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Important Notice

Case report
Year : 2024 | Month : May | Volume : 18 | Issue : 5 | Page : ED11 - ED13 Full Version

A Rare Case of Granular Cell Tumour of Tongue in a 13-year-old Girl

Published: May 1, 2024 | DOI:
Archana Shivamurthy, Archana Muniswamyreddy, Vijaya Basavaraj

1. Assistant Professor, Department of Pathology, Sri Madhusudhan Sai Institue of Medical Sciences and Research, Chikkaballapur, Karnataka, India. 2. Assistant Professor, Department of Pathology, JSS Medical College, JSSAHER, Mysuru, Karnataka, India. 3. Professor, Department of Pathology, JSS Medical College, JSSAHER, Mysuru, Karnataka, India.

Correspondence Address :
Dr. Archana Muniswamyreddy,
No. 21, First Stage, Forth Cross, Brindavan Extension, Mysuru, Karnataka, India.


Granular Cell Tumour (GCT) is a rare benign tumour that usually affects adults in the third to sixth decades, with females being more commonly affected. Here, a rare case of GCT occurring in a 13-year-old child is presented. A young girl presented with a painless swelling on the right-side of her tongue. Her past medical history was unremarkable. Upon examination, a firm, well-defined, non pulsatile, non tender lesion measuring 1×0.8 cm was noted, involving the right lateral border of the tongue. The lesion was excised and sent for histopathological examination. Gross examination revealed a 1×0.8×0.6 cm mass with a grey-white cut surface. Microscopy showed hyperplastic stratified squamous epithelium overlaying a submucosal tumour composed of polygonal cells arranged in sheets. The tumour cells had central small round nuclei with abundant granular eosinophilic cytoplasm and intervening skeletal muscle fibres. No necrosis, atypia, or increased mitosis were observed. Immunohistochemistry showed strong nuclear positivity and bright granular cytoplasmic positivity for S-100, confirming the diagnosis of GCT. The patient was followed-up for 14 months after surgery and reported no recurrences. GCTs typically manifest in older individuals, with the highest prevalence in the fifth and sixth decades of life. However, this case deviates from the norm, being noted in a 13-year-old child. This exceptionally unusual presentation should prompt the inclusion of GCT in the differential diagnosis of tongue neoplasms in paediatric patients as well. Periodic follow-up of these patients is recommended to detect malignant transformations and late recurrences at early stages.


Abrikossof’s tumour, S-100, Tongue neoplasms

Case Report

A 13-year-old girl presented to the otorhinolaryngology clinic with a swelling on the right-side of her tongue that had been present for two months. It had an insidious onset, was slowly progressive in size, and was painless. Her past medical history was unremarkable. Local examination revealed a solitary swelling measuring 1×0.8 cm involving the right lateral border of the tongue (Table/Fig 1). The swelling was non pulsatile, non tender, firm, with fairly well-defined borders, and the overlying mucosa appeared normal. General physical examination was within normal limits, and there was no other significant personal or family history. A clinical differential diagnosis of neurofibroma, lipoma, schwannoma, and GCT was considered. Based on these findings, a decision was made to excise the lesion. The swelling was excised under general anaesthesia, appropriate sutures were placed, and haemostasis was achieved. The specimen was sent for histopathological examination. On gross examination, the lesion measured 1×0.8×0.6 cm with a grey-white cut section. Microscopy revealed hyperplastic stratified squamous epithelium overlying a submucosal tumour composed of polygonal cells arranged in sheets. Individual tumour cells had central small round nuclei with abundant granular eosinophilic cytoplasm and intervening skeletal muscle fibres (Table/Fig 2). There was no necrosis, atypia, or increased mitosis observed. These features were suggestive of GCT. Immunohistochemical staining with S-100 was performed to confirm the diagnosis. The tumour cells showed strong nuclear positivity and bright granular cytoplasmic positivity for S-100 (Table/Fig 3), thereby confirming the diagnosis of GCT. In the present case, complete healing occurred in one week, and no recurrence was observed after 14 months of follow-up.


The GCT is a rare, benign tumour that commonly affects the tongue. With an incidence of approximately 1 in 1,000,000 population per year, it affects adults between the second and sixth decades of life (1),(2). It is often accidentally detected and has a female preponderance. It presents as a small lesion, with a size ranging from 1-3 cm. It is a painless, slow-growing tumour that is often asymptomatic at presentation. The reported prevalence ranges from 0.019-0.03% of all human neoplasms (2),(3),(4). The present case, reports a GCT of the tongue in a 13-year-old girl.

GCT is also known as Abrikossoff’s tumour, named after Russian pathologist Alexei Ivanovich Abrikossoff who described it in 1926 (1),(5). Historically, different terminologies were applied to this lesion, which include myoblastoma, granular cell nerve sheath tumour, and granular cell schwannoma owing to the uncertain histogenesis of this tumour, said to be derived from adult or embryonic muscle cells or Schwann cells (1),(2),(4),(6). In 1948, Fust and Custer were the first to point out its neurologic origin and stated that these tumours arise from Schwann cells. Later, using electron microscopy and histochemical techniques, Fischer and Wechsler provided convincing evidence that the cell of origin is the Schwann cell and suggested that these lesions be called granular cell schwannomas (6).

