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1</a>a&#41;&#46; After surgery we observed a macroscopically intense pigmentation affecting the deep dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#44; so we sent the sample to the Pathology Department with a suspected diagnosis of malignant melanoma&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Histologically we observed one intensely pigmented lesion&#44; localized in the dermis and subcutaneous tissue&#44; with no intraepidermal component or connection to the epidermis&#44; which was flattened and showed some hyperkeratosis&#46; The lesion itself showed a pattern of expanding growth with various lobes &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>a&#41;&#46; The central area consisted of numerous melanophages &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>a&#41;&#44; while the periphery and deep lesion area were composed of melanocytic cells with an epithelioid or slightly fusocellular nature&#44; moderate cytologic atypia and pleomorphism &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>b&#41;&#46; Those cells were of medium and large size&#44; with slightly eosinophilic cytoplasm and vesicular nuclei&#44; most of them with a central nucleolus or smaller sized nucleoli&#46; We counted up to four mitoses per HPF in the proliferation areas&#44; some of them showing atypia &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>c&#41;&#46; A scarce inflammatory component &#8211; consisting of lymphocytes interspersed with tumor cells &#8211; was observed&#46; We did not observe any foci of necrosis or images of lymphovascular or perineural invasion&#46; An immunohistochemistry analysis using red chromogen revealed that the melanocytic cells were only found in the deep and peripheral part of the lesion&#44; while the rest of the cells corresponded entirely to melanophages &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>b&#41;&#46; With these findings&#44; the diagnosis of malignant blue nevus &#40;MBN&#41; was established&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">We carried out an extension study on the patient&#44; including a complete clinical examination to rule out the presence of another pigmented lesion that could be considered the primary tumor&#44; but it turned out completely normal&#46; We also performed blood tests with WBC&#44; biochemistry and liver panels&#44; and also a PET-CT with no altered results from average normal ranges&#58; no systemic disease was evident&#46; Twelve months after surgery&#44; no signs suggesting recurrence or systemic disease were observed and the patient remains asymptomatic from all points of view&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Malignant blue nevus &#40;MBN&#41; was a term introduced by Allen and Spitz to describe lesions that are similar to blue nevi but can show a malignant behavior&#44; even lethal&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Currently&#44; the term MBN is controversial and some authors like Ackerman<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> do not recommend its use&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">This diagnosis has been used in different clinicopathologic situations&#44; such as melanomas that arise on a blue nevus&#44; usually on the cellular ones&#46; This probably could be our case if we consider the evolution of the lesion&#46; MBN could also refer to new melanomas that contain elements that are reminiscent of a blue nevus&#46; Critics of the term claim the supposed incongruity of referring to a malignant nevus&#44; when the definition of nevus includes benignity&#46; Therefore&#44; a synonym for MBN that has been proposed today is <span class="elsevierStyleItalic">blue-nevus-like melanoma</span>&#46; This seems to be the most appropriate way to refer to these lesions&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">We can find several published series of MBN where the lesions had the same clinical outcomes as <span class="elsevierStyleItalic">regular</span> malignant melanomas&#44; especially if we consider survival and recurrence&#46; But some isolated studies suggest that the clinical course is usually more aggressive in the MBN cases&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The histological criteria for the diagnosis of MBN are not well defined&#44; but almost every published paper agrees that these lesions show cytological atypia&#44; a high mitotic index and the presence of atypical mitosis&#46; They usually present necrosis and an infiltrative growth rate&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#8211;3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Differential diagnosis of these lesions can be complicated&#46; First&#44; distinguishing between nodular and metastatic melanoma is needed&#46; This is mandatory in order to determine if the lesion we are studying is a skin metastasis or a primary tumor&#46; Some imaging tests may be helpful for this task&#44; such as PET-CT&#46; 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Case and Research Letter
Malignant Blue Nevus: A Challenge for Dermatologists and Dermatopathologists
Nevus azul maligno: un desafío para dermatólogos y dermatopatólogos
T. Toledo-Pastranaa,
Autor para correspondencia
ttoledop@gmail.com

Corresponding author.
