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PATOL-148; No. of Pages 3 Rev Esp Patol. 2011;xxx(xx):xxxxxx

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Patologa
R E V I S TA E S PA O L A D E

www.elsevier.es/patologia

BRIEF REPORT

Adenoid cystic carcinoma of Bartholins glandA rare entity likely to be misdiagnosed

Rajnish Kumar , Mini Singhal, Rajiv Acharya, Sandip Kudesia, Nitin Chawla
Shri Guru Ram Rai Institute of Medical and Health Sciences, Patel Nagar, Dehradun, PIN-248001 Uttarakhand, India Received 10 May 2011; accepted 24 May 2011

KEYWORDS
Adenoid cystic carcinoma; Fine needle aspiration; Cytology; Bartholins gland

Summary Primary carcinomas of Bartholins gland are very rare. Adenoid cystic carcinoma, a variant of adenocarcinoma is even rarer than the other histological types at this site. This locally aggressive malignant neoplasm is usually found in postmenopausal females and occurs only very occasionally in women under forty years of age. A 35-year-old patient presenting with dyspareunia was admitted at our gynaecology outpatients department. The lesion had been previously misdiagnosed as an inammatory lesion. The diagnosis of adenoid cystic carcinoma was made on ne needle aspiration cytology which was conrmed on histopathological examination. We report the cyto- and histopathological features of this rare tumour occurring in a young patient together with a review of the relevant literature. 2011 SEAP y SEC. Published by Elsevier Espaa, S.L. All rights reserved.

PALABRAS CLAVE
Carcinoma adenoide qustico; Puncin aspiracin con aguja na; Glandula de Bartolino

Carcinoma adenoide quistica de la glandula de bartolino. una entidad poco comun sujeta a diagnosticos erroneos
Resumen Los carcinomas primarios de la glandula de Bartolino son muy infrecuentes. El carcinoma adenoide qustico, una variante de adenocarcinoma, es todava ms raro que otros tipos histolgicos en este rgano. Este tumor maligno es localmente agresivo, ocurre en mujeres postmenopusica y muy raramente se encuentra en mujeres menores de 40 aos. n Una mujer de 35 aos consult por dispareunia en el departamento ginecolgico del ambun latorio de nuestro Instituto. La lesin haba sido diagnosticada previamente como un proceso inamatorio. El diagnostic de carcinoma adenoide qustico fue realizado mediante puncin aspiracin con aguja na y conrmado histopatolgicamente. Comunicamos los rasgos citohistolgicos de este raro tumor, diagnosticado en una paciente joven. Realizamos una revisin de la literatura pertinente. 2011 SEAP y SEC. Publicado por Elsevier Espaa, S.L. Todos los derechos reservados.

Corresponding author. E-mail address: rajnishkumar1965@gmail.com (R. Kumar).

1699-8855/$ see front matter 2011 SEAP y SEC. Published by Elsevier Espaa, S.L. All rights reserved. doi:10.1016/j.patol.2011.05.003

Please cite this article in press as: Kumar R, et al. Adenoid cystic carcinoma of Bartholins glandA rare entity likely to be misdiagnosed. Rev Esp Patol. 2011. doi:10.1016/j.patol.2011.05.003

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Introduction
Adenoid cystic carcinoma (ACC) is a highly malignant, locally aggressive neoplasm which frequently shows perineural invasion. It occurs often in salivary and respiratory tract glands and less commonly in the breast, skin and head and neck regions. However, ACC of the Bartholins gland is exceedingly rare5 and the majority of cases are found in peri or postmenopausal women. There are very few reports of the diagnosis being made with ne needle aspiration cytology (FNAC). We present an extremely rare case of ACC of Bartholins gland in a young patient diagnosed with FNAC and subsequently conrmed by histopathology.

Case report
A 35-year-old female patient with a 2-year history of marked dyspareunia and occasional blood stained vaginal discharge was admitted at the gynaecology out patients department at our Institute. Her symptoms had appeared following the delivery of her last child, during which she underwent an episiotomy. An inammatory lesion was diagnosed clinically and antibiotic and anti-inammatory treatment prescribed, but with no relief. On per speculum, examination, a 2 2 cm extremely tender, rm swelling was seen at the vaginal introitus, extending from 4 oclock to 6 oclock position. No inguinal lymph nodes were palpable and routine haematology was normal. FNAC of the lesion was performed under sedation. ACC was diagnosed and subsequently, complete excision with wide surgical margins was performed.

Figure 1 Cellular smear showing tri-dimensional aggregates of small uniform cells having scanty cytoplasm and hyperchromatic nuclei around characteristic magenta coloured globules MGG stain, 100 (inset MGG stain 400).

