Syringocystadenocarcinoma papilliferum: a systematic review of clinical characteristics, reappraisal of associations, diagnostic pitfalls and management challenges.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare and aggressive malignant adnexal tumor originating from apocrine or pluripotent appendageal glands, often associated with a preceding syringocystadenoma papilliferum (SCAP) or nevus sebaceus (NS). This systematic review rigorously examines SCACP through an analysis of 78 cases documented between 1980 and 2024. The study aims to provide a comprehensive review of the clinical manifestations, diagnosis, treatment modalities, and outcomes associated with SCACP, while also reappraising its associations, particularly with NS. SCACP predominantly affects older adults, with an average age of 66.3 years and a slight male predominance, commonly presenting as ulcerated nodules or plaques on the scalp. This review highlights the aggressive nature of SCACP, evidenced by significant rates of metastasis and recurrence. Treatment is primarily surgical, with Mohs micrographic surgery offering potential benefits in terms of margin control and cosmetic outcomes. The association of SCACP with NS is critically evaluated, suggesting a complex etiopathogenesis and underscoring the importance of recognizing this association for timely diagnosis and management. Our review also briefly discusses potential pitfalls faced by clinicians in the diagnosis of SCACP. Our findings emphasize the need for standardized treatment protocols and further research into targeted therapies to improve patient outcomes in SCACP.

Syringocystadenoma papilliferum of the scalp.

Syringocystadenoma papilliferum is a rare, benign adnexal tumor of eccrine or apocrine origin that typically presents at birth or before puberty. Syringocystadenoma papilliferum is associated with a nevus sebaceus in about 40% of cases. We present a 50-year old woman with a pink-orange plaque and nodule on the scalp, consistent with syringocystadenoma papilliferum that arose within a nevus sebaceus.

Hidradenoma papilliferum of the hymen: a case report.

BACKGROUND: Hidradenoma papilliferum is a rare benign neoplasm arising from apocrine glands. It occurs commonly on the anogenital region of middle-aged women. It usually presents as a slow growing, solitary asymptomatic, skin colored or red nodule less than 1 cm in diameter. CASE PRESENTATION: The case is a 38-year-old, white woman who presented with a painful nodule occurring within a month in the himenal region of the posterior vaginal introitus. The nodule was excisied and the histology revealed a hidradenoma papilliferum. The diagnosis and treatment of hidradenoma papilliferum is possible with surgical removal and histopathological evaluation of nodules. CONCLUSION: When an adult woman presents with a noduler lesion in the anogenital area, sexually transmitted diseases and other benign and malignant vulvar lesions, as well as malignant transformation is very rare but,should be kept in mind; however because it has been reported and long-term clinical follow-up is suggested.

Vulval hidradenoma papilliferum: a clinical and dermoscopic study.

Hidradenoma papilliferum (HP) is a rare, slow-growing, benign adnexal tumour with apocrine differentiation. It usually affects the anogenital region in adult women and is clinically polymorphous, mimicking other benign or malignant neoformations. The dermoscopic features of vulval HP have not been reported yet. We report the clinical and dermoscopic features in a case series of histopathologically proven vulval HPs. Dermoscopy may be a useful tool for the diagnosis of vulval HP. To our knowledge, our paper represents the first report of dermoscopic findings in a series of vulval HPs in a multiracial population.

Tumoraciones con distribución lineal en la región escapular de un varón joven / Tumors Distributed Linearly Near the Scapula in a Young Man

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Syringocystadenocarcinoma Papilliferum: A Rare Malignant Sweat Gland Tumor.

A 73-year-old Hispanic woman presented to the clinic with an erythematous, friable 3.0 cm × 2.7 cm × 0.7 cm mass located on the posterior vertex of the scalp (Figure 1). The lesion had been present since birth but had recently begun to bleed intermittently, prompting evaluation. A biopsy was obtained, revealing a tumor with irregular, complex papillomatous invaginations lined by a two-layered epithelium. Atypical and crowded columnar cells lined the luminal aspect, while cuboidal cells lined the epithelium adjacent to the stroma (Figure 2). Decapitation secretion was noted in several areas on the luminal surface.

Eyelid syringocystadenoma papilliferum: A novel presentation with major review.

A major review of the literature of syringocystadenoma papilliferum's (SCAP's) presentation and management is presented. In addition, a case report of this unique diagnosis presenting as a corneal abrasion in a 66-year-old-male is included. This benign adnexal tumor of the apocrine glands is most commonly found in the face and neck. When found on the eyelids, these lesions are commonly misdiagnosed as basal cell carcinoma or cysts. Diagnosis is made based on histopathology. Treatment is complete excision of the lesion and this has a low recurrence rate. While predominantly benign, there have been cases of basal cell carcinoma development or other malignant transformations. Of the 26 reported cases of SCAP of the eyelids, none have caused a corneal abrasion. The authors present the only known presentation of eyelid SCAP, causing corneal abrasions, and provide a review of literature with discussion of clinical presentation, natural history, diagnosis, and treatment of this rare, eyelid lesion with potential for malignant transformation.

Syringocystadenoma papilliferum combined with a tubular apocrine adenoma.

Syringocystadenoma papilliferum and tubular apocrine adenoma are rare benign sweat gland tumors. Syringocystadenoma papilliferum occurs alone or in association with other tumors. Although it is rare, the association of tubular apocrine adenoma with syringocystadenoma papilliferum developing in a sebaceous nevus on the scalp is well documented. However, the co-existence of these two tumors without the background of a sebaceous nevus has not been frequently reported. Syringocystadenoma papilliferum and tubular apocrine adenoma may have a histopathological overlap, but a few cases of a syringocystadenoma papilliferum combined with a tubular apocrine adenoma have been reported. Herein we describe an unusual case of syringocystadenoma papilliferum co-existing with a tubular apocrine adenoma located on the back of a 14-year-old patient in the absence of a pre-existing sebaceous nevus.

Syringocystadenoma papilliferum combined with a tubular apocrine adenoma

Abstract: Syringocystadenoma papilliferum and tubular apocrine adenoma are rare benign sweat gland tumors. Syringocystadenoma papilliferum occurs alone or in association with other tumors. Although it is rare, the association of tubular apocrine adenoma with syringocystadenoma papilliferum developing in a sebaceous nevus on the scalp is well documented. However, the co-existence of these two tumors without the background of a sebaceous nevus has not been frequently reported. Syringocystadenoma papilliferum and tubular apocrine adenoma may have a histopathological overlap, but a few cases of a syringocystadenoma papilliferum combined with a tubular apocrine adenoma have been reported. Herein we describe an unusual case of syringocystadenoma papilliferum co-existing with a tubular apocrine adenoma located on the back of a 14-year-old patient in the absence of a pre-existing sebaceous nevus.

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.