scholarly journals Neurosarcoidosis and the Pituitary

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A593-A594
Author(s):  
Samina Afreen ◽  
Ashima Mittal ◽  
Farhad Hasan
Keyword(s):  
Symptomatic Relief ◽  
Cranial Nerves ◽  
Visual Field Loss ◽  
High Dose ◽  
Pituitary Hormone ◽  
Central Hypothyroidism ◽  
Hilar Lymph Node ◽  
Delay In Diagnosis ◽  
New Findings ◽  
Bilateral Hilar Lymphadenopathy

Abstract Background: Sarcoidosis involves the nervous system in 3-10% of cases. Neurosarcoidosis (NS) can mimic other diseases, leading to delay in diagnosis and management. We present 2 cases of hypothalmo-pituitary (HP) NS with widely differing initial presentations. Case Presentations: Case 1: A 58 year old African American (AA) male presented with decreased libido and hot flashes of 8-10 months duration. He also reported headaches, pain in the face and behind the eyes and dyspnea on exertion. On evaluation, he had central hypogonadism. MRI brain showed thickening and enhancement of pituitary infundibulum (PI). CT chest showed lung lesions and bilateral hilar adenopathies. EBUS with biopsies of lung and hilar lymph node showed non caseating granulomas (NCG). He was started on Prednisone and testosterone leading to symptomatic relief. However, he stopped prednisone after 3 months due to weight gain. He was then started on azathioprine. 9 months later, he presented with recurrence of neurologic symptoms and weight loss of 30 lbs. Labs showed new findings of low prolactin, low IGF-1, central hypothyroidism and central adrenal insufficiency (AI). MRI pituitary showed diffuse thickening of 3rd and 5th cranial nerves and of PI. He was re-started on Prednisone and levothyroxine (LT4) was started with improvement of symptoms. Plan was to start him on Infliximab instead of Azathioprine. Case 2: A 26 year old AA female presented with near syncope and visual impairment in the left eye that started a few weeks prior. Testing showed superior visual field loss and evidence of optic neuropathy, disc edema with mild dyschromatopsia in the left eye. She had had polyuria and polydipsia for the preceding 2-3 months and amenorrhea for 2 years. Lab testing revealed hyperprolactinemia and central DI, hypothyroidism, AI and hypogonadism. Pituitary MRI revealed a suprasellar mass measuring 20 x 19 x 11 mm, displacing the optic chiasm. CT scan showed bilateral hilar lymphadenopathy and lesions in liver and spleen. EBUS and biopsy of mediastinal lymph nodes showed NCG. Patient was treated with IV steroids leading to improved vision. She was started on DDAVP and LT4 and initiated on methotrexate and prednisone on discharge. Discussion: Pituitary hormone abnormalities due to HP NS include anterior hypopituitarism, hyperprolactinemia and DI. The most common (MC) presentation is of DI. Among anterior pituitary hormone deficits, central hypogonadism is the MC. Systemic high dose glucocorticoids (GC) are the mainstay of initial treatment. Optic atrophy is the MC neuro-ophthalmologic manifestation of NS. Only 10 - 20 % of patients have been found to have biochemical improvement of HP axis in response to high-dose GC treatment. Since pituitary involvement commonly leads to permanent endocrine deficits, it is essential to recognize it early and to treat it aggressively to prevent further permanent hormonal and neurologic deficits.

scholarly journals MON-462 Rebel Nodule or Reluctant Gland? the Challenge Presented by Heterophile Antibodies

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Pranjali Sharma ◽  
Linda Ding ◽  
Megan Elizabeth McGarvey
Keyword(s):  
Equilibrium Dialysis ◽  
Diagnostic Testing ◽  
High Dose ◽  
Pituitary Hormone ◽  
Thyroid Function Tests ◽  
Biochemical Tests ◽  
Central Hypothyroidism ◽  
Heterophile Antibody ◽  
Related Substances ◽  
Specific Symptoms

