Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Granular Cell Tumor of Newborn: A Case Report

Roozrokh M.¹, Ansari G.², Jadali F.³, Kermani N.²

¹. Department of Pediatric Surgery, Mofid Children’s Hospital.

². Department of Pedodontics Dental School

³. Department of Pathology, Mofid Children’s Hospital,

Shahid Beheshti University MC.

Tehran, Iran

 

Correspondence:

Dr Ghassem. Ansari

Associate Professor

Department of Pedodontics, Dental School, Shahid Beheshti University MC; Tehran, Iran

Tel: (98) 21 222 555 37, Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Granular cell tumor (GCT) is a rare benign soft tissue neoplasm. The most common place is the oral cavity. The most frequent occurrence stage is 4th to 6th decade of life with a rare prevalence in children. A 3 days old female patient was admitted to Mofid Children`s Hospital, Tehran with a protruding mass out of the mouth. She underwent a surgical removal of lesion under general anesthesia. The mass was completely excised and sent to pathology department for future assessment and definitive diagnosis.

Clinical appearance represented a lobulated, capsular mass measuring 3.5 X 1.5 X 2.5 cm, pedunculated from labial area of upper right maxilla. The histological assessment confined the diagnosis of granular cell tumor. A 3 to 6 months follow-up revealed no recurrence, the child was gaining weight and the feeding was reasonable. The case was diagnosed as GCT based on histopathological findings. Differential diagnosis with congenital granular tumor should be considered.

Key words: granular cell tumor, newborn.

  

Introduction

The granular cell tumor (GCT) is an uncommon benign soft tissue neoplasm. The histogenesis of this lesion has long been debated [1]. Abrikosoff (1926) was first who described GCT as granular cell myoblastoma as it was believed to be of skeletal muscle origin [2]. GCT is mostly common in the oral cavity and then on the skin. The most common site is the tongue (one third to one half of all cases) and the second most frequent site is buccal mucosa. It occurs most frequently at the fourth to sixth decades of life and is quite rare in children. The tumor is usually asymptomatic. It is best treated by conservative local excision and recurrence is uncommon [1].The aim of this report is to identify this isolated case and provide new data for science.

granular cell tumor 1 granular cell tumor 2
Figure 1. The preoperative picture of the tumor Figure 2. Tumor is being removed

 

granular cell tumor 3 granular cell tumor 4
Figure 3. Post operative view of the site of surgical operation; note the underlying bone natural position Figure 4. Histological preparation of the tumoral tissue, note the plasmocytoid cells and abundant eosinophilic cytoplasm

Case report

A newborn female was referred to the outpatient clinic at Mofid Children’s Hospital, Tehran in fall 2007. The patient presented a mass of tissue standing out of the mouth. The child was referred a day after birth due to the presence of this large lesion protruding from her upper maxillary right area, obstructing the feeding and disruption for seal achievement in sucking process. There was no complication on airways, based on the parents report.

Clinical examination revealed a large mass of soft tissue projected from upper anterior maxillary area on the right quadrant (Fig.1).

The tumor’s texture could be described of an elastic nature, pedunculated and firmly connected to the alveolar ridge. Surgical removal was decided, under general anesthesia. A sharp (No.11) scalpel was employed along with an electro cauterizing unit to control bleeding (Fig.2, 3). Excised tissue was then placed into a pathology sample container of formaldehyde and immediately transferred to histopathology lab for assessment and identification. Pathology report indicated that the specimen being a soft tissue mass consisting of a lobulated, capsular round tan-brown elastic tissue fragment with the total measurement of 3.5 X 2.5 X 1.5 cm.

Result

Pathology report indicated that the solid and light tan color on cut section as showing squamous epithelium overlying a neoplasm composed of plasmocytoid cells. Abundant eosinophilic cytoplasm was also observed on microscopic examination. The case was therefore concluded as a Granular Cell Tumor (Fig. 4). Patient was discharged the day after the surgical excision. A 3 to 6 months follow-up revealed no recurrence, the child was gaining weight and the feeding was reasonable. The jaws development appeared to be of normal pattern and therefore no other intervention was needed. Such cases are followed-up every 3 months, for at least a year after the operation.

Discussion

After Abrikosoff (1926) who named GCT as granular cell myoblastoma, Thompson (1984) in an ultra structural study showed that the GCT is in direct continuity to the striated muscle [2]. The theory of neurogenic origin was supported by Holland et al, who demonstrated s-l00 staining in Schwann cells but not in myoblasts [3]. There is a 2:1 female predilection and more than half of the cases have been reported as occurred in African American patients [1, 3, 4, 5].

