Interesting coincidence of atypical TSH-secreting pituitary adenoma and chronic lymphocytic leukemia.

Thyrotropin-secreting adenomas (TSH-oma) are very rare pituitary tumours. They are macroadenomas usually presenting with signs and symptoms of hyperthyroidism, and mass effects. They can co-secrete other hormones such as growth hormone or prolactin. Different malignancies, including haematological ones, are reported in patients with pituitary diseases. Chronic lymphocytic leukemia (CLL) occurs mostly in older patients, more often in males. CLL is associated with increased risk of second malignancies such as other blood neoplasms, skin and solid tumours. We present a successful neurosurgical outcome in a patient with an interesting coincidence of atypical TSH-oma and asymptomatic CLL.

An image-guided, noninvasive method of cannulation of the foramen ovale for awake, percutaneous radiofrequency rhizotomy.

The authors describe a simple and low-cost technique for image-guided cannulation of the foramen ovale during treatment of medically refractory trigeminal neuralgia using percutaneous radiofrequency rhizotomy. Computed tomography-guided neuronavigation and the noninvasive ear, nose, and throat Small Active Frame system were applied for visualization of cannulation of the foramen ovale in the awake patient. Use of invasive head-clamp fixation was unnecessary. Three patients were treated using this technique in which the foramen ovale was reached using a single tract. No adverse effects of this technique were reported. The potential benefits and disadvantages of this method are presented.

[Surgical treatment for Rathke cleft cysts]. / Leczenie chirurgiczne torbieli kieszonki Rathke'go.

Surgical treatment for Rathke cleft cysts--Intrasellar and suprasellar cysts are often lesions observed in the neuroradiological examinations. The majority of them (craniopharyngioma, cystic pituitary adenoma) are histologically neoplasmic. The others originate from the remnants of the embryonic diverticulum which arise from the roof of the stomodeum. Rathke cleft cyst is the most common cystic developmental lesion of the pituitary gland. It is located within the sella turcica as well as over the diaphragm, near the stalk. Occasionally, they may become large enough to cause typical symptoms and signs headaches, adenohypophysis disfunction, diabetes insipidus and visual field defects. Asymptomatic Rathke cleft cyst should be observed (detailed endocrinological and radiological examination of the pituitary gland). Magnetic resonance imaging (MRI) is the preferred neurodiagnostic study in evaluating the Rathke cleft cyst. Aspiration of cyst contents and cyst wall biopsy or it's total removal are the operative treatment of the symptomatic cysts. Transsphenoidal approach is usually effective. Reccurence of Rathke cleft cyst after surgical treatment is rare. The authors present 16 cases of Rathke cleft cysts operated on during the period from January 1996 to December 2002. We observed 12 intrasellar cysts and 4 suprasellar lesions. Fourteen patients underwent transsphenoidal procedure and four patients were operated on via transcranial route. Histological examination confirmed Rathke cleft cyst. There were no serious complications in our group. Follow-up examination comprises periodic endocrinological and radiological investigations.

[Transsphenoidal surgery of secretory and invasive pituitary adenomas (somatotropinoma or corticotropinoma)--own experiences]. / Zastosowanie dostepu przezklinowego w leczeniu operacyjnym czynnych hormonalnie (wydzielajacych GH lub ACTH) inwazyjnych guzów przysadki mózgowej--doswiadczenia wlasne.

