Aggressive Cushing disease five years after the treatment of a nonfunctional pituitary adenoma

2016 ◽  
Vol 62 (5) ◽  
pp. 77-78
Author(s):  
Minodora Betivoiu ◽  
Sorina Martin ◽  
Iulia Soare ◽  
Alexandra Nila ◽  
Simona Fica
Keyword(s):  
Pituitary Adenoma ◽  
Gamma Knife ◽  
Pituitary Adenomas ◽  
Histopathological Examination ◽  
Case Reports ◽  
Dependent Diabetes Mellitus ◽  
Ki 67 ◽  
The Right ◽  
Gamma Knife Therapy

Introduction. Cushing's disease (CD) represents 10%–12% of all pituitary adenomas and is seen predominantly in women, with a female-to-male ratio of 8:1. Although most patients with ACTH-secreting adenomas present with benign, small tumors, some have invasive macroadenomas. Rarely, nonfunctional pituitary adenomas (NFPAs) may gain secretory function, but there have been a few case reports of metamorphosis to CD.Case report. We report the case of a 59-year-old female diagnosed in 2007 with a NFPA and panhypopituitarism. She had two transsphenoidal surgeries and Gamma Knife therapy and started replacement treatment with levothyroxine 75mcg/day and prednisone 5mg/day. The postoperative course was favorable and imagistic follow-up between 2007-2014 showed progressive reduction of the residual tumor and empty sella. From personal history we note noninsulin-dependent diabetes mellitus, postmenopausal osteoporosis treated with bisphosphonates. In January 2015 she suffered visual loss on the right eye. Pituitary MRI showed supra and parasellar tumor recurrence of 27/24/17mm, infiltrating the right side of the cavernous sinus, extending around the right internal carotid artery and optic nerve, compressing the optic chiasm. In March 2015 a third transsphenoidal partial excision of the tumor was performed and in August Gamma Knife therapy was repeated. The histopathological examination was consistent with a pituitary adenoma but immunohistochemical staining for ACTH was positive, with Ki-67=25%. She had no non-specific cushingoid features. Laboratory test: glucose=116mg/dl, HbA1c=7.5%, FSH=3.34mIU/ml, LH=0.585mIU/ml TSH=0.044mcIU/ml, FT4=1.13ng/dl. Prednisone replacement therapy was stopped and CD was confirmed: 8AMcortisol=13.3mcg/dl, 23PMcortisol=11.3mcg/dl, ACTH=70.2pg/ml, 8AMcortisol after 1mg dexamethasone overnight=13.8mcg/dl. Ophthalmic exam: blindness in the right eye, slightly decreased visual field in the left eye. Pituitary MRI 8-month postsurgery revealed a 28/31/28mm invasive tumor. We started treatment with Cabergoline 3mg/week and recommended closely biological and imagistic follow-up, hoping for a good response to radiotherapy.Conclusions: Our case stresses the importance of regular, lifelong follow-up of patients with NFPAs. Chiloiro et al have reported that pituitary adenomas with Ki-67≥1.5% have a higher risk of recurrence. Although the characteristics of patients with CD have been well known for decades, the diagnosis and management of this disease are often challenging.

Ki-67/MIB-1 and Recurrence in Pituitary Adenoma

2021 ◽  
Author(s):  
Kent Tadokoro ◽  
Colten Wolf ◽  
Joseph Toth ◽  
Cara Joyce ◽  
Meharvan Singh ◽  
...  
Keyword(s):  
Systematic Review ◽  
Pituitary Adenoma ◽  
Recurrence Rate ◽  
Pituitary Adenomas ◽  
Cellular Proliferation ◽  
Published Data ◽  
Ki 67 ◽  
Institutional Data ◽  
Mib 1

