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CASE REPORT| Volume 56, ISSUE 2, P171-173, March 2003

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Malignant melanoma arising in a sebaceous nevus of the scalp

      Abstract

      This is the first report of malignant melanoma arising from sebaceous nevus (SN) of the scalp, although it has been known that various benign and malignant neoplasms may develop in association with SN. After the excisional biopsy for SN with 5 mm free margin, pathological examination revealed the coexistence of nodular melanoma invading to the reticular dermis (IV level of Clark) for a maximal depth of 4 mm. After resection with another 20 mm free margin, there has been no evidence of local recurrence or distant metastasis for nine years.

      Keywords

      SN is an uncommon skin malformation first reported by Jadassohn in 1895.
      • Jadassohn J
      Bemerkungen zur Histologie der systematisierten Naevi und über ‘Talgdrüsen-Naevi’.
      Its clinical importance lies mostly in the potential for the development of various malignant or benign neoplasms
      • Mehregan A.H
      • Pinkus H
      Life history of organoid nevi. Special reference to nevus sebaceous of Jadassohn.
      as well as in its association with nevus sebaceous syndrome.
      • Mardem P.M
      • Venters Jr, H.D
      A new neurocutaneous syndrome.
      We report a case of malignant melanoma in a SN of the scalp. To our knowledge, malignant melanoma arising in a SN has not been described before.

      1. Case report

      A 47-year-old Japanese woman had had a hairless yellowish plaque on her back scalp from birth. A black nodular lesion appeared on the SN in 1992. Physical examination in 1993 revealed a yellowish mass, 8×20 mm in size, with a black lesion, 6×7 mm, which we suspected clinically to be a basal cell carcinoma (BCC) in SN (Fig. 1) . The regional lymph nodes were not involved. The lesion was excised with 5 mm border of normal scalp, including the epicranial aponeurosis, and closed simply. The histopathological diagnosis was nodular malignant melanoma (NM) in SN with invasion of the reticular dermis (Clark IV level), for a maximal depth of 4 mm (Fig. 2) . There was one mitotic figure per 10 high power fields and the cells were large spindle- or polygonal-shaped with prominent nucleoli.
      Figure thumbnail gr1
      Fig. 1Yellowish verrucous and nodular mass, 8×20 mm2 in size, with black and nodular lesion, 6×7 mm, on the scalp.
      Figure thumbnail gr2
      Fig. 2Histologic examination demonstrating malignant melanoma arising from preexisting sebaceous nevus. Nevus tissue was deviated by nodular melanoma, which has typical histopathological features of malignant melanoma. Hematoxylin and eosin stain. Original magnification × 15 (left), × 100 (right).
      Surrounding normal skin was widely resected, with a 2.0 cm margin around the previous scar including the epicranial aponeurosis. The area was then covered with meshed split thickness skin graft with pathological confirmation of disease-free margin. One cycle of neoadjuvant and two cycles of adjuvant combination chemotherapy with dacarbazine (DTIC), nimustine hydrochloride (ACNU), vincristine (VCR) and interferon beta were performed. The patient has had no evidence of local recurrence or distant metastasis and is disease free after nine years of follow-up.

