scholarly journals Giant Pituitary Adenoma Presenting with Foster-Kennedy Syndrome in a 21-Year Old Ethiopian Patient: A Rarely Reported Phenomenon: A Case Report

2020 ◽  
Vol 30 (2) ◽  
Author(s):  
Biniyam Ayele ◽  
Abenet Mengesha ◽  
Abdi Wotiye ◽  
Yodit Alemayehu

BACKGROUND: Disorders of the pituitary gland and the sellar region present a wide spectrum of clinical problems. A variety of lesions in this area tend to present with similar problems; namely, headache, hormonal disorders, and loss of vision. Benign adenomas of the pituitary gland are by far the most common disorders of sellar region. Rarely, patients with pituitary adenoma may present with blindness in one eye and visual field cut (temporal hemianopia) in the contralateral eye. This rare clinical entity is called Foster-Kennedy Syndrome (FKS).CASE PRESENTATION: We present a 21-year-old male patient, presented with progressive visual disturbance of the left eye, associated with global headache. The headache was refractory to over-counter medications. In addition, he had history of sleep disturbance and loss of appetite. Neurological examination was pertinent for left side visual loss with optic atrophy and right eye temporal visual field cut with disc edema. Brain MRI showed 4 cm X 3.5 cm sized lobulated intra and supra sellar mass with heterogeneous contrast enhancement and minimal surrounding edema. Serum prolactin level was 6,705 ng/mL. Otherwise, the other pituitary hormones were in normal range.CONCLUSION: This case highlighted that pituitary adenoma may present with the full picture of Foster Kennedy syndrome. Therefore, we recommend considering pituitary adenoma as a possible differential diagnosis of intracranial lesions presenting as Foster Kennedy syndrome, as early detection and management could potentially salvage patients’ vision and quality of life.

2021 ◽  
Vol 3 (4) ◽  
pp. 244-249
Author(s):  
Norazlida Ibrahim ◽  
Raja Norliza Binti Raja Omar ◽  
Mae-Lynn Catherine Bastion

Pituitary apoplexy in pregnancy is a potentially fatal condition caused by acute ischaemic infarction or haemorrhage of pre-existing pituitary adenoma or within a physiologically enlarged pituitary gland. It has a wide spectrum of clinical presentations ranging from a mild headache to sudden collapsed. Here, we report a life-threatening case of pituitary apoplexy in a non-functioning pituitary macroadenoma occurring during pregnancy that presented with bilateral blurring of vision. Visual field showed bitemporal superior quadrantanopia. Urgent non-contrast brain MRI revealed an acute expansion of a hemorrhagic pituitary lesion complicated with local compression to the optic chiasm. The patient underwent an uneventful right supraorbital craniotomy and excision of the tumour under general anaesthesia with no foetal loss. The repeated visual field at 2 weeks after surgery showed recovering visual field defect. Hence, early neurosurgical intervention is advisable to prevent mortality and morbidity due to permanent visual field loss.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Abhilasha Singh ◽  
Massiell German ◽  
Anu Sharma

