Neurosurgical implications of Carney complex

2000 ◽  
Vol 92 (3) ◽  
pp. 413-418 ◽  
Author(s):  
Joe C. Watson ◽  
Constantine A. Stratakis ◽  
Peter K. Bryant-Greenwood ◽  
Christian A. Koch ◽  
Lawrence S. Kirschner ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Pituitary Adenomas ◽  
Carney Complex ◽  
Cushing's Syndrome ◽  
Spinal Tumors ◽  
Common Component ◽  
Content Type ◽  
Cardiac Myxomas ◽  
Insight Into ◽  
Fine Print

Object. The authors present their neurosurgical experience with Carney complex. Carney complex, characterized by spotty skin pigmentation, cardiac myxomas, primary pigmented nodular adrenocortical disease, pituitary tumors, and nerve sheath tumors (NSTs), is a recently described, rare, autosomal-dominant familial syndrome that is relatively unknown to neurosurgeons. Neurosurgery is required to treat pituitary adenomas and a rare NST, the psammomatous melanotic schwannoma (PMS), in patients with Carney complex. Cushing's syndrome, a common component of the complex, is caused by primary pigmented nodular adrenocortical disease and is not secondary to an adrenocorticotropic hormone-secreting pituitary adenoma.Methods. The authors reviewed 14 cases of Carney complex, five from the literature and nine from their own experience. Of the 14 pituitary adenomas recognized in association with Carney complex, 12 developed growth hormone (GH) hypersecretion (producing gigantism in two patients and acromegaly in 10), and results of immunohistochemical studies in one of the other two were positive for GH. The association of PMSs with Carney complex was established in 1990. Of the reported tumors, 28% were associated with spinal nerve sheaths. The spinal tumors occurred in adults (mean age 32 years, range 18–49 years) who presented with pain and radiculopathy. These NSTs may be malignant (10%) and, as with the cardiac myxomas, are associated with significant rates of morbidity and mortality.Conclusions. Because of the surgical comorbidity associated with cardiac myxoma and/or Cushing's syndrome, recognition of Carney complex has important implications for perisurgical patient management and family screening. Study of the genetics of Carney complex and of the biological abnormalities associated with the tumors may provide insight into the general pathobiological abnormalities associated with the tumors may provide insight into the general pathobiological features of pituitary adenomas and NSTs.

Cavernous sinus sampling in patients with adrenocorticotrophic hormone—dependent Cushing's syndrome with emphasis on inter- and intracavernous adrenocorticotrophic hormone gradients

1998 ◽  
Vol 89 (5) ◽  
pp. 762-768 ◽  
Author(s):  
Akira Teramoto ◽  
Yoichi Yoshida ◽  
Naoko Sanno ◽  
Shigeru Nemoto
Keyword(s):  
Standard Deviation ◽  
Cavernous Sinus ◽  
Cushing’S Syndrome ◽  
False Negative ◽  
Cushing's Syndrome ◽  
Adrenocorticotrophic Hormone ◽  
Posterior Portion ◽  
Content Type ◽  
Catheterization Technique ◽  
Fine Print

Object. As an alternative method to inferior petrosal sinus sampling with administration of corticotropin-releasing hormone (CRH), the authors have developed a method of selective venous sampling directly from the cavernous sinus, which is performed using a superselective catheterization technique. The goal of this study is to assess this method. Methods. Catheterization to the cavernous sinus was performed in 44 patients with clinical and biochemical features of Cushing's syndrome. Forty of these patients in whom there was a definite clinical and/or histological diagnosis were evaluated. In 35 patients, a pituitary microadenoma was detected on magnetic resonance imaging and/or during surgery. Ectopic lesions were suspected in five patients, who later developed nonpituitary tumors. The central/peripheral (C/P) ratios of adrenocorticotrophic hormone (ACTH) without CRH administration ranged from 5.2 to 448.1 (mean ± standard deviation, 66.7 ± 91.2) in cavernous sinuses and from 1.1 to 52 (mean ± standard deviation, 8.5 ± 9.9) in the inferior petrosal sinuses. The petrosal sinus sampling contained false negative results in eight patients (23%) based on a cutoff point of 2. On the contrary, patients with ectopic lesions did not show any increase in ACTH levels in either sampling portion. The intercavernous gradients of ACTH, ranging from 1.2 to 506.4, indicated the correct lateralization of microadenoma in the lateral wing in all 30 patients. The interpetrosal gradients, ranging from 1 to 31.4, did not give a sufficient value (≥ 1.4) in four patients (13%) with a lateral lesion. In five cases of midline tumors, the intercavernous and interpetrosal gradients indicated false laterality in three and four cases, respectively. The intracavernous (posterior—anterior) gradients, ranging from 1.04 to 60.7 (mean 14.9), showed a higher concentration of ACTH in the posterior portion of the sinus in all patients. Conclusions. These results suggest that 1) cavernous sinus sampling without CRH administration can demonstrate hypersecretion of ACTH from the pitutary gland with a high diagnostic accuracy; 2) intercavernous gradients will indicate the correct lateralization in laterally localized microadenomas; and 3) sampling should be performed from the posterior portion of the cavernous sinus.

