Congenital granular cell lesion: A rare tumor of new born (original) (raw)

Congenital granular cell lesion: A rare tumor of new born

Rajesh Kumar, Shradha Jaiswal 1{ }^{1}, Ankit Singhal 2{ }^{2}, Ridhi Garg 3{ }^{3}
Departments of Periodontology, 1{ }^{1} Oral and Maxillofacial Pathology, Sri Aurobindo College of Dentistry, Indore, 2{ }^{2} Endodontics, People Dental Academy ,Bhopal, Madhya Pradesh, 2{ }^{2} Orthodontics and Dento Facial Orthopedics, Dr. BR Ambedkar Institute of Dental Sciences and Hospital, Patna, Bihar India

Address for correspondence:
Dr. Rajesh Kumar,
Department of Periodontology, Sri Aurobindo
College of Dentistry, Indore - 453 555, Madhya Pradesh, India.
E-mail: drkumar.perio78@gmail.com

Abstract

Congenital granular cell tumor (CGCT), or congenital epulis, is an uncommon benign soft tissue lesion that usually arises from the alveolar mucosa of neonates and may cause respiratory and feeding problems. The histogenesis and clinical history of the lesion remains obscure. Treatment involves surgical excision and recurrences are rare. The present report describes a case of congenital granular cell lesion (CGCL) in the anterior segment of maxillary alveolar ridge of a 2-month-old female. This lesion was causing feeding problem and was excised under local anesthesia, with no recurrence even after 3 years. Key words: Congenital epulis, congenital granular cell lesion, Newman’s tumor

INTRODUCTION

Congenital granular cell lesion (CGCL) is a rare benign soft tissue tumor of the newborn. It is also synonymously known as gingival granular cell tumor (GCT) of the newborn and was first described by Newmann in 1871, hence also called as Newmann’s tumor. [1]{ }^{[1]} CGCL is a rare lesion of the newborn with an incidence of just 0.0006%.[2]0.0006 \% .{ }^{[2]} To the best of our knowledge only 50 cases have been reported so far. [3]{ }^{[3]} Though benign, this tumor may cause hindrance in breathing and suckling, depending upon the size and position of the growth. Although spontaneous regression has been documented, but the recommended treatment is surgical excision with no reports of recurrence after resection. [4]{ }^{[4]}

This case report is unique as differential diagnosis has been included so as to help the clinician to arrive at a more conclusive diagnosis thereby helping him to plan the treatment accordingly. Also, more the number of case reports on this entity more is the likelihood that we will be able to build a conclusive base for the histogenesis of this uncommon and obscure lesion.

CASE REPORT

A 2-month-old female child was referred with the chief complaint of difficulty in feeding since a few days. On
img-0.jpeg
examination a 2×2×1 mm2 \times 2 \times 1 \mathrm{~mm} pedunculated growth was observed arising from the left side of maxilla in the canine region. The lesion was pink in color, similar to that of the surrounding mucosa, surface was smooth without any ulcerations and was firm in consistency. The patient was uncomfortable because of this small growth which was possibly hindering proper suction during breast feeding.[Figure 1]. Surgical excision of the growth was performed under local anesthesia. There was minimal bleeding during the procedure and the infant was comfortable with breast feeding from the very next day. The gingival mass was fixed in formalin and sent for histopathological examination.

Histopathological examination

Hematoxylin and Eosin (H and E) stained section showed keratinized stratified squamous epithelium and underlying connective tissue stroma. High power view showed delicate Collagen fibers, numerous young fibroblasts and granular cells [Figure 2]. The granular cells were large oval cells with an eosinophillic and granular cytoplasm and single basophilic nucleus; these were seen to be scattered amidst the connective tissue [Figure 3]. Clinicopathological correlations were suggestive of CGCL.

DISCUSSION

Epulis is a Greek word literally meaning “of the gums” and is used to describe a variety of gingival overgrowths. CGCL is a rare, gingival tumor that usually arises in the incisor-canine region of the maxilla (maxilla: mandible ratio is 3:1). [5]{ }^{[5]} This soft tissue tumor appears as smooth-surfaced, pedunculated growth which may be lobulated at times and arises from the gingival mucosa of maxilla or mandible. [6]{ }^{[6]} It ranges in size

img-1.jpeg

Figure 1: Clinical photograph showing small pedunculated gingival mass
img-2.jpeg

Figure 3: Large oval cells with granular cytoplasm and single basophilic nucleus which were scattered between collagen fibers and fibroblasts. (H&E stain, ×100\times 100 )
from few millimetres to 9 cm (as reported till date), with a female preponderance of 8:1.[7]8: 1 .{ }^{[7]}

They are usually firm in consistency, covered by a smooth or erythematous mucosa and are attached by means of a stalk to the underlying tissue. Early diagnosis can be made by prenatal ultrasonography or magnetic resonance imaging (MRI). However, MRI is considered to be superior to ultrasonography, showing its gingival origin without local extension. [8]{ }^{[8]} Treatment of choice is surgical excision.

