Dermatology Online Journal

Rapidly growing extraocular sebaceous carcinoma occurring during pregnancy: A case report
Sudip Kumar Ghosh MD DNB DDermat¹, Debabrata Bandyopadhyay MD¹, Sandipan Gupta MS MCh², Gobinda Chatterjee MD¹, Arghyaprasun Ghosh MD¹
Dermatology Online Journal 14 (8): 8

1. Department Of Dermatology, Venereology, & Leprosy, R.G.Kar Medical College, Kolkata, India. dr_skghosh@yahoo.co.in
2. Department Of Plastic Surgery, Medical College, Calcutta, Kolkata, India

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Abstract

Sebaceous carcinoma is a very rare, aggressive, malignant tumor derived from the adnexal epithelium of sebaceous glands. It is traditionally classified into two groups: tumors arising from the ocular adnexa and those arising in extra-ocular sites. Despite the widespread anatomic distribution of sebaceous glands, extraocular sebaceous carcinoma is very rare, comprising only ¼ of all reported cases of sebaceous carcinoma. Extraocular sebaceous carcinoma in the post-auricular region or in the external auditory canal is very rarely described in the literature. A case of a rapidly growing sebaceous carcinoma of the pinna occurring in a pregnant woman is reported here for its rarity.

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Introduction

Sebaceous carcinoma (SC), which accounts for less than 1 percent of all cutaneous malignancies, is a rare, aggressive, malignant tumor derived from the adnexal epithelium of sebaceous glands. Sebaceous carcinoma is traditionally classified into 2 groups: tumors arising from the ocular adnexa, particularly the meibomian glands and glands of Zeiss, and those arising in extraocular sites. Ocular SC represents 1 percent to 5.5 percent of all eyelid malignancies. Despite the widespread anatomic distribution of sebaceous glands, extraocular sebaceous carcinoma is very rare comprising only 25 percent of all reported cases of sebaceous carcinoma [1].

A case of a rapidly growing sebaceous carcinoma of the pinna occurring in a pregnant woman is reported herein.

Report

Figure 1	Figure 2
Figure 1. A moist, yellowish-pink, cauliflower-like, oval-shaped growth on the outer aspect of left pinna with slightly verrucous surface is shown. There is evidence of mild bleeding from the lesion. Figure 2. Exudation of scanty purulent material is observed.

A 33-year-old previously healthy pregnant woman presented with a rapidly growing swelling on the outer aspect of her left pinna for 5 months, roughly the duration of her amenorrhea. She had a history of a small, asymptomatic, elevated lesion at that site since her childhood for which no medical opinion was sought. Over the preceding 5 months prior to the presentation, the lesion started growing rapidly. Although the growth was asymptomatic, there was a history of mild bleeding after trauma. Her past medical history was unremarkable and there was no family history of similar lesions. Examination revealed a moist, yellowish pink cauliflower-like, exuberant growth (Fig. 1) on the outer aspect of her left pinna with an oval configuration (3 x 4 cm). The surface of the lesion was slightly verrucous (Fig. 2) with exudation of scanty purulent material on manipulation. The lesion was non-tender and there was no regional lymphadenopathy. Systemic examination, including examination of breasts and rectal examination, revealed no other abnormality. Routine blood, urine, stool analysis, and biochemistry panel were within normal limits and ultrasonography of the whole abdomen did not reveal any pathology.

Histopathological examination of a lesional incisional biopsy showed lobules (Fig. 3) of finely vacuolated and foamy cells separated by scanty stroma, extending deeply into the subcutaneous tissue. The cells had large hyperchromatic nuclei and scattered mitoses. The overlying epidermis was thinned out at places but was otherwise normal. Based on the clinical picture and histopathology, the diagnosis of sebaceous carcinoma of the pinna was made. A wide excision and reconstructive surgery was performed within 2 weeks of presentation. The excised specimen also revealed similar histopathological features.

