Granular cell tumor mimicking a subglottic hemangiomaMichele Torre a,⁎, Francisca Yankovic b, Oscar Herrera c, Cecilia Borel d,Juan José Latorre e, Pedro Aguilar f, Patricio Varela g
aPediatric Surgery Department, Istituto G. Gaslini, 16148 Genoa, ItalybPediatric Surgery Department, Calvo Mackenna Hospital, 8320000 Santiago, ChilecBronchopulmonary Unit, Calvo Mackenna Hospital, 8320000 Santiago, ChiledENT Unit, Calvo Mackenna Hospital, 8320000 Santiago, ChileePathology Unit, Calvo Mackenna Hospital, 8320000 Santiago, ChilefPediatric Department, Hospital del Pino, 8320000 Santiago, ChilegPediatric Surgery Department, Calvo Mackenna Hospital and Clinica Las Condes, 8320000 Santiago, Chile
Received 27 June 2010; revised 28 July 2010; accepted 28 July 2010
Laryngotracheal tumors are uncommon in the pediatricage group. Subglottic hemangioma is the most frequentlyoccurring lesion. Granular cell tumor (GCT) of the upperairway is very rare in children, as only a few cases have beenreported in the literature [1-6]. In a small percentage of cases,the tumor is multifocal [1,7]. Predominance in black femalesof school age is reported [2,5]. We present a case ofsubglottic GCT mimicking a hemangioma. Preoperativeworkup allowed us to make the correct diagnosis and tochoose open surgical resection. We underline the importanceof considering GCT in the differential diagnosis of an airwayobstruction, to address the correct treatment.
⁎ Corresponding author. Pediatric Surgery, Largo G. Gaslini, 16148enova, Italy. Tel.: +39 0105636392.E-mail address: [email protected] (M. Torre).
A 4-year-old white girl with inspiratory stridor since thefirst year of life was referred to our Airway Unit. She had ahistory of numerous admissions to the emergency depart-ment for acute respiratory episodes interpreted as recurrentlaryngitis and treated medically by adrenalin, bronchodila-tors, and steroids, with only temporary and partial success.The patient underwent endoscopic airway evaluation using a0° 4-mm Hopkins rod telescope. Under spontaneousventilation, the vocal cord movements appeared normal,but a tumor was observed arising from the left posterolateralsubglottic wall causing 80% lumen obstruction (Fig. 1). Themass appeared covered by normal mucosa and extendedcaudally up to the first tracheal ring. The distal trachea andbronchi were normal. Computerized tomography (CT) scan
Fig. 3 The tumor has been exposed and dissected from cricoid.
E10 M. Torre et al.
revealed a mass partially occluding the airway withoutinvasion of paralaryngeal tissues or lymphadenopathy(Fig. 2). The mass appeared solid and did not take up muchcontrast enhancement. Open resection by cervical anteriorapproach was performed. The airway was opened from thelower third of thyroid cartilage to the first 5 tracheal rings,widely exposing the tumor. Amucosal plane was opened, anda submucosal flap was lifted. The tumor was attachedposteriorly to the cricoid cartilage and was completelyresected (Figs. 3 and 4). Histopathologic examination of themass revealed the presence of polyhedric cells, withintracytoplasmic granules (Fig. 5). Immunohistochemicalstudy was positive for S-100, vimentin, periodic acid–Schiff,and neuronal specific enolase (NSE). These features wereconsistent with a diagnosis of GCT.
The patient was extubated 48 hours after the procedure,and she was discharged on the fifth postoperative day ingood health. Endoscopic evaluation performed at day 10 and
Fig. 2 Computed tomography scan of the neck showing the mass.
1, 6, and 12 months after tumor resection revealed normalfindings without other foci of disease. At 3 years and 6months of follow-up, the patient is well and free fromrespiratory troubles.
2. Discussion
Granular cell tumor is an uncommon tumor, and only afew reports in pediatric age group have been published [1-7].Female prevalence, as for subglottic hemangioma, has beenobserved [2,5]. Although the pathogenesis of GCT wasdebated in the past, its neural origin from Schwann cells is
Fig. 5 Polyhedric cells with intracytoplasmic granules.
