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Congenital Neurofibromatosis; Multiple Subcutaneous Tumors With Spontaneous Regression In Twins

Eeg-Olofsson & Lindskog, 1983Other/Unknown

Acta Paediatrica Scandinavica 72(5): Sep 1983: 779-780

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Abstract

Paragangliomas are rare neoplasms, most of which are benign, but malignant paragangliomas are occasionally encountered. The authors report a case of spontaneous remission of biopsy-proven metastatic pulmonary lesions from a malignant paraganglioma arising in the neck. We have no explanation for the regression of disease in our patient. We consider the case interesting because of the histologic confirmation of the metastatic lesions in the lung and because the regression began five months after chemotherapy had been stopped because of lack of response.

Case Details

Personal Characteristics

Second born male identical twin, weighed 2,430 grams at birth, no cafe-au-lait spots at birth but developed one on the abdominal wall, hyperactive behaviour

Clinical Characteristics

Multiple subcutaneous tumors at birth

Remission Characteristics

The subcutaneous tumors completely disappeared by the age of seven months

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

Removal of one tumor

Additional Notes

No family history of neurofibromatosis