Gastrin in pituitary tumours

1987 ◽  
Vol 115 (3) ◽  
pp. 419-422 ◽  
Author(s):  
Linda Bardram ◽  
Jörgen Lindholm ◽  
Jens F. Rehfeld
Keyword(s):  
Cushing’S Syndrome ◽  
Pituitary Adenomas ◽  
Cushing's Syndrome ◽  
Pituitary Tumours ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

Abstract. Twelve of 87 pituitary adenomas from patients with acromegaly, Cushing's syndrome, Nelson's syndrome, hyperprolactinaemia and without symptoms of hormone hypersecretion contained gastrin in concentrations from 0.5 to 166 pmol/g. Only ACTH-producing tumours contained gastrin, which occurred in forms smaller than those present in the normal adenohypophysis. The results indicate that corticotropic tumours may synthesize gastrin in moderate amounts.

Recurrent intracranial solitary fibrous tumor with cerebrospinal fluid dissemination

2004 ◽  
Vol 101 (6) ◽  
pp. 1045-1048 ◽  
Author(s):  
Katsuyoshi Miyashita ◽  
Yutaka Hayashi ◽  
Hironori Fujisawa ◽  
Mitsuhiro Hasegawa ◽  
Junkoh Yamashita
Keyword(s):  
Cerebrospinal Fluid ◽  
Malignant Transformation ◽  
Solitary Fibrous Tumor ◽  
S100 Protein ◽  
Spinal Tumors ◽  
Content Type ◽  
Csf Dissemination ◽  
Fibrous Tumor ◽  
Fine Print ◽  
Mib 1

✓ Solitary fibrous tumor (SFT) is a benign and rare neoplasm. To date, only 37 patients with intracranial SFTs have been reported. Although a number of the tumors were recurrent and some later underwent malignant transformation, none of these lesions progressed to cerebrospinal fluid (CSF) dissemination. In this paper the authors report a case of SFT in which the lesion recurred several times and ultimately was disseminated by the CSF. The patient was a 63-year-old woman with multiple intracranial and spinal tumors. Fifteen years before this presentation, at the age of 48 she had been hospitalized for resection of a falcotentorial tumor. During the ensuing 15 years she underwent multiple surgeries and sessions of radiation therapy for recurrent lesions. The exclusive location of her tumors in the subarachnoid space at the end of this 15-year period indicate CSF dissemination of the tumor. The tumor that was resected when the patient was 48 years old and the latest resected lesion were analyzed by performing immunohistological CD34, epithelial membrane antigen, vimentin, S100 protein, and reticulin staining, and determining the MIB-1 labeling index (LI). Most of the results were identical, and both tumors were diagnosed as SFT according to a staining pattern that showed a strong and diffuse positive reaction for CD34. Nevertheless, the authors noted that the MIB-1 LI increased from less than 1% in the original tumor to 13% in the latest tumor. The increased proliferation of MIB-1 indicates that the malignant transformation could have occurred during tumor recurrence with CSF dissemination.

Monostotic fibrous dysplasia of the thoracic spine: clinopathological description and follow up

2004 ◽  
Vol 100 (4) ◽  
pp. 378-381 ◽  
Author(s):  
Mehmet Arazi ◽  
Onder Guney ◽  
Mustafa Ozdemir ◽  
Omer Uluoglu ◽  
Nuket Uzum
Keyword(s):  
Fibrous Dysplasia ◽  
Thoracic Spine ◽  
Histopathological Examination ◽  
En Bloc Resection ◽  
Spinal Tumors ◽  
En Bloc ◽  
Content Type ◽  
Thoracic Spinal ◽  
Fine Print

✓ The authors report the case of a 53-year-old woman with monostotic fibrous dysplasia of the thoracic spine. The patient presented with a 1-month history of pain in the thoracic spinal region. En bloc resection of the lesion was successfully performed via a transthoracic approach, and a histopathological examination confirmed the diagnosis of fibrous dysplasia. At 24-month follow-up examination, pain and vertebral instability were absent. The findings in this case illustrate that, although very rare, monostotic fibrous dysplasia of the thoracic spine should be considered in the differential diagnosis of spinal tumors. Although a consensus for management of this disease has not been achieved, the authors recommend radical removal of all involved bone as well as internal fixation or bone graft—assisted fusion to achieve long-term stabilization.

