Granular cell (Abrikossoff) tumor in the head and neck: a series of 5 cases.
We evaluated a series of 5 patients--3 men and 2 women, aged 39 to 70 years (mean: 54.4)--with a granular cell tumor (GCT) of the head and neck in an effort to better define the clinical presentation, imaging characteristics, and surgical management of this type of tumor. In all cases, the diagnosis was established by pathologic analysis. There were 2 cases of laryngeal GCT and 1 case each of GCT arising in the nostril, hypopharynx, and the tongue base. The clinical findings were variable, depending on the location and extent of each lesion. Four of these patients underwent endoscopic examination, and in 2 cases computed tomography was performed. Treatment included wide surgical excision of the lesion in all cases. Otolaryngologists should be familiar with this unusual tumor. Although an accurate preoperative diagnosis is extremely difficult to make, appropriate therapeutic intervention is associated with a cure rate that is quite high.
Granular cell tumor (GCT), also called Abrikossoff tumor, is an unusual, slowly growing neoplasm. (1) It is almost always benign. (2,3) GCT can occur anywhere in the body; half of these tumors arise in the head and neck region, especially in the tongue. (4,5) The origin of this tumor has been debated in the literature. Most authors believe that the tumor is of neural origin. (6,7)
GCT occurs most frequently in the third to sixth decades of life. (8) It usually arises as a solitary tumor; multiple tumors occur in approximately 7 to 16% of cases. (9,10) The only examination that can confirm the clinical diagnosis is a histologic evaluation. The appropriate treatment is excision of the lesion.
In this article, we describe 5 cases of GCT involving the head and neck, and we discuss their clinical presentation, diagnosis, and surgical treatment. In 2 of our patients, the tumor's site of origin was extremely rare--the nasal cavity and the hypopharynx.
We reviewed the cases of 5 patients--3 men and 2 women, aged 39 to 70 years (mean: 54.4)--with GCT whose records were contained in the archives of the ENT Department at the General Hospital of Volos "Achillopoulio" in Volos, Greece. Among the 5 cases were 2 cases of laryngeal GCT and 1 case each of GCT arising in the nostril, hypopharynx, and the tongue base (table).
The clinical presentation of the lesions varied. Of the 2 lesions in the larynx, 1 manifested as gradually increasing hoarseness (patient 1) and 1 as periodic dyspnea and hoarseness (patient 2). The GCT of the nostril was asymptomatic (patient 3). The patient with hypopharyngeal GCT (patient 4) and the patient with lingual GCT (patient 5) had similar symptoms; they both complained of dysphagia and a foreign-body sensation in the throat.
In the 2 cases of laryngeal GCT, endoscopic examination was performed. In patient 1, a small, round, subepithelial mass was found at the anterior portion of the right vocal fold. In patient 2, the lesion was quite large, and it had arisen from the right false vocal fold (figure 1). In the case of GCT of the nostril (patient 3), the clinical examination revealed thickness of the skin of the nasal vestibule and the external surface of the nasal ala (figure 2).
In patient 4, endoscopic examination revealed a submucosal mass bulging from the posterior wall of the hypopharynx and pushing the right arytenoid cartilage forward without impairing vocal fold mobility (figure 3, A). Computed tomography (CT) demonstrated a solid, homogeneous submucosal lesion that had a maximum diameter of 22.4 mm (figure 3, B). The mass displayed well-defined margins and poor enhancement after contrast administration. Finally, in patient 5, endoscopy located a round subepithelial lesion in the right vallecula (figure 4); the CT findings were similar to those of patient 4.
In the case of GCT of the nostril, a small biopsy of the lesion confirmed the diagnosis preoperatively. In the rest of the cases, we proceeded directly to total excision of the lesion with a margin of normal surrounding tissue. The choice of surgical approach depended on the location of the lesion. In the 2 patients with laryngeal GCT, the tumor was resected under microlaryngoscopy with a CO, laser. In the case involving the nostril, a wide excision was performed; repair of the surgical defect was performed at the same time with a local nasolabial reverse flap. In the other 2 patients, a laryngoscope was used to obtain good exposure of the lesion, and an en bloc surgical excision was carried out with the use of the laser. In all cases, the diagnosis was based on histologic examination (figure 5).
