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CASE REPORT
Year : 2018  |  Volume : 36  |  Issue : 3  |  Page : 319-323
 

Congenital granular cell tumor of the newborn – Spontaneous regression or early surgical intervention


Oral Health Sciences Centre, Unit of Pedodontics and Preventive Dentistry, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication24-Sep-2018

Correspondence Address:
Manojkumar Jaiswal
Unit of Pedodontics and Preventive Dentistry, Oral Health Sciences Centre, Postgraduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JISPPD.JISPPD_1187_17

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   Abstract 


Congenital granular cell tumor of the newborn is a benign, relatively innocuous hamartomatous lesion arising from the alveolar ridge. Large lesions often warrant prompt surgical removal owing to the associated difficulty in breastfeeding and respiration of the infant. However, for medium-sized lesions, another approach could be observed for spontaneous regression. The present case report highlights the use of a combination approach for management of tumor in a 2-day-old female child presenting with a large protruding mass from the mouth associated with difficulty in breastfeeding. A combination of observation for regression in size followed by electrosurgical removal of the tumor was done. The infant was followed up regularly over a long period of 3 years to assess any adverse effects of surgical intervention on the maxillary primary anterior dentition of which none were recorded.


Keywords: Congenital granular cell tumor, electrocautery, epulis


How to cite this article:
Dhareula A, Jaiswal M, Goyal A, Gauba K. Congenital granular cell tumor of the newborn – Spontaneous regression or early surgical intervention. J Indian Soc Pedod Prev Dent 2018;36:319-23

How to cite this URL:
Dhareula A, Jaiswal M, Goyal A, Gauba K. Congenital granular cell tumor of the newborn – Spontaneous regression or early surgical intervention. J Indian Soc Pedod Prev Dent [serial online] 2018 [cited 2019 Nov 14];36:319-23. Available from: http://www.jisppd.com/text.asp?2018/36/3/319/241959





   Introduction Top


Congenital granular cell tumor (CGCT) of the newborn is a rare benign hamartomatous soft-tissue tumor which was first described by Newmann in 1871.[1] The tumor often arises from the maxillary alveolar ridge (3:1), corresponding to the future location of the canine and lateral incisor, though other intraoral sites such as the mandible and tongue have also been reported.[2] Clinically, the tumor presents as a smooth, sessile or pedunculated, solitary mass varying in size from a few millimeters to as large as 9 cm, usually with normal overlying mucosa. Females are mostly affected (8–10 females: 1 male) with a predisposition for the Caucasian race.[3] Multiple lesions are sporadic and rare. Exact histogenesis of the tumor is still unclear with various proposed hypothesis including a neurogenic, fibroblastic, myoblastic, odontogenic, histiocytic, and a possible endocrinologic origin based on the types of cells found in the histological sections.[4] Histologically, the tumor resembles adult granular cell tumor comprising sheets of large, closely packed cells showing fine, granular, eosinophilic cytoplasm except the absence of pseudoepitheliomatous hyperplasia.[5] Depending on the size, the tumor may pose difficulties in feeding or may obstruct the airway of the infant leading to respiratory distress.

Management options vary from observation for spontaneous regression of the smaller lesions to immediate surgical excision of large tumors without wide dissection, with negligible reoccurrence rates. With advances in neonatal surgical practices and use of electrocautery, the excision has further become quick and hassle-free, with minimal blood loss, limited potential scarring, and rapid healing rates.

The present case report highlights the use of a combination approach, namely, observation for spontaneous regression for 2 months followed by surgical excision of the tumor using a bipolar electrocautery with a long follow-up period of 3 years. The combination approach allowed the tumor to reduce in size while simultaneously allowing the infants' physiological functions to mature adequately, especially pertaining to the immature liver functions.


   Case Report Top


A 2-day-old postnatal female was referred to the clinic of pediatric dentistry for examination of a large solitary mass protruding the mouth. The infant was born at 38.5 weeks of gestation through vaginal delivery following an uneventful pregnancy and weighed 4120 g. The infant was a result of nonconsanguineous marriage with no other associated congenital anomaly or malformations. Clinical examination revealed the presence of two fleshy smooth-surfaced sessile masses – the larger one being attached to the left anterior maxillary alveolar ridge encompassing both the labial and palatal mucosa and a smaller one corresponding to the maxillary labial frenum almost crossing the midline, the combined widths of the lesions being approximately 3.5 cm × 2 cm × 4 cm [Figure 1]a and [Figure 1]b. Both masses were nontender, nonfluctuant, firm in consistency; smooth with normal overlying mucosa and no associated lymphadenopathy. Based on the location and clinical presentation of the growth, a provisional diagnosis of CGCT was made. Due to the prominence and size of the masses, the infant was unable to maintain adequate lip seal, leading to difficulty in feeding. Consultation from pediatric plastic surgery was sought, and it was decided to defer the surgical excision of the lesion, allowing for spontaneous regression. Meanwhile, infant's mother was trained in feeding the child using a spoon maintaining an upright position, and the child was reviewed regularly on an outpatient basis for regression of the lesion.
Figure 1: (a and b) Preoperative extraoral and intraoral view

