Syringocystadenoma Papilliferum Developed on Sebaceous Nevus
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Eur. J. Pediat. Dermatol. 19, 83-6, 2009 Syringocystadenoma papilliferum developed on sebaceous nevus. Three pediatric cases. Guareschi E., Di Lernia V. U.O. Dermatologia, Arcispedale Santa Maria Nuova Reggio Emilia (Italy) Summary Sebaceous nevus is a congenital organoid nevus. It is mainly located on the scalp and appears as a hairless yellowish plaque at birth or soon after. Sebaceous nevus changes with growth being sebaceous glands responsive to hormones at puberty. Since benign and ma- lignant tumors can arise within sebaceous nevus, short follow-up and surgical excision are probably the better medical behavior to manage these lesions when changes appear. Here are reported three pediatric cases of syringocystadenoma papilliferum developed in the con- text of sebaceous nevus. Key words Sebaceous nevus, syringocystadenoma papilliferum. ebaceous nevus is a common congenital these tumors increases with age, in particular af- lesion occurring mainly on the head, par- ter puberty. ticularly on the scalp. It was firstly descri- Another significant association of sebaceous Sbed in 1895 from Jadassohn as a hamartomatous nevus is a neurocutaneous syndrome known as lesion predominantly composed of sebaceous linear nevus sebaceus syndrome (Schimmelpen- glands. ning-Feuerstein-Mims syndrome, Solomon syn- Pinkus defined this disease as “organoid ne- drome), which is a subtype of the wider epidermal vus” being the anomalies not only relative to se- nevus syndrome. The commonest extracutaneous baceous glands but also to sweat glands and hair manifestations in patients with epidermal nevus follicles (8). syndrome involve the central nervous system, Sebaceous nevus appears as an alopecic yel- ocular and skeletal systems (5). lowish patch at birth or early childhood and tends Here we report three cases of SCAP arising to become raised, papillomatous at puberty as a on sebaceous nevus in childhood and discuss the result of the effect of the androgen hormones on therapeutic approach to these complex lesions. the sebaceous glands. Numerous benign and malignant tumors are known to arise within sebaceous nevus (5). The Case report commonest benign neoplasms are syringocysta- denoma papilliferum (SCAP) and trichoblasto- We present three cases of SCAP arising on se- ma, whereas the commonest malignant one is baceous nevus in pediatric patients. The data of basal cell carcinoma (BCC). The incidence of our patiens are summarized in Table 1. 83 Guareschi, Di Lernia Table 1: Data of patients with syringocystadenoma papilliferum arisen on sebaceous nevus. Case 1 Case 2 Case 3 Tumor SCAP SCAP SCAP (Fig. 1) Age (years) 12 10 15 Site of SN Trunk Left temporal area Frontal (hairline) Clinical presentation Nodule Nodule (Fig. 2) Nodule Excision Entire lesion Nodule Entire lesion Discussion glands with small hair follicles, which remain primordial. Often also apocrine glands with dila- Sebaceous nevus is an organoid nevus present ted lumina and hyperplasia of eccrine glands can mostly at birth or early childhood in 0.05-1% be found. The third, adult stage, is characterized of newborns (1). It presents as an alopecic yel- by epidermal hyperplasia, large sebaceous glands lowish plaque mainly located on the scalp and and ectopic apocrine glands. The hair follicles re- face. Other localizations are rare but possible. main primordial. In the last stage several benign Three clinico-pathological stages have been and malignant tumors can occur (7). identified in the evolution of this congenital le- Among the benign tumors arising on seba- sion. In the first, infantile stage, clinically it ap- ceous nevus Shapiro et Al. (7) include 1- tumors pears with smooth surface and yellowish-orange with pilar differentiation such as trichoblastoma, color. Hystopathologically it shows slightly acan- desmoplastic trichilemmoma, trchoepitelioma, thotic and hyperpigmented epidermis and in the follicular and pilar hamartoma, proliferating tri- dermis hair follicles are small and incompletely chilemmal cyst, follicular infundibuloma; 2- ba- formed, often represented by solid cords of basa- saloid neoplasms with follicular differentiation loid, undifferentiated cells. Sebaceous glands are such as epidermal inverted follicular keratosis; not prominent. In the second, adolescent stage, 3- tumors with sebaceous differentiation such sebaceous nevus becomes clinically thicker with as sebaceous adenoma, sebaceous epithelioma; surface nodules or verrucous hyperkeratosis. Hi- 4- tumors with apocrine differentiation such as stologically, there is verrucous epidermal hyper- tubular apocrine adenoma, apocrine cystadeno- plasia and in the dermis hyperplasia of sebaceous ma, apocrine hidrocystoma, apocrine epithelio- Fig. 1 Fig. 2 Fig. 1, 2: SCAP (Fig. 1) on sebaceous nevus -case 3-. In Fig. 2 pathological findings of SCAP -case2-. 