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Adenoid cystic carcinoma of Bartholin's gland—A rare entity likely to be misdiagnosed
Rev Esp Patol. 2011;44(4):213---215
Patología R E V I S TA
E S PA Ñ O L A
D E
www.elsevier.es/patologia
BRIEF REPORT
Adenoid cystic carcinoma of Bartholin’s gland----A 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 Available online 22 July 2011
KEYWORDS Adenoid cystic carcinoma; Fine needle aspiration; Cytology; Bartholin’s gland
PALABRAS CLAVE Carcinoma adenoide quístico; Punción aspiración con aguja fina; Glandula de Bartolino
∗
Summary Primary carcinomas of Bartholin’s 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 inflammatory lesion. The diagnosis of adenoid cystic carcinoma was made on fine needle aspiration cytology which was confirmed 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 España, S.L. All rights reserved.
Carcinoma adenoide quístico de la glándula de Bartolino. Una entidad poco común sujeta a diagnósticos erróneos Resumen Los carcinomas primarios de la glandula de Bartolino son muy infrecuentes. El carcinoma adenoide quístico, una variante de adenocarcinoma, es todavía más raro que otros tipos histológicos en este órgano. Este tumor maligno es localmente agresivo, ocurre en mujeres postmenopáusica y muy raramente se encuentra en mujeres menores de 40 a˜ nos. Una mujer de 35 a˜ nos consultó por dispareunia en el departamento ginecológico del ambulatorio de nuestro Instituto. La lesión había sido diagnosticada previamente como un proceso inflamatorio. El diagnosticó de carcinoma adenoide quístico fue realizado mediante punción aspiración con aguja fina y confirmado histopatológicamente. Comunicamos los rasgos citohistológicos de este raro tumor, diagnosticado en una paciente joven. Realizamos una revisión de la literatura pertinente. © 2011 SEAP y SEC. Publicado por Elsevier España, S.L. Todos los derechos reservados.
Corresponding author. E-mail address: [email protected] (R. Kumar).
1699-8855/$ – see front matter © 2011 SEAP y SEC. Published by Elsevier España, S.L. All rights reserved. doi:10.1016/j.patol.2011.05.003
214
R. Kumar et al.
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 Bartholin’s 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 fine needle aspiration cytology (FNAC). We present an extremely rare case of ACC of Bartholin’s gland in a young patient diagnosed with FNAC and subsequently confirmed 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 inflammatory lesion was diagnosed clinically and antibiotic and anti-inflammatory treatment prescribed, but with no relief. On per speculum, examination, a 2 × 2 cm extremely tender, firm swelling was seen at the vaginal introitus, extending from 4 o’clock to 6 o’clock 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.
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 difficult to appreciate.
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).
neoplasms and less than 1% of female genital tract tumours.1,6 Only 10---15% of carcinomas occurring in Bartholin’s 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 Bartholin’s gland.7 The mean age of diagnosis of ACC is 49 years with a range of 25---80.2 The usual presenting signs and symptoms are nonspecific and may include pain, a burning sensation, dyspareunia bleeding, a palpable mass, etc.1 Clinically, the painful enlargement of the Bartholin’s gland may be confused with more common entities --- bartholinitis, cyst or abscess, as occurred in the present case.2
Histopathology findings 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 Bartholin’s gland in the labia minora was first described by Caspar Bartholin, a Dutch anatomist, in 1677. Retention cysts and abscesses are the common lesions found in the Bartholin’s gland.4 Primary carcinomas of the gland are rare, accounting for only 0.1---7% of all vulvar
Figure 2 Adenois cystic carcinoma of Bartholin’s gland showing tubulo-cribriform arrangement of tumour cells around acellular spaces. H E stain, ×100 (inset --- PAS stain).
Adenoid cystic carcinoma of Bartholin’s gland ACC of Bartholin’s 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 Bartholin’s gland should only be made when (1) the tumour involves the area of the Bartholin’s 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 fine needle aspiration cytology.3,5 Characteristic findings 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.
Conclusion Primary adenoid cystic carcinoma of Bartholin’s gland is a rare, painful, slow-growing but locally aggressive neoplasm. FNAC can be used successfully in its difficult differential diagnosis with common inflammatory lesions and other neoplasms.
