CASE REPORT

Granular cell tumor in two oral anatomic sites

Marianna Sampaio Serpa1 • Hugo Costa-Neto1 • Patrıcia Teixeira de Oliveira1 •

Ericka Janine Dantas da Silveira1 • Ana Miryam Costa de Medeiros1

Received: 30 November 2015 / Accepted: 21 March 2016 / Published online: 23 March 2016

� Springer-Verlag Berlin Heidelberg 2016

Abstract Granular cell tumor (GCT) is an uncommon

benign soft tissue neoplasm, which usually presents as a

solitary nodule, appearing especially in the tongue. There

are few cases of multiple oral GCT in the English-language

literature, with only three cases reported in the last

20 years. This report describes a case of two oral GCT in a

young patient and provides a literature review from 1995 to

2015. Clinical characteristics of the reviewed cases were

retrieved and compared with the present case. Exception-

ally, the current case was the only one that occurred in an

adolescent and solely affected the oral cavity. Besides the

oral cavity, the other cases also observed GCT lesions in

the skin. Although rare, it is important to know multiple

oral GCT clinical and histopathological characteristics so

they can be correctly diagnosed, treated and followed up.

Keywords Oral granular cell tumor � Clinicalcharacteristics � Diagnosis � Treatment

Introduction

Granular cell tumor (GCT) is an uncommon benign neo-

plasm that has been thought to be of neural origin due to its

strong and consistent stain for S-100 protein [1]. Clinically,

GCT is typically an asymptomatic sessile nodule of pinkish

color, occurring most frequently between the fourth and

sixth decades of life [2]. Although usually solitary, multi-

ple GCT lesions can occur in up to 25 % of the cases, being

most often located in the intradermal or subcutaneous tis-

sue [3]. Few cases of multiple oral GCT have been reported

in the English-language literature. We report herein a case

of GCT occurring in the dorsum and lateral tongue of a

young patient and provide a literature review from 1995 to

2015.

Case presentation

A 12-year-old female patient was referred to the Depart-

ment of Stomatology of the Federal University of Rio

Grande do Norte, complaining of asymptomatic swellings

in the tongue of unknown evolution time. Medical history

was not helpful and extraoral physical exam showed no

alterations. Intraoral examination revealed two lesions with

nodular aspect, sessile implantation, normal mucosa color

and firm consistence (Fig. 1). The lesions were located in

the dorsum and lateral border of the tongue, measuring,

respectively, 0.8 and 0.5 cm in diameter. According to the

report, no trauma occurred in these areas. Clinical diag-

nosis hypotheses were GCT, neurilemmoma and fibroma.

Excisional biopsy of the lesions was performed and

microscopic examination revealed both specimens were

nonencapsulated neoplasms composed of polygonal cells

with large cytoplasm, remarkably granular appearance and

indistinct margins, presenting ovoid and pycnotic nuclei.

Close relationship between the skeletal muscle and the

neoplastic cells was also observed (Fig. 2a). Immunohis-

tochemical analysis of both lesions revealed strong posi-

tivity of the tumor cells for S-100 protein (rabbit

polyclonal; dilution 1:2000; Dako, Glostrup, Denmark)

(Fig. 2b). Periodic acid-Schiff (PAS) was performed to

& Marianna Sampaio Serpa

mariannaserpa@hotmail.com

1 Postgraduate Program in Oral Pathology, Department of

Dentistry, Federal University of Rio Grande do Norte,

Avenida Senador Salgado Filho, 1787, Lagoa Nova, Natal,

Rio Grande do Norte CEP 59.056-000, Brazil

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Eur Arch Otorhinolaryngol (2016) 273:3439–3441

DOI 10.1007/s00405-016-4006-5

further characterize this cell population (Fig. 2c). In view

of these findings, the histopathological diagnosis was GCT.

The patient has been under follow-up for 1 year without

any signal of recurrence.

Discussion

Three cases of multiple oral GCT were identified in the

English-language literature from a search on PubMed and

Scopus from 1995 to 2015. These cases, along with the

current case are listed in Table 1. Additionally, all clinical

features of the cases are summarized in Table 2.