GCT usually involves adults between the second and sixth decades of life, and its incidence in children is rare. A thorough search of literature to identify published cases of GCT of the tongue in the paediatric age group (<15 years) was done, and the results are tabulated (Table/Fig 4) (5),(7),(8),(9),(10),(11),(12). Females are known to be affected 2-3 times more frequently when compared to men. GCT can affect any part of the body (1),(3),(13). About 45-65% of the GCTs involve the head and neck region, with 70% of these noted in the intraoral region. The tongue is the most common intraoral region affected, followed by the lips, retrocommissural area, buccal mucosa, and hard palate in order of frequency. The tongue, being rich in nerve fibres encased by Schwann cells, can be the cells from which GCTs arise. GCTs can be seen at various other locations like the skin, subcutaneous tissue, central nervous system, trachea, larynx, bladder, uterus, and vulva (2),(6),(14). In the present case, the lesion involved the right lateral border of the tongue.

On intraoral examination, GCTs of the tongue appear as solitary asymptomatic nodules, pink in colour, occasionally being yellowish. They are small lesions and are often less than three cm in size. They involve the subcutaneous or submucosal tissues. A few patients may present with a past history of inflammation or trauma at the involved site. Clinical differential diagnosis include fibroma, lipoma, neurofibroma, and schwannoma (2),(8),(15). On histopathological examination, GCTs are characterised by submucosal proliferation of polygonal cells, arranged in sheets. The abundant granular eosinophilic cytoplasm and small round to oval monotonous nuclei are characteristic features. These histologic features noted in present case helped us consider a diagnosis of GCT and rule out clinical differential diagnoses of lipoma, schwannoma, and neurofibroma.

Approximately 1-2% of benign GCTs can show metastasis. The characteristic features of malignant GCTs are described by Fanburg-Smith. These criteria include spindling of the tumour cells, high nuclear-cytoplasmic ratio, vesicular nuclei with large nucleoli, nuclear pleomorphism, increased mitotic rate (>2/10 hpf), and necrosis. Malignant GCTs show three or more of these features. The common sites for metastasis include the bone, lung, breast, lymph nodes, and peritoneum. The present case didn’t show any features of malignant transformation. Immunohistochemically, these tumours are positive for neuron-specific enolase and S-100. The neurogenic origin is supported by the immunohistochemical localisation of these markers in the tumour cells. Genetically, loss of heterozygosity involving chromosomes 9p and 17p has been described (2),(4),(5),(14).

The histopathological differential diagnoses include histiocytosis, amelanotic melanoma, oral granular cell leiomyoma, Pecoma (perivascular epithelioid cell tumour), xanthoma, alveolar soft part sarcoma, Rosai-Dorfman disease, and primitive non neural GCT (16),(17),(18),(19),(20). Correlation of clinicopathological parameters and immunohistochemical marker expression helps differentiate GCT from the other tumours. The differential diagnosis of oral lesions with cells having granular cytoplasm, their distinguishing features from GCT, and Immunohistochemistry (IHC) findings are tabulated (Table/Fig 5).

As GCTs are non encapsulated and a small risk of recurrence (7%) is associated with positive margins, a complete surgical excision with margin clearance is warranted. Long-term follow-up of these patients is advised as there is a risk of malignant transformation in about 10% and distant metastasis in about 2% of cases. Laser excision surgery has also been used as a treatment of choice in a few cases. The advantages include a reduction in bleeding and postoperative pain. It is also associated with more rapid healing.

Some authors have also stated that the vaporisation effect on remnant tissues could eliminate GCT cells on the surgical bed, thus hypothetically leading to a lower rate of recurrence (3),(5),(21). Lafuente Ibáñez de Mendoza I et al., conducted a multicentric study and a systematic review of oral GCTs in Spain and Brazil (22). A total of 272 cases of oral GCT were studied. Only 12 cases out of 272 were GCTs of the tongue in children <15 years of age. This study proves the rarity of GCTs of the tongue in the paediatric age group. A high index of suspicion, thorough clinical examination backed by histopathological and IHC studies help us arrive at a diagnosis. Excision is the treatment of choice, and periodic follow-up of these patients is recommended to detect malignant transformations and late recurrences at early stages.


GCT is a rare tumour that occurs on the tongue. The clinicopathological features of this lesion are derived from isolated case reports or case series. Categorising GCTs as benign and malignant is further challenging. GCTs typically manifest in older individuals with the highest prevalence in the fifth and sixth decades of life. However, the present case deviates from the norm and was noted in a 13-year-old child. This exceptionally unusual presentation should prompt the inclusion of GCT in the differential diagnosis of tongue neoplasms in paediatric patients as well.


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DOI and Others

DOI: 10.7860/JCDR/2024/70014.19427

Date of Submission: Feb 08, 2024
Date of Peer Review: Mar 04, 2024
Date of Acceptance: Mar 25, 2024
Date of Publishing: May 01, 2024

• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

• Plagiarism X-checker: Feb 09, 2024
• Manual Googling: Mar 19, 2024
• iThenticate Software: Mar 22, 2024 (13%)

ETYMOLOGY: Author Origin


JCDR is now Monthly and more widely Indexed .
  • Emerging Sources Citation Index (Web of Science, thomsonreuters)
  • Index Copernicus ICV 2017: 134.54
  • Academic Search Complete Database
  • Directory of Open Access Journals (DOAJ)
  • Embase
  • EBSCOhost
  • Google Scholar
  • HINARI Access to Research in Health Programme
  • Indian Science Abstracts (ISA)
  • Journal seek Database
  • Google
  • Popline (reproductive health literature)