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.

Rate of Benign and Malignant Secondary Tumors Associated With Nevus Sebaceous: A Systematic Review and Meta-Analysis.

BACKGROUND: Nevus sebaceous (NS) is a rare congenital skin lesion affecting approximately 0.3% of all newborns. Although benign, NS lesions can harbor malignant secondary tumors. The published rate of development of these malignant tumors varies. This meta-analysis aimed to identify the rate of malignant and benign secondary neoplasms occurring in NS. METHODS: A literature search was conducted using PubMed, Embase, and Web of Science from inception to April 2023. Eligible studies reported incidence or risk of secondary neoplasms in patients with NS. Two independent reviewers screened studies, extracted data, and assessed the quality of included studies. The primary outcome was the pooled incidence of secondary neoplasms. Studies with sample sizes greater than 50 patients were eligible for meta-analysis using the random-effects model. RESULTS: Twenty-eight studies were identified, 22 of which were eligible for meta-analysis. The overall rate of secondary neoplasms was 12.8% (95% confidence interval [Cl], 9.2%-17.6%). The rates of development of malignant and benign tumors were 2.4% (95% CI, 1.4%-4.1%) and 10.3% (95% CI, 7.5%-13.9%), respectively. The rate of development of basal cell carcinoma was 1.7% (95% CI, 0.9%-3.2%), whereas the rate of the development of syringocystadenoma papilliferum was 3.6% (95% CI, 2.5%-5.3%) and that if trichoblastoma was 2.6% (95% CI, 1.7%-3.8%). CONCLUSIONS: Although the rate of development of malignant tumors within a primary NS lesion is low, it is not negligible. Prophylactic early excision remains a viable approach to prevent secondary malignant neoplasms, address cosmetic and functional complications, and preempt the need for complex reconstruction in the future. We propose that resection of NS lesions in childhood remains a reasonable first-line option in the appropriate patient keeping in mind that it may leave an undesirable scar.

Nevo sebáceo de Jadassohn. Localización atípica y síndromes asociados / Nevus sebaceus of Jadassohn. Atypical location and associated syndromes

Hamartoma benigno que puede aparecer desde el nacimiento. Se localiza sobre todo en cuero cabelludo y cara; origina una placa alopécica, amarillenta, de superficie rugosa. Tiene tres etapas, una de hiperplasia epidérmica, luego una puberal con desarrollo de glándulas sebáceas y una tardía con desarrollo de tumores benignos o malignos, en especial siringocistadenoma papilífero y tricoblastomas. Puede presentarse asociado a síndromes genéticos. Su exéresis quirúrgica es objeto de controversia (AU)

Benign hamartoma that can appear from birth. It is located mainly on the scalp and face; It causes an alopecic, yellowish plaque with a rough surface. It has three stages, one of epidermal hyperplasia, then a pubertal stage with development of sebaceous glands, and a late stage with development of benign or malignant tumors, especially papillary syringocystadenoma and trichoblastoma. It can appear associated with genetic syndromes. Its surgical excision is controversial. (AU)

Sebaceous Nevus of the Scalp.

Excisions of scalp nevus sebaceous (NS) presents a unique challenge due to limited soft tissue laxity, hair-bearing skin, and convex surfaces which often leave the surgeon and patient underwhelmed with the reconstructive outcome. In this study, the authors conducted an institutional review board-approved retrospective review of patients who underwent excision of pathologically proven scalp primary NS from 2003 to 2017 at our institution to better define the reconstructive outcomes and options for treatment of pediatric scalp NS. 92 patients were included in the study, 54 males (58.7%) and 38 females (41.3%). The average age at surgery was 7.24 years (0.5-16.0; SD 4.7). Local tissue undermining/galeal scoring with primary closure (LTUGS) was used for lesions with average surface area of 3.6 cm, rotational or transposition flaps (RF/TF) for lesions averaging 4.3 cm, completed serial excision for lesions averaging 13.9 cm, and tissue expansion (TE) for lesions averaging 21 cm (P <0.001). One or more poor outcomes were experienced by 35 patients (38%), with a significant difference between the surgical groups; LTUGS 37.2% (29/78), RF/TF 60% (3/5), serial excision 100% (3/3), TE 0% (0/6) (P = 0.022). Univariant binary regression analysis within the LTUGS and RF/TF groups showed that lesion size was a significant predictor of poor outcomes (P = 0.012). All specimens in this study were negative for carcinoma. Therefore, most pediatric nevus sebaceous of the scalp can be managed by a single-phase procedure though risk of poor outcomes increase with nevus size with high rates of poor outcomes even with small lesions.

Squamous cell carcinoma with perineural involvement in nevus sebaceus.

Nevus sebaceus (NS), also known as an organoid nevus, is a congenital skin hamartoma involving the pilosebaceous unit, epidermis, and adnexa, frequently occurring in the scalp area. During childhood, the lesion remains unchanged but may subsequently increase in size during puberty in response to hormonal release, with possible tumor development. We report a case of squamous cell carcinoma (SCC) development in an NS with histologic evidence of perineural involvement on the anterior central scalp of a 13-year-old adolescent girl. We also review the management and prognosis.

Verruciform xanthoma overlying inflammatory linear verrucous epidermal nevus and in broad segmental distribution.

