Case Reports

Melanocytic Matrical Carcinoma in a Solid-Organ Transplant Recipient

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Practice Points

  • Melanocytic matrical carcinoma (MMC) is an extremely rare adnexal malignancy that can present as a hyperpigmented papule with or without ulceration.
  • Histologically, the lesion resembles a matrical carcinoma with admixed, banal-appearing dendritic melanocytes.
  • Solid-organ transplant recipients are at an increased risk of cutaneous malignancies, including rare cancers such as MMC, and these neoplasms should remain in the clinician’s differential diagnosis.


 

References

To the Editor:

A 68-year-old white man presented with a firm, gradually enlarging, mildly tender, grayish black papule with central ulceration on the left dorsal wrist of 4 months’ duration (Figure 1). His relevant medical history included multiple basal cell carcinomas (BCCs) and squamous cell carcinomas, as well as a single-lung transplant 2 years prior, for which he was on chronic immunosuppressive therapy with azathioprine, everolimus, tacrolimus, and prednisone. The clinical differential diagnosis included pigmented BCC, malignant melanoma, and ulcerated squamous cell carcinoma.

Figure 1. Clinical appearance of the melanocytic matrical carcinoma, a grayish black papule on the distal dorsal wrist with central ulceration.

Histologic examination of the lesion (Figure 2) demonstrated irregular nodules of basaloid tumor cells with rounded nuclei, visible nucleoli, and scant cytoplasm involving the dermis. The tumor produced abrupt matrical-type keratinization, forming ghost cells. The lesion also contained frequent mitotic figures, apoptotic cells, focal areas of necrosis, and abundant melanin pigment. Admixed throughout the lesion were pigmented and dendritic melanocytic cells. The overlying epidermis was focally ulcerated with an adjacent localized connection between the tumor and the epidermis. Keratinocyte atypia was found in the surrounding epidermis, which contained melanophages, solar elastosis, and scattered chronic inflammatory cells. An immunohistochemical study (Figure 3) for tyrosinase demonstrated abundant admixed melanocytic cells. β-Catenin expression was shown in both nuclear and cytoplasmic distributions, and there was focal labeling on BerEP4 staining. Based on these findings, a diagnosis of melanocytic matrical carcinoma (MMC) was made.

Figure 2. A, Histologic section of a shave biopsy demonstrated an infiltrative basaloid neoplasm with focal epidermal connections (H&E, original magnification ×2). B, Focal necrosis was found within 1 of the small nests (H&E, original magnification ×200). C, Basaloid tumor cells elaborating matrical-type keratin with abundant melanin pigment and dendritic melanocytes (H&E, original magnification ×400).


Figure 3. A, Immunohistochemical staining revealed abundant admixed melanocytic cells populating the lesion (tyrosinase, original magnification ×100). B, There was nuclear and cytoplasmic expression of β-catenin (original magnification ×100).


The lesion was subsequently treated with wide local excision. The patient has not had recurrence to date.

Melanocytic matricoma (MM), a rare adnexal tumor, was first described in 1999 by Carlson et al.1 A PubMed search of articles indexed for MEDLINE using the terms melanocytic and matricoma yielded 24 reported cases in the English-language literature.1-17 It consists of an admixed population of basaloid matrical and supramatrical cells, ghost cells, and dendritic melanocytes in a well-circumscribed dermal nodule, typically without epidermal or adnexal connection. In comparison to the more commonly described pilomatricoma, which can be uncommonly pigmented, MM typically has only focal areas of ghost cells and lacks cystic architecture.1,9,10,18 A granulomatous reaction to keratinaceous debris is variably present.1,9,10 Histologically, the scattered dendritic melanocytes are classically benign, but cases demonstrating melanocyte atypia have been reported.10,13 Melanocytic matricoma appears most commonly as a black or gray papule on sun-damaged skin in older men and tends not to recur following complete excision; thus, MM is considered to be a clinically benign neoplasm. Given the demographics and distribution of the lesions, exposure to UV radiation is thought to play a contributory role in the pathogenesis.2,10,19 Melanocytic matricoma is believed to recapitulate the hair follicle in the anagen phase, where there is close interplay between matrical keratinocytes and melanocytes prior to cessation of melanogenesis during the catagen phase.5,6,8,20,21 Evidence demonstrating highly conserved β-catenin and downstream lymphoid enhancer binding factor 1 (LEF1) expression, as well as pleckstrin homology-like domain, family A, member 1 (PHLDA1) expression (as a marker for follicular stem cells), points to constitutive activity in the Wnt signaling pathway in follicular stem cells of the bulge area as a major agent of tumorigenesis.12

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