Unusual spinal metastases from an adenoid cystic carcinoma of the maxillary sinus.

Adenoid cystic carcinoma (ACC), the second most common cancer occurring in the sinonasal tract, is an aggressive malignancy with a poor five-year survival rate. Spinal metastases to the vertebral column related to this cancer are rare. This report presents a patient with maxillary sinus carcinoma with vertebral metastases at the thoracic level and compression of the spinal cord seven years after surgical resection of the primary tumor. Eleven years after detection of the primary tumor the patient is still able to walk. The role of decompression and/or fusion in spinal metastases with neurologic deficits is still under debate, although recent studies have confirmed the beneficial role of surgical intervention in selected patients. This report represents an example of modern individual treatment of an aggressive tumor in a palliative situation. The epidemiology, clinical findings, treatment and outcome of this atypical distant metastasis in long-term survivors are presented.

[Acute abdomen in abdominal actinomycosis]. / Akutes Abdomen bei abdomineller Aktinomykose.

The case of a female patient aged 34 suffering from abdominal actinomycosis is reported. She underwent emergency surgery for an abscess-forming tumour infiltrating the lateral abdominal wall and giving rise to the clinical picture of acute abdomen. The casuistry is complemented by two further case histories of actinomyces infections in patients with fistulas of the anal region and pilonidal sinus respectively. Abdominal actinomycosis presents itself as a rare chronic bacterial inflammation, usually located in the right lower abdomen, with local abscess or fistula formation as well as tumour-like infiltration of the surrounding tissues. Exceptionally, the diagnosis is ascertained prior to surgery. Postoperative antibiotic therapy with penicillin for several months ist essential.

Extraadrenal intralymphonodular myelolipoma.

An extraadrenal myelolipoma inside a lymph node in a 70-year-old man is reported as the first description of this tumour in the given localization.

Greig syndrome in a large kindred due to reciprocal chromosome translocation t(6;7)(q27;p13).

We report on cases of Greig syndrome segregating in a large kindred over four generations due to reciprocal translocation t(6;7)(q27;p13) and on a patient from this pedigree with a severe malformation syndrome due to duplication 7(p13----pter). The clinical findings are discussed as possible consequence of a gene mutation due to the break at 7p13.

[Cryptococcosis of the skin]. / Hautkryptokokkose.

In a woman patient who had received a kidney transplant 9 years previously, we diagnosed a cryptococcosis of the skin on the right arm. The results of clinical and mycological investigations and the forms of treatment applied are discussed.

Selective decontamination of the digestive tract and fungal infection in acute leukemia patients.

For prevention of infection we used an SD design including antibacterial (trimethoprim 480 mg/daily, sulfamerazine 720 mg/daily, and polymyxin 0.25 mg/daily) and antifungal (4-6 million IU nystatin/daily) components. We analyzed retrospectively 138 treatment periods in 108 patients. The intensified chemotherapy resulted in severe granulocytopenia below 0.1 x 10(9)/liter over 25.2 days. In 19 patients there was suspicion of major fungal infection; therefore they were given amphotericin B and 5-fluocytosine. Fourteen of them died; major fungal infections were documented in 5 cases. In 18% of all the deceased we found major fungal infections. There was a correlation between fungal infection, the late stages of the hematological malignancy, and the lesions on the oropharyngeal mucosa. However, in terms of the serological and culture findings no correlation appeared to exist between the group with and the group without fungal infection. The SD regime is meant to suppress the Candida cell concentration in the digestive tract but has no influence on Aspergillus in the respiratory tract.

Isochromosome (18q) in siblings.

A report is presented on a familial occurrence of isochromosome (18q) in a newborn infant and in a fetus in the 24th week of gestation after amniocentesis.

[Morphology of IgA nephritis (Berger disease). Immunohistologic, light- and electron microscopic findings]. / Morphologie der IgA-Nephritis (Morbus Berger). Immunohistologische, licht- und elektronenmikroskopische Befunde.

An IgA nephritis was diagnosed in 109 (24%) out of 445 renal biopsies with glomerular disease in the years from 1977 to 1985. The mean age of the patients in question was 29 years. The male:female sex ratio was 2.6:1. Immunohistologically, the characteristic branching mesangial IgA deposits were uniformly present. In addition 96% showed mesangial C3 and 54% mesangial IgG and/or IgM deposits. Besides, immunoglobulin and/or C3 deposits could be detected at glomerular basement membranes (20%). The histological types of IgA nephritis were minor glomerular abnormalities (10%), focal/segmental glomerulonephritis (29%), diffuse mesangioproliferative (58%), membranoproliferative (1 case), and sclerosing glomerulonephritis (2 cases). Additional tubulointerstitial lesions were found in 55%. Electron microscopically, in 53 cases under study, mesangial electron dense immunodeposits were seen. At the glomerular basement membranes, deposits could be found only in 23 examined cases (43%). The comparison of clinical data with morphological findings showed relationships between the degree of proteinuria and creatinine level increase with the histological type of IgA nephritis and with the degree of glomerular sclerosis, tubulointerstitial lesions, and electron microscopically proved glomerular basement membrane deposits. Hematuria seemed not to correlate with the morphological picture.

Fungal infections in acute leukemia patients during selective decontamination of the digestive tract--a clinical, laboratory and pathological study.

Selective decontamination (SD) is used for prevention of bacterial as well as fungal infection in acute leukemia patients during remission induction therapy. We analyzed 138 treatment periods of 108 patients. The intensified chemotherapy resulted in severe granulocytopenia below 0.1 X 10(9) l for over 25.2 days. In 19 patients there was a suspect of major fungal infection, for which they were given antimycotics (in 15 cases amphotericin B and 5-fluocytosin). Fourteen of them died. Major fungal infections were documented in 5 cases. In 18% of the deceased we found major fungal infection (7 cases of Candida sp., 5 cases of Aspergillus, one case of Candida as well as mucor). There was a correlation between fungal infection, the late stages of the haematological malignancy and the lesions appearing on the oropharyngeal mucosa. However, no correlation appeared to exist in the serological and culture findings between the groups with and without fungal infection. The SD-regime is meant to suppress the Candida cell concentration in the digestive tract but has no influence on Aspergillus in the respiratory tract. The clinical occurrence of major fungal infections published in the E.O.R.T.C.--Gnotobiotic Project Group was 8.2%. According to the literature there is in the eighties a tendency for an increased incidence of aspergillosis. The use of the SD-regime as prophylaxis as well as early antifungal therapy appear to be of great advantage in reducing the frequency of fungal infections in immunocompromised patients.