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PubMed1989

Endocrine function in patients with large pituitary tumors treated with operative decompression and radiation therapy.

Large pituitary tumors are still a common problem. Thirty consecutive patients underwent operative decompression and radiation therapy for large sellar and suprasellar tumors. They were studied prospectively in terms of their endocrine outcome. Ten of the patients had panhypopituitarism both before and after treatment. The other 20 patients had partial hormonal deficits prior to treatment. Ten (50%) of the 20 patients who had partial preoperative deficits went on to develop delayed onset of worsening in their endocrine function; 9 of the 10 developed panhypopituitarism, and 1 patient developed decreased thyroid function. The mean time from surgery until the onset of delayed worsening in endocrine function was 26.1 months. The mean age of those patients who developed delayed onset of worsening in pituitary function was significantly higher than that of those who did not develop further hormonal loss (40.5 +/- 3.1 years versus 52.0 +/- 4.1 years, P less than 0.05). None of the 10 patients with delayed onset of worsening in pituitary function had anatomical evidence by computed tomographic scan of tumor recurrence. Delayed onset radiation effect is the most likely cause of the late onset of worsening in endocrine function.

Combined Modality Therapy↗
PubMed1994

Functioning ectopic supradiaphragmatic pituitary adenomas.

Pituitary adenomas arising far from the pituitary gland occur rarely as a result of defects in embryological migration. Likewise, there have been reports of isolated suprasellar adenomas (clinically nonfunctioning), presumably derived from cells of the pars tuberalis. In this report, we present four cases of functional pituitary adenomas (three adrenocorticotrophic hormone, one prolactin) confined to the supradiaphragmatic region. In each case, the tumors were initially treated unsuccessfully by operations via the transsphenoidal route because of expected intrasellar processes with suprasellar extension.

Adenoma↗
PubMed1994

Magnetic resonance imaging and pathological analysis of a pituicytoma: case report.

A case of a pituicytoma is presented that describes the clinical, pathological, and magnetic resonance imaging features of a rare tumor of the neurohypophysis. A 26-year-old woman presented with a 4-month history of dizziness and visual obscuration. A magnetic resonance image revealed a pituitary mass with suprasellar extension. The specimen obtained from a transsphenoidal decompression identified the mass as an astrocytoma of the posterior pituitary (pituicytoma). Immunohistochemical staining was positive for glial fibrillary acidic protein, S-100, and vimentin. Electron microscopy identified intermediate filaments, numerous broad cell junctions, no secretory granules, and two cellular populations with either an electron-dense or lucent cytoplasmic matrix. This case is unique in that other documented cases originating in the posterior pituitary have all been pilocytic astrocytomas, whereas this neoplasm was not a pilocytic variant. This is also the first case in the literature of a pituicytoma documented by magnetic resonance imaging. This report reviews the cytological elements of the neurohypophysis and the origin of pituicytomas and stresses the proper use of the term "pituicytoma" in relation to tumors of the posterior pituitary.

Adult↗
PubMed1994

Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome.

Pathological and clinical data from 56 patients operated on for craniopharyngioma since 1981 were analyzed to determine the utility of dividing patients with this tumor into distinct clinical groups based on recognized pathological type and to determine the prognostic import of brain invasion. Of the tumors in the 30 adult patients, 66% were adamantinomatous, 28% were squamous papillary, and the remainder were mixed. However, of the tumors in the 26 children, 96% were adamantinomatous and none were pure squamous papillary (P < 0.01). Forty-six percent of the children compared with 17% of the adults had brain invasion (P < 0.01). Brain invasion was present in 37% of the adamantinomatous but in only 13% of the squamous papillary tumors. Seventy-seven percent of the children underwent gross total resection (GTR) compared with 27% of the adults (P < 0.01). Sixty-three percent of the squamous papillary tumors underwent GTR compared with 54% of the adamantinomatous and mixed tumors. Follow-up ranged from 7 to 187 months (mean, 49 mo). After subtotal resection, with or without radiation therapy, 58% of the tumors recurred compared with 17% recurrence after GTR (P < 0.01), with a mean time to recurrence of 34 months. In both tumor histological types, subtotal resection was associated with a higher rate of tumor recurrence compared with gross total resection. Among the subtotally resected craniopharyngiomas, 2 of the 3 (67%) squamous papillary and 11 of the 21 (52%) adamantinomatous and mixed tumors recurred. In contrast, among the totally resected tumors, none of the 5 squamous papillary and only 5 of the 25 (20%) adamantinomatous and mixed tumors recurred. There were no significant differences in Karnofsky performance status score, mortality rate, or visual and endocrine outcomes when comparing patients based on histological tumor type. When controlling for age and extent of resection, we found that brain invasion had no significant effect on recurrence rate in totally resected tumors. Based on the limited number of patients in this series, we conclude as follows. 1) Contrary to previous reports, squamous papillary craniopharyngiomas, like adamantinomatous tumors, may recur when subtotally resected. 2) For both tumor variants, the most significant factor associated with craniopharyngioma recurrence is the extent of surgical resection rather than histopathological subtype. 3) Contrary to prior hypotheses, brain invasion in totally resected tumors does not predict higher recurrence. 4) GTR is associated with a significantly lower recurrence rate and can be achieved without sacrificing functional outcome.