Conflict of Interest The authors declare they have no conflict of interest.
Responsabilidades éticas Protección de personas y animales. Los autores declaran que para esta investigación no se han realizado experimentos en seres humanos ni en animales.
215 Confidencialidad de los datos. Los autores declaran que han seguido los protocolos de su centro de trabajo sobre la publicación de datos de pacientes y que todos los pacientes incluidos en el estudio han recibido información suficiente y han dado su consentimiento informado por escrito para participar en dicho estudio. Derecho a la privacidad y consentimiento informado. Los autores han obtenido el consentimiento informado de los pacientes y/o sujetos referidos en el artículo. Este documento obra en poder del autor de correspondencia.
References 1. Cardosi RJ, Speights A, Fiorica JV, Grendys Jr EC, Hakam A, Hoffman MS. Bartholin’s gland carcinoma: a 15-year experience. Gynecol Oncol. 2001;82:247---51. 2. DePasquale SE, McGuinness TB, Mangan CE, Husson M, Woodland MB. Adenoid cystic carcinoma of Bartholin’s gland: a review of the literature and report of a patient. Gynecol Oncol. 1996;61:122---5. 3. Frable WJ, Goplerud DR. Adenoid cystic carcinoma of Bartholin’s gland diagnosis by aspiration biopsy. Acta Cytol. 1975;19: 152---3. 4. Henryk S, Katarzyna S, Agnieszka H. The pathomorphology of Bartholin’s gland analysis of surgical data. Pol J Pathol. 2007;58:99---103. 5. Jethwani D, Mohile N, Gupta VK. Cytodiagnosis of adenoid cystic carcinoma of Bartholin’s 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 Bartholin’s gland: case report with review of the literature. Gynecol Obstet Invest. 2005;59: 54---8. 7. Sook YVY, Jeong WL, Woo SK, Kyoung LJ, Sun JL, Je HL, et al. Adenoid cystic carcinoma of the Bartholin’s gland: report of two cases and review of the literature. Gynecol Oncol. 2006;100:422---5. 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 Classification of Tumours; 2003. p. 316---25.
Patología R E V I S TA
E S PA Ñ O L A
D E
www.elsevier.es/patologia
BRIEF REPORT
Adenoid cystic carcinoma of Bartholin’s gland----A 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 Available online 22 July 2011
KEYWORDS Adenoid cystic carcinoma; Fine needle aspiration; Cytology; Bartholin’s gland
PALABRAS CLAVE Carcinoma adenoide quístico; Punción aspiración con aguja fina; Glandula de Bartolino
∗
Summary Primary carcinomas of Bartholin’s 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 inflammatory lesion. The diagnosis of adenoid cystic carcinoma was made on fine needle aspiration cytology which was confirmed 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 España, S.L. All rights reserved.
Carcinoma adenoide quístico de la glándula de Bartolino. Una entidad poco común sujeta a diagnósticos erróneos Resumen Los carcinomas primarios de la glandula de Bartolino son muy infrecuentes. El carcinoma adenoide quístico, una variante de adenocarcinoma, es todavía más raro que otros tipos histológicos en este órgano. Este tumor maligno es localmente agresivo, ocurre en mujeres postmenopáusica y muy raramente se encuentra en mujeres menores de 40 a˜ nos. Una mujer de 35 a˜ nos consultó por dispareunia en el departamento ginecológico del ambulatorio de nuestro Instituto. La lesión había sido diagnosticada previamente como un proceso inflamatorio. El diagnosticó de carcinoma adenoide quístico fue realizado mediante punción aspiración con aguja fina y confirmado histopatológicamente. Comunicamos los rasgos citohistológicos de este raro tumor, diagnosticado en una paciente joven. Realizamos una revisión de la literatura pertinente. © 2011 SEAP y SEC. Publicado por Elsevier España, S.L. Todos los derechos reservados.
Corresponding author. E-mail address: [email protected] (R. Kumar).
