Abstract
Objective
Should one evaluate the asymptomatic nonspecific hypopigmented interlabial area in a menopausal woman attending a corporate health check clinic?
Case Report
A menopausal woman aged 66 years, a para 2, reported for a voluntary routine health check including gynaecological evaluation. Examination revealed a hypopigmented interlabial area without any itching, itch marks, redness or thickened skin. There was no altered sensation. There were no skin lesions elsewhere on the body nor was any significant lymphadenopathy. Toluidine blue-directed vulvar biopsy was consistent with mycosis fungoides. Gene rearrangement of T cell receptor by PCR and gel electrophoresis suggested clonal nature of T cell lymphoproliferative disorder. Immunohistochemistry markers CD3 and CD4 were positive.
Conclusion
Cutaneous T cell lymphomas are aggressive and prone to delayed or misdiagnosis and have a high mortality rate. Malignant lymphomas in the pelvis are rare, and the vulvar primary involvement is only 4% of pelvic lymphomas. Therefore, any vulvar hypopigmented or hypertrophied areas need to be subjected to histopathological evaluation.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Notes
The laboratory reports of the work-up are provided as Electronic Supplementary Material.
References
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–85.
Aarset H, Skarsvag S, Ryggen K. Mycosis fungoides. Early diagnosis and evaluation of treatment by polymerase chain reaction. Tidsskr Nor Laegeforen. 1997;117:3391–2.
Lukowsky A, Muche JM, Sterry W, Audring H. Detection of expanded T cell clones in skin biopsy samples of patients with lichen sclerosus et atrophicus by T cell receptor-gamma polymerase chain reaction assays. J Invest Dermatol. 2000;115:254–9.
Weinstock MA, Gardstein B. Twenty-year trends in the reported incidence of mycosis fungoides and associated mortality. Am J Public Health. 1999;89:1240–4.
Nam JH, Park MC, Lee KH, Yoon C, Park HR, Chun BK. Primary non-Hodgkin’s malignant lymphoma of the vulva. J Korean Med Sci. 1992;7:271–5.
Andrea L, Buras AL, McSwain AN, Quin Y, Meunier JK. Primary mycosis fungoides of the vulva: the first reported case. Gynaecol Oncol Rep. 2015;12:7–8.
Vang R, Medeiros LJ, Malpica A, Levenback C, Deavers M. Non-Hodgkin’s lymphoma involving the vulva. Int J Gynecol Pathol. 2000;19:236–42.
Whittaker S. Molecular genetics of cutaneous lymphomas. Ann NY Acad Sci. 2001;941:39–45.
Scarisbrick JJ, Woolford AJ, Russell-Jones R, Whittaker SJ. Allelotyping in mycosis fungoides and Sezary syndrome: common regions of allelic loss identified on 9p, 10q and 17p. J Invest Dermatol. 2001;117:663–70.
Panda S. Mycosis fungoides: current trends in diagnosis and management. Indian J Dermatol. 2007;52:5–20.
Buras AL, McSwain AN, Qin Y, Meunier JK. Primary mycosis fungoides of the vulva: the first reported case. Gynecol Oncol Rep. 2015;12:7–8.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
Authors have no conflicts of interest to declare.
Ethics Committee Approval
Permission was obtained for presentation.
Informed Consent
Informed consent was obtained from the participants.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Nithya, P.J., Parimkayala, R., Saha, D. et al. Vulvar Mycosis Fungoides: A Rare Non-Hodgkin’s Cutaneous Lymphoma. Indian J Gynecol Oncolog 15, 17 (2017). https://doi.org/10.1007/s40944-017-0107-3
Received:
Revised:
Accepted:
Published:
DOI: https://doi.org/10.1007/s40944-017-0107-3