Endocrine Abstracts

Introduction: Schwannomas are tumors derived from myelin sheat of nerves which can displace and compress nerves causing pain, weakness and numbness. Very rarely they become malignant.

Case report: Thirty-one years old patient was hospitalized at our Department to evaluate hypopituitarism. He was born at term by cesarean section, with hydrocephalus and did not start to breath spontaneously. A few months later, he was diagnosed with scoliosis thoracalis duplex and ostium secundum atrial septal defect. Hydrocephalus spontaneously resolved at the age of four and at the age of five he was diagnosed with hypopituitarism – GH deficiency, hypogonadotropic hypodonadism and hypothyroidism. Endorinological testing showed adequate cortisol response in ACTH test and insulin tolerance test. At the age of nine due to congenital spinal curves he underwent a surgery but significant spine gibus remained. At ten, he was diagnosed with Asthma bronchiale and congenital heart defect was surgically treated. From 11th to 16th year of age while on GH replacement therapy he grew from 92 to 154 cm. At the age of 12, a CT scan of the head was performed: Empty sella and a CT scan of the abdomen: agenesis of the right kidney. At the age of 27, due to a constant pain in his left shoulder and MRI was performed. It showed a tumefaction of 16×10×8.5 cm, located predominantly in the musculus infraspinatus and musculus teres minor and enlarged regional lymph nodes. The surgery was performed and pathohistological diagnosis was schwanoma malignum, low grade, with zones of myxoid transformation. Immunohistochemistry was performed in search for androgen receptors. The result was negative and his regular androgen therapy was continued. He has regular check ups at the Orthopedic Clinic with no signs of relapse. He takes the advised hormonal subsitution therapy and in general feels well.

Conclusion: Malignant schwannomas are very rare and are usually associated with neurofibromatosis type I. According to the available literature our patient is the first with the occurance of malignant schwanomma with hypopituitarism.