UNLABELLED: Invasive pituitary adenomas with extension to the cavernous sinus are rare and comprise 6-10% of all pituitary tumors. Their effective surgical treatment is very difficult. Cavernous sinus invasion increases the morbidity and mortality associated with surgical procedures. Prolonged medical treatment and radiotherapy are an alternative to the surgery. PURPOSE: Efficiency of the extended transnasal transsphenoidal approach in the treatment of the invasive pituitary adenomas with extension to the cavernous sinus. MATERIAL AND METHOD: During the past two years (from January 2000 to December 2001) authors carried out 24 surgical operation in the medial compartment of the cavernous sinus (extended transnasal transsphenoidal approach). Our experience is based on the analysis of 24 patients (19 patients with GH-secreting tumors and 5 patients with ACTH-secreting adenomas--2 with Cushing disease and 3 with Nelson's syndrome) with invasive macroadenomas expanding to the cavernous sinus. Mean age was 46.21 years (range 28-68, SD +/-9.54 years) in our group. Mean serum GH level was 43.95 microg/l (range 9-170 microg/l, SD +/-39.68 microg/l) and mean serum ACTH level in the group of Nelson's syndrome was 1180 pg/ml (range 790-1780 pg/ml, SD +/-527.35 pg/ml). Parasellar extension of the tumor was measured according to Knosp's scale. There were 16 patients in stage III and 9 patients in stage IV. RESULTS: Total surgical removal of invasive GH-secreting adenoma (according cure criteria: basal serum GH level below 2.5 microg/l, OGTT<1 microg/l, normal sex and age-related IGF-I level) we achieved in 6 patients. Basal serum GH level was below 5 microg/l (postoperative mean serum IGF-I level was significantly lower but abnormal) in the next five patients. Remission (normalization of 24-hour urine-free cortisol (UFC) and its metabolites) was achieved in one patient with Cushing disease. Out of 3 patients with Nelson's syndrome two were cured. Their postoperative serum ACTH level was below 80 pg/ml. There were no mortality and no deterioration of cranial and optic nerves. CSF leakage occurred in one patient and was treated surgically. Deterioration of pituitary function and diabetes insipidus occurred in one patient. CONCLUSION: Extended transnasal transsphenoidal approach is the safe surgical procedure in the treatment of invasive GH or ACTH-secreting pituitary adenomas. The overall morbidity rate of this surgical intervention is rare. We suppose the extended transnasal transsphenoidal approach is an alternative for prolonged medical treatment and radiotherapy.

[Own experience in surgical treatment of the pineal region and midbrain tumors via the infratentorial approach]. / Wlasne doswiadczenia w leczeniu chirurgicznym guzów okolicy szyszynki i sródmózgowia z zastosowaniem dostepu podnamiotowego.

Nine cases of tumours located in the pineal and midbrain region in adults operated on between November 1998 and July 2002 in Dept. of Neurosurgery, Central Military Hospital in Warsaw are reported. The patients (2 men and 7 women) were aged from 27 and 69 years (mean age 43.6 years). Their main initial symptoms were caused by hydrocephalus. The histopathological examination revealed anaplastic pinealoma in 2 cases, and pineocytoma, pineal cyst, mesencephalic glial cyst, protoplasmatic astrocytoma, epidermoid cyst, unclear glial scar, and papillary ependymoma in single cases. Five patients had been treated, usually elsewhere, with shunt implantation prior to the surgery. Occipito-suboccipital osteoplastic craniotomy was performed in each case and tumours were totally removed microsurgically via the infratentorial epicerebellar approach. In one case a part of the glial periaqueductal tumour was resected additionally via the fourth ventricle and aqueduct in one stage. Postoperative haematomas in the third and fourth ventricle were found in 2 cases. Main complaints after the surgery included transient diplopia. All the patients improved significantly and resumed their previous life activities. Follow-up ranged from 3 to 44 months. Three patients with pineal tumours and one with a small postoperative ependymoma recurrence were irradiated after the surgery. One patient had been irradiated prior to surgery. Very good results of the surgical treatment of tumours in this area suggest that such patients should be referred earlier to one stage surgical management, as the procedure is easier to perform and shunt implantation may be avoided.

[Surgical treatment of invasive pituitary adenomas (somatotropinoma or corticotropinoma)]. / Leczenie operacyjne czynnych hormonalnie (wydzielajacych GH lub ACTH) inwazyjnych gruczolaków przysadki mózgowej.