Cytology
The aspirate was hemorrhagic but particulate. The May Grunwald Giemsa (MGG) stained smears were hypercellular and showed the characteristic magenta hyaline globules (acellular basement membrane material) surrounded by tightly cohesive cell clusters, 3D structures and a few discretely lying cells. The cells were small, monomorphic to mildly pleomorphic with small hyperchromatic ovoid nuclei (Fig. 1). The magenta globules stained weakly with Papanicolaou stain and were difcult to appreciate.

neoplasms and less than 1% of female genital tract tumours.1,6 Only 1015% of carcinomas occurring in Bartholins gland are ACC, the others are squamous cell carcinoma, adenocarcinoma adenosquamous carcinoma and transitional cell carcinoma.8 Extensive search of the literature revealed only 62 other cases of ACC of Bartholins gland.7 The mean age of diagnosis of ACC is 49 years with a range of 2580.2 The usual presenting signs and symptoms are nonspecic and may include pain, a burning sensation, dyspareunia bleeding, a palpable mass, etc.1 Clinically, the painful enlargement of the Bartholins gland may be confused with more common entities bartholinitis, cyst or abscess, as occurred in the present case.2

Histopathology ndings
The tumour cells were arranged predominantly in a cribriform pattern. The lumina in the cribriform spaces showed Periodic acid Schiff (PAS) positive basement membrane material. The tumour cells were small, uniform and basaloid, with scanty cytoplasm and hyperchromatic nuclei with little intervening stroma (Fig. 2).

Discussion
Bartholins gland in the labia minora was rst described by Caspar Bartholin, a Dutch anatomist, in 1677. Retention cysts and abscesses are the common lesions found in the Bartholins gland.4 Primary carcinomas of the gland are rare, accounting for only 0.17% of all vulvar

Figure 2 Adenois cystic carcinoma of Bartholins gland showing tubulo-cribriform arrangement of tumour cells around acellular spaces. H E stain, 100 (inset PAS stain).

Please cite this article in press as: Kumar R, et al. Adenoid cystic carcinoma of Bartholins glandA rare entity likely to be misdiagnosed. Rev Esp Patol. 2011. doi:10.1016/j.patol.2011.05.003

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Adenoid cystic carcinoma of Bartholins gland ACC of Bartholins gland is a slow-growing but locally aggressive neoplasm with characteristic perineural and lymphatic invasion. This explains the pronounced dyspareunia and the frequent recurrences associated with this lesion. A histopathological diagnosis of a primary tumour of Bartholins gland should only be made when (1) the tumour involves the area of the Bartholins gland; (2) there is apparent transition from normal to neoplastic elements; although this is not always seen, especially in advanced disease and (3) there is no evidence of a primary tumour elsewhere.2 Frable, Goplerud and others successfully diagnosed ACC with ne needle aspiration cytology.3,5 Characteristic ndings include magenta coloured hyaline globules on MGG stained smears surrounded by tightly cohesive, monomorphic cell clusters, 3D structures and a cribriform pattern. FNAC can also differentiate between ACC and other types of carcinomas and common lesions i.e. bartholinitis, cyst and abscess. Localized tumour may be treated with simple local excision or radical vulvectomy, with or without partial or complete regional lymphadenectomy.1,6 However, nodal metastases are seen in only 10% cases and lymph node dissection could be reserved for those patients felt to be at high risk for nodal involvement. The overall long-term survival is good.

Conict of Interest
The authors declare they have no conict of interest.

References
1. Cardosi RJ, Speights A, Fiorica JV, Grendys Jr EC, Hakam A, Hoffman MS. Bartholins gland carcinoma: a 15-year experience. Gynecol Oncol. 2001;82:24751. 2. DePasquale SE, McGuinness TB, Mangan CE, Husson M, Woodland MB. Adenoid cystic carcinoma of Bartholins gland: a review of the literature and report of a patient. Gynecol Oncol. 1996;61:1225. 3. Frable WJ, Goplerud DR. Adenoid cystic carcinoma of Bartholins gland diagnosis by aspiration biopsy. Acta Cytol. 1975;19: 1523. 4. Henryk S, Katarzyna S, Agnieszka H. The pathomorphology of Bartholins gland analysis of surgical data. Pol J Pathol. 2007;58:99103. 5. Jethwani D, Mohile N, Gupta VK. Cytodiagnosis of adenoid cystic carcinoma of Bartholins gland in vulva a case report. J Cytol. 2005;22, 00-00. 6. Nasu K, Kawano Y, Takai N, Kashima K, Miyakawa I. Adenoid cystic carcinoma of Bartholins gland: case report with review of the literature. Gynecol Obstet Invest. 2005;59: 548. 7. Sook YVY, Jeong WL, Woo SK, Kyoung LJ, Sun JL, Je HL, et al. Adenoid cystic carcinoma of the Bartholins gland: report of two cases and review of the literature. Gynecol Oncol. 2006;100:4225. 8. Wilkinson EJ, Teixeira MR. Epithelial tumours. In: Tavassoli FA, Devilee P, editors. Pathology and genetics of tumours of the breast and female genital organs. Lyon, France: IARC Press; World Health Organization Classication of Tumours; 2003. p. 31625.

Conclusion
Primary adenoid cystic carcinoma of Bartholins gland is a rare, painful, slow-growing but locally aggressive neoplasm. FNAC can be used successfully in its difcult differential diagnosis with common inammatory lesions and other neoplasms.

Please cite this article in press as: Kumar R, et al. Adenoid cystic carcinoma of Bartholins glandA rare entity likely to be misdiagnosed. Rev Esp Patol. 2011. doi:10.1016/j.patol.2011.05.003