Pituitary germ cell tumor with pancytopenia accompanied by gelatinous degeneration of bone marrow

Abstract

A previously healthy 14-year-old girl complained of headache, appetite loss and depression. She had lost 36% (13.5 kg) of her weight over six months owing to low food intake and frequent vomiting. CT revealed an obstructive hydrocephalus due to a 6 cm mass occupying the third ventricle. She was diagnosed as having pituitary germ cell tumor (germinoma with STGC) on the basis of surgical biopsy results and serum marker levels. Her condition was complicated by panhypopituitarism, diabetes insipidus, anemia (Hb 9.0 g/dL), and thrombocytopenia (Plt count, 58,000/μL). Bone marrow examination revealed marked hypoplasia accompanied by gelatinous degeneration. We performed reduced dose chemotherapy and proton beam therapy targeting the whole ventricle and tumor bed, excluding the craniospinal region. The gelatinous degeneration of the bone marrow was resolved by sufficient nutritional control, hormone replacement therapy, and treatment of the brain tumor. Her blood cell count also normalized subsequently. Most reported cases of gelatinous bone marrow degeneration are found among patients with anorexia nervosa. Not only significant malnutrition but panhypopituitarism may have contributed to her condition.