Gigantism: microsurgical treatment by transsphenoidal approach and prognostic factors.

PURPOSE: We present the results of transsphenoidal microsurgical treatment in 14 patients with gigantism. The influence on the prognosis of factors such as the tumor size and preoperative levels of GH and IGF-1 is also quantified. MATERIALS AND METHODS: The patients, operated between 1982 and 2004, were reviewed retrospectively in June 2022. All patients had complete endocrinological studies in the preoperative period and a postoperative control between 6 days and 3 weeks. Follow-up has been supported with annual check-ups between 3 and 31 years. We have compared the preoperative levels of GH and IGF-1 of these patients with the levels of a series of acromegalic patients operated on in the same Center. RESULTS: In this series there were 4 women and 10 men. The age ranged between 14 and 21 years. In 6 patients, postoperative hormone levels achieved the disease control criteria (42.8%). The CT/MRI studies revealed the existence of invasive tumors in 10 of the patients (71.4%). Postoperative CT/MRI showed no tumor tissue in 3 patients but in 7 patients there were tumor remains. The remaining 4 patients had abnormal images although not considered as tumor. A statistical comparison of preoperative serum GH and IGF-1 levels in patients with gigantism and patients with acromegaly showed a significant elevation in the former. CONCLUSION: Pituitary adenomas that cause gigantism are generally large and invasive, which makes them difficult to cure. High preoperative levels of GH and IGF-1 are also factors that decrease remission.

Acromegaly: surgical results in 548 patients.

PURPOSE: The goal of this study was to quantified the results of microsurgery, in all the patients with acromegaly treated by the same endocrinologist and the same surgeon between 1975 and 2015. METHODS: A series of 548 patients with acromegaly were operated and followed-up from 6 months to 40 years. Patients were selected according to five criteria: (1) Operated by the same surgeon. (2) No previous treatment. (3) Complete endocrinological preoperative studies including GH, OGTT, IGF-I, PRL test and TC/MRI. (4) Complete postoperative endocrinological evaluation for at least one determination of GH, OGTT, PRL test and IGF-I six months after surgery. (5) All the patients were supervised by the same endocrinologist. RESULTS: Microadenomas were present in 119 patients and 109 (91,5%) achieved remission. Non invasive macroadenomas were present in 200 patients and 164 achieved remission (82%). Results were worse for invasive macroadenomas but even with great invasions some patients achieved clinical remission. Follow-up range from 6 months to 40 years (mean 3.3 ± 2.3) A long term follow-up of 15 years was achieved in 61 patients. Four of them had a recurrence 4, 7, 8, 12 years after surgery (6.5%). There was not mortality and the rate of complications was low. CONCLUSIONS: Surgery remains the first line of therapy for a majority of acromegalic patients. This series proves to be very valuable in circumscribed adenomas but also in invasive tumours. Levels of GH and IGF-I were decreased in almost all the patients without remission.

Neurocognitive long-term impact of two-field conventional radiotherapy in adult patients with operated pituitary adenomas.

PURPOSE: To assess the long-term impact of postoperative two-field-conventional radiotherapy (RT) on neurocognitive functions of adult patients with operated pituitary adenomas (PA). METHODS: We selected 124 adult patients with operated PA-56 of whom had also received RT-recorded their main clinical data and performed a neuropsychological assessment in all of them that included 15 standardized tests, and a cerebral SPECT in eight patients. Comparative analyses were carried out on major clinical and neurocognitive domains between irradiated and not irradiated patients, and on cerebral SPECT source. RESULTS: Compared with non-irradiated patients, irradiated patients performed significantly worse on Barcelona's story recall test (P < 0.001) and arithmetic problems (P < 0.03) and on five categories of the Wisconsin card sorting test, especially on perseverative answers and errors (P < 0.001) without differences in other examined functional domains. RT was the only factor associated with worse results in these tests regardless other clinical and treatment-related variables. Kaplan-Meier analysis suggested that the probability of achieving poorer results with time was related to RT total dose and field-size, type of PA and age at the time of RT. Four of the five SPECTS performed in irradiated patients revealed a similar altered perfusion in the left temporal lobe cortical region. CONCLUSIONS: In adult patients with operated PA, RT was independently associated with an impairment on verbal memory and executive function, when compared to non-irradiated patients. Our data suggest that diagnosis of acromegaly or Cushing's disease, and age at the time of RT were able to modulate this long-term radio-induced neurocognitive sequelae.

Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up / Resultados de la reintervención quirúrgica en la enfermedad de Cushing. Seguimiento a largo plazo

OBJECTIVE: Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. PATIENTS AND METHODS: Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. RESULTS: Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. CONCLUSIONS: When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors

OBJETIVO: La cirugía transesfenoidal (TE) es el tratamiento de elección en primera línea en la enfermedad de Cushing (EC). Sin embargo, se desconoce cuál es el tratamiento más adecuado cuando el hipercortisolismo persiste o recidiva. El objetivo del estudio es analizar el resultado a corto y largo plazo de la reintervención TE e identificar factores predictores de respuesta. PACIENTES Y MÉTODOS: Se revisaron retrospectivamente los datos de 26 pacientes con hipercortisolismo persistente (n = 11) o recidivado (n = 15) reintervenidos por un mismo cirujano entre 1982 y 2009. Se consideró remisión a la normalización del cortisol libre urinario (CLU) y recidiva a la presencia de CLU elevado después de una remisión. Como potenciales predictores de respuesta se analizaron los siguientes factores: función adrenal tras la cirugía inicial (persistencia o recidiva), visibilidad del tumor en las pruebas de imagen, grado de hipercortisolismo antes de la reintervención, mismo/diferente cirujano en ambas cirugías y tiempo hasta la reintervención. RESULTADOS: Doce pacientes remitieron inmediatamente tras la reintervención (46,2%). De los 10 con seguimiento a largo plazo recidivaron 5 (mediana de tiempo hasta la recidiva: 13 meses). Se indujeron nuevos déficits hormonales en 7 pacientes (37%) y fístula de líquido cefalorraquídeo en 2. No se observaron otras complicaciones. Ninguno de los factores estudiados se asoció con la respuesta. CONCLUSIONES: Comparada con la cirugía inicial, la reintervención TE en la EC se asocia con una menor tasa de remisión y un riesgo mayor de recidivas y complicaciones. Son necesarios más estudios para definir factores predictores de respuesta

Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up.

OBJECTIVE: Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. PATIENTS AND METHODS: Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. RESULTS: Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. CONCLUSIONS: When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors.

Chondromyxoid fibroma of the lumbar spine: case report and literature review.

PURPOSE: Chondromyxoid fibroma (CMF) is a benign tumour of the bone that typically occurs in long bone metaphysis. Spinal involvement is uncommon, but more frequent in the cervical and thoracic segments. Lumbar involvement is extremely rare. We report the ninth case of lumbar CMF and the first one involving the articular process of the vertebra. A review of the literature is also intended making special emphasis on the differential diagnosis with other benign spinal tumours of the bone. METHODS: A 21-year-old Caucasian male suffering from low back pain that increased with sports and interrupted sleep was diagnosed with a tumoural lesion in the right inferior articular process of L5. RESULTS: Complete surgical excision of the tumour was accomplished. Histological diagnosis confirmed a CMF. The patient remains asymptomatic at 1-year follow-up. CONCLUSION: Despite the low incidence of CMF in the lumbar spine, differential diagnosis must include this subtype of lesion among other benign tumours of the bone and cartilage. Histological diagnosis is essential in order to provide the patient with an accurate management of the pathology. Recurrence rate is to be considered even in the case of complete surgical excision. Radiotherapy administration is controversial due to suspicion of malignant transformation of the tumour.

Malignant prolactinoma with multiple bone and pulmonary metastases. Case report.

Malignant prolactinomas, as with other pituitary carcinomas, are rare tumors. The authors describe a 14-year-old boy who presented with visual loss caused by a pituitary prolactinoma. He underwent transsphenoidal surgery, radiotherapy, and dopamine agonist therapy, but 6 years after the initial diagnosis his pituitary tumor regrew and bone and pulmonary metastases developed. The authors review the literature and discuss the clinical presentation of malignant prolactinomas, their rarity, histological characteristics, distribution of the metastases, different treatment approaches, and their poor prognosis.