1/370. Epithelioid cell histiocytoma: a simulant of vascular and melanocytic neoplasms.Epithelioid cell histiocytoma (ECH) is an unusual and still poorly recognized variant of benign fibrous histiocytoma. Epithelioid cell histiocytoma differs from most benign fibrous histiocytomas in five important ways: the predominance of epithelioid cells, relative lack of secondary elements (such as giant cells, foamy, or hemosiderin-laden macrophages), relative sharp circumscription, prominent vascularity, and centering in the papillary dermis in most cases. A strong resemblance to melanocytic and vascular lesions has been noted, and a recent case was reported with features suggesting endothelial origin. Fifteen new cases of ECH, including one example of the rare deep cellular variant, are presented herein, with emphasis on features mimicking vascular and melanocytic neoplasms. Labeling with endothelial markers, including previously unreported CD-31 labeling, showed abundant vascular staining, which may be challenging to interpret, but which does not indicate an endothelial origin of ECH.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/370. 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) |
3/370. A case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified COL1A1 and PDGFB sequences.dermatofibrosarcoma protuberans (DFSP) is a cutaneous tumour of borderline malignancy, the cytogenetic features of which include the translocation t(17;22)(q22;q13) or, more commonly, supernumerary ring chromosomes containing material from 17q22 and 22q13. These rearrangements result in the COL1A1/PDGFB fusion gene. Here, we describe a case of DFSP displaying a ring chromosome 5 together with a large marker chromosome composed of chromosome 22 alphoid dna, material from distal 12q and amplified COL1A1 and PDGFB sequences. This is the first case of DFSP with multiple copies of COL1A1 and PDGFB not confined to ring chromosomes, showing that DFSP is similar to other borderline malignant mesenchymal tumours, where rings and giant markers are alternative vehicles for amplified material.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/370. Multinucleate-cell angiohistiocytoma occurring in a patient with mycosis fungoides.Multinucleate-cell angiohistiocytoma (MCAH) is a benign vascular proliferation of unknown etiology. Clinically, MCAH presents as grouped, erythematous, or violaceous papules on the extremities in older women. These lesions often resemble Kaposi's sarcoma. Histologic examination reveals characteristic bizarre-shaped, multinucleate giant cells, some of which contain three to six nuclei arranged in a ring-like or overlapping pattern, which stain positively for factor xiiia. In addition, there is a proliferation of dermal capillaries and venules with a mild lymphohistiocytic infiltrate. To our knowledge, this is the first reported case of MCAH occurring in a patient with mycosis fungoides.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/370. Giant cavernous hemangioma of the scalp.In this article, a quite rare giant cavernous hemangioma located in right frontoparietal region of a 16 year-old boy is presented. Cavernous hemangiomas are commonly seen in extremities, neck, face and sometimes scalp, cutaneous or subcutaneous in localization. Giant cavernous hemangioma in the right frontoparietal region have been seen in two cases so far. In this article, we discussed the differential diagnosis, characteristics and therapy methods of giant cavernous hemangioma of the scalp.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
6/370. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin.We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/370. Giant congenital multiple patch-like glomus tumors.Glomus tumors can be classified into solitary and multiple glomus tumors. Congenital multiple glomus tumors are extremely rare, and only 12 cases have been reported in the world literature. We describe the first case with giant congenital multiple patch-like glomus tumors that show partial atrophy.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
8/370. 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) |
9/370. Basal cell carcinoma with tumor epithelial and stromal giant cells: a variant of pleomorphic basal cell carcinoma.A case of basal cell carcinoma with giant cells of the central epithelial and surrounding stromal components is presented. The lesion was an 8-mm dome-shaped papule on the ear of a 66-year-old man. The giant cells of the epithelial component shared the immunophenotype of the more typical cells of the basal cell carcinoma (keratin, smooth muscle actin, and bcl-2 positive), whereas the stromal giant cells were positive only for bcl-2. This case represents a peculiar variant of pleomorphic basal cell carcinoma, the significance of which is unknown.- - - - - - - - - - ranking = 7keywords = giant (Clic here for more details about this article) |
10/370. 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) |
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