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Anal malignant melanoma and soft-tissue malignant fibrous histiocytoma in neurofibromatosis type 1.

A 48-year-old man with the nonfamilial form of neurofibromatosis type 1 (NF-1) developed a malignant fibrous histiocytoma in the deep soft tissue of the calf. Following excision and local radiotherapy, the tumor did not recur. At age 59, due to rectal bleeding, a polypoid malignant melanoma of the anal canal was detected and resected. One year later a local recurrence was removed, a pelvic mass was seen on computed tomography, and the patient died. The malignant melanoma was amelanotic and negative for HMB-45 (a melanoma-specific antibody), and the recurrent tumor showed areas of desmoplasia and prominent perineural and intraneural spread. The latter properties underscore the similarities of this melanoma to malignant peripheral nerve sheath tumor. In addition to neurofibroma and malignant schwannoma, NF-1 is associated with other neuroectodermal tumors, including brain tumors and pheochromocytoma. However, it is still controversial whether cutaneous malignant melanoma is more prevalent in NF-1 patients. Anorectal melanoma and malignant fibrous histiocytoma are exceedingly rare in NF-1. Second primary cancers are probably more frequent in NF-1 patients compared to the general population and may be related to alterations in the NF-1 gene.

Anus Neoplasms↗

Origins of heterogeneous ovarian carcinomas. A molecular cytogenetic analysis of histologically benign, low malignant potential, and fully malignant components.

It is unclear whether ovarian carcinomas develop from malignant transformation of benign precursors or whether they arise de novo. Thus, histologically benign or low malignant potential components found in heterogeneous ovarian carcinomas may be remnants of pre-existing lesions that progressed to malignancy or, alternatively, elements that arose independently (de novo). In a third possible interpretation, they represent areas of malignant epithelium that matured. We evaluated clonal relationships of histological components in 10 heterogeneous ovarian carcinomas using fluorescence in situ hybridization and confocal microscopy. Detailed analysis of aneuploidy for chromosomes 8, 12, and 17 on intact paraffin sections revealed that two tumors were aneuploid in all components, two lacked abnormalities in benign-appearing components, and one lacked aneuploidy in both histologically benign and low malignant potential components. Histological appearance was significantly related to aneuploidy (P < 0.05). The distribution of aneuploidy among tumor components strongly supports the tumor progression theory and demonstrates that the de novo hypothesis is highly unlikely (P < 0.001). Results also indicate that benign-appearing components in heterogeneous ovarian carcinomas do not represent maturation of malignant tissue and suggest that some benign tumors that become cancerous may have genetic aberrations that predispose them to malignant transformation.

Aneuploidy↗

Glycogen-rich malignant melanomas and glycogen-rich balloon cell malignant melanomas: frequency and pattern of PAS positivity in primary and metastatic melanomas.

OBJECTIVE: After identifying a metastatic glycogen-rich balloon cell malignant melanoma, originally thought to be a benign clear cell tumor of the lung, we investigated the extent of positive reactions, or "positivity," of malignant melanoma to periodic acid-Schiff (PAS) staining. METHODS: Frequency, intensity, and distribution of PAS positivity was studied in 61 excisional biopsy specimens from 58 patients with malignant melanoma. For comparison, 17 benign nevi from 10 patients were examined. RESULTS: Positivity for PAS was seen in all cases. All malignant melanomas and benign nevi were characterized by weak, diffuse, diastase-resistant PAS positivity. Additionally, focal or diffuse, strong diastase-sensitive PAS positivity was observed in 9 of 61 melanomas (15%); 7 were metastatic and 2 were primary invasive melanomas. Strong diastase-sensitive PAS positivity was seen in all lesions with 30% or more balloon cell features and only in advanced primary or metastatic lesions. The presence of glycogen was confirmed by transmission electron microscopy. CONCLUSIONS: Cutaneous malignant melanomas have weak, diastase-resistant PAS positivity. Strong diastase-sensitive PAS positivity, consistent with the presence of intracytoplasmic glycogen, is seen in many primary and metastatic melanomas with balloon cell features. Depending on the content of the balloon cells, these melanomas are best categorized as either glycogen-rich malignant melanomas or glycogen-rich balloon cell malignant melanomas. Because many tumors with clear cell features contain glycogen, such content often is an unreliable differential feature.

Biopsy↗

[Thoracoscopic video-assisted talcum pleurodesis (TTP)--effective treatment of malignant pleural effusion].

INTRODUCTION: Aim of the palliative therapeutic procedure should be a fast, efficient and pain free treatment of the malignant pleural effusions. PATIENTS AND METHOD: Since 1995 the results of malignant pleural effusions treatment were analyzed retrospectively in 46 patients who underwent 51 video-assisted TTP. The most frequent origin of malignant pleural effusion was breast cancer, followed by bronchial carcinoma. Precondition for video-assisted TTP was the verification of a malignant pleural effusion by cytology. RESULTS: Following TTP one patient suffered from pneumonia, two recurrent diseases occurred. Four patients died due to their severe primary malignancy. Average hospitalization was 8 days (3-55). CONCLUSION: Video-assisted TTP is the standard procedure in palliative treatment of malignant pleural effusion; the recurrence rate is low and the technique minimal invasive.

