Int. J. Oral Surg. 1979: 8: 128-132 (Key words: myobfastoma, granular cell; surgery, oral)
Granular cell tumor of the tongue G. BEEMSTER, W. A. M. VAN DER KWAST, J. SCHOEN AND 1. VAN DER WAAL Departments of Oral Surgery and Oral Pathology, Free University, Amsterdam Department of Oral Surgery, Centraal Ziekenhuis, Alkmaar, The Netherlands
The granular cell tumor has been and still is a debatable lesion in several aspects. The etiology and pathogenesis are still unknown. Granular cell tumors may occur everywhere in the body, but the oral cavity is a favorite location. The majority of the oral lesions are found in the dorsum and the borders of the anterior two-thirds of the tongue. Seven patients with a granular cell tumor of the tongue are described. The clinical and microscopic findings have been discussed. In all cases an excisional biopsy had been aimed at. In spite of incomplete excision in six out of the seven cases, local recurrences were noticed in only one patient. In one patient "metastatic" occurrence of granular cell tumors was noticed. .uJSTR.ACT -
(Received for publication 1 February, accepted 5 May 1978)
The granular cell tumor has been described as one of the most unusual and mysterious lesions in the realm of human pathology14. The disease is known under many synonyms such as Abrikossoff tumor!, granular cell myoblastoma, myoblastic mama and granular rhabdomyoma. The term granular cell tumor has been used by the World Health Organization 2D • According to SLAVKIN, GLAZER & CATONE26 about 2 % of the granular cell tumors have to be considered malignant. By some authors those tumors are called alveolar soft part sarcomas, while others prefer the term malignant granular cell tumor, considering the alveolar soft part sarcoma a separate entity l0,2D. For many years the granular cell tumor was considered to be a neoplasm of myo-
genic ongm. Many authors, however, have seriously questioned the myogenic origin and prefer a neurogenic one instead, reflected in a term such as granular cell Schwannoma. Others have ascribed a histiocytic nature to the granular cells15• Also a salivary gland origin was proposed in the pastl 2 • Shear25 suggested that the lesion might represent a metabolic change rather than a neoplastic one, affecting muscle cells as well as fibroblasts. Some authors believe that the granular cell tumor is the result of trauma 3,31.
Material and methods Of a total of about 35,000 patients referred to
one department of oral surgery, three cases of granular cell tumor were recorded, all located in the tongue. Four other patients were re-
0300--9785/79/020128-05$02.50/0 © 1979 Munksgaard, Copenhagen
GRANULAR CELL TUMOR
Fig. 1. Granular cell tumor at the anterior
right border of the tongue in a 45-year-old man. The lesion is slightly elevated.
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the diagnosis granular cell tumor had been considered the most likely diagnosis. In all other cases some kind of a fibrotic lesion was first thought of. All the cases were reported microscopically as benign granular cell tumor (Fig. 2). Cellular and nuclear pleomorphism were absent, as was any mitotic activity. There were no signs of encapsulation. In none of our cases was a distinct border observed between the granular cells and the overlying epithelium. Pseudoepitheliomatous changes were noticed in three cases (Fig. 3). In not one single case was radical excision established. In only one patient, no, 3, was an immediate attempt made to remove the remaining tumor cells. The follow-up period varied from 1 to 12 years. In patient no,7, two local recurrences were excised within a year. Both times, the microscopic aspects had not changed and did not l'eveal any signs' of malignancy. Six years after the tongue lesion in patient no. 4 had
ferred to us for advice or follow-up care after the lesion of the tongue had been treated. All the patients were white. Duration of symptoms varied from some months up to 1 year. They had all noticed a slow growing nodule in their tongue. The history did not reveal any severe trauma. All the patients were otherwise healthy. The clinical appearance was recorded as a pink or yellowish, slightly elevated, not freely movable nodule in the tongue, the surface being intact (Fig. 1). None of the lesions measured more than 1 em in their largest diameter. The age, sex and exa.ct location of the lesions are recorded in Table 1. In all the cases an excisional biopsy was performed. All specimens were routinely prepared into H&E sections, except in one case, no. 5, in which tissue was prepared for electron microscopic examination as well. The last patient was the only one in which preoperatively Table 1. Granular cell tumor of the tongue Patient 1 2
3
4 5 6 7
Sex
Age
F
29 35 12
F
M M M F
F
24 45
26 26
Location right border right border dorsum, left of the midline right border right border right border right border
Fig. 2. Granular cell tumor of the tongue in
direct contact with the overlying epithelium, Note the pseudo-epitheliomatous hyperplasia (PER). Tn this magnification the granules of the tumor cells (GTC) are hardly visible (H&E X 130),
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BEEMSTER, VAN DER KWAST, SCHOEN AND VAN DER WAAL
Fig. 3. High-power view showing the granular
cells (GTe), as well as normal muscular structures (M), Cellular or nuclear pleomorphism is absent, as are mitotic figures. There is a somewhat gradual transition between tumor cells and muscles (between arrows). (H&E X 330).
been excised, two new lesions developed at the same time in the cheek mucosa and on the dorsum of one of the feet. Both lesions were excised; the one on the foot, however, appeared again 4 years later. In the remaining five patients no signs of local recurrence or "metastatic" behavior were observed.
