Subject(s)
Calcinosis/etiology , Dermatomyositis/complications , Skin Diseases/etiology , Subcutaneous Tissue/pathology , Adolescent , Anti-Bacterial Agents/therapeutic use , Calcinosis/drug therapy , Calcinosis/surgery , Combined Modality Therapy , Cutaneous Fistula/etiology , Dermatomyositis/drug therapy , Diphosphonates/therapeutic use , Drug Resistance , Female , Humans , Immunosuppressive Agents/therapeutic use , Pamidronate , Skin Diseases/drug therapy , Skin Diseases/surgery , Skin Diseases, Bacterial/etiology , Skin Diseases, Bacterial/prevention & controlSubject(s)
Abnormalities, Multiple/pathology , Ectodermal Dysplasia/pathology , Limb Deformities, Congenital/pathology , Scalp Dermatoses/congenital , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/embryology , Abnormalities, Multiple/genetics , Adolescent , Blood Vessels/embryology , Child , Consanguinity , Ectodermal Dysplasia/diagnosis , Ectodermal Dysplasia/embryology , Ectodermal Dysplasia/genetics , Female , Foot Deformities, Congenital/genetics , Humans , Limb Deformities, Congenital/diagnosis , Limb Deformities, Congenital/embryology , Limb Deformities, Congenital/genetics , Male , Scalp Dermatoses/diagnosis , Scalp Dermatoses/embryology , Scalp Dermatoses/genetics , Scalp Dermatoses/pathologyABSTRACT
INTRODUCTION: Granular cell tumor (GCT), also known as Abrikossoff tumor, is a rare and benign neoplasm that more commonly affects the oral cavity in adults. We report here 2 childhood cases of a cutaneous granular cell tumor. CASE REPORTS: An 8- and a 12-year-old girl, with no past medical history, presented with pigmented nodular lesions located, respectively, in the left scapular region and the left iliac crest. Histological examination showed sheets and clusters of infiltrating tumor cells with morphologic and immunohistochemical features consistent with granular cell tumor. In both cases, the lesions were excised with 2-cm margins. The patients are presently being reviewed at 6-month and 2-month intervals to evaluate for recurrence and any malignant transformation. They were in good health with no signs of further tumor development. DISCUSSION: GCT is a relatively uncommon benign neoplasm probably of neural origin derived from Schwann cells. The tongue and buccal mucosa are commonly affected. It develops between the second and sixth decades of life, more frequently among women and blacks. It can also occur in childhood, but this is rare. The cutaneous forms, as in the present observations, are exceptional. Benign granular cell tumours are generally seen as a solitary asymptomatic nodule less than 3 cm in size involving the subcutaneous or submucosal tissues. Histologically, the tumor is not encapsulated but is well circumscribed. The tumor cells may show a degree of infiltration of the surrounding connective tissue. The cells are polygonal, have abundant cytoplasm, are granular and weakly eosinophilic. The nuclei are small, vesicular, and highly chromatic. Mitotic figures are rare. A key element for the histological diagnosis is the expression of S100 protein, neuron specific enolase (NSE), and vimentin on immunohistochemistry. The treatment of choice is a conservative surgical excision of the lesion. However, as the GCT has a poorly defined margin, it is suggested that the tumor should be excised along with portions of adjacent tissue. A low rate of recurrence of the lesion has been reported. CONCLUSION: The specific value of these rare cases is the occurrence in 2 children and the cutaneous location.
