Abstract
Introduction
Adenoid cystic carcinoma of vulva (ACC-vulva) is an extremely rare entity with <100 cases reported in the literature so far.
Objective
To study the clinical profile and outcome of ACC-vulva treated at a tertiary cancer care centre in South India.
Methods
This is a retrospective, record-based study of histopathologically confirmed cases of ACC-vulva treated at our centre from January 2005 to March 2016.
Results
Only four patients were diagnosed with ACC-vulva during the 11-year period under study. The longest duration of follow-up was 129 months. The age at diagnosis ranged from 32 to 43 years, with a median of 40 years. All patients were married, parous and premenopausal and presented with a painless unilateral vulval swelling. All patients had involvement of the Bartholin’s gland site with normal overlying skin. In all patients, wide excision was performed. Unilateral inguinal node dissection was done in one case. Perineural infiltration was documented in two cases, while positive excision margins were present in three cases. None of the patients had any lymph node involvement at diagnosis or during follow-up. Two patients had recurrence of disease. The disease-free interval was 23 months for one patient and 118 months for the other. In both, local (vulval) and distant (multiple lung) metastases were detected simultaneously.
Conclusion
Adenoid cystic carcinoma of vulva is an extremely rare, slowly progressing neoplasm mostly involving the Bartholin’s gland. The usual treatment includes wide excision and adjuvant radiotherapy (if required). There may be late local and distant recurrence.
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‘Leena Rose Johnson, Rema. P, Suchetha. S, Rari P Mony, Jayapriya G., Aswin Kumar and Iqbal M. Ahamed’ declare that they have no conflict of interest.’
Ethical Statement
All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008.
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Leena Rose Johnson has done her MBBS, DGO, DNB(OBG). She is Assistant Professor of Obstetrics and Gynecology at SUT Academy of Medical Sciences,Thiruvananthapuram. Rema Prabhakaran Nair is Additional Professor, Division of Surgical Oncology, Regional Cancer Centre; Suchetha Sambasivan is Associate Professor, Division of Surgical Oncology, Regional Cancer Centre; Rari P. Mony is Senior Resident, Division of Pathology, Regional Cancer Centre; Jayapriya Gangadharan is Senior Resident, Division of Pathology, Regional Cancer Centre; Aswin Kumar is Additional Professor, Division of Radiation Oncology, Regional Cancer Centre; Iqbal M. Ahamed is Professor and Head of the Department, Division of Surgical Oncology, Regional Cancer Centre.
The study was conducted during her tenure as Senior Resident in Gynecologic Surgical Oncology at Regional Cancer Centre, Thiruvananthapuram.
This study has been approved by the Institutional Review Board, Regional Cancer Centre, Thiruvananthapuram.
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Johnson, L.R., Nair, R.P., Sambasivan, S. et al. Adenoid Cystic Carcinoma of Vulva-11 Years’ Single-Institution Experience. J Obstet Gynecol India 67, 196–201 (2017). https://doi.org/10.1007/s13224-016-0935-6
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DOI: https://doi.org/10.1007/s13224-016-0935-6