84 Syringocystadenoma papilliferum on sebaceous nevus ma, syringocystadenoma papilliferum, apocrine gnant one. Although it is somewhat difficult to hamartoma, apocrine nevus; 5- tumors with ec- differentiate BCC from trichoblastoma arising in crine differentiation such as spiradenoma, no- sebaceous nevus, some studies have outlined a dular hidradenoma/acrospiroma, syringoma and number of morphologic criteria useful in diffe- 6- other tumors such as piloleiomyoma, osteoma, rentiating these two neoplasms (4). nevocellular nevus, proliferating epidermal cyst, For a pediatric dermatologist the matter is to epithelioma adenoides cysticum. decide if a child with sebaceous nevus must be Among the malignant tumors arising in seba- followed up during years or if he/she must be ceous nevus the same Authors (7) include: basal addressed to surgery. Probably the correct beha- cell carcinoma, squamous cell carcinoma, kera- vior is to inform the patient and/or his/her parents toacanthoma, apocrine carcinoma, eccrine po- about the possible occurrence of this complica- rocarcinoma, sweat gland carcinoma, anaplastic tion and its rate according to the age. When a tu- salivary gland adenocarcinoma, sebaceous carci- mor or bleeding occurs, the patient should contact noma, adnexal carcinoma with pilar differentia- the dermatologist. In case of suspected tumor, the tion and mucoepidermoid carcinoma. latter will advise removal of the entire nevus or Many works have been done to define the risk only the tumor, according to the individual case. of development of tumors in the context of a se- After puberty the patient should be informed baceous nevus. However, during the past years about the need for a dermatological examination there was no agreement between the Authors. in case of clinical changes of sebaceous nevus. In Only recently different large studies reached the this event surgical excision of the lesion must be same conclusions. Cribier et Al. (2) found a total undertaken. of 13.6% of benign tumors -less than 2% in seba- Indeed it is of importance to underline that, ceous nevus excised in children, 0% under 10 ye- even if the most frequent tumor arising in seba- ars- and 0.8% of malignant -none in children- in ceous nevus is a benign tumor as SCAP, it can ex- their 596 cases. Santibanez-Gallerani et Al. found ceptionally evolve in its malignant counterpart, no basal cell carcinoma (BCC) in their sebaceous syringocystoadenocarcinoma papilliferum, with nevus series of 658 cases of patients aged 16 important consequences on the prognosis (3). years or less. They did not found either benign In conclusion, our 3 cases confirm the data of tumors (6). Kaddu et Al. analized 316 cases of the literature regarding the age of risk for develo- SN and found 7.6% of benign tumors -only in ping of tumors within congenital sebaceous ne- adults- and two malignant tumors -only in adults vus. Our youngest patient was 10-years-old and as well- (4). Munoz-Perez et Al. found 18% of the other 2 patients were older. Also the histologic benign tumors in their series of 226 patients, all type of tumor of our patients is in accordance with in subjects above the age of fourteen (5). These the data reported from the literature, being SCAP Authors agree about the conclusion that tumors the most frequent neoplasm which can arise in a arising on SN are exceptional under the age of sebaceous nevus, developing in until 19% of the ten years and almost rare until puberty. After this of sebaceous nevus (7). Interestingly, two of our period the incidence increases with age. patient had a sebaceous nevus of the head, while In adulthood also malignant tumors can ap- the third had a sebaceous nevus of the trunk. The pear in until 40% of sebaceous nevus (1). SCAP latter localization is quite peculiar because seve- and trichoblastoma are the most frequent benign ral Authors agree that most tumors in sebaceous tumors, while BCC is the most frequent mali- nevus are usually located on the scalp (2). Address to: Dr. Vito Di Lernia U.O. Dermatologia, Arcispedale Santa Maria Nuova, Viale Risorgimento 80 42100 Reggio Emilia (Italy) e-mail: [email protected] 85 Guareschi, Di Lernia References 1) Baykal C., Buyukbabani N., Yazganoglu D., Saglik E. study. J. Eur. Acad. Dermatol. Venereol. 16, 319-24, - Tumors associated with nevus sebaceous. 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