Primary intracranial meningeal melanomas are rare. Diagnosing primary meningeal melanomas mostly involves comprehensive assessment through clinical and radiological means. This evaluation should encompass a detailed dermal and ophthalmic examination. Any suspicious lesion must be biopsied and examined microscopically. This is crucial not only to differentiate primary intracranial melanoma from other brain tumors but also to rule out metastases from potential sources of primary cutaneous or non-cutaneous melanomas. Surgery is considered the mainstay of treatment. Despite melanomas being generally considered radio- and chemo-resistant tumors, adjuvant radiotherapy and chemotherapy still play a crucial role in their management. The treatment landscape for primary meningeal melanoma is continually evolving, with ongoing research aiming to improve outcomes for patients with this challenging disease.

BACKGROUND: Pigmented fungiform papillae of the tongue (PFPT) is a rare benign pigmentary disorder of the tongue. In dark-skinned individuals, PFPT appears to be relatively common. However, limited data exist on PFPT in Korean patients.
OBJECTIVE: We aimed to investigate the clinical characteristics of PFPT in Korean patients.
METHODS: Patients diagnosed with PFPT between 1995 and 2021 at the Pusan National University Hospital were included. Clinical characteristics of PFPT, dermoscopic findings, and comorbidities were reviewed.
RESULTS: A total of 19 patients diagnosed with PFPT were enrolled. The male to female ratio was approximately 1:5. The mean age at diagnosis was 41.1 years (range, 8~67 years). According to Holzwanger\'s classification, Type I was the most common (89.5%). PFPT was commonly concomitant with pigmentary disorders, including mucosal melanotic macules, Laugier-Hunziker syndrome, melasma, and melanonychia (6/19, 31.6%). Preceding oral infection or inflammatory lesions were found in four patients (21.1%), and systemic diseases and infectious diseases existed in two patients (10.5%). Dermoscopic examination was performed in seven patients; pigmented border with dichotomized vessels (rose petal pattern, 71.4%) and diffuse pigmentation (cobblestone pattern, 71.4%) were common findings.
CONCLUSIONS: Our study shows PFPT can coexist with pigmentary disorders. Concomitant pigmentary disorder shows an association with sex hormone or susceptibility to abnormal pigmentation may be a possible cause of PFPT.

Ependymomas have rarely been described to contain pigment other than melanin, neuromelanin, lipofuscin or a combination. In this case report, we present a pigmented ependymoma in the fourth ventricle of an adult patient and review 16 additional cases of pigmented ependymoma from the literature. A 46-year-old female showed up with hearing loss, headaches, and nausea. Magnetic resonance imaging revealed a 2.5 cm contrast-enhancing cystic mass in the fourth ventricle, which was resected. Intraoperatively, the tumor appeared grey-brown, cystic, and was adherent to the brainstem. Routine histology revealed a tumor with true rosettes, perivascular pseudorosettes and ependymal canals consistent with ependymoma, but also showed chronic inflammation and abundant distended pigmented tumor cells that mimicked macrophages in frozen and permanent sections. The pigmented cells were positive for GFAP and negative for CD163 consonant with glial tumor cells. The pigment was negative for Fontana-Masson, positive for Periodic-acid Schiff and autofluorescent, which coincide with characteristics of lipofuscin. Proliferation indices were low and H3K27me3 showed partial loss. H3K27me 3 is an epigenetic modification to the DNA packaging protein Histone H3 that indicates the tri-methylation of lysine 27 on histone H3 protein. This methylation classification was compatible with a posterior fossa group B ependymoma (EPN_PFB). The patient was clinically well without recurrence at three-month post-operative follow-up appointment. Our analysis of all 17 cases, including the one presented, shows that pigmented ependymomas are most common in the middle-aged with a median age of 42 years and most have a favorable outcome. However, one patient that also developed secondary leptomeningeal melanin accumulations died. Most (58.8%) arise in the 4th ventricle, while spinal cord (17.6%) and supratentorial locations (17.6%) were less common. The age of presentation and generally good prognosis raise the question of whether most other posterior fossa pigmented ependymomas may also fall into the EPN_PFB group, but additional study is required to address that question.

First described by Miller in 1932, melanocytic schwannoma (MS) (melanotic schwannoma, pigmented schwannoma) is a rare variation of peripheral nerve sheet tumours with ectodermal origin occurring predominantly in somatic, but also in the autonomic peripheral system with around two hundred cases in the literature. Predominantly benign tumours, MS are still imaging and pathological challenge and can be easily misdiagnosed with more aggressive peripheral nerve tumours.We report a case of melanocytic schwannoma on L3 sensory rootlet with systematic literature review of nearly 200 cases presented in intracranial, paraspinal region, thoracic, abdominal or pelvic cavities and skin. Two-thirds of cases are part of Carney complex.We present a case of a 61-year-old male with a 3-month history of low back pain, progressive numbness and stiffness in the right thigh, shin and knee, tibial and peroneal paresis causing gait disturbance and neurological claudication. MRI findings present \"sand clock\" type intradural extramedullary tumour formation with extension to the L3 rootlet through right L3-L4 foramen, hypointense on T2 and hyperintense on T1. Pathological diagnosis of sporadic type melanocytic schwannoma was made via immunohistological and ultrastructural analysis. Thirteen months after total resection there was clinical and MRI evidence of recurrence of the tumour. Total resection and radiosurgery was performed with a recurrence free period of 14 months.A gold standard for melanocytic schwannoma treatment is gross total surgical resection. Despite being considered benign tumours, MS have a local or metastatic recurrence of around 13%. MRI imaging in most of the cases is insufficient and only exhaustive pathological and immunohistological examination is the key to diagnosis. Need of postoperative radiation therapy is still controversial. For the first time, a criterion for postoperative adjuvant therapy was established.