A complete ENT examination and esophagogastric endoscopy were performed for all our patients to rule out the presence of a concomitant lesion. During a follow-up that ranged from 1 to 4 years, no patient experienced a recurrence.
The clinical presentation of GCT varies according to the location and extent of the lesion. Patients with laryngeal GCT present with hoarseness, dysphagia, cough and, less frequently, dyspnea and stridor. Symptoms of pharyngeal GCT include dysphagia, mild dysphonia, dyspnea, and a foreign-body sensation in the throat.
Macroscopically, GCTs appear as firm, nodular lesions covered with mucosa, which is usually intact. Typically these masses are small (<2 cm) and well circumscribed but not encapsulated. (8) In 2 of our cases (patient 2 and patient 4), the lesion was unusually large. Some GCTs have ill-defined margins and show a locally infiltrative pattern of growth so that enucleation from adjacent tissues is difficult. Moreover, in 50 to 65% of cases, a covering of squamous, pseudoepitheliomatous hyperplasia overlies the epithelium. (8)
On CT, GCTs are solid and homogeneously enhancing. (11) On magnetic resonance imaging (MRI), radiologic characteristics include (1) hypointensity on T1-weighted imaging with homogeneous contrast enhancement and (2) a heterogeneously increased signal on T2-weighted imaging. (11) However, radiologic imaging does not display any specific typical finding that is suggestive of the diagnosis of GCT. As a result, establishing the diagnosis of GCT preoperatively is extremely difficult.
The diagnosis of GCT is based on the histopathologic examination. Microscopically, the tumor cells are polygonal and arranged in sheets and strands. The defining histologic characteristic is the abundant, eosinophilic cytoplasm that is filled with periodic acid-Schiff-positive and Sudan black B-positive granules. (12)
In general, the appropriate treatment for benign GCT is surgical excision. The extent of surgery ranges from a simple local enucleation to excision with a wide margin; most authors favor wide excision. (13,14) However, even with adequate resection, there is a 2 to 8% recurrence rate, usually at the primary site. (15) In our series, all patients underwent wide excision of the lesion in order to reduce the risk of recurrence. In most of our cases, the C[O.sub.2] laser was used. No recurrences were noted during follow-up. Radiation therapy and chemotherapy are not recommended for GCT. (16)
After excision, regular long-term follow-up is required to look for recurrences. In our series, the first follow-up examination was performed 6 months postoperatively. The timing of the follow-up was based on the fact that GCTs grow slowly and evolve on average between 6 and 7 months before patients perceive a sensible discomfort and seek medical advice. (17)
In conclusion, although GCTs are rare and typically benign tumors, otolaryngologists must be aware of the need to make an accurate diagnosis and to provide appropriate treatment. Surgical excision with free margins is highly recommended to minimize the risk of recurrence. Careful follow-up is necessary for early detection should a recurrence arise.
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Petros Koltsidopoulos, MD, PhD; Konstantinos Chaidas, MD; Paschalis Chlopsidis, MD; Charalambos Skoulakis, MD, PhD
From the ENT Department, General Hospital of Volos "Achillopoulio," Volos, Greece (Dr. Koltsidopoulos); the ENT Department, General Hospital of Athens "G. Gennimatas," Athens, Greece (Dr. Chaidas); the ENT Department, Augsburg General Hospital, Augsburg, Germany (Dr. Chlopsidis); and the ENT Department, University Hospital of Larissa, Larissa, Greece (Dr. Skoulakis). The cases described in this article occurred at General Hospital of Volos "Achillopoulio."
Corresponding author: Dr. Petros Koltsidopoulos, ENT Department, General Hospital of Volos "Achillopoulio," Polimeri 134, 38222 Volos, Greece. E-mail: firstname.lastname@example.org
Table. Case summaries Pt. Age/sex Location Presentation Follow-up 1 58/M Larynx Hoarseness 2 yr 2 70/F Larynx Periodic dyspnea, 1 yr hoarseness 3 39/M Nostril Asymptomatic mass 4 yr 4 46/F Flypopharynx Dysphagia, 2 yr foreign-body sensation 5 59/M Tongue Dysphagia, 1 yr foreign-body sensation
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Koltsidopoulos, Petros; Chaidas, Konstantinos; Chlopsidis, Paschalis; Skoulakis, Charalambos|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Case study|
|Date:||Jan 1, 2016|
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