Pure pineal germinomas: analysis of gender incidence.

OBJECTIVE: Pure pineal germinomas have been rarely reported in girls. Gender incidence and differences of pure pineal germinomas are not well known. The authors report a series of pure pineal germinoma and its gender characteristic is reviewed. METHODS AND RESULTS: Of a total of 50 germ cell tumors operated on between 1988 and 2004 we found 26 cases (median age at diagnosis, 12 years) of pineal germ cell tumors. Of these, 14 cases (male/female ratio: 13/1) were pure pineal germinomas, and 12 cases (male/female ratio: 12/0) were non-germinoma germ cell tumors. In pure pineal germinomas, the main clinical presentations were intracranial hypertension and cranial nerve dysfunction. Imaging studies disclosed a homogeneous type of tumor (n = 10) and associated hydrocephalus (n = 6). Cases were managed with biopsy and subsequent radiation therapy and chemotherapy. After a follow up of 10 years, pure germinoma cases have no neurological deficits and tumor recurrence. The literature on gender incidence of pure pineal germinomas is analyzed and possible causes are discussed. CONCLUSIONS: Although rare, pure pineal germinoma can be found in female subjects. On the basis of the literature review, the male/female ratio in cases of pure pineal germinoma is between 5:1 and 22:1 (mean 14:1). In our series, the male/female ratio was 13:1.

hCG-secreting pineal teratoma causing precocious puberty: report of two patients and review of the literature.

Two boys are described with precocious puberty (PP) due to pineal immature teratoma associated with choriocarcinoma. Patient 1 was a 7 year-old boy with a 2-year history of PP. He had elevated CSF and plasma beta-hCG levels. Magnetic resonance imaging (MRI) showed a 3.0 cm pineal mass. He was initially submitted to a trial with radiotherapy, followed by radical surgical resection, stereotactic radiotherapy and chemotherapy. Long-term follow up included the appearance of acute hydrocephalus requiring CSF shunting, local hemorrhage and extensive radionecrosis. Death occurred 1.5 years after diagnosis. Patient 2 was a 7 year-old boy with an 8-month history of PP. He had elevated CSF and plasma beta-hCG and alpha-fetoprotein levels. MRI showed a 1.0 cm pineal mass. He was submitted to radical surgical resection (which caused normalization of levels of markers) and prophylactic chemotherapy. The boy is doing well 1.5 years after diagnosis. An extensive review of the literature corroborates the idea that this last treatment paradigm (surgery and chemotherapy) probably represents the best treatment regimen for these patients.

El mareo como síntoma de los tumores encefálicos. Estudio de 60 casos / Dizziness as a symptom of encephalic tumors. A review of 60 cases

Se procedió a estudiar la incidencia de manifestaciones de mareos en una muestra de 60 casos de pacientes de ambos sexos, con edades entre 6 y 76 años, con tumores encefálicos de diversa localización e histopatología. La ubicación de los tumores se estableció por métodos radiológicos y se confirmaron tras la intervención quirúrgica. Se estudió especialmente la relación del síntoma mareo con la ubicación de los tumores encefálicos. Comprobamos que el mareo aparece asociado especialmente a los tumores de fosa posterior. En los casos de tumores localizados en hemisferios cerebrales el mareo se manifiesta con menor incidencia. Estos casos se ubican en áreas próximas a los centros vestibulares corticales

Endocrine disorders in 66 suprasellar and pineal tumors of patients with prepubertal and pubertal ages.

Tumor oncotypes, initial symptoms and endocrine disturbances before and/or 1 month after surgery were studied in 66 patients with prepubertal and pubertal ages having suprasellar or pineal intracranial tumors. Neoplasms found in patients of prepubertal age were: 15 craniopharyngiomas (CRA), 24 neuroepithelial-cell-derived tumors (NEC), 5 germ cell tumors (GERM) and 4 other lesions (OTHER). In patients of pubertal age, there were 7 CRA, 7 pituitary tumors (PIT), 2 NEC, 1 GERM and 1 OTHER. Approximately 90% of patients had visual abnormalities as one of the initial signs and symptoms, while 59% had increased intracranial pressure. Short stature was observed in only 10% of patients. Before surgery, somatotropic function was found to be deficient (by 2 pharmacological tests) in 90-100% of patients with CRA, PIT or GERM and in 40% of patients with NEC. Overt hypothyroidism was found in 5-25% of CRA, NEC or GERM but in 40% of PIT. Abnormal TSH responses to TRH were observed in 64% of CRA and in 29% of NEC. Low basal serum cortisol was found in 21 or 6% of patients with CRA or NEC, but in 100 or 60% of patients with PIT or GERM, respectively. Diabetes insipidus was diagnosed in 13.6% of all patients. Surgery produced few additional disturbances in endocrine function, except for the incidence of diabetes insipidus which was doubled. Gonadotropic deficiency was found in most patients of pubertal age with CRA and PIT. They were readily differentiated by the high prolactin or growth hormone (GH) levels of the latter.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnostic difficulties in a case of true precocious puberty

A case of true precocious puberty of cerebral origin is reported in a girl with hydrocephaly, calcification of the pineal region and previous pulmonary tuberculosis

Pubertad precoz, como única manifestación de un tumor intracraneano en un niño de siete años / Precocious puberty as the only manifestation of intracraneal tumon in a 7 year old boy

Se presente el primer caso de germinoma suprasellar en el Hospital de Niños "J.M. de Los Ríos". Masculino, 7 años de edad, con macrogenitosomía precoz y cambio de voz. La TAC mostró lesión de ocupación de espacio suprasellar, el gammagrama fue hipercaptante en la misma región. Los dosificación hormonal mostró elevación de LH y de gonadotropina coriónica, así como la testosterona, androstenodiona y dehidroepiandrosterona. El paciente fue irradiado y el TAC de control disminuyó la lesión. La pneumografía fue normal. Sigue bajo control de Endocrinología y se sugirió quimioterapia. El seguimiento total sido de 9 meses