Apocrine carcinoma developed in nevus sebaceus of Jadassohn

ARTICLE

Auteur(s) : Stéphane DALLE, François SKOWRON, Brigitte BALME, Henri PERROT

Service de Dermatologie, Hôpital de l‘Hôtel‐Dieu, 1 place de l‘Hôpital, 69288 Lyon cedex 02 France

Article accepted 22\5\2003

Apocrine skin carcinoma (ASC) is a rare tumor. It usually occurs in apocrine gland areas, in order of frequency in the axilla, external ear, eyelid and anogenital regions. When it occurs on the scalp it is always secondary to a nevus sebaceus of Jadassohn (NSJ).

Case report

A 66‐year‐old man was referred to our unit for a tumour of the scalp which had first appeared six months previously (Fig. 1). The patient described a pre‐existing hairless plaque since childhood. Clinical examination of the occipital scalp revealed two nodules, respectively 0.3 and 0.8 cm in diameter, on a yellowish plaque measuring 3 × 2 cm. We also noticed a right sub maxillary adenopathy, measuring 1.5 × 1.5 cm. The plaque with the two nodules was surgically excised and histopathologic analysis (Fig. 2) of the plaque revealed a typical aspect of nevus sebaceus with epidermal hyperplasia, hyperplastic sebaceous glands and presence of apocrine glands. The first nodule on the plaque was a basaloid proliferation with clefts in a fibrotic stroma, aggregations with smooth borders and a symmetrical aspect corresponding to a trichoblastoma. The second nodular formation (Fig. 3), with a multilobulated aspect over the nevus, was a well‐differentiated adenocarcinoma forming large glandular lumina with decapitation secretion of lining neoplastic cells. It was an exophytic and endophytic tumor, some glandular lumina were cystically dilated and contained eosinophilic material resembling apocrine gland lumina. Atypical cells were organized in solid nests. Their nuclei were large, with prominent nucleoli. Some mitotic figures were observed. Infiltrative growth of tumor cells was observed in almost all the thickness of the dermis. Intracytoplasmic granules were periodic‐acid‐Schiff‐positive and diastase‐resistant. Immunohistochemistry of these cells showed a positive staining with carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), and monoclonal anticytokeratin 7 antibody. Neoplastic cells were also positive with CD15 (Leu M1). From these observations we conclude that there was a cutaneous apocrine carcinoma arising from a nevus sebaceus and associated with a trichoblastoma. Others investigations, which were limited to a chest radiography and abdominal ultrasonography, were negative. Further investigation was not possible as the patient refused any other examinations or treatment.

Discussion

Nevus sebaceus, described by J. Jadassohn in 1895 [1], is a hamartoma that combines epidermal, follicular, sebaceus and apocrine gland abnormalities. It presents as a congenital hairless plaque, located either on the head or neck. Its surface classically becomes irregular during puberty. In adulthood various appendage tumors frequently develop within about 14 % of NSJ, but these are mainly benign [2, 3]. The most frequent benign lesions are syringocystadenoma papilliferum (5 %) and trichoblastoma (4.5 %) [4], while trichilemmoma (2.5 %) and sebaceoma (2.1 %) are less frequent [4, 6, 7].

Fewer than 1 % of NSJ are complicated by malignant tumors according to recent retrospective studies [7‐9]. The most frequently described carcinomas are basal cell carcinomas (BCC) and squamous cell carcinomas (SCC) [10‐13]. BCC is an uncommon neoplasm in nevus sebaceus, and most of the lesions that have previously been interpreted as BCC were in fact trichoblastomas [4, 5]. Recently one case of sebaceus carcinoma and one case of tricholemmal carcinoma were found in adults in association with NSJ [14, 15]. There has only been one published case of an adult patient with a metastatic porocarcinoma [16]. There have been 5 cases of ASC developed within a NSJ in adult subjects reported, two of which were already metastatic and one case of sweat‐gland carcinoma [17, 18, 19].

ASC is a rare tumor. Its clinical presentation as an asymptomatic cutaneous or subcutaneous nodule is non specific. It usually occurs in areas rich in apocrine or modified apocrine glands (cerumina and Moll‘s glands) although in some instances ASC can occur in areas usually lacking apocrine glands, such as the wrist [20, 21]. ASC of the scalp is always associated with NSJ. Local recurrences and regional lymph node metastases may even occur many years after the first excision, and can ultimately be fatal [22].

Malignant degeneration of NSJ during childhood or teenage years is exceptional. We found only two cases of malignant tumor in a child, a BCC occurring in a 7‐year‐old boy [13] and a squamous cell carcinoma in a 15‐year‐old girl [23]. Recent retrospective studies which analysed the histologic findings on NSJ removed during childhood, adolescence or in young adults found little malignant degeneration. However, in the largest study, malignant tumors were found on 1.2 % of the 19 to 40‐year‐old group and up to 2.1 % when patients were older than 40 years [8]. We believe that the development of aggressive tumors on NSJ in adults such as the ASC in our case, justifies the preventive surgical excision of the lesions, or at least a prolonged surveillance of these patients.

