An Unusual Case of Recurrent Cutaneous Carcinomas Over Extragenital Multicentric Pigmented Bowen’s Disease

Abstract

Bowen’s disease is a slowly progressive squamous cell carcinoma (SSC) in situ with high potential for malignant transformation. In this case, we describe a patient with multicentric Bowen’s disease for the past 26 years, developing growths over his left buttock. The patient had a previous history of growth developing over his right thigh, and was diagnosed with metatypical basal cell carcinoma (BCC). The points that make this case noteworthy are recurrent cutaneous carcinomas over the multicentric generalized occurrence of in situ SCC of extragenital type, the rarity of the site, and the nature of its morphological presentation in the skin of color.

Introduction

Bowen’s disease is a premalignant lesion regarded as squamous cell carcinoma (SCC) in situ.[1] The propensity for malignant transformation is 3–5% in the extragenital type and 10% in the genital type,[2] due to which early diagnosis and incessant monitoring are indispensable.[3] We describe a case of a senior man who, over a period of 26 years, developed multiple cutaneous carcinomas over the extragenital sites, fitting the multicentric form of the disease. The unusual site and history of presentation and the presence of recurring cutaneous carcinomas over underlying multicentric premalignant lesions make this case unique.

Case Report

A sixty-year-old man with Fitzpatrick skin phototype IV reported a few ulceroproliferative, non-tender growths over his left buttock for six months with no prior history suggestive of immunosuppression and exposure to heavy metals or radiation [Figure 1a and b]. Dermoscopy of the lesion revealed fissuring, erythema, and thick adherent scaling and crusting [Figure 1c]. Immunohistochemistry of the lesion exhibited PanCK (pan-cytokeratin), p40 (Delta Np63, a truncated product of p63), and Ki-67 (proliferative index marker) positivity, substantiating the diagnosis of multifocal microinvasive SCC. Dysplastic squamous cells were observed to invade the papillary dermis in groups [Figure 1d and e]. A wide local excision was performed with primary closure of the lesions.

Figure 1.

(a) Ulceroproliferative growths over the left buttock (b) Enlarged image of the same with fissuring and thick scaling (c) (DermLite Foto 3 Gen polarized): dermoscopic image of the growth with yellow-white scaling and fissuring (d) Dysplastic cells (black arrow) with large blood vessel (yellow arrow) (H&E, 10x) (e) Dysplastic cells (black arrow) with mitotic figures and large blood vessels (yellow arrow) in the upper dermis (H&E, 40x)

According to the patient’s earlier medical records, he had reddish-brown scaly lesions all over his body for 26 years. They were well-defined, erythematous, scaly, and hyperpigmented plaques of varied sizes [Figure 2a-e]; histopathology supported the diagnosis of an in situ tumor with a classic “windblown” appearance [Figure 2f-i]. Over his right thigh, he eventually developed a butterfly-shaped plaque 7 cm by 3 cm in size [Figure 3a]. Liquid nitrogen cryotherapy with two freeze–thaw cycles was administered [Figure 3b]. Significant regression was observed in the lesions. The patient abandoned follow-up after the lesion improved. The patient subsequently presented with a tender ulceroproliferative mass over the same lesion, that bled on touch [Figure 3c and d]. Records showed that histopathology from the right thigh mass revealed aggressive nests of cancerous cells penetrating deeper tissues. The localized clumps of squamous cells and keratin pearls in the center of the cell nests, which had basaloid rimming, were suggestive of metatypical basal cell carcinoma (BCC) [Figure 3e and f].

Figure 2.