AIM OF THE STUDY: To evaluate efficiency of the transcranial epidural approach in the treatment of invasive GH- or ACTH-secreting pituitary adenomas with extension to the cavernous sinus. MATERIAL AND METHODS: During the past two years (from January 2000 to December 2001) 14 patients with invasive GH- or ACTH-secreting pituitary adenomas extending to the cavernous sinus were operated on using the transcranial epidural approach. Our experience is based on an analysis of 12 patients with GH-secreting tumors and 2 patients with ACTH-secreting adenomas. The patients' mean age was 45.36 years (range 28-66, SD +/- 10.26 years). Parasellar extension of the tumor was measured using the Knosp scale--in all the cases there was an extension to the cavernous sinus, in stage III (4 patients) or stage IV (10 patients). RESULTS: In none of the cases a total surgical removal of the invasive GH-secreting adenoma was attained (according the following cure criteria: basal serum GH level below 2.5 micrograms/l, OGTT < 1 microgram/l, normal sex- and age-related IGF-I level). In four patients the surgery resulted in a reduction of the basal serum GH level to below 5 micrograms/l (their postoperative mean serum IGF-I level was 530 micrograms/l--significantly lower, but still abnormal, p < 0.05). In a single case of a female patient the basal serum GH level was below 10 micrograms/l, while in other 7 patients the GH level remained above 10 micrograms/l. Remission (normalization of 24-hour urine-free cortisol (UFC) and its metabolites) was achieved in one patient with the Cushing disease. There were no complications involving case fatality. A transient deterioration of the third cranial nerve function observed in one patient disappeared within 3 months from the surgery. There was no deterioration of pituitary function and no cases of diabetes insipidus in our group. CONCLUSION: Transcranial epidural approach is an alternative to radiotherapy and/or prolonged medication in the treatment of invasive GH- or ACTH-secreting pituitary adenomas.

[Transsphenoidal surgery of intrasellar and perichiasmatic tumors--personal experience]. / Leczenie operacyjne guzów okolicy skrzyzowania wzrokowego z dostepu przez zatoke klinowa--doswiadczenia wlasne po trzydziestu latach stosowania dostepu w Polsce.

Microsurgical transsphenoidal approach has been for decades an established standard surgical treatment of pituitary adenomas. Actually it offers a safe and rapid midline access to the sellar region with a minimal risk of injury to vascular vessels, the optic chiasm or nerves and the brain. Major limitations of this approach are due to the narrow and deep operating field, with the related possible complications of the pituitary insufficiency and the cerebrospinal fluid rhinorrhea. In this paper the authors present their own experience with transsphenoidal surgery they performed in over 1500 cases during the past 28 years. The history and evolution of the microsurgery are described, including the operative technique, indications and counter-indications, as well as relative advantages and disadvantages of the transsphenoidal approach. Moreover, current modifications of the neurosurgical technique (extended transsphenoidal approaches) and their application in the treatment of non-glandular perichiasmatic tumors are outlined and discussed.

[Colloid cysts of the pituitary gland]. / Wystepowanie torbieli koloidowych przysadki mózgowej.

Colloid cysts of the pituitary gland are very rare pathological lesions occurring in sellar region. Their pathogenesis is not clear. They are located between the anterior and posterior lobe of the pituitary. Colloid cysts of the pituitary gland are space occupying lesions and induce hypopituitarism, diabetes insipidus, visual disturbances, etc. They cause pituitary apoplexy too. Magnetic resonance imaging is the preferred neurodiagnostic method in evaluating these lesions. 503 transsphenoidal procedures were performed from January 1996 to February 2001. Hypothalamo-hypophyseal dysfunction was caused by colloid cyst in seven cases. The most frequent presenting symptoms were headaches, oligomenorrhea and loss of libido. Diabetes insipidus was found in two cases before surgery. Colloid cyst induced pituitary apoplexy in one patient. Two patients presented visual disturbances. All our patients were operated on. We performed six transsphenoidal procedures and one transcranial operation. Postoperatively, the endocrine function of the pituitary gland was evaluated. We concluded that transsphenoidal operation is a safe method for treating colloid cyst located in the sellar region.

[Prognostic value of the Knosp scale in trans-sphenoidal surgery for pituitary adenomas]. / Wartosc prognostyczna skali Knosp'a w operacjach przezklinowych gruczolaków przysadki mózgowej.

Various surgical approaches are available for the treatment of somatotroph pituitary adenomas. The treatment of choice remains surgical excision via transsphenoidal route. The results of that operation depend on the volume of the tumour, its suprasellar and parasellar extension, especially to the cavernous sinus. Modern neuroimaging by magnetic resonance provides visualization of the size and extension of the tumour, but invasion of the cavernous sinus space remains still a surgical diagnosis. According to intraoperative observations and proliferation marker (Ki-67) Knosp has evolved out radiological classification describing parasellar extension of pituitary adenomas. We present a series of 142 somatotroph pituitary adenomas surgically treated by transsphenoidal route. The radiological features of the tumours are described using Knosp classification. The results of surgery are analyzed with special reference to preoperative magnetic resonance findings. We found Knosp scale useful for prognostic classification for transsphenoidal surgery cases.

