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Giant extraocular sebaceous carcinoma: case report and a brief review of a literature

  • Author(s): Torres, José Saulo
  • Amorim, Ana Cristina
  • Hercules, Flavio Marcondes
  • Kac, Bernard Kawa
  • et al.
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Giant extraocular sebaceous carcinoma: case report and a brief review of a literature
José Saulo Torres1 MD, Ana Cristina Amorim1 MD, Flavio Marcondes Hercules1 MD, Bernard Kawa Kac2 MD
Dermatology Online Journal 18 (11): 7

1. Department of Dermatology, Mário Kröeff Hospital, Associação Pele Saudável, Rio de Janeiro, Brazil
2. Section of dermatopathology at Anatomical Lab, Rio de Janeiro, Brazil


Abstract

Extraocular sebaceous carcinoma (ESC) is a rare, aggressive malignant tumor that originates from the adnexal epithelium of the sebaceous gland. We present herein one of the largest cases reported in the literature until now. We also review briefly the relevant aspects including clinical presentation, differential diagnosis, histopathological features, inmunohistochemical profile, and therapeutic options.



Introduction

A 59-year-old previously healthy male, was seen with an enormous, rapidly growing, painless, ulcer localized in the anterior chest area. The lesion initially appeared two years earlier and had relatively slow growth at first, with a history of mild bleeding after trauma. His past medical history was unremarkable and there was no family history of relevance.

At physical examination there was on the anterior chest wall an ulcer of 20 cm x 18 cm, that was well-circumscribed and had slightly elevated bright red borders (Figure 1).


Figure 1Figure 2
Figure 1. An ulcerated lesion of 20 x 18 cms, with well-circumscribed and slightly elevated bright red edges.

Figure 2. A non-encapsulated tumor composed of sheets of cells separated by a fibrovascular stroma. The cells of the tumor are extending through the dermis and showing atypical cells with enlarged, hypercromatic, pleomorphic nuclei with finely peripherically vacuolated cytoplasm. (HE x400)

Figure 3Figure 4
Figure 3. Atypical cells showing variable sebaceous differentiation with centrally situated nuclei and vacuolated cytoplasm. (HE x1000)

Figure 4. Atypical cells showing variable sebaceous differentiation with centrally situated nuclei and vacuolated cytoplasm (HE x1000)

Initial diagnostic hypotheses included invasive cutaneous basal cell carcinoma, pyoderma gangrenosum, and cutaneous metastasis. An incisional biopsy was performed for histopathological examination with hematoxylin and eosin staining (Figures 2 through 4) revealing a non-encapsulated tumor composed of sheets of cells separated by a fibrovascular stroma. The cells of the tumor were extended through the dermis and showed variable sebaceous differentiation with enlarged, hypercromatic, pleomorphic nuclei with finely peripherically vacuolated cytoplasm.


Figure 5
Figure 5. Tumoral cells present multifocal reactive expression of Epithelial Membrane Antigen (x40)

Immunohistochemistry revealed multifocal reactivity for Epithelial Membrane Antigen (EMA) and CA15.3. Staining was negative for BerEp4 and S100 protein confirming the diagnosis of undifferentiated sebaceous carcinoma (Figure 5).

Contrast-enhanced computed tomography (CT) of the chest, abdomen, and pelvis, colonoscopy, and upper digestive endoscopy were performed and were negative, which most likely excludes an association with the Muir-Torre syndrome (MTS).

Because of the size of the primary lesion surgery was not considered as a therapeutic option. The patient was referred to the radiotherapy department for palliative treatment.


Discussion

Sebaceous carcinoma (SC) accounts for less than 1 percent of all cutaneous malignancies. It affects predominantly elderly people with a median age of 73 years at the time of diagnosis, showing a slightly predominance of men 54 percent and predilection for caucasians [1, 2].

SC can present as an isolated entity or it could be a part of a manifestation of the MTS [3].

MTS is an autosomal dominantly inherited disease defined as the presence of one or more sebaceous neoplasms in association with at least one internal malignancy. The skin neoplasms can occur prior, concurrent with, or after the diagnosis of visceral neoplasms [4, 5].

Sebaceous carcinoma can be classified either as ocular (OSC) or extraocular sebaceous carcinoma (ESC) depending on the involvement of the eyelid structures.

OSC represents approximately 75 percent of all SC; the Meibomian gland is the site of origin for approximately half of all cases of OSC. The upper lid is approximately three times more frequently involved than the lower [3]. The ESC counts approximately for the other 25 percent of cases [6] and the most frequent localization is the head and neck because of the presence of the highest concentration of sebaceous glands. Nevertheless, sebaceous carcinoma can arise from any site where sebaceous glands are present [7, 8].

The histopathological exam shows a dermal and nonencapsulated tumor separated by a fibrovascular stroma [9]. The tumor cells exhibit high mitotic activity, with variable sebaceous differentiation [3, 9].

EMA, BER-EP4, CA15.3, androgen receptor, and adipophilin are some immunohistochemical markers that appear to be consistently positive in these tumors and help to differentiate them from other poorly differentiated appendage tumors [9, 10, 11].

SC is an aggressive carcinoma with local recurrence that varies from 9 to 36 percent in five years with surgical excision [6]. Nodal metastasis occurs in approximately 8 – 28 percent of cases [6, 12]. The main prognostic factor is the time to diagnosis; when the diagnosis is made in the first six months the mortality rate is about 14 percent. After the sixth month the mortality rates rises to 38 percent [6].

The first line treatment is surgical excision with wide margins. Mohs surgery appears to be a good alternative therapeutic option [13]. Radiotherapy also has been used for curative intent and has an important role for palliative cases [6, 14].

This is one of the largest cases reported of sebaceous carcinoma in the medical literature, confirming the local aggressive behavior of the tumor and highlighting the importance of early diagnosis.

ACKNOWLEDGEMENTS: The authors wish to express their sincere gratitude to Control Lab and Anatomical Lab for helping them with the pathology high resolution pictures of this paper.

References

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