Localisation of gene for the naevoid basal-cell carcinoma syndrome.
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Biomedical subjects
Publications and source records attributed to W Groth.
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The validity of current staging systems for malignant melanoma was analyzed in a prospective study on 220 patients with extremity melanoma. Patients were followed 2 to 9 years after a wide excision of the primary, regional cytostatic perfusion and dissection of regional lymph nodes. The "original three-stage" system yielded statistically significant differentiation, but with a distinct preference for Stage I. The classification into IA/B, and IIIA/B/C according to the M.D. Anderson system does not distribute the patients into significantly different tumor stages. Using the American Joint Committee of Cancer Staging and End Results Reposting (AJCC) system, 40 patients were classified as Stage I, 95 as Stage II, 53 as Stage III, and 32 patients as Stage IV. The 5-year survival rate was 96% in Stage I, 90% in Stage II, 68% in Stage III, and 30% in Stage IV. According to the UICC staging system there was a numerical preference of Stage II and III. The differentiation of Stage I and II was not significant. It is the authors' opinion that the AJCC staging system allows the best differentiation of melanoma patients into tumor stages that are evenly distributed and significant for prognoses.
We report on a 20-year-old man and a 14-year-old girl showing multiple basaloid follicular hemartomas on face and upper back. Both patients were members of a family with Gorlin-Goltz syndrome.
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Patients with level V melanoma have a very bad prognosis on account of their high rate of lymph node and distant metastases. From Jan. 1979 to Dec. 1987, a total of 251 patients suffering from malignant melanoma of the extremities (including 28 patients with level V melanoma = 11.2%) underwent excision of the primary tumor, lymph node dissection, as well as regional hyperthermic perfusion with cytostatics. The prognosis of our patients with level V melanoma could be improved considerably by therapy.
We report on an 80-years-old woman suffering from subacute prurigo simplex, who had developed a painless subcutaneous tumor on her right upper back, 8 cm in diameter. In palpation, the tumor was firm and did not adhere to the overlying skin. The movability of her arms and back was not restricted. The tumor could not be distinguished from its surrounding tissue by means of ultrasound. Surgery revealed that the tumor was firmly attached to the thoracic fasciae, periosteum, and ligaments, which prevented its complete removal. On histological examination, the tumor was diagnosed as elastofibroma dorsi. We discuss our microscopical findings of this very rare neoplasm in detail.
We report on a 49-year-old female patient suffering from Pacinian neurofibroma on her right middle finger. The microscopical findings of this rare neoplasm are discussed in detail.
To evaluate the effectiveness of regional hyperthermic cytostatic perfusion in patients with malignant melanomas of the extremities, 107 patients were included in a prospective randomized study. In a control group (A, n = 54) the tumors were widely excised, and the regional lymph nodes were dissected. The patients in the perfusion group (B, n = 53) received additional hyperthermic (42 degrees C) perfusion with melphalan. The disease-free survival time was chosen as the criterion for success. An intermediate evaluation (average follow-up observation period of 550 days) revealed a highly significant difference between the groups (P = 0.0001): 21 recurrences in the control group versus four recurrences in the perfusion group. In a second analysis 3 1/2 years after premature discontinuation, 26 recurrences were diagnosed in Group A, whereas only six recurrences were noted in Group B (P = 0.0001). A retrospective analysis of the entire test group revealed the following figures. In Group A seven recurrences in Stage I were diagnosed, seven in Stage II, and 12 in Stage III. In Group B one was observed in Stage I, one in Stage II, and four in Stage III. The level of significance was calculated to be P = 0.05 in Stage I, P = 0.05 in Stage II, and P = 0.01 in Stage III. The results of the study show that additional perfusion in the treatment of extremity melanomas is superior to conventional methods.