Abstract Objectives Ki-67/MIB-1 is a marker of cellular proliferation used as a pathological parameter in the clinical assessment of pituitary adenomas, where its expression has shown utility in predicting the invasiveness of these tumors. However, studies have shown variable results when using Ki-67/MIB-1 association with recurrence. The purpose of this study is to determine if a high Ki-67/MIB-1 labeling index (LI) is predictive of recurrence in pituitary adenomas. Methods A retrospective chart review was performed for patients undergoing pituitary adenoma resection with at least 1 year of follow-up. Additionally, systematic data searches were performed and included studies that correlated recurrence rate to Ki-67/MIB-1 LI. Our institutional data were included in a synthesis with previously published data. Results Our institutional review included 79 patients with a recurrence rate of 26.6%. We found that 8.8% of our patients had a high Ki-67/MIB-1 LI (>3%); however, high Ki-67/MIB-1 was not associated with recurrence. The systematic review identified 244 articles and 49 full-text articles that were assessed for eligibility. Quantitative analysis was performed on 30 articles including our institutional data and 18 studies reported recurrence by level of Ki-67/MIB-1 LI. Among studies that compared Ki-67/MIB-1 ≥3 vs. <3%, 10 studies reported odds ratios (OR) greater than 1 of which 6 were statistically significant. A high Ki-67/MIB-1 had higher odds of recurrence via the pooled odds ratio (OR = 4.15, 95% confidence interval [CI]: 2.31–7.42). Conclusion This systematic review suggests that a high Ki-67/MIB-1 should prompt an increased duration of follow-up due to the higher odds of recurrence of pituitary adenoma.

Gamma Knife surgery for adrenocorticotropic hormone–producing pituitary adenomas after bilateral adrenalectomy

2007 ◽  
Vol 106 (6) ◽  
pp. 988-993 ◽  
Author(s):  
William J. Mauermann ◽  
Jason P. Sheehan ◽  
Daniel R. Chernavvsky ◽  
Edward R. Laws ◽  
Ladislau Steiner ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Gamma Knife ◽  
Pituitary Adenomas ◽  
Cranial Nerve ◽  
Adrenocorticotropic Hormone ◽  
Gamma Knife Surgery ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Follow Up Studies

Object Patients with adrenocorticotropic hormone (ACTH)–secreting pituitary adenomas may require a bilateral adrenalectomy to treat their Cushing's disease. Approximately one third of these patients, however, will experience progressive enlargement of the residual pituitary adenoma, develop hyperpigmentation, and have an elevated level of serum ACTH. These patients with Nelson's syndrome can be treated with Gamma Knife surgery (GKS). Methods The prospectively collected University of Virginia Gamma Knife database of patients with pituitary adenomas was reviewed to identify all individuals with Nelson's syndrome who were treated with GKS. Twenty-three patients with a minimum of 6 months of follow up were identified in the database. These patients were assessed for tumor control (that is, lack of tumor growth over time) with neuroimaging studies (median follow-up duration 22 months) and for biochemical normalization of their ACTH levels (median follow-up duration 50 months). Neuroimaging follow-up studies were available for 22 patients, and endocrine follow up was available for 15 patients in whom elevation of ACTH levels was documented prior to GKS. In the 22 patients in whom neuroimaging follow-up studies were available, 12 had a decrease in tumor size, eight had no tumor growth, and two had an increase in tumor volume. Ten of 15 patients with elevated ACTH levels prior to GKS showed a decrease in their ACTH levels at last follow up; three of these 10 patients achieved normal ACTH levels (< 50 pg/ml) and the other five patients with initially elevated values had an increase in ACTH levels. Ten patients were thoroughly evaluated for post-GKS pituitary function; four were found to have new pituitary hormone deficiency and six did not have hypopituitarism after GKS. One patient suffered a permanent third cranial nerve palsy and four patients are now deceased. Conclusions Gamma Knife surgery may control the residual pituitary adenoma and decrease ACTH levels in patients with Nelson's syndrome. Delayed hypopituitarism or cranial nerve palsies can occur after GKS. Patients with Nelson's syndrome require continued multidisciplinary follow-up care. Given the difficulties associated with management of Nelson's syndrome, even the modest results of GKS may be helpful for a number of patients.

Gamma Knife surgery for pituitary adenomas: factors related to radiological and endocrine outcomes

2011 ◽  
Vol 114 (2) ◽  
pp. 303-309 ◽  
Author(s):  
Jason P. Sheehan ◽  
Nader Pouratian ◽  
Ladislau Steiner ◽  
Edward R. Laws ◽  
Mary Lee Vance
Keyword(s):  
Pituitary Adenoma ◽  
Radiation Dose ◽  
Tumor Growth ◽  
Gamma Knife ◽  
Pituitary Adenomas ◽  
Gamma Knife Surgery ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency

Object Gamma Knife surgery (GKS) is a common treatment for recurrent or residual pituitary adenomas. This study evaluates a large cohort of patients with a pituitary adenoma to characterize factors related to endocrine remission, control of tumor growth, and development of pituitary deficiency. Methods A total of 418 patients who underwent GKS with a minimum follow-up of 6 months (median 31 months) and for whom there was complete follow-up were evaluated. Statistical analysis was performed to evaluate for significant factors (p < 0.05) related to treatment outcomes. Results In patients with a secretory pituitary adenoma, the median time to endocrine remission was 48.9 months. The tumor margin radiation dose was inversely correlated with time to endocrine remission. Smaller adenoma volume correlated with improved endocrine remission in those with secretory adenomas. Cessation of pituitary suppressive medications at the time of GKS had a trend toward statistical significance in regard to influencing endocrine remission. In 90.3% of patients there was tumor control. A higher margin radiation dose significantly affected control of adenoma growth. New onset of a pituitary hormone deficiency following GKS was seen in 24.4% of patients. Treatment with pituitary hormone suppressive medication at the time of GKS, a prior craniotomy, and larger adenoma volume at the time of radiosurgery were significantly related to loss of pituitary function. Conclusions Smaller adenoma volume improves the probability of endocrine remission and lowers the risk of new pituitary hormone deficiency with GKS. A higher margin dose offers a greater chance of endocrine remission and control of tumor growth.

scholarly journals Hypopituitarism after Gamma Knife radiosurgery for pituitary adenomas: a multicenter, international study

2019 ◽  
Vol 131 (4) ◽  
pp. 1188-1196 ◽  
Author(s):  
Diogo Cordeiro ◽  
Zhiyuan Xu ◽  
Gautam U. Mehta ◽  
Dale Ding ◽  
Mary Lee Vance ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Gamma Knife ◽  
Pituitary Adenomas ◽  
Proportional Hazards Model ◽  
Gamma Knife Radiosurgery ◽  
Cox Proportional Hazards Model ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
New Onset

OBJECTIVERecurrent or residual adenomas are frequently treated with Gamma Knife radiosurgery (GKRS). The most common complication after GKRS for pituitary adenomas is hypopituitarism. In the current study, the authors detail the timing and types of hypopituitarism in a multicenter, international cohort of pituitary adenoma patients treated with GKRS.METHODSSeventeen institutions pooled clinical data obtained from pituitary adenoma patients who were treated with GKRS from 1988 to 2016. Patients who had undergone prior radiotherapy were excluded. A total of 1023 patients met the study inclusion criteria. The treated lesions included 410 nonfunctioning pituitary adenomas (NFPAs), 262 cases of Cushing’s disease (CD), and 251 cases of acromegaly. The median follow-up was 51 months (range 6–246 months). Statistical analysis was performed using a Cox proportional hazards model to evaluate factors associated with the development of new-onset hypopituitarism.RESULTSAt last follow-up, 248 patients had developed new pituitary hormone deficiency (86 with NFPA, 66 with CD, and 96 with acromegaly). Among these patients, 150 (60.5%) had single and 98 (39.5%) had multiple hormone deficiencies. New hormonal changes included 82 cortisol (21.6%), 135 thyrotropin (35.6%), 92 gonadotropin (24.3%), 59 growth hormone (15.6%), and 11 vasopressin (2.9%) deficiencies. The actuarial 1-year, 3-year, 5-year, 7-year, and 10-year rates of hypopituitarism were 7.8%, 16.2%, 22.4%, 27.5%, and 31.3%, respectively. The median time to hypopituitarism onset was 39 months.In univariate analyses, an increased rate of new-onset hypopituitarism was significantly associated with a lower isodose line (p = 0.006, HR = 8.695), whole sellar targeting (p = 0.033, HR = 1.452), and treatment of a functional pituitary adenoma as compared with an NFPA (p = 0.008, HR = 1.510). In multivariate analyses, only a lower isodose line was found to be an independent predictor of new-onset hypopituitarism (p = 0.001, HR = 1.38).CONCLUSIONSHypopituitarism remains the most common unintended effect of GKRS for a pituitary adenoma. Treating the target volume at an isodose line of 50% or greater and avoiding whole-sellar radiosurgery, unless necessary, will likely mitigate the risk of post-GKRS hypopituitarism. Follow-up of these patients is required to detect and treat latent endocrinopathies.

scholarly journals Cushing’s Disease (CD) Due to ACTH-Secreting Pituitary Microadenoma Incidentally Discovered on a Sestamibi Scan for Primary Hyperparathyroidism

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A579-A580
Author(s):  
Hassan Mehmood ◽  
Murray B Gordon
Keyword(s):  
Pituitary Adenoma ◽  
Primary Hyperparathyroidism ◽  
Pituitary Adenomas ◽  
Case Reports ◽  
Adrenal Hyperplasia ◽  
Pituitary Microadenoma ◽  
Free Cortisol ◽  
Sestamibi Scan ◽  
The Right ◽  
Acth Secreting

Abstract Introduction: MIBI scintigraphy is commonly being used for the preoperative localization of parathyroid adenomas. Multiple studies showed MIBI uptake in pituitary adenomas are likely due to higher metabolic activity. When hyperfunctioning pituitary adenomas were reported, both had CD [1,2]. We present the third case of increased pituitary uptake on a MIBI scan later confirmed as CD. CaseA 64-year-old Caucasian female s/p renal transplantation for RPGN who presented for evaluation of hypercalcemia. Evaluation confirmed primary hyperparathyroidism with persistently elevated PTH levels 74-108 pg/ml (11-68), serum calcium levels 10.0-10.4 mg/dl (8.4-10.3), albumin 4.1-4.3 g/dl (3.6-5.1), phosphorus 3.0-3.2 mg/dl (2.5-4.5), creatinine 0.94-1.07 mg/dl. 24-hour urine calcium 60 mg/day (35-250). Vitamin D-25 OH level was 37 ng/dL (30-100). A sestamibi scan showed uptake in the right lower parathyroid, the midsternal chest region and the pituitary gland. MRI of the pituitary revealed a 7mm cystic pituitary microadenoma in the right posterior pituitary. CD was confirmed by the findings of persistently elevated 8 AM serum cortisol levels of 28.4 and 24.2 mcg/dl (4-22), ACTH levels of 59 and 39 pg/ml (10-48), and an elevated plasma free cortisol of 1.43 mcg/dl (0.07-0.93). CT of the abdomen showed L adrenal thickening suggesting adrenal hyperplasia from CD. Plasma cortisol suppressed to 1.2 mg/dl following 1 mg of dexamethasone. 24 urine for free cortisol 26.7 mcg/day (4-50). The patient had no proximal muscle weakness, striae or Cushingoid facial features. She had no hyperglycemia or hypertension. Patient was diagnosed with an ACTH secreting pituitary microadenoma with mild CD and adrenal hyperplasia. Her DXA scan showed osteoporosis. Genetic testing for MEN1 mutation was negative. Patient did not wish surgery for either her hyperparathyroidism or her CD and is being evaluated for medical treatment of hypercortisolism. Conclusion: There are two prior case reports of an incidentally discovered pituitary adenoma on sestamibi scan later diagnosed as CD [1,2]. Corticotrophs may have a strong affinity for sestamibi. Our case is the first, to our knowledge, of pituitary MRI confirmation of the ACTH secreting pituitary incidentaloma initially suspected by pituitary uptake on a sestamibi scan in a patient with hyperparathyroidism. Reference1. Kuhadiya ND et al. Incidentally Discovered ACTH-Secreting Pituitary Adenoma on a Sestamibi Scan in a Patient With Hyperparathyroidism. AACE Clinical Case Reports. 2015;1(3):e152-5. 2. Gierach M et al. The case of Cushing’s disease imaging by SPECT examination without manifestation of pituitary adenoma in MRI examination. Nuclear Medicine Review. 2005;8(2):137-9.

scholarly journals Pediatric Pituitary Adenoma: Case Series, Review of the Literature, and a Skull Base Treatment Paradigm

2018 ◽  
Vol 79 (01) ◽  
pp. 091-114 ◽  
Author(s):  
Avital Perry ◽  
Christopher Graffeo ◽  
Christopher Marcellino ◽  
Bruce Pollock ◽  
Nicholas Wetjen ◽  
...  
Keyword(s):  
Systematic Review ◽  
Pituitary Adenoma ◽  
Skull Base ◽  
Pituitary Adenomas ◽  
Pediatric Population ◽  
Csf Leak ◽  
Persistent Disease ◽  
Treatment Paradigm ◽  
Surgical Cure

Background Pediatric pituitary adenoma is a rare skull base neoplasm, accounting for 3% of all intracranial neoplasms in children and 5% of pituitary adenomas. Compared with pituitary tumors in adults, secreting tumors predominate and longer disease trajectories are expected due to the patient age resulting in a natural history and treatment paradigm that is complex and controversial. Objectives The aims of this study were to describe a large, single-institution series of pediatric pituitary adenomas with extensive long-term follow-up and to conduct a systematic review examining outcomes after pituitary adenoma surgery in the pediatric population. Methods The study cohort was compiled by searching institutional pathology and operative reports using diagnosis and site codes for pituitary and sellar pathology, from 1956 to 2016. Systematic review of the English language literature since 1970 was conducted using PubMed, MEDLINE, Embase, and Google Scholar. Results Thirty-nine surgically managed pediatric pituitary adenomas were identified, including 15 prolactinomas, 14 corticotrophs, 7 somatotrophs, and 4 non-secreting adenomas. All patients underwent transsphenoidal resection (TSR) as the initial surgical treatment. Surgical cure was achieved in 18 (46%); 21 experienced recurrent/persistent disease, with secondary treatments including repeat surgery in 10, radiation in 14, adjuvant pharmacotherapy in 11, and bilateral adrenalectomy in 3. At the last follow-up (median 87 months, range 3–581), nine remained with recurrent/persistent disease (23%).Thirty-seven publications reporting surgical series of pediatric pituitary adenomas were included, containing 1,284 patients. Adrenocorticotropic hormone (ACTH)-secreting tumors were most prevalent (43%), followed by prolactin (PRL)-secreting (37%), growth hormone (GH)-secreting (12%), and nonsecreting (7%). Surgical cure was reported in 65%. Complications included pituitary insufficiency (23%), permanent visual dysfunction (6%), chronic diabetes insipidus (DI) (3%), and postoperative cerebrospinal fluid (CSF) leak (4%). Mean follow-up was 63 months (range 0–240), with recurrent/persistent disease reported in 18% at the time of last follow-up. Conclusion Pediatric pituitary adenomas are diverse and challenging tumors with complexities far beyond those encountered in the management of routine adult pituitary disease, including nuanced decision-making, a technically demanding operative environment, high propensity for recurrence, and the potentially serious consequences of hypopituitarism with respect to fertility and growth potential in a pediatric population. Optimal treatment requires a high degree of individualization, and patients are most likely to benefit from consolidated, multidisciplinary care in highly experienced centers.

Metastatic Basal Cell Carcinoma: Four Case Reports, Review of Literature, and Immunohistochemical Evaluation

2006 ◽  
Vol 130 (1) ◽  
pp. 45-51
Author(s):  
Diana N. Ionescu ◽  
Muammar Arida ◽  
Drazen M. Jukic
Keyword(s):  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Case Reports ◽  
Small Sample ◽  
Ki 67 ◽  
Immunohistochemical Markers ◽  
Metastatic Basal Cell Carcinoma ◽  
Metastatic Sites

Abstract Context.—Metastatic basal cell carcinoma (BCC) is relatively rare and is seldom considered a complication in the routine treatment and follow-up of patients with BCC. Although multiple studies have tried to distinguish aggressive from nonaggressive BCCs, to our knowledge, no consistent clinical, histopathologic, or immunohistochemical features have yet been reported. Objective.—To report 4 cases of metastatic BCCs and to evaluate these in addition to known nonmetastatic BCCs with specific immunostains in an attempt to find distinct morphologic or immunohistochemical patterns that could be helpful in identifying aggressive BCCs. Design.—We reviewed 4 cases of metastatic BCCs and recorded the clinical and morphologic findings. We then searched our archives for 14 cases of BCC that followed the usual nonaggressive course. We evaluated these 18 cases with immunohistochemical stains for Ki-67, p53, and bcl-2. Results.—In metastasizing BCC, Ki-67 staining was slightly higher in metastatic sites than in primary sites (average 63% and 51%, respectively). p53 was expressed in 3 of 4 primary sites and 2 of 4 metastatic sites. Bcl-2 was positive in both primary and metastatic sites in 3 of 4 cases. In the 14 cases of nonaggressive BCC, staining for Ki-67 averaged 38%, p53 was positive in 11 cases, and Bcl-2 staining was noted in 13 cases. Conclusions.—Overall, in the small sample that we evaluated, the immunohistochemical markers for Ki-67, p53, and Bcl-2 did not distinguish between metastatic and nonaggressive BCCs.

scholarly journals Alternative Leprosy Treatment Using Rifampicin Ofloxacin Minocycline (ROM) Regimen – Two Case Reports

2019 ◽  
Vol 11 (3) ◽  
pp. 89-93
Author(s):  
Yohanes Widjaja ◽  
Khairuddin Djawad ◽  
Saffruddin Amin ◽  
Widyawati Djamaluddin ◽  
Dirmawati Kadir ◽  
...  
Keyword(s):  
Clinical Improvement ◽  
Alternative Treatment ◽  
Histopathological Examination ◽  
Case Reports ◽  
Erythematous Plaque ◽  
First Case ◽  
Satisfactory Outcome ◽  
Significant Clinical Improvement ◽  
The Right

Abstract Introduction. Leprosy is a disease that predominantly affects the skin and peripheral nerves, resulting in neuropathy and associated long-term consequences, including deformities and disabilities. According to the WHO classification, there are two categories of leprosy, paucibacillary (PB) and multibacillary (MB). The standard treatment for leprosy employs the use of WHO MDT (Multi Drug Treatment) regimen, despite its multiple downsides such as clofazimine-induced pigmentation, dapsone-induced haematological adverse effects, poor compliance due to long therapy duration, drug resistance, and relapse. Multiple studies and case reports using ROM regimen have reported satisfactory results. Nevertheless, there are still insufficient data to elucidate the optimum dosage and duration of ROM regimen as an alternative treatment for leprosy. Previous experience from our institution revealed that ROM regimen given three times weekly resulted in a satisfactory outcome. Case Reports. We report two cases of leprosy treated with ROM regimen from our institution. The first case was PB leprosy in a 64-year-old male who presented with a single scaly plaque with erythematous edge on the right popliteal fossa. Sensibility examination showed hypoesthesia with no peripheral nerve enlargement. Histopathological examination confirmed Borderline Tuberculoid leprosy. ROM regimen was started three times weekly for 6 weeks and the patient showed significant clinical improvement at the end of the treatment with no reaction or relapse until after 6 months after treatment. The second case was MB leprosy in a 24-year-old male patient with clawed hand on the 3rd-5th phalanges of the right hand and a hypoesthetic erythematous plaque on the forehead. Histopathology examination confirmed Borderline leprosy. The patients received ROM therapy 3 times a week with significant clinical improvement after 12 weeks. Conclusion. ROM regimen given three times weekly for 6 weeks in PB leprosy and 12 weeks in MB leprosy resulted in a significant clinical improvement. Thus, ROM regimen could be a more effective, safer, faster alternative treatment for leprosy.

