Klinische und morphologische Aspekte zur Cortison-Therapie von Hämangiomen

Summary

Until now 36 haemangiomata without thrombocytopenia, treated with prednisone, have been reported by 8 authors. With a dosage of 2 to 3 mg prednisone per kilogram bodyweight (about 20 mg daily or 40 mg every second day) recession of the haemangiomata, or a stationary course, was observed in 50% of the patients within 10 to 40 days after the onset of this treatment. Recurrent growth was seen, but no serious complications, when the treatment was limited to 6 to 8 weeks. For a final evaluation of this procedure the number of cases is still too small, the type of the lesions too different, and the time of the follow-up studies too short.

Therefore two further cases are reported, which allow certain conclusions in respect of the indications, as well as the mechanism of the steroidtherapy. In case 1, an 8 week old infant (Figs. 1 and 2), a haemangioma had grown from a small lesion to a large pulsating mass which caused severe and already dangerous obstruction of the face and neck. 15 mg prednisolone (Deltacortril) daily were given. After 7 days there was a marked recession in the size of the tumor, which felt solid on the 10th day, and seemed perfectly thrombosed and confined to the parotid gland on the 15th day. The prednisolone-medication was slowly reduced. On the 25th day the skin was pale, the auditory canal open, and the rest of the haemangioma was palpable as a walnut-sized parotid tumour. Altogether 293.7 mg prednisolone were given. The child lost 400 gram in weight but no complications were observed. Within 4 days after recession of the medication the haemangioma started to grow and to pulsate again (Figs. 3 and 4). Therefore an immediate parotidectomy with preservation of the facial nerve was performed, and because of the involution of the haemangioma it was able to be executed as if under normal conditions.

The second case (Figs. 3 and 4) had a similar course. Recession of the haemangioma, after 4 weeks of treatment (243 mg prednisolone) appeared to be complete; the tumour appeared also thrombosized and of walnut-size. After the prednisolone-treatment was stopped, the haemangioma started to grow again. Therefore a total parotidectomy with preservation of the facial nerve was performed, as in case 1.

The histological examination showed the parotid acini nearly completely displaced by a typical haemangioma of a mixed type. In spite of a very intensive search no thrombosis could be seen, but there were in both cases special arteries with thick walls, narrow lumena and an intima which was padded out with epitheloid cells. The full picture suggests that these blocked anteries did not develop with the angioma, but were locked instead with the parotid with the lobes of the haemangioma. The course and the findings in these cases do not support the presumption of Zarem and Edgerton that the steroid therapy leads to a recession of the haemangioma by an increased fragility of the capillaries and the venules, and by spontaneous haemorrhage and thrombosis, because this mechanism would lead first to a primary enlargement, with discoloration, and then to a shrinkage of the tumour only after weeks. In our patients the recession occured after only a few days and there was no thrombotic obliteration and no difference in the morphological picture before and after steroid treatment. The anaemia producing effect of steroid compounds are well known, and even used for testing. A similar mechanism, a vasoconstriction of the arteries which are becoming blocked, as of the peripheral vessels, seem to be the most likely cause of recession of the haemangioma during steroid therapy.

Prednisolone seems indicated in dangerously enlarging haemangioma, e.g. in the parotid region, where a preoperative involution seems the inescapable condition for a safe resection of the tumour. Because the effect of prednisone is uncertain in small infants and is probably only temporary, and because of the possible cortisone-complications, this therapy should at present be restricted to babies with rapidly growing tumours which may endanger vital functions.

Zusammenfassung

Die Steroidtherapie kann bei bedrohlich wachsenden Hämangiomen hilfreich und zur Operationsvorbereitung sehr wirksam sein. Im Hinblick auf den fraglichen Dauereffekt und die zweifelhafte Wirkung bei älteren Kindern, sowie wegen der möglichen Nebenwirkungen, sollte diese Behandlung vorläufig auf exzessive Einzelfälle beschränkt werden.