Abstract Introduction: Interpretation of thyroid function tests (TFTs) in background of non-specific symptoms concerning for hypothyroidism, has become challenging with assay interference. Heterophile antibody interferences are both test and laboratory platform dependent. We present a case of a toxic nodule erroneously diagnosed as isolated central hypothyroidism due to heterophile antibody interference. Case: A 38 year old lady was referred to Endocrinology for possible central hypothyroidism with symptoms of progressive fatigue, 40lb unintentional weight loss, poor appetite, nausea, intermittent diarrhea/constipation, occasional cold intolerance and intermittent lightheadedness. TFTs suggested low TSH 0.175 mcIU/ml (N 0.35-3.8 mcIU/ml) and low FT4 0.74 ng/dl (N 0.8-1.8 ng/dl). Pituitary hormone evaluation revealed normal ACTH, AM cortisol, IGF-1, prolactin and FSH. A pituitary MRI was normal. Two months later, she had worsening fatigue, anxiety with palpitations and tremors, and 14lb weight gain. TFTs again confirmed low TSH (0.575 mcIU/ml) and lower FT4 (0.70 ng/dl). Empirical weight-based levothyroxine (LT4) supplementation was initiated for central hypothyroidism. Six weeks later, TFTs showed hyperthyroidism (TSH <0.005 mcIU/ml, FT4 1.57 ng/dl). The patient endorsed worsening lightheadedness and palpitations on LT4, which resolved on LT4 discontinuation. One month later, TFTs were abnormal again (TSH 0.047 mcIU/ml, FT4 0.74 ng/dl). Due to persistent discordance in symptoms and biochemical tests, TFTs were evaluated for heterophile antibody interference. While HAMA-treated TSH remained low (0.04 mIU/l), FT4 by equilibrium dialysis (FT4 ED) was normal (1.1 ng/dl, regular assay 0.74 ng/dl), suggesting subclinical hyperthyroidism. A thyroid uptake scan confirmed an autonomous toxic right thyroid nodule with suppressed remaining gland. Patient ultimately underwent 15 mCi RAI ablation of the nodule. TFTs done 1 month post-RAI suggested normal TSH (1.2 mcIU/ml) and normal FT4 ED (1 ng/dl). Previous symptoms have since resolved. Conclusion: Current methods of TFTs are generally reliable in diagnosing and monitoring thyroid disease. Rarely, medications, supplements, and endogenous antibodies can bind to TSH, T4 or lab reagent, resulting in inaccurate values. This has become increasingly common with use of high dose biotin. TSH with HAMA/heterophile antibodies and FT4 ED are more accurate forms of diagnostic testing. When approaching patients with symptoms not consistent with typical hypo- or hyperthyroidism and are not responding as expected to therapy, it is important to consider more accurate testing to rule out assay errors. References: 1. Soh SB, Aw TC. Laboratory Testing in Thyroid Conditions - Pitfalls and Clinical Utility. Ann Lab Med. 2019;39(1):3-14. doi:10.3343/alm.2019.39.1.3 2. Spencer C., PhD. Assay of Thyroid Hormones and Related Substances

scholarly journals Characterizing Immune Checkpoint Inhibitor-Induced Hypophysitis and Obstacles in Diagnosis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A624-A625
Author(s):  
Jake Johnson ◽  
Dua’A Abdallah ◽  
Anupam Kotwal ◽  
Apar Kishor Ganti ◽  
Whitney Sears Goldner
Keyword(s):  
Immune Checkpoint ◽  
Checkpoint Inhibitors ◽  
Small Cell Lung Carcinoma ◽  
Serum Cortisol ◽  
Pituitary Hormones ◽  
High Dose ◽  
Pituitary Hormone ◽  
Central Hypothyroidism ◽  
Cell Lung Carcinoma

