Granular cell tumour (Abrikossoff’s tumour) of the tongue (2024)

Granular cell tumour (Abrikossoff’s tumour) of the tongue (1)

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Granular cell tumour (Abrikossoff’s tumour) of the tongue

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  1. http://orcid.org/0000-0003-0753-1608Pedro Nuno Dias Ferraz1,
  2. Vladislav Danu1,
  3. Rui Almeida2 and
  4. José Figueiredo1
  1. 1Stomatology, Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal
  2. 2Pathology, Centro Hospitalar e Universitário de Coimbra EPE, Coimbra, Portugal
  1. Correspondence to Dr Pedro Nuno Dias Ferraz; pedrodferraz{at}icloud.com

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    • dentistry and oral medicine
    • mouth
    • head and neck cancer
    • oral and maxillofacial surgery

    Description

    We report a case of a 43-year-old female patient, referred to the stomatology consultation by her general and family medicine doctor, for the observation of a 1-year-old lesion on the tongue. The patient reported that the lesion was painless and slow growing.

    She had no relevant personal history or relevant usual medication. There were no smoking and/or alcohol habits.

    Physical examination revealed a firm, well-circ*mscribed whitish lesion of about 1.5 cm in diameter, located at the posterior limit of the middle third of the dorsum of the tongue (figure 1).

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    Figure 1

    Image of lesion.

    Thus, an incisional biopsy was performed, whose histological study revealed the diagnosis of granular cell tumour (GCT) in the dorsal region of the tongue.

    Given the result, we opted to perform an excisional biopsy under local anaesthesia (figure 2). The macroscopic sample had the dimensions of 1.5×0.9×0.4 cm, with whitish surface. Its anatomopathological study confirmed the diagnosis of GCT in the dorsal region of the tongue, and it also reported a submucosal lesion, consisting of proliferation of polygonal cells with large clarified granular cytoplasm Periodic acid–Schiff (PAS+), and with central, oval and monotonous nucleus. There was pseudoepitheliomatous hyperplasia (PH) of the lining. Regarding immunohistochemical analysis, the cells had marked and diffuse immunostaining for S100 protein (figures 3–5).

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    Figure 2

    Excisional biopsy.

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    Figure 3

    H&E (100× and 400×): poorly defined lesion composed of sheets separated by collagenous band. Cells are polygonal or spindle, with abundant and granular eosinophilic cytoplasm, and small nuclei.

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    Figure 4

    H&E (100× and 400×): poorly defined lesion composed of sheets separated by collagenous band. Cells are polygonal or spindle, with abundant and granular eosinophilic cytoplasm, and small nuclei.

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    Figure 5

    Immunohistochemistry S100 (100×): lesional cells expressing diffuse positivity for S100 protein.

    In the postoperative period, the patient evolved without intercurrences. There are no signs of recurrence of the lesion 1 year after the surgery.

    GCT is an uncommon benign neoplasm that still reveals some controversial aspects. GCT can commonly occur in the oral cavity, particularly in the anterior part of the tongue.1 Also known as Abrikossoff’s tumour, is characteristically asymptomatic, with slow growth, and is often accidentally detected. It is characterised by the presence of a small, well-defined, submucosal nodular mass, about 1–3 cm in size, of firm consistency and usually covered by intact mucosa.2 The estimated incidence of oral GCT is approximately 1:1.000 000 population per year. There are no distinct geographical or racial differences. Apparently, there is no explanation for the well-known female predilection.2 The diagnostic hypothesis was fibroma, lipoma, neurofibroma, schwannoma and GCT. Neurofibroma and schwannoma should be the main considerations for tongue injuries. Lipoma and other benign mesenchymal neoplasms may present intraorally as asymptomatic nodules similar to the GCT. Traumatic fibroma is a common reactive lesion that should be included in the differential diagnosis.3

    The persistence of the presence of S100 protein (pathognomonic marker for peripheral nerve sheath tumours) associated with anatomical similarities with peripheral nerve fibres supports this theory. In this case, the immunohistochemical analysis showed a positive chain for protein S100, which is considered sufficient for the proposed diagnosis4 (figure 5). The phenomenon of PH is uncommon in other benign connective tissue tumours but is frequent in GCT.1

    Although aggressive and malignant variants of this neoplasm have been described, most of the GCTs are benign. Complete excision of the lesion may not be always possible due to absence of capsule. Therefore, it is advisable to perform an excision with enough safety margin to reduce the probability of recurrence.

