Ectopic intracavernous sinus adrenocorticotropic hormone—secreting microadenoma: could this be a common cause of failed transsphenoidal surgery in Cushing disease?

2003 ◽  
Vol 98 (6) ◽  
pp. 1312-1317 ◽  
Author(s):  
Louis J. Kim ◽  
Jeffrey D. Klopfenstein ◽  
Ming Cheng ◽  
Murugasu Nagul ◽  
Stephen Coons ◽  
...  
Keyword(s):  
Transsphenoidal Surgery ◽  
Adrenocorticotropic Hormone ◽  
Sella Turcica ◽  
Cardiac Events ◽  
Cushing Disease ◽  
Rapid Reduction ◽  
Content Type ◽  
Negative Findings ◽  
Acth Secreting ◽  
Fine Print

✓ Despite diagnostic advances, it remains difficult to identify intrasellar and ectopic parasellar adrenocorticotropic hormone (ACTH)—secreting microadenomas. The authors present the case of a 61-year-old woman with Cushing disease in whom a significant central-to-peripheral and lateralized right-sided ACTH gradient was demonstrated on inferior petrosal sinus sampling; no discernible abnormality was seen on magnetic resonance imaging. She underwent transnasal transsphenoidal surgery. No tumor was found on sellar exploration and a total hypophysectomy was performed, yet her hypercortisolemia persisted. The patient died of cardiac events 17 days postsurgery. Autopsy revealed an isolated, right-sided, intracavernous ACTH-secreting adenoma with no intrasellar communication. This case represents the first failed transsphenoidal surgery for Cushing disease in which there is postmortem confirmation of a suspected intracavernous sinus lesion. It supports the hypothesis that Cushing disease associated with nondiagnostic imaging studies, a strong ACTH gradient on venography, and negative findings on sellar exploration may be caused by an ectopic intracavernous ACTH-secreting adenoma. There are no premortem means of confirming the presence of such lesions, but these tumors could underlie similar cases of failed surgery. Radiation therapy targeting the sella turcica and both cavernous sinuses, possibly supplemented with medical treatment, is suggested for similar patients in whom transsphenoidal hypophysectomy has failed. Adrenalectomy may also be appropriate if a rapid reduction in ACTH is necessary.

Transsphenoidal surgery in Cushing disease: 10 years of experience in 34 consecutive cases

2004 ◽  
Vol 100 (4) ◽  
pp. 634-638 ◽  
Author(s):  
Charlotte Höybye ◽  
Eva GrenbäcK ◽  
Marja Thorén ◽  
Anna-Lena Hulting ◽  
Lars Lundblad ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Transsphenoidal Surgery ◽  
Cushing Syndrome ◽  
Preoperative Evaluation ◽  
Clinical Signs ◽  
Pituitary Hormone ◽  
Years Of Experience ◽  
Cushing Disease ◽  
Content Type ◽  
Fine Print

Object. Cushing disease is a rare disorder. Because of their small size the adrenocorticotropic hormone (ACTH)—producing tumors are often not detectable on neuroimaging studies. To obtain a cure with transsphenoidal surgery (TSS) may therefore be difficult. In this report the authors present 10 years of experience in the treatment of patients with Cushing disease who were followed up with the same protocol and treated by the same surgeon. Methods. Thirty-four patients, 26 of them female and eight of them male (mean age 40 years, range 13–74 years) were studied. All had obvious clinical signs and symptoms of Cushing syndrome. Magnetic resonance (MR) imaging was performed in all patients, and inferior petrosal sinus (IPS) sampling was done in 14. In 12 patients MR imaging indicated a pituitary tumor; 10 were microadenomas and two were macroadenomas. In six patients with no visible tumor, the results of IPS sampling supported the diagnosis. All patients underwent TSS; the mean follow-up duration was 6 ± 0.5 years. Selective adenomectomy was performed in 32 and hemihypophysectomy in the other two patients. A cure was obtained in 31 patients (91%) after one TSS and in two more patients after further TSS; one patient was not cured despite two TSSs and one underwent bilateral adrenalectomy. Disease recurrence was seen in two patients after 3 years, and they were successfully treated with stereotactic gamma knife surgery. Half of the patients had an ACTH deficiency postoperatively, whereas one third had other pituitary hormone insufficiencies. There were no serious complications attributable to the surgical intervention. Conclusions. Transsphenoidal surgery with selective adenomectomy is an effective and safe treatment for Cushing disease. In the patients presented in this study, the surgical outcome seemed to depend on careful preoperative evaluation and the surgeon's experience. For optimal results in this rare disease the authors therefore suggest that the endocrinological, radiological, and surgical procedures be coordinated in a specialized center.

