Tumors of the Nail Apparatus and Adjacent Tissues

Epithelioid hemangioendothelioma is a unique tumor of adult life which is characterized by an "epithelioid" or "histiocytoid" endothelial cell. Forty-one cases of this rare tumor have been recognized at the Armed Forces Institute of Pathology. They may occur in either superficial or deep soft tissue, and in 26 cases appeared to arise from a vessel, usually a medium-sized or large vein. They are composed of rounded or slightly spindled eosinophilic endothelial cells with rounded nuclei and prominent cytoplasmic vacuolization. The latter feature probably represents primitive lumen formation by a single cell. The cells grown in small nests or cords and only focally line well-formed vascular channels. The pattern of solid growth and the epithelioid appearance of the endothelium frequently leads to the mistaken diagnosis of metastatic carcinoma. The tumor can be distinguished from a carcinoma by the lack of pleomorphism and mitotic activity in most instances and by the presence of focal vascular channels. Ultrastructural study in four cases confirmed the endothelial nature of the tumor in demonstrating cells surrounded by basal lamina, dotted with surface pinocytotic vesicles, and occasionally containing Weibel-Palade bodies. Follow-up information in 31 cases indicated that 20 patients were alive and well following therapy; three developed local recurrences and six metastases. It is suggested the term epithelioid hemangioendothelioma be used to designate these biologically "borderline" neoplasms. The significance of the epithelioid endothelial cell is not entirely clear. Since it may be observed in both benign and malignant vascular lesions, its presence alone does not define a clinicopathologic entity.

The aneurysmal bone cyst is the result of a specific pathophysiologic change, which is probably the result of trauma or a tumor-induced anomalous vascular process. In approximately one third of cases, the preexisting lesion can be clearly identified. The most common of these is the giant cell tumor, which accounts for 19-39% of cases in which the preceding lesion is found. Other common precursor lesions include osteoblastoma, angioma, and chondroblastoma. Less common lesions include fibrous dysplasia, fibroxanthoma (nonossifying fibroma), chondromyxoid fibroma, solitary bone cyst, fibrous histiocytoma, eosinophilic granuloma, and even osteosarcoma. Interestingly, some of the controversy surrounding this lesion may be the result of a change in how the lesion was defined by Lichtenstein in 1953, when intramedullary lesions were added to the previously described juxtacortical (superficial) lesions. Members of the AFIP have suggested that many of the intramedullary lesions in which no previous lesion can be identified may represent giant cell tumors of bone. Their similarity to proved giant cell tumors in skeletally immature patients can be striking and seems more than coincidental. Appropriate treatment of an aneurysmal bone cyst requires the realization that it results from a specific pathophysiologic process, and identification of the preexisting lesion, if possible, is essential. Clearly an osteosarcoma with superimposed secondary aneurysmal bone cyst change must be treated as an osteosarcoma, and giant cell tumor with secondary features of aneurysmal bone cyst would be expected to be more likely to recur locally. The vast majority (approximately 80%) of patients presenting with aneurysmal bone cystlike findings are less than 20 years old. More than half of all such lesions occur in long bones, with approximately 12-30% of cases occurring in the spine. The pelvis accounts for about half of all flat bone lesions. Most patients present with pain and/or swelling, with symptoms usually present for less than 6 months. The imaging appearance of aneurysmal bone cyst reflects the underlying pathophysiologic change. Radiographs show an eccentric, lytic lesion with an expanded, remodeled "blown-out" or "ballooned" bony contour of the host bone, frequently with a delicate trabeculated appearance. Radiographs may rarely show flocculent densities within the lesion, which may mimic chondroid matrix. CT scanning will define the lesion and is especially valuable for those lesions located in areas in which the bony anatomy is complex, and which are not adequately evaluated by plain films. Fluid-fluid levels are common and may be seen on CT scans and MR images.(ABSTRACT TRUNCATED AT 250 WORDS)

Although the histopathological subtype of melanoma has not been clearly proven to carry independent prognostic significance, acral lentiginous melanoma (ALM) seems to confer a poorer prognosis mainly because disease is often more advanced at the time of diagnosis. To investigate the distinctive epidemiological and clinical characteristics of ALM, a peculiar histological entity, and to identify prognostic factors. We performed a register-based review of cases from a single large referral centre, the University Hospital Department of Dermatology, Lyons, France. We reviewed patient demographics, the initial presentation of the lesion, and clinical outcome. ALM-specific and disease-free survival were estimated using the KaplanMeier method and compared using the log-rank test. A Cox model was used to identify prognostic factors. One hundred and twenty-six patients were identified as having histopathology-proven ALM in our melanoma patient register from 1996 to 2004. There were 46 (37%) subungual ALM and 80 (63%) ALM on soles, palms and nonvolar sites. The mean age at diagnosis was 63 years. There were 44 (35%) men and 82 (65%) women, sex ratio M/F 1 : 1.86. The mean Breslow thickness was 2.51 mm (range: in situ to 20 mm). There was no evidence of overexposure to ultraviolet radiation, nor was there found a predisposing genetic trait. Only 16 (13%) patients recalled a history of trauma. Thirty-four ALM (28%) were unpigmented. The median ALM-specific and disease-free survival were 13.5 and 10.1 years, respectively. The 5-year survival rate was 76%. Multivariate analysis identified tumour thickness, male gender and amelanosis as independent clinical prognostic factors for both ALM-specific and disease-free survival. Our study provides specific information on the clinical characteristics and outcome of this uncommon histological subtype of melanoma. However, the pathogenesis remains unknown. Breslow thickness, male gender and amelanosis were significantly associated with a poorer prognosis.