After 4 months, our patient delivered a healthy female baby by caesarian section. The postpartum period was uneventful. Upper and lower gastrointestinal endoscopy performed 3 months postpartum revealed no abnormality. There was no evidence of recurrence for 1½ years; the operation site was absolutely healthy (Fig. 4) and we are still in regular touch with the patient.

Figure 3	Figure 4
Figure 3. Healthy operative site during follow up at the end of 1½ years Figure 4. Histopathology showing lobules of malignant cells with vacuolated and foamy appearance separated by scanty stroma. (H&E, original magnification x100)

Discussion

Extraocular sebaceous carcinomas most commonly involve the head and neck region, in which sebaceous glands are most plentiful, followed by external genitalia, the parotid and submandibular glands, the external auditory canal, the trunk and upper extremity, sole, the dorsum of the great toe, and laryngeal or pharyngeal cavities [2]. Sebaceous carcinoma does appear to have a higher incidence in the Asian population. The sex distribution of extraocular SC appears to be about equal for male and female patients and the mean age of occurrence is 63 years [2]. The disease occurred in our patient at a much younger age.

The disease exhibits such a variety of clinical presentations and histologic growth patterns that the diagnosis is often delayed for months to years. It may appear on top of pre-existing dermatoses, like nevus sebaceous [3] and actinic keratosis [4], or may follow radiation therapy for other diseases [5]. It may also occur in Muir-Torre syndrome (MTS), characterized by occurrence of sebaceous tumors in association with visceral malignancies [6]. Regardless of the location, sebaceous malignancies must be considered as an aggressive neoplasm with a potential for regional and distant metastasis.

The clinical appearance of extraocular SC is not pathognomonic, but the lesion may be a pink to red-yellow nodule. Our patient had the undiagnosed primary lesion since her childhood and this tumor showed rapid growth during pregnancy. In view of the fact that sebaceous carcinoma can occur during generalized immunosupression, aggressive growth during pregnancy in our patient can be similarly explained. Bleeding has been reported in approximately 1/3 of the cases of sebaceous carcinoma [2]. Increased vascularity associated with pregnancy might also be a contributory factor of recurrent bleeding in our patient. Extraocular sebaceous carcinoma, in association with nevus sebaceous, in the post-auricular region [7] or in the external auditory canal is very rarely described in the literature. Extraocular sebaceous carcinoma may clinically resemble pyogenic granuloma, hemangioma, or squamous cell cancer. Although extraocular sebaceous carcinoma, compared to orbital SC, is generally considered less aggressive, visceral metastasis has been reported [8]. Draining lymph nodes may be involved in a few cases.The possibility of MTS must be considered in every case of sebaceous tumor. Criteria for the diagnosis of MTS include the presence of at least one sebaceous adenoma, sebaceous epithelioma, or sebaceous carcinoma (sebaceous hyperplasia and nevus sebaceous of Jadassohn are generally excluded), and at least one visceral cancer [9].

In our patient there was neither any regional lymphadenopathy nor any evidence of other internal malignancy as associated with MTS. However, immunohistochemistry, an important aid to the diagnosis of MTS, could not be performed on the tumor to look for expression of mismatch repair genes because of local nonavailability and financial constraints. Because cutaneous features may precede the internal malignancies associated with MTS, regular follow-up is required on a long-term basis.

Histopathologically two distinct forms of sebaceous carcinoma are seen, namely, true sebaceous gland carcinoma and basal cell epithelioma with sebaceous differentiation. The former is more malignant with well-circumscribed lobules composed of atypical sebaceous cells located deep in the dermis. On the other hand, the latter is less aggressive and its occurrence at a younger age suggests a nevoid or hamartomatous origin; the epidermis is thinned with lobulated downgrowths occurring from the basal layer into the dermis. Treatment of sebaceous carcinoma requires wide surgical excision with removal of involved regional lymph nodes. There are diverse opinions regarding the use of postoperative irradiation or chemotherapy [10]. Although complete excision with reconstructive surgery was performed, our patient requires long-term follow-up to determine recurrence or development of other internal malignancies.

References

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