| Abstract|| |
Granular cell tumors (GCTs) are rare and approximately half of the all lesions arise from head and neck, especially from the tongue. However, they are rarely seen in the vulva. They can occur in patients of any age, but peak age incidence is in the fourth to sixth decades. They generally occur as small, slow growing, and skin-colored nodule. GCTs are usually benign, but malignant cases were reported. Recurrence can be seen in benign tumors with clear margins, but rates increase with positive margins. The treatment is complete surgical excision. We report a case of benign GCT of the vulva. A 41-year-old patient presented with vulvar mass, and biopsy was consisted with GCT.
Keywords: Benign, granular cell, neoplasia, vulva
|How to cite this article:|
Sonmez FC, Koroglu N, Guler B, Arici DS. Vulvar granular cell tumor. Indian J Pathol Microbiol 2016;59:389-91
| Introduction|| |
Granular cell tumor (GCT) is rare mesenchymal neoplasm of neurogenic origin and was first described on tongue by Abrikossoff in 1926., Although GCT arises mostly from the head and neck region, particularly the tongue, it is not usual in the vulva where 5–15% of all GCTs occur., The most common presenting symptom is an asymptomatic mass in the vulva. The mean age at presentation is approximately 50 years, but also it has been reported in children. Although vulvar GCTs are mostly benign, malignant changes occur in 2% of the cases.,
In this paper, we presented a patient with benign GCT of the vulva.
| Case Report|| |
A 41-year-old patient presented with a mass on the right labium majus. On cutaneous examination, skin colored, nodular mass, measuring 1 cm × 1 cm × 0.5 cm in diameter was detected on vulva. There was no regional lymphadenopathy on physical examination. The patient underwent surgical excision of the vulvar mass. Grossly, 12 mm × 10 mm × 7 mm sized, firm, nodular subcutaneous mass was detected. The cut surface was well-defined, but not encapsulated and gray-yellowish colored with focal central hemorrhage. Microscopically, the tumor was composed of large cells with round to oval nuclei with small, inconspicuous nucleoli and large eosinophilic granular cytoplasm [Figure 1]. These cells were diffusely involving the connective tissues and arranging as sheets and nests in some areas. The cytoplasmic granules were periodic acid-Schiff (PAS) positive [Figure 2]. Immunohistochemically, tumoral cells were diffuse and strongly reactive for S-100 protein [Figure 3]. The overlying epithelium was slightly hyperplastic [Figure 4]. The diagnosis was “benign GCT” with all these findings. Features as nuclear pleomorphism, high nucleocytoplasmic ratio, increased mitotic activity, and necrosis suggesting malignancy were not detected in our case. However, microscopic examination revealed that tumor was extending to resection margin. The tumor was well circumscribed, grossly, but irregular, microscopically. We suggested re-excision to prevent recurrence, but the patient refused re-operation. She has been followed for seven months after excision, and no finding of recurrence has been detected. She has been informed about that the tumor may recur after excision so she should have routine physical examination regularly for any evidence of recurrence.
|Figure 3: Diffuse and strongly staining with S-100 protein, immunohistochemically|
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|Figure 4: Slightly hyperplastic epithelium and diffuse tumoral infiltration in subepithelial tissue (H and E, ×40)|
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| Discussion|| |
GCT was first described on tongue by Abrikossoff in 1926 as granular cell myoblastoma. GCTs are thought to be neurogenic origin as Schwann cells. Immunohistochemical features firmly identify that they are neural lesions, but it is sufficiently distinctive to be seperated from neurofibroma and schwannoma. Although rare in children, GCTs occur in patients of any age but are most common in fourth to sixth decades of life. GCTs are encountered in females two times more common than men., GCTs are located mostly in the dermis or subcutis, and less frequently in the submucosa, smooth muscle, striated muscle, and the internal organs. They occur primarily in the head and neck region, particularly in tongue, but may also occur in genital tract as presented in our case. Although ovary and cervix have been reported sporadically, genital tract GCTs are situated primarily in vulva, comprising 5–15% of all GCTs.
