Introduction

Nevus sebaceous of Jadassohn (NSJ), first described by Joseph Jadassohn in 1895, is an organoid nevus of pilosyringosebaceous and adnexal origin that combines sebaceous gland abnormalities with anomalies of the epidermal, follicular, and apocrine glands (1,2).

NSJ typically presents at birth as linear/ovoid plaque most commonly on the face and scalp and progresses through self-differentiation into multiple neoplasms in late adulthood (3-5). However, reports of more than three simultaneous tumors from a single nevus and their malignant transformation are extremely rare (6,7). Here, we describe the features of multiple synchronous benign and malignant neoplasms arising from a long-standing nevus sebaceous of the scalp.

Case Presentation

A 70-year-old male patient was admitted with a complaint of a painful slow-growing ulcer in the right buccal mucosa and swelling in the scalp, which had grown slowly over years to its current size with occasional itching. The patient had a history of chronic tobacco use and betel quid chewing. On physical examination, a lesion that was irregularly surfaced, ulcerated, and proliferating onto the mandibular alveolar process was detected along with asymptomatic solitary dome-shaped nodular growth measuring approximately 7.5x4.5 cm over the left parietooccipital region of the scalp (Figure 1). The lesion was hyperpigmented and soft to firm. An incisional biopsy of the oral lesion was performed and the histopathological evaluation was consistent with a well-differentiated squamous cell carcinoma (stage IVa). The intra-oral lesion was treated by wide excision with hemimandibulectomy; nerve and vein sparing type of neck dissection and reconstruction of the defect were carried out with pectoralis major myocutaneous flap.

Fine-needle aspiration of the lesion on the scalp was reported as syringocystadenoma papilliferum (SCAP). The lesion on the scalp was surgically excised and the defect was covered with a split-thickness skin graft.

Histopathological examination of the lesion revealed multiple lesions in the primary NSJ, namely SCAP, tubular apocrine adenoma (TAA), eccrine poroma (EP), apocrine cystadenoma (ACA), cutaneous apocrine carcinoma (CAC) and cutaneous apocrine carcinoma, with invasive areas (Figure 2A-G). Immunohistochemical studies ruled out basal cell carcinoma and malignant melanoma. S100 for nerve sheath tumors and malignant melanoma, CK 17 and cytokeratin screening for epithelial origin tumors, and CD 99 for mesenchymal tumors/soft tissue sarcomas were studied during histopathological evaluations.

Discussion

NSJ follows three phases of its natural history designated by Mehregan and Pinkus (8) as infancy, pubertal stimulation, and post-pubertal histological degeneration; usually appears at birth (0.1-0.3% among newborns) or early childhood as a well-circumscribed, smooth, slightly raised, yellowish-brown alopecic patch that progresses through puberty, assuming a more verrucous texture, possibly due to the expression of various hormonal receptors (4). There have been only a few reported cases in the literature describing the simultaneous development of 5 or more neoplasms in a solitary lesion (2).

SCAPs are the most common hamartoma tumors associated with nevus sebaceous, which originate from the apocrine sweat glands (4). Stavrianeas report an incidence of 5 to 19% of the nevi complicated with SCAP (1). Section from our patient’s slides revealed a stratified squamous epithelium with immature sebaceous glands and areas of basaloid hyperplasia along with immature hair structures and dilated ductal infundibulum in the dermis (Figure 2B).

TAA is a minor variant of SCAP consisting of a benign appendage tumor of apocrine origin. TAA and SCAP rarely develop together in the nevus sebaceous, and only a few such cases had been reported in 2004 (9). Sections of our slides showed a well-circumscribed dermal neoplasm comprising lobules of dilated tubules lined by two rows of cuboidal to columnar epithelial cells with eosinophilic cytoplasm and round bland nuclei (Figure 2C).

EP is a benign tumor originating from the epidermal sweat glands and occurs in middle-aged individuals on the sole and hands. However, on the scalp is extremely rare, with 18 cases reported until the year 2012 (10). Sections of our slides showed a downward proliferation of basaloid cells from the epidermis with delicate fibrovascular stroma in the form of anastomosing cords and nests with pigmentation (Figure 2D).

ACA is a rare, benign, solitary adenomatous cystic tumor of the skin. Sections of our slide revealed cystic areas in the dermis lined by columnar cells with small round basal nuclei and abundant eosinophilic cytoplasm arranged in a papillary pattern with a fibrovascular core (Figure 2E).

CAC is a rare malignancy with unknown etiology and only eight cases are reported in 2012 (6). We found many layers of pleomorphic cells with irregular hyperchromatic nuclei, prominent nucleoli, and abundant granular eosinophilic cytoplasm (Figure 2F). There was the presence of atypical mitoses, decapitation secretion, and tumor tissue invasion into the papillary and reticular dermis (Figure 2G).

Conclusion

The current case is in agreement with most authors who have suggested that NSJ should be surgically excised, histologically examined, and should be kept on a close follow-up to prevent malignant transformation.

Ethics

Informed Consent: Informed written consent has been obtained from the patient for publication.

Peer-review: Externally and internally peer-reviewed.

Authorship Contributions

Surgical and Medical Practices: D.S., A.D., N.K., D.U.S., A.A., Concept: D.S., A.D., N.K., D.U.S., Design: D.S., A.D., Data Collection or Processing: D.S., A.A., S.B., Analysis or Interpretation: D.S., A.D., N.K., D.U.S., A.A., Literature Search: D.S., S.B., Writing: D.S., S.B.

Conflict of Interest: No conflict of interest was declared by the authors.

Financial Disclosure: The authors declared that this study received no financial support.