Basal Cell Carcinoma and Syringocystadenoma Papilliferum Arising in Nevus Sebaceous on Face—A Rare Entity

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprising 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 to develop two different types of tumor, benign and malignant, to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 27-year-old male with two neoplastic proliferations including a syringocystadenoma papilliferum a benign tumor and basal cell carcinoma a malignant tumor arising in a long-standing nevus sebaceus on the face. Neoplastic changes are common in nevus sebaceous present on scalp but our case is unique due to the presence of two different types of neoplasm in a nevus sebaceous which was present on face.     

Rippled-pattern trichoblastoma with apocrine differentiation arising in a nevus sebaceus: report of a case and review of the literature

A 36-year-old man developed a papular growth in a portion of a nevus sebaceus on the post-auricular scalp. Excision showed typical histologic changes of nevus sebaceus including epidermal papillomatosis with reduced numbers of hair follicles as well as numerous sebaceous glands high in the dermis that focally emptied directly to the overlying epidermis. Histologic sections of the papular growth at the superior pole of the nevus sebaceus showed a proliferation of cytologically bland basaloid epithelial tumor lobules both in the superficial dermis, with multiple connections to the epidermis, and within the deeper dermis in a nodular growth pattern demonstrating papillary mesenchymal bodies. Ductal structures with apocrine-type decapitation secretion were present. There was prominent palisading of nuclei in rows parallel to one another, alternating with bands of homogenous eosinophilic stromal material forming a ripple pattern resembling the Verocay bodies of schwannoma. The histologic features resembled those of rippled-pattern trichoblastoma with apocrine differentiation arising in a nevus sebaceus, an association not previously described. We discuss this case as well as review the literature on rippled-pattern trichoblastoma.     

Syringocystadenoma papilliferum with sebaceous differentiation in an intradermal tubular apocrine component. Report of a case

Syringocystadenoma papilliferum is a benign tumor most commonly located on the scalp or face, and it often arises within a nevus sebaceus of Jadassohn. We describe a 61-year-old man with syringocystadenoma papilliferum developing within a nevus sebaceus with sebaceous differentiation in an intradermal tubular apocrine component of the syringocystadenoma papilliferum. Although some adnexal neoplasms that develop in association with a nevus sebaceus may exhibit conjoint sebaceous, follicular, or apocrine differentiation, reflecting close embryological relations of the folliculosebaceous-apocrine unit, the feature we report on has not previously been described to the best of our knowledge.     

Extensive cerebriform nevus sebaceus: an unusual presentation

Nevus sebaceus is an epidermal nevi composed of sebaceous glands, which usually presents as single yellowish plaque over the head and neck. A rare morphological variant, namely cerebriform nevus sebaceus was first reported in 1998. As with other nevi, the occurrence of nevus sebaceous is related to genetic mosaicism and the shape and distribution of the nevus is determined by the stage at which mosaicism has arisen. We report a patient with cerebriform nevus sebaceus having an unusually widespread distribution over the head, face, and trunk, but without any other organ system involvement.     

Ten cases of sebaceous carcinoma arising in nevus sebaceus

Although nevus sebaceus is known to develop various types of secondary neoplasms, it rarely causes carcinoma and only 14 cases of secondary sebaceous carcinoma have been reported. In this study, 10 cases of sebaceous carcinoma arising in nevus sebaceus were collected. The clinicopathological features and results of immunohistochemical examinations with adipophilin, perilipin and p53 were summarized. Sebaceous carcinoma arising in nevus sebaceous predominantly occurred on the scalp (8/10) of elderly women (mean age, 67.7 years). No case was associated with Muir–Torre syndrome. We found several pathological features of sebaceous carcinoma; that is, made up mainly of germinative cells, moderate nuclear atypia without pleomorphism and many mitoses (4–28/10 high‐power field). Adipophilin and perilipin antibodies highlighted lipid drops in the cytoplasm of the malignant cells in all cases. Overexpression of p53 was seen in all cases. In two cases there were coexisting benign‐looking sebaceous lesions at the periphery of the main cancer nodule, and in these lesions p53 showed low positivity compared with the clearly malignant area. There was co‐occurrence of another neoplasm in three cases with trichoblastoma, sebaceoma and syringocystadenoma papilliferum, respectively. All cases were treated by excision of the malignant lesion, with or without inclusion of the nevus sebaceus. In a follow‐up period of 1–7 years, there was no case of recurrence, lymph node metastases or distant metastases. With these specific pathological and immunohistochemical findings using adipophilin, perilipin and p53, we have to consider the possibility that there is a tendency to underdiagnose secondary sebaceous carcinomas in nevus sebaceus. These clinicopathological features of sebaceous carcinomas developing in the nevus sebaceus seem to indicate different biological entities from de novo sebaceous carcinoma.     

