Acromegaly and diabetes mellitus associated with hyperthyroidism.

A 66-year-old woman with acromegaly and diabetes mellitus as well as primary hyperthyroidism is described. Serum GH Levels were inappprpriately high.MRI revealed an enlarged sella turcica with intrasellar mass. Her HbAlc was 12.2% and fasting blood glucose 8.89 mmol/l. Thyroid hormone levels in serum and thyroidal radioiodine uptake values were elevated, while TSH measurements in serum were low. Anti TPO antibodies were negative, TSH receptor antibodies were normal. Thyrotoxicosis as the first presenting illness in acromegaly was particulary uncommon. An ultrasound thyroid scan showed a multinodular goiter. Histology of the pituitary lesion showed a typical eosinophilic adenoma which only secreted GH when tested with specific immunostain. Post-operatively, the patient's clinical conditions improved, however, secondary hypoadrenalism appeared.

Outcomes of endoscopic papillectomy in elderly patients with ampullary adenoma or early carcinoma.

Outcomes for 14 elderly (age ≥ 70 years) patients (79.4 ± 1.0 years) who underwent endoscopic papillectomy for ampullary tumors were compared with those of 22 younger (age < 70 years) patients (52.5 ± 1.9 years). There were no differences in procedural success (100%), bleeding (5/14 vs. 6/22), adenoma recurrence (0/14 vs. 2/22) and median survival (24.0 vs. 25.5 months) between the elderly and younger patients. In younger patients, although adenoma recurrences (n=2) were managed endoscopically, invasive adenocarcinomas (n=3) were treated by pancreatoduodenectomy. All elderly patients with invasive (T2) tumors (n=5) were not surgically fit and underwent successful palliation with further endoscopic resection and stenting. Whilst all younger patients survived, five elderly patients died but three of these deaths were not cancer-related. Advanced age, therefore, did not adversely influence the outcomes of endoscopic papillectomy, suggesting it may be a treatment of choice for elderly patients with ampullary tumors or early cancer who are deemed unfit for surgery.

Apoplexy accompanying pituitary adenoma as a complication of preoperative anterior pituitary function tests.

Pituitary apoplexy occurs as a very rare complication of the pituitary function test. We have experienced two cases of pituitary apoplexy following anterior pituitary function tests for preoperative assessment: a triple bolus test and a TRH test. To elucidate such a rare complication, we outline our two cases and review 28 cases from the literature. The clinical characteristics, etiology, pathophysiology, and diagnostic and therapeutic implications are also discussed. The combined data suggest that pituitary function tests have the potential to precipitate pituitary apoplexy, and its manifestations range from a clinically benign event to a catastrophic presentation with permanent neurological deficits or even death, although most patients may fortunately have a good outcome. We suggest that the pituitary function test should not be done as a routine test, and when such a test is planned, the patient should be observed with caution for any symptomatic changes for at least 2 hours following the test for appropriate treatment. Further, MRI, especially enhanced studies, may provide an earlier diagnosis of the pituitary apoplexy since CT scan images often fail to demonstrate either density changes or obvious enlargement of the pituitary adenoma at the acute stage.

Ectopic growth hormone-releasing adenoma in the cavernous sinus--case report.

A 55-year-old woman presented a rare ectopic pituitary adenoma in the right cavernous sinus manifesting as acromegaly. The tumor was removed via transsphenoidal approach. Intraoperative observation showed the adenoma was located entirely within the right cavernous sinus, and separated from the normal pituitary gland by the medial wall of the cavernous sinus. There was no communication between the tumor and the pituitary. Histological examination showed a growth hormone-releasing adenoma. Including our case, only eight of 86 reported ectopic adenomas have occurred in the cavernous sinus. Such ectopic presentation may be responsible for failed transsphenoidal surgery for endocrinologically active tumors.

Blastoma adrenal en adulto / Adrenal blastoma in an adult

El blastoma adrenal es una neoplasia excepcional de la que hay una única publicación en un niño de 21 meses. Se presenta un caso similar desarrollado en un varón de 68 años. Se revisan los criterios anatomopatológicos e inmunohistoquímicos y el diagnóstico diferencial. En conclusión, el blastoma adrenal no es un tumor exclusivo de los niños (AU)

Long-acting peptidomimergic control of gigantism caused by pituitary acidophilic stem cell adenoma.

