CASE REPORT SOLITARY SEBACEOUS NEVUS OF JADASSOHN COMPLICATED BY SQUAMOUS CELL CARCINOMA AND BASAL CELL CARCINOMA

Transcription

1 CASE REPORT Dennis H. Kraus, MD, Section Editor SOLITARY SEBACEOUS NEVUS OF JADASSOHN COMPLICATED BY SQUAMOUS CELL CARCINOMA AND BASAL CELL CARCINOMA Ahmad Ridzwan Arshad, FRCS, 1 Wan S. Azman, MS, 1 Ayadurai Kreetharan, MPath 2 1 Department of Plastic and Reconstructive Surgery, Hospital Kuala Lumpur, Jalan Pahang, Kuala Lumpur, Malaysia. araprs@hkl.gov.my 2 Department of Pathology, Hospital Kuala Lumpur, Jalan Pahang, Kuala Lumpur, Malaysia Accepted 29 May 2007 Published online 30 October 2007 in Wiley InterScience ( DOI: /hed Abstract: Background. Sebaceous nevus is a benign congenital epidermal nevus. Its association with basal cell carcinoma is well known. Method. This is a case report of sebaceous carcinoma complicated by both basal cell carcinoma and squamous cell carcinoma. Results. The behavior of this tumor is very aggressive, resulting in poor prognosis. Conclusions. All sebaceous nevi should be excised early. VC 2007 Wiley Periodicals, Inc. Head Neck 30: , 2008 Keywords: sebaceous nevus; basal cell carcinoma; organoid nevus; squamous cell carcinoma; Jadassohn Sebaceous nevus or organoid nevus is a benign congenital epidermal nevus, which can present as a solitary lesion or in association with other benign or malignant skin lesions as well as with other system involvement. 1,2,3 The association of Correspondence to: A. R. Arshad VC 2007 Wiley Periodicals, Inc. sebaceous nevus with benign and malignant skin lesions are well documented. Basal cell carcinoma is the most common malignant lesion that can arise from a sebaceous nevus. There have been limited reports on other malignant skin tumors in association with sebaceous nevus. We report a patient in whom both squamous cell carcinoma and basal cell carcinoma arose in a solitary sebaceous nevus. Its aggressive behavior justifies early excision of these lesions. CASE REPORT A 55-year-old man presented with an ulcerative growth of 5 months duration on the right temporoparietal scalp. The growth developed within an alopecic patch that had been present since birth. The lesion was 5 cm 3 4 cm in size, had an everted edge and irregular surface, looking typically like squamous cell carcinoma. The base was surrounded by a verrucous, warty lesion, looking like a sebaceous nevus (Figures 1 and 2). There was no local or regional lymphadenopathy. Wide excision 544 Sebaceous Nevus Complicated with BCC and SCC HEAD & NECK DOI /hed April 2008

2 FIGURE 1. A 55-year-old man with a fungating lesion in a sebaceous nevus in right temporal scalp. [Color figure can be viewed in the online issue, which is available at wiley.com.] FIGURE 2. Close-up view of lesion. [Color figure can be viewed in the online issue, which is available at com.] was performed, and the defect was covered with split-thickness skin graft. Histopathologically, the specimen was composed of invasive clusters and sheets of malignant squamous cells. These cells were large and polygonal, exhibiting pleomorphic vesicular nuclei with prominent nucleoli and a moderate amount of eosinophilic cytoplasm. An increase in mitotic activity was evident. A separate area shows a second tumor composed of relatively uniform oval-shaped basophilic cells, exhibiting peripheral pallisading. It also showed an increase in mitotic activity. In addition, there were features of papillomatosis and acanthosis (Figures 3 6). Also noted was the enlargement of sebaceous glands, which are seen high up in the dermis. The tumor was therefore reported as moderately differentiated squamous cell carcinoma and basal cell carcinoma arising in nevus sebaceous. Three months later, the patient presented with multiple swellings behind his right ear (Figure 7). Further wide excision in continuity with a right radical neck dissection was performed, followed by radiotherapy. This specimen was reported as a recurrent squamous cell carcinoma with a single level IV cervical node metastasis. Five months later, the patient returned with lung metastases (Figure 8) and died within 2 months, 15 months after the first diagnosis. FIGURE 3. Photomicrograph shows features of sebaceous nevus and basal cell carcinoma (original magnification 3 4). Sebaceous Nevus Complicated with BCC and SCC HEAD & NECK DOI /hed April

3 FIGURE 4. Photomicrograph shows features of sebaceous nevus and basal cell carcinoma (original magnification 3 4). FIGURE 6. Squamous cell carcinoma with adjacent area showing features of sebaceous nevus ( magnification). DISCUSSION Sebaceous nevus, also called organoid nevus, 3 is a benign, well-circumscribed, verrucose, raised, irregular, hairless plaque, most commonly seen in the scalp, less often on the face, neck and trunk. It was first described by Jadassohn in It appears at birth and largely remains benign. It has been described as having 3 stages in its life history. 3 5 The early infantile stage is characterized by papilomatous epithelial hyperplasia and underdevelopment of hair. During puberty, there is massive overdevelopment of sebaceous glands, with epidermal hyperplasia and maturation of apocrine glands. Later in life, in the fourth to seventh decade, they may develop neoplasm of epidermal, adnexal, or mesenchymal origin, the most common being basal cell carcinoma. FIGURE 5. Squamous cell carcinoma with adjacent areas showing features of sebaceous nevus (4 3 3 magnification). FIGURE 7. Appearance of enlarged right postauricular lymph nodes 3 months after excision and grafting. [Color figure can be viewed in the online issue, which is available at wiley.com.] 546 Sebaceous Nevus Complicated with BCC and SCC HEAD & NECK DOI /hed April 2008