Acknowledgements. We want to thank Keith Veitch for his English review of the manuscript.

References

1 . Jadassohn J. II Bemerkungen zur Histologie der systematisierten Naevi und ueber Talgduesen‐Naevi. Arch Dermatol Syph 1895 ; 33 : 355‐394.

2 . Yoon DH, Jang IG, Kim TY, Kim HO, Kim CW. Syringocystadenoma papilliferum, basal cell carcinoma and trichilemmoma arising from nevus sebaceous of Jadassohn. Acta Derm Venereol 1997 ; 77 : 242‐3

3 . Rosenblum GA. Nevus sebaceous, syringocystadenoma papilliferum, and basal cell epithelioma. J Dermatol Surg Oncol 1985 ; 11 : 1018‐20.

4 . Kaddu S, Schaeppi H, Kerl H, Soyer HP. Trichoblastoma and sebaceoma in nevus sebaceus. Am J Dermatopathol 1999 ; 21 : 552‐6.

5 . Roson E, Gomez Centeno P, Sànchez‐Aguilar D, Peteiro C, Toribio J. Desmoplastic trichilemmoma arising within a nevus sebaceus. Am J Dermatopathol 1998 ; 20 : 495‐7.

6 . Hunt SJ, Kilzer B, Santa Cruz DJ. Desmoplastic trichilemmoma: histologic variant resembling invasive carcinoma. J Cutan Pathol 1990 ; 17 : 45‐52.

7 . Jaqueti G, Requena L, Sanchez Yus E. Trichoblastoma is the most common neoplasm developed in nevus sebaceous of Jadassohn: a clinicopathologic study of a series of 155 cases. Am J Dermatopathol 2000 ; 22 : 108‐18.

8 . Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceous: a study of 596 cases. J Am Acad Dermatol 2000 ; 42 : 263‐8.

9 . Alessi E, Wong SN, Advani HH, Ackerman AB. Nevus sebaceous is associated with unusual neoplasms. An atlas. Am J Dermatopathol 1988 ; 10 : 116‐27.

10 . Smolin T, Hundeiker M. Plattenepithel‐und Basazellkarzinome im Naevus sebaceus (Jadassohn). Z Hautkr 1986 ; 61 : 267‐82.

11 . Lillis PJ, Ceilley RI. Multiple tumors arising in nevus sebaceus. Cutis 1979 ; 23 : 310‐4.

12 . Braitman M. Supervention of a pigmented basal‐cell carcinoma upon a nevus sebaceous. J Dermatol Surg Oncol 1979 ; 5 : 232‐3.

13 . Turner CD, Shea CR, Rosoff PM. Basal cell carcinoma originating from a nevus sebaceous on the scalp of a 7‐year‐old boy. Am J Pediatr Hematol Oncol 2001 ; 23 : 247‐9.

14 . Rinaggio J, McGuff HS, Otto R, Hickson C. Postauricular sebaceous carcinoma arising in association with nevus sebaceus. Head Neck 2002 ; 24(2) : 212‐6.

15 . Misago N, Narisawa Y. Tricholemmal carcinoma in continuity with trichoblastoma within nevus sebaceus. Am J Dermatopathol 2002 ; 24(2) : 149‐55.

16 . Tarkan II, Domingo J. Metastazing eccrine porocarcinoma developing in a sebaceous nevus of Jadassohn. Report of a case. Arch Dermatol 1985 ; 121 : 413‐5.17

. Domingo J, Helwig EB. Malignant neoplasms associated with nevus sebaceous of Jadassohn. J Am Acad Dermatol 1979 ; 1 : 545‐6.

18 . Jacyk WK, Requena L, Sanchez Yus E, Judd MJ. Tubular apocrine carcinoma arising in a nevus sebaceous of Jadassohn. Am J Dermatopathol 1998 ; 20 : 389‐92.

19 . Ansai S, Koseki S, Hashimoto H, Hozumi Y, Kondo S. A case of ductal sweat gland carcinoma connected to syringocystadenoma papilliferum arising in nevus sebaceus. J Cutan Pathol 1994 ; 21 : 557‐63. 20

. Paties C, Taccagni L, Papotti M, Valente G, Zangrandi A, Aloi F. Apocrine carcinoma of the skin. Cancer 1993 ; 71 : 375‐81.

21 . Katagiri Y, Ansai S. Two cases of cutaneous apocrine ductal carcinoma of the axilla. Dermatology 1999 ; 199 : 332‐37.

22 . Warkel L, Helwig EB. Apocrine gland adenoma and adenocarcinoma of the axilla. Arch Dermatol 1978 ; 114 : 198‐203.

23 . Hidvegi NC, Kangesu L, Wolfe KQ. Squamous cell carcinoma complicating naevus sebaceous of Jadassohn in a child. Br J Plast Surg 2003 Jan ; 56 : 50‐2.