[Ectopic pituitary adenoma. Case report and review of literature]. / Ektopowy gruczolak przysadki. Opis przypadku i przeglad pismiennictwa.

Ectopic pituitary adenomas are very rare neoplasms. They are usually located in the sphenoid sinus or nasopharynx. Ectopic tumors derive from a residual pituitary fragment in the craniopharyngeal duct. They are similar in morphology, immunohistochemistry and hormonal activity to the typical pituitary adenoma. One third ectopic pituitary tumors reported in the literature are endocrine inactive, while the remaining ones indicate a hormonal activity and usually secrete ACTH, causing the Cushing disease. About 50% of pituitary adenomas are located at the skull base and present as aggressive neoplasms. The computed axial tomography (CAT) and magnetic resonance imaging (MRI) are very useful in revealing the presence of these tumors. Preoperative diagnosis of ectopic pituitary adenomas located at the skull base is difficult. Diagnostic procedures usually suggest another type of neoplasm, e.g. chordoma, nasopharyngeal carcinoma, or a metastatic tumor. Pathological examination including immunohistochemical studies of neuroendocrine markers is important in establishing the diagnosis. A unique case of ectopic, nonfunctioning pituitary adenoma involving the sphenoid sinus and nasopharynx and causing palsy of cranial nerves is presented. MRI examination revealed the presence of a large invasive tumor. The neoplasm was partially resected by transnasal approach. The diagnosis was confirmed by a pathological examination including immunohistochemical smears.

[Thyrotropin--TSH secreting pituitary tumor]. / Tyreotropinoma--gruczolak przysadki wydzielajacy TSH.

Thyrotropin-releasing pituitary tumors represent 0.9 to 2.8% of all pituitary adenomas. They cause secondary or central hyperthyroidism. The diagnosis of these tumors has been increasing in the past 20 years. It was produced by introduction of the sensitive immunoradio-metric assay of TSH and better radiological imaging (magnetic resonance imaging). TSH--secreting pituitary adenomas are aggressive and invasive neoplasms. Most reports describe a poor outcome after pharmacological therapy, surgery and radiation therapy. Presently the diagnosis of thyrotropin-secreting pituitary tumor is based on the lack of: a. inhibition of TSH levels in the presence of increased free thyroid hormones; b. response of TSH to stimulation with TRH; c. and presence of a abnormal, neoplastic(adenomatous) intrasellar or parasellar mass. Surgical excision (selective adenomectomy) by the transsphenoidal route is the first treatment. Craniotomy should be reserved for parasellar tumors with significant lateral extension. Pharmacological pretreatment with long acting somatostatin analogues is recently a standard before surgery. This medical treatment of the TSH-omas is effective in reducing TSH and free thyroid hormone plasma levels. Administration of the somatostatin analogues causing tumor mass shrinkage and changes consistency. This pretreatment is effective therapy and improves surgical outcome especially in patients harbouring macroadenomas. Radiotherapy is noncurative and produces long term complications (hypopituitarism). Authors present and discuss current cure criteria of TSH-omas with reference to their clinical experience.

[Acromegaly--do we already know everything about treatment of the somatotroph pituitary adenomas?]. / Akromegalia--czy wiemy juz wszystko o leczeniu gruczolaków somatotropowych przysadki?

Acromegaly--do we already know everything about treatment of the somatotroph pituitary adenomas?--Acromegaly, an insidious neuroendocrine disorder, reduces life expectancy and leads to 3.5-fold increase in mortality. The main causes are cardiovascular, pulmonary and enhanced prevalence of deaths from malignancy. Successful therapy ought to normalize GH, IGF-I secretion, remove adenoma mass and its local pressure effects and preserve pituitary function to improve systemic morbidity and normalize mortality. The primary therapy for most patients with acromegaly caused by somatotroph pituitary adenoma is still transsphenoidal resection of the tumor. The results of the transsphenoidal surgery for pituitary adenoma for acromegaly depend on the volume of the tumor, its suprasellar and parasellar extension, especially to the cavernous sinus. The aim of this paper was to present the diagnostic criteria and principle therapy in these tumors. Our experience was based on the analysis of more than 150 patients operated on during the last five years and review of the literature.