Large Suprasellar Mass Research Articles

BRAF-mutated suprasellar glioblastoma mimicking craniopharyngioma: illustrative case.

Suprasellar masses commonly include craniopharyngiomas and pituitary adenomas. Suprasellar glioblastoma is exceedingly rare with only a few prior case reports in the literature. Suprasellar glioblastoma can mimic craniopharyngioma or other more common suprasellar etiologies preoperatively. A 65-year-old male with no significant history presented to the emergency department with a subacute decline in mental status. Work-up revealed a large suprasellar mass with extension to the right inferior medial frontal lobe and right lateral ventricle, associated with significant vasogenic edema. The patient underwent an interhemispheric transcallosal approach subtotal resection of the interventricular portion of the mass. Pathological analysis revealed glioblastoma, MGMT partially methylated, with a BRAF V600E mutation. Malignant glioblastomas can mimic benign suprasellar masses and should remain on the differential for a diverse set of brain masses with a broad range of radiological and clinical features. For complex cases accessible from the ventricle where the pituitary complex cannot be confidently preserved via a transsphenoidal approach, an interhemispheric approach is also a practical initial surgical option. In addition to providing diagnostic value, molecular profiling may also reveal therapeutically significant gene alterations such as BRAF mutations.

THU101 A Challenging Case Of Recurrent Suprasellar Epidermoid Cyst With Visual Field Deficits

Abstract Disclosure: S. Ahsun: None. A. Atri: None. R. Bhat: None. C. eaton: None. N. Blocher: None. A challenging case of recurrent suprasellar epidermoid cyst with visual field deficits. Introduction: Intracranial epidermoid cysts account for approximately 1% of all intracranial tumors. The most common location of occurrence is cerebellopontine angle. Suprasellar region is the second most common location. We present a case for suprasellar epidermoid cyst with visual field deficits. Case Presentation: A 52 y/o female presented to an ophthalmologist for blurring of vision and was found to have bitemporal hemianopia. MRI of the brain showed a large suprasellar mass of 6.9x4.2x3.3 cm with extension into the surrounding structures. A portion of the cyst wall was hemorrhagic. There was significant mass-effect on the optic chiasm and bilateral optic tracts. Patient underwent elective craniotomy with left frontotemporal approach. A characteristic pearly wide epidermoid capsule was visualized through the oculomotor triangle. The optic nerve was noted to be quite distorted in its course, exiting from the optic canal and being pushed superiorly by the epidermoid tumor. Accessible tumor capsule was resected, however due to the dense adhesions of the epidermoid cyst to the surrounding vasculature and the optic nerves, complete resection was not attempted. Postoperatively, patient developed right-sided hemiparesis due to an acute infarct. Patient also developed some dementia, hyperphagia and aggressive behavior following her surgery. She started her rehabilitation therapy but had persistent dementia and cognitive decline. Repeat vision testing showed right superior homonymous hemianopia. MRI of the brain a year after the initial surgery showed growth of the residual cyst and some obstructive hydrocephalus but without any symptoms. She underwent a second debulking procedure that was successful with complete excision of the residual tumor but the patient had delayed recovery. She subsequently underwent tracheostomy and PEG tube placement and was transferred to the rehabilitation facility. She is now awake but still nonverbal. Patient also developed panhypopituitarism and is currently on the replacement for cortisol and thyroid hormone. She is also on DDAVP for her diabetes insipidus which seems to be permanent in her case. Conclusion: This case highlights some important facts: 1. Epidermoid cysts are challenging from the management standpoint. Only 50-80% can be resected completely. Incomplete removal increases the chances for recurrence which are variable at 1-54%. 2. Even in cases of recurrent growth repeat surgery should only be performed if patient has significant symptoms from the tumor. 3.As per the literature patients with adjuvant radiotherapy have improved outcomes. As in our case radiotherapy should have been considered in the setting of recurrence of the tumor. 4.Endoscopic endonasal approach is a new modality that has shown a very low rate of complications after resection. Presentation: Thursday, June 15, 2023

Ojo con la neurofibromatosis

We present the case of glioma in both optic nerves in a patient with a medical history of neurofibromatosis type 1. The patient presented constant bilateral retro-ocular pain refractory to analgesic treatment and blurred vision. On examination, Lisch nodules were seen on the surface of the iris and pale optic nerves in the fundus. Nuclear magnetic resonance revealed a large suprasellar mass separate from the pituitary glands.

Hypothalamic Inflammation and Hypothalamic Obesity: Case Report and Mini-Review

Context: Autoimmune hypothalamitis (AHT) is extremely rare and appears on the Magnetic resonance Imaging (MRI) as a predominant suprasellar mass. There are only a few case reports of AHT in the literature and none was presented together with hypothalamic obesity (HO). Methods of prediction of hypothalamus (HT) damage are included as the pathogenesis of HO. Case description: A 19-year-old female was operated with a transcranial operation due to a large suprasellar mass with symptoms of visual deficiencies and headache. The diagnosis of AHT was confirmed in specimen of lymphocyte-dominated infiltration with plasma cells, neutrophils and fibrocytes. The operation reduced the AHT mass and a further reduction was accomplished by long-term oral Prednisone. Directly after operation she suffered from hypothalamic obesity which worsened after the introduction of Prednisone treatment. During Prednisone treatment a reduction in the inflammation mass and improvement of vision was recorded. She was treated with glucagon-like peptide 1 receptor agonist and Metformin as treatment for her diabetes mellitus type II and with supplementations of her pituitary insufficiencies. The patient exhibits dramatic weight reduction while on a strict caloric diet at two follow-up occasions. However, this effect was reversed once the patient had stopped the calorie restricted diet. Conclusion: The coexistence of AHT and HO is extremely difficult to treat as the former involves Prednisone treatment. Pharmacological intervention of HO has had limited effect on weight, but extreme diet was successful, however with short endurance.

Retinoblastoma Extending up to the Optic Chiasma and Presenting as a Suprasellar Mass

Retinoblastoma is the most common ocular malignancy of childhood. It is present in childhood with leukocoria and strabismus. Most patients are diagnosed under 3 years of age. Funduscopic examination can reveal an intraocular mass, but imaging is essential for complete evaluation of the lesion. Although ultrasound is a non-invasive and relatively inexpensive screening tool, cross-sectional imaging is required to assess the involvement of optic nerve and intracranial spread. We report a case of retinoblastoma in a 5 year old male child who presented with headache diminution of vision in both eyes. Contrast enhanced magnetic resonance imaging (MRI) revealed an enhancing soft tissue mass in the right eye with involvement of optic nerve and optic chiasma and contiguous with a large suprasellar mass causing hydrocephalus. Non-contrast computed tomography (CT) showed extensive calcifications in the mass lesion.

Underappreciation of the “Hook Effect” leading to Mismanagement of a Patient, a Tale of Preventable Disaster

Abstract Introduction: When evaluating pituitary adenomas, one needs to be cognizant of the “hook effect” - an assay artifact leading to under-reporting of extremely high prolactin levels. We report a case of a patient with a macroprolactinoma with an initial reported prolactin level of less than 1.0 ng/dl. Such an extreme case of the “hook effect” in a prolactinoma has not been reported yet. Case Presentation: A 53-year-old male with a medical history of type II DM initially presented in 2017 with blurred vision. Vital signs and labs, including pituitary hormone levels, were all within normal range. Prolactin level was reported as less than 1.0 ng/dl (normal 2-18 ng/dl). MRI brain showed a large pituitary mass and he was diagnosed with a non-secreting pituitary macroadenoma with mass effect. The patient subsequently underwent transsphenoidal resection of the pituitary adenoma. Prolactin level was noted to be elevated at 1608 ng/dl after the procedure. Unfortunately, the patient was lost to follow up. Two years later, the patient presented again with persistent headaches and worsening vision. MRI brain revealed a large suprasellar mass, measuring at 4.9 x 4 x 3 cm, with extension into the third ventricle and left cavernous sinus, indicating a recurrence of the pituitary adenoma. Prolactin levels measured were elevated 12,030 ng/dl. At this time, the patient was started on cabergoline with significant improvement in symptoms and reduction in prolactin levels to 1792 ng/dl within 4 weeks after initiation of therapy. He is currently still in remission with weekly cabergoline therapy. Conclusion: The most used prolactin assay typically reports the presence of detectable heterotrimeric immune complex formations made up of prolactin antigens sandwiched between ‘capture antibodies‘ at one end and ‘reported antibodies’ at the other end of the prolactin antigen. In prolactinomas, prolactin levels are usually present in relative excess to the two assay antibodies. This leads to the oversaturation of antibody sites with most of prolactin being complexed to only a single antibody instead of two antibodies, thus impairing adequate immune complex formation. Only a few remaining prolactin antigen are “sandwiched” to form the heterotrimeric complex and are therefore reported as “detectable.” As a result, true prolactin levels are substantially underreported, potentially leading to incorrect diagnosis, treatment delay, exposure to surgical risk and complications and increased economic burden to the patient and the healthcare system. The best method to avoid the “hook effect” and to correctly interpret prolactin values would be to obtain prolactin levels in appropriately diluted serum specimens (10-100-fold dilution of the sample). This will lead to appropriate immune complex formations and increase the sensitivity and specificity of the prolactin assay.

