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Physical examination revealed a hard&#44; polypoid lesion of 0&#46;3<span class="elsevierStyleHsp" style=""></span>mm diameter&#44; with a translucent surface&#46; With a possible diagnosis of fibrous papule&#44; milium cyst&#44; or adnexal tumor &#40;trichodiscoma&#41;&#44; the lesion was excised&#46; Histopathology revealed a circumscribed proliferation of cells in the superficial and mid dermis&#44; surrounded by an epithelial collarette &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The cells&#44; arranged in an interlinked fascicular pattern&#44; had a poligonal morphology with abundant&#44; granular eosinophilic cytoplasm and large vesicular nuclei &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Mitotic figures were present&#46; No ulceration or necrosis was observed&#46; Immunohistochemistry was positive for CD68 and negative for AE1-AE3&#44; S-100&#44; Melan A&#44; CD34&#44; desmin&#44; actin&#44; and smooth muscle&#46; On the basis of these findings we made a diagnosis of primitive polypoid granular cell tumor&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Granular cells can be found in a varied group of tumors and reflect the intracytoplasmic accumulation of lysosomes and other components of the Golgi aparatus&#46; Traditional and conventional nomenclature makes reference to the cutaneous and mucosal granular cell tumor&#44; known as Abrikossoff tumor&#44; a benign neoplasm of neural origin derived from Schwann cells&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> However&#44; other granular cell tumors of non-neural origin exist&#44; including the congenital gingival granular cell tumor and the primitive polypoid granular cell tumor&#46; In addition&#44; numerous tumors can present granular cells&#44; including myogenic tumors&#44; melanocytic lesions&#44; dermatofibroma&#44; dermatofibrosarcoma protuberans&#44; basal cell carcinoma&#44; atypical fibroxanthoma&#44; angiosarcoma&#44; fibrous papule&#44; ameloblastoma&#44; and adnexal tumors with eccrine or apocrine differentiation&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Primitive polypoid granular cell tumor is a rare neoplasm or uncertain origin that affects middle-aged adults&#46; It usually arises on the trunk or limbs as a polypoid or elevated lesion with a smooth surface&#59; its size can be variable&#46; The distinctive characteristic that differentiates it from conventional granular cell tumor is its histopathology&#44; which shows a tumor in the mid dermis that is clearly surrounded by an epidermal collarette&#59; the tumor is formed of large polygonal&#44; round or spindle-shaped cells with marked nuclear pleomorphism&#44; an elongated nucleus&#44; abundant eosinophilic cytoplasm containing fine granules&#44; and mitotic activity of around 1 to 3 mitoses per mm&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> usually with no atypia and minimal or absent epidermal hyperplasia&#46; These histopathology findings satisfy the criteria proposed by Enzinger and Weiss for the classification of malignant or atypical granular cell tumor&#44; but the tumors can be differentiated by immunohistochemistry&#44; as primitive polypoid granular cell tumor is usually negative for S-100 and positive for CD68 and neuron-specific enolase&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Despite the histological characteristics&#44; this is a tumor of low&#8211;grade malignancy&#46; In the series reported there is only 1 case of metastasis&#44; which arose in the cheek 25 months after excision of the lesion and did not present an epidermal collarette on histology&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">We have presented a new case of primitive polypoid granular cell tumor&#44; a variant that is not clearly distinguished from the conventional tumor&#46; Its atypical histological characteristics allow it to be classified as a new entity and distinguished from granular cell tumor of neural origin&#46;</p></span>"
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Case and Research Letters
Primitive Polypoid Granular-Cell Tumor
Tumor primitivo polipoide de células granulares
M.T. López-Villaescusaa,
Autor para correspondencia
lopezvillaescusa@hotmail.com

Corresponding author.