Abstract Background: Immune checkpoint inhibitors (ICI) are used in the treatment of multiple cancers. ICI-induced endocrinopathies are known side effects of many of these agents due to their immunogenic properties. The primary aim of this single institution analysis was to characterize the population who developed hypophysitis and secondary adrenal insufficiency (AI) related to ICI therapy, and identify diagnostic challenges. Methods: We performed a retrospective cohort study of adult cancer patients who received ICI therapy from 12/1/2012- 12/31/2019. We identified 839 patients who received CTLA-4, PD-1, PDL-1 inhibitors, or a combination during this time. Hypophysitis (H) was defined as low ACTH and low serum cortisol ± other pituitary hormone deficiencies. Results: Sixteen patients (1.9%; 16/839) developed hypophysitis after a median of 7 months from ICI initiation. The underlying cancers included: uroepithelial (1/48; 2.1 %), neuroendocrine (1/10; 10%), melanoma (9/156; 5.8%), renal cell carcinoma (4/74; 5.4%), and non-small cell lung carcinoma (1/247; 0.4%). Four patients were excluded since they had isolated secondary AI due to exogenous steroids. Two patients with hypophysitis also had exogenous steroid usage. However, these patients had central hypothyroidism + secondary AI so were included. The median age at start of ICI was 61.3 years and 57% were males. Patients who developed hypophysitis were younger compared to the non-hypophysitis (NH) patients (median age: 57 years vs. 65 years; p=0.0113). Time from initiation of ICI to death/last follow-up was longer in the H group (20 months vs. 10.8 months; p=.0013). 87.5% of group H as compared to 47.3% of NH were alive at end of data collection (p=0.0008). In the H cohort, 43.8% (7/16) received combination CTLA-4/PD-1 inhibitors, 43.8% (7/16) PD-1 monotherapy, 6.25% (1/16) CTLA-4 monotherapy, and 6.25% (1/16) PD-L1 monotherapy. Forty-six percent (6/13) of group H had radiographic evidence of hypophysitis on MRI that resolved on follow up imaging. Six patients had concurrent thyroiditis, 2/16 had concurrent type 1 diabetes, 9/16 had central hypothyroidism, 1/16 had secondary hypogonadism, and 2/16 had GH deficiency. In 15/16 patients, secondary AI presented as the first endocrinopathy, while one patient presented with central hypothyroidism. Conclusions: Development of hypophysitis following ICI therapy was associated with a longer survival. Deficiencies in multiple pituitary hormones occurred in the majority and non-pituitary endocrinopathies occurred in half the cases. High dose steroid usage can also present as secondary AI, making the diagnosis of ICI induced hypophysitis difficult. Further pituitary evaluation must be conducted to differentiate secondary AI due to exogenous steroids from hypophysitis from ICI therapy.

Zygomatic Approach to Cavernous Sinus Chordoma: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Antonio Aversa ◽  
Ossama Al-Mefty
Keyword(s):  
Carotid Artery ◽  
Cavernous Sinus ◽  
Infratemporal Fossa ◽  
Cranial Nerves ◽  
Radical Resection ◽  
High Rate ◽  
Petrous Apex ◽  
High Dose ◽  
Middle Fossa ◽  
Cavernous Carotid Artery

Abstract Chordoma is not a benign disease. It grows invasively, has a high rate of local recurrence, metastasizes, and seeds in the surgical field.1 Thus, chordoma should be treated aggressively with radical resection that includes the soft tissue mass and the involved surrounding bone that contains islands of chordoma.2–5 High-dose radiation, commonly by proton beam therapy, is administered after gross total resection for long-term control. About half of chordoma cases occupy the cavernous sinus space and resecting this extension is crucial to obtain radical resection. Fortunately, the cavernous sinus proper extension is the easier part to remove and pre-existing cranial nerves deficit has good chance of recovery. As chordomas originate and are always present extradurally (prior to invading the dura), an extradural access to chordomas is the natural way for radical resection without brain manipulation. The zygomatic approach is key to the middle fossa, cavernous sinus, petrous apex, and infratemporal fossa; it minimizes the depth of field and is highly advantageous in chordoma located mainly lateral to the cavernous carotid artery.6–12 This article demonstrates the advantages of this approach, including the mobilization of the zygomatic arch alleviating temporal lobe retraction, the peeling of the middle fossa dura for exposure of the cavernous sinus, the safe dissection of the trigeminal and oculomotor nerves, and total control of the petrous and cavernous carotid artery. Tumor extensions to the sphenoid sinus, sella, petrous apex, and clivus can be removed. The patient is a 30-yr-old who consented for surgery.

