Metastasizing Adenocarcinoma and Multiple Neoplastic Proliferations Arising in a Nevus Sebaceus

ArticleinAmerican Journal of Dermatopathology 29(5):462-6 · November 2007with6 Reads
DOI: 10.1097/DAD.0b013e31814a50c6 · Source: PubMed
Nevus sebaceus of Jadassohn is a hamartoma of multiple skin structures. Many neoplasms have been reported to arise in association with nevus sebaceus, most commonly trichoblastoma/basal cell carcinoma and syringocystadenoma papilliferum. We report a case of a 66-year-old woman with an adenocarcinoma as well as multiple neoplastic proliferations arising in a long standing nevus sebaceus on the scalp, with subsequent occipital neck metastatic disease. On histologic evaluation, the epidermis showed changes reminiscent of tumor of the follicular infundibulum as well as basaloid proliferations resembling superficial trichoblastoma. A focus suggestive of syringofibroadenoma was also present. A small dermal collection of basaloid and more mature sebocytes was consistent with a sebaceoma/sebaceous epithelioma. Most of the lesion was composed of an adenocarcinoma with areas showing ductal differentiation with decapitation secretion, well-formed papillae and focal cribriform structures. Other portions demonstrated a high-grade neoplasm with prominent nuclear atypia and a solid pattern of growth resembling high-grade breast carcinoma. Anti-epithelial membrane antigen strongly labeled tumor cells and highlighted ductal structures. Less than 1% of cells expressed progesterone or estrogen receptors. Her2/neu reactivity was focally present, showing 1+ membranous reactivity in 10% of cells. Anti-p63 labeled basaloid cells surrounding the tumor lobules. A breast primary was ruled out by clinical and radiologic examination. This report illustrates an extraordinary case of adnexal neoplasia displaying various lines of differentiation arising in association with nevus sebaceus.
    • "There are reports of benign as well as malignant tumors developing in NS. Basal cell carcinoma, syringocystadenoma papilliferum, trichoblastoma, and hidradenoma are documented to arise in NS. [9] In a report, authors described six tumors including syringocystadenoma papilliferum, tubular apocrine adenoma, sebaceoma, pigmented trichoblastoma, and superficial epithelioma with sebaceous differentiation and tumor of follicular infundibulum appearing in NS. [10] Because of benign nature of NS, some authors are an opinion that excision is not mandatory. [11] Hence, it is important and necessary to follow-up the lesions of NS when excision is not planned during childhood. "
    Full-text · Article · Mar 2016
    • "To the best of our knowledge, only a few cases arising in a nevus sebaceus have been reported in the literature, ours being the youngest case reported to date [2, 3]. In pediatric patients with NS only, previous reports have shown that excision is not necessary [4, 5]. However, in secondary tumors developing within the NS, clinically observed features are insufficient to make the exact diagnosis of benign or malignant lesions. "
    [Show abstract] [Hide abstract] ABSTRACT: A 14-year-old girl presented with a new, rapidly growing, pigmented tumor on a previously existing yellowish, verrucous plaque on the scalp. The patient received complete surgical excision. Routine histology ruled out basal cell carcinoma (BCC) and the histological diagnosis was pigmented trichoblastoma arising in nevus sebaceous (NS). It is important to define management for new lesions developing in pediatric patients with existing nevus sebaceus.
    Full-text · Article · Jul 2014
    • "Nearly all lesions occur on the scalp, forehead or face and appear clinically as smooth yellowish, hairless patches.1,2 Most lesions are plaques of 1-6 cm in diameter.1 Characteristic morphologic changes occur with puberty and lesions assume a verrucoid appearance.2 "
    [Show abstract] [Hide abstract] ABSTRACT: Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual for more than four tumors to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 70-year-old woman with six neoplastic proliferations including a syringocystadenoma papilliferum, pigmented trichoblastoma, tubular apocrine adenoma, sebaceoma, tumors of follicular infundibulum and superficial epithelioma with sebaceus differentiation arising in a long standing nevus sebaceus on the scalp. Our case is extraordinary because a single nevus sebaceus contained six neoplastic proliferations with differentiation toward the folliculosebaceous-apocrine unit.
    Full-text · Article · Dec 2013
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