SYRINGOCYSTADENOMA PAPILLIFERUM – A RARE CASE REPORT

Syringocystadenoma papilliferum is a rare benign adnexal tumor of apocrine or eccrine glands. It is a childhood tumor with a relatively higher incidence at birth. The common sites of occurrence are the face and scalp. The propensity of the nodular lesion is towards the trunk, but here in our case, we present a case of nodular syringocystadenoma papilliferum of the scalp. The tumor can arise de novo or from pre-existing naevus sebaceous. The tumor rarely has a malignant course, most often basal cell carcinoma. Herein, we present an adolescent with the nodular type of syringocystadenoma papilliferum of the scalp, treated with surgical excision.

Syringocystadenoma Papilliferum at an Unusual Location: a Case Report

A case of 15year old female presented with lesion over back since childhood, with occasional bleeding and oozing from lesion without any associated systemic complaints. There were multiple verrucous coalescing papules forming plaque with overlying erosion present over left lower back- diagnosed provisionally as angiokeratoma circumscriptum and was biopsied. Histopathology revealed findings consistent with Syringocystadenoma papilliferum. Surgical excision was done and closed with rotation flap. Syringocystadenoma is benign cutaneous adnexal tumor presenting clinically with many morphologies such as warty papules, nodules, plaques with oozing of serous material. Lesion is usually seen in head and neck area in most cases however can also occur on extremities, buttocks, anogenital region. It is characterized by multiple invaginations from skin surface in association with hair follicles lined by cuboidal to columnar epithelium on luminal aspect and myoepithelial cells on outside. There is papillary architecture and dermal ductal component.

Contiguous squamous proliferations in syringocystadenoma papilliferum: A retrospective study of 14 cases

Background: Syringocystadenoma papilliferum is a benign adnexal neoplasm. Contiguous squamous proliferation has been rarely described in syringocystadenoma papilliferum. Aims: This study aimed to evaluate the spectrum and pathogenesis of contiguous squamous proliferation in syringocystadenoma papilliferum. Materials and Methods: All cases of syringocystadenoma papilliferum diagnosed over the past 12 years were screened for contiguous squamous proliferation. Cases with associated nevus sebaceous were excluded from the study. Immunohistochemistry for GATA3, CK7, BRAFV600E and p16 was performed. PCR for human papilloma virus, type 16 and 18, was carried out. Results: Of a total of 30 cases, 14 cases showed associated contiguous squamous proliferation which included four cases of verrucous hyperplasia, six cases with papillomatosis, two cases with mild squamous hyperplasia and one case each of Bowen’s disease and squamous cell carcinoma. In the cases with non-neoplastic contiguous squamous proliferations, the squamous component did not express CK7 or GATA3. However, the squamous component of premalignant and malignant lesions expressed CK7 and GATA3 concordant with the adenomatous component. BRAF was positive in adenomatous component in five cases while the contiguous squamous proliferation component was negative for BRAF in all but one case. p16 was negative in both components of all cases and PCR for human papilloma virus was negative in all cases. Limitations: Due to the rarity of disease, the sample size of our study was relatively small with two cases in the 2nd group, that is, syringocystadenoma papilliferum with malignant contiguous squamous proliferation. Detailed molecular studies such as gene sequencing were not performed. Conclusion: Syringocystadenoma papilliferum with contiguous squamous proliferation is underreported, and most commonly displays verrucous hyperplasia. The premalignant and malignant contiguous squamous proliferations likely arise from syringocystadenoma papilliferum while the hyperplastic contiguous squamous proliferations likely arise from the adjacent epidermis. Relationship with high-risk human papilloma virus is unlikely. However, further molecular analysis of larger number of cases is required to establish the pathogenesis.