LINC-04. POSSIBLE ROLE OF NEOADJUVANT CHEMOTHERAPY IN METASTATIC PURE GERMINOMA IN LOW AND MIDDLE INCOME COUNTRIES. A PRO POS OF A CASE

BACKGROUNDCNS germ cell tumors represent about 3–5 % of pediatric brain tumors, 60% are pure germinomas. Germinomas are very sensitive to chemotherapy which has helped to reduce volume and dose of radiotherapy in localized disease while maintaining excellent survival. In metastatic disease the SIOP GCT-96 trial showed no benefit with addition of chemotherapy to craniospinal irradiation alone. Radiotherapy maybe not readily available in Low/Middle Income Countries (LMIC).METHODWe describe a patient in which the use of neoadjuvant chemotherapy helped to rescue vision. The patient is a 9 year old female with a 3 months history of morning headaches and vomits. Visual decline was noticed a month before admission when the child had completely loss vision of the right eye and left eye was partially affected. MRI showed a large suprasellar mass with ventricular nodules. Beta-hGC in CSF was mildly elevated. Patient received 2 cycles of carboplatin/etoposide. After first cycle there was a complete vision recovery in both eyes. After the second course the MRI showed complete response in primary and metastatic disease. Patient received CSI (24Gy + 16 Gy Boost) after 2 cycles of chemotherapy. Chemotherapy was very well tolerated without side effects. Patient vision is 20/20 in both eyes without deficit in visual fields.CONCLUSIONAlthough the addition of chemotherapy in metastatic germinoma has no clear role in reducing radiotherapy it could possible help selected patients in attempt to rescue vision when radiotherapy is not readily available.

MON-260 Sinonasal Papilloma Masquerading as a Pituitary Macroadenoma

Background: Sinonasal tumors are rare, with annual worldwide incidence of approximately 1 in 100,000, and are not commonly considered in the differential diagnosis of pituitary tumors (1). Sinonasal tumors are well known for their invasiveness, tendency to recur and association with malignancy. We present a case of sinonasal papilloma presenting as a large suprasellar mass. Clinical Case: A 61 year-old male with a past medical history including type 2 diabetes mellitus presented with chief complaints of headaches and visual disturbances over the past 6 months. Prior to admission he experienced episodes of left eye midline deviation associated with diplopia. New onset dysphagia associated with leftward tongue deviation prompted him to seek medical attention. The social history was notable for chemical exposures in his work at a hair salon; he is sexually active with his husband. He has had no sexually transmitted infections and has been vaccinated against human papilloma virus (HPV). CT of the brain showed a large sellar mass.A subsequent MRI of the pituitary demonstrated a large destructive mass centered on the clivus elevating the pituitary gland into the suprasellar cistern. The mass measured 6 cm x 4.5 cm in the axial plane with displacement without invasion of the cavernous sinuses. The mass extended anteriorly into the ethmoid sinuses and extended posteriorly into the prepontine cistern displacing the basilar artery.Pituitary hormonal analysis included a 250 mcg Cosyntropin stimulation test resulting with a random cortisol of <1.0 ug/dl rising to 17.7 ug/dl following Cosyntropin administration. Additional anterior pituitary results included FSH of 3.8 mIU/ml (1.5-14 mIU/ml), LH of 1.3 mIU/ml (1.4-7.7 mIU/ml), total testosterone of 230 ng/dl (300-700 ng/dl), and prolactin 11.1 ng/ml (2.6-13 ng/ml). Ophthalmology was consulted for visual field testing which proved normal, however a partial left cranial nerve VI palsy was noted likely secondary to cavernous sinus involvement. A biopsy of the sellar mass was obtained by bedside nasal endoscopy. The initial biopsy was consistent with a non-dysplastic, inverted sinonasal papilloma with negative HPV and P16 serologies. The patient underwent resection of the pituitary mass, with surgical pathology showing superficially invasive squamous cell carcinoma arising from sinonasal papilloma. Conclusion: This is one of the very few cases reported in the literature of a sinonasal papilloma masquerading as a pituitary mass. Sinonasal papilloma should be considered when evaluating large destructive suprasellar tumors. Although a benign tumor, the local aggressiveness of sinonasal papilloma and the potential to give rise to squamous cell carcinoma highlights the significance of identifying this lesion.