scholarly journals Ruptured Dissecting Aneurysm of the Carotid Artery 12 Years after Gamma Knife Therapy for Pituitary Adenoma

2020 ◽  
Author(s):  
Takahiko Taniguchi ◽  
Terushige Toyooka ◽  
Masataka Miyama ◽  
Satoru Takeuchi ◽  
Naoki Otani ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Carotid Artery ◽  
Gamma Knife ◽  
Dissecting Aneurysm ◽  
Gamma Knife Therapy

scholarly journals SAT-269 A Rare Case of Functioning Gonadotroph Producing Pituitary Macroadenoma in Teenage Male

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Siroj Dejhansathit ◽  
Subhanudh Thavaraputta ◽  
Genevieve LaPointe ◽  
Ana Marcella Rivas Mejia
Keyword(s):  
Transcription Factor ◽  
Pituitary Adenoma ◽  
Pituitary Adenomas ◽  
Complete Blood Count ◽  
Case Reports ◽  
Biologically Active ◽  
Total Testosterone ◽  
Sellar Mass ◽  
Delay In Diagnosis ◽  
Orbitozygomatic Approach

Abstract Background: Unlike nonfunctioning gonadotroph pituitary adenomas, functioning gonadotroph pituitary adenomas (FGA) are an uncommon type of pituitary tumors that secrete biologically active gonadotropins (LH, FSH, or both). Clinical Case: A 23-year-old man with no previous medical history presented to the emergency department with three months history of progressive decreased vision and decreased libido. He denied headache, seizure, erectile dysfunction, or weakness. On physical examination, his visual acuity was significantly reduced on the right eye and was only able to perceive light. He had a visual field narrowing on the left eye. Gynecomastia was noted bilaterally and testicles were found to be enlarged (Orchidometer &gt;25 mL). Complete blood count was significant for hemoglobin of 19.2 g/dL (N, 13.7-17.5 g/dL), and hematocrit of 57.0% (N, 40.1-51.0%). Pituitary function tests were as follow: FSH &gt;200.0 mIU/mL (N, 1.5-12.4 mIU/mL), LH 17.0 mIU/mL (N, 1.7-8.6 mIU/mL), total testosterone 41.3 nmol/L (N, 8.6-29.0 nmol/L), free testosterone 1.263 nmol/L (N, 0.148-0.718 nmol/L), and bioavailable testosterone 29.609 (N, 2.110-8.920 nmol/L). Prolactin, TSH, GH, and ACTH were all within the normal range. Magnetic resonance imaging (MRI) showed a sellar mass involving the planum sphenoidal measuring 5.8 x 5.2 x 5.6 cm with optic chiasm displaced superiorly, in addition, subfalcine herniation with mild hydrocephalus was also noted. The patient underwent orbitozygomatic approach resection of the pituitary tumor. Histological examination was consistent with a pituitary adenoma. Cells stained positive for transcription factor steroidogenic factor 1 (SF 1), FSH, LH, and alpha-subunit consistent with a gonadotroph adenoma. They were negative for transcription factor Pit 1 stain and the remaining pituitary hormones including ACTH, GH, prolactin, and TSH stain. Postsurgical hormone assessment showed a significant decline in FSH and LH to 2.3 and 0.4 mIU/mL, respectively and testosterone level decreased to &lt; 0.087 nmol/L on postoperative day 18. The patient’s vision improved postoperatively prior to discharge but he lost follow up thereafter. Conclusion: Most patients with functioning gonadotroph pituitary adenoma present with large tumors that are detected based on the occurrence of symptoms of compression that result from the enlarging sellar mass. Most patients, particularly men and postmenopausal women, do not develop symptoms of hormone excess and the lack of symptomatology results in delay in diagnosis. Our patients presented with significant polycythemia which resulted from excess testosterone and could have prompted earlier tumor detection if he had presented in an earlier stage. The incidence of polycythemia in male patients with functioning gonadotroph pituitary adenoma has previously been described in the literature in a few case reports.

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