Adenoid Cystic Carcinoma of the Bartholin’s Gland: A Diagnostic …downloads.hindawi.com/journals/criog/2019/1784949.pdf · 2019-08-18 · CaseReport Adenoid Cystic Carcinoma of

Case ReportAdenoid Cystic Carcinoma of the Bartholinrsquos GlandA Diagnostic Dilemma

Charmaine C W Lo 1 Jerome B Y Leow2 K Naing3

Ken Jaaback 3 and Thanuja Thachil 2

1Faculty of Health and Medicine University of Newcastle Newcastle New South Wales Australia2Central Coast Cancer Centre Gosford District Hospital Gosford New South Wales Australia3John Hunter Hospital Newcastle New South Wales Australia

Correspondence should be addressed toThanuja Thachil tanu70rediffmailcom

Received 31 March 2019 Accepted 22 July 2019 Published 18 August 2019

Academic Editor John P Geisler

Copyright copy 2019 Charmaine C W Lo et al This is an open access article distributed under the Creative Commons AttributionLicense which permits unrestricted use distribution and reproduction in any medium provided the original work is properlycited

Adenoid cystic carcinomas of the Bartholinrsquos gland are extremely rare and are often misdiagnosed There are currently no definitetreatment guidelines This article describes the case of a 33-year-old female who was managed at our centre for adenoid cysticcarcinoma of the Bartholinrsquos gland She presented with a prolonged history of a vulvar lesion which was eventually diagnosed asadenoid cystic carcinoma of the Bartholinrsquos gland She was subsequently treatedwith wide local excision of the primary and inguinallymph node dissection followed by adjuvant radiotherapy and chemotherapy She had gross perineural invasion on MRI imagingThe present case highlights the diagnostic dilemma in this extremely rare cancer and the literature further explores the naturalhistory and treatment options

1 Introduction

Primary Bartholinrsquos gland cancer (BGC) is rare and con-sists of 0001 of all female genital cancers [1] The vari-ety of subtypes for BGCs include squamous cell ade-noid epitheloid-myoepithelial neuroendocrine Merkel celllymphoepithelium-like transitional cell and adenoid cysticcarcinoma [2] Adenoid cystic carcinomas usually arise fromminor and major salivary glands [3] The other sites of origininclude the nasopharynx [4] lacrimal glands [5] skin [6]trachea [7] breast [8] and vulva [9] As of 2018 only 350cases of the adenoid cystic subtype have been reported in theBartholinrsquos gland [10] BGCs are often misdiagnosed becausethey are so rare but also often present as a mimic of a benignBartholinrsquos gland pathology There are currently no clinicalguidelines for the treatment of BGCs Below we describe acase of the adenoid cystic subtype of a BGC seen at our centerThe aim of the present study is to highlight the importanceof early diagnosis of this rare cancer and to explore thetreatment options in order to improve the prognosis Ourcase interestingly showed pudendal nerve invasion which

could have been missed if the natural history was not wellexplored

2 Case Presentation

A 33-year-old ECOG 0 female was referred to our centre foropinion and management of postoperative locally invasiveadenoid cystic carcinoma of the Bartholinrsquos gland (ACCBG)She was nulliparous on an oral contraceptive with menarcheat the age of 15 Gynaecological and family history wasotherwise unremarkable

She first presented to a hospital in 2009 for a rightvulvar lesion Biopsy of this lesion was benign and the lesionresolved without intervention In 2013 the patient noticed anew right perineal lesionCTMRI and bone scanswere orga-nized by her gynaecologist Investigationswere unremarkableexcept for a lesion in the ischial tuberosity onMRIThe lesionwas deemed benign after review by orthopedic surgeons4 years later the patient began to experience dyspareuniaassociated with a burning sensation An MRI performed in

HindawiCase Reports in Obstetrics and GynecologyVolume 2019 Article ID 1784949 4 pageshttpsdoiorg10115520191784949

2 Case Reports in Obstetrics and Gynecology

Figure 1 Preoperative T2 axial MRI though the pelvis demonstrat-ing ACCBG lesion in the right vulva

June 2018 showed soft tissue swelling in the perineal regionand a 14x13x13mm rounded soft tissue mass at the rightposterolateral margin on the vaginal introitus consistent witha Bartholinrsquos cystThe lesion appeared to infiltrate the vaginalwall in the anteromedial margin but this did not cross themidline (Figure 1)