, M. Rodríguez-Vázqueza, M. García-Arpab, R. García-Angelc
a Servicio de Dermatología, Complejo Hospitalario Universitario de Albacete, Albacete, Spain
b Servicio de Dermatología, Hospital de Ciudad Real, Ciudad Real, Spain
c Servicio de Anatomía Patológica, Complejo Hospitalario Universitario de Albacete, Albacete, Spain
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Cells with a polygonal morphology&#44; abundant granular eosinophilic cytoplasm&#44; and large vesicular nuclei&#46; The cells are arranged in an interlinked fascicular pattern&#46; Hematoxylin-eosin&#44; original magnification &#215;40&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">In contrast to conventional granular cell tumor &#40;Abrikossoff tumor&#41;&#44; primitive polypoid granular cell tumor was first identified by LeBoit et al&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> in 1991 and subsequently endorsed by Chaudhry and Calonje<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> as a dermal tumor of granular cells of non-neural origin&#46; The tumor has a polypoid morphology and presents numerous mitoses&#44; cytologic atypia&#44; and a primitive immunophenotype&#46; We present a new case and review the characteristics of this rare and poorly known tumor&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Our patient was a woman aged 44 years&#44; with no past medical or family history of interest&#46; She consulted for an asymptomatic lesion that had appeared 4 months earlier at the right nasolabial angle&#46; Physical examination revealed a hard&#44; polypoid lesion of 0&#46;3<span class="elsevierStyleHsp" style=""></span>mm diameter&#44; with a translucent surface&#46; With a possible diagnosis of fibrous papule&#44; milium cyst&#44; or adnexal tumor &#40;trichodiscoma&#41;&#44; the lesion was excised&#46; Histopathology revealed a circumscribed proliferation of cells in the superficial and mid dermis&#44; surrounded by an epithelial collarette &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The cells&#44; arranged in an interlinked fascicular pattern&#44; had a poligonal morphology with abundant&#44; granular eosinophilic cytoplasm and large vesicular nuclei &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Mitotic figures were present&#46; No ulceration or necrosis was observed&#46; Immunohistochemistry was positive for CD68 and negative for AE1-AE3&#44; S-100&#44; Melan A&#44; CD34&#44; desmin&#44; actin&#44; and smooth muscle&#46; On the basis of these findings we made a diagnosis of primitive polypoid granular cell tumor&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Granular cells can be found in a varied group of tumors and reflect the intracytoplasmic accumulation of lysosomes and other components of the Golgi aparatus&#46; Traditional and conventional nomenclature makes reference to the cutaneous and mucosal granular cell tumor&#44; known as Abrikossoff tumor&#44; a benign neoplasm of neural origin derived from Schwann cells&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> However&#44; other granular cell tumors of non-neural origin exist&#44; including the congenital gingival granular cell tumor and the primitive polypoid granular cell tumor&#46; In addition&#44; numerous tumors can present granular cells&#44; including myogenic tumors&#44; melanocytic lesions&#44; dermatofibroma&#44; dermatofibrosarcoma protuberans&#44; basal cell carcinoma&#44; atypical fibroxanthoma&#44; angiosarcoma&#44; fibrous papule&#44; ameloblastoma&#44; and adnexal tumors with eccrine or apocrine differentiation&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Primitive polypoid granular cell tumor is a rare neoplasm or uncertain origin that affects middle-aged adults&#46; It usually arises on the trunk or limbs as a polypoid or elevated lesion with a smooth surface&#59; its size can be variable&#46; The distinctive characteristic that differentiates it from conventional granular cell tumor is its histopathology&#44; which shows a tumor in the mid dermis that is clearly surrounded by an epidermal collarette&#59; the tumor is formed of large polygonal&#44; round or spindle-shaped cells with marked nuclear pleomorphism&#44; an elongated nucleus&#44; abundant eosinophilic cytoplasm containing fine granules&#44; and mitotic activity of around 1 to 3 mitoses per mm&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> usually with no atypia and minimal or absent epidermal hyperplasia&#46; These histopathology findings satisfy the criteria proposed by Enzinger and Weiss for the classification of malignant or atypical granular cell tumor&#44; but the tumors can be differentiated by immunohistochemistry&#44; as primitive polypoid granular cell tumor is usually negative for S-100 and positive for CD68 and neuron-specific enolase&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Despite the histological characteristics&#44; this is a tumor of low&#8211;grade malignancy&#46; In the series reported there is only 1 case of metastasis&#44; which arose in the cheek 25 months after excision of the lesion and did not present an epidermal collarette on histology&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">We have presented a new case of primitive polypoid granular cell tumor&#44; a variant that is not clearly distinguished from the conventional tumor&#46; Its atypical histological characteristics allow it to be classified as a new entity and distinguished from granular cell tumor of neural origin&#46;</p></span>"
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2015 Abril 15 8 23
2015 Marzo 20 9 29
2015 Febrero 14 1 15
2015 Enero 9 2 11
2014 Diciembre 8 3 11
2014 Noviembre 3 0 3
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Actas Dermo-Sifiliográficas
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¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?