The Spectrum of Epidermal Nevi: A Case of Verrucous Epidermal Nevus Contiguous with Nevus Sebaceus

During the normal development of skin, pluripotential cells give rise to keratinocytes, sebaceous glands, hair follicles, apocrine glands, and eccrine glands. In epidermal nevi, these components emerge in an abnormal mixture within a circumscribed site. Many authors have categorized epidermal nevi based on their predominant component; however, there is often notable overlap that occurs within a single area or within contiguous areas. We report a verrucous epidermal nevus contiguous to a nevus sebaceus of Jadassohn. The categories of epidermal nevi are somewhat artificial. Our case supports the view that epidermal nevi have a spectrum of manifestations, including verrucous epidermal nevi and nevus sebaceus of Jadassohn.     

A rare combination of sebaceoma with carcinomatous change (sebaceous carcinoma), trichoblastoma,and poroma arising from a nevus sebaceus

We report a case of a 48‐year‐old Malay female who presented with multiple tumors arising from a large nevus sebaceus on her right parieto‐temporal scalp. Histologically, the tumors corresponded to a sebaceoma with carcinomatous change, a poroma and a trichoblastoma. Immunohistochemical staining of the sebaceous tumor with p53 showed strong within the areas of carcinomatous change, while there was negative to weak staining within the sebaceoma‐like areas. A discussion on the potential secondary neoplasms from a nevus sebaceus ensues, with a review of this literature on multiple tumors from a nevus sebaceus.     

Sebaceous carcinoma arising in nevus sebaceus of Jadassohn: a clinicopathological study of five cases

The authors report a small series of sebaceous carcinoma developing in nevus sebaceus of Jadassohn (also known as organoid nevus) and analyze similar cases reported in the literature. All of our patients were females (age range 57-71 years; median 60 years) who had the organoid nevus on the scalp, face, or nuchal area, the rest of which was recognized clinically and/or histologically. All sebaceous carcinomas manifested unquestionable architectural (asymmetry, invasive growth) and cytological features of a carcinoma (cellular and nuclear pleomorphism, plentiful atypical mitoses, necroses en masse), demonstrated unambiguous sebaceous differentiation in the form of mature sebocytes, and lacked other differentiations. The sebaceous carcinoma was always accompanied by other benign or malignant adnexal lesions such as sebaceoma, syringocystadenoma papilliferum, syringocystadenocarcinoma papilliferum, trichoblastoma, tricholemmoma, desmoplastic tricholemmoma, or syringoma. In three cases, prominent mucinous metaplasia of sweat ducts and glands was seen. In two of these cases, sweat ducts exhibited hyperplastic changes. The analysis of the previously published material and our cases indicates that sebaceous carcinoma arising in organoid nevus has a female predilection and tends to occur in elderly patients. It may involve any site where nevus sebaceus typically occurs. Clinically, the tumor presents as a solitary nodule, ulcerated tumor, or mass, often with a recent history of rapid growth. It may arise alone, but it occurs more frequently as part of multiple benign and malignant adnexal tumors. The lesion does not seem to be associated with Muir-Torre syndrome. The rest of organoid nevus is usually recognized both clinically and microscopically, although large tumors may overgrow and mask the nevus. The tumor seems to be a low-grade carcinoma in terms of clinical behavior.     