A biopsy was performed with histological features con-sistent with adenoid cystic carcinoma followed by widelocal excision of the right vulva The specimen spanned43x25x32mm and the tumour involved the excisionmarginsOn histology the specimen had foci of perineural inva-sion and invaded fibrous tissue fat and skeletal muscles(Figure 2) One month later 8 lymph nodes were dissectednone of which were positive

PET scan 2 months after surgery showed FDG uptakeconsistent with postsurgical changes and uptake in a rightaxillary node that was likely inflammatory rather than adistant metastasis Further MRI contrast scans to assessextent of perineural invasion showed linear enhancementalong the course of the perineal branch of the right pudendalnerve terminating before Alcockrsquos canal (Figure 3)

The consensus of the Gynaecologic Oncology Multi-disciplinary Tumour Board was to proceed with adjuvantchemoradiation therapy The patient received radiotherapypostoperatively to the tumour bed to a total dose of 66 Gyand to the right pudendal nerve to 594 Gy in 33 fractionsby a VMAT technique with concurrent weekly 40 mgm2

cisplatin chemotherapy which she tolerated well The ovarieswere spared to prevent premature ovarian dysfunction At her3 month posttreatment follow-up there was clinically andradiologically no evidence of locoregional recurrence Thepatient is planned for close surveillance

3 Discussion

31 Etiology andDisease Progression Theetiology of ACCBGis unknown though a series of ACCBG cases by Copelandhas suggested that pregnancy may be an independent risk

Figure 2 Histological slide of the ACCBG specimen Cribriformand tubular glands infiltrating the submucosa of the vulvaThere arepseudoglandular spaces with excess basement membrane materialand mucin Foci of perineural invasion are present The tumourinvolves excision margins

factor [11] ACCBGs can present as both painless and painfulmasses [10 12] and are usually solitary and unilateralClinically they are associated with pruritus and a burningsensation which reflects its nature to invade the perineumbut has also presented with bleeding dyspareunia anddischarge from abscesses [13] They usually occur in womenbetween 40 and 60 years old with an age range of 29 to 76years [7]

Histologically most ACCBG occurs in a lsquocribriformrsquo pat-tern [14] and is diagnosed using the haematoxylin and eosinstain [15] As described by Copeland [11] the lsquocribriformrsquopattern is characterized by anastomosing cords of cellssurrounded by acellular spaces containingmucin andhyalineTumour cells are small and basaloid [1] with scant cytoplasm

Case Reports in Obstetrics and Gynecology 3

Figure 3 Postoperative T2 axial MRI through the pelvis demon-strating high T2 signal in the right pudendal nerve

and normal nuclei They usually have extensive perineuraland skeletal muscle invasion [1 11 16] ACCBGCs are distinctfrom other subtypes of BGCs and seem to arise frommyoepithelial cells of the Bartholinrsquos gland [17]

ACCBGrsquos are slow growing but are locally aggressiveand have a high recurrence rate [18] Usually several localrecurrences precede distant metastasis Distant metastaticsites include the lungs [18] and less commonly brain [12] andbone [16] Patients with ACCBG treated with radical localexcisions can have 5 10 and 15 year progression-free ratesof 47 38 and 13 respectively [11] Overall survival ratesat 5 10 and 15 years are 71 50 and 51 as reported byCopeland et al [11]

32 Management of ACCBGs There are no standard treat-ment guidelines for these extremely rare tumours Surgeryis currently the mainstay of treatment Surgical optionsreported include wide local excisions hemivulvectomy sim-ple vulvectomy and radical vulvectomy with and withoutinguinal andor femoral lymphadenectomy [11 16 19 20]There is no consensus as to the best surgical approachWhile some advocate for radical vulvectomy [13 21] as itresults in lower rates of positive margins others advocatefor a more conservative surgical approach to reduce delay tochemoradiation as ACCBGs have early local disseminationand margin status does not affect recurrence rates [13]However up to 8 of BGCs cannot be treated surgically [22]

Adjuvant radiation is also used to treat ACCBGs with arange of doses though there is no consensus regarding therole extent or total dose of radiation [23] It is recommendedespecially in patients with positive margins on resection[24 25] Previous case reports [12 26ndash28] have reported

radiotherapy doses ranging from 504 Gy to 66 Gy [27]Extrapolating fromHead andNeck adenoid cystic carcinomaseries it is worthwhile to rule out gross perineural invasionand to treat the entire grossly involved nerve [3 29ndash31] Clin-icians should discuss fertility options with premenopausalpatients prior to adjuvant radiotherapy

There has been one report which stated a similar overallsurvival with primary radiation or chemoradiation comparedto other surgical series [32] but to date this is not thepreferred approach

