scholarly journals Extrapituitary Parasellar Microadenoma in Cushing’s Disease

1999 ◽  
Vol 84 (8) ◽  
pp. 2912-2923 ◽  
Author(s):  
Ryszard M. Pluta ◽  
Lynnette Nieman ◽  
John L. Doppman ◽  
Joseph C. Watson ◽  
Nancy Tresser ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Cushing’S Disease ◽  
Anterior Lobe ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Pars Tuberalis ◽  
Serial Sections ◽  
Resonance Imaging ◽  
High Incidence ◽  
Acth Secreting

Negative sellar exploration (despite the results of endocrine evaluation indicating Cushing’s disease), the high incidence of failure of total hypophysectomy, and remission of Cushing’s syndrome after unsuccessful hypophysectomy and sellar irradiation suggest that the etiology of refractory Cushing’s disease, in some patients, lies near the sella but not in the pituitary gland. We present 5 patients, out of 626 who received surgery for Cushing’s disease, in whom an ACTH-secreting extrapituitary parasellar adenoma was identified: 2 after unsuccessful total hypophysectomy for the treatment of refractory Cushing’s disease, 2 after unsuccessful hemihypophysectomy (the first, 2 yr before treatment at the NIH for Nelson’s syndrome; and the second, with recurrent Cushing’s disease 5 yr after negative transsphenoidal exploration), and 1 with a preoperative diagnosis of an intraclival microadenoma, which was cured by resection of the tumor. In all cases, an extrapituitary parasellar microadenoma was confirmed unequivocally as the cause of the disease, by negative pathology of the resected pituitary gland (patients 1, 2, 3, and 5), and/or the remission of the disease after selective resection of the extrasellar adenoma (patients 3, 4, and 5). Three of 5 patients had a partial empty sella. These patients support the thesis that ACTH-secreting tumors can arise exclusively from remnants of Rathke’s pouch, rather than from the adenohypophysis (anterior lobe or pars tuberalis of the pituitary gland) and can be a cause of Cushing’s disease. In the sixth presented case, an extrapituitary tumor was suspected at surgery after negative pituitary exploration, but serial sections of the hemihypophysectomy specimen revealed a microscopic focus of tumor at the margin of the resected gland. This case demonstrates the importance of negative pituitary histology to establish the presence of an extrapituitary parasellar tumor as an exclusive source of ACTH, and it supports the value of clinical outcome to establish the diagnosis with selective adenomectomy of an extrapituitary parasellar tumor. In patients with negative pituitary magnetic resonance imaging, especially in the presence of a partial empty sella, the diagnostic and surgical approach in Cushing’s disease should consider the identification and resection of extrapituitary parasellar adenoma, which can avoid total hypophysectomy, as was possible in 3 of our 5 patients.

scholarly journals Does pituitary compression/empty sella syndrome contribute to MRI-negative Cushing’s disease? A single-institution experience

2020 ◽  
Vol 48 (6) ◽  
pp. E3
Author(s):  
Benjamin T. Himes ◽  
Adip G. Bhargav ◽  
Desmond A. Brown ◽  
Timothy J. Kaufmann ◽  
Irina Bancos ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Relative Reduction ◽  
Single Institution ◽  
Empty Sella Syndrome ◽  
Tissue Diagnosis ◽  
Acth Secreting

OBJECTIVECushing’s disease arises from functioning adrenocorticotropic hormone (ACTH)–secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing’s disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing’s disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing’s disease.METHODSA single-institution database of Cushing’s disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing’s disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI.RESULTSOf the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61–6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002).CONCLUSIONSEmpty sella syndrome is associated with an increased rate of MRI-negative Cushing’s disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.

Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease

2014 ◽  
Vol 121 (3) ◽  
pp. 518-526 ◽  
Author(s):  
Gautam U. Mehta ◽  
Kamran D. Bakhtian ◽  
Edward H. Oldfield
Keyword(s):  
Pituitary Gland ◽  
Cushing’S Disease ◽  
Pituitary Surgery ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Csf Leak ◽  
Empty Sella Syndrome ◽  
Csf Leaks ◽  
Primary Empty Sella

Object Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD). Methods Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height > 2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point. Results Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p < 0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27). Conclusions Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.