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The clinical course of a 50-year-old woman with oral lichen ruber planus (Irp) and prolonged dysphagia is described. The swallowing problems of this patient were related to an inflammatory lesion of the middle third of the esophagus, leading to stenosis. Distal to this area, the esophagus was covered with mucosa of the cardia type, as seen in endobrachyesophagus. Apart from reflux disease, the Irp may have accounted for the stenosis of the mid-esophagus. The mucosal lesions disappeared after administration of etretinate (Tigason). After endoscopic dilatation the patient was able to swallow normally again.
A malignant blue nevus of the right upper arm with hematogeneous lung metastases is presented. Histological examination showed that the tumor was composed of spindle, dendritic, and globular cells with hyperchromatic and polymorphic nuclei, atypical mitoses, and tumor cell necrosis. There was no proliferation of atypical melanocytes at the dermoepidermal junction. Histologically, malignant blue nevus should be distinguished from benign cellular blue nevus, primary cutaneous malignant melanoma, cutaneous metastases of malignant melanoma, and clear cell sarcoma.
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Roentgen morphological, ultrasonographical and CT findings in a patient with mammary cancer metastasising hematogenously to the duodenum and colon and in four patients with malignant melanoma metastasising hematogenously to the stomach, duodenum, small bowel and colon.
To evaluate the effectiveness of regional hyperthermic cytostatic perfusion in patients with malignant melanomas of the extremities 107 patients were included in a prospective randomized study. In a control group (A, n = 54) the tumors were excised widely and the regional lymph nodes were dissected. The patients in the perfusion group (B, n = 53) received additional hyperthermic (42 degrees C) perfusion with Melphalan. We chose the disease-free-survival time as the criterion for success. An intermediate evaluation (mean follow-up observation period of 550 days) revealed a highly significant difference between the groups (p = 0.0001) of 21 resp. 4 recurrences. A second evaluation, 2 1/2 years after prematurely discontinuation, also shows a highly significant difference in favour of perfusion (p = 0.0001).
Cutaneous metastases of malignant melanoma were seen as small red papules with central hair resembling the typical clinical picture of folliculitis. This type of cutaneous metastasis was observed in a 51-year-old woman 12 months and in a 42-year-old man 30 months after wide excision of cutaneous malignant melanoma, in both cases located on the lower extremity. The first case presented a reflux metastasis, the second case a hematogenous metastasis on the contralateral thigh. Histological findings concurrently revealed metastases of malignant melanoma located at the cutaneous-subcutaneous interface beneath the hair follicle. This type of cutaneous metastasis could be easily excised in toto by means of a 6 mm diameter punch biopsy.
The concentrations of indole melanogens have been measured in 24-hour urine samples of 44 patients suffering from malignant melanoma, clinical stages I to IV, and 23 healthy test persons (control group). With regard to the controls, the urinary concentration of indole melanogens amounted to 3.8 +/- 1.329 micrograms/ml; the excretion was 5.41 +/- 1.941 mg daily. In patients with localized malignant melanoma, the concentration of indole melanogens did not differ from the control group. Highly significant levels, however, were measured in patients with generalized disease (7.76 micrograms/ml and 6.85 mg daily). Tyrosin orally given (7 g on the 2nd, 3rd, and 4th day of the collecting period) did not elevate the urinary excretion of indole melanogens in any of the test groups. These results indicate that the applied procedure does not present any fundamental advantage for clinical oncology.
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A human malignant melanoma was grown in nude mice. The tumour showed an exponential growth for several weeks which gradually slowed down, until following week 8 the tumour growth ceased. The reasons for this growth pattern were examined by labelling techniques (PLM, LI). The tumour cell production as quantified by the growth fraction, showed only a moderate reduction which, taken alone, could not explain the growth cessation. The important mechanism seems to be an increased loss of tumour cells during the intermitotic interval. While the loss of cells/h remains constant the total intermitotic cell loss is increased because the cell cycle times are prolonged by 50% from the exponential phase (45 h in week 3) to the plateau phase (66 h in week 8).