4 FIGURE 8. Chest X-ray taken 5 months after radical neck dissection shows left pleural effusion. The treatment of sebaceous nevus of Jadassohn has been a subject of much debate. Surgical treatment of this benign lesion is by simple excision, and for a much bigger lesion, especially of the scalp, the defect may need to be skin grafted. When sebaceous nevi are not removed, they may continue to remain benign or they may develop other tumors secondarily within them, such as syringiocystadenoma papilliferum, nodular hidradenoma, syringoma, sebaceous epithelioma, chondroid syringoma, trichilemmoma, and trichilemmal cyst. 1 Various malignant tumors may also arise from sebaceous nevus, namely basal cell carcinoma and, rarely, squamous cell carcinoma. Basal cell carcinoma has been observed in 5% to 7% of sebaceous nevus, 1 but there are others who say that it is variable. 3 Domingo and Helwig 5 in 1979 reviewed 997 cases and found 1 case of sebaceous nevus on the scalp that developed squamous cell carcinoma. This patient was treated with surgery and radiotherapy. In spite of the aggressive treatment given, the patient developed generalized metastases and died within 11 months of diagnosis. Wilson Jones and Heyl 3 in their review of 140 cases of nevus sebaceous, cited Schirren and Pfistinger who reported a case of squamous cell carcinoma arising in sebaceous nevus, which again showed a very aggressive behavior in the form of recurrences, after excision and radiotherapy, and lymph node metastasis. Hidvegi et al 4 reported squamous cell carcinoma complicating naevus sebaceous in a child, but they have not reported their follow-up findings and disease progress. Jaqueti et al, 6 in a multicentre study of 155 cases of nevus sebaceous, was unable to identify any malignant lesion arising from them, but found other hamartomas, hyperplasia, and benign neoplasms that include syringoadenoma papillifarum, sebomatricoma, apocrine gland cyst, poroma, different histologic variants of warts, primitive follicular induction, and ductal induction. Based on these findings, they recommend that early excison, to prevent development of malignancy, is inappropriate. We report a case in which both squamous cell carcinoma and basal cell carcinoma arose from a solitary sebaceous nevus on the scalp of an adult. To our knowledge, this is only the third case that has been reported in the literature. 7 As has been described by previous authors, 5 squamous cell carcinomas, when they arise from sebaceous naevus, have a very aggressive behavior. Within a few months of initial treatment, they have developed local recurrences and metastasized to regional lymph nodes. The enlargement of this recurrence was also very rapid. A wide excision of this recurrence in continuity with a radical neck dissection was performed. The patient was subsequently referred to the oncologist for radiotherapy. While under our follow-up, this patient developed spread to the lungs, rapidly deteriorated, and died within 15 months after being initially seen. The rapid clinical progress of this case followed almost the same course as the case reported by Domingo and Helwig 5. We therefore would like to suggest that every case of sebaceous nevus should be excised early, preferably before puberty. In conclusion, this is a case report of a sebaceous nevus of Jadassohn that was complicated by the presence of squamous cell carcinoma and basal cell carcinoma. It exhibited a very aggressive behavior, leading to its poor prognosis. It is therefore recommended that early excision of sebaceous nevus should be done in every case. REFERENCES 1. Elder D, Elenitsas R, Ragsdale BD. Tumour of the epidermal appendages. In: Elder D, Elenitsas R, Jaworsky C, Johnson B, Editor. Lever s histopathology of the skin, 8th ed. Philadelphia: Lippincot-Raven; pp Weedon D, Strutton G. Skin pathology, 2nd ed. New York: Churchill Livingstone; pp Sebaceous Nevus Complicated with BCC and SCC HEAD & NECK DOI /hed April

5 3. Wilson Jones E, Heyl T. Naevus sebaceous. A report of 140 cases with special regard to the development of secondary malignant tumours. Br J Dermatol 1970;82: Hidvegi NC, Kangesu L, Wolfe KQ. Squamous cell carcinoma complicating naevus sebaceous of Jadassohn in a child. Br J Plast Surg 2003;56: Domingo J, Helwig E. Malignant neoplasms associated with nevus sebaceous of Jadassohn. J Am Acad Dermatol 1979;1: Jaqueti G, Requena L, Yus ES. 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: Ball EAM, Hussein M, Moss ALH. Squamous cell carcinoma and basal cell carcinoma arising in a naevus sebaceous of Jadassohn: case report and literature review. Clin Exp Dermatol 2005;30: Sebaceous Nevus Complicated with BCC and SCC HEAD & NECK DOI /hed April 2008