Journal of Obstetrics and Gynaecology

ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20

Dermatofibrosarcoma protuberans of the vulva G. Bogani, A. Cromi, S. Uccella, M. Serati, J. Casarin, L. Cimetti, N. Donadello & F. Ghezzi To cite this article: G. Bogani, A. Cromi, S. Uccella, M. Serati, J. Casarin, L. Cimetti, N. Donadello & F. Ghezzi (2015) Dermatofibrosarcoma protuberans of the vulva, Journal of Obstetrics and Gynaecology, 35:2, 209-210, DOI: 10.3109/01443615.2014.935726 To link to this article: http://dx.doi.org/10.3109/01443615.2014.935726

Published online: 14 Jul 2014.

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Date: 18 October 2015, At: 23:46

Gynaecology Case Reports 209 rax can recur in ⬎ 50%, despite hormonal therapy (Joseph and Sahn 1996). Hormonal treatment appears to be more effective for catamenial haemoptysis than catamenial pneumothorax (Wang et al. 2000). Ultimately, definitive surgical management would be ideal for this patient if she was willing to lose fertility. The patient consented to the presentation of this case report and the report was exempt from the IRB review. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

low-grade sarcoma. DFSP is a rare tumour accounting for ⬍ 0.1% of all malignancies. Generally, it occurs on the trunk, head and neck and on the proximal (both upper and lower) extremities (Hammonds and Hendi 2010). Vulvar localisations are infrequent and fewer than 50 cases have been previously reported (Edelweiss and Malpica 2010). DFSP is characterised by a high rate of local recurrences. In fact, it is estimated that the recurrence rate after primary excision ranges between 30% and 70% (Ohlinger et al. 2004). Here we describe a case of DFSP arising in the vulva. Furthermore, we conducted an analysis of the current literature.

Case report

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References Akbulut S, Sevinc MM, Bakir S et al. 2010. Scar endometriosis in the abdominal wall: a predictable condition for experienced surgeons. Acta Chirurgica Belgica 110:303–307. Alifano M, Roth T, Broët SC et al. 2003. Catamenial pneumothorax: a prospective study. Chest 124:1004–1008. Alifano M, Trisolini R, Cancellieri A et al. 2006. Thoracic endometriosis: current knowledge. Annals of Thoracic Surgery 81:761–769. Bagan P, Le Pimpec Barthes F, Assouad J et al. 2003. Catamenial pneumothorax: retrospective study of surgical treatment. Annals of Thoracic Surgery 75: 378–381. Bobbio A, Carbognani P, Ampollini L et al. 2007. Diaphragmatic laceration, partial liver herniation and catamenial pneumothorax. Asian Cardiovascular and Thoracic Annals 15:249–251. Bulletti C, Coccia ME, Battistoni S et al. 2010. Review endometriosis and infertility. Journal of Assisted Reproduction and Genetics 27:441–447. Ciriaco P, Negri G, Libretti L et al. 2009. Surgical treatment of catamenial pneumothorax: a single centre experience. Interactive Cardiovascular and Thoracic Surgery 8:349–352. Hensen JH, Van Breda Vriesman AC, Puylaert JB. 2006. Abdominal wall endometriosis: clinical presentation and imaging features with emphasis on sonography. American Journal of Roentgenology 186:616–620. Joseph J, Sahn S. 1996. Thoracic endometriosis syndrome: new observation from an analysis of 110 cases. American Journal of Medicine 100:164–169. Kirchner PA. 1998. Porous Diaphragm Syndromes. Chest Surgery Clinics of North America 8:449–472. Pryshchepau M, Gossot D, Magdeleinat P. 2010. Unusual presentation of catamenial pneumothorax. European Journal of Cardio-Thoracic Surgery 37:1221. Rousset-Jablonski C, Alifano M, Plu-Bureau G et al. 2011. Catamenial pneumothorax and endometriosis-related pneumothorax: clinical features and risk factors. Human Reproduction 26:2322–2329. Shiraishi T. 1991. Catamenial pneumothorax: report of a case and review of the Japanese and non-Japanese literature. Thoracic and Cardiovascular Surgeon 39:304–307. Triponez F, Alifano M, Bobbio A et al. 2010. Endometriosis-related spontaneous diaphragmatic rupture. Interactive Cardiovascular and Thoracic Surgery 11:485–487. Wang HC, Kuo PH, Kuo SH et al. 2000. Catamenial hemoptysis from tracheobronchial endometriosis: reappraisal of diagnostic value of bronchoscopy and bronchial brush cytology. Chest 118:1205.

