Mohs micrographic surgery for vulvar malignancies: A systematic review
Authors: Samantha Shwe, Ashley N. Elsensohn, Camila Ortiz, Christina N. Kraus
Journal: Journal of the American Academy of Dermatology (2021)

To the Editor: Mohs micrographic surgery (MMS) is a promising treatment for vulvar cutaneous malignancies, allowing tumor-free margins while preserving structural and functional integrity. In a recent study evaluating MMS for male genital tumors, low local recurrence rates and high patient-reported satisfaction were observed,1Lukowiak T.M. Perz A.M. Aizman L. et al.Mohs micrographic surgery for male genital tumors: local recurrence rates and patient-reported outcomes.J Am Acad Dermatol. 2021; 84: 1030-1036Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar and other reviews have shown favorable outcomes of MMS for genital malignancies.2Zhu T.H. Zhu T.R. Routt E. Ciocon D. Mohs micrographic surgery for genital skin cancer: a review of cases and reconstructive techniques.J Drugs Dermatol. 2021; 20: 311-319Crossref PubMed Google Scholar However, a systematic review of vulvar MMS is lacking. Our review included 29 articles representing 107 cases (Table I, Supplemental Fig 1; available via Mendeley at https://doi.org/10.17632/r4y9g4sm3h.1). MMS was indicated for the following vulvar malignancies: squamous cell carcinoma (SCC) not involving lymph nodes (38), basal cell carcinoma (BCC) (25), extramammary Paget disease (EMPD) (31), dermatofibrosarcoma protuberans (DFSP) (9), verrucous carcinoma (2), basosquamous carcinoma (1), and nodular infiltrating adenocarcinoma of mammary-like glands (1).Table IDemographic and preoperative information on vulvar skin cancers treated with Mohs micrographic surgeryCancer typeNo. of casesMedian age, yCoexisting inflammatory conditionsAverage tumor size (mm)No. P, No. RTreatments prior to MMSImmunostainsAncillary imagingStudy designSCC3864.5Lichen simplex chronicus (1)18.85, 3Excision (3), imiquimod (2), laser (1), radiation (2), partial vulvectomy (1), total vulvectomy (3), hysterectomy (1), and abdominal-perineal resection (1)n/aPET (1) and CT (2)Case report (4), review (1), and prospective original research (1)BCC2568Lichen sclerosus (1)15.311, 2Curettage (1), imiquimod (1), WLE (1), and topical corticosteroids (1)n/an/aCase report (4), case series (2), and review (1)EMPD3168.8n/a57.86, 9CO2 laser (1), imiquimod (1), MMS (2), partial vulvectomy (1), total vulvectomy (1), and WLE (4)CK7 (11), EMA (1), and S-100 (1)Colonoscopy (5), Mammogram (5), cystoscopy (5), pelvic ultrasound (1), pap smear (1), sigmoidoscopy (1), pyelogram (1), and CXR (1)Review (4), case series (2), case report (1), prospective cohort (1), and prospective original research (1)DFSP949n/a48.94, 1WLE (3)CD34 (2), Vimentin (1), PDGFRA (1), and PDGFRB (1)MRI (1) and CXR (1)Review (5), and case report (3)VC252.5Lichen sclerosus (1)101, 0n/an/aPelvic ultrasound (1), cytology (1), and CXR (1)Review (1), and case series (1)BSC170n/a15n/an/an/an/aReview (1)Adenocarcinoma of mammary-like glands151n/a101, 0n/aCK7 (1), CEA (1), and GCDFP-15 (1)CT pelvis (1)Case report (1)Total107 cases28, 1529 articlesBCC, Basal cell carcinoma; BSC, basosquamous cell carcinoma; CD34, cluster of differentiation 34; CEA, carcinoembryonic antigen; CK7, cytokeratin 7; CT, computerized tomography; CXR, chest X-ray; DFSP, dermatofibrosarcoma protuberans; EMA, epithelial membrane antigen; EMPD, extramammary Paget disease; GCDFP-15, gross cystic disease fluid protein 15; MMS, Mohs micrographic surgery; MRI, magnetic resonance imaging; n/a, not applicable; P, primary; PDGFRA, platelet derived growth factor receptor alpha; PDGFRB, platelet derived growth factor receptor beta; PET, positron emission tomography; R, recurrent; SCC, squamous cell carcinoma; VC, verrucous carcinoma; WLE, wide local excision. Open table in a new tab BCC, Basal cell carcinoma; BSC, basosquamous cell carcinoma; CD34, cluster of differentiation 34; CEA, carcinoembryonic antigen; CK7, cytokeratin 7; CT, computerized tomography; CXR, chest X-ray; DFSP, dermatofibrosarcoma protuberans; EMA, epithelial membrane antigen; EMPD, extramammary Paget disease; GCDFP-15, gross cystic disease fluid protein 15; MMS, Mohs micrographic surgery; MRI, magnetic resonance imaging; n/a, not applicable; P, primary; PDGFRA, platelet derived growth factor receptor alpha; PDGFRB, platelet derived growth factor receptor beta; PET, positron emission tomography; R, recurrent; SCC, squamous cell carcinoma; VC, verrucous carcinoma; WLE, wide local excision. No recurrences were reported for BCC, DFSP, verrucous carcinoma, basosquamous carcinoma, and nodular infiltrating adenocarcinoma of mammary-like glands. For BCC, all but 1 case reported outcomes; there was no recurrence at a mean follow-up of 45.6 months. For SCC, there were 4 recurrences (10.53% [4/38]). Of 29 EMPD cases treated with Mohs and reporting on recurrence, recurrence occurred in 4 (13.8%) cases. In 1 