Syringocystadenocarcinoma papilliferum in situ of the penis

ARTICLE

ejd.2012.1723

Auteur(s) : Mathew A. Plant¹, Shachar Sade², Collin Hong³, Danny MD Ghazarian⁴ Danny.Ghazarian@uhn.on.ca

¹ Department of Surgery, Toronto General Hospital,

² Department of Pathology Sunnybrook Health Sciences Centre, Toronto, Canada

³ Cosmetic, Plastic and Reconstructive Surgeon, Toronto, Canada

⁴ Department of Pathology, Toronto General Hospital, 190 Elizabeth St, Toronto, M5G 2C4, Canada

Syringocystadenocarcinoma papilliferum (SCACP) is the malignant version of syringocystadenoma papilliferum (SCAP), which is a benign tumor of eccrine, apocrine or apoeccrine origins that typically occurs on the scalp or face [1-3]. Clinically SCAP lesions are skin-colored to yellowish papules and nodules that remain stable for years. Histologically SCAP is comprised of an intracystic proliferation of micropapillary structures lined by a two-cell layer of either columnar or cuboidal epithelium which transitions to squamous epithelium towards the surface. There is typically a marked infiltration of plasma cells within the stroma [1, 3, 5]. SCACP in situ (SCACP-IS) has the same appearance as SCACP without evidence of stromal invasion or ulceration through the epithelium

An 83-year-old male presented with a non-healing, ulcerated lesion on his penis measuring 1.2×1 cm (figure 1A). The lesion was excised and, at a private laboratory, was given a differential diagnosis of plasmacytosis mucosae and squamous cell carcinoma in situ with ulceration. The slides were then sent to the senior author for a second opinion.

On microscopy there was a central epidermal lesion showing reticulated acanthosis with focal surface areas demonstrating papillary formations. The dermal component showed mild dilatation in areas suggestive of a cystic glandular/adnexal lesion. Glandular lumena were also apparent. The cells within the lesion demonstrated severe full-thickness epithelial dysplasia with severe cytologic atypia, cellular crowding, loss of polarity as well as numerous mitoses at all levels. There was prominent pagetoid spread at the periphery both in the form of single cells and aggregates. The surface was eroded with focal ulceration and crusting. The dermis demonstrated a prominent plasmacytic infiltrate with fibrosis and vascular proliferation superficially. There was no evidence of dermal invasion and the lesion was completely excised (figure 1B).

Immunohistochemical staining was positive for LMWK, CK7 and EMA; and negative for p63 and CK5/6 (both highlighting the intact basal cell layer). BRST-2, ER and AR were also negative. Monoclonal CEA is negative within the cells and only highlights the acrosyringeal ducts seen within the tumor (figure 1D) [4]. Molecular diagnosis demonstrated positivity for oncogenic HPV DNA (subtype 68) via linear array analysis.

SCACP is quite a rare phenomenon with approximately 10 cases worldwide having been described. The vast majority of these arose from longstanding SCAP [3]. Once SCAP undergoes malignant degeneration it again begins to enlarge and can bleed or ulcerate. Histologically the structure becomes increasingly more asymmetrical, the boundary between the lesion and the normal tissue becomes unclear, the organization of the double-layered epithelium disappears and the individual cells exhibit crowded nuclei with variable atypia and numerous mitoses [5].

SCACP-IS is even rarer with 4 cases having been described and only one in the genital area [6]. To the best of our knowledge no prior case of SCACP or SCACP-IS has tested positive for HPV DNA.

This case is interesting as this is the fifth published case of SCACP-IS and the first on the penis. Furthermore, there are fewer than ten cases of cutaneous tumours of glandular origin on the penis described previously [1]. It also represents both the first documented glandular penile cutaneous tumour and the first case of SCACP (in any location) to test positive for HPV. Finally, the lesion on this patient arose de novo, whereas the majority of SCACP lesions in the literature have arisen from an existing SCAP lesion.Whether there is a causal relationship between HPV and SCACP cannot be determined based on a single case, however the association between the two that is demonstrated by this case indicates that this relationship deserves further study.

Disclosure

Financial support : none. Conflict of interest : none.

References

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3. Langner C, Ott A. Syringocystadenocarcinoma papilliferum in situ originating from the perianal skin. APMIS 2009; 117: 147-50.

4. Yamamoto O, Doi Y, Hamada T, Hisakoa M, Sasaguri Y. An immunohistochemical and ultrastructural study of syringocystadenoma papilliferum. Brit J Dermatopathol 2002; 147: 936-45.

5. Ishida-Yamamoto A, Sato K, Wada T, Takahashi H, Iizuka H. Syringocystadenocarcinoma papilliferum: Case report and immunohistochemical comparison with its benign counterpart. J Am Acad Dermatol 2001; 45(5): 755-9.

6. Goshima J, Hara H, Okada T, Suzuki H. Syringocystadenoma papilliferum arising on the scrotum. Eur J Dermatol 2003; 13(3): 271.