SCALP syndrome: sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: a distinct syndromic entity

Nevus sebaceus syndrome (SNS) is a constellation of nevus sebaceus with extracutaneous findings, including the ophthalmologic nervous, and musculoskeletal systems. Didymosis aplasticosebacea is a recently described entity consisting of aplasia cutis congenita and nevus sebaceus, implying twin spotting (didymosis). We describe a neonate with a nevus sebaceus on the scalp and a limbal dermoid on her left eye. Contiguous with the nevus sebaceus was a giant congenital melanocytic nevus and numerous areas of membranous aplasia cutis congenita. We propose the acronym SCALP (nevus sebaceus, central nervous system malformations, aplasia cutis congenita, limbal dermoid, pigmented nevus) to summarize the unique features of this case and review the two similar cases in the literature.     

Metastasizing adenocarcinoma and multiple neoplastic proliferations arising in a nevus sebaceus

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.     

Trichoblastoma and sebaceoma in nevus sebaceus

We report two cases of an unusual combined adnexal neoplasm arising in a nevus sebaceus (NS). Clinically, both neoplasms presented in two women (46 and 78 years) as single, partially ulcerated nodules within NS situated on the scalp. Histopathologically, each neoplasm showed distinctive aggregations of basaloid cells with features of trichoblastoma adjacent to aggregations of neoplastic cells exhibiting features of sebaceoma. In both cases, typical features of NS were present. To the best of our knowledge, this unusual combined adnexal neoplasm comprised of trichoblastoma and sebaceoma could not be assigned to any previously described histopathologic entity. This "complex" adnexal neoplasm should be distinguished histopathologically from basal cell carcinoma with sebaceous differentiation and trichoblastoma with sebaceous differentiation.     

Trichoblastoma,syringocystadenoma papilliferum,desmoplastic trichilemmoma and tumor of the follicular infundibulum with signet‐ring cells,all arising in nevus sebaceus

Herein, we describe a 63‐year‐old male with multiple tumors arising within a nevus sebaceus on the posterior scalp. On histopathologic examination, four distinct tumors were identified: trichoblastoma, syringocystadenoma papilliferum, desmoplastic trichilemmoma and tumor of the follicular infundibulum (TFI). Within the TFI component of the nevus sebaceus, there was intracytoplasmic accumulation of eosinophilic keratin, as shown on pancytokeratin‐stained sections, imparting a signet‐ring appearance to the cells. To our knowledge, this is the first report of signet‐ring cells arising within a TFI occurring in a nevus sebaceus. We discuss this case as well as review the literature on multiple tumors arising within nevus sebaceus and signet‐ring cell changes in primary cutaneous tumors.     

Schimmelpenning-Feuerstein-Mims syndrome with hypophosphatemic rickets

The Schimmelpenning-Feuerstein-Mims syndrome (SFM syndrome) is a rare and variable multisystem defect consisting of congenital, extensive linear nevus sebaceus and associated abnormalities in different neuroectodermal organ systems. We present the history of a 52-year-old female patient with disproportionate hyposomia and asymmetric constitution. From birth she suffered from a right-sided, extensive nevus sebaceus following Blaschko's lines extending on the scalp, neck, right arm and trunk. At the age of 5 years, she developed a generalized growth retardation, along with deformations of bones. At the age of 11, hypophosphatemic rickets was diagnosed causing this growth retardation. Moreover, the patient developed a precocious puberty at the age of 9 years. When we saw the patient 40 years after the diagnosis had been made, phosphaturia had returned to normal. Specific therapy of hypophosphatemic rickets is straightforward and efficient in preventing late complications like growth retardation. We suggest to conduct appropriate laboratory tests in early childhood in patients with an extensive systematized sebaceous nevus or with additional signs of growth retardation or skeletal involvement, in order to exclude hypophosphatemic rickets associated with SFM syndrome.     

Nevus sebaceus associated with major ophthalmologic abnormalities

Nevus sebaceus rarely occurs as part of a syndrome consisting of central nervous system and ophthalmologic abnormalities. We describe a case of nevus sebaceus associated with an epibulbar complex choristoma and colobomas of the optic disc and peripapillary choroid, and review the dermatologic, ophthalmologic, and neurologic literature on the nevus sebaceus syndrome. When associated with other developmental abnormalities, nevus sebaceus and epidermal nevus have erroneously been considered to be a single entity. In this article, we delineate and emphasize the ophthalmologic abnormalities associated with the nevus sebaceus syndrome.     