Discussion Most authors credit ABRIKOSSOFF with reporting the first cases of granular cell tumors1 • However, WEBER had published on this sllbject in 18543 °, MrcHEAu, GERARD-MARCHANT & CACHIN described a series of 12 granular cell tumors and 1,931 squamous cell carcinomas of the tongue1a. In our material we have identified seven granular cell tumors and 40 squamous cell carcinomas of the tongue. Those different ratios are not easily explained since the incidence of oral cancer in France com-
pared to the Dutch figure is only slightly different. There must be, therefore, a different explanation, probably of some statistical nature. The sex distribution and the age of our patients are in accordance with the findings in 325 granular cell tumors reviewed by Peterson23 • Three cases have been described 6 occurring in children of 4, 5 and 10 years, respectively. A congenital granular cell tumor of the tongue was published in 1897 22 , In 1975 two cases were reported8 of congenital granular cell tumors of the tongue associated with a granular cell tumor of the anterior maxilla and the mandible, respectively. Those cases question the almost uniform agreement in the literature to consider the congenital granular cell tumor of the alveolar ridge a separate lesion that should be excluded from granular cell tumors in other lesions. Almost all granular cell tumors of the tongue that have been reported in the literature measured less than 2 cm. This was also true in our patients. We have encountered only one report of an exceptionally large granular cell tumor of the tongue7 • Ulceration of the overlying epithelium is rare lD , The dorsum and border of the tongue are common locations, in contrast to the ventral surface13 • LIEBERMAN & SIMRlNG16 reported a case of a man who had five nodules in his tongue at the same time, A case of multiple lesions was reported ~in the tongue tt• BERNSTEIN, MURNANE & ~h LONEy5 reported a case of granular cell :tumol' of the tongue associated with multiple lesions of the skin. A multiple occurrence of granular cell tumor is found in about 2 % of all cases 2 . More recently figures from 7-10 % and even higher have been mentioned 2D , It remains obscure whether there is multifocality or metastatic spread. In malignant metastatic granular cell tumor
GRANULAR CELL TUMOR
the metastatic sites are, in descending order, the regional lymph nodes, the lungs, the liver and the hones; in multiple lesions of benign granular cell tumors, however, the aforementioned areas are usually not involved, suggesting a multifocal rather than a metastatic behavior 14 . The spontaneous regression, especially in multiple lesions 4,21, remains another unsolved aspect, that the onset of secondary tumors was preceded by handling a primary growth 20 • In patient no. 4, the lesions appeared one after another, suiting the concept of MOSCOVIC & AZAR 20 of a selected implantation or action of an agent released from the tumor, The microscopic study of our material using electron microscopy in one case only did not change in any way the debatability of the origin of the granular cells. As mentioned by others and also by one of us in a thesis on carcinoma of the tongue28 such epithelial changes can easily be misdiagnosed as squamous cell carcinoma. Histochemical reactions, as reported by others 27 , and also applied in our cases, are not of significance in making a diagnosis of benign granular cell tumor. Electron microscopic examination may help to differentiate cases of alveolar soft part sarcoma, carotid body tumor, malignant melanoma and granular cell tumor9 • Since we have done electron microscopic examination in one case only, we do not feel qualified to support or attack the aforementioned statement. Interestingly, a malignant granular cell tumor rarely occurs in the oral cavity. In a series of 53 of such cases, only one was located intraorally17. In spite of proven incomplete excision in six of our seven cases, local recurrences were noticed in only one patient, no. 7, This is more or less in accordance with the findings of others. Although wide local excision is the treatment of choice, it seems justifiable to follow the patient closely for a year or so, when complete excision has
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not been obtained at the first intervention. There seems to be no place for postoperative radiotherapy. The few cases of radiotherapy of granular cell tumor that were reported have not been successfup,24. Acknowledgment - We thank Dr. D. H. J. WACHTERS, dermatologist and Dr. P. R. J. PINXTER, general surgeon, both practicing in Amsterdam, for the permission (0 publish cases 3 and 4.
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22. PENDL, F.: Ober ein congenitales Rhabdomyom der Zunge. Ztsch. Heilk. 1897: 18: 457-468. 23. PETERSON, L. l.: Granular cell tumor. Oral Surg. 1974: 37: 728-735. 24. ROTHCHILD, T. P. E. & CRARY, H.: Granular cell myoblastoma. Ann. Surg. 1953: 137: 530. 25. SHEAR, M.: The histogenesis of the socalled "granular cell myoblastoma". J. Pathai. Bact. 1960: 80: 225-228. 26. SLAVKIN, D. M., GLAZER, R. 1. & CATON"', G. A.: Bilateral granular cell myoblastoma of the lip: report of case. J. Oral Surg. 1973: 31: 848-851. 27. TOTO, P. D. & RESTARSKl, J.: Histogenesis of the granular cell myoblastoma. Oral Surg. 1967: 24: 384-387. 28. WAAL VAN DER, 1.: Carcinoma of the tongue. Thesis, Amsterdam, 1973. 29. WAHl, P. N.: Histological typing of oral and oropharyngeal tumors. World Health Organization, Geneva, 1971. 30. WEBER, C. 0.: Anatomische Untersuchungen einer hypertropischen Zunge nebst Bemerkungen tiber die Neubildung quergestreiften Muskelfasern. Arch. Path. Anat. 1854: 7: 115-125. 31. WILLIS, R. A.: Pathology oj tumors. Butterworth and Co. Inc., Washington 1960.
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r.
van der Waal Hospital of the Free University Depts. of Oral Surgery and Oral Pathology de Boelelaan 1117 1081 HV Amsterdam Holland