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Lingual granular cell tumor
Lingual granular cell tumor D. E. KYRMIZAKIS, MD, DDS, A. A. NIKOLIDAKIS, MD, J. G. PANAYIOTIDES, MD, P. CHRISTODOULU, MD, and E. HELIDONIS, MD, FACS, Crete, Greece
A
62-year-old man with a history of moderate smoking and drinking presents with a slightly red, elevated, firm, asymptomatic lesion 1.5 cm in diameter, on the dorsal surface of his tongue (Fig 1). He had no history of illness or hospitalization. Results of the remaining examination of the oral cavity, pharynx, and larynx were normal. He had no cervical adenopathy. An incisional biopsy was performed. Histologic findings showed the lesion to be a granular cell tumor with pseudoepitheliomatous hyperplasia of the overlying squamous epithelium. A few days later, the tumor was excised with a CO2 laser with the patient under local anesthesia. The histopathology report of the surgical specimen confirmed the diagnosis as well as the need for complete excision of the lesion. DISCUSSION
The granular cell tumor (GCT), formerly known as granular cell myoblastoma, is an uncommon benign tumor of unknown cause. Abrikossoff first reported the GCT in 1926 as a “Myoblastenmyom” suggesting a skeletal muscle origin.1 Other investigators have also suggested an origin from skeletal muscle, macrophages, undifferentiated mesenchymal cells, and pericytes. However, these theories have been incompletely supported. More recently, the unique granular cells that make up the tumor are believed to be of neural (Schwann-cell) origin based predominantly on immunohistochemical studies. This theory is also debatable.2 The GCT appears in a range of patients from children to the elderly but usually involves individuals in their fifth to sixth decades of life. Lesions can occur both on skin and subcutaneous tissue, and occasionally some may be identified in lung, gastrointestinal track, and heart tissue. In the head and neck, the tongue is by far the most common location for GCT.
From the Departments of Otolaryngology (Drs Krymizakis, Nikolidakis, Christodoulu, and Helidonis) and Pathology (Dr Panayiotides), University Hospital of Heraklion. Reprint requests: D. E. Kyrmizakis, MD, DDS, 25 Papanastasiou St, Heraklion, Crete, Greece; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:422-3 Copyright © 2001 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/78/115665 doi:10.1067/mhn.2001.115665 422
Fig 1. Face view of the tongue shows a lingual granular cell tumor on the left side of the dorsum.
Typically, the lesions occur as small (less than 2 cm in diameter), well-circumscribed, solitary, painless, uninflamed, and nonulcerated nodules. The overlying epithelium is usually intact. Multiple lesions have occasionally been described.3 Pseudoepitheliomatous hyperplasia of the overlying oral epithelium is seen in about half the cases. This may be such a prominent feature that subjacent GCT are overlooked and a superficial biopsy from a such a lesion may be erroneously diagnosed as well-differentiated squamous cell carcinoma (SCC).3 Differential diagnosis would include, except for SCC, connective tissue lesions as neurofibroma and Schwannoma, minor salivary gland tumors, lymphoma, traumatic fibroma and in children a related lesion known as congenital gingival granular cell tumor (congenital epulis).3 Rarely encountered malignant GCT comprise approximately 3% of the entire group. The synchronous occurrence of SCC and GCT has also been reported.4
Otolaryngology– Head and Neck Surgery Volume 125 Number 4
Spontaneous regression has been noted but is apparently a rare event. Benign GCT are treated by complete surgical excision and have little chance of recurrence.3,4 REFERENCES 1. Lack EE, Worshan GF, Callihan MD, et al. Granular cell tumor: a clinicopathologic study of 110 patients, J Surg Oncol 1980;13:301-16.
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2. Papageorgiou S, Litt JZ, Pomeranz JR. Multiple granular cell myoblastomas in children. Arch Dermatol 1967;96:168-71. 3. Regezi JA, Sciubba JJ. Connective tissue lesions. In: Regezi JA, Sciubba JJ, editors. Oral pathology clinical-pathologic correlations, 3rd ed. Philadelphia: WB Saunders; 1999. p. 176-216. 4. Said-al-Naief N, Brandwein M, Lawson W, et al. Synchronous lingual granular cell tumor and squamous carcinoma: a case report and review of the literature. Arch Otolaryngol Head Neck Surg 1997;123:543-7.