    Learning points

    • The importance of the relationship between clinical and histological and immunohistochemical aspects, all of which are essential to establish the correct diagnosis of granular cell tumour (GCT).

    • Immunohistochemical analysis makes it possible to deepen the knowledge of the aetiopathogenesis of GCT as well as the possible association with other tumours.

    • It is possible to perform a correct medical-surgical approach to this type of injury.

    Acknowledgments

    I would like to thank Dr José Paiva Amorim for his support.

    References

      1. Freitas J,
      2. Mata L, et al

      . Tumor de células granulares intra-orais: apresentação de dois casos clínicos. Rev Port Estomatol Med Dentária e Cir Maxilofac 2019;60:49.

      OpenUrl

      1. van de Loo S,
      2. Thunnissen E,
      3. Postmus P, et al

      . Granular cell tumor of the oral cavity; a case series including a case of metachronous occurrence in the tongue and the lung. Med Oral Patol Oral Cir Bucal 2015;20:e303.doi:10.4317/medoral.19867pmid:http://www.ncbi.nlm.nih.gov/pubmed/24880452

      OpenUrlPubMed

      1. Serpa MS,
      2. Costa-Neto H,
      3. de Oliveira PT, et al

      . Granular cell tumor in two oral anatomic sites. Eur Arch Otorhinolaryngol 2016;273:343941.doi:10.1007/s00405-016-4006-5pmid:http://www.ncbi.nlm.nih.gov/pubmed/27007285

      OpenUrlPubMed

      1. Ferreira JCB,
      2. Oton-Leite AF,
      3. Guidi R, et al

      . Granular cell tumor mimicking a squamous cell carcinoma of the tongue: a case report. BMC Res Notes 2017;10:16.doi:10.1186/s13104-016-2325-7

      OpenUrlCrossRef

    Footnotes

    • Contributors PNDF: wrote the manuscript. VD: contribution in the acquisition of data. RA: made his contribution in his help of anatomopathological and histological analysis. JF: made his contribution in conduct and reporting the case.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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    Granular cell tumour (Abrikossoff’s tumour) of the tongue (2024)

    FAQs

    Granular cell tumour (Abrikossoff’s tumour) of the tongue? ›

    Granular cell tumor, also known as Abrikossoff's tumor, is characteristically asymptomatic, with slow growth and is often accidentally detected. It is characterized by the presence of a small, well-defined, submucosal nodular mass, about 1 to 3 cm in size, of firm consistency and usually covered by intact mucosa [7].

    How do you treat granular cell tumors on the tongue? ›

    Treatment is with surgical resection. With benign granular cell tumors, local surgical excision is curative, if complete resection is achieved; however, recurrence is possible even withclear margins.

    What is a granular cell tumor in abrikossoff? ›

    A rare type of soft tissue tumor that usually begins in Schwann cells (cells that hold nerve cells in place). It can occur anywhere in the body, but it usually occurs in or under the skin of the head and neck (especially the mouth or tongue).

    What is a GCT tumor on the tongue? ›

    GCTs are relatively rare benign tumors that can occur throughout the body. The tongue is involved in ≥60% of oral GCTs, although these tumors can also be found in the head and neck region, buccal mucosa, hard palate, lips and gingiva [3].

    What is the prognosis for granular cell tumors? ›

    The outcomes of granular cell tumors depend in part on whether the lesions are malignant or benign. Benign tumors have excellent outcomes with wide local excision and rarely recur or metastasize. On the other hand, patients with large, malignant lesions and metastatic disease have dismally poor outcomes.