scholarly journals SAT-241 Pituitary Stalk Interruption Syndrome Presenting as Neonatal Hypotonia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Nordie Anne Bilbao
Keyword(s):  
Pituitary Gland ◽  
Neonatal Period ◽  
Hormone Replacement ◽  
Brain Mri ◽  
Rare Condition ◽  
Pituitary Stalk ◽  
Pituitary Hormone ◽  
Thyroid Function Tests ◽  
Acth Stimulation ◽  
Central Hypothyroidism

Abstract Pituitary stalk interruption syndrome (PSIS) is a rare condition that include congenital anatomic abnormalities of the pituitary gland and hypopituitarism. There is a wide variety of clinical presentation, with the age at presentation encompassing from neonatal period to adulthood and including one or more pituitary hormone deficiencies. In recent literature there is increasing recognition of PSIS presenting in the neonatal period, mostly involving hypoglycemia. Our patient is a full-term male infant who presented in the newborn period with hypotonia and hypothermia. He also had hypoglycemia, which was initially thought to be associated to hyperinsulinism in the context of gestational diabetes. Micropenis was noted on physical exam. As part of the study for hypotonia, serial thyroid function tests were obtained revealing central hypothyroidism. A low dose ACTH stimulation test was performed which revealed adrenal insufficiency. The patient was started on cortisol and thyroid hormone replacement. Brain MRI showed an ectopic neurohypophysis located along the floor of the hypothalamus, a small anterior pituitary gland, and a partially absent infundibulum, findings consistent with pituitary stalk interruption syndrome. The patient received testosterone injections for micropenis and is being followed for development of other pituitary hormone deficiencies. PSIS is a rare congenital condition that is increasingly recognized in neonates manifesting with signs of hypopituitarism.

scholarly journals SUN-432 A Case of Sellar Plasmacytoma Masquerading as Pituitary Carcinoma with Hypopituitarism

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Christine Lee ◽  
Marilyn Tan
Keyword(s):  
Multiple Myeloma ◽  
Complete Blood Count ◽  
Rapid Development ◽  
Clinical Manifestations ◽  
Pituitary Carcinoma ◽  
Pituitary Hormone ◽  
Mri Brain ◽  
Imaging Characteristics ◽  
Cranial Nerve Palsies ◽  
New Findings

Abstract Background: Sellar plasmacytomas are rare tumors arising from clonal plasma cells localized to the sellar/parasellar region. They may mimic other pituitary pathology such as pituitary adenomas, chordomas, meningiomas or metastatic carcinomas in both clinical manifestations and radiographic evidence. Certain features such as predominance of cranial nerve palsies and absence of hypopituitarism appear to be more common with sellar plasmacytomas. We present an interesting case of a sellar plasmacytoma, initially mistaken for pituitary carcinoma, with rapid development of hypopituitarism and systemic multiple myeloma. Case: A 75 year old healthy male presented with sudden onset left eye diplopia and retro-orbital pain. Left eye ptosis and diplopia with left lateral and upward gaze were noted on exam. Initial MRI brain revealed a 17mm soft tissue mass involving pituitary and clivus, extending into the left superior orbital fissure, concerning for pituitary carcinoma. With the exception of low LH (1.2) and testosterone (25), baseline pituitary hormone labs, complete blood count and metabolic panel were within normal limits. Within 1 month, he acutely presented with fever, confusion, weakness, and bony pains. Repeat pituitary labs showed low ACTH < 5, AM cortisol 4.3, TSH 0.07, FT4 0.5. New findings of acute renal injury, hypercalcemia, anemia with elevated Mspike, Kappa light chain 39.7 (0.3-2.0mg/dl), kappa/lamda ratio 66.2 (0.3-1.6) and beta2microglobulin 4484 were found. Repeat MRI brain noted multifocal osseous metastatic disease with left sellar and cavernous sinus lesion contiguous with clival osseous lesion displacing pituitary gland, concerning for plasmacytoma. PET/CT showed focal lytic lesions in clivus and posterior left iliac bone. Bone marrow biopsy confirmed 70% myeloma involvement with kappa monotypic plasma cell population. He was started on hydrocortisone, levothyroxine, chemotherapy and XRT. Conclusion: The rarity of plasmacytomas, combined with their clinical presentation and imaging characteristics similar to other sellar tumors, can often result in misdiagnosis. Accuracy of diagnosis is critical, as sellar plasmacytomas and underlying multiple myeloma (present in 50% of cases) require XRT and systemic chemotherapy respectively rather than surgery. Although pituitary function is usually preserved in plasmacytomas, our case shows that acute anterior pituitary dysfunction can be an early presenting sign.

