British Journal of Derrmtoloffy (1991) 124, 28 3-284.
Aix)Ni.s UOO7O96391OOO65B
Sebaceous carcinoma: an unusual cause of a rapidly enlarging rhinophyma R.).MOTLEY. A.F.DOUGLAS-JONI-S* AND P.J.A.HOLT IklMirtments ol Dermtitolonff iind ^hitlwhtin, Universiln llmpita! of Wales. Hcutli I'ark. Ciirdijf. U.K. Accepted for publication 22 August
Summary
We describe a patient who developed sebaceous carcinoma within a long-standing rhinophyma with rapid enlargement of the lesion. We believe the development of this tumour was a random octiirrence but the possibility of tumour development should be considered in any rhinophyma showing a rapid mcrease in size.
Most cases of true sebaceous gland carcinoma develop on the eyelids, but extra-ocular lesions may present as nodular tumours on the head and neck of elderly patients. These tumours may metastasize and should be treated with wide local excision. We describe a patient who developed this unusual tumour in a long-standing rhinophyma.
Case report
The tumour consisted of irregular lobules of cells separated by tine tibrovascular stromal septae (Fig. 2). Many of the cells had abundant foamy cytoplasm and there was considerable variation in the size and shape of the nuclei. A number of mitotic figures were also seen. Stains for keratin, low-molecular-weigbt keratin (Cam S 2). glycogen. mucin. prostatic acid phosphatase and S-l()() were negative. Stains for carcinoembryonic antigen showed strong positive staining in individual and groups of tumour cells but the majority of the tumour
A 91 -year-old man presented with an ulcerated massive rhinophyma (Fig. 1). The lesion had been present for over 20 years and had caused him little concern. Over the preceding 12 months, however, it had enlarged progressively, culminating in ulceration and a persistent serous discharge. The increased size of the lesion had produced difficulties in breathing, and prompted his referral for treatment. He had a past medical history of a benign oesphageal diverticulum and intermittent dysphagia. but was otherwise well. On examination, the rhinophyma measured 6 cm in diameter and there was ulceration distally and redundant tissue partially occluding the nares. Physical examination was otherwise unremarkable. In view of the size and vascularity of the lesion he was treated with a carbon dioxide laser. Under general anaesthesia an attempt was made to pare the lesion using a focused carbon dioxide laser beam. Progress was very slow and subsequently changed to cutting diathermy to resect the hulk of the tissue. The patient was symptomatically much improved by this procedure. The resected material was submitted for routine histological examination and we were surprised to discover the presence oi a tumour within the rhinophyma tissue. Correspondence: l)r R.J.Motley
Figure 1. Prcopertitive frontal view of the iilceritled rhinophymii.
283
284
R.J.MOTLEYcl«/.
Discussion
Figured. Hislological appearanctof the tumour composetJ ol'iobules of cells with aliundant foamy cytoplasm. There is coiisideriible variation in nuclear size and shape, and several mitotic figures are seen.
was negative. Fat stains could not be performed as all the material had been embedded in paraffin. The tumour was unconnected with the epidermis and there was no evidence of basal cell carcinoma-like change in any of the sections. On the basis of these findings a histological diagnosis of sebaceous gland carcinoma was made. Iixtcnsive investigations of the patient revealed no evidence of malignancy elsewhere in the body. He died 4 months later from bronchopncumonia: a post-mortem examination was not performed.
Sebaceous carcinoma is an uncommon neoplasm most frequently seen on the eyelids where it accounts for 1% of malignant tumours at this site.' Extra-ocular sebaceous carcinomas appear to arise almost exclusively in middle-aged or elderly patients with a peak in the seventh decade of life. Males are affected twice as frequently as females. The majority of the lesions arise on the head and neck where they usually appear as a nodule which may ulcerate.- A patient with sebaceous carcinoma of the nose, said clinically to resemble a rhinophyma. has been previously described.' Our patient had a rhinophyma for many years, but it is signiiicant that he had noticed progressive enlargement over the 12 months prior to his presentation. This enlargement was clearly due to the growing tumour. There arc no specific markers lor sebaceous carcinoma and the diagnosis is made by its distinguishing features of sebaceous cells with foamy cytoplasm and the cellular features of malignancy. The differential diagnosis includes sebaceous adenoma, basal cell carcinoma with sebaceous differentiation and metastatic clear cell tumour. Many different tumours may produce clear cell' metastases. the most common being renal and prostatic carcinomas. True sebaceous carcinomas have the ability to metastasize and should be excised with a wide margin. The paucity of previous reports suggests that the development of sebaceous carcinoma in rhinophyma tissue is purely a random occurrence. Rapid enlargement of a rhinophyma should be considered as a sign of possible tumour development within the tissue.
References 1 Riu) N. Hidayat AA. .McLean IW. 'Zimmerman I,H. Sebaceous carcinomas of the ocular adnexae. Hum Pallid 19S2: 1 J: I t 3-22. 2 Wick MR. Goellner JR. Wolfe JT, Su WPl). Adru-val carcinomas of the skin. II. Fxtraocular sebaceous carcinomas. Cniu'i-r 198S; 56: 1 1(15-72. 3 Rulon DB, Helwig KI3, Cutaneous sebaceous neoplasms. Cancer 1974: 3J: 82-102.
