Giant Non-Functioning Pituitary Adenoma: Clinical Characteristics and Therapeutic Outcomes.

BACKGROUND: Giant pituitary adenoma (≥4 cm) is a rare tumor whose clinical features and prognosis are not well known. AIM: To evaluate the clinical characteristics and therapeutic outcomes of giant non-functioning PA (gNFPA). PATIENTS AND METHODS: A retrospective multicenter study of gNFPA patients diagnosed in a 12-year period was performed. In each patient, clinical data and therapeutic outcomes were registered. RESULTS: Forty patients (24 men, age 54.2 ± 16.2 years) were studied. The maximum tumor diameter [median (interquartile range)] was 4.6 cm (4.1-5.1). Women had larger tumors [4.8 cm (4.2-5.4) vs. 4.5 cm (4.0-4.9); p=0.048]. Hypopituitarism [partial (n=22, 55%) or complete (n=9, 22.5%)] at diagnosis was present in 77.5% of the patients. Visual field defects were found in 90.9%. The most used surgical technique was endoscopic endonasal transsphenoidal (EET) surgery (n=31, 77.5%). Radiotherapy was used in 11 (27.5%) patients (median dose 50.4 Gy, range 50-54). Thirty-seven patients were followed for 36 months (10-67 months). Although more than half of these patients showed tumor persistence (n=25, 67.6%), tumor size was significantly reduced [0.8 cm (0-2.5); p<0.001]. At last visit, 12 patients (32.4%) showed absence of tumor on MRI. Hypopituitarism rate was similar (75.0%), although with significant changes (p<0.001) in the distribution of the type of hypopituitarism. The absence of tumor at the last visit was positively associated with positive immunohistochemical staining for FSH (p=0.01) and LH (p=0.006) and negatively with female sex (p=0.011), cavernous sinus invasion (p=0.005) and the presence of Knosp grade 4 (p=0.013). CONCLUSION: gNFPAs are more frequent in men but tumors are larger in women. Surgical treatment is followed by a complete tumor resection rate of approximately 30%. Positive immunostaining for gonadotropins is associated with tumor absence at last revision, while female sex and invasion of the cavernous sinuses with tumor persistence.

Giant Prolactinoma in Men: Clinical Features and Therapeutic Outcomes.

The aim of the study was to evaluate the clinical features and long-term therapeutic outcome of giant prolactinoma (gPRLoma) in men and to compare them with those of a group of male patients with non-gPRL macroprolactinomas (non-gPRLomas). A retrospective and multicenter study of gPRLomas in men diagnosed in a 20-year period was performed. Clinical data and treatment outcome were registered. The diagnosis of gPRLoma was established when the maximal tumor diameter was ≥40 mm or the tumor had ≥20 mm of suprasellar extension associated to hyperprolactinemia (PRL>1000 ng/ml). Non-gPRLoma was considered when tumor diameter was ≥ 10 mm and<40 mm associated to hyperprolactinemia (PRL≥200 ng/ml). Twenty-three patients with gPRLoma (age 38.3±13.5 years) followed for at least 3 months (follow-up 87.1±60.5 months, range 3-211 months) were evaluated. A group of 42 patients with non-gPRLoma (age 42±16.6 years; NS; follow-up 89±65.9 months, range 3-222 months; NS) served as a control group. More than half (56.5%) of the gPRLoma patients were younger than 40 years at diagnosis. Visual disturbances were significantly more common in gPRLoma than in non-gPRLoma patients (65.2 vs. 25.6%; p=0.004). Prevalence of hypopituitarism was similar in both groups of patients (73.9% vs. 80.9%; gPRLoma vs non-gPRLoma; NS). Serum PRL concentrations were significantly higher in gPRLoma than in non-gPRLoma patients [median (IR), 3978 ng/ml (1179-9012) vs. 907 ng/ml (428-3119); p<0.001]. Maximum tumor diameter in gPRLomas was 4.8±0.8 cm and 2.4±0.7 cm in non-gPRLoma (p<0.001). All patients were treated with dopamine agonists (DA). Twelve (52.2%) gPRLoma patients and 32 (73.8%) non-gPRLoma patients were treated with DA as monotherapy (p=0.045). Surgery was used in 12 (52.2%) gPRLoma patients and in 12 (28.6%) non-gPRLoma patients (p=0.054). Lastly, radiotherapy was used in 5 (21.7%) gPRLoma patients and in 6 (14.2%) non-gPRLoma patients (NS). At last visit, PRL was similar in both groups of patients [16 ng/ml (4-30) vs. 11 ng/ml (4-25); gPRLomas vs. non-gPRLomas; ns] and tumor size decreased significantly (p<0.001) in both groups of patients. Clinical cure (maintained normoprolactinemia without therapy for>1 year and no radiological evidence of pituitary tumor) was achieved in 2 (8.7%) gPRLoma patients and in 2 (4.8%) non-gPRLoma patients (NS). gPRLomas in men are usually diagnosed at a mean age of 40 years, an age similar to that of non-gPRLomas. The only clinical difference with non-gPRLomas is their greater prevalence of visual disturbances. The therapeutic approaches and tumor outcomes were similar to those obtained in patients with non-gPRLomas. Complete cure in gPRLoma is rare, but similar to that achieved in non-gPRLomas, reached in less than 10% of patients.

