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. 2023 Jun;17(2):401-408.
doi: 10.1007/s12105-022-01515-9. Epub 2022 Dec 31.

PRAME Staining in Sinonasal Mucosal Melanoma: A Single-Center Experience

W F Julius Scheurleer #  1 , W Weibel Braunius #  2   3 , Bernard M Tijink  2 , Karijn P M Suijkerbuijk  4 , Miranda P Dierselhuis  3 , Ruud W J Meijers  5 , Willeke A M Blokx  5 , Remco de Bree  2 , Gerben E Breimer  5 , Johannes A Rijken  2
Affiliations

PRAME Staining in Sinonasal Mucosal Melanoma: A Single-Center Experience

W F Julius Scheurleer et al. Head Neck Pathol. 2023 Jun.

Abstract

Background: Sinonasal mucosal melanoma (MM) is a rare, aggressive melanoma subtype. Complete surgical excision, with or without adjuvant radiotherapy, remains the cornerstone of treatment and yields adequate locoregional control. Metastatic MM is managed similarly to metastatic cutaneous melanoma but with poorer survival. PReferentially expressed Antigen in MElanoma (PRAME) has been identified as a potential diagnostic marker and therapeutic target in the treatment of cutaneous melanoma.

Methods: Retrospective analysis of the clinical characteristics and immunohistochemical features of all sinonasal MM patients referred to the department of Head and Neck Surgical Oncology, UMC Utrecht Cancer Center, between 2011 and 2021 was performed. Single nucleotide polymorphism (SNP) array and next-generation sequencing (NGS) were performed in selected cases.

Results: A total of 26 patients with an MM were included. The median follow-up duration was 15 months. At the end of follow-up, 13 patients had died due to progression of their disease, and one patient died of intercurrent disease. PRAME immunohistochemistry was performed in 23 out of 26 cases, all displaying PRAME expression. In two cases PRAME expression was present both within the melanoma cells and in melanocytes in adjacent mucosa. SNP array showed ≥ 5 copy number variants (CNV) in all tested cases, with a median of 29.5 CNVs (IQR 23.25-40). The three most common mutations identified by NGS were NRAS (7 cases) and NF1 (2 cases).

Conclusion: We show that expression of PRAME is common in sinonasal MM, making PRAME a useful ancillary diagnostic tool and a potential therapeutic target in sinonasal MM. The demonstrated occurrence of extensive presence of PRAME-positive melanocytes in the surrounding mucosa of sinonasal MM might explain the multifocal nature of melanoma in the (sinonasal) mucosa, and would be an extra argument for a PRAME targeting treatment in preventing local disease recurrence.

Keywords: Field-melanomization; Immunotherapy; Mucosal melanoma; Nasal mucosa; PRAME; Sinonasal cancer.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Endoscopic view of the right nasal cavity. Nasal septum (*), melanoma posterior nasal septum (M), polyp lateral wall with melanoma (P), adjacent pigmented lesion of the lateral wall (←) which proved to be non-invasive, but PRAME positive
Fig. 2
Fig. 2
Immunohistochemical staining of the index patient. A H&E stain of melanoma; B PRAME immunohistochemical stain of melanoma; C H&E stain of separate focus with invasive and in situ melanoma; D corresponding PRAME immunohistochemical stain; E H&E stain of surrounding mucosa with PRAME positive intra-epithelial melanocytes; F corresponding PRAME immunohistochemical stain. H&E hematoxylin and eosin stain; PRAME PReferentially expressed Antigen in Melanoma
See this image and copyright information in PMC

References

    1. Ascierto P, Accorona R, Botti G, et al. Mucosal melanoma of the head and neck. Crit Rev Oncol Hematol. 2017;112:136–152. doi: 10.1016/j.critrevonc.2017.01.019. - DOI - PubMed
    1. Hernandez-Prera JC. Update from the 5th Edition of the World Health Organization classification of head and neck tumors: the neck and lymph nodes, metastasis, and melanocytic tumors. Head Neck Pathol. 2022;16(1):110–122. doi: 10.1007/s12105-022-01433-w. - DOI - PMC - PubMed
    1. Brierley JD, Gospodarowicz MK, Wittekind C. The TNM classification of malignant tumours. 8. Hoboken: Wiley; 2016.
    1. Christopherson K, Malyapa RS, Werning JW, et al. Radiation therapy for mucosal melanoma of the head and neck. Am J Clin Oncol. 2015;38(1):87–89. doi: 10.1097/COC.0b013e31828d73bf. - DOI - PubMed
    1. Owens JM, Roberts DB, Myers JN. The role of postoperative adjuvant radiation therapy in the treatment of mucosal melanomas of the head and neck region. Arch Otolaryngol Head Neck Surg. 2003;129(8):864–868. doi: 10.1001/archotol.129.8.864. - DOI - PubMed