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Five extracardiac rhabdomyomas were studied by light and electron microscopy: two of the adult type, two of the fetal type, and one of the female genital tract type. The three types can be distinguished histologically and ultrastructurally. Adult type rhabdomyomas are encapsulated and reveal histologically closely packed, large tumor cells with granular or vacuolated cytoplasm and only scant stroma. Electron microscopically, myofibrils are haphazardly arranged with rodlike Z-band material. Sometimes a sarcomeric arrangement of myofibrils is seen. The tumor cells are packed with mitochondria bearing lamellar inclusions. Fetal type rhabdomyomas contain a mixture of muscle cells in different stages of differentiations; the amount of stroma is increased. The myofilaments are usually disorganized and in only a few cells is rodlike Z-band material found. The cytoplasm reveals few mitochondria. In the rhabdomyoma of the female genital tract, the tumor cells are large and resemble more mature muscle fibers; the stroma is abundant and the myofilaments are usually arranged in an orderly fashion. Generally, the prognosis for rhabdomyomas is good. Metastases are not known. Two of the five rhabdomyomas studied recurred. One rhabdomyoma was found at autopsy. Embryonal rhabdomyosarcoma must be considered in differential diagnosis particularly for fetal type rhabdomyoma.

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(29. 111. 45.) (113. Mitteilung')).Allgemeines. I n zwei friiheren M i t t e i l~n g e n~)~) haben wir uber die Isolierung von zwei epimeren d 16-Androsten-olen-(3) am Schweinetestes-Bxtrakten und fiber ihre partialsynthetische Herstellung berichtet. Beide Verbindungen zeichnen sich durch einen charakteristischen Geruch, den wir als ,,moschusahnlich" beschrieben haben, aus. Einen ahnlichen Geruch besitzen auch die Dihydro-Derivate dieser ungesattigten Alkohole, die epimeren Androstan-ole-(3). Das entsprechende ungesattigte Keton, das d 16-Androstenon-on-( 3 ) zeigt dagegen einen anderen, ebenfalls charakteristischen Geruch, den wir meiterhin kurz als ,,Harngeruch" bezeichnen wollen4)).Die Tatsache, dass gewisse Steroide mit einer Sauerstoff-Funktion einen recht intensiven Geruch besitzen, war iiberraschend. Wir haben deshalb eine Reihe iihnlicher Verbindungen hergestellt, urn festzustellen, durch welche konstitutiven Merkmale der Geruch in der Steroid-Reihe bedingt ist. Von vornherein war nicht zu erwarten, dass sehr viele Steroide Riechstoffeigenschaften besitzen, da bis vor kurzem keine von den bekannten Verbindungen aus dieser Gruppe durch ihren Geruch aufgefallen war.Tatsachlich konnten wir den ,,moschusahnlichen" Geruch bisher bei keiner der neu hergestellten Verbindungen wiederfinden. Schon eine Umkehrung der Konfiguration am Kohlenstoffatom 5 fiihrte von den beiden epimeren A 16-Androsten-olen-(3) zu den praktisch geruchlosen epimeren d 16-Atio-cholen-olen-(3) (V und VI). Die DihydroDerivate dieser Verbindungen, die Atio-cholan-ole-( 3) (VIII und I X ) sind ebenfalls ohne Geruch. Dagegen konnte der , , H a r n g e r u c h " des ~I~~--Anllrosten-ons-(3) b e i m e h r e r e n d e r n e u u n t e r s u c h t e n S t e r o i d e f e s t g e s t e l l t werden. So zeigten das Androstan-on-(3) (X) und das d*J6-Androstadien-on-(3) (111) denselben Geruch wie das friiher beschriebene d 16-Androsten-on-(3). Ein besonderer Einfluss der Doppelbindung in l) 112. Mitt. Helv. 21, 389 (1945).

Background. Wilms' tumor is rare in adults. The recommended treatments for patients with Stage II adult Wilms' tumor with favorable histology (FH) nephroblastomas are conflicting. Methods. Two patients with Stage II, favorable histology, adult nephroblastomas are described. Current treatment modalities are discussed and the literature is reviewed. Results. The first patient, a 52‐year‐old woman, probably had a late local relapse of a Wilms' tumor 21 years after nephrectomy because of a renal tumor originally diagnosed as reticular sarcoma. In this case, a recurring or an extrarenal Wilms' tumor should have been considered. After the tumor was removed, the patient received adjuvant chemotherapy with dactinomycin and vincristine and was disease free 44 months after diagnosis. The Wilms' tumor in the second patient, a 33‐year‐old woman, was discovered accidentally and classified as Stage II/FH based on preoperative biopsy. She was treated with radical nephrectomy and adjuvant chemotherapy with dactinomycin and vincristine. This patient was disease free 24 months after surgery. Conclusions. Surgery and two‐drug chemotherapy with dactinomycin and vincristine is suggested for patients with Stage II adult Wilms' tumor with FH nephroblastomas. Cancer 1995;75:545‐51.

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A case of Wolman's disease is described in a German infant who died at the age of 4 months. Hepatosplenomegaly, abdominal distention, gastrointestinal symptoms, dyserythropoietic changes in the bone marrow, but not adrenal calcification on X-ray were present. Stored lipid material could be demonstrated in liver, spleen, intestine, adrenals, thymus, kidneys, blood cells, but not in the central nervous system. Cholesterylesters and triglycerides were markedly increased in liver and spleen. Lysosomal acid lipase was found to be decreased in leucocytes and liver to less than 10% of normal, when measured with synthetic and natural substrates.