scholarly journals Low FT4 Concentrations around the Start of Recombinant Human Growth Hormone Treatment: Predictor of Congenital Structural Hypothalamic-Pituitary Abnormalities?

2018 ◽  
Vol 89 (2) ◽  
pp. 98-107 ◽  
Author(s):  
Laura van Iersel ◽  
Hanneke M. van Santen ◽  
Gladys R.J. Zandwijken ◽  
Nitash Zwaveling-Soonawala ◽  
Anita C.S. Hokken-Koelega ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Treatment ◽  
Recombinant Human Growth Hormone ◽  
Human Growth ◽  
Hormone Treatment ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Gh Treatment ◽  
Central Hypothyroidism ◽  
Pituitary Disease

Background: Growth hormone (GH) treatment may unmask central hypothyroidism (CeH). This was first observed in children with GH deficiency (GHD), later also in adults with GHD due to acquired “organic” pituitary disease. We hypothesized that newly diagnosed CeH in children after starting GH treatment for nonacquired, apparent isolated GHD points to congenital “organic” pituitary disease. Methods: Nationwide, retrospective cohort study including all children with nonacquired GHD between 2001 and 2011 in The Netherlands. The prevalence of CeH, hypothalamic-pituitary (HP) abnormalities, and neonatal congenital hypothyroidism screening results were evaluated. Results: Twenty-three (6.3%) of 367 children with apparent isolated GHD were prescribed LT4 for presumed CeH within 2 years after starting GH treatment. Similarly to children already diagnosed with multiple pituitary hormone deficiency, 75% of these 23 had structural HP abnormalities. In children not prescribed LT4, low pre- or post-GH treatment FT4 concentrations were also associated with structural HP abnormalities. Neonatal screening results of only 4 of the 23 children could be retrieved. Conclusion: In children with nonacquired, apparent isolated GHD, a diagnosis of CeH after, or a low FT4 concentration around the start of GH treatment, is associated with congenital structural HP abnormalities, i.e., “organic” pituitary disease. Neonatal values could not be judged reliably.

scholarly journals ML-05 A CASE OF NEUROLYMPHOMATOSIS ARISED SECONDARILY FROM PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii33-ii33
Author(s):  
Keishi Horiguchi ◽  
Hirofumi Asano ◽  
Tadashi Osawa ◽  
Masahiko Tosaka ◽  
Yuhei Yoshimoto
Keyword(s):  
Spinal Cord ◽  
Fdg Pet ◽  
Peripheral Nerves ◽  
Initial Treatment ◽  
Cervical Spinal Cord ◽  
Intrathecal Injection ◽  
Cranial Nerves ◽  
Central Nervous System Lymphoma ◽  
High Dose ◽  
Whole Brain Irradiation