      2. Discussion

      All constituents of human skin are derived from either ectoderm or mesoderm. The epithelial structures (epidermis, pilo-sebaceous-apocrine unit, eccrine unit and nails) are ectodermal derivatives. Nerves and melanocytes emanate from neuroectoderm and neural crest. Mesenchymal structures (collagen, reticulum, and elastic fibers, blood vessels, muscles, and fat), originate from mesoderm.
      SN can be defined as a hamartoma,
      • Albrecht E
      Ueber Hamartome.
      representing an organizational or embryogenetic defect resulting in an abnormal mixture of tissue of varying types. A variety of tumors arising in SN have been reported, originating from ectodermal or, less often, mesodermal tissues.
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      Table 1 shows the increased number of SN frequently observed in this hamartoma as summarised by Burden et al
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      where they divided these secondary tumors into six categories according to embryonic aspects. This is the first report that melanocytes, dendritic cells, from neural crest cells of ectodermal derivation had malignant changes in SN.
      Table 1Benign and Malignant Tumors Reported in Organoid Nevi
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      Epidermal Germ OriginFollicular Germ OriginEccrine Germ OriginEpithelial Origin—ControversialMesodermal OriginNeural Crest Origin
      Epidermal nevusHair differentationSyringomaBasal cell carcinomaPiloleimyomaNevocellular
      Squamous cellTumor of the follicularHidradenomaOsteomanevus
      carcinomainfundibulumMixed tumor (chondroid
      Trichilemmomasyringoma)
      Inverted follicular keratosis
      Pilomatricoma
      Proliferating trichilemmal cyst
      Keratoacanthoma
      Sebaceous differentation
      Sebaceous adenoma
      Sebaceous epithelioma
      Sebaceous carcinoma
      • Goldstein G.D
      • Whitaker D.C
      • Argenyi Z.B
      • Bardach J
      Basal cell caricinoma arising in a sebaceous nevus during childhood.
      Apocrine differentation
      Apocrine nevus
      Syringocystadenoma papilliferum
      Apocrine cystadenoma
      Apocrine adenoma
      Apocrine carcinoma
      The incidence of development of secondary tumour reported by several investigators was from 30 to 50%.
      • Jones E.W
      • Heyl T
      Naevus sebaceous: A report of 140 cases with special regard to the development of secondary malignant tumors.
      • Alessi E
      • Wong S.N
      • Advani Ackerman A.B
      Nevus sebaceous is associated with unusual neoplasm. An Atlas.
      In 1962, Michalowski suggested that SN should be considered a premalignant lesion after he noted 34 carcinomas arising out of total of 160 cases of SN (22%).
      • Michalowski R
      Nævus séæübacé de Jadassohn-un état précancéreux.
      124 secondary tumours (31.9%) including 61 carcinomas (15.7%) in 389 cases of SN were the result of three large pooled studies.
      • Mardem P.M
      • Venters Jr, H.D
      A new neurocutaneous syndrome.
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      • Jones E.W
      • Heyl T
      Naevus sebaceous: A report of 140 cases with special regard to the development of secondary malignant tumors.
      • Bonvalet D
      • Barrandon Y
      • Foix C
      • Civatte J
      Tumeurs annexielles bénignes de survenue tardive sur nævus verruco-sébacé (Jadassohn).
      Most of the associated malignancies are BCC, but a few cases of squamous cell carcinoma, apocrine gland carcinoma
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      and adnexal carcinoma,
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      and one case of sebaceous caricinoma
      • Burden P.A
      • Gentry R.H
      • Fitzpatrick J.E
      Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
      have been reported. We would like to include a case of malignant melanoma in this category.
      However, the malignant change of melanocytes in SN could conceivably occur by one of two different mechanisms: it is possible that this change occurs spontaneously in melanocytes in SN; alternatively the change may occur under the induction of SN. The mechanism by which this abnormal induction occurs is not known. Ackerman suggests that most malignant melanoma in situ develop de novo, but many surely develop in preexisting melanocytic nevi.
      • Ackerman A.B
      The concept of malignant melanoma in situ.
      Considering his suggestion, we speculate that malignant change of melanocytes occurs within melanocytes of SN without induction of SN.
      On the basis of our own experience and reviews of the literature, it must be stressed that local excision should be carried out early in the patient's life, preferable before puberty, while the lesions are small in size and the likelihood of malignant degeneration is small.
      • Goldstein G.D
      • Whitaker D.C
      • Argenyi Z.B
      • Bardach J
      Basal cell caricinoma arising in a sebaceous nevus during childhood.
      Moreover, early removal of the lesion reduces the likelihood of cosmetic problems for the patient.

      References

        • Jadassohn J
        Bemerkungen zur Histologie der systematisierten Naevi und über ‘Talgdrüsen-Naevi’.
        Arch Dermatol Syphilol. 1895; 33: 355-394
        • Mehregan A.H
        • Pinkus H
        Life history of organoid nevi. Special reference to nevus sebaceous of Jadassohn.
        Arch Dermatol. 1965; 91: 574-588
        • Mardem P.M
        • Venters Jr, H.D
        A new neurocutaneous syndrome.
        Am J Dis Child. 1966; 112: 79-81
        • Albrecht E
        Ueber Hamartome.
        Ver Deutsch Ges Path. 1904; 7: 153-157
        • Burden P.A
        • Gentry R.H
        • Fitzpatrick J.E
        Piloleiomyoma arising in an organoid nevus: a case report and review of the literature.
        J Dermatol Surg Oncol. 1987; 13: 1213-1218
        • Jones E.W
        • Heyl T
        Naevus sebaceous: A report of 140 cases with special regard to the development of secondary malignant tumors.
        Br J Dermatol. 1970; 82: 99-117
        • Alessi E
        • Wong S.N
        • Advani Ackerman A.B
        Nevus sebaceous is associated with unusual neoplasm. An Atlas.
        Am J Dermatopathol. 1988; 10: 116-127
        • Michalowski R
        Nævus séæübacé de Jadassohn-un état précancéreux.
        Dermatologica. 1962; 124: 326-340
        • Bonvalet D
        • Barrandon Y
        • Foix C
        • Civatte J
        Tumeurs annexielles bénignes de survenue tardive sur nævus verruco-sébacé (Jadassohn).
        Ann Dermatol Vénéréol. 1983; 110: 337-342
        • Ackerman A.B
        The concept of malignant melanoma in situ.
        Pigment cell. 1987; 8: 205-210
        • Goldstein G.D
        • Whitaker D.C
        • Argenyi Z.B
        • Bardach J
        Basal cell caricinoma arising in a sebaceous nevus during childhood.
        J Am Acad Dermatol. 1988; 18: 429-430

      Biography

      The Authors
      Seishu Abe MD, Consultant Plastic Surgeon
      Yoshiteru Yamamoto, Registrar in Plastic Surgery
      Sin-ichi Uno, Registrar in Plastic Surgery
      Masakatu Andou, Consultant in Clinical Pathology
      Department of Plastic and Reconstructive Surgery, Asahikawa Red-Cross Hospital, 1-1 Akebono, Asahikawa 070-8530, Japan
      Toshihide Akasaka, Professor in Dermatology
      Iwate Medical University, Morioka, Japan
      Martin C. Mihm Jr., Professor in Dermatology
      Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA