scholarly journals The Coexistence of an Intrasellar Adenoma, Lymphocytic Hypophysitis, and Primary Pituitary Lymphoma in a Patient with Acromegaly

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Jose Hernan Martinez ◽  
Mariel Davila Martinez ◽  
Marcos Mercado de Gorgola ◽  
Luis F. Montalvo ◽  
Jaime E. Tome

The concomitant presence of three histopathologically different entities in the pituitary gland is a rare occurrence. Most publications identify at least two distinct pathologies, mainly, a pituitary adenoma coexisting with a second intrasellar lesion. We present a case of a 71-year-old female referred for evaluation and treatment of acromegaly. Questioning revealed she was experiencing facial palsy, visual disturbances, and syncopal spells for several weeks. When laboratory evaluation showed elevated somatomedin (IGF-I) levels and an oral glucose tolerance test failed to demonstrate any suppression of her growth hormone (GH) values, an MRI of the pituitary revealed a sellar mass. A presumptive diagnosis of pituitary adenoma was established. The patient underwent transsphenoidal resection of the sellar mass, which proved to be a large B-cell lymphoma (Stage I-E) associated with areas of adenoma and lymphocytic hypophysitis.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Satoshi Yamagata ◽  
Shinobu Takayasu ◽  
Koshi Makita ◽  
Kazunori Kageyama ◽  
Yuta Okawa ◽  
...  

Abstract Background: McCune-Albright syndrome (MAS) is a rare disorder. MAS is caused by an activating postzygotic somatic mutation in the GNAS, and, is classically defined by the occurrence of fibrous dysplasia (FD), café-au-lait skin macules, and precocious puberty. Autonomous GH and/or PRL production in MAS has been reported. However, there have been no reports of ACTH excess in MAS. Method: Plasma ACTH and serum cortisol (F) levels were assessed using electrochemiluminescence immunoassays (Eclusys ACTHTM and Eclusys Cortisol IITM, respectively; Roche Diagnostics K.K., Tokyo, Japan).Clinical Cases: Case1; 42-year-old man showed craniofacial deformities and suffered from multiple bone fractures. He was diagnosed with FD at the age of 23 years. Café-au-lait macules were found on his back. He had slightly acromegaloid features. He showed no cushingoid features. Pituitary adenoma or hyperplasia was not detected by MRI. The diagnosis of GH excess was confirmed by no suppression of serum GH levels by a 75-g oral glucose tolerance test (nadir GH: 2.34 ng/mL) and an elevated serum IGF-I level (307 ng/mL; normal range: 92-257 ng/mL). The patient was treated with monthly subcutaneous lanreotide injection and then GH excess was well controlled. Basal ACTH and F levels in blood were 40.6-63.4 pg/mL and 8.0-10.5 μg/dL, respectively. The urinary free cortisol (UFC) level was 53 μg/day. Autonomous F excess was excluded by the level of midnight F (1.2 μg/dL) and the level of F (0.2 μg/dL) after a low-dose (1 mg) dexamethasone suppression test (DST). Case2; A 32-year-old man was diagnosed with MAS and gigantism at the Pediatrics Department at the age of 5 years. Treatment of GH excess was well controlled by monthly octreotide depot. He had no acromegaloid features and no cushingoid features. Café-au-lait macules were observed from the left flank to the back. Pituitary adenoma or hyperplasia was not detected by MRI. Basal ACTH and F levels in blood were 35.5-73.1 pg/mL and 7.0-11.7 μg/dL, respectively. The UFC level was 61 μg/day. Autonomous F excess was excluded by the level of F (<0.2 μg/dL) after a low-dose (0.5 mg) DST.Possibility of primary adrenal insufficiency was excluded by ACTH stimulation test and/or insulin tolerance test in both cases. The involvement of 11β-HSD1 by GH excess and PC1/3 deficiency were also excluded. Gel exclusion chromatography was then performed. POMC and pro-ACTH were detected and the aberrant ACTH/normal ACTH ratio was 42% in both cases. Conclusion: This is the first report of the presence of aberrant ACTH precursors, particularly POMC, in MAS. A high ratio of circulating ACTH to F may suggest secretion of inactive ACTH precursors in MAS. Further investigations are required to determine whether GNAS mutations or other mechanisms are involved in the presence of aberrant ACTH precursors in MAS.


2019 ◽  
Vol 7 (1) ◽  
pp. 31-34
Author(s):  
Kaveh Ebrahimzadeh ◽  
Kurosh Mojtabavi ◽  
Sasan Mohammadi ◽  
Mehrdad Farahani ◽  
Zohreh Gholizadeh Ghozloujeh ◽  
...  

Background: Acromegaly defines as chronic elevations of insulin-like growth factor-1 (IGF-1) and growth hormone (GH), which results in enlargement of organs and soft tissues. Cardiovascular complications of acromegaly such as cardiomegaly, hypertension contributing to a high risk of cardiovascular events. This study aimed to identify the determinants of the prevalence of cardiomegaly as cardiovascular comorbidity of acromegaly and the potential effect of the curative intervention. Methods: A total of 160 patients with acromegaly due to pituitary adenoma participated in this study. Acromegaly diagnosed was based on clinical manifestations, age-adjusted plasma IGF-I, and elevated plasma GH levels, not suppressible during an oral glucose tolerance test (75 g). Electrocardiogram and chest X-ray obtained in all patients. Treatment approaches included transsphenoidal surgery and hormonal evaluation performed before and six months after surgery. Results: The GH serum level was elevated in all patients before surgery, with a mean of 33.7 ng/ mL that reached 5.7 ng/mL after surgery. Mean IGF-1 was 794.1 ng/mL that reached 395.6 ng/mL postoperatively. The prevalence of cardiomegaly was 15% that improved in 5% of patients after trans sphenoidal surgery. Conclusion: Appropriate surgical intervention in acromegaly that complicated by cardiomegaly may result in significant improvement of the cardiac structure.


Author(s):  
Shinichiro Teramoto ◽  
Yuichi Tange ◽  
Hisato Ishii ◽  
Hiromasa Goto ◽  
Ikuko Ogino ◽  
...  

Summary A 67-year-old woman with a past history of type 2 diabetes mellitus presented with worsening glycemic control. She had some acromegaly symptoms and magnetic resonance imaging demonstrated a pituitary tumor. Endocrinological examination found the resting growth hormone (GH) level within the normal range, but elevated insulin-like growth factor 1 level. A 75 g oral glucose tolerance test showed inadequate suppression of nadir GH levels. Acromegaly due to GH-secreting pituitary tumor was diagnosed. The patient underwent endoscopic transsphenoidal surgery resulting in gross total removal of the tumor and recovered well postoperatively. Histological examination of the tumor showed coexistence of relatively large gangliocytoma cells and pituitary adenoma cells, suggesting mixed gangliocytoma-pituitary adenoma. In addition, colocalization of GH and GH-releasing hormone (GHRH) in pituitary adenoma cells was revealed, so the adenomatous components were more likely to produce GHRH in our mixed gangliocytoma-pituitary adenoma case. Mixed gangliocytoma-pituitary adenoma is very rare, and the present unique case demonstrated only the adenomatous components associated with GHRH production. Learning points: Sellar gangliocytoma coexisting with pituitary adenoma is recognized as a mixed gangliocytoma-pituitary adenoma and is very rare. A proposed developmental mechanism of growth hormone (GH)-secreting mixed gangliocytoma-pituitary adenoma involves GH-releasing hormone (GHRH) produced by the gangliocytic components promoting the growth of tumor including GH-secreting adenomatous components. Since our present case indicated that the adenomatous components of mixed gangliocytoma-pituitary adenoma could secrete both GH and GHRH simultaneously, progression of GH-secreting mixed gangliocytoma and pituitary adenoma may involve exposure to spontaneously produced GHRH due to the adenomatous components.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A886-A886
Author(s):  
Aurelie Pare ◽  
Michael Tamilia

Abstract Background: We present a case of coexisting acromegaly and medullary thyroid cancer. As both conditions are rare, the association raises the possibility of causality (non-genomic) as opposed to pure coincidence. Clinical Lesson: A 73 year-old man presented to our clinic with an incidental finding of a left thyroid nodule. The patient underwent an ultrasound-guided fine needle aspiration biopsy which revealed medullary thyroid carcinoma. Shortly afterward, the patient underwent a total thyroidectomy with bilateral cervical lymph node dissection. The patient tested negative for mutations involving RET exons 8, 10, 11, 13, 14, 15, and 16. On initial presentation, the patient was also noticed to have an appearance typical of acromegaly, with spade-like hands and characteristic acromegalic facial features, including prognathism and macroglossia. These findings were long-standing. He was found to have elevated IGF-1 (121.4 nmol/L, reference range 6.1 - 26.9 nmol/L) and hyperprolactinemia (78.2 mcg/L, reference range 2.7 - 16.9 mcg/L). His growth hormone (GH) levels did not suppress following a 75 g oral glucose tolerance test. Magnetic resonance imaging of the sella turcica revealed a prominent pituitary gland and stalk with a rightward deviated infundibulum, with a suspected 1.1 cm left-sided pituitary macroadenoma. The patient declined surgical management, and he was instead managed medically with octreotide. Conclusion: Coexistance of GH-producing pituitary adenoma and medullary thyroid cancer is a rare occurrence. We identified ten cases previously described in the literature. Specifically, this is the second reported case of a patient presenting with both medullary thyroid cancer and pituitary adenoma causing acromegaly. The advanced acromegalic phenotype suggested long standing disease and therefore prolonged tissue exposure to elevated levels of IGF-I. Although acromegaly is more commonly associated with nodular goiter of follicular-cell origin, the possibility of a thyroidal C-cell neoplasm must be entertained especially if GLP-1 agonist therapy is being considered for the treatment of hyperglycemia.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Louna S El-Zein ◽  
Dania Abushanab ◽  
Bayan Chaker

Abstract We are reporting a case of SIADH hyponatremia and pan-hypopituitarism in an otherwise stable appearing non-functional pituitary macroadenoma on MRI that suddenly developed apoplexy. Patient is a 52-year-old hispanic male with a history of a pituitary adenoma diagnosed initially on 06/2017 during MRI head for chronic headaches. MRI Pituitary on 07/2018 showed “well-circumscribed lesion” measuring approximately 12.7 x 14.8 x 13.4 mm in AP. Hormonal work up was normal in 06/2017, 01/2018, and 07/2018. In 07/2018 labs showed: IGF-1 247 ng/mL (65–222), ACTH 28 pg/mL (7–69), random cortisol 8.58 microg/dL (3.09–22.4), TSH 2.3 mIU/mL (0.4–5.5), fT4 1.06 ng/dL (0.8–1.8), LH 4.6 mIU/mL (0.98–79.7), FSH 6.2 MIU/mL (1–18), prolactin 2.92 ng/mL (2.1–24), and testosterone 310 ng/dL (87–814). Oral glucose tolerance test was done and showed: GH was 0.34 ng/mL at baseline and 1.01 ng/mL after 75 g oral glucose (equivocal result). Repeat IgF1 was normal after that. Subsequently, patient was admitted on 09/24/2018 with severe headaches, nausea, vomiting, polyuria, and polydipsia. He was hypotensive and tachycardic and was found to be hyponatremic with a Na of 124, lowest at 119 where hypertonic saline was given at that point. Urine output was 1.5 L in 24 hours while he was on an 0.8 L fluid restriction, and then 6 L the following day while he was on 1.5 L fluid restriction, serum osmolality was 251, urine osmolality was 1003, urine specific gravity was >1.030. Nephrology agreed to the diagnosis of SIADH. Blood work on 09/24 showed: random cortisol 0.67 microg/dL, TSH 0.19 mIU/mL, free T4: 0.63 ng/dL, ACTH 11 pg/mL, LH 2.4 mIU/mL, FSH 4.7 MIU/mL, testosterone 13 ng/dl. Patient was started on Levothyroxine 125 mcg and hydrocortisone stress dose. MRI brain was done and showed a pituitary macro-adenoma, measuring approximately 14 x 11 x 11 mm with no significant change in size since 6/30/2017, without optic nerve compression, no hemorrhage was mentioned. Work up for other causes of panhypopituitarism, i.e. hemochromatosis was done and was negative: Ferritin: 258 ng/mL (22–322). CT abdomen and pelvis was also done and showed normal adrenal glands. We thought about granulomatous infiltrative diseases like sarcoidosis since 1–25 (OH) vitamin D was high at 120 pg/mL (19.9–79.3). CT neck and chest was done but was negative for sarcoidosis or lymphadenopathy. Patient was seen outpatient by neurosurgery in 1/2019 and a repeat MRI was done which reported resolution of cystic and blood material from a pituitary adenoma, and per neurosurgery there is resolution of a pituitary cystic adenoma with apoplexy that now looks like a partial empty sella. Patient currently continues to be pan-hypopituitary requiring hydrocortisone, levothyroxine and testosterone replacement. SIADH has resolved. An otherwise non-functioning stable pituitary macroadenoma on MRI, can suddenly present as symptomatic apoplexy with SIADH and pan-hypopituitarism.


2005 ◽  
Vol 11 ◽  
pp. 28
Author(s):  
Fanny Rodriguez Vallejo ◽  
Juan Manuel Rios Torres ◽  
Francisco J. Gomez-Pérez ◽  
Juan A. Rull Rodrigo ◽  
Bernardo Pérez Enriquez

1994 ◽  
Vol 72 (03) ◽  
pp. 434-437 ◽  
Author(s):  
E Bruckert ◽  
A Ankri ◽  
P Glral ◽  
G Turpin

SummaryPlasminogen activator inhibitor type-1 (PAI-1) is a key determinant of the fibrinolytic capacity. Its activity correlates with most of the characteristic features of insulin resistance syndrome, i. e. obesity, high blood pressure and hyperlipidemia.We measured plasma PAI-1 antigen levels in 131 asymptomatic men (aged 44.2 ± 11 years) who had been referred for hyperlipidemia. Those taking medication and those with a secondary hyperlipidemia were excluded.We confirmed the correlation between PAI-1 levels and the following variables: body mass index, blood pressure, triglyceride concentration, and blood glucose and insulin levels before and after an oral glucose tolerance test. We also found a significant and independent correlation between PAI-1 and the concentration of the hepatic enzymes glutamyl transferase, alanine aminotransferase and aspartate aminotransferase.Mild liver abnormalities (presumably steatosis) may thus be one of the factors accounting for high plasma PAI-1 levels in hyperlipidemic patients.


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