Abstract Background Pituitary hyperplasia (PH) is a non-neoplastic increase in one or more cell subtype of the adenohypophysis. It is physiologically seen in pregnancy from resultant lactotroph hyperplasia and pathologically in organ dysfunctions that lead to loss of negative feedback on hypothalamus like primary hypothyroidism and primary adrenal insufficiency. Although primary hypothyroidism is common, PH is an under-recognized consequence of long-standing hypothyroidism. Case Description A 26-year-old female with class 3 obesity, type 2 diabetes mellitus, and primary hypothyroidism on replacement presented for evaluation of secondary amenorrhea. Two months prior to presentation, she underwent a brain MRI for new-onset headache which revealed diffusely enhancing homogenous pituitary tissue measuring 2.3 cm with upward lifting of the optic chiasm. Serum prolactin was elevated at 86.2 ng/ml (2.8-26 ng/ml). This was presumed to be secondary to a prolactinoma and cabergoline was started by the referring provider. Subsequent workup revealed elevated thyroid-stimulating hormone (TSH) at 494.11 mU/L (0.30- 4.00 mU/L) and a low free thyroxine (FT4) of 0.2 ng/dl (0.8-1.7 ng/dl). Other pituitary hormones were within normal limits. The visual field examination was normal. It was noted she had a persistently elevated TSH over the past five years. On further questioning, the patient ran out of levothyroxine replacement 6 months ago and had been generally non-adherent to therapy for years due to nausea. Improved medication adherence resulted in the normalization of prolactin and reduction in TSH level. Repeat MRI 2 months later revealed a reduction in her pituitary hyperplasia to 2.1 cm. Discussion In severe primary hypothyroidism, there is loss of negative feedback to the hypothalamus due to low circulating FT4 and triiodothyronine (T3) concentrations. As a result, there is stimulation of thyrotropin-releasing hormone (TRH) which promotes thyrotroph hyperplasia. A strong correlation exists between TSH concentration and the degree of pituitary hyperplasia. The typical MRI finding is a diffusely enhancing homogenous pituitary mass. Despite optic chiasm contact, physiologic pituitary hyperplasia rarely causes visual field defects. High concentrations of TRH stimulates prolactin release with resultant mild hyperprolactinemia. Although PH is reversible with therapy, rare cases with subsequent development of panhypopituitarism while on therapy have been reported in the setting of longstanding hypothyroidism. Very rarely, chronically untreated primary hypothyroidism can stimulate the growth of a thyrotroph adenoma. Long-term biochemical and radiological monitoring is therefore recommended until resolution. This case highlights the physiologic responses manifested in severe primary hypothyroidism and the fact that these changes improve with adequate replacement.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A567-A567
Author(s):  
Young Ki Kim ◽  
In Sun Goak ◽  
Yu Ji Kim ◽  
Heung Yong Jin ◽  
Tae Sun Park ◽  
...  

Abstract Background: Nonfunctioning pituitary adenomas (NFPAs) are most common pituitary tumors, and primary pituitary gland malignancies are extremely rare. Most malignant pituitary gland lesions metastasize from other sites. Primary malignant lesions, such as sarcomas, usually develop after radiotherapy or chemotherapy for other diseases. We report a rare case of primary sellar leiomyosarcoma (LMS) without prior therapy that arose concurrently with a pituitary adenoma. Clinical Case: A 56-year-old woman with ptosis of the right eye, headache, and progressive visual deficits visited our neurosurgery department. She had no medical history besides hypertension. Twelve months ago, she was referred to us because of decreased visual acuity and a 3.5×3.6-cm-sized pituitary mass detected on brain MRI. Normal pituitary functions with mild hyperprolactinemia suggested a nonfunctioning pituitary mass with stalk compression. After transsphenoidal surgery, histopathology revealed a pituitary adenoma; MRI immediately post-surgery revealed no grossly remnant lesion. However, during the current visit, sellar MRI revealed a re-growing mass in the pituitary fossa extending to the sphenoid sinus and compressing the optic chiasm with a suprasellar extension. Functional endoscopic sinus surgery was performed, and histopathology revealed a composite tumor, i.e., a mesenchymal tumor with a pituitary adenoma. On immunohistochemical staining, smooth muscle actin, synaptophysin, and chromagranin were positive; tumor cell mitosis was observed at 7/10 high-power fields. Finally, a composite tumor of myxoid leiomyosarcoma and pituitary adenoma was diagnosed. Hence, systemic chemotherapy with radiotherapy was planned for the remnant lesion. Hormonal replacement therapy with hydrocortisone and thyroxine was also started for subsequent hypopituitarism. Conclusion: NFPA is benign and has good prognosis if it is not grow in size or is completely resected. Conversely, primary sarcomas, such as LMS, show rapid extension and aggressive local invasion. Although their incidence is extremely rare, few primary pituitary sarcoma cases with or without pituitary adenoma have been reported. In the former case, initial diagnosis of pituitary adenoma may lead to delayed diagnosis of combined malignant lesions. Thus, clinicians should consider this possibility and high index of suspicion is required when diagnosing a pituitary mass.