Prevalence, Clinical Features, and Natural History of Incidental Clinically Non-Functioning Pituitary Adenomas.

The objective of the study was to assess the prevalence and clinical features of incidentally discovered clinically non-functioning pituitary adenoma (CNFPA) and to analyze its natural history. A multicenter retrospective study in patients diagnosed with incidental CNFPA periodically followed-up in 3 specialized neuroendocrinology units from 1992 to 2015 was performed. Out of a total of 189 CNFPA patients, 57 cases (30.1%; 29 women; age at diagnosis 55.8±16.7 years) were incidental. Most patients (n=55, 96.5%) were diagnosed by magnetic resonance imaging (MRI). A sum of 71.9% (n=41) were macroadenomas; 2 of them (3.5%) were giant adenomas (≥4 cm). Patients with macroadenomas were older than those with microadenomas (59.5±16.7 vs. 46.4±18.1 years, p=0.007). Macroadenomas were more common in men (85.7% vs. 58.6%, p=0.023). Twenty-eight patients (49.1%) showed suprasellar extension; of these, 19 were accompanied by chiasmatic compression. Hypopituitarism was present in 14 (24.6%) patients; which was partial in 13 patients (22.8%) and complete in one patient (1.8%). The gonadal axis was the most frequently affected (n=13, 22.8%). Twenty four patients (42.1%) underwent surgery. Of the non-operated patients, 26 patients could be evaluated morphologically after a median follow-up of 15.5 months (interquartile range, 5.7-32.7 months). No significant changes were found in the maximum tumor diameter at the end of follow-up (1.2±0.6 vs 1.2±0.7 cm). The majority of CNFPAs evaluated (n=23, 88.5%) did not show any changes in size. In 2 cases (7.7%) tumor size decreased, and in one patient (3.8%) increased. In our series of CNFPA patients, approximately one-third were incidental. These tumors were diagnosed by MRI preferably from the 5th decade of life without sex predilection. Most of them were macroadenomas, more commonly diagnosed in men and at an older age, compared to microadenomas. The suprasellar extension with chiasmatic compression and hypopituitarism were frequent at diagnosis. Most of the non-operated incidental CNFPAs remain with stable tumor size over time, growth being an unusual event.

Non-functioning pituitary adenoma underwent surgery: A multicenter retrospective study over the last four decades (1977-2015).

OBJECTIVE: To assess clinical features, diagnostic procedures, therapies and outcomes in patients with clinically non-functioning pituitary adenomas (NFPAs) surgically treated over the last four decades. DESIGN AND METHODS: A multicenter retrospective study in NFPA patients periodically followed up in specialized neuroendocrinology units who underwent surgery in the period 1977-2015 was performed. RESULTS: A total of 131 patients were studied [66 women (50.4%); mean age 52.6±14.8years (range, 15-82)]. Median diameter of the adenoma was 2.6cm (interquartile range, 2.0-3.1). The most frequently type of surgery used was endoscopic endonasal surgery (58.5%) followed by microscopic transsphenoidal surgery (37.4%). Radiation therapy was used in 19 patients (14.5%). Ki-67 labeling index performed in 54 patients was ≤2% in 70% samples. After a median follow-up time of 57months (25 to 128months), tumor diameter significantly decreased to 0.9cm (0-1.8cm), p<0.001. Multivariant analysis showed that endoscopic endonasal surgery (HR 2.74, 1.06-6.87, p=0.036) and radiotherapy (HR 0.04, 0.02-0.65, p=0.024) behaved as positive and negative predictors, respectively, of tumor absence in the follow-up. Endoscopic endonasal surgery (HR 6.71, 1.45-31.05, p=0.015) was the only positive predictor for complete cure in the follow-up. CONCLUSION: NFPAs surgically treated in Spain are usually macroadenomas diagnosed around the sixth decade of life with no sex predilection. Type of surgery is associated with clinical outcome. Endoscopic endonasal surgery behaves as a positive predictor for the absence of tumor imaging and complete cure in the follow up.