Aix)Ni.s UOO7O96391OOO65B
Sebaceous carcinoma: an unusual cause of a rapidly enlarging rhinophyma R.).MOTLEY. A.F.DOUGLAS-JONI-S* AND P.J.A.HOLT IklMirtments ol Dermtitolonff iind ^hitlwhtin, Universiln llmpita! of Wales. Hcutli I'ark. Ciirdijf. U.K. Accepted for publication 22 August
Summary
We describe a patient who developed sebaceous carcinoma within a long-standing rhinophyma with rapid enlargement of the lesion. We believe the development of this tumour was a random octiirrence but the possibility of tumour development should be considered in any rhinophyma showing a rapid mcrease in size.
Most cases of true sebaceous gland carcinoma develop on the eyelids, but extra-ocular lesions may present as nodular tumours on the head and neck of elderly patients. These tumours may metastasize and should be treated with wide local excision. We describe a patient who developed this unusual tumour in a long-standing rhinophyma.
Case report
The tumour consisted of irregular lobules of cells separated by tine tibrovascular stromal septae (Fig. 2). Many of the cells had abundant foamy cytoplasm and there was considerable variation in the size and shape of the nuclei. A number of mitotic figures were also seen. Stains for keratin, low-molecular-weigbt keratin (Cam S 2). glycogen. mucin. prostatic acid phosphatase and S-l()() were negative. Stains for carcinoembryonic antigen showed strong positive staining in individual and groups of tumour cells but the majority of the tumour
A 91 -year-old man presented with an ulcerated massive rhinophyma (Fig. 1). The lesion had been present for over 20 years and had caused him little concern. Over the preceding 12 months, however, it had enlarged progressively, culminating in ulceration and a persistent serous discharge. The increased size of the lesion had produced difficulties in breathing, and prompted his referral for treatment. He had a past medical history of a benign oesphageal diverticulum and intermittent dysphagia. but was otherwise well. On examination, the rhinophyma measured 6 cm in diameter and there was ulceration distally and redundant tissue partially occluding the nares. Physical examination was otherwise unremarkable. In view of the size and vascularity of the lesion he was treated with a carbon dioxide laser. Under general anaesthesia an attempt was made to pare the lesion using a focused carbon dioxide laser beam. Progress was very slow and subsequently changed to cutting diathermy to resect the hulk of the tissue. The patient was symptomatically much improved by this procedure. The resected material was submitted for routine histological examination and we were surprised to discover the presence oi a tumour within the rhinophyma tissue. Correspondence: l)r R.J.Motley
Figure 1. Prcopertitive frontal view of the iilceritled rhinophymii.
283
284
R.J.MOTLEYcl«/.
Discussion
Figured. Hislological appearanctof the tumour composetJ ol'iobules of cells with aliundant foamy cytoplasm. There is coiisideriible variation in nuclear size and shape, and several mitotic figures are seen.
was negative. Fat stains could not be performed as all the material had been embedded in paraffin. The tumour was unconnected with the epidermis and there was no evidence of basal cell carcinoma-like change in any of the sections. On the basis of these findings a histological diagnosis of sebaceous gland carcinoma was made. Iixtcnsive investigations of the patient revealed no evidence of malignancy elsewhere in the body. He died 4 months later from bronchopncumonia: a post-mortem examination was not performed.
Sebaceous carcinoma is an uncommon neoplasm most frequently seen on the eyelids where it accounts for 1% of malignant tumours at this site.' Extra-ocular sebaceous carcinomas appear to arise almost exclusively in middle-aged or elderly patients with a peak in the seventh decade of life. Males are affected twice as frequently as females. The majority of the lesions arise on the head and neck where they usually appear as a nodule which may ulcerate.- A patient with sebaceous carcinoma of the nose, said clinically to resemble a rhinophyma. has been previously described.' Our patient had a rhinophyma for many years, but it is signiiicant that he had noticed progressive enlargement over the 12 months prior to his presentation. This enlargement was clearly due to the growing tumour. There arc no specific markers lor sebaceous carcinoma and the diagnosis is made by its distinguishing features of sebaceous cells with foamy cytoplasm and the cellular features of malignancy. The differential diagnosis includes sebaceous adenoma, basal cell carcinoma with sebaceous differentiation and metastatic clear cell tumour. Many different tumours may produce clear cell' metastases. the most common being renal and prostatic carcinomas. True sebaceous carcinomas have the ability to metastasize and should be excised with a wide margin. The paucity of previous reports suggests that the development of sebaceous carcinoma in rhinophyma tissue is purely a random occurrence. Rapid enlargement of a rhinophyma should be considered as a sign of possible tumour development within the tissue.
References 1 Riu) N. Hidayat AA. .McLean IW. 'Zimmerman I,H. Sebaceous carcinomas of the ocular adnexae. Hum Pallid 19S2: 1 J: I t 3-22. 2 Wick MR. Goellner JR. Wolfe JT, Su WPl). Adru-val carcinomas of the skin. II. Fxtraocular sebaceous carcinomas. Cniu'i-r 198S; 56: 1 1(15-72. 3 Rulon DB, Helwig KI3, Cutaneous sebaceous neoplasms. Cancer 1974: 3J: 82-102.
