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Extramammary Paget's disease

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Condyloma acuminatum associated with syringocystadenoma papilliferum.Am J Dermatopathol. 1994 Dec;16(6):628-30.

A 70-year-old man presented with a keratotic lesion of the right buttock. Histologic examination revealed an endophytic cystic and papillary lesion of the dermis. The papillae were lined by two layers of cuboidal epithelial cells with a dense plasmacytic infiltrate of the stroma, consistent with syringocystadenoma papilliferum (SCAP). The overlying epidermis showed acanthosis, papillomatosis, and hyperkeratosis. There were multiple foci of prominent koilocytotic atypia. In situ DNA probes for HPV type 6/11 were positive in many epidermal nuclei. The concurrent occurrence of SCAP and CA may be coincidental; however, the occurrence of CA may be related to the environment at the surface of the SCAP. Syringocystadenoma papilliferum often occurs in association with nevus sebaceus (NS), but it has also been reported from most body sites. When it is not seen in association with NS, the epidermis overlying SCAP may be hyperkeratotic or verrucous. The verrucous changes in our case had features of condyloma acuminatum (CA), which were confirmed by in situ DNA probes.

Syringocystadenocarcinoma papilliferum: case report and immunohistochemical comparison with its benign counterpart.J Am Acad Dermatol. 2001 Nov;45 (5): 755-9.

Syringocystadenocarcinoma papilliferum (SCACP) is the malignant counterpart of syringocystadenoma papilliferum (SCAP), although only a few cases have been reported in the literature and its clinical and histologic characteristics are not well known. We report a case of SCACP that started as an enlarging nodule over 10 years in the perianal area of a 61-year-old man. Macroscopically, the lesion was a black exophytic tumor, 6 cm in diameter, with a granular surface. Histologically, it was an in situ adenocarcinoma, showing cytologic atypia and pagetoid spread in the surrounding epithelia, although the clinicopathologic features were distinct from extramammary Paget's disease. The tumor lacked the typical double-layered pattern of SCAP but had some similar histopathologic features to SCAP. Decapitation secretion was apparent and there was positive immunoreactivity to epithelial membrane antigen and human milk fat globules subclass 2. SCACP is a rare cutaneous tumor but nevertheless represents a specific dermato- pathologic entity.

Syringocystadenoma Papilliferum

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Syringocystadenoma papilliferum. A case located on the knee.Ann Dermatol Venereol. 1994;121(4):323-4.

Syringocystadenoma papilliferum is an infrequent hamartoma of the sweat glands, usually of the scalp or temporal area. We observed a syringocystadenoma papilliferum in a young patient from Senegal. The lesion was unique and presented as a round 4 cm plaque with a red smooth surface. The diagnosis was based on the histologic examination. Complete surgical exeresis was performed. Localization on the knee is very unusual.

Syringocystadenoma papilliferum as a differential diagnosis of a carcinoma of the lower lip. Z Hautkr. 1985 Feb 15;60(4):348-55.

A 56-year-old patient showed a skin-colored tumor of the lower lip which had been photographically documented since his 10th year of age. On account of the localisation, increase of size during the last few months, as well as irregular epithelial hyperplasia observed in a punch biopsy from the periphery, we suspected the tumor to be a squamous cell carcinoma. Histological examination, however, revealed syringocystadenoma papilliferum.

Syringocystadenoma papilliferum: light and electron microscopic studies.Acta Derm Venereol. 1976;56(5):327-36.

Light microscopic and electron microscopic studies of a lesion displaying a typical histopathological picture of syringocystadenoma papilliferum (S.P.) are reported. Light microscopic serial sections showed that the tumor parenchyma extended to the epithelium just above the intrafollicular sebaceous duct. The tumor thus presumably develops from the epithelial region corresponding to the intrafollicular duct of the apocrine sweat gland. Electron microscopy revealed a non-keratinized intracytoplasmic cavity and intercellular canaliculi apparently formed as a continuation of this cavity. Keratinized cells were absent from the glandular and duct epithelium and even from the superficial epithelial portion of acanthotic areas. Neither myoepithelial cells nor secretory granules were identified in areas showing tubular, glandular, or sinusoidal structures. It was concluded that the tumor differentiates towards both the intrafollicular and intradermal duct of the embryonic apocrine sweat gland apparatus.

 
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