Adding up the four cases, a total of 28 GCT were found;

the range was 2–12 lesions per patients, and the mean

number of lesions was seven. Of the 28 lesions, 16 lesions

were located in the oral cavity and the tongue was the most

common site (56.25 %). The mean age was 29.5 and all

cases occurred in melanodermic women. Furthermore, the

present case was the only one to solely affect the oral

cavity. In the other cases, gastrointestinal tract and, par-

ticulary, subcutaneous involvement was common, involv-

ing hands, feet, elbow, face, groin, perineum and vulva. In

cases 1 and 2, after surgical excision and histopathological

diagnosis, more GCT lesions appeared in other locations,

including oral and extraoral sites.

Multiple GCT are uncommon lesions and most com-

monly affect melanodermic women between 30 and

50 years old [2], although the present case affected an

adolescent patient. As for the location, solitary oral GCT

has been described to mainly affect the dorsal surface of

Fig. 1 Intraoral clinical exam. Nodular lesions in dorsum (a) and

lateral border (b) of the tongue

Fig. 2 Photomicrographs of GCT. a Polygonal cells with large

granular cytoplasm and small nuclei. Close relationship between

neoplastic cells and muscle tissue is also observed (hematoxylin–

eosin stain; 9200 lm). b Strong and uniform positivity for S-100

protein (immunohistochemical stain, 920 lm). c Cytoplasmic gran-

ules highlighted (PAS stain, 920 lm)

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the tongue [7]. Based on this review, it was observed

multiple oral GCT appear to involve most frequently the

lateral border of the tongue, following the dorsum.

Except for the present case, besides the oral region, the

other cases also involved lesions in the skin and gastroin-

testinal tract. Moreover, because of the patient’s young age,

and previous reports of new GCT lesions appearing after

histopathological diagnosis, it is possible that as the patient

gets older, other GCT lesions show up in other locations

besides the oral cavity.

Multiple GCT have previously been associated with

systemic disorders, such as growth retardation, lentiginosis,

cafe-au-lait spots and neurofibromatosis type I, occurring

mainly in children or adolescents [8]. Due to that, it is

important to consider other abnormalities in a young

patient with multiple GCT. In the current case, the patient

reported no relevant medical history and no other physical

alterations were observed in clinical examination, dis-

carding any possible systemic disorder.

Surgical excision with margin of safety is the treatment

of choice and recurrences are rare [1]. In this review, there

was no case of recurrence, however, in two cases more

GCT lesions showed up after histopathological diagnosis,

which suggests the need for a long-term follow-up.

Compliance with ethical standards

Funding None.

Conflict of interest All authors declare that they have no conflict of

interest.

Ethical approval All procedures performed in this study involving

the patient were in accordance with the ethical standards of the

institutional and/or national research committee and with the 1964

Helsinki declaration and its later amendments or comparable ethical

standards.

Informed consent Informed consent was obtained from the patient

in this case report.

References

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Table 1 Report of multiple oral granular cell tumors in the last 20 years

Case no. Authors Sex Age Skin color Oral site Total no. of oral lesions

1 Collins and Jones [4] F 39 Melanodermic Tongue (6), mouth floor (2) 8

2 Sargenti-Neto et al. [5] F 41 Melanodermic Tongue (1), lips (1) 2

3 Varghese et al. [6] F 26 Melanodermic Lips (4) 4

4 Present case—2015 F 12 Melanodermic Tongue (2) 2

Table 2 Clinical features of multiple oral granular cell tumors

Location of oral GCT (n = 16)

Tongue 9 (56.25 %)

Dorsal surface 1 (11.11 %)

Ventral surface 1 (11.11 %)

Lateral border 7 (77.77 %)

Lips 5 (31.25 %)

Floor of the mouth 2 (12.5 %)

Age (years, n = 4)

Mean 29.5

Range 12–41

Sex (n = 4)

Female 4 (100 %)

Male 0 (0 %)

Skin color (n = 4)

Melanodermic 4 (100 %)

Leucodermic 0 (0 %)

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