The tumor occurs most frequently in the forth to sixth decades of life. Surgical excision is the treatment of choice and recurrence is uncommon unless incomplete excision of the lesion [1]. Based on the literature it is clear that GCT is rare in newborn and also rare in alveolar ridge but the other soft tissue lesion which is more common in newborn alveolar ridge is known as congenital epulis. The current case was first diagnosed as epulis using clinical features of the tumor however misdiagnosis could take place if only clinical findings are referred to. The use of histopathology data is confirmed as the most reliable method to identify cases of unknown nature. To date around 200 cases of epulis have been described in the literature [6]. Rare examples of epulis also have been described on the tongue which is the most common site for GCT, this is why several investigators have given the term of congenital cell lesion (CGCT) or gingival granular cell tumor of the newborn to this identity [1,7,9].

CGCT is a differed entity from those of the GCT. It has a marked female predominance of 8-10:1 and mostly occurs on maxillary alveolar ridge [7, 10, 11]. The treatment of GCT is mostly excision with little to no chance of recurrence and possible damage to the future dentition [1].

Histopathological it is known that congenital epulis consists of granular cells and is similar to the true granular cell tumor, but there are some differences including lesser vascularity with often pseudo epithelialomatous hyperplasia. It also has more conspicuous nerve bundles than congenital epulis. It is of great interest to note that immune-histo-chemical findings with antiserum to S100 protein are only positive for GCT and not for CGCT [12].

Several other differential diagnoses could be considered including neuroectodermal tumor of infancy, hemangioma, fibroma, granuloma, rhabdomyosarcoma and squamous cell carcinoma [1, 12, 13, 14]. GCT is best treated by conservative local excision and recurrence is quite uncommon [1].

Conclusion

The case of Granular Cell Tumor is presented in a newborn female with no relapse after complete surgical removal within the first year follow up. Dentists may initially be consulted in such cases, emphasizing the need for proper differential diagnosis.

 

 

REFERENCES

1. Neville BW, DAMM DD, Allen CM, Bouquot JE:Oral&Maxillofacial Pathology.2nd ed.Philadelphia:W.B. Saunders Co.2002; 12:465-467.

2. Kamal SA, Othman EO. Granular cell tumor of the larynx. J laryngol Otol 1998; 112:83-85.

3. Holland RS, Abaza N, Balsara G, lesser R. Granular cell tumor of the larynx in a six year old child: case report and review of the literature. Ear Nose Throat 1998; 77:652-654.

4. Victoria LV, Hoffman HT, Robinson RA, Granular cell tumor of the larynx. J laryngol Otol 1998; 112:373-376.

5. Kudawara I, Ueda T, Yoshikawa H. Granular cell tumor of the subcutis: CT and MRI findings- a report of three cases. Skeletal Radial 1999; 28:96-99.

6. Leocata P, Bifaretti G, Saltarelli S,Corbacelli A.Ventura L. Congenital (granular cell ) epulis of the newborn: a case report with immunohistochemical study on the histogenesis. Ann Saudi Med 1999;19(6):527-9.

7. Dash JK, Sahoo PK, Das SN. Congenital granular cell lesion congenital epulis – report of a case. J Indian Soc Pedod Prev Dent 2004;22(2):63-7.

8. Yavuzer R, Ataoglu O, Sari A. Multiple congenital epulis of the alveolar ridge and tongue. Ann Plast Surg. 2001; 47(2):199-202.

9. Packeisen J, Nowak M, Kruger A. Epulis eines Neugeborenen. Histogenetischer vergleich Zum Granularzell tumor des Erwachsenen. Pathologe 2002; 23(2):145-8.

10. Inan M, Yalcin O, Pul M. Congenital fibrous epulis in the infant. Yonsei Med J 2002; 43(5):675-7.

11. Damm DD, Cibull ML, Geissler RH, et al. Investigation into histogenesis of congenital epulis of the newborn. Oral Surg Oral Med Oral Pathol 1993; 76: 205-212.

12. Witman PM, Rogers RS. Pediatric oral medicine. Dermatol Clin 2003; 21(1):157-70.

13. Tokar B, Boneval C, Mirapoglu S,Tetikkurt S,Salman T. Congenital granular cell tumor of the gingiva. Peditr Surg Int 1998; 13: 594-6.