A 17-year-old woman presented with moderately itchy, non-progressive, linearly arranged verrucous plaques over dorsum of left foot since early childhood. Two years ago, she developed slowly increasing, verrucous exophytic growth in posterior most aspect of linear verrucous plaque. One year ago, she also developed multiple, linearly arranged, fleshy plaques with surface crusting over lateral aspect of right leg extending to thigh. Biopsy from both verrucous lesion on left foot and fleshy plaque on right leg showed an exophytic growth with significant papillomatosis, neutrophils in stratum corneum, acanthosis and infiltration of papillary dermis with foamy macrophages that were CD 68 positive, features compatible with verruciform xanthoma (VX). Biopsy from linear verrucous plaque over left foot was consistent with inflammatory linear verrucous epidermal nevus (ILVEN). A diagnosis of segmental VX and VX overlying ILVEN was made.

Management of naevus sebaceous: a national survey of UK dermatologists and plastic surgeons.

Naevus sebaceous (NS) is a congenital cutaneous hamartoma, which typically occurs on the head and neck. Historically, the treatment of choice was excision in infancy because of the potential for malignant transformation; however, recent studies suggest that this risk is < 1% and unlikely in childhood. We sent a questionnaire to UK dermatologists and plastic surgeons to investigate current management practice of NS. We found that almost a third of dermatologists still recommend excision for malignancy prevention, while over 90% of plastic surgeons consider excision, with 64% citing malignancy prevention as the reason. Plastic surgeons most commonly recommended excision in childhood, whereas dermatologists waited until adulthood. We have shown there is significant variation in practice across the UK in the management of naevus NS. It is important that patients across the UK receive the same standard of care, and therefore we advocate the development of evidence-based guidance for treatment of naevus NS.

Syndromic sebaceous nevus: current findings.

BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan. METHODS: This is a retrospective study reviewing the cases of syndromic sebaceous nevus treated in the Department of Orthopedic Plastic Pediatric Surgery in Montpellier, France, and the Department of Pediatric Surgery in Lausanne, Switzerland, between 1994 and 2016. RESULTS: The files of six patients with syndromic sebaceous nevus were analyzed. The average age at the first consultation was 4 months. The location was craniofacial in all cases. Cerebral radiological imaging was performed on all patients; two showed abnormal findings. Four patients underwent ophthalmic examination, which all revealed abnormalities. Three patients had other associated malformations. Three patients presented with epilepsy or learning difficulties in the course of follow-up. CONCLUSION: All patients presenting with extensive sebaceous nevus of the craniofacial region should benefit from cerebral imagery and ophthalmic examination since there is a very high probability of associated abnormalities. The developmental problems encountered could not be definitively associated with the skin malformations.

Picosecond 532-nm neodymium-doped yttrium aluminum garnet laser-a promising modality for the management of verrucous epidermal nevi.

The verrucous epidermal nevus (VEN) is the most common type of epidermal nevi. As lesions can be disfiguring, treatment is often sought. Many therapeutic approaches have been reported, with variable efficacy and safety. Picosecond (PS) lasers are novel laser devices designated to target small chromophores. A side effect of these lasers is blistering due to epidermal-dermal separation. We aimed to harness this side effect of the PS lasers to treat patients with VEN. The purpose of this study was to report our experience treating VEN using a PS 532-nm laser. We present a retrospective case series of six patients with large VEN who were treated using a PS 532-nm laser (2-6 treatments, 8-10 weeks apart). Response in clinical photographs was assessed by two independent dermatologists and graded on a scale of 0 (exacerbation) to 4 (76-100% improvement). Patient satisfaction was recorded on a scale of 1-5. All patients demonstrated significant improvement. Average improvement was 3.7 on the quartile scale of improvement. Patient satisfaction rate averaged 4.7. The PS 532-nm laser is a promising novel modality for the treatment of large VEN.

Pigmented trichoblastoma developed in a sebaceous nevus: HRAS mutation as a common molecular driver.

A 40-year-old woman presented with a pigmented nodule on a previously existing yellowish, verrucous plaque on the scalp. The histological diagnosis was consistent with a pigmented trichoblastoma developed within a sebaceous nevus (SN). A multigene hotspot mutational profiling of the BRAF, NRAS, HRAS and KRAS genes was carried out, and a shared G13R HRAS mutation in both the trichoblastoma and the sebaceous nevus components was found. These data support a common molecular landscape of the two lesions.

Massive Vulvar Linear Verrucous Epidermal Nevus Presenting at Menarche.

BACKGROUND: Epidermal nevi are benign hamartomatous growths of the skin that present at birth and develop in early childhood often linearly along the "lines of Blaschko." Verrucous linear epidermal nevi are the most common epidermal nevi and often are located on the trunk or extremities. There is minimal evidence regarding vulvar involvement and subsequent management of the associated cosmetic deformity in this anatomic location. CASE: An 11-year-old menarchal girl who underwent full-thickness dermal excision of a 9.6 × 4.5 × 3.2 cm left benign vulvar epidermal nevus (noninflammatory type) with primary reconstruction and closure using a mons rotational skin flap. SUMMARY AND CONCLUSION: Surgical excision of large vulvar nevi can be achieved with desirable cosmetic outcomes with use of rotational skin flaps and primary closure.

Complex limbal choristoma in linear nevus sebaceous syndrome managed with scleral grafting.

Linear nevus sebaceous syndrome (LNSS) is characterized by nevus sebaceous, mental retardation, seizures, and ocular abnormalities such as complex limbal choistoma. A young male with history of mass in right eye and blackish discoloration of skin over right and left side of forehead since birth presented with foreign body sensation and diminished vision in right eye. Ocular examination showed mass over epibulbar region with chorioretinal coloboma and posterior staphyloma in right eye and megalocornea in left eye. Histopathology report revealed complex limbal choristoma with compound melanocytic nevus. The case was managed by surgical excision of the limbal mass and filling the gap with scleral graft.