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Background

Syringocystadenoma papilliferum (SP) is usually first noted at birth or during early childhood and occurs most commonly on the scalp, consisting of either one papule, several papules in a linear arrangement, or a solitary plaque. At puberty, SP may increase in size and become papillomatous and crusted. It is frequently associated with nevus sebaceus. Basal cell carcinomas may occur in up to 10% of cases.

OUTLINE

Pathogenesis  
Histopathological Features and Variants  
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PATHOGENESIS CHARACTERIZATION

Syringocystadenoma Papilliferum A Study of Potential Tumor Suppressor Genes

Roland Böni, etal.

Am J Dermatopathol 2001;23:87-89 Abstract quote

Syringocystadenoma papilliferum (SP) is a benign tumor most commonly located on the scalp or face, which frequently arises from a nevus sebaceus (NS). Transition of SP to basal cell carcinoma (BCC) and, albeit rarely, to metastatic adenocarcinoma may occur. Allelic deletions of the human homologue of the drosophila patched gene (PTCH) occur in both NS and BCC.

To search for genetic changes in SP, a microdissection-based genetic analysis using polymorphic markers at 9q22 (PTCH; D9S15, D9S303, D9S287, D9S252) as well as markers at 9p21 flanking the tumor suppressor gene p16 (IFNA, D9S171) was performed. Glandular epithelium consisting of two rows of cells as well as adjacent normal tissue or inflammatory infiltrates in the stroma, when present, was dissected and subjected to single-step DNA extraction and loss of heterozygosity (LOH) analysis.

Two of 10 informative SP cases showed LOH at 9q22 (PTCH). Three of 7 informative SP cases showed allelic deletions at 9p21 (p16). Allelic loss at 9q22 is consistant with the clinical observation of transition of SP to BCC. The finding of frequent allelic loss at 9p21 is unlikely to be related to the rare transition of SP to metastatic adenocarcinoma.

Our study supports the hypothesis of a gatekeeper role of the tumor suppressor gene p16 in a variety of benign and malignant tumors, including SP.

 

HISTOLOGICAL TYPES CHARACTERIZATION
General

Cystic invagination of the epidermis into the underlying dermis surrounding abundnant plasma cells

Cysts often lined by 2-3 layers of squamous cells with apocrine differentiation

VARIANTS  
HIDRADENOMA COMPONENT  


Mixed tubulopapillary hidradenoma and syringocystadenoma papilliferum occurring as a verrucous tumor.

Hsu PJ, Liu CH, Huang CJ.

Department of Dermatology, China Medical College Hospital, Taichung, Taiwan.

`J Cutan Pathol 2003 Mar;30(3):206-10 Abstract quote

Tubulopapillary hidradenoma (TPH)1 is a term proposed to describe morphological dermal ductal tumors with both eccrine and apocrine differentiation. The term TPH encompasses a spectrum of lesions that includes tubular apocrine adenoma (TAA) and papillary eccrine adenoma (PEA):2 PEA and TAA can be indistinguishable both clinically and histologically.

We described a case of TPH with both prominent eccrine and apocrine differentiation combined with syringocystadenoma papilliferum (SCAP) over the distal extremity. This rarely encountered dermatopathological phenomenon is the sixth reported case from the literature in which PEA or TAA and SCAP were present in the same lesion.3-7 Furthermore, the tumor had a warty surface, which is histologically consistent with a typical viral verruca.

Although PCR and DNA probe hybridization for human papilloma virus (HPV) types 2, 6/11, 16 and 18 failed to reveal positive results, the location and clinicopathologic correlation convinced us that superimposed HPV could not be excluded.

MALIGNANT  

Ductal carcinoma arising from a syringocystadenoma papilliferum in a nevus sebaceus of Jadassohn.

Hugel H, Requena L.

Dermatohispathologische Gemeinschaftspraxis, Friedrichshafen, Germany.


Am J Dermatopathol. 2003 Dec;25(6):490-3 Abstract quote.  

We present an example of ductal carcinoma connected to a syringocystadenoma papilliferum situated in a nevus sebaceus of Jadassohn on the scalp of a 22-year-old woman. The ductal carcinoma involved the entire thickness of the dermis and extended to the subcutaneous fat. Because syringocystadenoma papilliferum is considered a hamartoma with apocrine differentiation, the ductal carcinoma here described was interpreted as an apocrine ductal carcinoma.

Syringocystadenocarcinoma papilliferum is an exceedingly rare neoplasm, most examples of which seem to have arisen in its benign counterpart, syringocystadenoma papilliferum. From a histopathologic point of view, syringocystadenocarcinoma papilliferum usually shows a papillary configuration similar to that of syringocystadenoma papilliferum. In contrast, the case here described a ductal carcinoma superficially connected to a syringocystadenoma papilliferum, but mostly composed of small ductal structures embedded in a desmoplastic stroma and involving the full thickness of the dermis.

We review the literature about the malignant neoplasms arising in the nevus sebaceus of Jadassohn.

Syringocystadenocarcinoma papilliferum: Case report and immunohistochemical comparison with its benign counterpart

Akemi Ishida-Yamamoto, MD, PhD
Katsuhiko Sato, MD
Takashi Wada, MD
Hidetoshi Takahashi, MD, PhD
Hajime Iizuka, MD, PhD

Asahikawa, Japan

J Am Acad Dermatol 2001;45:755-9 Abstract quote

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 dermatopathologic entity.

Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.


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Last Updated 12/15/2003

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