Journal of Cytology
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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 25  |  Issue : 3  |  Page : 108-110
Vulval schwannoma: A cytological diagnosis


Department of Pathology, Sri Devaraj Urs Medcal College, Sri Devaraj Urs University, Tamaka, Kolar, India

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   Abstract 

Schwannomas are the most common peripheral nerve sheath tumors, the frequent sites of involvement being the head and neck, the flexor aspect of the limbs, especially near the elbow, wrist, knee, and trunk. Involvement of the female genital tract is extremely rare. We present one such rare case of a solitary vulval schwannoma which was diagnosed by fine needle aspiration cytology in a 48 year-old female, and confirmed by histopathological examination.

Keywords: Aspiration cytology; schwannoma; vulva.

How to cite this article:
Das S, Kalyani R, Harendra Kumar M L. Vulval schwannoma: A cytological diagnosis. J Cytol 2008;25:108-10

How to cite this URL:
Das S, Kalyani R, Harendra Kumar M L. Vulval schwannoma: A cytological diagnosis. J Cytol [serial online] 2008 [cited 2019 Aug 21];25:108-10. Available from: http://www.jcytol.org/text.asp?2008/25/3/108/44048



   Introduction Top


Causing 800 deaths annually in the US, vulval cancer represents 3-4% of the malignancies of the female genital tract. [1] Squamous cell carcinomas accounts for more than 90% of the cases. Mesenchymal tumors such as fibrosarcomas, liposarcomas, angiosarcomas, and schwannomas are extremely rare, accounting for 1-2% of all vulval lesions. [1]


   Case Report Top


A 48 year-old female presented with a vulval swelling of two years' duration that had been gradually increasing in size. There was no history of bleeding per vagina or any urinary or bowel symptoms. The patient was para three with her last childbirth having occurred 19 years back. Past and family history was noncontributory. On general examination, the patient was found to be of average build and no clinical features indicative of neurofibromatosis (NF1 and NF2) were noted. Per vagina examination revealed a swelling which was firm in consistency, immobile, nontender, and arising from the labia majora. The cervix was healthy, the uterus was of normal parous size, and the fornices were free. Results of routine investigations were within normal limits and a clinical diagnosis of leiomyoma was made. Fine needle aspiration cytology (FNAC) was performed using a 23 gauge needle. Both Papanicolaou and May-Grünwald-Geimsa stained smears were examined. Cytology smears showed satisfactory cell yields with benign spindle cells showing palisading nuclei with pointed ends in a fibrillary background, suggestive of a spindle cell tumor, probably a schwannoma [Figure 1].

The lesion was excised and gross examination revealed the excised mass to be well encapsulated, measuring 4 x 3 x 2 cm in size. The cut surface was grayish white with focal myxoid areas [[Figure 2], inset]. Multiple hematoxylin and eosin (H and E) stained sections were examined. The slides showed characteristic features of a schwannoma with thin, wavy nuclei, fibrillary cytoplasm, and nuclear palisading at places [Figure 2]. A final diagnosis of a schwannoma was made; immunohistochemical stains showed positivity for S-100.

The patient had an uneventful postoperative recovery and is being regularly followed up in the outpatient department without any complications.


   Discussion Top


The female genital tract is a common site for epithelial lesions including malignancies. However, mesenchymal tumors such as liposarcomas, rhabdomyosarcomas, neurofibrosarcomas, and malignant schwannomas constitute just 1-2% of all vulval malignancies. [1]

Vulval lesions are uncommon and unpleasant but potentially curable even in elderly females. Schwannomas can occur at all ages but occur most commonly in the age group of 20-40 years with female predominance. They arise mostly from the cranial, spinal, and the peripheral nerves involving the face, neck, and the scalp. Sites such as the tongue, larynx, palate, and female genital organs including the vulva are involved in 1-2% of the cases. [1] In a population-based study of schwannomas, 90% were observed to be sporadic, 3% occurred in patients with neurofibromatosis (NF-2), 2% occurred in association with schwannomatosis, and 5% in association with multiple meningiomas in patients without NF2. [1] Rarely do schwannomas occur in patients with NF-1. [1]

Cytomorphological features of FNA smears of schwannomas have been described in literature. These include the presence of spindle cells with fibrillary cytoplasm, fishhook nuclei, Verocay bodies, and pseudoinclusions in the nuclei. However, several pitfalls for the diagnosis of neural lesions may be seen in the cytological smears, [2] including the varied cytomorphological appearance in the various subtypes of schwannoma along with degenerative changes and the sparsely cellular aspirate. [2] In two large series of FNAC of schwannoma, whereas Dahl et al . [3] have not addressed the sensitivity of FNAC in the diagnosis of schwannomas, Renick et al . [4] observed that inadequate cell yield/paucicellularity are the most common findings when these tumors are aspirated. Ancient schwannoma, another diagnostic pitfall, was initially mentioned by Ackerman and Taylor [5] as a degenerative change occurring in a long-standing schwannoma and is characterized by nuclear hyperchromasia, mild nuclear pleomorphism with multinucleation, stromal edema, fibrosis, and xanthomatous changes leading to a misdiagnosis of malignancy in the aspirates.