Pigmented actinic keratosis is an uncommon variant of actinic keratosis that can mimic melanocytic lesions. A 54-year-old man who presented with a dark lesion on his nasal tip is described; biopsy of the lesion revealed a pigmented actinic keratosis that was treated with cryotherapy using liquid nitrogen. Pigmented actinic keratoses typically appear on sun-exposed areas of the skin as flat hyperpigmented lesions that grow in a centrifugal pattern. Dermoscopy reveals one or more pseudonetworks with hyperpigmented dots or globules. Histopathology shows atypical keratinocytes in the epidermal basal layer and increased melanin content in the epidermis and dermis. Treatment options include liquid nitrogen cryotherapy for solitary lesions and curettage, 5-fluorouracil, imiquimod, ingenol mebutate, photodynamic therapy, or superficial peels for extensive lesions.

BACKGROUND: Porokeratosis is a benign hyperkeratotic skin tumour due to a clonal proliferation of keratinocytes and is characterised by a telltale annular threadlike configuration along the border of a skin-colored to erythematous papule that can expand centrifugally.
METHODS: We are presenting a clinical and dermoscopic case of pigmented disseminated superficial actinic porokeratosis (DSAP) limited to the upper trunk of a white man with sun-damaged skin. Literature Review and Conclusion: A thorough review of PubMed failed to identify any previous reports on the dermoscopic appearance of pigmented porokeratosis. On dermoscopy, the presence of black dots limited to the periphery of the lesions is due to pigment incontinence and melanophages within the superficial papillary dermis limited to the area below the cornoid lamella. Pigmented DSAP is a unique morphological presentation of porokeratosis, and it is essential to be familiar with its clinical and dermoscopic presentation.

BACKGROUND: Choroid plexus papillomas (CPPs) are rare benign tumors, and the pigmented subtype is observed even more rarely.
METHODS: We present the case of a 43-year-old woman with complaints of headache and progressive left monocular visual deterioration, whose initial plain computed tomography CT scan showed an ovate high-density tumor located within the insellar region. Magnetic resonance imaging revealed a homogeneously contrast-enhancing tumor extending from the sella turcica to the suprasellar cistern. Single-nostril transsphenoidal endoscopic resection followed by subfrontal subtotal resection was performed in this patient. Postoperative histology revealed that the tumor consisted of hyperchromatic tissue with papillary features. Higher-resolution examination of the tissue revealed this tissue was composed of hyperplastic columnar epithelial cells with hyperchromatic cytoplasmic pigment. Subsequent immunohistochemistry identified the lesion as a pigmented choroid plexus papilloma. Here we review the current literature, discuss the origin of the tumor, the differential diagnosis, and the roles of surgery and radiotherapy.
CONCLUSIONS: This case study provides important clinical information for the evaluation, diagnosis, and treatment of pigmented CPP in the sellar region.

We present a unique case of separate and independent adjacent fibroepitheliomas of Pinkus (FeP) arising from the umbilicus in an 83-year-old man. Of further interest, one is pigmented and the other nonpigmented. Clinical, dermatoscopic and histopathological images are provided. A review of the published literature is undertaken to attempt to assess the incidence of pigmented versus nonpigmented FeP. Of 24 published FeP cases, 10 (41.7%) have been pigmented. Thus to date pigmented FeP comprise approaching one-half of all reported cases.

Pigmented extramammary Paget\'s disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget\'s disease affecting skin that is rich in apocrine sweat glands such as the axilla, perianal region and vulva. It most commonly occurs in postmenopausal women and presents as a superficial pigmented scaly macule, mimicking a melanocytic lesion. The histological presentation is adenocarcinoma in situ with an increased number of melanocytes scattered between the Paget\'s cells. Therefore, PEMPD may be misdiagnosed as a melanocytic tumour both clinically and histologically. The tumour cells are usually positive for cytokeratin 7, epithelial membrane antigen, Cam 5.2, HER2, and mucicarmine stain while S100 and human melanoma black-45 highlight the processes of reactive dendritic cells. The association between Paget\'s cells and intratumoural reactive melanocytes is still unclear. We report our first case of PEMPD associated with an intradermal naevus involving the axilla in a 63-year-old woman.

Keratocystic odontogenic tumor (KCOT) is a relatively rare benign neoplasm of odontogenic origin. The squamous epithelium of KCOT usually does not contain melanocytes, however, pigmented KCOT has been documented, albeit extremely rarely. In the present study, we described an additional case of pigmented KCOT and review the clinicopathological features of this extremely rare lesion. A 23-year-old Japanese female presented with a relatively well-circumscribed round unilocular radiolucency that impacted the third molar in her right mandibula. Surgical resection was performed subsequent to a clinical diagnosis of KCOT. Histopathological study of the resected mandibular cyst showed that it was covered by a parakeratinized stratified squamous epithelium, which had slightly enlarged hyperchromatic nuclei. On the luminal surface, a wavy layer of parakeratin was observed. In addition, dendritic melanocytes without atypia were observed in approximately half of the squamous epithelium. Immunohistochemical analyses revealed that these melanocytes were positive for S-100 protein, Melan-A and HMB-45. Therefore, a diagnosis of pigmented KCOT was made. Review of the clinicopathological features of the previously reported cases of pigmented KCOT as well as the present case revealed that: i) this lesion occurs mostly in young persons (average age, 18 years) and shows female predominance; ii) most cases are solitary and involve the mandibula; and iii) the reported incidence is 0.36-10.6% and this difference may be associated with ethnicity. Thus, we described the ninth reported case of pigmented KCOT. The mechanism by which melanocytes appear and the difference in ethnic prevalence remain unclear. Additional clinicopathological studies are needed to clarify these issues.