Adult↗

[Malignant neuroleptic syndrome and malignant hyperthermia--a comparison].

Malignant Hyperthermia is a specific potentially fatal condition which occurs in susceptible individuals in response to various triggering mechanisms, of which anesthetic agents have been found to be the most common offenders. Leading symptoms are generalized muscular rigidity and hyperpyrexia. The etiology seems to be associated with some inherited disturbance in muscle metabolism related to calcium regulation. This syndrome is known for 25 years, the mechanism of triggering, the genetics and the treatment have been able to get examined by animal model as malignant hyperthermia syndrome may occur in swine as well. Pharmacological in-vitro studies on biopsy specimen of muscle fragments are presently one of the most accepted means for pre-anesthetic diagnosis, the hydantoin derivate dantrolene sodium is the only known specific drug in treatment and prophylaxis. Neuroleptic Malignant Syndrome which occurs in patients treated with neuroleptics shows almost identical symptoms. Although the pathogenesis is still unknown, most authors believe a neuroleptic-induced alteration of central neuroregulatory mechanisms is involved. Alternative etiologic mechanisms are suggested by the striking similarities noted between the Neuroleptic Malignant Syndrome and Malignant Hyperthermia. Both disorders might be based on common pathophysiologic mechanisms and might be induced in susceptible patients by a variety of pharmacologic agents. The authors present and compare the actual knowledge concerning symptoms, course and therapy of both syndromes on the basis of 126 case studies of the Neuroleptic Malignant Syndrome from literature.

Adult↗

Risk of malignant hyperthermia among patients with neuroleptic malignant syndrome and their families.

The occurrence of malignant hyperthermia in 20 patients who had had neuroleptic malignant syndrome and in their 108 first-degree relatives was retrospectively studied. The patients had experienced a total of 20 courses of ECT and 12 surgical operations in which drugs that can cause malignant hyperthermia were used, but no cases of malignant hyperthermia had occurred, and no cases had occurred in the 37 relatives who had undergone surgery or ECT. The authors conclude that patients who have had malignant neuroleptic syndrome are not at considerably greater risk than others for developing malignant hyperthermia during surgery or ECT; the clinical data suggest that the two disorders are not related.

Adolescent↗

[Malignant hyperthermia susceptibility in 3 patients with malignant neuroleptic syndrome].

Hyperthermia, skeletal muscle rigidity, rhabdomyolysis, acidosis and multiple system insufficiency characterize malignant hyperthermia. Anaesthetic malignant hyperthermia follows halogenated volatile agents and/or depolarizing muscle relaxants utilization. Diagnosis is based on in vitro muscle contracture in response to halothane and/or caffeine exposure. Neuroleptic malignant syndrome affects patients taking neuroleptic drugs; clinical findings include hyperthermia, extrapyramidal rigidity, acidosis, neurovegetative instability and neurological signs. We report three neuroleptic malignant syndrome patients with positive muscle contracture tests which shows that muscle from neuroleptic malignant syndrome patients may in some instances show alterations similar to those of anaesthetic malignant hyperthermia.

Adult↗

[Malignant hyperthermia and neuroleptic malignant syndrome: report of 4 clinical cases].

Very numerous publication in the literature suggest the probable relationship between three clinical entities: peranesthesic malignant hyperthermia (PMH), exercitional malignant hyperthermia (EMH) and neuroleptic malignant syndrome (NMS). We briefly describe the clinical history and define, as subjects of research, the personal and familial histories of neuromuscular disease of 4 of our patients having presented with neuroleptic malignant syndrome, as recommended by the European group on malignant hyperthermia, the reference contracture test on neuromuscular fibers and anatomic and cytopathological investigations of neuromuscular fibers. We compare the results for our 4 patients having presented with neuroleptic malignant syndrome and their courses. We confirm the necessity of pursuing both case history and laboratory research in order to elucidate the debate on the hypothesis of a common etiopathogenesis for the 3 syndrome entities.

Adult↗

Oxidative stress in malignant and non-malignant phase hypertension.

Malignant hypertension (MHT) is a rare and severe form of hypertension characterised by arteriolar necrosis and severe vascular damage, leading to stroke, myocardial infarction and death. We hypothesised that in addition to endothelial damage, MHT may be associated with increased oxidative stress. Lipid hydroperoxides (LHP, an index of oxidative damage) and plasma von Willebrand factor (vWf, an index of endothelial damage/dysfunction) were measured in 16 patients with MHT and compared with 16 non-malignant essential hypertensives and 32 normotensive controls. vWf was greater in MHT (mean 117 iU/dL) than in non-malignant hypertensives (97 iU/dL) or normotensive controls (100 iU/dL) (ANOVA P = 0.017). However, although LHP were greater in MHT (mean 10.6 micromol/L) than in normotensives (4.5 micromol/L, P < 0.001), the levels in MHT were similar to those in non-malignant hypertension (12.3 micromol/L). In conclusion endothelial damage (raised vWf) was more evident in MHT compared with both normotensive controls and with non-malignant hypertension, whilst oxidative stress (raised LHP) was increased to a similar extent in both hypertension groups when compared with normotensive controls. These observations raise the possibility abnormal oxidative stress is probably not the mechanism responsible for the endothelial damage seen in malignant phase hypertension.

Analysis of Variance↗