Clinically, these tumors present slowly growing, mobile, and painless mass that may be solitary or multiple. The size of lesion varies from <1 to 12 cm in diameter, but usually, the nodule is smaller than 3 cm., The tumor nodule of our case was 1, 2 cm in diameter. Histopathologically, these tumors have corresponding features with other more common sites. A proliferation of cells with small uniform nuclei and abundant granular cytoplasm diffusely involve tissues. The cells may be polygonal or elongated and have indistinct cell membranes. Overlying squamous epithelium may go pseudoepitheliomatous hyperplasia and may be misdiagnosed as well-differentiated squamous cell carcinoma., In our case, overlying epithelium was slightly hyperplastic but not as pseudoepitheliomatous hyperplasia. These lesions are not encapsulated, and granular cells extend into adjacent tissues. Granules stain positively with PAS., Cells contain numerous lysosomes. The tumor cells are immunoreactive for S-100 protein, neuron-specific enolase, laminin, and CD 68.,,
GCTs rarely recur, even after conservative treatment. Positive deep margin was detected on microscopic examination of our case. We suggested re-excision because of potential local aggressive behavior of tumor. Patient preferred to be followed unless there was a recurrence. No recurrence has been detected after 7 months of follow-up in the patient. The majority (98%) of GCTs are benign. Malign GCTs constitute fewer than 2% of all GCTs. Some histological features are associated with increased risk of metastasis. These features are necrosis, increased mitotic activity (>2 mitosis/10 HPF), spindling, vesicular nuclei with prominent nucleoli, high nucleocytoplasmic ratio, and pleomorphism. Tumors with three or more of these are considered malignant. These features suggesting malignancy were not detected in our case. A case of malignant GCT of the vulva in a 17-year-old patient was reported. In addition, a case of a 28-year-old patient who presented with benign vulvar GCT, and malignant transformation after five months was reported in literature. Benign cases with development of local recurrence and inguinal node metastasis who had histopathologically benign vulvar GCTs were reported in literature. Complete surgical excision is the treatment of choice in benign tumors. Local excision has to be quite wide because of the common occurrence of groups of tumor cells beyond the apparent macroscopic limits of growth.
| Conclusion|| |
GCTs are uncommon, neurogenic soft tissue tumors with mostly benign behavior. We present a case of vulvar GCT because of the rarity of this location and emphasize its histopathological and clinical features.
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There are no conflicts of interest.
| References|| |
Bames L, Eveson JW, Reichart P, Sidransky D. World Health Organization, Classification of Tumours, Pathology and Genetics Head and Neck Tumours. Switzerland, Lyon: IARC Press; 2005. p. 185.
Abrikossoff A. Über myome, ausgehend von der quergestreiften willkürlichen muskulatur. Virchows Arch 1926;260:215-33.
Crum CP, Nucci MR, Lee KL. Diagnostic Gynecologic an Obstetric Pathology. 2nd
ed. Philadelphia: Elsevier Saunders; 2011. p. 181-2.
Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. 5th
ed. China: Mosby Elsevier; 2008. p. 878-87.
Tavassoli FA, Devilee P. World Health Organization, Classification of Tumours, Tumours of the Breast and Female Genital Organs. Lyon, France: IARC Press; 2003. p. 330.
Ramos PC, Kapp DS, Longacre TA, Teng NN. Malignant granular cell tumor of the vulva in a 17-year-old: Case report and literature review. Int J Gynecol Cancer 2000;10:429-34.
Tawfiq N, Sabri S, Saiss K, Bouchbika Z, Benchekroun N, Jouhadi H, et al.
Granular cell tumor: Report of a complicated vulvar localization of pulmonary metastases. Cancer Radiother 2013;17:671-4.
Fox H, Wells M. Haines and Taylor Obstetrical and Gynecological Pathology. 5th
ed. London: Churchill Livingstone; 2002. p. 122-3.
Dr. Fatma Cavide Sonmez
Department of Pathology, Faculty of Medicine, Bezmialem Vakif University, Istanbul
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]