Adolescent↗
PubMed1995

Gonadotropic pituitary carcinoma: case report.

A 37-year-old man developed multiple intracranial, intraspinal, and general metastases from an invasive nonfunctioning pituitary adenoma after surgery and radiation therapy. This is the first gonadotropic pituitary carcinoma reported in literature.

Adenoma↗
PubMed2004

Pituicytoma: report of two cases and clues regarding histogenesis.

OBJECTIVE AND IMPORTANCE: The pituicytoma is a rare primary tumor of the neurohypophysis. Although histologically benign, the location and vascular nature of these tumors can make surgical resection difficult. We present a report of two patients with pituicytomas and review the literature regarding treatment and prognosis for this unusual lesion. Possible histogenetic relationships of this tumor with other pituitary neoplasms are presented. CLINICAL PRESENTATION: Patient 1 was a 45-year-old man who presented with a 5-year history of decreased libido. He was found to have a 2-cm suprasellar mass on a magnetic resonance imaging scan. Patient 2 was a 48-year-old man who presented with multiple endocrine complaints. He was found to have an intrasellar mass on magnetic resonance imaging. INTERVENTION: Patient 1 underwent a right frontal craniotomy, with a subtotal resection of the suprasellar mass through the lamina terminalis. The residual tumor was treated with fractionated stereotactic radiotherapy. The intrasellar mass in Patient 2 was resected via a transsphenoidal approach. On surveillance magnetic resonance imaging, the tumor was found to have recurred and expanded into the suprasellar space. The patient underwent a right frontal craniotomy for decompression and a subtotal resection of the tumor. The patient experienced a second recurrence 7 years after the initial procedure and was subsequently treated with fractionated stereotactic radiotherapy. CONCLUSION: Pituicytomas are a distinct form of pituitary gland neoplasia that may recur if subtotally resected. These neurohypophysial tumors may contain a small subpopulation of previously unrecognized bcl-2-immunoreactive cells, whose role in the histogenesis of pituicytoma deserves further study.

Adenoma↗
PubMed1978

ACTH and cortisol response to bromocriptine, and results of long-term therapy, in Cushing's disease.

Plasma corticotrophin (ACTH) was lowered in 4 out of 5 patients with pituitary dependent Cushing's syndrome (one of whom was studied only after bilateral adrenalectomy) after a single oral dose of 2.5 mg bromocriptine, but plasma cortisols were unaltered in the 3 patients in whom it was measured. Three patients were observed during treatment with bromocriptine for 16 to 87 weeks. One improved symptomatically while maintained on a combination of metyrapone and bromocriptine, but plasma ACTH levels remained high even when the dose of bromocriptine was increased to 20 mg daily. Bromocriptine therapy was discontinued after 16 weeks in the second patient due to the development of mental depression. Her clinical features had not improved during this time. The third patient, who also underwent a course of pituitary irradiation, became, and remains, symptom free, with satisfactory plasma ACTH and cortisol levels for the 87 weeks he has received bromocriptine. The role of bromocriptine in the management of Cushing's disease seems limited despite the fact that plasma ACTH may fall after a test dose of the drug.

Administration, Oral↗
PubMed1989

Lack of diurnal rhythm of low molecular weight insulin-like growth factor binding protein in patients with Cushing's disease.

A specific radioimmunoassay with antibodies raised against the 25 kD insulin-like growth factor binding protein (25 kD IGFBP) in amniotic fluid was used to measure levels of cross-reacting protein in human serum and plasma. Plasma samples collected continually at 20-min intervals during 24-h in 6 healthy adults revealed a distinct diurnal rhythm in the concentration of 25 kD IGFBP. The lowest levels (9-13 micrograms/l) were found between 13.00 and 24.00 h with a rise after midnight to maximum levels (23-71 micrograms/l) between 03.00 and 09.00 h. There was no relation between the patterns of GH and 25 kD IGFBP. In 3 patients with active Cushing's disease, the levels of 25 kD IGFBP in plasma samples collected during 12 h, 19.00-07.00 h, were generally low and without nocturnal variations. One of the patients studied after extirpation of a pituitary adenoma displayed a nocturnal rhythm with maximum levels of 25 kD IGFBP between 03.00 and 07.00 h. Eight patients treated with stereotactic pituitary irradiation owing to Cushing's disease also showed a distinct nocturnal increase of 25 kD IGFBP. The results indicate the existence of a diurnal rhythm of 25 kD IGFBP in adults. Further, low levels and lack of diurnal rhythm of 25 kD IGFBP are demonstrated in Cushing's disease.