, I. Rodríguez Pérezb, P. Eguino Gorrochateguic
a Dermatology Department, Donostia University Hospital, San Sebastián, Spain
b Pathology Department, Donostia University Hospital, San Sebastián, Spain
c Dermatology Department, Bidasoa Regional Hospital, Fuenterrabía, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Melanocytic pigmented lesions pose a real challenge for clinicians and pathologists&#46; They are clinically and histologically very similar and it is essential to know the evolution of these lesions to establish a proper diagnosis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">An 87-year-old man came to the Dermatology Department for the evaluation of a tumor in the right shoulder&#46; The lesion had developed over 20 years and had presented changes in the last three months &#40;growth and hyperpigmentation&#41;&#46; He did not have other accompanying symptoms&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">We observed a 1&#46;7-cm indurated&#44; pigmented nodule&#44; which seemed embedded in the skin of the right shoulder with apparently no subcutaneous component&#46; A simple excision adjusted to palpable limits of the lesion was performed &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46; After surgery we observed a macroscopically intense pigmentation affecting the deep dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#44; so we sent the sample to the Pathology Department with a suspected diagnosis of malignant melanoma&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Histologically we observed one intensely pigmented lesion&#44; localized in the dermis and subcutaneous tissue&#44; with no intraepidermal component or connection to the epidermis&#44; which was flattened and showed some hyperkeratosis&#46; The lesion itself showed a pattern of expanding growth with various lobes &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>a&#41;&#46; The central area consisted of numerous melanophages &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>a&#41;&#44; while the periphery and deep lesion area were composed of melanocytic cells with an epithelioid or slightly fusocellular nature&#44; moderate cytologic atypia and pleomorphism &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>b&#41;&#46; Those cells were of medium and large size&#44; with slightly eosinophilic cytoplasm and vesicular nuclei&#44; most of them with a central nucleolus or smaller sized nucleoli&#46; We counted up to four mitoses per HPF in the proliferation areas&#44; some of them showing atypia &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>c&#41;&#46; A scarce inflammatory component &#8211; consisting of lymphocytes interspersed with tumor cells &#8211; was observed&#46; We did not observe any foci of necrosis or images of lymphovascular or perineural invasion&#46; An immunohistochemistry analysis using red chromogen revealed that the melanocytic cells were only found in the deep and peripheral part of the lesion&#44; while the rest of the cells corresponded entirely to melanophages &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>b&#41;&#46; With these findings&#44; the diagnosis of malignant blue nevus &#40;MBN&#41; was established&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">We carried out an extension study on the patient&#44; including a complete clinical examination to rule out the presence of another pigmented lesion that could be considered the primary tumor&#44; but it turned out completely normal&#46; We also performed blood tests with WBC&#44; biochemistry and liver panels&#44; and also a PET-CT with no altered results from average normal ranges&#58; no systemic disease was evident&#46; Twelve months after surgery&#44; no signs suggesting recurrence or systemic disease were observed and the patient remains asymptomatic from all points of view&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Malignant blue nevus &#40;MBN&#41; was a term introduced by Allen and Spitz to describe lesions that are similar to blue nevi but can show a malignant behavior&#44; even lethal&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Currently&#44; the term MBN is controversial and some authors like Ackerman<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> do not recommend its use&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">This diagnosis has been used in different clinicopathologic situations&#44; such as melanomas that arise on a blue nevus&#44; usually on the cellular ones&#46; This probably could be our case if we consider the evolution of the lesion&#46; MBN could also refer to new melanomas that contain elements that are reminiscent of a blue nevus&#46; Critics of the term claim the supposed incongruity of referring to a malignant nevus&#44; when the definition of nevus includes benignity&#46; Therefore&#44; a synonym for MBN that has been proposed today is <span class="elsevierStyleItalic">blue-nevus-like melanoma</span>&#46; This seems to be the most appropriate way to refer to these lesions&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">We can find several published series of MBN where the lesions had the same clinical outcomes as <span class="elsevierStyleItalic">regular</span> malignant melanomas&#44; especially if we consider survival and recurrence&#46; But some isolated studies suggest that the clinical course is usually more aggressive in the MBN cases&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The histological criteria for the diagnosis of MBN are not well defined&#44; but almost every published paper agrees that these lesions show cytological atypia&#44; a high mitotic index and the presence of atypical mitosis&#46; They usually present necrosis and an infiltrative growth rate&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#8211;3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Differential diagnosis of these lesions can be complicated&#46; First&#44; distinguishing between nodular and metastatic melanoma is needed&#46; This is mandatory in order to determine if the lesion we are studying is a skin metastasis or a primary tumor&#46; Some imaging tests may be helpful for this task&#44; such as PET-CT&#46; Another important differential diagnosis to consider is what is called &#8220;animal-type melanoma&#44;&#8221; which has a low mitotic index and a slight melanophagic component&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> The third differential diagnosis must be made with pigmented epithelioid melanocytoma &#40;PEM&#41;&#44; which is very similar to the animal-type melanoma and also to the benign blue nevus&#46; PEM is usually a &#8220;de novo&#8221; lesion&#44; more frequent in young patients&#46; It also shows a low mitotic index and melanophagic component&#46; Finally&#44; we should consider the differential diagnosis with the atypical cellular blue nevus&#44; which is usually a well-demarcated lesion with intermediate atypia and a low mitotic index&#44; but with no necrosis&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interests</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare no conflict of interests&#46;</p></span></span>"
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