Case Report | Open Access | Full Text

¹Resident Physician, Department of Gynecology and Obstetrics, at Ibn Rochd University Hospital, Casablanca, Morocco
²Professor in the Department of Gynecology and Obstetrics at the Ibn Rochd University Hospital in Casablanca, Morocco

*Corresponding author: Elomri Hajar, Resident Physician, Department of Gynecology and Obstetrics, at Ibn Rochd University Hospital, Casablanca, Morocco

Abstract

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Bowen's disease is an intraepidermal carcinoma that can progress to invasive carcinoma and can affect several organs, including the vulva. This clinical case presents a 45-year-old patient with vulvar BOWEN'S disease, with hyperpigmented, dyschromic vulvar and anal exophytic lesions on the clinical examination. The Skin and vulvar biopsy showed a squamous cell carcinoma in situ with no invasive sites. Cytological sampling of ASC-H cervix, showed at colposcopy: endocervical TAG2A, ZT3. The nail biopsy showed a squamous cell carcinoma in situ (Bowen's disease) with no signs of invasion. The patient was operated for a conization, and the histological examination revealed a squamous cell carcinoma in situ with microinvasive focus 3mm wide and 2mm deep with upper tumour border. Therefore, a total vulvectomy with hysterectomy without adnexal preservation was performed.
The aim of the current treatment is to have a conservative approach, and to preserve the anatomical and functional integrity of the vulva as far as possible.
Keywords: Bowen’s Disease, Carcinoma, Vulva, Tumour
Abbreviations: BD: Bowen’s Disease, HIV: Human Immunodeficiency Virus, FU: Fluorouracil

Introduction

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Bowen’s disease (BD) and Bowenoid papulosis (BP) are premalignant skin lesions, mostly appearing on sun-exposed areas. Lesions on the vulvar regions are relatively rare and there is still confusion about the various terms used to describe these similar disorders [1,2].
The relationship of Bowen’s disease to internal malignancy may still be indirect (via heredity, arsenic exposure, HPV, or other unidentified carcinogen), and further studies with long-term follow-up are necessary. The disease itself is easily treated and has an excellent prognosis [3].

Case report

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45-year-old female patient, one child born naturally, followed for type 2 diabetes on insulin, with undocumented history of recurrent genital infection. Referred from the dermatology department of the university hospital for vulvar and anal lesions that had been evolving for 10 years and had never been treated. Clinical examination revealed a patient in good general condition, with normal-colored conjunctivae, and a BMI of 27kg/m2. Examination of the genital tract revealed hyperpigmented, dyschromic vulvar and anal exophytic lesions (figures 1 and 2). The speculum examination showed the presence of a cervical lesion. The uterus size was normal, with no latero-uterine mass or palpable lymph node. Breast examination was normal. Her nails were scaly with a hard edge (figure 3). A skin and vulvar biopsy in the most suspicious sites (figure 4) showed histological evidence of squamous cell carcinoma in situ with no invasive sites (figures 5 and 6). Cytological sampling of the ASC-H cervix, showing at colposcopy: endocervical TAG2A, ZT3 (figures 7 and 8). Biopsy of the suspicious area of the cervix during colposcopy, showing squamous cell carcinoma in situ (figure 9). Nail biopsy: squamous cell carcinoma in situ (Bowen's disease) with no signs of invasion. Proctological examination: vulvo-anal lesion associated with Bowen's disease. Syphilis serology (TPHA), Serology (Elisa Ag-AC HIV), Hepatitis B serology (Elisa Ag-HBs), Hepatitis C serology (Elisa Ag-Ac-HCV) and HIV are negative. The patient was operated on for a conization within the histological final examination: squamous cell carcinoma in situ with microinvasive focus 3mm wide and 2mm deep with upper tumoral limit. A Total vulvectomy with hysterectomy without adnexal preservation was performed (figures 10 and 11) and post operating images (figures 12 and 13). The final histological results revealed:
1. Vulvectomy: Squamous cell carcinoma in situ with suspected invasive foci and associated low-grade VIN lesions, an immunohistochemical study is underway to evaluate the suspected invasive foci.
2. Hysterectomy: Squamous cell carcinoma in situ with foci of invasion. Recuts are underway to determine depth of invasion, posterior vaginal collar, isthmus and uterine wall uninvolved.