1699-8855/$ – see front matter © 2011 SEAP y SEC. Published by Elsevier España, S.L. All rights reserved. doi:10.1016/j.patol.2011.05.003
214
R. Kumar et al.
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 Bartholin’s 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 fine needle aspiration cytology (FNAC). We present an extremely rare case of ACC of Bartholin’s gland in a young patient diagnosed with FNAC and subsequently confirmed 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 inflammatory lesion was diagnosed clinically and antibiotic and anti-inflammatory treatment prescribed, but with no relief. On per speculum, examination, a 2 × 2 cm extremely tender, firm swelling was seen at the vaginal introitus, extending from 4 o’clock to 6 o’clock 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.
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 difficult to appreciate.
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).
neoplasms and less than 1% of female genital tract tumours.1,6 Only 10---15% of carcinomas occurring in Bartholin’s 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 Bartholin’s gland.7 The mean age of diagnosis of ACC is 49 years with a range of 25---80.2 The usual presenting signs and symptoms are nonspecific and may include pain, a burning sensation, dyspareunia bleeding, a palpable mass, etc.1 Clinically, the painful enlargement of the Bartholin’s gland may be confused with more common entities --- bartholinitis, cyst or abscess, as occurred in the present case.2
Histopathology findings 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 Bartholin’s gland in the labia minora was first described by Caspar Bartholin, a Dutch anatomist, in 1677. Retention cysts and abscesses are the common lesions found in the Bartholin’s gland.4 Primary carcinomas of the gland are rare, accounting for only 0.1---7% of all vulvar
Figure 2 Adenois cystic carcinoma of Bartholin’s gland showing tubulo-cribriform arrangement of tumour cells around acellular spaces. H E stain, ×100 (inset --- PAS stain).
Adenoid cystic carcinoma of Bartholin’s gland ACC of Bartholin’s 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 Bartholin’s gland should only be made when (1) the tumour involves the area of the Bartholin’s 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 fine needle aspiration cytology.3,5 Characteristic findings 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.
Conclusion Primary adenoid cystic carcinoma of Bartholin’s gland is a rare, painful, slow-growing but locally aggressive neoplasm. FNAC can be used successfully in its difficult differential diagnosis with common inflammatory lesions and other neoplasms.
Conflict of Interest The authors declare they have no conflict of interest.
Responsabilidades éticas Protección de personas y animales. Los autores declaran que para esta investigación no se han realizado experimentos en seres humanos ni en animales.
215 Confidencialidad de los datos. Los autores declaran que han seguido los protocolos de su centro de trabajo sobre la publicación de datos de pacientes y que todos los pacientes incluidos en el estudio han recibido información suficiente y han dado su consentimiento informado por escrito para participar en dicho estudio. Derecho a la privacidad y consentimiento informado. Los autores han obtenido el consentimiento informado de los pacientes y/o sujetos referidos en el artículo. Este documento obra en poder del autor de correspondencia.
References 1. Cardosi RJ, Speights A, Fiorica JV, Grendys Jr EC, Hakam A, Hoffman MS. Bartholin’s gland carcinoma: a 15-year experience. Gynecol Oncol. 2001;82:247---51. 2. DePasquale SE, McGuinness TB, Mangan CE, Husson M, Woodland MB. Adenoid cystic carcinoma of Bartholin’s gland: a review of the literature and report of a patient. Gynecol Oncol. 1996;61:122---5. 3. Frable WJ, Goplerud DR. Adenoid cystic carcinoma of Bartholin’s gland diagnosis by aspiration biopsy. Acta Cytol. 1975;19: 152---3. 4. Henryk S, Katarzyna S, Agnieszka H. The pathomorphology of Bartholin’s gland analysis of surgical data. Pol J Pathol. 2007;58:99---103. 5. Jethwani D, Mohile N, Gupta VK. Cytodiagnosis of adenoid cystic carcinoma of Bartholin’s 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 Bartholin’s gland: case report with review of the literature. Gynecol Obstet Invest. 2005;59: 54---8. 7. Sook YVY, Jeong WL, Woo SK, Kyoung LJ, Sun JL, Je HL, et al. Adenoid cystic carcinoma of the Bartholin’s gland: report of two cases and review of the literature. Gynecol Oncol. 2006;100:422---5. 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 Classification of Tumours; 2003. p. 316---25.