1/134. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease.We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/134. Epidermal nevi and localized cranial defects.We report on a girl with a congenital pigmented hairy nevus of the scalp, epidermal nevi of the right temple, and localized cranial defects. We have not found other reported cases of giant pigmented hairy nevus of the scalp occurring with absence of underlying cranial bone. We speculate that the localized cranial defects are undergrowth anomalies representative of a paracrinopathy from the overlying nevus or simultaneous bone/skin dysplasia, the former having been resorbed. In the absence of a familial history of epidermal nevi and/or seizures, our patient represents a sporadic case, perhaps a somatic mutation.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/134. Giant hairy nevus: preventable cause of amblyopia.An infant with a congenital giant hairy nevus causing occlusion of the visual axis of the right eye is presented. The nevus was removed at the early age of three weeks because of concern that the child would develop deprivation amblyopia. Since such nevi can undergo malignant changes, early removal may be justified for that reason alone.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/134. Adnexal-centered giant congenital melanocyte nevus with extensive ganglioneuromatous component and trisomy 7.Adequate interpretation of clinical and histopathologic features of giant congenital melanocytic nevus (GCMN) in newborns is a continued challenge. A GCMN with three large nodules and three polypoid exophytic tumors presented in the dorsum of a female full-term newborn, the borders exhibiting a spotted grouped pattern. Microscopic examination revealed a peculiar adnexal-centered (eccrine sweat gland ducts, acrosiringia, and hair infundibula) compound nevus expressing pagetoid intraepidermal spreading of epithelioid melanocytes. The nodules represented an extensive ganglioneuromatous component. The neurons and their neuropil were positive for neuron-specific enolase, S-100, synaptophysin, tyrosine hydroxilase, and PGP 9.5. In addition to these components, a poorly differentiated, fusiform, low-mitotic rate population of cells undergoing epithelioid differentiation (and probably neuronal differentiation) with nodular arrangement was also present in the polypoid tumors and deeper parts of the nevus, in part intermixed with the neurons. These cells were vimentin positive but S-100 negative. FISH studies revealed these cells to express three signals for the centromeric probe for chromosome 7 whereas the neuronal component showed just two. Adnexal-centered arrangement of melanocytes has not been emphasized in GCMN. Ganglioneuromatous differentiation has been rarely reported in this condition. trisomy 7 in GCMN has been reported only once previously.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
5/134. Secondary reconstruction of a giant congenital lentiginous dermal nevus with serial, large-volume tissue expansion.Giant congenital pigmented nevi pose a substantial reconstructive challenge for the treating physician. Due to the increased risk of malignant transformation in such lesions, complete excision with tissue expansion or skin grafting is the generally accepted treatment. These modalities can, however, leave the patient with secondary deformities that also require complex reconstructive procedures. The following case details a patient requiring secondary reconstruction with large-volume tissue expansion 12 years after excision of a giant nevus, and split-thickness skin grafting. This patient illustrates a severe secondary deformity and the usefulness of large-volume serial expansion in such patients.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
6/134. Malignant melanoma in the thymus.A case of malignant melanoma in the thymus is reported. diagnostic imaging demonstrated a left anterior mediastinal mass in a patient with giant pigmented nevus without malignant change. Histologic and cytologic specimens obtained from the tumor revealed that the tumor was malignant melanoma. Surgery revealed malignant melanoma in the left lobe of the thymus. Many cell nests of pigmented nevi were observed throughout the thymus. The malignant melanoma was thought to have originated from the nevocellular nevus in the thymus. This is the first report of malignant melanoma in the thymus.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/134. A newborn with nodular ulcerated lesion on a giant congenital nevus.The proliferative nodule is a lesion that develops within a congenital nevus. It is usually small (less than 5 mm), sometimes multiple, with a slow growth rate, and has a black or dark brown, smooth, shiny surface. It usually involutes spontaneously. We report a newborn infant who, at birth, had a giant congenital nevus with a nodular, ulcerative, hemorrhagic lesion within it. Physical and neurologic examinations were normal. Radiologic studies at birth and subsequently were normal. A fragment of the lesion was biopsied and histologic findings were compatible with a diagnosis of proliferative nodule in a giant congenital nevus. The rest of the nodule regressed spontaneously after 4 months.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
8/134. Giant congenital melanocytic nevus with underlying hypoplasia of the subcutaneous fat.A 17-week-old boy with a giant congenital melanocytic nevus (GCMN) of the left lower extremity was noted to have a reduction in circumference of the left lower extremity relative to the contralateral side. The skin overlying the GCMN was persistently warm when compared with the surrounding and contralateral skin. Comparative plain radiography, ultrasonography, and magnetic resonance imaging showed fat hypoplasia of the left lower extremity, with bone and muscle appearing unaffected. The possible role of cytokines produced by the nevus in fat hypoplasia in GCMN is discussed.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
9/134. Age-related changes of the MR appearance of CNS involvement in neurocutaneous melanosis complex.We report a case of giant congenital melanocytic nevi (GCMN) at risk of developing neurocutaneous melanosis (NCM) with age-related changes observable on MRI of the brain. However, although the usefulness of MR imaging in NCM is well known, age-related changes on MRI have rarely been reported. The prevalence of positive MRI findings and prognosis in GCMN accompanied by epilepsy and/or mental retardation awaits clarification. This case report may suggest the importance of serial brain MRI in cases of GCMN in assessing the risk of NCM.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/134. Giant congenital melanocytic naevus and symptomatic thoracic arachnoid cyst.A 43-year-old female with multiple congenital naevi presented with a 5-year history of right upper quadrant pain. magnetic resonance imaging demonstrated a large epidural thoracic arachnoid cyst. This case highlights the need to consider that the management of patients with giant congenital melanocytic naevus is variable depending on the age of the patient, the location of lesions and the presence of complications such as neurocutaneous melanosis. The value of magnetic resonance scanning as a screening device is discussed.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
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