Abstract A woman in her 40s. A biopsy of multiple intracranial lesions was performed, and the patient was diagnosed with DLBCL. As an initial treatment, 6 courses of high dose MTX therapy were performed and CR was achieved. Radiation therapy was not desired by the patient. On the 19th month after initial treatment, tumor recurrence was confirmed by MRI and added 2 courses of HD-MTX. On the 23rd month, second recurrence around the left basal ganglia were observed. One additional course of HD-MTX was performed, but due to the appearance of renal damage that was thought to be acute tubular necrosis, additional HD-MTX was not performed and whole brain irradiation was performed. She began complaining of pain in the trunk and extremities during radiation. When MRI and FDG-PET were performed in the 25th month, multiple lesions were found in the ganglia, plexus, and peripheral nerves from the cervical spinal cord to the sacral spinal cord. Cerebrospinal fluid cytology revealed atypical lymphocytes and lymphoma dissemination in the spinal cord. When intrathecal administration of the anticancer agent was performed nine times weekly, the CSF cytology was negative. Imaging findings showed that the lesions relapsed, although the lesions were temporarily reduced. After confirming that the renal function had recovered, two additional courses of HD-MTX were performed. Accumulation of FDG-PET in the lesion disappeared in the 29th month. However, peripheral neuropathic pain and paraplegia remained. Discussion: Neurolymphomatosis is considered to be a clinically rare disease that presents invasion of lymphoma into peripheral nerves including the cranial nerves, nerve roots or plexus. Diagnosis of NL has been required to be proved by nerve biopsy or autopsy, but noninvasive FDG-PET has been reported to be effective. In this case, CR was not obtained with anticancer drug intrathecal injection, and HD-MTX therapy was successful.

scholarly journals Primary Burkitt Lymphoma of the Fourth Ventricle in an Immunocompetent Young Patient

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Abdulrahman Alabdulsalam ◽  
Syed Z. A. Zaidi ◽  
Imran Tailor ◽  
Yasser Orz ◽  
Sadeq Al-Dandan
Keyword(s):  
Burkitt Lymphoma ◽  
Cranial Nerves ◽  
High Dose ◽  
Rare Entity ◽  
Diagnostic Challenge ◽  
Dose Methotrexate ◽  
The Central Nervous System ◽  
Blue Cell ◽  
The Brain ◽  
4Th Ventricle

Primary Burkitt lymphoma of the central nervous system (CNS) is rare, with only few cases reported in the literature. An 18 year-old immunocompetent male presented with multiple cranial nerves palsies and was found to have a mass predominantly in the 4th ventricle of the brain. Tumor was surgically removed and showed morphological and immunohistochemical features consistent with Burkitt lymphoma. The patient responded very well to anthracycline based chemotherapy with high dose methotrexate (HD MTX) and intrathecal (IT) chemotherapy delivered by Ommaya reservoir. Primary Burkitt lymphoma of the CNS is a rare entity that poses differential diagnostic challenge with other small round blue cell tumors.

scholarly journals MON-096 Endocrine Complications Following Cancer Treatment in Survivors of Pediatric Solid Tumors: 18 Years’ Experience of a Single Academic Center

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Yena Lee ◽  
Arum Oh ◽  
Jin-Ho Choi ◽  
Han-Wook Yoo
Keyword(s):  
Cancer Treatment ◽  
Solid Tumors ◽  
High Dose ◽  
Pituitary Hormone ◽  
Hypergonadotropic Hypogonadism ◽  
Hematopoietic Stem ◽  
Pediatric Solid Tumors ◽  
The Mean ◽  
Endocrine Complications

