Reactive Eccrine Syringofibroadenoma (ESFA) in Association With a Merkel Cell Carcinoma Excision Scar.

ABSTRACT: Eccrine syringofibroadenoma (ESFA) is a rare benign skin adnexal lesion of the acrosyringium of eccrine sweat ducts. Reactive ESFA, a subtype of ESFA, is usually associated with non-neoplastic cutaneous dermatoses or neoplastic skin tumors. Clinically, the lesions can be solitary or multiple, pink, or skin-colored coalescing papules or nodules of variable sizes. Histopathologically, this tumor is composed of numerous anastomosing cords of monomorphic cuboidal epithelial cells with eccrine duct formation. The association of reactive ESFA with benign conditions, such as psoriasis, diabetic polyneuropathy, scars, and leprosy, has been reported. However, the association of reactive ESFA with malignant tumors is extremely rare, with very few cases reported in the literature. We present a case of a 72-year-old woman who developed reactive ESFA associated with Merkel cell carcinoma excision scar. The ESFA tumors developed in the area of the surgical graft 10 months after the Merkel cell carcinoma had been excised. New ESFA tumors have continued to appear in the scar on a yearly basis while, so far, has been no recurrence of the original tumor. However, the presence of new tumor growths in the area suggested the possibility of recurrence of the Merkel cell carcinoma. That possibility was enhanced by the fact that PET scans revealed hypermetabolic activity in the ESFA papules.

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.

Primary cutaneous cribriform apocrine carcinoma: Case report and literature review.

Primary cutaneous cribriform apocrine carcinoma is a rare but distinct variant of primary cutaneous apocrine carcinoma and it is considered a low grade malignancy. We herein present a case of primary cutaneous cribriform apocrine carcinoma at the neck of a 26-year-old female. The tumor features a relatively well-circumscribed border and multiple aggregations of mildly pleomorphic epithelial cells with large ovoid nuclei, small nucleoli and abundant eosinophilic cytoplasms. Cribriform and tubular structures are the major architectural patterns. The primary differential diagnosis is cutaneous metastasis from a cribriform visceral carcinoma; others include primary secretory carcinoma of the skin, adenoid cystic basal cell carcinoma and primary cutaneous adenoid cystic carcinoma.

Syringocystadenocarcinoma papilliferum with coexisting trichoblastoma: A case report with review of literature.

We present a unique case of syringocystadenoma with malignant transformation into syringocystadenocarcinoma papilliferum and coexisting trichoblastoma arising de novo in a scalp swelling of long duration in a 78-year-old woman. Syringocystadenocarcinoma papilliferum was described by the World Health Organization as an extremely rare malignant adnexal tumor of apocrine origin with varied histology with some cases reported in literature.

The story of a deformed leprous foot.

Eccrine syringofibroadenoma (ESFA) is a rare adnexal tumour of eccrine ductal proliferation. A 50 year old treated case of leprosy presented with a chronic non healing ulcer of 5 years duration on the deformity laden right foot. Multiple verrucous papules and plaques were seen surrounding the ulcer which showed histopathological findings consistent with ESFA. Although ESFA constitutes a rare association with leprosy, considering the load of treated cases in our country and elsewhere, it may represent an under-reported entity which requires more attention in the post elimination era.