Gigantism is caused by GH hypersecretion occurring before epiphyseal long bone closure and usually is associated with pituitary adenoma. A 15-yr-old female patient presented with accelerated growth due to a large pituitary tumor that was surgically resected to relieve pressure effects. Second surgery to remove residual tumor tissue was followed by administration of octreotide LAR, a long-acting depot somatostatin analog, together with long-acting cabergoline. Height was over the 95th percentile, with evidence of a recent growth spurt. Serum GH levels were more than 60 ng/mL (normal, <10 ng/mL) with no suppression to 75 g oral glucose, and serum PRL (>8,000 ng/mL; normal, <23 ng/mL) and insulin-like growth factor I levels (845 ng/mL; age-matched normal, 242-660 ng/mL) were elevated. Histology, immunostaining, and electron microscopy demonstrated a pituitary acidophil stem cell adenoma. Tumor tissue expressed both somatostatin receptor type 2 and dopamine receptor type 2. The Gs alpha subunit, GHRH receptor, and MEN1 genes were intact, and tumor tissue abundantly expressed pituitary tumor transforming gene (PTTG). Serum GH and PRL levels were controlled after two surgeries, and with continued cabergoline and octreotide LAR GH, PRL, and insulin-like growth factor I levels were normalized. In conclusion, administration of long-acting somatostatin analog every 4 weeks in combination with a long-acting dopamine agonist biweekly controlled biochemical parameters and accelerated growth in a patient with gigantism caused by a rare pituitary acidophil stem cell adenoma.

Gamma Knife radiosurgery as a primary surgical treatment for hypersecreting pituitary adenomas.

OBJECT: To estimate the efficacy of Gamma Knife radiosurgery (GKR) especially as a primary surgical treatment for hypersecreting pituitary adenomas. METHODS: 274 patients were treated with GKR. The mean tumor volume was 1.86 cm(3). The mean peripheral dose was 28.7 Gy. RESULTS: 223 patients were followed up for an average of 31.6 months. The dose related to the tumor growth control and endocrinological normalization was detailed and statistical analysis of the data was performed. CONCLUSION: GKR as a primary surgical treatment for hypersecreting pituitary adenomas may be safe and effective.

[Sleep apnea syndrome in acromegalic patients]. / Zespól bezdechów podczas snu u chorych z akromegalia.

The aim of this report was to analyse the sleep apnea rate and its clinical picture in acromegaly patients before and after the surgical treatment. The authors investigated both hormones levels and spirometric coefficients. They found, that surgical removal of microadenoma from transphenoidal approach led to the fast regression of the syndrome.

Evaluación diagnóstica de la somatomedina C en la acromegalia / Diagnostic evaluation of somatomedine C in acromegaly

El diagnóstico de acromegalia se ha basado tradicionalmente en los niveles de hormona de crecimiento (GH) después de una carga de glucosa por vía oral (CGO). Esta prueba ha sido reemplazada paulatinamente con la estimación de la concentración del factor de crecimiento insulinoide (IGF-I) conocido como somatomedina C, ya que parece reflejar más directamente la acción de la GH, lo que a su vez podría constituir un mejor indicador de la actividad clínica en la acromegalia. Se estudiaron 47 pacientes acromegálicos en los que se realizó una CGO como parte del protocolo diagnóstico, midiéndose la concentración basal de IGF-I y GH; esta última también se midió en las demás muestras de sangre. Los pacientes fueron sometidos a cirugía transesfenoidal y el diagnóstico se confirmó histológicamente. Los niveles de IGF-I fueron altos en todos excepto en uno, lo que representa un valor predictivo de 98.7 por ciento. La concentración de GH estuvo elevada en 31 pacientes, lo cual significa un valor predictivo de 65.9 por ciento. Se concluye que la determinación de IGF-I, o comatomedina C, es una prueba diagnóstica confiable en la acromegalia y tiene ventaja adicional de un menor costo.

[Transsphenoidal microsurgical removal of GH-secreting pituitary adenoma in: a report of 200 cases].

From July 1979 to April 1992, 212 cases of GH-secreting pituitary adenoma were treated transsphenoidally by microsurgical technique, of whom two hundred cases monitored with their GH levels at preoperative and postoperative periods. The majority of cases totally 132 cases were of macroadenoma, and 68 cases were of microadenoma. 138 cases were operated on after April 1986. The cure and remission rate averaged to 72.5%, rising from 44.4% (before 1986) to 80.5% for microadenoma and from 31.4% to 69.1% for macroadenoma due to improvement of technique and accumulation of experience. One patient died of angiocardiopathy suddenly during operation. Mortality rate was 0.5%. The follow-up observation of postoperative GH levels in 52 cases revealed that the GH levels within 2 weeks after operation were the same as in 3-6 months. It is thought that the early GH levels may represent the surgical results. The surgical technique is described and factors influencing the surgical results, prevention and treatment of operative complications are discussed in this paper.

[Surgical treatment of pituitary adenoma].

Seventy-four patients with pituitary adenoma were treated by surgery, including 51 transsphenoidal and 23 transcranial approaches, without death, postoperative leakage of CSF and early-period intracranial infection. Many patients had a transient diabetes insipidus after operation. In discussion we attended the difference of attacking age of pituitary adenoma between male and female, the significances of CT and MRI as well as the indication and the important points of various approaches.

[Bleeding and complications related to hemorrhage during transsphenoidal removal of pituitary tumor].

