Based on the results of a retrospective study, which found blood vessel invasion to be the most important prognostic factor in clinical stage I nonseminomatous testicular germ cell cancer (NSTGCC I), a prospective study was started in 1985 which assigned NSTGCC I patients without evidence of vascular invasion to surveillance and patients with vascular invasion to two cycles of adjuvant chemotherapy with cisplatin, etoposide, and bleomycin. Twenty-two patients entered the surveillance group and 18 patients received adjuvant chemotherapy. Median follow-up is 30 months (3 to 50 months). Relapses occurred in three patients (7.5%), one in the surveillance group (4.5%), two in the chemotherapy group (11%). Thirty-eight patients (95%) are alive and without evidence of disease. Two patients of the adjuvant-treated group died, one of progressive germ cell cancer and one of lung cancer. We conclude that low- and high-risk NSTGCC I patients can be identified by considering blood vessel invasion. The presence of embryonal carcinoma and vascular invasion seem to be interrelated prognostic factors, because in 94% of vessel invasion the invading element was embryonal carcinoma. The exclusion of patients with vascular invasion from surveillance decreases relapse rates remarkably. Adjuvant chemotherapy diminishes relapse rates in high-risk patients but does not entirely prevent relapse.
In patients with intravascular lymphomatosis (IL) a broad spectrum of neuro-psychiatric disorders including dementia, focal neurological signs and seizures has been reported. Clinical diagnosis is difficult since neuroimaging findings are nonspecific. The clinical histories, biopsy and autopsy findings of three patients with IL, one with brain biopsy, are described. Two of them presented with rapidly progressive, fluctuating dementia. The third patient suffered from seizures followed by aphasia. Histology revealed large-cell lymphoma of the B cell type. The prognosis of IL, in general, is poor.
In a retrospective study the primary tumors of 33 patients with seminomas and 53 with nonseminomatous germ cell tumors were re-evaluated for vascular invasion. The significance of vascular invasion was analyzed in respect to the appearance of visceral metastases and the effect of adjuvant chemotherapy. Vascular invasion was demonstrated in 27 per cent of the patients with seminomas and 53 per cent with nonseminomatous germ cell testis tumors, while visceral metastases appeared in 9 and 32 per cent, respectively. Without adjuvant chemotherapy all 13 patients with nonseminomatous germ cell testis tumors and vascular invasion had metastases, compared to only 3 of 13 without vascular invasion (p less than 0.0005). Of 9 patients with seminoma and vascular invasion 3 had tumor progression, compared to 1 of 24 without vascular invasion (p greater than 0.05). With adjuvant chemotherapy only 1 of 15 patients (7 per cent) with nonseminomatous germ cell testis tumors and vascular invasion had metastases, compared to 100 per cent of 13 without this treatment. No significant correlation was noted between pT stage versus vascular invasion and pT stage versus tumor progression. The results demonstrate the importance of vascular invasion in the staging of and choice of treatment for early nonseminomatous germ cell testis tumors.
A patient with IgM-lambda plasmacytoma presented with continuous muscle fiber activity syndrome (CMFAS), which responded to carbamazepine therapy. This is the first report of CMFAS in association with plasmacytoma and IgM paraproteinemia.
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