Thyrotrophin-producing pituitary adenomas

1982 ◽  
Vol 57 (4) ◽  
pp. 515-519 ◽  
Author(s):  
Stephen A. Hill ◽  
James M. Falko ◽  
Charles B. Wilson ◽  
William E. Hunt
Keyword(s):  
Thyroid Hormone ◽  
Pituitary Adenomas ◽  
Preoperative Diagnosis ◽  
Pituitary Tumors ◽  
Recurrent Tumor ◽  
Hormone Production ◽  
Content Type ◽  
Aggressive Tumor ◽  
Tsh Levels ◽  
Fine Print

✓ Hyperthyroidism due to thyrotrophin (TSH)-secreting pituitary tumors is rare. Four cases are described, with the features that allow preoperative diagnosis. In all the patients, thyroid hormone production was consistently elevated despite antithyroid therapy, and TSH levels were inappropriately elevated. All patients were treated with both surgery and irradiation. Each patient had recurrent tumor with suprasellar, intrasphenoidal, or intraorbital spread. The combination of a recurrent, aggressive tumor complicated by thyrotoxicosis makes this a complex and difficult surgical problem.

Reticulin stain in differentiating astrocytomas from neurilemmomas on frozen sections

1979 ◽  
Vol 51 (1) ◽  
pp. 124-125 ◽  
Author(s):  
John J. Kepes ◽  
Robert A. Morantz ◽  
Anna Marie England
Keyword(s):  
Pituitary Adenomas ◽  
Frozen Sections ◽  
Content Type ◽  
Fine Print

✓ The authors recommend the use of a reticulin stain for frozen sections to differentiate between astrocytomas and neurilemmomas during surgery. This technique was first described for the diagnosis of pituitary adenomas.

Long-term results of gamma knife surgery for growth hormone—producing pituitary adenoma: is the disease difficult to cure?

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 119-123 ◽  
Author(s):  
Tatsuya Kobayashi ◽  
Yoshimasa Mori ◽  
Yukio Uchiyama ◽  
Yoshihisa Kida ◽  
Shigeru Fujitani
Keyword(s):  
Growth Hormone ◽  
Gamma Knife ◽  
Pituitary Adenomas ◽  
Gamma Knife Surgery ◽  
Long Term Results ◽  
Content Type ◽  
The Mean ◽  
Dose Radiation ◽  
Fine Print

Object. The authors conducted a study to determine the long-term results of gamma knife surgery for residual or recurrent growth hormine (GH)—producing pituitary adenomas and to compare the results with those after treatment of other pituitary adenomas. Methods. The series consisted of 67 patients. The mean tumor diameter was 19.2 mm and volume was 5.4 cm3. The mean maximum dose was 35.3 Gy and the mean margin dose was 18.9 Gy. The mean follow-up duration was 63.3 months (range 13–142 months). The tumor resolution rate was 2%, the response rate 68.3%, and the control rate 100%. Growth hormone normalization (GH < 1.0 ng/ml) was found in 4.8%, nearly normal (< 2.0 ng/ml) in 11.9%, significantly decreased (< 5.0 ng/ml) in 23.8%, decreased in 21.4%, unchanged in 21.4%, and increased in 16.7%. Serum insulin-like growth factor (IGF)—1 was significantly decreased (IGF-1 < 400 ng/ml) in 40.7%, decreased in 29.6%, unchanged in 18.5%, and increased in 11.1%, which was almost parallel to the GH changes. Conclusions. Gamma knife surgery was effective and safe for the control of tumors; however, normalization of GH and IGF-1 secretion was difficult to achieve in cases with large tumors and low-dose radiation. Gamma knife radiosurgery is thus indicated for small tumors after surgery or medication therapy when a relatively high-dose radiation is required.

Multiple pituitary adenomas in Cushing's disease

2000 ◽  
Vol 93 (5) ◽  
pp. 753-761 ◽  
Author(s):  
John K. Ratliff ◽  
Edward H. Oldfield
Keyword(s):  
Cushing’S Disease ◽  
Medical Records ◽  
Pituitary Adenomas ◽  
Cushing's Disease ◽  
Clinical Database ◽  
Null Cell ◽  
Content Type ◽  
Initial Surgery ◽  
Repeated Surgery ◽  
Fine Print

Object. Clinically evident multiple pituitary adenomas rarely occur. The authors assess the incidence and clinical relevance of multiple adenomas in Cushing's disease.Methods. A prospective clinical database of 660 pituitary surgeries was analyzed to assess the incidence of multiple pituitary adenomas in Cushing's disease. Relevant radiographic scans, medical records, and histopathological reports were reviewed.Thirteen patients with at least two separate histopathologically confirmed pituitary adenomas were identified. Prolactinomas (nine patients) were the most common incidental tumors. Other incidental tumors included secretors of growth hormone ([GH], one patient) and GH and prolactin (two patients), and a null-cell tumor (one patient). In two patients, early repeated surgery was performed because the initial operation failed to correct hypercortisolism, in one instance because the tumor excised at the initial surgery was a prolactinoma, not an adrenocorticotropic hormone—secreting tumor. One patient had three distinct tumors.Conclusions. Multiple pituitary adenomas are rare, but may complicate management of patients with pituitary disease.

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