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At 2 months of follow-up, the tumor showed little signs of regression in size, with continued difficulty in feeding and significant parental apprehension. The pros and cons of surgical excision were explained in detail to the parents and a combined decision was made to surgically excise the mass for which the written consent was obtained from the parents. Excision was carried out under general anesthesia with endotracheal intubation using a bipolar electrocautery [Figure 2]a and [Figure 2]b. Excised mass was sent for histopathological examination [Figure 3]b. Breastfeeding was initiated on the same day with both the infant and the mother being comfortable. At 1-week follow-up, a defect could be appreciated on the alveolous corresponding to the lateral incisor region with completely healed mucosa [Figure 4]a and [Figure 4]b. The child was followed up every 6 months with no reoccurrence of the tumor and at 3 years of follow-up showed uneventful eruption of all primary teeth with no evidence of any malformation, aplasia, or hypoplasia [Figure 3]a.
Figure 2: (a) Intraoperative view of the lesion. (b) Immediate postcauterization of the lesion

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Figure 3: (a) Eighteen months follow-up with complete eruption of the primary dentition. (b) H and E staining of the section revealing sheets of cells containing eosinophilic granular cell cytoplasm suggestive of congenital granular cell tumor of the newborn

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Figure 4: (a) One-week postcauterization. Notice the alveolar defect corresponding to the location of the lesion. (b) Eight months follow-up showing uneventful eruption of the primary dentition

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Histopathology confirmed the diagnosis of CGCT with sheets of cells containing eosinophilic granular cell cytoplasm [Figure 3]b. Furthermore, immune histochemical typing showed positivity for S-100, confirming the diagnosis of CGCT of the newborn.


   Discussion Top


CGGT, also known as congenital granular cell myoblastoma, Abrikossoff's tumor, congenital epulis or Newmann's tumor, is a lesion, exclusively seen in the newborns, arising at the future site of the maxillary canine or the lateral incisors.[2] The lesion however histologically differs from other granular cell lesions by the presence of scattered odontogenic epithelium, more elaborate and widespread vasculature along with the absence of interstitial cells.[5] Differential diagnosis includes congenital malformations including encephalocele and dermoid cysts/teratomas and neoplasms including hemangioma, lymphatic malformations, melanotic neuroectodermal tumors of infancy, and rhabdomyosarcoma.[6] Antenatal ultrasound imaging may help in prenatal diagnosis of large tumors that may be associated with polyhydramnios.[3] Postnatal magnetic resonance imaging and computed tomography scans may further serve as an adjunct to determine the extent and involvement of adjacent structures, especially when intracranial extension is suspected in case of large tumors.[7]

Management option for small lesions may include observation for spontaneous regression [Table 1] or immediate surgical excision. Despite the innocuous nature of the tumor, surgical excision under local or general anesthesia is immediately warranted when the tumor is large and interferes with feeding and airway, the treatment being definitive and curative. In the present case, a combined approach was used. Initially, the lesion was followed for spontaneous regression. This allowed for decrease in size of the tumor facilitating surgical excision with much less morbidity. Furthermore, this also allowed the liver function of the infant to mature completely negating the administration of parenteral Vitamin K and clotting factors which often is a prerequisite before contemplating any surgical procedure on an infant with an immature liver function. However, complete regression of the lesion is dependent on the initial size of tumor and may take several months.
Table 1: Reports of cases of congenital epulis managed conservatively

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In the present case, bipolar electrocautery was chosen over scalpel for excision of the mass to minimize the blood loss and negate the need of suture placement. Most of the authors that have reported cases of surgical excision of CGCT have used scalpel under GA followed by placement of sutures at the site, leading to an additional visit for removable of suture and delay in healing time. A search of English literature revealed the use of electrocoagulation for excision of CGCT by eight authors only [Table 2]. The child was followed up over a long period of 3 years till the complete primary dentition had erupted to monitor for any potential ill-effects of the surgical intervention including hypoplasia and aplasia, of which none occurred.
Table 2: Reports of cases of congenital epulis managed conservatively and using electrocautery

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   Conclusion Top


Based on the usual location of CGCT and the associated anxiety of the parents, pediatric dentists may be frequently consulted and thus should be aware of the differential diagnosis, potential for airway distress and feeding difficulties associated with large tumors. Combination approach may be suggested for lesions where the advantage of both spontaneous regression and surgical excision are offered. [23]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given their consent for images and other clinical information to be reported in the journal. The parents understand that name and initials of the patient will not be published and due efforts will be made to conceal patient's identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Neumann E. An instance of congenital Epulis. Arch Heilkd 1871;12:189-90.  Back to cited text no. 1
    