Abstract Background: Survival rates of pediatric cancer have been significantly improved over recent decades because of advances in chemotherapy and radiotherapy. The endocrine consequences of cancer treatment have become the major medical issues in the childhood cancer survivors. This study was performed to investigate the long-term endocrine complications in survivors of pediatric solid tumors. Methods: From 2000 to 2018, 402 patients were diagnosed with solid tumors including hepatoblastoma (n = 72), neuroblastoma (n = 117), Wilms tumor (n = 57), Ewing sarcoma (n = 40), osteosarcoma (n = 65), and rhabdomyosarcoma (n = 51) in our institute. Among them, 96 patients (24%) were expired during the follow-up period. Growth profiles and endocrinologic findings were analyzed by retrospective chart review in 306 survivors of solid tumors. Results: The median age at diagnosis of primary cancer was 3 years (range, 0 month to 18 years). The mean treatment duration was 11.7 ± 12.6 months, and the mean follow-up duration after cancer treatment was 7.1 ± 4.8 years. Short stature,which was defined by height-SDS below -2.0, was found in 39 patients (12.7%) with the mean height-SDS of -2.59 ± 0.45. Primary hypothyroidism was detected in 19 patients (6.2%), and 15 of them were treated with radiotherapy or 131I-MIBG therapy due to the metastatic neuroblastoma. Sixteen patients (5.2%) developed hypergonadotropic hypogonadism, whereas three patients (1%) were diagnosed with central precocious puberty. Vitamin D deficiency and osteoporosis were found in 4 patients (1.3%) and 3 patients (1%), respectively. Primary adrenal insufficiency was found in one patient who underwent bilateral adrenalectomy because of bilateral neuroblastoma. One patient with rhabdomyosarcoma in the nasal cavity underwent high dose radiotherapy (50.4 Gy) around the tumor site, eventually leading to multiple pituitary hormone deficiency. In multivariable analysis, longer duration of treatment (≥24 months) was associated with the endocrine complications (OR = 3.94; CI 1.41-11.06) and hematopoietic stem cell transplantation was a major risk factor for endocrine complications (OR = 4.70; CI 2.14-10.29). Conclusions: Various endocrine complications can occur in survivors of solid tumors in children and adolescents caused by treatment modalities including surgery, chemotherapy, and radiotherapy, rather than the tumor itself. Lifetime monitoring is necessary to detect endocrine consequences such as growth retardation, hypergonadotropic hypogonadism, and thyroid dysfunctions.

scholarly journals Co Existence of a Corticotroph Adenoma and a Multifunctional Pituitary Cyst

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A578-A578
Author(s):  
David John McDonnell ◽  
Carmel Kennedy ◽  
Yazan Qaoud ◽  
Karen Heverin ◽  
Diarmuid Smith ◽  
...  
Keyword(s):  
Urinary Free Cortisol ◽  
Needle Aspiration ◽  
Solid Component ◽  
High Dose ◽  
Pituitary Hormone ◽  
Cystic Fluid ◽  
Corticotroph Adenoma ◽  
High Dose Dexamethasone ◽  
Free Cortisol ◽  
Pituitary Cyst

Abstract The co-existence of a corticotroph adenoma and a pituitary cyst is very unusual. We present the case of a 50-year-old female who presented with a Cushingoid phenotype, severe hypokalaemia, hyperglycaemia and hypertension. Urinary free cortisol was markedly elevated at 50 fold. ACTH levels were elevated at 121.4 pg/ml. She failed both the low and high dose dexamethasone suppression tests. The CRF test did not show a satisfactory rise in ACTH levels but the Inferior petrosal sinus sampling revealed a central to peripheral ACTH gradient highly suggestive of Pituitary dependant Cushing’s. MRI pituitary revealed a large cystic lesion with a small solid component. Computed Tomography (CT) of thorax abdomen and pelvis was normal. A trans-sphenoidal hypophysectomy was performed, during which a fine needle aspiration of the intra-cystic fluid was obtained. This showed markedly elevated pituitary hormone levels of ACTH (1399pg/ml), prolactin (353,084mIU/L), TSH (217IU/L) FSH (205mIU/ml) and GH (519 ng/ml) consistent with a multifunctional pituitary cyst. Neuropathology of the solid component confirmed a corticotroph adenoma. Post-operative am cortisol levels were persistently suppressed to less than 50 nmol/l with marked improvement in clinical features. This case highlights the challenges in the work up of Cushing’s syndrome and the limitations of diagnostic tests. The coexistence of a corticotroph adenoma and a multifunctional pituitary cyst is very unusual and to our knowledge has not been reported before.

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