Sometimes, due to sampling error, only the epithelial component may be aspirated on FNAC, which can lead to an erroneous diagnosis. On reviewing the literature, we came across a single case of epithelioid malignant schwannoma reported with aspirated material. [6] However, we made a diagnosis of spindle cell tumor suggestive of schwannoma on the aspirated material and later substantiated it by histopathological examination.

Only a few cases of vulval schwannoma have been reported. Byran et al . [7] reported one such case. Most of the vulval schwannomas arise from the labia whereas Migliorini and Amato [8] reported a case located in the clitoris. Woodruff et al . [9] have reported a case of plexiform vulval schwannoma in a 26 year-old female patient. Terada et al . [10] have reported two cases of malignant vulval schwannoma arising from the preexisting case of vulval schwannoma in a 29 year-old female along with immunohistochemical confirmation showing positivity for the S-100 protein. Santos et al . [11] have described a case of plexiform vulval schwannoma in a five year-old girl.

A review of literature did not reveal any case of vulval schwannoma diagnosed after cytological examination. Here, we have highlighted the cytological features in the diagnosis of vulval schwannoma and have later confirmed the diagnosis by histopathological examination.


   Conclusions Top


Although vulval schwannoma is a rare tumor, it should be considered in the differential diagnosis of vulval lesions as a proper knowledge of this lesion will help us to avoid several diagnostic pitfalls. Our case report shows a relatively common lesion of schwannoma in a very unusual site involving the vulva along with the relevant cytomorphological features.

 
   References Top

1.Bigner DD, McLendon ER, Bruner MJ. Tumors of the cranial, spinal and peripheral nerve sheaths. In: Russell SD, Rubinstein JL, editors. Pathology of tumors of the nervous system. 6th ed. London: Edward Arnold; 1998. p. 141-93.   Back to cited text no. 1    
2.Henke CA, Solomao DR, Hughes JH. Cellular schwannoma mimics a sarcoma: An example of a potential pitfall in aspiration cytodiagnosis. Diagn Cytopathol 1999;20:312-6.  Back to cited text no. 2    
3.Dahl I, Hagmar B, Idvall I. Benign solitary neurilemomma (Schwannoma): A correlative cytological and histological study of 28 cases. Acta Pathol Microbiol Immunol Scand 1984;92:91-101.  Back to cited text no. 3    
4.Resnick JM, Fanning CV, Caraway NP, Varma DG, Johnson M. Percutaneous needle biopsy diagnosis of benign neurogenic neoplasms. Diagn Cytopathol 1997;16:17-25.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Ackerman LV, Taylor FH. Neurogenic tumors within the thorax: A clinicopathological evaluation of forty-eight cases. Cancer 1951;4:669-91.  Back to cited text no. 5  [PUBMED]  
6.Dodd LG, Scully S, Layfield LJ. Fine-needle aspiration of epithelioid malignant peripheral nerve sheath tumor (epithelioid malignant schwannoma). Diagn Cytopathol 1997;17:200-4.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Bryan WE. Neurilemmoma of the vulva. J Obstet Gynecol Br Emp 1955;62:949-50.  Back to cited text no. 7    
8.Migliorini A, Amato G. Neoplastic pathology of the vulva: unusual case of neurinoma of the clitoris. Minerva Ginecol 1978;39:543-5.  Back to cited text no. 8    
9.Woodruff JM, Marshall ML, Godwin TA, Funkhouser JW, Thompson NJ, Erlandson RA. Plexiform (multinodular) schwannoma: A tumor simulating the plexiform neurofibroma. Am J Surg Pathol 1983;7:691-7.  Back to cited text no. 9  [PUBMED]  
10.Terada KY, Schmidt RW, Roberts JA. Malignant schwannoma of the vulva: A case report. J Reprod Med 1988;33:969-72.  Back to cited text no. 10  [PUBMED]  
11.Santos LD, Currie BG, Killingsworth MC. Case report: Plexiform schwannoma of the vulva. Pathology 2001;33:526-31.  Back to cited text no. 11  [PUBMED]  

Top
Correspondence Address:
Subhashish Das
C/o Dr. Kalyani. R. MD, H. No.127/13, "Sri Ganesh", 4th Main, 4th Cross, P.C. Extension, Kolar - 563 101, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9371.44048

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    Figures

  [Figure 1], [Figure 2]

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