Silent necrosis of a pituitary corticotroph adenoma revealed by timely magnetic resonance imaging: a cause of spontaneous remission of Cushing's disease

1994 ◽  
Vol 130 (5) ◽  
pp. 469-471 ◽  
Author(s):  
Elisabeth Le Nestour ◽  
Jean-Paul Abécassis ◽  
Xavier Bertagna ◽  
André Bonnin ◽  
Jean-Pierre Luton
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Cushing’S Disease ◽  
Sella Turcica ◽  
Spontaneous Remission ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Resonance Imaging ◽  
Corticotroph Adenoma ◽  
Limited Mass

Le Nestour E, Abécassis J-P, Bertagna X, Bonnin A, Luton J-P. Silent necrosis of a pituitary corticotroph adenoma revealed by timely magnetic resonance imaging: a cause of spontaneous remission of Cushing's disease. Eur J Endocrinol 1994;130:469–71. ISSN 0804–4643 Spontaneous necrosis of a corticotroph adenoma is rare and is a very unlikely way of curing Cushing's disease. We report hereafter a case where magnetic resonance imaging of the pituitary provided clear evidence of the event. Successive and timely pituitary magnetic resonance imaging in this patient showed first a typical microadenoma as a well-limited mass with a low signal intensity before the necrosis, then a bright signal before gadolinium injection in the T1-weighted image at the time of the event and, finally, the aspect of an empty sella turcica with a small arachnoidocele 1 year later. The necrosis of a corticotroph adenoma is more frequent in macro- than in microadenomas, and is usually heralded by headache and visual disturbances. In this case, pituitary necrosis was entirely asymptomatic, and cured the patient as well as the surgeon's knife would have. Nevertheless, this exceptional occurrence does not rule out the possibility of a recurrence. X Bartagna, Clinique des Maladies Endocriniennes et Métaboliques, Hôpital Cochin, 27 rue due Faubourg Saint Jacques, 75014 Paris, France

In Vitro Secretion of Adenoma and Anterior Lobe Cells in Two Typical Cases of Cushing's Disease

Neurosurgery ◽  
1983 ◽  
Vol 12 (5) ◽  
pp. 549-554 ◽  
Author(s):  
Dieter K. Lüdecke ◽  
Martin Schabet ◽  
Wolfgang Saeger
Keyword(s):  
Cushing’S Disease ◽  
Anterior Lobe ◽  
Cushing's Disease ◽  
Acth Secretion ◽  
Acth Cell ◽  
Superfusion System ◽  
Adenomatous Tissue ◽  
Corticoid Feedback ◽  
Acth Secreting

Abstract Fragments of adrenocorticotropic hormone (ACTH) cell adenomas and anterior lobes of two patients with Cushing's disease were obtained by transnasal operation. Both patients showed the typical clinical course, with postoperative ACTH deficit and all other pituitary functions intact. Equivalent specimens of tissue were investigated by immunocytology and in a superfusion system. The majority of adenoma cells were ACTH-positive, whereas ACTH-secreting cells of the anterior lobes were mostly inactive and were reduced in number. In vitro, adenomatous tissue showed high ACTH secretion into the superfusion medium, which was increased significantly after vasopressin application. Corticoid feedback was impaired. Anterior lobe cells exhibited a significant spontaneous ACTH secretion that was reduced by cortisol, but not stimulated by vasopressin. These results support the concept of an impaired corticoid feedback at the adenoma level in the presence of suppressed ACTH secretion of the para-adenomatous anterior lobe.

scholarly journals Surgical Remission of Pituitary Adenomas Confined to the Neurohypophysis in Cushing’s Disease

2006 ◽  
Vol 91 (7) ◽  
pp. 2656-2664 ◽  
Author(s):  
Robert J. Weil ◽  
Alexander O. Vortmeyer ◽  
Lynnette K. Nieman ◽  
Hetty L. DeVroom ◽  
John Wanebo ◽  
...  
Keyword(s):  
Body Mass ◽  
Cushing’S Disease ◽  
Anterior Lobe ◽  
Cushing's Disease ◽  
Posterior Lobe ◽  
Repeat Surgery ◽  
Tertiary Referral Center ◽  
Acth Secreting