Thickening of sphenoid sinus mucosa during the acute stage of pituitary apoplexy

2001 ◽  
Vol 95 (5) ◽  
pp. 897-901 ◽  
Author(s):  
Kazunori Arita ◽  
Kaoru Kurisu ◽  
Atushi Tominaga ◽  
Kazuhiko Sugiyama ◽  
Fusao Ikawa ◽  
...  
Keyword(s):  
Transsphenoidal Surgery ◽  
Sphenoid Sinus ◽  
Pituitary Apoplexy ◽  
Sella Turcica ◽  
Acute Stage ◽  
Mr Images ◽  
Content Type ◽  
Subepithelial Layer ◽  
Sinus Mucosa ◽  
Fine Print

✓ The authors treated two patients with pituitary apoplexy in whom magnetic resonance (MR) images were obtained before and after the episode. Two days after the apoplectic episodes, MR imaging demonstrated marked thickening of the mucosa of the sphenoid sinus that was absent in the previous studies. The relevance of this change in the sphenoid sinus was investigated. Retrospective evaluations were performed using MR images obtained in 14 consecutive patients with classic pituitary apoplexy characterized by acute onset of severe headache. The mucosa of the sphenoid sinus had thickened predominantly in the compartment just beneath the sella turcica, in nine of 11 patients, as ascertained on MR images obtained within 7 days after the onset of apoplectic symptoms. This condition improved spontaneously in all four patients who did not undergo transsphenoidal surgery. The sphenoid sinus mucosa appeared to be normal on MR images obtained from three patients at the chronic stage (> 3 months after onset). The incidence of sphenoid sinus mucosal thickening during the acute stage was significantly higher in the patients with apoplexy than that in the 100 patients without apoplexy. A histological study conducted in four patients who underwent transsphenoidal surgery during the early stage showed that the subepithelial layer of the sphenoid sinus mucous membrane was obviously swollen. The sphenoid sinus mucosa thickens during the acute stage of pituitary apoplexy. This thickening neither indicates infectious sinusitis nor rules out the choice of the transsphenoidal route for surgery.

Surgical treatment of Cushing's disease

1987 ◽  
Vol 66 (2) ◽  
pp. 204-212 ◽  
Author(s):  
William F. Chandler ◽  
David E. Schteingart ◽  
Ricardo V. Lloyd ◽  
Paul E. McKeever ◽  
Gloria Ibarra-Perez
Keyword(s):  
Cushing’S Disease ◽  
Immunohistochemical Staining ◽  
Adrenocorticotropic Hormone ◽  
Cushing's Disease ◽  
Content Type ◽  
Acth Secreting ◽  
Endocrine Symptoms ◽  
Lung Carcinoid ◽  
Fine Print ◽  
Pharyngeal Pituitary

✓ The endocrinological, radiological, surgical, and pathological experience with 34 consecutive patients operated on for Cushing's disease is presented. Preoperative endocrine evaluation demonstrated that 19 patients had “typical” endocrine patterns for pituitary adrenocorticotropic hormone (ACTH)-dependent hypercortisolism and 11 had “atypical” testing. Pituitary pathology was found in 95% of the patients with typical preoperative endocrine testing but in only 55% of those with atypical testing. Eight of 34 preoperative computerized tomography scans demonstrated tumors, which correlated in all instances with the surgical findings. Microadenomas were removed from 25 patients, 22 of whom achieved postoperative remission of endocrine symptoms. Macroadenomas were found in three patients, only one of whom achieved remission after surgery. One patient had removal of an adenoma in the pharyngeal pituitary, and in another an ectopic lung carcinoid was excised; both of these patients were in postoperative remission. Four patients underwent transsphenoidal exploration but no definitive explanation was found for their hypercortisolism; these patients were not in remission. Immunohistochemical staining was performed on every specimen and all tumors showed excessive ACTH-secreting cells. A new rapid stain of the fibrovascular stroma is described.

Primary pituitary aspergillosis responding to transsphenoidal surgery and combined therapy with amphotericin-B and 5-fluorocytosine

1981 ◽  
Vol 54 (6) ◽  
pp. 839-841 ◽  
Author(s):  
Angelita Ramos-Gabatin ◽  
Richard M. Jordan
Keyword(s):  
Cerebrospinal Fluid ◽  
Amphotericin B ◽  
Transsphenoidal Surgery ◽  
Combined Therapy ◽  
Sella Turcica ◽  
Single Patient ◽  
Pituitary Abscess ◽  
Content Type ◽  
Elevated Protein ◽  
Fine Print

✓ Pituitary abscess is an unusual cause of sella turcica enlargement. Because its presentation closely mimics that of a pituitary tumor, the condition is seldom recognized preoperatively. Most cases have been of bacterial etiology; however, a single patient with a primary mycotic pituitary abscess secondary to Aspergillus species has been reported. That patient died of diffuse Aspergillus meningoencephalitis following a transfrontal craniotomy. In the present case, a woman with primary pituitary aspergillosis survived her infection with virtually intact pituitary function following a transsphenoidal approach which avoided contamination of cerebrospinal fluid. Postoperative amphotericin-B and 5-fluorocytosine therapy probably contributed greatly to her survival. Factors that should alert the clinician to the presence of a pituitary abscess in a patient with sella turcica enlargement are prior episodes of meningitis, sinusitis, or cerebrospinal fluid abnormalities, including pleocytosis, depressed glucose, and elevated protein.