E11Granular cell tumor mimicking a subglottic hemangioma
now widely accepted [4]. In children, the most frequentpresentation of GCT is congenital epulis, arising from themedian ridge of the newborn maxilla. Another presentationof GCT is the noninfantile GCT, more often noted during thethird to sixth decade of life, with half of them involving thehead and neck. This can occur during childhood, but it isextremely uncommon. In those cases, the tongue is the mostfrequent site of origin [4]. Only a few cases of airwayinvolvement, from supraglottic region to the bronchi, arereported in the pediatric age group [1-8]. The diagnosis ofGCT of the upper airway in children is often delayed [8]. Ourpatient was diagnosed at 4 years of age, but her symptomsstarted when she was 1 year old. Clinical presentation ofairway GCT includes stridor, cough, dyspnea, hoarse voice,asthma-like symptoms, and laryngitis. Unlike adults, GCThas never been malignant in children. Most of these lesionsare unifocal, but in around 10% of cases, they can present asmultifocal lesions. Although this is extremely rare inchildhood [1,7], long-term follow-up is recommended [4].
Apart from the rarity of laryngotracheal GCT in this agegroup, the interest of reporting our case derives from theunique clinical presentation of the tumor in this child, whichmimicked a subglottic hemangioma. The latter is much morefrequent than GCT and requires a different therapeuticapproach. In fact, symptoms were observed during the firstyear of life, even though the patient was referred to us at 4years of age. Early onset and the ethnic origin of the girl (notblack African) are not typical for GCT and could have beenmisleading. At endoscopic evaluation, the appearance of around mass in the typical location of hemangioma, that is,left posterolateral subglottis, might have reinforced thewrong diagnosis. However, in our patient, the suspicion ofGCT rose during endoscopic evaluation because the tumor
appeared to be a solid mass. The diagnosis of GCT wasconfirmed only at histopathologic examination of theresected specimen. However, the preoperative CT scan hadgiven us a clue by showing a solid noncontrasted lesion(Fig. 2). Hence, we underline the importance of the CT scanin the preoperative workup of a laryngeal mass. Otherauthors [4] advocate the use of laryngeal echography toavoid irradiation, but this is a very difficult examination in achild and many centers have no experience with this. Someauthors reported endoscopic resection of tracheal GCT [7],but we have preferred the open surgical approach because ofthe size of the tumor and its sessile aspect. Preserving thenormal mucosa above the tumor allowed for rapid epithe-lialization. This choice from an oncologic point of view wassupported by the fact that the mucosa is usually not involvedby the tumor [4].
Regarding histopathologic examination, if a GCT issuspected, the diagnosis is usually not difficult. In ourpatient, the mass was composed of round polygonal cellswith eosinophilic and granular cytoplasm. The characteristicstrong positivity for S-100 protein confirmed the diagnosis.
Follow-up of these tumors, as suggested by other authors,must be prolonged, because relapses have also beendescribed in the pediatric age group. However, it can be acontroversial matter if these children must undergo repeatedendoscopic evaluations even if asymptomatic. In our case,after the first postoperative year, we preferred a clinicalfollow-up.
This experience suggests that GCT must be considered inthe differential diagnosis of pediatric airway tumors.
References
[1] Burton DM, Heffner DK, Patow CA. Granular cell tumors of thetrachea. Laryngoscope 1992;102:807-13.
[2] Holland RS, Abaza N, Balsara G, et al. Granular cell tumor in a six-year-old child: case report and review of the literature. Ear Nose Throat J1998;77:652-4.
[3] Amar YG, Nguyen LH, Manoukian JJ, et al. Granular cell tumor of thetrachea in a child. Int J Pediatr Otorhinolaryngol 2002;62:75-80.
[4] Leboulanger N, Rouillon I, Papon JF, et al. Childhood granular celltumors: two case reports. Int J Pediatr Othorhinolaryngol 2008;72:279-83.
[5] Royal SA. Pediatric laryngeal granular cell tumor. Pediatr Radiol2000;30:869-70.
[6] Desai DP, Maddalozzo J, Holinger LD. Granular cell tumor of thetrachea. Otolaryngol Head Neck Surg 1999;120:595-8.
[7] Pernas FG, Younis RT, Lehman DA, et al. Management of pediatricairway granular cell tumor: role of laryngotracheal reconstruction. IntPediatr Otorhinolaryngol 2006;70:957-63.
[8] Vaos G, Zavras N, Priftis K, et al. Bronchial granular cell tumor in achild: impact of diagnostic delay on the type of surgical resection. JPediatr Surg 2006;41:1326-8.
