Some sources equate the term mole with melanocytic nevus, but there are also sources that equate the term mole with any nevus form. Our dermatology online brief report hhistopathological. Atypia and differential diagnosis in cellular blue nevi. Agminated heterogeneous papules on the neck mdedge. A case of epithelioid blue nevus article pdf available in the korean journal of pathology 486. Differentiation of some cellular blue nevi from melanoma can be. Epithelioid blue nevi have been reported in association with cardiac myxoma or in isolation. Dec 31, 2014 although epithelioid blue nevus ebn is a rare variant of blue nevus that was first reported in patients with carney complex, which encompasses cardiac and cutaneous myxomas, spotty skin pigmentation, endocrine overactivity, and psammomatous melanotic schwannomas, it has been also documented in the absence of such clinical features. Blue nevus versus pigmented epithelioid melanocytoma. In our case, the tumor was highly cellular, heavily pigmented and.
A case of epithelioid blue nevus pubmed central pmc. The main tumor cells of common blue nevus are elongated, dendritic melanocytes with. Epithelioid blue nevus is a rare variant of blue nevus that have been recently described mostly in patients with the carney complex 5. Melanocytes are arranged almost in solitary units scattered in the lower portion of the epidermis and absent in the outermost. May be part of carney complex, which includes cardiac myxoma, psammomatous melanotic schwannoma, multicentric blue nevi and. Other possible differential diagnoses include malignant blue nevus, cellular blue nevus and heavily pigmented melanoma. In blue nevus pigmentproducing cells are spindled and fusiform dendritic cells.
Clinically, they were darkly pigmented, domed, and small less than 1 cm and commonly interpreted as blue nevi. We report a case of pem in a previously well 26yearold caucasian woman who presented with a dark. Melanoma is the leading cause of death among cutaneous neoplasms. Nevus of ota associated with a primary uveal melanoma and. Histopathology images of epithelioid hemangioendothelioma. Epithelioid cells are admixed with dendritic cells and resemble clear cells of cellular blue nevus hematoxylineosin, original magnifications 320 a, c, and e and 3400 b, d, and f. The nevus cells are uniformly epithelioid throughout the lesion and there is no significant mitotic activity. Histologically, all lesions showed a small, relatively, intraepidermal proliferation of melanocytes that are often drop shaped, with a pale nucleus and an abundant, clear cytoplasm, with an epithelioid or pseudopagetoid appearance figs. Although uncommon, association with malignant melanoma is a complication that must be considered in the evaluation of patients with nevus of ota.
These lesions may cause diagnostic difficulty in case of large size, involvement of subcutaneous tissue, asymmetrical pigmentation and presence of lymphnode metastasis. Although epithelioid blue nevus ebn is a rare variant of blue nevus that was first reported in patients with carney complex, which. Melanocytes are arranged almost in solitary units scattered in the lower portion of the epidermis. Pdf blue nevi dermal melanocytomas and their variants are characterized by. Individual or groups of cells may be separated by a collagenous stroma but zonation, as seen in the cellular blue nevus, is not seen.
Pigmented epithelioid melanocytoma pem is a recently proposed term which encompasses those melanocytic tumours previously designated as animaltype melanoma or pigmentsynthesising melanoma and epithelioid blue nevus, the latter known to be associated with carneys complex. The differential diagnosis for pem includes melanocytic lesions characterized by both hypercellularity and prominent melanin synthesis, specifically cellular blue nevus, malignant blue nevus, deep penetrating nevus, plexiform spindle nevus, pigmented spindle and epithelioid cell spitz nevus, malignant melanoma arising in nevi of ito and ota. May be part of carney complex, which includes cardiac myxoma, psammomatous melanotic schwannoma, multicentric blue nevi and endocrine overactivity orphanet j rare dis 2006. Epithelioid blue nevusepithelioid and a few dendritic melanocytes with.
Melanoma is the fourth most common cancer in new zealand and incidence is increasing. Updates on molecular diagnostic assays in melanocytic. A biopsy was performed at the site 2 years prior for a single blue nevus, but the. According to the original description by tieche 33, to which little can be added, blue nevus is a dermalbased, benign melanocytic lesion histopathologically made up by variable proportions of ovalspindle and bipolar, usually heavily pigmented dendritic cells g. Spitz nevus is an infrequent, usually acquired melanocytic nevus composed of epithelioid andor spindle melanocytes that can occasionally be confused with melanoma.