Surgical management of adrenocorticotropic hormone—secreting macroadenomas: outcome and challenges in patients with Cushing's disease or Nelson's syndrome

2005 ◽  
Vol 103 (5) ◽  
pp. 825-830 ◽  
Author(s):  
Claudio De Tommasi ◽  
Mary Lee Vance ◽  
David O. Okonkwo ◽  
Alfa Diallo ◽  
Edward R. Laws
Keyword(s):  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Signs And Symptoms ◽  
Disease Recurrence ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Content Type ◽  
Nelson's Syndrome ◽  
Acth Secreting ◽  
Fine Print

Object. Adrenocorticotropic hormone (ACTH)—secreting pituitary macroadenomas are an uncommon cause of Cushing's disease (CD) and, subsequently, Nelson's syndrome (NS). They have been associated with low postoperative remission rates. The outcome of modern surgical treatment is unclear and thus was assessed in a series of 43 patients, with the goal of improving therapeutic results in patients with ACTH-secreting macroadenomas. Methods. Thirty-seven patients presented with CD and six with NS. They represented 15% of the patients surgically treated at the authors' institution for ACTH-secreting adenomas. The median patient age was 38 years (range 14–71 years), and the mean duration of follow up was 37 months (range 1–108 months). Remission occurred in 25 (67.6%) of 37 patients with CD, whereas the disease persisted in 12 (32.4%) of 37 patients. After an initial remission, three (12%) of 25 patients demonstrated signs and symptoms indicative of disease recurrence. Among patients with NS, only one (16.6%) displayed remission. Invasion of the dura mater by tumor was histologically demonstrated in 10 patients with CD and in two patients with NS. Conclusions. Comprehensive management of CD caused by ACTH-secreting macroadenomas through the appropriate use of combination therapy, including surgery, radiotherapy, radiosurgery, and adrenalectomy, can lead to outcomes similar to those for microadenomas. Disease recurrence and persistence rates are higher, often because of the invasiveness associated with macroadenomas. Remission of NS-associated macroadenomas is difficult to achieve. Strategies for the characterization and treatment of invasive macroadenomas are needed.

Delayed cure of Cushing's disease after transsphenoidal surgery of pituitary microadenomas

1978 ◽  
Vol 49 (4) ◽  
pp. 593-596 ◽  
Author(s):  
Stephen D. McDonald ◽  
Stanley E. Von Hofe ◽  
Steven G. Dorfman ◽  
Richard M. Jordan ◽  
James R. LaMorgese ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Normal Tissue ◽  
Adrenocorticotropic Hormone ◽  
Early Postoperative Period ◽  
Cushing's Disease ◽  
Content Type ◽  
Pituitary Microadenoma ◽  
Dexamethasone Suppression ◽  
Fine Print

✓ Transsphenoidal microdissection has been proposed as a preferred means of treating Cushing's disease. This procedure allows the surgeon to remove a pituitary microadenoma and at the same time to preserve normal tissue. Two cases described here were treated by this method. An interesting and important observation was that neither patient appeared to be cured for 2 to 6 weeks after surgery, as assessed by dexamethasone suppression. Later, normal suppressibility occurred and the course of each patient was compatible with cure. Patients treated by this method should not be automatically retreated because of adrenocorticotropic hormone (ACTH) non-suppressibility in the early postoperative period.

Transsphenoidal surgery of intrasellar germinomas

1983 ◽  
Vol 59 (6) ◽  
pp. 1063-1066 ◽  
Author(s):  
David S. Baskin ◽  
Charles B. Wilson
Keyword(s):  
Diabetes Insipidus ◽  
Transsphenoidal Surgery ◽  
Sella Turcica ◽  
Young Patients ◽  
Content Type ◽  
Patients With Diabetes ◽  
Transsphenoidal Operation ◽  
Diagnostic Possibility ◽  
Fine Print

✓ Two patients with diabetes insipidus, hypopituitarism, and an enlarged sella turcica underwent a transsphenoidal operation for the treatment of intrasellar germinomas. Successful transsphenoidal treatment of such neoplasms has not been reported previously. The cases indicate that the diagnostic possibility of intrasellar germinoma should be considered in young patients with combined diabetes insipidus and hypopituitarism, even when the sella is markedly expanded.