    What is the prognosis for SCC tongue? ›

    Prognosis for Oral Squamous Cell Carcinoma

    If carcinoma of the tongue is localized (no lymph node involvement), 5-year survival is > 75%. For localized carcinoma of the floor of the mouth, 5-year survival is 75%. Lymph node metastasis decreases survival rate by about half.

    Are granular cell tumors painful? ›

    Most often painless; can occasionally be mildly itchy or tender.

    Can a granular cell tumor become malignant? ›

    Most granular cell tumors are benign (not cancer), but some may be malignant (cancer) and spread quickly to nearby tissue. They usually occur in middle-aged adults. Also called Abrikossoff tumor.

    What is the survival rate for granulosa cell tumors? ›

    GCTs are considered to be tumors of low malignant potential. Approximately 90% of GCTs are at stage I at the time of diagnosis (see Staging for further detail). The 10-year survival rate for stage I tumors in adults is 90-96%. GCTs of more advanced stages are associated with 5- and 10-year survival rates of 33-44%.

    Is granulosa cell tumor fatal? ›

    Stage is the most important prognostic factor, with 10-year survivals of 84-95% for stage I tumors, decreasing to 50-65% for stage II disease, and to 17-33% for stages III and IV.

    What is the life expectancy of a person with GCT? ›

    Testicular germ-cell tumors (GCT) are highly curable. A multidisciplinary approach, including cisplatin-based chemotherapy has resulted in cure in the majority of patients with GCT. Thus, the life expectancy of survivors will extend to many decades post-diagnosis.

    Are tongue tumors ever benign? ›

    Benign mouth and tongue tumors usually occur alone and grow very slowly over a period of 2 to 6 years. Benign means they are not cancerous. They are unlikely to spread to other body parts. They can involve the lips, gums, roof or floor of the mouth, or tongue.

    What is the most common tongue tumor? ›

    What are the types of tongue cancer? The most common type of tongue cancer is squamous cell carcinoma (SCC). Squamous cells are the flat, skin like cells that cover the lining of the mouth, nose, larynx, thyroid and throat. Squamous cell carcinoma is the name for a cancer that starts in these cells.

    What tumor has the worst prognosis? ›

    Brain and pancreatic cancers have much lower median survival rates which have not improved as dramatically over the last forty years. Indeed, pancreatic cancer has one of the worst survival rates of all cancers. Small cell lung cancer has a five-year survival rate of 4% according to Cancer Centers of America's Website.

    Is a granulosa cell tumor benign or malignant? ›

    Are granulosa cell tumors benign or malignant? Granulosa cell tumors are usually malignant (cancerous). But most GCTs grow slowly. Most of the time, healthcare providers diagnose them in early stages when they have good treatment outcomes.

    What is the grading of granular cell tumors? ›

    Fanburg-Smith criteria: Necrosis, tumor cell spindling, vesicular nuclei with large nucleoli, > 2 mitoses/10 high power fields, high nuclear to cytoplasmic ratio and pleomorphism. 0: benign; 1 - 2: atypical; ≥ 3: malignant (Am J Surg Pathol 1998;22:779)

    What is the best treatment for squamous cell carcinoma of tongue? ›

    In general, early-stage tongue carcinoma (T1 or T2) can be treated successfully with single-modality therapy, namely surgery or radiation. However, when long-term morbidity of treatment, cost, and other factors are considered, surgery is recommended upfront.

    How are tongue tumors removed? ›

    This surgery is called a glossectomy. The surgeon removes the cancer and some of the healthy cells around it, called a margin. Removing the margin helps ensure that all the cancer cells are removed. How much of the tongue the surgeon removes depends on the size of the cancer.

    What is the treatment for granular cell? ›

    Treatment. The primary method for treatment is surgical, not medical. Radiation and chemotherapy are not needed for benign lesions and are not effective for malignant lesions.

    How do you treat precancerous cells on your tongue? ›

    Surgical Removal

    If you have moderate or severe dysplasia, which has a greater chance of becoming cancerous, doctors remove the lesion and a small margin of healthy tissue using a small scalpel or laser beam. They may use a local anesthetic to perform the surgery. You may return home the same day.

    References

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