The prognostic value of tumor mitotic rate and other clinicopathologic factors in patients with locoregional recurrences of melanoma.

BACKGROUND: Tumor mitotic rate (MRP) is an independent prognostic factor in clinically localized primary cutaneous melanoma, but the prognostic importance of mitotic rate in melanoma recurrences (MRR) is not known. In this study, we sought to determine the prognostic value of MRR and other clinicopathologic factors in recurrent melanoma. METHODS: Patients with primary cutaneous melanoma diagnosed between 1979 and 2006, who subsequently developed recurrence(s), were studied. Histologic sections of primary and first locoregional recurrences of melanoma were examined, and MRP and MRR were measured. Relationships between MRR, known prognostic parameters in melanoma, time to first recurrence (TTR), and postrecurrence survival were analyzed. RESULTS: A total of 279 patients (172 men, 107 women) had AJCC stage I (n = 97) or stage II (n = 182) melanoma. Median MRP and MRR were 4/mm(2) (0-34) and 4/mm(2) (0-51), respectively. There was weak association between MRP and MRR (R (2) = 0.02, p = 0.02). Independent predictors of poorer postrecurrence survival were shorter TTR (hazard ratio, 0.74; 95% confidence interval, 0.61-0.90, p = 0.003) and recurrence type (10-year postrecurrence survival for local, lymph node, and in-transit recurrences: 70%, 21.5%, and 11.1%, respectively; p = 0.04). MRR >0 was associated with poorer 10-year postrecurrence survival (39.1%) than if MRR = 0 (51.2%), but the difference did not reach statistical significance (p = 0.15). However, the difference in survival between patients with MRR >0 and those with MRR = 0 increased with time. CONCLUSIONS: TTR is an independent predictor of postrecurrence survival. Because survival in patients with MRR >0 decreases with time (relative to those with MRR = 0), MRR should be routinely measured so that future studies can determine whether MRR has any independent predictive value.

Myosin storage (hyaline body) myopathy: a case report.

Myosin storage myopathy/hyaline body myopathy is a rare congenital myopathy, with less than 30 cases reported in the literature. It is characterised by the presence of subsarcolemmal hyaline bodies in type 1 muscle fibres and predominantly proximal muscle weakness. Recently, a single mutation (Arg1845Trp) in the slow/beta-cardiac myosin heavy chain gene (MYH7) was identified in four unrelated probands from Sweden and Belgium. The clinical severity and age of onset was variable, despite the same disease-causing mutation and similar histological findings. Here, we report the clinical and morphological findings of two brothers of English/Scottish background with the Arg1845Trp mutation in MYH7. This case report adds to the clinical description of this rare disorder and confirms that Arg1845Trp is a common mutation associated with this phenotype, at least in the White European population.