Onychomatricoma (OM) is a fibroepithelial tumor of nail matrix that occurs in the digits of both the hands and feet. This was first reported by Baran and Kint. They initially described 3 cases, all of which demonstrated a filamentous tumor of matrix tissue that resulted in a thickened funnel-shaped nail. Although apparently benign, it is subject to recurrence, and long-term follow-up is recommended because it is not known whether there is a conversion to malignancy. Even though this neoplasm was first described more than 18 years ago, there remains a dearth of case reports (currently fewer than 50) in the literature. This is a single case report and literature review. Levels of Evidence: Therapeutic, Level IV
In our previous research, bcl-2-positive dendritic cells were seen in increased numbers in suprabasal areas of inverted follicular keratoses (IFKs) compared to seborrheic keratoses (SKs). The purpose of this study was twofold; firstly, to support that these dendritic cells are Langerhans cells and secondly, to contrast the epidermal dendritic cells in IFKs, squamous cell carcinomas (SCCs), and SKs.Ten IFKs and five SKs previously showing bcl-2-positive dendritic cells within the epidermis were stained with CD1a. Fifteen other IFKs were stained with CD1a alone. Ten SCCs were stained with bcl-2 and CD1a.In the 10 IFKs stained with both bcl-2 and CD1a, the density of CD1a-positive cells correlated well with the density of bcl-2-positive dendritic cells; in the five SKs, the density of CD1a-positive cells was similar to that seen in IFKs but was higher compared to the density of bcl-2-positive dendritic cells in SKs (p < 0.05). The IFKs had a significantly higher number of CD1a-positive cells compared to SCCs (p < 0.01). SCCs showed bcl-2-positive cells only within the basal layer of the normal epidermis flanking the carcinomas.These results suggest that there is different bcl-2 regulation of CD1a-positive cells in IFKs, SKs, and SCCs.
Ossifying fibromyxoid tumor (OFMTs) of soft parts is a rare soft tissue and bone tumor of borderline malignancy displaying an uncertain line of differentiation. The existence of fully malignant OFMT is controversial. To better understand the natural history and line of differentiation taken by OFMT, we studied 46 cases by light microscopic, immunohistochemical (IHC), genomic, proteomic, and fluorescence in situ hybridization (FISH) methods. Cases were classified according to the 2003 Folpe and Weiss system. Clinical and follow-up information was obtained. IHC for S-100 protein, desmin, epithelial membrane antigen (EMA), cytokeratins, smooth muscle actin (SMA), INI-1, neurofilament protein (NFP), CD56d excitatory amino acid transporter-4 (EAAT4), and MUC4 was performed on formalin-fixed, paraffin-embedded (FFPE) tissues. Gene expression profiling and proteomic studies were conducted on FFPE tissues from 13 and 5 cases, respectively. FISH for INI-1 was performed on 10 cases. The 46 tumors arose in 29 men and 17 women (median age, 52 y; range 39 to 63 y) and involved the proximal (N=17) and distal extremities (N=13), head and neck (N=9), and trunk (N=5). Median tumor size was 5.4 cm (range, 1.0 to 21.0 cm). Cases were classified as typical OFMT (26 of 46, 57%), atypical OFMT (5 of 46, 11%), and malignant OFMT (15 of 46 cases, 32%). Clinical follow-up (27 cases, median 55 months' duration) showed all patients with typical and atypical OFMT to be alive without disease. Adverse events, including 3 local recurrences, 3 metastases, and 3 deaths, were seen only in malignant OFMT. IHC results were as follows: S-100 protein (30 of 41, 73%), desmin (15 of 39, 38%), cytokeratin (4 of 35, 11%), EMA (5 of 32, 16%), SMA (2 of 34, 6%), INI-1 (lost in mosaic pattern in 14 of 19, 74%), EAAT4 (31 of 39, 80%), MUC4 (3 of 14, 21%), NFP (8 of 10, 80%) and CD56 (6 of 14, 43%). Gene expression profiling showed typical and malignant OFMTs to cluster together, distinct from schwannian tumors. Proteomic study showed expression of various collagens, S-100 protein, and neuron-related proteins. FISH showed INI-1 deletion in 5 of 7 (71%) cases. We conclude that malignant OFMTs exist and may be recognized by the previously proposed criteria of Folpe and Weiss. Expression of neuron-related markers, in addition to Schwann cell and cartilage-associated markers, suggests a "scrambled" phenotype in OFMTs. Loss of INI-1 or other genes on 22q is likely important in the pathogenesis of these rare tumors.
Purpose of review Lymphatic mapping and sentinel lymph node biopsy have been established as definitive procedures for the staging of cutaneous melanoma. Large-scale studies that have been recently conducted and that are ongoing suggest a therapeutic role for lymphatic mapping/sentinel node biopsy in the management and prognosis of melanoma patients with early lymph node metastases. Recent findings Sentinel node biopsy has been shown to extend disease free survival and increase melanoma-specific survival for patients with early nodal metastases according to interim analysis of the Multicenter Selective Lymphadenectomy Trial 1 (MSLT-1). The proper evaluation of sentinel lymph nodes requires histologic and immunohistochemical analysis of multiple levels. Immune modulation has been shown to play an important role in nodal metastasis. Summary There is increasing evidence for the efficacy of lymphatic mapping and sentinel lymph node biopsy in predicting prognosis, reducing the morbidity traditionally associated with regional lymph node dissection and increasing survival in subgroups of patients with cutaneous melanoma. Further study is needed to determine the role of the immune system in the spread of nodal metastases and the role of immunomodulatory therapy to prevent or possibly even reverse nodal metastases.