There is less evidence for chemotherapy in ACCBGcompared to adjuvant radiotherapy A variety of chemo-therapy regimens used include drugs such as paclitaxelcyclophosphamide cisplatinmethotrexate doxorubicin and5-fluorouracil [10 33 34] Chemotherapy regimens are usu-ally used in conjunction with radiotherapy or after recur-rence of ACCBGs after surgery and adjuvant radiotherapytreatment [10 34] Immunotherapy has also been used withanecdotal reports of excellent response [33]

4 Conclusion

Adenoid cystic carcinoma of the Bartholinrsquos gland is anextremely rare tumour with no established diagnostic ortherapeutic pathway We present a case of a 33-year-oldwoman with adenoid cystic carcinoma of the Bartholinrsquosgland treated with surgery followed by adjuvant concurrentchemoradiotherapy preserving ovarian function Our caseinterestingly showed invasion of the pudendal nerve andthe patient in fact had prolonged signs and symptoms fora few years before the eventual discovery of adenoid cysticcarcinoma This rare tumour often poses substantial diag-nostic and therapeutic dilemmas Case reports and multi-institutional pooled studies are imperative in contributing tofuture diagnostic and treatment guidelines of this extremelyrare malignancy

Consent

The patient has given written consent for publication of thecurrent case report

Conflicts of Interest

The authors declare that there is no conflict of interestregarding the publication of this paper

References

[1] M Akbarzadeh-Jahromi F S Aslani N Omidifar and SAmooee ldquoAdenoid cystic carcinoma of bartholinrsquos gland clin-ically mimics endometriosis A case reportrdquo Iranian Journal ofMedical Sciences vol 39 no 6 pp 580ndash583 2014

[2] S Neill and F Lewis Ridleyrsquos The Vulva Wiley 2009[3] M Huang D Ma K Sun G Yu C Guo and F Gao ldquoFactors

influencing survival rate in adenoid cystic carcinoma of thesalivary glandsrdquo International Journal of Oral and MaxillofacialSurgery vol 26 no 6 pp 435ndash439 1997


[4] X Liu C Xie H Li et al ldquoNasopharyngeal adenoid cysticcarcinoma magnetic resonance imaging features in ten casesrdquoChinese Journal of Cancer vol 32 no 5 pp 19ndash28 2013

[5] S-O Baek Y-J Lee S-H Moon Y-J Kim and Y-J JunldquoPrimary adenocarcinoma of the lacrimal glandrdquo Archives ofPlastic Surgery vol 39 no 5 pp 578ndash580 2012

[6] I D Tonev Y S Pirgova and N V Conev ldquoPrimary adenoidcystic carcinoma of the skin with multiple local recurrencesrdquoCase Reports in Oncology vol 8 no 2 pp 251ndash255 2015

[7] S Kumar N E M Iype S Thomas and U V Sankar ldquoAdenoidcystic carcinoma of the tracheardquo Indian Journal of SurgicalOncology vol 7 no 1 pp 62ndash66 2016

[8] K Miyai M R Schwartz and M K Divatia ldquoAdenoid cysticcarcinoma of breast Recent advancesrdquoWorld Journal of ClinicalCases vol 2 no 12 pp 732ndash741 2014

[9] L R Johnson R P Nair S Sambasivan et al ldquoAdenoid cysticcarcinoma of vulva-11 yearsrsquo single-institution experiencerdquo TheJournal of Obstetrics and Gynecology of India vol 67 no 3 pp196ndash201 2017

[10] S P Goh B McCully and M K Wagner ldquoA case of adenoidcystic carcinoma mimicking a bartholin cyst and literaturereviewrdquo Case Reports in Obstetrics and Gynecology vol 2018Article ID 5256876 3 pages 2018

[11] L J Copeland N Sneige D M Gershenson P B Saul C AllenStringer and J C Seskl ldquoAdenoid cystic carcinoma of bartholinglandrdquo Obstetrics amp Gynecology vol 67 no 1 pp 115ndash120 1986

[12] R Ramanah E Allam-Ndoul C Baeza and D RiethmullerldquoBrain and lung metastasis of Bartholinrsquos gland adenoid cysticcarcinoma a case reportrdquo Journal of Medical Case Reports vol7 article 208 2013

[13] S-Y V Yang J-W Lee W-S Kim et al ldquoAdenoid cysticcarcinoma of the Bartholinrsquos gland Report of two cases andreview of the literaturerdquo Gynecologic Oncology vol 100 no 2pp 422ndash425 2006