A 50-year-old multiparous woman underwent gynaecological examination due to the presence of an itching vulvar lesion that had enlarged gradually during the last 2 years. Her medical history was notable for type 2 diabetes mellitus, gastroesophageal reflux and arterial hypertension. Vulvoscopic examination showed the presence of a brownish-red coloured maculopapular sharply demarcated protruding lesion, measuring 2 ⫻ 1 cm at the left labium majus. Pap smear and colposcopic cervical evaluation were negative. Additionally, gynaecological and pelvic ultrasound examinations were normal. An excisional biopsy of the vulvar lesion was performed under local anaesthesia with about 0.5 cm free margins. Histological examination showed a population of neoplastic uniform, mildly atypical spindle-shaped cells distributed in the dermis and subcutaneous tissue with a storiform pattern (DFSP). Figure 1 shows the histological features. Mitotic activity was low (Ki-67/MIB-1 4%). Immunohistochemistry positivity of the neoplastic cells for CD34 was observed. The epidermis was uninvolved. Lateral margins were tumour free, but the lesion was close to the deeper surgical margin. Subsequently, the patient underwent clinical, dermatological and radiological examination. Chest and abdominal CT were negative and no other lesions were clinically detected. In order to ensure adequate resection of the tumour, surgical re-excision with about 4 cm free margins, including muscular fascia layer, was performed 1 month after excisional biopsy. No lesions were detected at pathological examination. Regular evaluations were undertaken for local recurrence and distant metastasis and at 24-month of follow-up, the patient was disease free.

Discussion First described in 1924 by Darier J. and Ferrand M., DFSP is a well-to-moderately differentiated sarcoma arising in the dermis and subcutaneous tissue (Edelweiss and Malpica 2010). It has an incidence of 3–6.5 cases/million persons per year (Lee et al. 2011). Trunk, head and neck and extremities are the most common sites of involvement, while vulvar localisation is uncommon (Gökden et al.

Dermatofibrosarcoma protuberans of the vulva G. Bogani1, A. Cromi1, S. Uccella1, M. Serati1, J. Casarin1, L. Cimetti2, N. Donadello1 & F. Ghezzi1 Departments of 1Obstetrics and Gynaecology and 2Human Morphology, University of Insubria, Del Ponte Hospital, Varese, Italy DOI: 10.3109/01443615.2014.935726 Correspondence: G. Bogani, Department of Obstetrics and Gynecology, University of Insubria, Piazza Biroldi, 1 - Varese, 21100, Italy. E-mail: [email protected]

Introduction Dermatofibrosarcoma protuberans (DFSP) represents a mesenchymal neoplasm of the dermis and subcutis regarded as a superficial

Figure 1. Histological features and immunohistochemical staining of dermatofibrosarcoma protuberans of the vulva. Histology shows the presence of dermal tumour composed of spindle cells extents to the subcutaneous tissue (a) and spindle-shaped cells with a storiform pattern (b). Strong and diffuse CD34 immunoreactivity (c); low proliferative index, Ki-67 (d).

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2003). Tumour cells are reactive for CD34 and are characterised by translocation t (17; 22) (q22; q13), generally in the form of a supernumerary ring chromosome, resulting in the formation of a chimeric fusion gene (COL1A1/PDGFB, i.e. collagen type I alpha I-platelet-derived growth factor beta) (Vanni et al. 2000). It generally affects young–middle-aged adults, and it is characterised by a significant risk of local recurrence and a low risk of distant spread. Interestingly, since several late recurrences have been described, long-term follow-up is required, especially in the light of the evidence suggesting the potential fibrosarcomatous evolution of the DFSP (Edelweiss and Malpica 2010). However, only limited data about DFSP arising in the vulva are still available. Historically, local treatments included different levels of radicality, ranging from excisional biopsy of the lesion to radical vulvectomy plus radiation therapy, while lymphadenectomy is generally not required (Hammonds and Hendi 2010). Several investigations suggested that large free margins are necessary to reduce risk of recurrence (Gloster et al. 1996; Edelweiss and Malpica 2010). Kimmel et al. (2007) suggested that 4 cm free margins achieve margins clearance in 95% of cases. Increasing evidence underlines that methods that ensure complete margin control, using frozen section, such as Mohs micrographic surgery (MMS), represent an effective and safe treatment for DFSP of the vulva (Hammonds and Hendi 2010; Galimberti et al. 2012). In 1996, Gloster et al. evaluated the recurrence rate after different types of surgical excision for the treatment of DFSP in the field of general dermatology. In this series, the cumulative relapse rate was higher for patients undergoing conservative excisions (43%) and wide surgical excisions (20%) than after MMS (1.6%). More recently, Thomas et al. (2007) reported excellent outcomes for 39 patients affected by DFSP of the skin who had MMS (no recurrences of disease were observed after a mean follow-up period of 39 months). DFSP is a rare occurrence, especially in the vulva. Usually, local excision of the mass represents the effective standard management. Due to the need to preserve vulvar tissue, the use of MMS thus represents a very effective method to achieve free margins with maximum tissue preservation and without subverting the anatomical function.