A type of melanocytic nevus, it affects the epidermis and dermis it is also known as an epithelioid and spindlecell nevus, and misleadingly as a benign juvenile melanoma. The deep penetrating nevus has microscopic features that overlap ordinary blue nevus, cellular blue nevus, and spindle and epithelioid cell spitz nevus 57. Blue nevus also known as blue neuronevus, dermal melanocytoma, nevus coeruleus and nevus bleu is a type of melanocytic nevus. The melanocytic epithelioid cell nevus of the thigh of. This chapter highlights the features that help to distinguish these histologically similar lesions. Departments of dermatology and pathology, university of alabama at. This is especially true for a variant of spitz naevus characterized by heavy pigmentation and large spindle cells confined to the epidermis and papillary dermis, the so.
He denied any personal or family history of skin cancer, cardiac abnormalities, or endocrine dysfunction. Multicomponent pattern, asymmetry and multiple colors. Previously known as animaltype melanoma, pigmented epithelioid melanocytoma pem was originally described as the sporadic counterpart to the epithelioid blue nevus in carneys complex. Care should be exercised to exclude nonmelanocytic lesions, and benign melanocytic entities, both of which can mimic melanoma histologically. A relatively well defined, round and bluish nodule is developed on the dorsum of right foot a, in set in a.
Significant dermal fibrosis is associated with a desmoplastic blue nevus. Epithelioid blue nevus epithelioid and a few dendritic melanocytes with. Congenital giant common blue nevus of the scalp a case report. Ebn is another variant of blue nevus that arises in the background of carney complex or may occur sporadically. The name juvenile melanoma is generally no longer used as it is neither a melanoma, nor does it occur only in children. Epitheliod hemangioendothelioma of liver is a rare lowgrade malignancy that can also occurs in other organs as a primary malignancy. In addition, the possibility of the lesion being a melanoma variant or epidermotropic metastasis should be considered. Spindle and epithelioid cell nevus definition of spindle. Epithelioid blue nevus ebn was first reported in association with carneys complex in 1996. Blue whitish veil 78%, grayish blue areas 76%, black dots 73%, irregular extensions and branched streaks 62%, cancer 2001. Instead of slender dendritic melanocytes, the cells of an epithelioid blue naevus are large, round and have a pale cytoplasm figure 4. However, the clinical, histologic, and prognostic features significantly differ between pem and other pigmented dermal melanocytic proliferations. For the great majority of melanocytic tumors histopathologic examination can reliably distinguish nevi from melanomas. Jul 10, 2018 previously known as animaltype melanoma, pigmented epithelioid melanocytoma pem was originally described as the sporadic counterpart to the epithelioid blue nevus in carneys complex.
Epithelioid blue nevus ebn was first described in patients with carney complex cnc and subsequently shown to also occur sporadically. A comparative evaluation of histopathologic and dermoscopic features was carried out on 52 melanocytic proliferations belonging to the morphologic spectrum of blue nevus. In this variant, the neoplastic cells show no maturation at the base of the lesion and, in contrast with the usual. An adolescent with a smooth, blue black nodule on the dorsal. New zealand has the highest rate of melanoma worldwide and risk is greatest for nonmaori men aged over 50 years. Although epithelioid blue nevus ebn is a rare variant of blue nevus that was first reported in patients with carney complex, which encompasses cardiac and cutaneous myxomas, spotty skin pigmentation, endocrine overactivity, and psammomatous melanotic schwannomas, it has been also documented in the absence of such clinical features. For such tumors, additional ancillary tests would be beneficial in adjudicating a more. The clinical lesion is usually an irregularly shaped, asymmetrical lesion with varying colors with a history of recent change in size, shape, colour or sensation.
The histopathological differential diagnoses of pem include cellular blue nevus, malignant blue nevus, spitz nevus, deep penetrating nevus and epithelioid blue. Epithelioid blue nevus is a distinctive variant that presents as a solitary, heavily pigmented, blueblack, domeshaped nodule. A 19yearold man presented to the dermatology clinic for evaluation of several new dark papules on the neck of 1 years duration. Histopathologic diagnosis may be difficult when the common blue nevus component and melanin production are sparse or inapparent. An update on cutaneous melanocytic lesions diagnostic. Epithelioid cell melanocy nevus also known as nevocytic nevus, nevus cell nevus and commonly as a mole is a type of melanocytic tumor that contains nevus cells. Benign melanocytic tumors malignant melanoma superficial spreading melanoma nodular melanoma lentigo maligna acral lentiginous melanoma desmoplastic melanoma melanoma arising from blue nevus melanoma arising in giant congenital nevus melanoma of childhood nevoid melanoma persistent melanoma. A case of epithelioid blue nevus europe pmc article. The cellular morphology may be difficult to appreciate without bleaching the section to expunge the copious melanin pigment. Epithelioid blue nevus is a distinctive variant that presents as a solitary, heavily pigmented, blue black, domeshaped nodule or as multiple nodules. Epithelioid hemangioendothelioma epithelioid hemangioendothelioma, liver. B excised tumor is heavily pigmented and relatively well demarcated in dermis with expansion to the subcutis. Oct 31, 2006 cellular blue nevus, with schwannian differentiation masson neuronevus a cellular blue nevus with neuroid fascicles of spindle cells with a wavy silhouette hypochromic blue nevus, myxoid cystic a commoncellular blue nevus with either intercellular mucin deposition or a central empty mucinous. Epithelioid blue nevus is composed of melaninladen polygonal epithelioid melanocytes.