case, the recurrence occurred 5 months after MMS, but no recurrence was documented following subsequent MMS at 25-month follow-up. Immunostaining with cytokeratin 7 was performed in 11 EMPD cases; no recurrence was observed. EMPD required the greatest number of stages (3.6) and had the largest postoperative defect size (101.4 mm). All 9 cases of DFSP reported outcomes, and there was no recurrence at a mean follow-up of 54 months. Staining for cluster of differentiation 34 was reported in 2 cases of DFSP. The treatments of SCC prior to MMS included the following: total vulvectomy (3), partial vulvectomy (1), excision (3), radiation (2), imiquimod (2), laser (1), hysterectomy (1), and abdominal-perineal resection (1) (Table II). Two cases of BCC reported treatment with topical imiquimod and excision prior to MMS. The prior treatment of EMPD included the following: total vulvectomy (1), partial vulvectomy (1), excision (4), MMS (2), topical imiquimod (1), and laser (1). In 3 cases of DFSP, excision was performed prior to MMS.Table IIOperative and follow-up information on vulvar skin cancers treated with Mohs micrographic surgeryCancer typeNo. of stagesDefect size (mm)Reconstruction typeAdjuvant treatmentOther specialties involvedPostop complicationsRecurrence no. (%)Follow-up (mo)SCC2.820.5Primary (2) and secondary intentions (3)Radiation therapy (1)Gyn (1), gyn onc (1)Cellulitis (1), DVT (1), metastasis (2), death from disease (2), and death from other causes (2)4 (10.53%)29.7BCC2.525.3Primary (7) and secondary intentions (3) as well as flap (1)Topical imiquimod (1)OBGYN (1)n/a0 (0%)45.6EMPD3.6101.4∗Defect size was only reported in 2 cases.Primary (1) and secondary intentions (3) as well as flap (7)Radiation therapy (2), partial vulvectomy (1)Gyn onc (13), plastics (10)n/a4 (13.79%)36.6DFSP2.660.7Primary (2) and secondary intentions (1) as well as flap (2)Debulking (1)Gyn onc (1)n/a0 (0%)54.3VC1.520Primary (1)n/an/an/a0 (0%)24BSC2.020n/an/an/an/an/an/aAdenocarcinoma of mammary-like glandsn/an/an/an/an/an/a0 (0%)26Total8 (7.69%)†In 3 of 107 cases, recurrence was not reported, and these were not included in the calculation of recurrence rate.BCC, Basal cell carcinoma; BSC, basosquamous cell carcinoma; DFSP, dermatofibrosarcoma protuberans; DVT, deep vein thrombosis; EMPD, extramammary Paget disease; Gyn, gynecology; Gyn onc, gynecology oncology; n/a, not applicable; OBGYN, obstetrics and gynecology; Postop, postoperative; SCC, squamous cell carcinoma; VC, verrucous carcinoma.∗ Defect size was only reported in 2 cases.† In 3 of 107 cases, recurrence was not reported, and these were not included in the calculation of recurrence rate. Open table in a new tab BCC, Basal cell carcinoma; BSC, basosquamous cell carcinoma; DFSP, dermatofibrosarcoma protuberans; DVT, deep vein thrombosis; EMPD, extramammary Paget disease; Gyn, gynecology; Gyn onc, gynecology oncology; n/a, not applicable; OBGYN, obstetrics and gynecology; Postop, postoperative; SCC, squamous cell carcinoma; VC, verrucous carcinoma. The conventional treatment of vulvar cutaneous malignancy is wide local excision/partial vulvectomy or total vulvectomy, procedures that have been known to be associated with disfigurement and functional deficits, with psychologic, social, and sexual ramifications.3Andersen B.L. Hacker N.F. Psychosexual adjustment after vulvar surgery.Obstet Gynecol. 1983; 62: 457-462PubMed Google Scholar The current National Comprehensive Cancer Network guidelines recommend MMS for BCC and DFSP. While the National Comprehensive Cancer Network guidelines recommend MMS for certain cases of penile SCC, they do not recommend MMS for vulvar SCC. Our review suggests that MMS is useful for certain cases of vulvar SCC (early stage) and EMPD. In prior studies of genital EMPD, higher rates of positive margins and recurrence have been found after wide local excision than after MMS.4Kim S.J. Thompson A.K. Zubair A.S. et al.Surgical treatment and outcomes of patients with extramammary paget disease: a cohort study.Dermatol Surg. 2017; 43: 708-714Crossref PubMed Scopus (33) Google Scholar However, in a recent study, it was found that women are more frequently offered total skinning procedures for genital EMPD, whereas men are more frequently offered MMS.5Rastogi S. Thiede R. Sadowsky L.M. et al.Sex differences in initial treatment for genital extramammary Paget's disease in the United States: a systematic review. Preprint. Published online April 20, 2019..J Am Acad Dermatol. 2019; https://doi.org/10.1016/j.jaad.2019.04.046Abstract Full Text PDF Google Scholar In conclusion, our review suggests that MMS is an efficacious modality for primary and recurrent vulvar cutaneous malignancies. We highlight the existing deficit in the surgical treatment of women with vulvar malignancies, most notably SCC. More robust studies of MMS for cutaneous vulvar malignancies are needed to ultimately establish guidelines for optimal patient care. None disclosed.