[14] Y G Xu M Hinshaw B J Longley H Ilyas and S NSnow ldquoCutaneous adenoid cystic carcinoma with perineuralinvasion treated by mohs micrographic surgery-a case reportwith literature reviewrdquo Journal of Oncology vol 2010 Article ID469049 5 pages 2010

[15] W A Khan V Mahajan S Sisodia and S H Ansari ldquoAdenoidcystic carcinoma of Bartholin1015840s gland Report of a case andreview of literaturerdquo South Asian Journal of Cancer vol 2 no1 p 18 2013

[16] S-T Hsu R-C Wang C-H Lu et al ldquoReport of two casesof adenoid cystic carcinoma of Bartholinrsquos gland and review ofliteraturerdquo Taiwanese Journal of Obstetrics and Gynecology vol52 no 1 pp 113ndash116 2013

[17] J C Felix R J Cote E E W Kramer P Saigo and G HGoldman ldquoCarcinomas of Bartholinrsquos gland histogenesis andthe etiological role of human papillomavirusrdquo The AmericanJournal of Pathology vol 142 no 3 pp 925ndash933 1993

[18] F M Woida and A Ribeiro-Silva ldquoAdenoid cystic carcinomaof the bartholin gland An overviewrdquo Archives of Pathology ampLaboratory Medicine vol 131 no 5 pp 796ndash798 2007

[19] A B Bhalwal A M Nick R dos Reis et al ldquoCarcinoma of thebartholin gland a review of 33 casesrdquo International Journal ofGynecologic Cancer vol 26 no 4 pp 785ndash789 2016

[20] L J Copeland N Sneige D M Gershenson V B Mcguffee FAbdul-Karim and F N Rutledge ldquoBartholin gland carcinomardquoObstetrics amp Gynecology vol 67 no 6 pp 794ndash801 1986

[21] V Anaf F Buxant F Rodesch et al ldquoAdenoid cystic carcinomaof Bartholinrsquos glandwhat is the optimal approachrdquo EuropeanJournal of Surgical Oncology (EJSO) vol 25 no 4 pp 406ndash4091999

[22] V Di Donato A Casorelli E Bardhi et al ldquoBartholin glandcancerrdquo Critical Review in OncologyHematology vol 117 pp 1ndash11 2017

[23] P Rosenberg E Simonsen and B Risberg ldquoAdenoid cysticcarcinoma of Bartholinrsquos gland A report of five new casestreated with surgery and radiotherapyrdquo Gynecologic Oncologyvol 34 no 2 pp 145ndash147 1989

[24] S E Depasquale T B Mcguinness C E Mangan M Hussonand M B Woodland ldquoAdenoid cystic carcinoma of Bartholinrsquosgland A review of the literature and report of a patientrdquoGynecologic Oncology vol 61 no 1 pp 122ndash125 1996

[25] K Nasu Y Kawano N Takai K Kashima and I MiyakawaldquoAdenoid cystic carcinoma of Bartholinrsquos gland Case reportwith review of the literaturerdquo Gynecologic and Obstetric Inves-tigation vol 59 no 1 pp 54ndash58 2005

[26] L Agolli M F Osti V Armosini V De Sanctis M ValerianiandRM Enrici ldquoAdenoid cystic carcinomaof Bartholinrsquos glandreceiving adjuvant radiation therapy case reportrdquo EuropeanJournal of Gynaecological Oncology vol 34 no 5 pp 487-4882013

[27] AMakhija A D Desai BM Patel and C D Parekh ldquoAdenoidcystic carcinoma of bartholinrsquos gland misdiagnosed as benignvulvar adnexal tumourrdquo Indian Journal of Gynecologic Oncologyvol 16 no 1 p 1 2018

[28] M Momeni Y Korotkaya G Carrasco and M Prasad-HayesldquoAdenoid cystic carcinoma of Bartholinrsquos gland case reportrdquoActa Medica Iranica vol 54 no 12 pp 820ndash822 2016

[29] C L Ellington M Goodman S A Kono and W GristldquoAdenoid cystic carcinoma of the head and neckrdquo Cancer vol118 no 18 pp 4444ndash4451 2012

[30] ANDantas E F deMorais RAMacedo JM Tinoco andMd Morais ldquoClinicopathological characteristics and perineuralinvasion in adenoid cystic carcinoma a systematic reviewrdquoBrazilian Journal of Otorhinolaryngology vol 81 no 3 pp 329ndash335 2015

[31] J Ko J Siever D Hao R Simpson and H Lau ldquoAdenoid cysticcarcinoma of head and neck clinical predictors of outcomefrom a Canadian centrerdquo Current Oncology vol 23 no 1 pp26ndash33 2016