Encephalocraniocutaneous Lipomatosis

A 5-year-old boy presented with worsening headaches for 3 months. On examination, he was found to have a hairless fatty tissue nevus of the scalp (nevus psiloliparus), subcutaneous soft tissue masses on the right side of his face, neck, mandible and right buttock and epibulbar dermoid of the right eye (choristoma) (). Magnetic resonance imaging revealed a large suprasellar mass, which was debulked and found to be a pilocytic astrocytoma. Testing was not performed for the BRAF/KIAA1549 fusion or BRAFV600E mutation. Seven years later, he was started on adjuvant chemotherapy for gradual tumor progression. Over the ensuing 3 years, he had further disease progression despite treatment with 3 frontline chemotherapy regimens: vinblastine, carboplatin/vincristine, and irinotecan/bevacizumab. Targeted sequencing of tissue from the right gluteal mass, revealed a mosaic activating FGFR1 c.1966A>G (p.Lys656Glu) mutation, absent in normal left gluteal tissue, confirming the diagnosis of encephalocraniocutaneous lipomatosis (ECCL), belonging to the family of RASopathies (including neurofibromatosis type I, Noonan syndrome, Costello syndrome), with constitutive activation of the mitogen-activated protein kinase (MAPK) pathway, and an increased risk of developing neoplasms. He was started on trametinib, a MEK inhibitor, off-label, targeting the MAPK pathway downstream from FGFR1, with stable tumor size at last follow-up, after 6 months on therapy.

Diencephalic syndrome: a rare cause of failure to thrive

Timely diagnosis of diencephalic syndrome is not often the case for patients presenting with failure to thrive (FTT) because of its rarity and lack of specific symptoms. Herein, we report...

Large suprasellar mass: answer

Teratomas are rare intracranial neoplasms that make up approximately 0.4% of all primary intracranial tumours [1]. They can be divided into extra-axial and intra-axial teratomas [2]. Extra-axial tumours usually arise in the suprasellar or pineal regions and typically present in childhood with a recent case series demonstrating a mean age of 14.5 years [3]. In contrast, intra-axial teratomas are the most common intracranial foetal tumour and present in the antenatal period with a mean gestational age at diagnosis of 27 weeks [4,5]. Intra-axial teratomas are more commonly supratentorial with a predilection for the frontal lobes [5]. Regardless of location, extragonadal teratomas are believed to develop from ectopic, totipotent primordial germ cells that have either been misplaced or undergone aberrant migration during early embryogenesis [2,6]. Because of this cellular origin, teratomas can contain diverse mixed tissues with up to 90% of childhood teratomas containing elements derived from all three embryonic layers (ectodermal, mesodermal and endodermal) [2]. This is in contrast to epidermoid and dermoid cysts which are both derived from ectodermal origin only [2]. Importantly, although dermoid cysts have sebaceous glands which produce liquid oily sebum appearing as fat density on CT scan and on MRI demonstrating T1 shortening, chemical shift artefact and signal attenuation on fat suppressed sequences, they do not contain solid adipose tissue as lipocytes are mesodermal in origin [2,7]. Similarly, dermoid cysts may contain semi-formed teeth but do not contain cartilage or bone [8]. It should be noted, however, that the absence of detectable fat or bone on imaging does not exclude the diagnosis of teratoma [9]. In this patient, the detection on CT scan of fatty tissue and bone within the mass is the key for diagnosis (Question: Fig. 1a–c). The presence of these tissues within a mixed cystic and solid enhancing mass is strongly suggestive of a pre-operative diagnosis of teratoma, as contrast enhancement is rarely seen in dermoid cysts [10]. MRI confirmed the presence of fat adjacent, but separate to, the right posterior clinoid process identified on CT scan as an area of intrinsic high T1 signal with surrounding chemical shift artefact on the T2 sequence (Question: Fig. 1d–f). Histology confirmed the radiological diagnosis of intracranial teratoma (Fig. 1).