2020 ◽  
pp. 26-30
Author(s):  
Bogdan Ionut David ◽  
Ligia Gabriela Tataranu ◽  
Vasile Gheorghe Ciubotaru ◽  
Aurelia Mihaela Sandu ◽  
Radu Mircea Gorgan

Synchronous tumours can be found all along the entire neuraxis, however, some lesions are far less likely to coexist. One of these extremely rare associations is between GH-pituitary adenomas and suprasellar meningiomas. A wide spectrum of transcranial and transsphenoidal approaches were described in the literature for either sellar, suprasellar and parasellar lesions, but no agreement has been reached for the cases of simultaneous occurring lesions. We present a rare case of a woman with GH-secreting pituitary adenoma and concomitant suprasellar meningioma. The strategy chosen was sequential transsphenoidal surgeries. However, after the first surgery, the remaining tumour mass did not mobilize as expected due to gravity, hence we decided to perform a transcranial subfrontal unilateral approach. Surprisingly, the second surgery revealed a different histopathological result. Association of a GH-pituitary adenoma and suprasellar meningioma is very rare, only 17 cases being reported in the relevant literature so far.  Different authors prefer different strategies, ranging from only transsphenoidal to simultaneous transsphenoidal and transcranial approaches, but no general consensus was established. In conclusion, the existence of synchronous tumours of the sellar region should be taken into account when imaging studies reveal an intracranial mass developing both sellar and suprasellar. The surgical strategy should be tailored to every specific patient and experience of the neurosurgeon.


2021 ◽  
Vol 14 (8) ◽  
pp. e242353
Author(s):  
Franziska Geissler ◽  
Irene Hoesli ◽  
Monya Todesco Bernasconi

Pituitary apoplexy is caused by haemorrhage or infarction of the pituitary gland. Presenting signs and symptoms often include severe headache, visual disturbance, ophthalmoplegia, altered consciousness and impaired pituitary function. The management of pituitary apoplexy has very rarely been described during pregnancy and there is no existing data for further pregnancies of affected women. We present a case of a woman with a recurrent pituitary apoplexy due to haemorrhages in a pituitary adenoma in her third and fourth pregnancies. In both pregnancies, the pituitary apoplexy was managed conservatively, but due to therapy-resistant headaches, a preterm delivery was implemented.


2020 ◽  
Vol 2 (Supplement_2) ◽  
pp. ii4-ii5
Author(s):  
Christopher Hong ◽  
Declan McGuone ◽  
E Zeynep Erson-Omay ◽  
Sacit Bulent Omay

Abstract Metastasis to the pituitary gland and surrounding sellar region from systemic tumors is a rare occurrence. Patients may present with signs of endocrine dysfunction secondary to pituitary involvement, as well as mass effect-related symptoms including headaches and visual deficits. Despite a small but accumulating body of literature describing the clinical and histopathological correlates for sellar metastases from systemic tumors, the genetic alterations underlying sellar spread have not been previously described. We describe a 68 year-old female with history of a resected lung carcinoid tumor, followed by chemoradiation, who was diagnosed with a sellar lesion on surveillance PET-CT and subsequent brain MRI. Her tumor was resected via an endoscopic endonasal approach, and final pathology was consistent with neuroendocrine origin, including positive immunohistochemistry for synpatophysin, CK7, TTF-1, and CAM5.2 with a Ki-67 index of 8–12%. Whole-exome sequencing of the sellar specimen demonstrated large-scale deletions of chromosomes 3, 6, and 9 and focal deletions on chromosomes 1,2, 11, 15, and 16. Mutational signature analysis was enriched for COSMIC Signature 4, seen in multiple primary lung cancers. Among 91 total somatic alterations, 7 had been previously associated with oncogenesis (MYO18A, PTCH1, BCOR, CLIC6, TLL2, COL1A1, PTPRK). Notably, mutations in BCOR and PTCH1 have been previously implicated in both systemic neuroendocrine tumors as well as primary tumors of the pituitary gland, while MYO18A, FGF4, and PTPRK mutations had not been reported in systemic neuroendocrine tumors but have been implicated in tumor migration and pituitary adenoma progression. In summary, these data demonstrated an expected mutational pattern indicating a systemic lung neuroendocrine origin but also revealed new mutations previously implicated in primary pituitary pathologies that may have evolutionarily drove divergence from the primary tumor. Further genome studies of these rare lesions may yield further insight into the genetic alterations underlying metastasis to the sellar region.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A577-A578
Author(s):  
Noor AlNasrallah ◽  
Maryam Almurshed ◽  
Khaled Aljenaee ◽  
Sulaiman Hajji