Pigmented epithelioid melanocytoma pem is a recently described entity encompassing epithelioid blue nevus of carney complex and most tumors earlier considered as socalled animaltype melanoma. The blue nevus is a melanocytic nevus variant in which the lesional cells are found deep within the connective tissue. The histopathological differential diagnoses of pem include cellular blue nevus, malignant blue nevus, spitz nevus, deep penetrating n epithelioid blue nevus. All blue nevus cases diagnosed between 2000 and 2014 at our pathology. Nevus of ota is a blue, hyperpigmented, benign dermatosis of the skin and mucosae that most often occurs unilaterally in the distribution of the ophthalmic v1 and maxillary v2 branches of the trigeminal nerve. Histopathology of malignant melanoma linkedin slideshare.
Blue nevus bn is a common specimen in routine pathology practice. Pigmented epithelioid melanocytoma is a recently introduced entity comprising a subset of lesions previously included in the spectrum of socalled animaltype melanoma. Histopathology a compound nevus possesses features of both a junctional and an intradermal nevus. Dendritic cells cannot be appreciated without melanin pigmentation. Pdf blue nevus versus pigmented epithelioid melanocytoma. Epithelioid cell melanocy epithelioid melanocytoma pem is a recently described entity encompassing epithelioid blue nevus of carney complex and most tumors earlier considered as socalled animaltype melanoma. Over 50% of patients with cnc harbor mutations in the gene prkar1a, which codes for protein kinase a regulatory subunit 1. Its characteristic blue hue is attributed to the tyndall effect the scattering of light as it passes through a colloidal suspension. Mar 10, 2015 prof histopathology of malignant melanoma by dr ajeta 2. In recent years, several histopathologic variants of blue nevus have been identified, whose clinical and dermoscopic correlates need further clarification. Congenital giant common blue nevus of the scalp a case. Although epithelioid blue nevus ebn is a rare variant of blue nevus that was first reported in patients with carney complex, which encompasses cardiac and cutaneous myxomas, spotty skin pigmentation, endocrine overactivity, and psammomatous melanotic schwannomas,1 it has been also documented in. Epithelioid and fusiform blue nevus of chronically sun.
He also denied any recent changes in health or use of medication. We report a case of pem in a previously well 26yearold caucasian. Blue naevus was first described by tiesche in 1906. Blue nevus is a tumor of dermal dendritic melanocytic origin, and common blue nevus and cellular blue nevus are the most common variants. A new concept encompassing animaltype melanoma and epithelioid blue nevus article pdf available in bmj case reports 20mar22 1 march 20 with 679 reads. Blue nevus it is a small slate blue to blue black macule or papule found in the extremities, acquired after infancy. Messina, md september 16, 2017 melanoma versus nevus. The differentiating feature between pem and cellular blue nevus is the presence of abundant.
A number of pigmented lesions are difficult to classify and raise the possibility of a melanoma diagnosis. An adolescent with a smooth, blueblack nodule on the dorsal wrist. The blue nevus in benign pigmented lesion that presents as a dark blue domeshaped papule or as a flat macule on the skin or mucosa. Similar to cellular blue nevus, the lesspigmented spindle and epithelioid cells toward the base of the lesion may concentrate around blood vessels and nerves producing a plexiform pattern of growth. Epithelioid blue naevus is also called pigmented epithelioid melanocytoma. Microscopically, they featured heavily pigmented, poorly circumscribed, dermal lesions that displayed two types of melanocytes. Currently, there are no immunohistochemical markers or molecular biology techniques that can be used to make an entirely safe diagnosis of spitz nevus or melanoma in problematic cases. A case of epithelioid blue nevus linkedin slideshare. Pigmented epithelioid melanocytoma and epithelioid blue nevus. The blue colour is caused by the pigment being deeper in the skin than in ordinary nevi.
Multiple epithelioid spitz nevi or tumors with loss of bap1 expression. The main tumor cells of common blue nevus are elongated, dendritic melanocytes with reactive melanophages and only a few. An adolescent with a smooth, blue black nodule on the. Jan 29, 2020 melanoma is the leading cause of death among cutaneous neoplasms. A biopsy was performed at the site 2 years prior for a single blue nevus, but the patient.
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