Histogenesis of this tumor is unclear and the proposed cells of origin are probably derived from one of the sources, that is, odontogenic epithelium, undifferentiated mesenchymal cells, pericytes, fibroblasts, smooth muscle cells, nerve related cells and histiocytes. [4,9]{ }^{[4,9]} An endogenous hormonal stimulus has been proposed as the reason for its occurrence in neonates and for female predominance. But this theory
img-3.jpeg

Figure 2: Photomicrograph showing delicate collagen fibers Abundant fibroblasts and granular cells (H&E stain, ×200\times 200 )
was not accepted because of the lack of detectable estrogen and progesterone receptors within the lesion. [8,9]{ }^{[8,9]}

Histologically, CGCL shows a remarkable similarity with the more common GCTs. [10]{ }^{[10]} However, there are striking microscopic and epidemiological differences between the two. GCT is comparatively less vascular with an occasional component of pseudoepitheliomatous hyperplasia and contains more conspicuous nerve bundles as compare to CGCL. CGCL solely occurs on the gum pads of infants; whereas, GCT occurs in adults and may involve multiple organs. [10]{ }^{[10]} Immunohistochemically, GCTs are reactive to S-100 protein and laminin, while these are absent in CGCL. GCTs also show positive staining for NGFR/p75 and inhibin- α\alpha, while CGCL does not. [11,12]{ }^{[11,12]}

Differential diagnosis includes melanotic neuroectodermal tumor of infancy (MNTI), hemangioma, fibroma, embryonal rhabdomyosarcoma, granuloma, malignant granular cell myoblastoma, chondrogenic and osteogenic sarcoma and schwannoma. [13]{ }^{[13]}

MNTI is an osteolytic pigmented neoplasm primarily affecting jaws of newborns. However, MNTI and CGCL are distinctly different as MNTI is infiltrating, contains melanin pigment and tumor mass comprises of alveolar spaces lined by cuboidal or large polygonal cells having pale cytoplasm. Hemangioma is primarily a vascular lesion with budding endothelial cells and essentially not present at birth; whereas, CGCLs manifest within the 1st 1^{\text {st }} month of life. Fibroma is more common in 5th 5^{\text {th }}, 6th 6^{\text {th }} and 7th 7^{\text {th }} decade. It consists of bundles of interlacing collagen fibers and no granular cells. Embryonal rhabdomyosarcoma is a malignant tumor of striated muscle and uncommon in the oral cavity. Granuloma is primarily a focal collection of epithelioid cells, lymphocytes and giant cells. Malignant granular cell myoblastoma is commonly seen between 30 and 60 years of age. Histologically, granular cells are in ribbons separated

by septa giving an appearance of invasion or infiltration into underlying tissues. Chondrogenic and osteogenic sarcoma are malignant tumors characterized by chondroid or osteoid tissue, respectively. Schwannoma is a tumor of neural origin which shows Antoni A and B areas and Verocay bodies.

In conclusion, CGCL can be diagnosed by prenatal sonography, but prenatal findings are not specific. [14]{ }^{[14]}

REFERENCES

  1. Newmann E. Ein fall von Kongenitalu epulis. Arch Heilkd 1871;12:189-90.
  2. Bosanquet D, Roblin G. CGCL: A case report and estimation of incidence. Int J Otolaryngol 2009;31:198-9.
  3. Dash JK, Sahoo PK, Das SN. Congenital granular cell lesion “congenital epulis”- Report of a case. J Indian Soc Pedod Prev Dent 2004;22:63-7.
  4. Fuhr AH, Krogh PH. CGCL of the newborn: centennial review of the literature and a report of case. J Oral Surg 1972;30:30-5.
  5. Lack EE, Perez-Atayde AR, McGill TJ, Vawter GF. Gingival granular cell tumour of the newborn (congenital epulis): ultrastructural observations relating to histogenesis. Hum Pathol 1982;13:686-9.
  6. Lapid O, Shaco-Levy R, Krieger Y, Kachko L, Sagi A. Congenital epulis. Pediatrics 2001;107:e22.
  7. Kanotra S, Kanotra SP, Paul J. Congenital epulis. J Laryngol Otol 2006;120:148-50.
  8. Silva GC, Vieira TC, Vieira JC, Martins CR, Silva EC. Congenital granular cell tumour (congenital epulis): a lesion of multidisciplinary interest. Med Oral Patol Cir Bucal 2007;12:E428-30.
  9. Raissaki MT, Segkos M, Prokotakis EP, Haniotis V, Velegrakis GA, Gourtsoyiannis N. Congenital granular cell tumour (epulis): post natal imaging appearances. J Comp Assist Tomogr 2005;29:520-3.
  10. Damm DD, Cibull ML, Geissler RH, Neville BW, Bowden CM, Lehmann JE. Investigations into histogenesis of congenital epulis of newborn. Oral Surg Oral Med Oral Pathol 1993;76:205-12.
  11. 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;19:527-9.
  12. Vered M, Dobriyan A, Buchner A. Congenital granular cell epulis presents an immunohistochemical profile that distinguishes it from granular cell tumour of the adult. Virchows Arch 2009;454:303-10.
  13. Rajendran R, Shivpathasundaram B, editors. Shafer’s Textbook of Oral Pathology, 6th 6^{\text {th }} ed. Amsterdam: Elsevier Publication.
  14. Tokar B, Boneval C, Mirapoglu S, Tetikkurt S, Aksöyek S, Salman T, et al. Congenital granular cell tumour of gingiva. Pediatr Surg Int 1998;13:594-6.

How to cite this article: Kumar R, Jaiswal S, Singhal A, Garg R. Congenital granular cell lesion: A rare tumor of new born. J Oral Maxillofac Pathol 2013;17:440-2.
Source of Support: Nil. Conflict of Interest: None declared.