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Syringocystadenoma papilliferum: An unusual location

  • Author(s): Elfatoiki, Fz
  • Khadir, K
  • Ouakadi, A
  • Azzouzi, S
  • Bahechar, N
  • Benchikhi, H
  • et al.
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Syringocystadenoma papilliferum: Unusual location
Fz Elfatoiki1, K Khadir1, A Ouakadi2, S Azzouzi2, N Bahechar3, H Benchikhi1
Dermatology Online Journal 17 (6): 7

1. Department of Dermatology
2. Department of Anatomic pathology
3. Department of Plastic Surgery
Ibn Rochd UHC of Casablanca, Morocco


Abstract

Syringocystadenocarcinoma papilliferum (SCAP) is rare. We describe a 55-year-old man with a partially eroded lesion on the trunk that developed over one year. Histopathological examination revealed syringocystadenoma papilliferum. A wide excision was performed to remove the tumor. The patient has been well without relapse or metastasis for 8 months.



Introduction

Syringocystadenoma papilliferum is a rare benign neoplasm, which occurs either de novo or within some organoid nevus such as nevus sebaceus. The clinical presentation varies widely but the histopathologic appearance is uniform and characteristic forming the basis of diagnosis.

We report a case of SCAP presenting on the trunk, clinically misdiagnosed because of the unusual location in a 55-year-old man.


Case report


Figure 1Figure 2
Figure 1. Location in the trunk

Figure 2. Circumscribed and sessile nodule

Figure 3Figure 4
Figures 3 and 4. Cystic and branched tubular projections

A 55-year-old man presented with a partially eroded nodule localized on the trunk that started 1 year prior to presentation as a small papule and gradually grew in size. Recently, the lesion became more noticeable and ulcerated. Some local medications were applied but the tumor never regressed.

On examination, a soft nodule was seen on the middle of the trunk. It was a circumscribed and sessile nodule measuring 3 cm in diameter, with an erythematous base surrounding it and a red, ulcerated surface. The rest of the skin examination was normal and there was no regional lymphadenopathy.


Figure 5Figure 6
Figures 5, 6 and 7. Plasma cell in the stroma of papillary projections

Figure 7Figure 8
Figure 8. Endophytic invaginations of the epithelium into the dermis


Figure 9
Figure 9. Cystic and branched tubular projections

Diagnoses of verrucous tuberculosis, pyogenic granuloma, amelanotic melanoma, adnexal neoplasm, B cell lymphoma, and pseudolymphoma were considered.

Skin biopsy exhibited hyperplastic epidermis with cystic invaginations into the dermis. The epidermis showed prominent hyperkeratosis. Multiple papillary projections were noted in the lumen of the cystic invagination. These were lined by a bilayered epithelium composed of a luminal row of columnar and cuboidal cells. A fair number of the columnar cells showed decapitation secretion. A dense inflammatory infiltrate composed of plasma cells was noted. There was no evidence of nevus sebaceus. A final diagnosis of SCAP was entertained.

The nodule was excised and the histopathology showed the same findings.


Discussion

The peculiarity of our case lies in the rarity of syringocystadenoma papilliferum, its location on the trunk, and its occurrence in a 55-year-old man.

The tumor started 1 year prior to presentation as a small papule and no lesion was reported to be present before at the site. The ulcerated nodule on the middle of the trunk was solitary. Skin biopsy was characteristic and the nodule was excised without relapse or metastasis.

Syringocystadenoma papilliferum (SCAP) is a benign adnexal neoplasm occurring during childhood or adolescence. It usually presents as a papule or a smooth hairless plaque on the scalp and forehead; 75 percent of the cases are reported in the head and neck region, occurring de novo or associated with nevus sebaceus [1, 2, 3].

Uncommon sites of occurrence include trunk, arms, breast, eyelids, axilla, scrotum, lower limb, and inguinal and perineal regions. Most of these are sporadic cases diagnosed on histopathology, the clinical presentation being non-specific and misleading [2, 3].

Morphologically, SCAP is characterized by endophytic invaginations of the epithelium into the dermis. These are duct-like structures leading into the dermal cystic spaces. Papillary projections of variable shape and size protrude into the lumen of these spaces. These are lined by double-layered outer cuboidal and luminal high columnar epithelium. Dilated capillaries and a dense infiltrate of plasma cells are noted in the stroma of these papillary projections.

Tumor cells show a positive staining reaction with carcinoembryonic antigen and gross cystic disease fluid protein-15 [1, 3].

One-third of all the cases arise in an organoid nevus, such as nevus sebaceus. Coexisting basal cell carcinoma is noted in 10 percent of the cases and very rarely a transformation to adenocarcinoma may also occur [2, 3, 4].

The only treatment for SCAP is excisional biopsy, which also confirms the diagnosis.

CO2 laser excision of SCAP of the head and neck is a clinical treatment option in anatomic areas unfavorable to excision and grafting. Syringocystadenocarcinoma papilliferum has been successfully treated with Mohs micrographic surgery [3, 4].


Conclusion

Syringocystadenoma papilliferum, although well described, is a relatively rare tumor and the trunk is an uncommon location. Solitary tumors in unusual locations generate multiple differential diagnoses and must be sent for histopathological examination.

References

1. Akhdari N., Habibeddine S., Azzouzi S., Lakhdar H. Le syringocystadénome papillifère : À propos d'une observation à disposition linéaire. Les Nouvelles dermatologiques 2003, vol. 22, no4. 249-250

2. Ghosh SK, Bandyopadhyay D, Chatterjee G, Bar C. Syringocystadenoma papilliferum: An unusual presentation Pediatr Dermatol. 2009 Nov-Dec;26(6):758-9. [PubMed]

3. Malhotra P, Singh A, Ramesh V. Syringocystadenoma papilliferum on the thigh: An unusual location. Indian J Dermatol Venereol Leprol 2009 ; 75:170-2. [PubMed]

4. S. Rammeh-Rommani, B. Fezaa, E. Chelbi, M.R. Kamoun , S. Baltagi Ben Jilani. Syringocystadénome papillifère de localisation inhabituelle. Ann Dermato Venereol 2006;133:301-2

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