(a) Multicentric in situ squamous cell carcinoma over the chest (b) Multicentric in situ squamous cell carcinoma over the back (c) Multicentric in situ squamous cell carcinoma over the left forearm dorsum (d) Multicentric in situ squamous cell carcinoma over the left thigh (e) Multicentric in situ squamous cell carcinoma over the right retro-auricular area (f) (H and E 10X): Dysplastic cells restricted to the epidermis, showing the classic “windblown” appearance (black bracket) (g) (H and E 40X): Dysplastic cells restricted by the basement membrane of the epidermis (black arrows) (h) (H and E 40X): Dysplastic cells restricted by the basement membrane of the epidermis (black arrows) (i) (H and E 40X): Dysplastic cells showing the classic “windblown” appearance (yellow arrow)

Figure 3.

(a) In situ tumor over the right thigh (b) Following cryotherapy (c) Fungating mass that recurred over the same site on the right thigh (d) Following surgical excision of the tumour (e) (H and E 10X): Cell nests having basaloid rimming (black arrow) with focal aggregates of squamous cells and keratin pearls in the center (yellow arrow) (f) (H and E 40X): Typical clefting between the basaloid cells (black arrow)

Discussion

Bowen’s disease is a gradually progressive in situ cutaneous malignancy whose period for full expression can vary from 2 years to a maximum of 40 years.[4,5] It is predominantly solitary and localized over photo-exposed areas, especially over the face and extremities.[5,6,7] Our patient had lesions over photo-protected areas such as the trunk, waist, back, and buttocks, in contrast to most reports.[8]

Among the extragenital types of Bowen’s disease, 3–5% of cases transform into SCC, making it the most typical type of malignancy of Bowen’s disease.[2] Rarely do Bowen’s lesions transform into BCC, and the occurrence of the metatypical type of BCC on Bowen’s is a rarity and a case that has not been reported so far. Metatypical BCC or basosquamous BCC is an aggressive form of BCC than classic BCC, with a reported incidence of 1.2%–2.7% and an increased risk of metastases and recurrence.[9]

Histologically, BCC shows basaloid nests with artifactual clefting in between the nests, SCC shows features of dyskeratosis such as squamous keratin pearls with nuclear pleomorphism and hyperchromatism, and basosquamous carcinoma has a transition zone between basaloid and squamoid cells showing both basaloid and squamoid differentiation.[10]

On immunohistochemistry, BerEp4 (EpCAM, Epithelial Cell Adhesion Molecule), a monoclonal antibody against glycoproteins of epithelial membrane, and AE1/AE3, a PanCK antibody against epithelial cytokeratin markers AE1 and AE3, are markers for metatypical BCC, the former being the classic marker. The cytokeratin and cell adhesion molecule markers, AE1/AE3 and CAM5.2 (Cytokeratin marker against secretory glandular epithelium and epithelial tumours), respectively, are markers for SCC and the squamoid part of metatypical BCC, which also show variable staining for EMA (epithelial membrane antigen).[9,11]

The mainstay of treatment of SCC in this case remains surgical excision. Mohs micrographic surgery has a high cure rate and saves the most tissue possible. Electrodesiccation and curettage, radiation therapy, cryosurgery, topical treatment such as imiquimod, photodynamic therapy, and systemic therapy are additional options.[12]

Our patient presented with the classic features of multicentric generalized pigmented Bowen’s disease but developed microinvasive SCC with a recorded history of basosquamous carcinoma. Though Bowen’s disease may be called SCC in situ, it may mimic invasive carcinomas because both present in a very similar fashion with itching, pain, and bleeding at presentation.[13] Immunohistochemistry helps in determining the type of tumor, where, in this case due to financial restrictions, the patient could not afford to undergo the same for the excised tumor.

Any case of Bowen’s disease, be it solitary or multicentric, should be kept under surveillance to ascertain its transformation into malignancy at the earliest and enhance the patient’s prognosis and survival.

In conclusion, we would like to draw attention to the following significant characteristics in this case:

• The rarity of extragenital generalized multicentric Bowen’s disease.

• The occurrence of the lesions over photo-protected areas such as the thighs, chest, back, and buttocks.

• The recurrence of cutaneous nonmelanoma malignancies over such in situ tumors.

• The pigmented type has yet to be extensively debated in the literature, especially in the skin of color.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.