[32] E Lopez-Varela E Oliva J F McIntyre and A F FullerJr ldquoPrimary treatment of Bartholinrsquos gland carcinoma withradiation and chemoradiation A report on ten consecutivecasesrdquo International Journal of Gynecological Cancer vol 17 no3 pp 661ndash667 2007

[33] P Zhan G Li B Liu and X-G Mao ldquoBartholin glandcarcinoma A case reportrdquo Oncology Letters vol 8 no 2 pp849ndash851 2014

[34] G Yoon H-S Kim Y-Y Lee et al ldquoAnalysis of clinical out-comes of patients with adenoid cystic carcinoma of Bartholinglandsrdquo International Journal of Clinical and ExperimentalPathology vol 8 no 5 pp 5688ndash5694 2015

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PPAR Research

Hindawi Publishing Corporation httpwwwhindawicom Volume 2013Hindawiwwwhindawicom

The Scientific World Journal

Volume 2018

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Evidence-Based Complementary andAlternative Medicine

Volume 2018Hindawiwwwhindawicom

Submit your manuscripts atwwwhindawicom


Figure 1 Preoperative T2 axial MRI though the pelvis demonstrat-ing ACCBG lesion in the right vulva

June 2018 showed soft tissue swelling in the perineal regionand a 14x13x13mm rounded soft tissue mass at the rightposterolateral margin on the vaginal introitus consistent witha Bartholinrsquos cystThe lesion appeared to infiltrate the vaginalwall in the anteromedial margin but this did not cross themidline (Figure 1)

A biopsy was performed with histological features con-sistent with adenoid cystic carcinoma followed by widelocal excision of the right vulva The specimen spanned43x25x32mm and the tumour involved the excisionmarginsOn histology the specimen had foci of perineural inva-sion and invaded fibrous tissue fat and skeletal muscles(Figure 2) One month later 8 lymph nodes were dissectednone of which were positive

PET scan 2 months after surgery showed FDG uptakeconsistent with postsurgical changes and uptake in a rightaxillary node that was likely inflammatory rather than adistant metastasis Further MRI contrast scans to assessextent of perineural invasion showed linear enhancementalong the course of the perineal branch of the right pudendalnerve terminating before Alcockrsquos canal (Figure 3)

The consensus of the Gynaecologic Oncology Multi-disciplinary Tumour Board was to proceed with adjuvantchemoradiation therapy The patient received radiotherapypostoperatively to the tumour bed to a total dose of 66 Gyand to the right pudendal nerve to 594 Gy in 33 fractionsby a VMAT technique with concurrent weekly 40 mgm2

cisplatin chemotherapy which she tolerated well The ovarieswere spared to prevent premature ovarian dysfunction At her3 month posttreatment follow-up there was clinically andradiologically no evidence of locoregional recurrence Thepatient is planned for close surveillance

3 Discussion

31 Etiology andDisease Progression Theetiology of ACCBGis unknown though a series of ACCBG cases by Copelandhas suggested that pregnancy may be an independent risk

Figure 2 Histological slide of the ACCBG specimen Cribriformand tubular glands infiltrating the submucosa of the vulvaThere arepseudoglandular spaces with excess basement membrane materialand mucin Foci of perineural invasion are present The tumourinvolves excision margins

factor [11] ACCBGs can present as both painless and painfulmasses [10 12] and are usually solitary and unilateralClinically they are associated with pruritus and a burningsensation which reflects its nature to invade the perineumbut has also presented with bleeding dyspareunia anddischarge from abscesses [13] They usually occur in womenbetween 40 and 60 years old with an age range of 29 to 76years [7]

Histologically most ACCBG occurs in a lsquocribriformrsquo pat-tern [14] and is diagnosed using the haematoxylin and eosinstain [15] As described by Copeland [11] the lsquocribriformrsquopattern is characterized by anastomosing cords of cellssurrounded by acellular spaces containingmucin andhyalineTumour cells are small and basaloid [1] with scant cytoplasm










4 Conclusion


Consent




References








































of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of




























4 Conclusion


Consent




References








































of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of























































of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of























of




Disease Markers



OncologyJournal of





PPAR Research



Volume 2018


Journal of

ObesityJournal of



















Documents

Adenoid Cystic Carcinoma of the Bartholin’s Gland: A Diagnostic …downloads.hindawi.com/journals/criog/2019/1784949.pdf · 2019-08-18 · CaseReport Adenoid Cystic Carcinoma of