Large suprasellar mass: question

Large suprasellar mass: question

Intracranial Germinoma Manifesting as Cavernous Sinus Syndrome

An 11-year-old girl presented with a rare case of neurohypophyseal germinoma manifesting as cavernous sinus syndrome with left abducens nerve palsy. Magnetic resonance (MR) imaging showed a mass located in the left cavernous sinus. Biopsy revealed an inflammatory reaction with lymphocytic infiltration. Postoperative MR imaging showed regression of the mass. Her eye movements were also restored. However, after 5 months, she developed right oculomotor and abducens nerve palsy, and MR imaging showed a large suprasellar mass invading the bilateral cavernous sinuses. The transcranial biopsy sample showed positive staining for placental alkaline phosphatase and c-kit, and the diagnosis of germinoma was confirmed. Chemotherapy and radiotherapy were administered, and the patient has been in complete remission for over 4 years.

Case seminar: a young female with acute hyponatremia and a sellar mass

In familial cases of combined pituitary hormone deficiency the most common mutations are that of Prophet of Pit 1 (PROP1) gene. PROP1 mutations are associated with deficiencies of growth hormone, thyrotropin, prolactin, and gonadotropins (follicle-stimulating hormone and luteinizing hormone), with evolving adrenocorticotropin (ACTH) deficiency in some cases. On imaging in most patients the pituitary gland is hypoplastic, but occasionally transient pituitary enlargement is found. We report a 22-year-old female initially diagnosed at age 12 with familial hypopituitarism due to PROP1 mutation, who presented with coma and respiratory arrest (acute hyponatremia). She was urgently treated in Intensive Care Unit of Emergency Center with hypertonic saline and stress doses of hydrocortisone, which resulted in the fast increase of plasma osmolality resulting in the osmotic demyelination syndrome. Simultaneously and incidentally on computed tomography scan a large sellar and suprasellar mass were reported as possible Rathke's cleft cyst or craniopharyngioma. Once the patient was stable, ACTH deficiency was documented. She remained replaced with hydrocortisone and subsequently underwent transphenoidal surgery. The removed sellar content revealed no pituitary adenoma or pituitary cells, but only an eosinophilic, colloid-like mass, and necrotic acellular debris. Her sister with hypopituitarism had an empty sella. Genetic testing in both sisters revealed the same homozygous c.150delA mutation in PROP1 gene. Here we report two sisters with the same PROP1 mutation who presented in adulthood with different pituitary morphology, one of them with a large sellar and suprasellar mass, in which transphenoidal surgery provided an extremely rare opportunity for a histopathological analysis of the sellar content. Due to the lack of endocrine care during the transition period hypocortisolism which evolved, a consequence of PROP1 mutation, was not recognized. Empirical use of hydrocortisone in the Intensive Care in our patient with life-threatening acute hyponatremia was appropriate but because glucocorticoid therapy on its own corrects hyponatremia even after stopping hypertonic saline infusion, the risk for over-correction of hyponatremia in ACTH deficiency is high.