Adult↗
PubMed1993

Radical removal of craniopharyngioma: a long-term follow-up.

Radical removal of craniopharyngiomas was attempted as the primary treatment in 12 adult and six child patients. No postoperative radiotherapy was given to patients undergoing total removal, while those undergoing subtotal removal received 50-60 Gy. Overall evaluation showed 15 cases of total removal and three of subtotal removal. One patient died of septicemia 2 months postoperatively and another developed a recurrent tumor which was removed totally at a second operation. The mean follow-up period was 7 years. The quality of life was "excellent" in 10 patients, "good" in five, and "fair" in two. Visual losses were minimized and endocrinological disturbances treated medically. These satisfactory results indicate that radical surgical removal is the first choice for treatment of craniopharyngioma irrespective of the age of patients.

Adolescent↗
PubMed1990

Long term androgen replacement therapy does not preclude gonadotrophin-induced improvement on spermatogenesis.

A previously hypophysectomized man with azoospermia, who was on androgen replacement therapy since 11 years, was studied with regard to his intratesticular steroid conversion capacity in vitro after which he was given combined hCG/hMG therapy. Before therapy a steroid conversion pattern similar to that seen in prepubertal boys was found, i.e. a high proportion of 20 alpha-dihydroprogesterone was produced while only smaller amounts of 17 alpha-hydroxyprogesterone was produced from the substrate 3H-progesterone. After only five weeks of hCG/hMG treatment, sperm counts and serum testosterone levels increased dramatically and a child was conceived. The steroid conversion pattern simultaneously switched to the mature, adult type with a low production of 20 alpha-dihydroprogesterone and large amounts of 17 alpha-hydroxyprogesterone being produced in vitro. Thus gonadotrophin substitution therapy may still be very effective after long term androgen replacement.

Chorionic Gonadotropin↗
PubMed1992

Microsurgical management of craniopharyngiomas.

A retrospective analysis of 34 patients who underwent microsurgical therapy for craniopharyngioma from 1975 to 1989, a period when CT imaging was routinely used, is presented. Mean follow-up was 6.4 years with no patients lost to follow-up. Those who underwent subtotal resection with adjuvant radiation had a significantly better recurrence-free interval compared with those who either underwent total or subtotal surgical resection only (p < 0.05 and p < 0.025). Among patients treated with surgery alone, the total resection group had a recurrence rate of 20% and those with a subtotal resection 60%. Those with subtotal resection and radiation had a 12% rate of recurrence. Endocrine and visual deficits were common after surgery. Based on this review, our results suggest that with a policy of attempted total resection where possible, subtotal removal along with adjuvant radiation, in cases where total resection was deemed unsafe, may be more effective than aggressive total resection alone as the initial management of craniopharyngioma.

Adolescent↗
PubMed1992

Paediatric craniopharyngiomas: a regional review.

Forty paediatric craniopharyngioma cases treated between 1956 and 1987 by conservative surgery (15), radical surgery (10), conservative surgery and radiotherapy (9) and shunting (6) are reviewed. The conservative surgery and radiotherapy group's local control and survival (100%) is significantly better than that of any other group. This group also achieved the most consistent level of employment or tertiary education. Overall morbidity was high. Overall survival has improved since 1976. Whether given as an adjuvant or salvage a radiotherapy dose of TDF 83 or greater gave a significantly better survival (100%) than lower doses.

Adolescent↗
PubMed1994

Prolactinomas in men masquerading as invasive skull base tumours.

Four giant prolactin-secreting tumours invading the skull base are described. All of them occurred in men. The presenting features were sufficiently diverse to be clinically misleading. We advocate the estimation of prolactin levels before embarking on complicated skull base surgery in men where doubt remains regarding the diagnosis after clinical and radiological study.

Adolescent↗
PubMed1994

Cervical lymph nodes metastases from a pituitary carcinoma.

The authors report a case of a pituitary carcinoma which was locally invasive and which metastasized to cervical lymph nodes more than 9 years after the initial presentation. Cells from the tumour and metastasis immunostained with antibodies to prolactin and growth hormone, even though there was no clinical or biochemical evidence that the tumour was secreting prolactin or growth hormone. In addition, ultrastructural studies showed a monomorphic tumour with secretory granules much smaller than those normally associated with prolactin and growth hormone secretion. The clinical and pathological features suggest that the tumour is probably an acidophil stem cell adenoma, which although known to be aggressive in its clinical behaviour has not been previously reported to metastasize.

Adenoma, Chromophobe↗