Discussion

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Colloid carcinoma is a rare histological entity of breast carcinoma, first described in 1982 by Geschickter [5]. It is a histological type that accounts for 7% of all malignant tumors of the breast after age 75 and 1% before age 35 [6].
In the literature, no significant difference has been found between colloid carcinoma, tubular carcinoma and breast CCI [7]. Pure colloid carcinomas often present as well-limited, mobile, even lobulated masses [8], which may thus be mistaken for benign formations; a sensation of suffler, crease or fluctuation is reported when colloid carcinomas of the breast are palpated [9]. The average tumor diameter is 1.5 cm, with extremes ranging from 0.3 to 19 cm [14]. The majority of tumors (96%) were TNM stage T1 or T2 [9].
On mammography, the presence of mucin translates into a relatively well-defined, low-density lobular mass. Occasionally, they may have partially faded or obscured margins. Up to 20% of lesions may be occult on mammography.
Calcification may be rare in pure mucinous types. On ultrasonography, mucinous carcinomas often show mixed echogenicity with mixed solid and cystic components, distal enhancement and microlobulated margins are commonly found in mucinous carcinomas. A mixed mucinous carcinoma tends to be more hypoechoic. On MRI, they are one of the few cancers to have very high signal intensity on T2-weighted images involving the mucinous component, compared with other malignant breast tumors [10].
Lymph node metastases in pure colloid carcinomas of the breast are their frequency increases with tumor size. Immunohistochemical studies of hormone receptors for estrogen and progesterone have often revealed a strong presence, particularly of estrogen (91% of cases) [11].
Treatment is based on surgery with or without adjuvant chemotherapy and hormone therapy. Conservative surgical treatment (lumpectomy) is recommended for T1 and small T2 cases, followed by radiotherapy [12]. Partial and accelerated irradiation of the breast is currently the most recommended after conservative surgery [3]. Exclusive radiotherapy may be attempted in inoperable forms for local or general reasons [12]. Poortmans [12] reported a 70% reduction in the risk of locoregional recurrence in patients treated with irradiation, irrespective of age, tumour characteristics and systemic administration of treatment.
Most authors agree on the favorable evolution of pure colloid carcinoma compared to other forms of breast malignancy, in particular ductal carcinoma which is by far the most common histological type [11]. The onset of metastases in pure colloid carcinomas is delayed late. The average time to metastasis is ten years [13].

Conclusion

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Bowen’s disease is an intraepidermal squamous cell carcinoma (carcinoma in situ) as originally described by John T. Bowen , this disease has the potential to progress to an invasive carcinoma and may affect both the skin and the mucous membrane of the glans penis, vulva, and oral mucosa [3].
Bowen’s disease may occur at any age, but patients older than 60 years are typical, and it can occur anywhere in the body, It usually affects sexually active adults, is more common in women, and is more common in people infected with the human immunodeficiency virus (HIV [4].
The lesion is more often solitary than multiple and may develop on exposed or non-exposed skin. The time interval from observed onset to diagnosis is approximately six years in the patient with a typical lesion [5].
The most prevalent symptoms were itching and pain. The striking feature of many cases, especially those involving the vulva, is pruritus. The lesions may be confused with a variety of dermatologic lesions such as syphilis, tuberculosis, eczema, Paget's disease and psoriasis [4].
The following characteristics should arouse suspicion of Bowen's disease: discrete or confluent, flat or slightly elevated, crusted papulo-squamous patches with circinate or serpiginous margins. Surrounding induration of the skin is minimal [5].
The vulvar lesions in the early stages may suggest leucoplakia or kraurosis [6] Diagnosis can only be made after multiple biopsies under local anesthesia with Kevorkian biopsy forceps. Invasion is not diagnosed in 7- 22% of cases. Cervicovaginal exploration is essential in the context of this pathology, due to an associated increase in dysplasia.
A variety of different modalities can be used to treat Bowen’s disease, which can be categorized as: surgical and destructive therapies, topical therapies, and non-surgical ablative therapies. Excision, cryotherapy, and curettage with or without cautery fall under the category of surgical and the destructive therapy.
Topical therapies involve the use of 5-fluorouracil (5-FU) or 5% imiquimod. Radiotherapy, laser ablation therapy, and PDT constitute non-surgical ablative therapies. The treatment decision making should taken into account multiple factors including the location, the size, and the number of the lesion, the clinician’s expertise, the patient’s age and the immune status, cosmetic outcome, and the patient’s preference [7].

References

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