Efectiveness of long-term home parenteral nutrition with peripherally inserted central catheter: a case report / Eficacia de la nutrición parenteral domiciliaria de larga evolución con catéter de acceso periférico: a propósito de un caso

The use of home parenteral nutrition (HPN) in patients who can not obtain their nutritional requirements by the enteral route is increasing in recent years, allowing normalization lifestyle of patients. Neoplasm and mesenteric ischaemia are some of the diseases that most frequently require HPN in Spain. However, HPN is one of the cornerstones of the treatment of much less frequent illnesses as in the case of encapsulating peritoneal sclerosis. We present the case of a patient with encapsulating peritoneal sclerosis and HPN support for more than 7 years with a peripherally inserted central catheter (PICC) for over 6 years without complications and the autonomy to perform his normal business activity. Given the exceptional nature of the case we refer it to its publication.

El uso de la nutrición parenteral domiciliaria (NPD) en pacientes que no pueden alcanzar sus requerimientos nutricionales por la vía enteral está aumentando en los últimos años, permitiendo la normalización del estilo de vida de los pacientes. Entre las patologías que más frecuentemente precisan de la NPD en España destacan la neoplasia y la isquemia mesentérica. Sin embargo, la NPD constituye uno de los pilares básicos del tratamiento de enfermedades mucho menos frecuentes como es el caso de la esclerosis peritoneal encapsulante. A continuación presentamos el caso de un paciente diagnosticado de esclerosis peritoneal encapsulante con soporte NPD de más de 7 años de NPD con un catéter central de inserción periférica (PICC) para la NPD sin complicaciones y pudiendo realizar su actividad laboral habitual. Dado lo excepcional del caso lo remitimos para su publicación.

Eficacia de la nutrición parenteral domiciliaria de larga evolución con catéter de acceso periférico: a propósito de un caso / Effectiveness of long-term home parenteral nutrition with peripherally inserted central catheter: a case report

El uso de la nutrición parenteral domiciliaria (NPD) en pacientes que no pueden alcanzar sus requerimientos nutricionales por la vía enteral está aumentando en los últimos años, permitiendo la normalización del estilo de vida de los pacientes. Entre las patologías que más frecuentemente precisan de la NPD en España destacan la neoplasia y la isquemia mesentérica. Sin embargo, la NPD constituye uno de los pilares básicos del tratamiento de enfermedades mucho menos frecuentes como es el caso de la esclerosis peritoneal encapsulante. A continuación presentamos el caso de un paciente diagnosticado de esclerosis peritoneal encapsulante con soporte NPD de más de 7 años de NPD con un catéter central de inserción periférica (PICC) para la NPD sin complicaciones y pudiendo realizar su actividad laboral habitual. Dado lo excepcional del caso lo remitimos para su publicación (AU)

The use of home parenteral nutrition (HPN) in patients who can not obtain their nutritional requirements by the enteral route is increasing in recent years, allowing normalization lifestyle of patients. Neoplasm and mesenteric ischaemia are some of the diseases that most frequently require HPN in Spain. However, HPN is one of the cornerstones of the treatment of much less frequent illnesses as in the case of encapsulating peritoneal sclerosis. We present the case of a patient with encapsulating peritoneal sclerosis and HPN support for more than 7 years with a peripherally inserted central catheter (PICC) for over 6 years without complications and the autonomy to perform his normal business activity. Given the exceptional nature of the case we refer it to its publication (AU)