450 cases who undertook the transsphenoidal removal of pituitary tumors from 1982 to 1992 were reviewed. The bleeding during operation and the complications related to the bleeding were reported in 19 cases. The intercavernous sinuses were traumatized during operation in 9 cases. The possibility of abnormal dural arteries existed in 3 cases. The bleeding happened in the course of dissociation of nasal septum in 2 cases. The cause of bleeding in 3 cases were unclear. There was no much bleeding or bleeding record during operation, but postoperative bleeding complications appeared in other 2 cases. Heavy bleeding came from nose repeatedly in 2 cases, one of which was confirmed as pseudoaneurysm. The postoperative subarachnoid haemorrhage in 2 cases, one blindness and one paralysis of abductor nerve happened. The causes of bleeding and complication were discussed.

[Bladder cancer associated with growth hormone-producing pituitary tumor: a case report].

A case of bladder cancer associated with a growth hormone (GH) producing pituitary tumor is reported. A 67-year-old male underwent hypophysectomy for a GH producing pituitary tumor, 31 months earlier. Although the serum GH decreased postoperatively, recurrence of pituitary tumor with increased serum GH was found and followed by a neurosurgeon. He was referred to our department for asymptomatic gross hematuria. Cystoscopy revealed a papillary pedunculated tumor. Under the diagnosis of superficial bladder tumor, transurethral resection (TUR) was performed. Pathological examination showed transitional cell carcinoma. As the bladder cancer recurred with a high level of serum GH seven months later, TUR was repeated. We reviewed and discussed the relationship between GH or GH producing tumor and bladder cancer.

Sudden blindness from pituitary apoplexy: a report of two cases.

Two cases of acidophil adenoma of the pituitary causing sudden blindness from pituitary apoplexy are presented. The tumours had been clinically silent, without producing any symptoms of endocrine dysfunction. Radiological evidence was very conclusive. Transfrontal craniotomy with decompression resulted in quick and dramatic visual improvement. The interesting syndrome of clinical manifestations is discussed.

Immunohistochemistry as a predictor of clinical outcome in patients given postoperative radiation for subtotally resected pituitary adenomas.

There is general agreement that postoperative radiation therapy is beneficial for patients with subtotally resected pituitary adenomas. We have identified 41 such patients treated during a 20-year period who received postoperative irradiation for a pituitary adenoma. The usual dose was 5040 cGy in 28 fractions. The mean follow-up time was 10.3 years. On routine hematoxylin and eosin (H&E) staining, there were thirty-three chromophobe, seven eosinophilic, and one basophilic adenoma. Tissue blocks were stained for growth hormone (GH), luteinizing hormone (LH), thyroid-stimulating hormone (TSH), prolactin (PRL), and/or adrenocorticotropin (ACTH) using the peroxidase-antiperoxidase immunohistochemistry (IHC) method. Routine H&E staining was a poor predictor of the IHC stain. While most patients with a known clinical endocrine syndrome stained positive on IHC for the suspected offending hormone, many patients without a clinical syndrome also stained positive indicating the presence of hormonally occult adenomas in this locally invasive group. The IHC stain results were compared to clinical outcome. The presence of positive GH IHC staining decreased the 15-year progression-free survival (PFS) from 100% to 64% compared to GH negative adenomas (p = 0.06). There was a trend toward decreased 15-year PFS in patients who did not stain for LH. Positive staining for prolactin, ACTH, or TSH had no influence on the progression-free survival. We conclude that additional prognostic information can be obtained in this subset of patients (by performing IHC analysis) that is not known by the clinical presentation or appearance on H&E stain.

Double adenomas of the pituitary: a clinicopathological study of 11 tumors.

Of more than 3000 cases of surgically removed pituitary adenomas, 11 were defined as "double adenomas," i.e., 2 morphologically or immunocytologically distinct tumors. In 8 cases, the lesions exhibited differing histological features and immunophenotypes; in 2 specimens, distinct ultrastructural features were noted as well. In another instance, despite histological and immunocytological uniformity, the two neoplastic components demonstrated distinct ultrastructure. In yet another case, the two adenomas were consecutively removed; despite similar histological features, they differed in immunocytological and ultrastructural characteristics. Last, in one case, the adenoma was histologically uniform, but a portion of the mass exhibited immunoreactivity by ultrastructural features distinct from those of the remainder of the lesion. Hormonal excess attributed to both tumors could be correlated with endocrine manifestations in two cases. Double adenomas of the pituitary occur infrequently. In routine histological sections of surgical material, they are often difficult if not impossible to identify. Presented herein are clinical and endocrinological data on 10 cases of double pituitary adenomas correlated with morphological and immunocytochemical results. The literature regarding multiple adenomas is reviewed as are the diagnostic and therapeutic difficulties associated with these rare lesions.

Effect of long-term treatment with somatostatin analogue (SMS 201-995) on pituitary tumor shrinkage in acromegaly--report of two cases.

The effect of long-term somatostatin analogue (SMS 201-995) treatment in two acromegalic patients is reported. Continuous tumor shrinkage was observed even after 129 and 139 weeks of treatment with 600 micrograms of SMS 201-995 daily. A huge and firm adenoma underwent shrinkage during treatment with SMS 201-995. No serious side effect appeared during 160 weeks of treatment. SMS 201-995 has a longterm tumor shrinkage effect and improves endocrinopathies.