Transsphenoidal decompression of the optic nerve and chiasm

1977 ◽  
Vol 46 (6) ◽  
pp. 717-722 ◽  
Author(s):  
Edward R. Laws ◽  
James C. Trautmann ◽  
Robert W. Hollenhorst
Keyword(s):  
Pituitary Adenoma ◽  
Optic Nerve ◽  
Transsphenoidal Surgery ◽  
Visual Loss ◽  
Sella Turcica ◽  
Recent Experience ◽  
Content Type ◽  
Visual Status ◽  
Fine Print

✓ A review of recent experience with transsphenoidal surgery for lesions in and about the sella turcica establishes the value of this approach for the management of patients with visual loss. The lesions encountered consisted of pituitary adenoma in 45 cases, craniopharyngioma in 10 cases, and miscellaneous tumors involving the sella in the remaining seven cases. Sixty of the 62 patients in this series had quantitative determination of preoperative and postoperative visual status; after surgery, vision was improved in 81%, unchanged in 11%, and worse in 5%. Two patients (3%) died during the immediate postoperative period before their visual status could be evaluated.

Sellar reconstruction with resorbable vicryl patches, gelatin foam, and fibrin glue in transsphenoidal surgery: a 10-year experience with 376 patients

2000 ◽  
Vol 93 (5) ◽  
pp. 762-765 ◽  
Author(s):  
Rolf W. Seiler ◽  
Luigi Mariani
Keyword(s):  
Fibrin Glue ◽  
Transsphenoidal Surgery ◽  
Operating Time ◽  
Sella Turcica ◽  
Morbidity Rate ◽  
Surgical Incision ◽  
Content Type ◽  
Csf Leakage ◽  
Sellar Reconstruction ◽  
Fine Print

Object. Closure of the sella turcica after transsphenoidal surgery is mainly accomplished with autologous muscle fascia and fat or muscle; this requires a second surgical incision. The authors review the results of using resorbable vicryl patches, gelatin foam, and fibrin glue for sellar reconstruction.Methods. A review was conducted of 376 consecutive patients who underwent surgery for pituitary adenomas, cysts, or subdiaphragmatic craniopharyngiomas in the sella turcica that the senior author (R.W.S.) had performed or directly supervised over the last 10 years. The sellar reconstruction was performed with a commercially available, synthetic absorbable patch composed of polyglactin 910/poly-p-dioxanone, gelatin foam, and fibrin glue. The patch is essentially resorbed in 2 to 3 months and replaced by fibrous collagen tissue. There were 117 small, 112 medium-sized, and 147 large lesions. The overall nonendocrine postoperative morbidity rate was 2.8%, and included visual deterioration, meningitis, secondary epistaxis, nasal septum complication, and cerebrospinal fluid (CSF) leakage. Two patients with macroadenomas needed reoperation for persistent CSF leakage, which comprised 0.5% of the whole series or 0.8% of the 259 patients with medium-sized or large lesions. There was no mortality and no morbidity related to the implanted material, and in particular no delayed empty sella syndrome.Conclusions. Closure of the sella turcica with a synthetic absorbable vicryl patch, gelatin foam, and fibrin glue after transsphenoidal surgery is safe and very effective in preventing postoperative CSF fistulas. The use of this technique obviates the need for a second surgical incision and shortens the operating time. Because of the progressive resorption of the substitute material, the interpretation of postoperative magnetic resonance studies was not significantly hindered.

Suture knot on the repair splint: a simple method to facilitate reconstruction of the sella turcica during endonasal endoscopic transsphenoidal surgery

2005 ◽  
Vol 102 (5) ◽  
pp. 938-939 ◽  
Author(s):  
Shigeki Kubo ◽  
Hiroshi Hasegawa ◽  
Toshihiko Inui ◽  
Shinsuke Tominaga ◽  
Toshiki Yoshimine
Keyword(s):  
Transsphenoidal Surgery ◽  
Sella Turcica ◽  
Endoscopic Approach ◽  
Tumor Removal ◽  
Endoscopic Transsphenoidal Surgery ◽  
Simple Method ◽  
Content Type ◽  
Narrow Space ◽  
Sellar Floor ◽  
Fine Print

✓ Reconstruction of the sellar floor after pituitary tumor removal is sometimes difficult because the repair graft is difficult to handle in the narrow space. This is especially problematic if the endonasal endoscopic approach is used. The authors devised a technique to facilitate this procedure by placing a suture knot on the repair splint. This allows the material to be grasped securely with forceps and improves manipulation even within the narrow nasal cavity. This technique has proved useful when performing the endonasal endoscopic approach, and it is also expected to be useful when conducting the conventional sublabial transsphenoidal approach.

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