An unusual location of a vulvar leiomyoma F. Yazici Yilmaz1, Ö. Şengül1, K. N. Salİhoğlu1, F. S. Dede1, İ. Dölen1 & H. Ökten2 Departments of 1Obstetrics and Gynecology and 2Pathology, Etlik Zubeyde Hanim Women’s Health Training and Research Hospital, Ankara, Turkey DOI: 10.3109/01443615.2014.936841 Correspondence: F. Yazıcı Yılmaz, Department of Obstetrics and Gynecology, Etlik Zubeyde Hanim Women’s Health Training and Research Hospital, Yeni Etlik Caddesi No:55 06010 Etlik, Keçiören, Ankara, Turkey. E-mail: [email protected]

Introduction Leiomyomas of the external genitalia are uncommon soft tissue tumours. There are limited reports in the literature. These tumours are considered to originate from smooth muscle within erectile tissue, blood vessel walls, the round ligament, the dartos muscle or the erector pili muscle. The most common locations are the Bartholin gland area and labia majora (Nielsen et al. 1996). Vulvar leiomyomas are very rare and labia minora location even more so. We report the case of a 27-year-old woman a with vulvar leiomyoma on the right labium minora.

Case report A 27-year-old gravida 3, para 3 woman was admitted to our hospital with a protruding mass on the right labium minora and she complained of vulvar pain for approximately 4 months. No serious illness was identified in her history. General physical and abdominal examinations were normal. On pelvic examination, the mass was

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Edelweiss M, Malpica A. 2010. Dermatofibrosarcoma protuberans of the vulva: a clinicopathologic and immunohistochemical study of 13 cases. American Journal of Surgical Pathology 34:393–400. Galimberti G, Montaño AP, Kowalczuk A et al. 2012. Outcomes in 11 patients with dermatofibrosarcoma protuberans treated with Mohs micrographic surgery. International Journal of Dermatology 51:89–93. Gloster HM Jr, Harris KR, Roenigk RK. 1996. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. Journal of the American Academy of Dermatology 35:82–87. Gökden N, Dehner LP, Zhu X et al. 2003. Dermatofibrosarcoma protuberans of the vulva and groin: detection of COL1A1-PDGFB fusion transcripts by RTPCR. Journal of Cutaneous Pathology 30:190–195. Hammonds LM, Hendi A. 2010. Dermatofibrosarcoma protuberans of the vulva treated using Mohs micrographic surgery. Dermatologic Surgery 36: 558–563. Kimmel Z, Ratner D, Kim JY et al. 2007. Peripheral excision margins for dermatofibrosarcoma protuberans: a meta-analysis of spatial data. Annals of Surgical Oncology 14:2113–2120. Lee HJ, Kim MJ, Choi J et al. 2011. Dermatofibrosarcoma protuberans arising on the skin of the breast. Breast Journal 17:93–95. Ohlinger R, Kühl A, Schwesinger G et al. 2004. Dermatofibrosarcoma protuberans of the vulva. Acta Obstetricia et Gynecologica Scandinavica 83: 685–686. Thomas CJ, Wood GC, Marks VJ. 2007. Mohs micrographic surgery in the treatment of rare aggressive cutaneous tumors: the Geisinger experience. Dermatologic Surgery 33:333–339. Vanni R, Faa G, Dettori T et al. 2000. A case of dermatofibrosarcoma protuberans of the vulva with a COL1A1/PDGFB fusion identical to a case of giant cell fibroblastoma. Virchows Archiv 437:95–100.

Figure 1. Vulvar leiomyoma on the right labium minora.

Dermatofibrosarcoma protuberans of the vulva.

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