Sebaceous gland neoplasia

Neoplasms with sebaceous differentiation cover a broad spectrum. They range from benign sebaceous hyperplasia and adenomas to locally infiltrative sebaceous epitheliomas which have biologic behaviors similar to basal cell carcinomas, to sebaceous carcinomas which can have very malignant biologic behaviors. In this paper, we have purposely excluded the nevus sebaceus of Jadassohn, a nevoid tumor composed of several cutaneous elements. As sebaceous glands are only one part of this tumor, it is better classified as an organoid nevus.     

Apocrine cystadenoma arising in a nevus sebaceus of Jadassohn

An increased incidence of benign and malignant adnexal tumors has been noted to occur within the nevus sebaceus of Jadassohn. We report a case of a 41-year-old white woman with a blue cyst on her scalp which had recently developed within a congenital "birthmark." Excision of the lesion revealed that the nodule was an apocrine cystadenoma within a nevus sebaceus of Jadassohn. The clinical features of nevus sebaceus with apocrine cystadenoma are discussed and the various tumors which may arise within the nevus sebaceus are reviewed.     

PNA-binding sites in sebaceous tumors

The presence and distribution of peanut agglutinin (PNA)-binding sites was studied in normal sebaceous glands, nevus sebaceous, senile sebaceous hyperplasia, sebaceous adenoma, sebaceous epithelioma, and sebaceous carcinoma. Cell surface staining and sponge-like cytoplasmic staining was observed in sebaceous glands, nevus sebaceus and sebaceous hyperplasia. In sebaceous adenoma, sebaceous epithelioma, and sebaceous carcinoma, diffuse cytoplasmic staining was observed in addition to cell surface staining.     

Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases

Nevus sebaceus of Jadassohn is a hamartoma that combines epidermal, follicular, sebaceous, and apocrine gland abnormalities. Classically, several types of cutaneous neoplasms have been associated with this hamartoma, with basal cell carcinoma being the most frequently described malignancy. We studied a series of 155 examples of nevus sebaceus of Jadassohn with clinicopathologic correlation. Several histopathologic changes related to the age of the patients were found. In our series, we could not identify any cases of authentic basal cell carcinoma. In contrast, several examples of primitive follicular induction and of trichoblastomas were seen. Other cutaneous hamartomas, hyperplasias, and neoplasms found in our series of nevus sebaceus of Jadassohn included syringocystadenoma papilliferum, sebomatricoma, apocrine gland cyst, poroma, different histopathologic variants of warts (classic warts, tricholemmoma, and desmoplastic tricholemmoma), primitive follicular induction, and ductal induction. In our series, no examples of malignant neoplasms were identified. On the basis of these findings, the classically recommended treatment for this hamartoma, which consists of early excision to prevent the development of malignancy, seems to be inappropriate.     

Multiple tumors arising in nevus sebaceus.

A forty-three year old woman with nevus sebaceus of Jadassohn involving the scalp is described. The unusual features of this case include the presence of adenoidal basal cell epithelioma, pigmented basal cell epithelioma, syringocystadenoma papilliferum, and nevocytic nevus. This case suggests the importance of prophylactic removal and close follow-up of nevus sebaceus.     

The effect of sebocytes cultured from nevus sebaceus on hair growth

Sebaceous glands are known to affect hair growth. Nevus sebaceus, a sebaceous gland hamartomas, presents as hairless patches. In this study, cultures of nevus sebaceus sebocytes (NSS) and normal scalp hair follicle sebocytes (NS) were used in performance of microarray, RT‐PCR, western blot assay and immunofluorescence staining. NSS‐ and NS‐conditioned media were also added to the culture of outer root sheath cells (ORSCs), dermal papilla cells (DPCs) or normal scalp hair follicle sebocytes. Results of this study showed a decrease in the survival rate of ORSCs and DPCs and hair growth in the NSS‐conditioned medium‐treated group, compared with the control and NS‐conditioned medium‐treated groups. An increase in expression of fibroblast growth factor (FGF)‐5, Dickkopf‐1 and inflammatory cytokines and a decrease in expression of Wnt10b and Lef1 were observed. In conclusion, NSS showed an increase in expression of hair growth‐suppressing bioactive factors, including FGF‐5, and a decrease in expression of hair growth‐stimulating factors.