A Case of Syringocystadenoma Papilliferum of External Auditory Canal

Ceruminous glands are specialized sudoriferous glands located in the external auditory canal (EAC). Tumors originating from these ceruminous glands are rare lesions of EAC; in particular, syringocystadenoma papilliferum (SCAP) is an extremely rare ceruminous glands neoplasm to occur in the EAC. Although it is rare, the association of SCAP with tubular apocrine adenoma in the background of nevus sebaceous on the body, extremities, and scalp is well documented. In this article, we report on a 60-year-old male who presented a mass in EAC, which was surgically removed. The final diagnosis of SCAP was determined by histological analysis and the patient has been well without recurrence.

A Rare Occurrence of Sebaceous Carcinoma, Sebaceoma, Syringocystadenoma Papilliferum, and Trichoblastoma in a Single Nevus Sebaceous Lesion

Nevus sebaceous (NS) is a benign tumor with the potential to develop secondary benign and malignant neoplasms. It is a rare phenomenon to develop 2 or more skin tumors in a single NS lesion. We report a case of multiple secondary tumors, such as sebaceoma, sebaceous carcinoma, syringocystadenoma papilliferum, and trichoblastoma, in a single NS lesion.

Inverted Ductal Papilloma of the Salivary Gland

This is a case consult of slides stated to be from an excision of a buccal mucosa mass in a 58-year-old-man. The specimen was described as a 3 cm diameter roughly oval tan-gray tissue with a 2 x 1.5 cm mucosal ellipse on the surface that has a central ulcerated punctum. Cut section showed an underlying 1.7 cm diameter roughly oval well-circumscribed mass with a granular tan surface. Histological sections show a papillary lesion with an orifice on the mucosal surface and with epithelial nests invaginating into the underlying lamina propria in a non-infiltrative pattern. (Figure 1) The lesion is composed of papillary epithelial fronds with cleft-like spaces between the fronds. (Figure 2) The papillary fronds are lined by non-keratinizing basaloid stratified squamous cells with a superficial layer of columnar glandular cells along with mucous goblet cells interspersed among the squamous cells. (Figure 3) All the cellular components are devoid of cytologic atypia and mitoses. Based on these microscopic features we signed the case out as inverted ductal papilloma (IDP). Ductal papillomas are uncommon benign epithelial tumors with a papillary configuration that originate from the excretory ductal system of salivary gland acini.1-3 The World Health Organization recognizes two sub-types depending on the growth pattern: an intraductal papilloma (IP) and an IDP.1 An IDP usually presents as an asymptomatic submucosal nodule, measuring about 1.5 centimeters in diameter, and most commonly involving the buccal mucosa, followed by the lips, palate, and floor of the mouth.2,3 Histological sections typically show an unencapsulated though well-circumscribed epithelial proliferation with a papillary configuration on the luminal surface, and a nodular, endophytic or invaginating (“inverted”) configuration at its interface with the underlying lamina propria.2 Both the papillary and the invaginating areas are composed of basaloid, non-keratinizing stratified squamous epithelium that are often covered with a cuboidal or columnar ductal cell layer.2 Scattered among these are mucous goblet cells which can form microcysts.1,2 There is an overall morphological similarity to the sinonasal inverted papilloma.3 A relationship to trauma has been proposed.1,4 Association with Human Papilloma Virus (HPV) has also been reported.1 Others, however, have not been able to demonstrate this association.4 Differential diagnoses primarily include IP - which is differentiated from IDP architecturally by being a unicystic intraluminal papillary proliferation within a dilated excretory duct 2 – and sialadenoma papilliferum – which is predominantly polypoid and pedunculated with a verrucoid surface rather than a submucosal nodule, and an over-all morphologic similarity to the cutaneous tumor syringocystadenoma papilliferum.1,4 An important differential diagnosis that has to be ruled out is mucoepidermoid carcinoma (MECA) because of the presence of both squamous and mucin-secreting cells. MECA is distinguished by poor circumscription, and an infiltrative solid-cystic growth pattern.2,4 IDP is benign and non-recurrent. Unlike the nasal tumor, there has been no report of malignant transformation.2,3 Complete surgical excision is considered curative.1,2 Reporting these cases is encouraged to further our knowledge of the entity and elucidate a potential association with HPV.