British Journal of Dermatology (1978) 98, 645.
Primary mucinous carcinoma of the skin DANIEL J.SANTA-CRUZ, JAMES H.MEYERS, DOUGLAS R.GNEPP AND BLANCA M. PEREZ* Department of Pathology, Division of Surgical Pathology, Washington University Medical Center, Barnes Hospital Plaza, St Louis, Missouri 63110, U.S.A. Accepted for publication 7 December 1977
SUMMARY
Only twenty-seven cases of primary mucinous carcinoma of the skin have been reported. Three additional cases are included in this paper. The clinical presentation is distinctive: they are found most commonly in middle-aged, hlack males in the head and neck region as painless nodules, usually less than 3 cm in diameter. Previous reports have indicated the probable sweat gland origin of these tumours; one of our cases demonstrates a transition from a solid hidradenoma-Hke pattern to nests of tumour cells floating in mucin lakes, adding support for the sweat gland hypothesis. The clinical behaviour is relatively benign; late recurrences are common but metastases are rare. Only one case had widespread metastases. Although the histological appearance is distinctive, other primary sites first must be excluded. Local excision is the treatment of choice.
Primary mucin producing carcinomas ofthe skin are rare; most mucinous carcinomas presenting in the skin are subsequently found to be metastatic. The largest series of primary mucinous carcinomas, reported by Mendoza & Helwig (1971), comprised 14 cases spanning 27 years; only thirteen others have been reported (See Table i). The sweat-gland origin of these tumours has been supported by several authors using histochemical staining reactions, enzyme histochemistry, and electron microscopy. A pattern of nests and cords of tumour cells forming small lumens floating in large mucin lakes has been seen in many instances. The purpose of this paper is to include three additional cases, two with long term follow-up. One of our cases will demonstrate a transition from a solid hidradenomalike pattern to the typical pattern noted above, adding additional support to the theory of sweat gland histogenesis. The pathological implications of such a transitional phase will be discussed. A review of the previously reported cases with a discussion of the clinical characteristics, and gross and microscopic pathological presentation, is also presented. MATERIALS AND METHODS
The three current cases were taken from the files of the Division of Surgical Pathology, Barnes and Jewish Hospitals, St Louis, Missouri. Sections from the tumours were stained with haematoxylin * Jewish Hospital, St Louis, Missouri 63 n o , U.S.A. 0007-0963/78/0600-0645 S02.00 (g) 1978 British Association of Dermatologists
645
646
D.J.Santa-Cruz et ai TABLE I
Case
Age 26
59 69 76 64 66
Mendo2a& Helwig (1971) -
72 53 57 8 43 69 59
Sex Race M M M M M M
B J — W B W
F M M M
w B
F M
B B — W B
M
Lermox, Pearse & Richards(1952) Grossman & Izuno (1974)
57 67
M F
W W
65
M
w
Rodrigucs et al. (1973)
52
M
B
Lund (1957) Wolfe & Stemberg (1954)
53 62
M F
B —
Miller (1967) Metz ei al. (1974)
60 63
M M
— —
F
—
M
—
70
M
—
— —
— — W
M
' 44 Headington (1977) Berg & McDivitt C1968)
Yeung & Stinson (1977)
59
— — M
Ours
80
M
B B
M
B
Primary mucinous carcinoma of the skin DANIEL J.SANTA-CRUZ, JAMES H.MEYERS, DOUGLAS R.GNEPP AND BLANCA M. PEREZ* Department of Pathology, Division of Surgical Pathology, Washington University Medical Center, Barnes Hospital Plaza, St Louis, Missouri 63110, U.S.A. Accepted for publication 7 December 1977
SUMMARY
Only twenty-seven cases of primary mucinous carcinoma of the skin have been reported. Three additional cases are included in this paper. The clinical presentation is distinctive: they are found most commonly in middle-aged, hlack males in the head and neck region as painless nodules, usually less than 3 cm in diameter. Previous reports have indicated the probable sweat gland origin of these tumours; one of our cases demonstrates a transition from a solid hidradenoma-Hke pattern to nests of tumour cells floating in mucin lakes, adding support for the sweat gland hypothesis. The clinical behaviour is relatively benign; late recurrences are common but metastases are rare. Only one case had widespread metastases. Although the histological appearance is distinctive, other primary sites first must be excluded. Local excision is the treatment of choice.
Primary mucin producing carcinomas ofthe skin are rare; most mucinous carcinomas presenting in the skin are subsequently found to be metastatic. The largest series of primary mucinous carcinomas, reported by Mendoza & Helwig (1971), comprised 14 cases spanning 27 years; only thirteen others have been reported (See Table i). The sweat-gland origin of these tumours has been supported by several authors using histochemical staining reactions, enzyme histochemistry, and electron microscopy. A pattern of nests and cords of tumour cells forming small lumens floating in large mucin lakes has been seen in many instances. The purpose of this paper is to include three additional cases, two with long term follow-up. One of our cases will demonstrate a transition from a solid hidradenomalike pattern to the typical pattern noted above, adding additional support to the theory of sweat gland histogenesis. The pathological implications of such a transitional phase will be discussed. A review of the previously reported cases with a discussion of the clinical characteristics, and gross and microscopic pathological presentation, is also presented. MATERIALS AND METHODS
The three current cases were taken from the files of the Division of Surgical Pathology, Barnes and Jewish Hospitals, St Louis, Missouri. Sections from the tumours were stained with haematoxylin * Jewish Hospital, St Louis, Missouri 63 n o , U.S.A. 0007-0963/78/0600-0645 S02.00 (g) 1978 British Association of Dermatologists
645
646
D.J.Santa-Cruz et ai TABLE I
Case
Age 26
59 69 76 64 66
Mendo2a& Helwig (1971) -
72 53 57 8 43 69 59
Sex Race M M M M M M
B J — W B W
F M M M
w B
F M
B B — W B
M
Lermox, Pearse & Richards(1952) Grossman & Izuno (1974)
57 67
M F
W W
65
M
w
Rodrigucs et al. (1973)
52
M
B
Lund (1957) Wolfe & Stemberg (1954)
53 62
M F
B —
Miller (1967) Metz ei al. (1974)
60 63
M M
— —
F
—
M
—
70
M
—
— —
— — W
M
' 44 Headington (1977) Berg & McDivitt C1968)
Yeung & Stinson (1977)
59
— — M
Ours
80
M
B B
M
B