2.
Loyola AM, Gatti AF, Pinto DS Jr., Mesquita RA. Alveolar and extra-alveolar granular cell lesions of the newborn: Report of case and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:668-71.  Back to cited text no. 2
    
3.
Canavan-Holliday KS, Lawson RA. Anaesthetic management of the newborn with multiple congenital epulides. Br J Anaesth 2004;93:742-4.  Back to cited text no. 3
    
4.
Nagpal R, Suryawanshi P, Malshi N, Godse A, Kalrao V. Congenital epulis: Case report and literature review. Indian J Neonatal Med Res 2013;1:18-20.  Back to cited text no. 4
    
5.
Neville BW, Douglas DD, Allen CM, Bouquot J. Oral and Maxillofacial Pathology. 3rd ed. St. Louis: Saunders/Elsevier Health Sciences; 2009.  Back to cited text no. 5
    
6.
Welbury RR. Congenital epulis of the newborn. Br J Oral Surg 1980;18:238-43.  Back to cited text no. 6
    
7.
Lenes-Voit F, Sheyn A, Weinberger D. Congenital granular cell tumor in a child with tetralogy of fallot. Ear Nose Throat J 2016;95:264-6.  Back to cited text no. 7
    
8.
de Siqueira AS, das Pinheiro MG, Monteiro AC, de Carvalho MR, Pinherio LR, de Pinherio JJ. Congenital epulis with auto-resolution: Case report. Rev Gaúcha Odontol Porto Alegre 2014;62:315-8.  Back to cited text no. 8
    
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Bhatia SK, Goyal A, Ritwik P, Rai S. Spontaneous regression of a congenital epulis in a newborn. J Clin Pediatr Dent 2013;37:297-9.  Back to cited text no. 9
    
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Ritwik P, Brannon RB, Musselman RJ. Spontaneous regression of congenital epulis: A case report and review of the literature. J Med Case Rep 2010;4:331.  Back to cited text no. 10
    
11.
Mabongo M, Wood NH, Lemmer J, Feller L. Congenital epulis: A case report. SADJ 2008;63:350-1.  Back to cited text no. 11
    
12.
Ruschel HC, Beilke LP, Beilke RP, Kramer PF. Congential epulis of newborn: Report of a spontaneous regression case. J Clin Pediatr Dent 2008;33:167-9.  Back to cited text no. 12
    
13.
Sakai VT, Oliveira TM, Silva TC, Moretti AB, Santos CF, Machado MA, et al. Complete spontaneous regression of congenital epulis in a baby by 8 months of age. Int J Paediatr Dent 2007;17:309-12.  Back to cited text no. 13
    
14.
Marakoglu I, Gursoy UK, Marakoglu K. Congenital epulis: Report of a case. ASDC J Dent Child 2002;69:191-2, 125.  Back to cited text no. 14
    
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Jenkins HR, Hill CM. Spontaneous regression of congenital epulis of the newborn. Arch Dis Child 1989;64:145-7.  Back to cited text no. 15
    
16.
O'Brian FV, Pielou WD. Congenital epulis: Its natural history. Arch Dis Child 1971;46:559-60.  Back to cited text no. 16
    
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Bewley A, Bloom JD, Kherani S, Pawel BR. Congenital epulis. Ear Nose Throat J 2010;89:299-300.  Back to cited text no. 17
    
18.
Sürmeli-Onay Ö, Api A. A premature newborn with intraoral tumor. Turk Pediatri Ars 2014;49:353-5.  Back to cited text no. 18
    
19.
Gupta D, Panda SS, Yadav DK, Kumar B, Bagga D, Acharya SK. Congenital epulis: A rare lesion. J Clin Neonatol 2014;3:220-2.  Back to cited text no. 19
  [Full text]  
20.
Hiradfar M, Zabolinejad N, Gharavi M, Sebt S. Multiple congenital epulis of the mandibular ridge: A case report. Iran J Otorhinolaryngol 2012;24:193-6.  Back to cited text no. 20
    
21.
Roozrokh M, Ansari G, Jadali F, Kermani N. Granular cell tumor of newborn: A case report. J Pediatr Surg Spec 2008;2:26-8.  Back to cited text no. 21
    
22.
Saeed MS, Ani MA. Congenital epulis (congenital gingival granular cell tumor). Ann Coll Med 2010;36:153-6.  Back to cited text no. 22
    
23.
Olson JL, Marcus JR, Zuker RM. Congenital epulis. J Craniofac Surg 2005;16:161-4.  Back to cited text no. 23
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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