Abstract Background: Gonadotroph adenomas are the most common type of non-functioning pituitary adenomas. However functioning gonadotroph adenomas are rare with only a few cases reported in literature. Clinical Case: A 42-year old man was admitted as a case of COVID-19 when an incidental finding of a pituitary gland mass was detected. Upon evaluation, the patient was found to have blurred vision and dizziness since few years, however no history of headache, seizures, visual field deficit were reported, nor clear symptoms related to anterior pituitary hypo- or hyperfunction. Pituitary MRI revealed a pituitary gland mass measuring 30x23x22 mm expanding the pituitary fossa and extending to suprasellar cistern. Initial laboratory investigations revealed low free T3 (1.8 pmol/L, normal: 3.1-6.8 pmol/L) and low free T4 (6.6 pmol/L, normal: 12-22 pmol/L) with normal TSH (3.54 mIU/L, normal: 0.27-4.2 mIU/L), high FSH (31.4 IU/L, normal: 1-18 IU/L), and high total testosterone (50.31 nmol/L, normal 8.3-32.9 nmol/L). Testicular ultrasound showed bilateral enlarged testicles (right testis 38.14 cm3 and left testis 33.38 cm3). Visual Field testing revealed monocular superior temporal defect affecting only the left eye. Patient was treated medically with levothyroxine and hydrocortisone and surgically by minimally invasive transsphenoidal resection of pituitary adenoma. Histopathology assessment confirmed the diagnosis of gonadotroph secreting adenoma, with positive immunostain for FSH. At 6 weeks postoperatively, insulin tolerance test was performed after which hydrocortisone was only prescribed during periods of stress. Anterior pituitary profile was repeated which revealed normalization of free T4 (14.3 pmol/L) and FSH (1.2 IU/L) and decrease in testosterone (0.66 nmol/L). Assessment of visual field revealed marked improvement of previous findings. Pituitary MRI was also repeated with findings of enhancing soft tissue remnants. At 12 weeks postoperatively, anterior pituitary profile revealed normalization of testosterone (11.3 nmol/L). Testicular ultrasound was repeated and showed a marked decrease in testicular volume (right testis 17.93 cm3 and left testis 21.13 cm3). Conclusion: Functional gonadotroph adenomas are a rare subtype of pituitary adenomas which pose a diagnostic challenge and require meticulous clinical evaluation and multidisciplinary therapeutic approach.


QJM ◽  
2019 ◽  
Vol 113 (1) ◽  
pp. 59-60
Author(s):  
R Almazrouei ◽  
A Qayum ◽  
D Hope ◽  
E Hatfield

2010 ◽  
Vol 68 (3) ◽  
pp. 350-354 ◽  
Author(s):  
Aldo Pereira-Neto ◽  
André Meireles Borba ◽  
Paulo Andrade de Mello ◽  
Luciana Ansanelli Naves ◽  
Antônio Santos de Araújo Jr ◽  
...  

Intrasellar pressure (ISP) measurement technique has recently opened a new line of research in neuroendocrinology. The absolute and mean ISP values were investigated in 25 patients consecutively operated at the Brasilia University Hospital (DF). These data were correlated with serum prolactin levels, number of visual quadrant affected, tumor size, quality of life (measured through the SF-36 scale) and graded headache (measured through the HIT-6 scale). No correlation was observed. The p values were 0.887; 0.137; 0.892; 0.812 and 0.884; respectively. The HIT-6 values were inversely and moderately correlated with total RAND SF-36 and its mental and physical dimensions.


2016 ◽  
Vol 77 (S 02) ◽  
Author(s):  
Yoshikazu Ogawa ◽  
Kenichi Sato ◽  
Yasushi Matsumoto ◽  
Teiji Tominaga

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