A
62-year-old man with a history of moderate smoking and drinking presents with a slightly red, elevated, firm, asymptomatic lesion 1.5 cm in diameter, on the dorsal surface of his tongue (Fig 1). He had no history of illness or hospitalization. Results of the remaining examination of the oral cavity, pharynx, and larynx were normal. He had no cervical adenopathy. An incisional biopsy was performed. Histologic findings showed the lesion to be a granular cell tumor with pseudoepitheliomatous hyperplasia of the overlying squamous epithelium. A few days later, the tumor was excised with a CO2 laser with the patient under local anesthesia. The histopathology report of the surgical specimen confirmed the diagnosis as well as the need for complete excision of the lesion. DISCUSSION
The granular cell tumor (GCT), formerly known as granular cell myoblastoma, is an uncommon benign tumor of unknown cause. Abrikossoff first reported the GCT in 1926 as a “Myoblastenmyom” suggesting a skeletal muscle origin.1 Other investigators have also suggested an origin from skeletal muscle, macrophages, undifferentiated mesenchymal cells, and pericytes. However, these theories have been incompletely supported. More recently, the unique granular cells that make up the tumor are believed to be of neural (Schwann-cell) origin based predominantly on immunohistochemical studies. This theory is also debatable.2 The GCT appears in a range of patients from children to the elderly but usually involves individuals in their fifth to sixth decades of life. Lesions can occur both on skin and subcutaneous tissue, and occasionally some may be identified in lung, gastrointestinal track, and heart tissue. In the head and neck, the tongue is by far the most common location for GCT.
From the Departments of Otolaryngology (Drs Krymizakis, Nikolidakis, Christodoulu, and Helidonis) and Pathology (Dr Panayiotides), University Hospital of Heraklion. Reprint requests: D. E. Kyrmizakis, MD, DDS, 25 Papanastasiou St, Heraklion, Crete, Greece; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:422-3 Copyright © 2001 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/78/115665 doi:10.1067/mhn.2001.115665 422
Fig 1. Face view of the tongue shows a lingual granular cell tumor on the left side of the dorsum.
Typically, the lesions occur as small (less than 2 cm in diameter), well-circumscribed, solitary, painless, uninflamed, and nonulcerated nodules. The overlying epithelium is usually intact. Multiple lesions have occasionally been described.3 Pseudoepitheliomatous hyperplasia of the overlying oral epithelium is seen in about half the cases. This may be such a prominent feature that subjacent GCT are overlooked and a superficial biopsy from a such a lesion may be erroneously diagnosed as well-differentiated squamous cell carcinoma (SCC).3 Differential diagnosis would include, except for SCC, connective tissue lesions as neurofibroma and Schwannoma, minor salivary gland tumors, lymphoma, traumatic fibroma and in children a related lesion known as congenital gingival granular cell tumor (congenital epulis).3 Rarely encountered malignant GCT comprise approximately 3% of the entire group. The synchronous occurrence of SCC and GCT has also been reported.4
Otolaryngology– Head and Neck Surgery Volume 125 Number 4
Spontaneous regression has been noted but is apparently a rare event. Benign GCT are treated by complete surgical excision and have little chance of recurrence.3,4 REFERENCES 1. Lack EE, Worshan GF, Callihan MD, et al. Granular cell tumor: a clinicopathologic study of 110 patients, J Surg Oncol 1980;13:301-16.
KYRMIZAKIS et al
423
2. Papageorgiou S, Litt JZ, Pomeranz JR. Multiple granular cell myoblastomas in children. Arch Dermatol 1967;96:168-71. 3. Regezi JA, Sciubba JJ. Connective tissue lesions. In: Regezi JA, Sciubba JJ, editors. Oral pathology clinical-pathologic correlations, 3rd ed. Philadelphia: WB Saunders; 1999. p. 176-216. 4. Said-al-Naief N, Brandwein M, Lawson W, et al. Synchronous lingual granular cell tumor and squamous carcinoma: a case report and review of the literature. Arch Otolaryngol Head Neck Surg 1997;123:543-7.