the malignancy rate between patients with a solitary nodule and patients with two to six thyroid nodules at ultrasonography, between patients with unilateral nodule(s) and patients with bilateral nodules, or between patients with at least one nodule > 1.0 cm and patients without any nodules > 1.0 cm (p  =  .870, .578, and .361, respectively). CONCLUSION: This study demonstrates that the likelihood of thyroid cancer is independent of the number of thyroid nodules. Moreover, our data show that the malignancy rate is not influenced by the distribution of the nodules or their size.

Congenital aural atresia: bone-anchored hearing aid vs. external auditory canal reconstruction.

OBJECTIVE: To compare the audiologic outcome and feasibility of bone-anchored hearing aid (BAHA) and external auditory canal reconstruction (EACR) surgeries in pediatric patients presenting a congenital aural atresia (CAA). METHODS: A retrospective chart review of 40 patients operated in our tertiary pediatric care center between 2002 and 2010 was performed. 20 patients underwent EACR, whereas another 20 patients were implanted with a BAHA device. Air conduction (AC), bone conduction (BC), pure tone average (PTA) and speech discrimination score (SDS) were compared preoperatively, and hearing gain (HG) postoperatively at 6 and at 12 months at frequencies of 500, 1000, 2000 and 4000 Hz. Operative time, complications and associated microtia were documented as well. EACR patients were graded retrospectively upon Jahrsdoerfer's classification. RESULTS: Preoperative AC were significantly different between groups, at 500, 1000 and 2000 Hz but not at 4000 Hz. BAHA group compared postoperatively to EACR group showed significantly a superior HG of 46.9 ± 7.0 dB (p<0.001) and of 39.8(7) ± 7.2(6.9)dB (p<0.001) at 6 months and at 1 year, respectively. Moreover, aided air thresholds from the EACR group revealed an audiologic status similar to those of the BAHA group patients, at 6 months and one year postoperatively. Both groups had a similar evolution of their BC, as well as of the incidence of complications. We report one case of transient facial paralysis in the EACR group. Total operative time is significantly lower (p<0.001) for a BAHA implantation (56 ± 21 min) than for EACR surgery (216 ± 174 min). No preoperative or postoperative correlation (Pearson correlation test; p>0.05) was found between patient's Jahrsdoerfer's score and their audiologic outcome. HG does not seem to be influenced by the presence of microtia. CONCLUSION: EACR, although constituting an attractive option, does not give acceptable results alone. It can however, when combined to conventional air conduction hearing aids, provide excellent audiologic outcomes comparable to BAHA. BAHA implantation is a reliable, safe and efficient therapeutic option that allows a significantly better audiologic outcome when compared to unaided EACR for patients with CAA.

Solitary fibrous tumor arising in the mesentery: a case report.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm usually found in the pleura, soft tissues and visceral organs. We describe one case arising in the mesentery, which is an exceptional localization. CASE PRESENTATION: A 71-year-old man was referred to our establishment for a painless hypogastric mass. Further investigation revealed a vascular tumor, which was resected en bloc. Pathological findings confirmed solitary fibrous tumor of the mesentery. CONCLUSION: This is the second case of solitary fibrous tumor of the small intestine mesentery ever reported. It was managed by en bloc resection and close follow up considering the high risk of recurrence. Investigation should be made regarding the use of adjuvant systemic therapy to improve long-term survival for these patients.