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.
"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.
The Korean Journal of Pathology 12/2013; 47(6):569-74. DOI:10.4132/KoreanJPathol.2013.47.6.569 · 0.17 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Squamous cell carcinoma developing within a naevus sebaceus of Jadassohn is rare. We report the fifth such case in the English literature to highlight the importance of early excision of any naevus sebaceus of Jadassohn with a history of change. Other cases have been reported, but the clinical and histopathological details were not well documented.
The American Journal of dermatopathology 07/2008; 30(3):269-70. DOI:10.1097/DAD.0b013e318169cc69 · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The development of a squamous cell carcinoma within a nevus sebaceous of Jadassohn is very rare. We report here for the first time, the simultaneous occurrence of two squamous cell carcinomas within a single nevus sebaceous of Jadassohn.
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