Abstract Context: Partial or total removal of the pituitary cures 60–80% of patients with Cushing’s disease (CD) in whom an adenoma cannot be identified at surgery. Many patients who fail complete or partial hypophysectomy are cured by sellar and parasellar irradiation. Design/Patients: As part of a series of prospective studies of CD, we identified 12 patients (34.5 ± 19.9 yr; 11 females; four children) with tumors located completely within the neurohypophysis among 730 patients undergoing surgery for CD. Setting: The study was conducted at a tertiary referral center at a clinical research hospital. Results: All 12 patients had clinical and biochemically defined CD. Tumor was visible at surgery in 11 patients; all 12 tumors were positive for ACTH by immunohistochemistry. Two tumors were excised at repeat surgery because of persistent hypercortisolism within 14 d of negative exploration of the adenohypophysis. There were no long-term complications. At follow-up of 71.9 ± 34.2 months (range, 30–138 months), all patients are in remission of CD. Adult patients have had significant improvement in weight and body mass indices, with restoration of normal menses in all women. In the four pediatric patients, height, weight, and body mass indices have been restored toward normal by surgical remission of CD. Hypopituitarism or long-term neurohypophysial dysfunction has not occurred. Conclusion: We report a new subset of patients with CD, ACTH-secreting adenomas that arise wholly within the posterior lobe of the pituitary gland. In cases of CD in which an adenoma is not identified in the adenohypophysis and in patients with persistent hypercortisolism after complete or partial excision of the anterior lobe, tumor within the neurohypophysis should be considered; selective adenomectomy of a neurohypophyseal, ACTH-secreting tumor can produce long-term remission.

Endocrinological evaluation of ACTH-secreting pituitary microadenomas: their location and α-melanocyte stimulating hormone immunoreactivity

1992 ◽  
Vol 76 (6) ◽  
pp. 944-947 ◽  
Author(s):  
Takashi Nagaya ◽  
Akio Kuwayama ◽  
Hisao Seo ◽  
Nobuhiro Tsukamoto ◽  
Nobuo Matsui ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Anterior Lobe ◽  
Cushing's Disease ◽  
Lower Sensitivity ◽  
Intermediate Lobe ◽  
Posterior Lobe ◽  
Content Type ◽  
Melanocyte Stimulating Hormone ◽  
Acth Secreting ◽  
Stimulating Hormone

✓ It has been hypothesized by Lamberts and coworkers in their analysis of 15 cases that adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas may be derived from either the anterior lobe or the intermediate lobe. The intermediate lobe type of Cushing's disease is thought to be controlled through a hypothalamic pathway and is characterized by hyperprolactinemia, suppressibility of Cortisol with bromocriptine, and lower sensitivity to dexamethasone. The authors investigated the validity of this hypothesis in 125 cases of ACTH-secreting pituitary microadenomas by analyzing the endocrine findings, the locations of the microadenomas, and α-melanocyte stimulating hormone (α-MSH) immunoreactivity in the adenoma cells. No significant differences in the basal hormone levels, Cortisol suppressibility with bromocriptine, sensitivity to dexamethasone, and recurrence rate were observed between patients with the microadenoma adjacent to the posterior lobe (considered typical of the intermediate lobe-derived tumor) or those with the microadenoma located in the anterior lobe. The locations of the microadenoma were not correlated with α-MSH immunoreactivity in the adenoma cells. No significant differences in endocrine findings were noticed between adenomas positive or negative for α-MSH. Thus, Cushing's disease cannot be simply divided into either the anterior lobe type or the intermediate lobe type by endocrinological evaluation as described by Lamberts, et al.

scholarly journals Progression of an Invasive ACTH Pituitary Macroadenoma with Cushing’s Disease to Pituitary Carcinoma

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Clarissa Groberio Borba ◽  
Rafael Loch Batista ◽  
Nina Rosa de Castro Musolino ◽  
Vanielle Carvalho Machado ◽  
Ana Elisa Evangelista Alcantara ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Pituitary Carcinoma ◽  
Cushing's Disease ◽  
Resonance Imaging ◽  
Ki 67 ◽  
P53 Expression ◽  
Acth Secreting ◽  
Pituitary Carcinomas

Pituitary carcinomas are very rare tumors that in most cases produce prolactin and adrenocorticotropic hormone (ACTH). It is a challenge to diagnosis of a pituitary carcinoma before disclosed symptomatic metastasis. We report the case of a female patient with Cushing’s disease who underwent three transsphenoidal surgeries, with pathological findings of common ACTH pituitary adenoma including Ki-67 expression <3%. She achieved hypocortisolism after the 3rd surgery although ACTH levels remained slightly elevated. The patient returned some time later with fast worsening of hypercortisolism. Magnetic resonance imaging showed clivus invasion, which led to a fourth surgery and radiation. This time, immunohistochemistry revealed strong Ki-67 (10% to 15%) and p53 expression. Liver and lumbar spine metastases were found on workup. The patient died after few months due to lung infection. Pituitary carcinomas are rare, and the transformation of an ACTH-secreting pituitary adenoma into a carcinoma is exceptional. The difficulty of defining markers for the diagnosis of carcinoma, before metastasis diagnosis, in order to change the management of the disease, is a challenge.

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