Pituitary blastoma: a unique embryonal tumor

Pituitary blastoma, a recently described tumor of the neonatal pituitary, exhibits differentiation to Rathke epithelium and adenohypophysial cells of folliculostellate and secretory type, a reflection of arrested pituitary development and unchecked proliferation (Scheithauer et al. in Acta Neuropathol 116(6):657-666, 2008). Herein, we report the pathologic features of three additional cases, all ACTH-producing. One involved a 9-month-old male presenting with progressive right ophthalmoplegia, MRI findings of a large suprasellar mass with cavernous sinus invasion, and elevated plasma ACTH levels. The second was nonfunctioning and occurred in a 13-month-old female with right third nerve palsy. The third had been previously published as a "pituitary adenoma" in a 2-year-old female (Min et al. in Pathol Int 57(9):600-605, 2007). The subtotally resected tumors were subject to histochemical, immunohistochemical and, in two cases, ultrastructural study. Histologically, the complex tumors consisted of glands of varying from rosettes to glandular structures resembling Rathke epithelium, small undifferentiated-appearing cells (blastema), and large secretory cells. Mucin-producing goblet cells were noted in case 3. Cell proliferation was high in two cases and low in case 3. Immunoreactivity of the secretory cells included synaptophysin, chromogranin, various keratins and, to a lesser extent, ACTH and beta endorphin. MGMT immunolabeling was 40-60%. Mitotic activity was moderate to high in cases 1 and 2 and was low in case 3. The same was true for MIB-1 labeling. Germ cell markers were lacking in all cases. One tumor ultrastructurally consisted of three cell populations including (a) small, polyhedral, primitive-appearing cells (blastema) with scant cytoplasm, abundant glycogen and few organelles, (b) folliculostellate cells and (c) large corticotroph cells containing rough endoplasmic reticulum, golgi membranes, spherical, 150-400 nm secretory granules and occasional perinuclear, intermediate filament bundles. A second example (case 3) lacked a blastema and glandular component. The clinical and morphologic features of our three cases were those of pituitary blastoma. The finding of cellular elements of adenohypophysial development is consistent with a diagnosis of pituitary blastoma and aligns it with blastomas of other organs. It also suggests an underlying specific genetic abnormality. Marked variations in cellular proliferative activity suggest blastomas occur in low- and higher-grade form. Variable MGMT reactivity suggests an incomplete response to temozolomide therapy. Literature regarding similar morphologically complex, infantile, Cushing disease-associated lesions is briefly reviewed.

An endoscopic modification of the simultaneous ‘above and below’ approach to large pituitary adenomas

Surgical resections of large-to-giant pituitary adenomas (PA) are technically challenging procedures. Tumors with a fibrous consistency or ‘hour-glass’ configurations are particularly difficult to remove completely and safely through the transsphenoidal route alone. Although the transcranial approach can facilitate the removal of a large suprasellar mass, it may be associated with significant bleeding within the intradural space. A simultaneous microscopic transcranial and transsphenoidal approach has been described as an alternative surgical strategy. We have further modified this ‘above and below’ approach by adopting endoscopic techniques for the transsphenoidal part of the procedure. This modified approach has the advantages of requiring only one operating microscope, and permitting freer maneuvers and easier orientation for both surgical teams. We present two patients successfully treated with this approach. Complete tumor removal was achieved and both patients achieved satisfactory functional recovery.

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Benign histiocytic proliferations are identified by their component cells and classified as either Langerhans cell histiocytosis or non-Langerhans cell histiocytosis. We report a 58-year-old Caucasian woman who presented with diabetes insipidus and was found to harbor a large suprasellar mass. Histopathological analysis was consistent with non-LCH. The differential diagnoses included juvenile xanthogranuloma, adult-onset xanthogranuloma, xanthoma disseminatum, Rosai-Dorfman disease, and Erdheim-Chester disease. Immunohistochemical examination demonstrated a proliferation of large lipid-laden histiocytic cells which were positive for CD68, negative for S100 protein, and showed only faint, background staining for CD1a. We present a case of an autopsy-confirmed non-Langerhans cell histiocytosis limited to the central nervous system and evaluated with both immunohistochemical and ultrastructural studies. Based on the multifocality, anatomic distribution, and immunostaining features, a diagnosis of Erdheim-Chester disease was made. This is only the second reported case of Erdheim-Chester disease with intracranial involvement but absence of extracerebral manifestations. Given the overlapping clinicopathologic, radiographic, and immunohistochemical profiles, differentiating between these rare histiocytic disorders can often present a significant diagnostic challenge. A systematic approach using all available clinical, laboratory, radiographic, histologic, immunohistochemical and ultrastructural data is essential for proper discrimination between the numerous histiocytoses.

Non-Metastatic Suprasellar Germinoma in A Child: Case Report

Eleven-year-old girl presented with visual disturbance, diabetes insipidus and hypothyroidism. The lesion in magnetic resonance imaging (MRI) was established as a large suprasellar mass stretching the optic chiasm and pituitary stalk. The lesion showed intense contrast enhancement by intravenous administration of a contrast media. MRI examination of whole spine with contrast showed no pathology. Tumor markers such as alpha-fetoprotein (AFP) and human chorionic gonadotrophin (BHCG) in the serum and cerebrospinal fluid were at normal range. Paranasal or bone invasion and spread of subarachnoid space were not detected. Total resection of the lesion was successfully achieved. Histopathological examination revealed germinoma. After surgery, the patient was treated with the combined approach of adjuvant chemotherapy and radiotherapy.

Picture quiz: Diabetes insipidus in a woman with breast cancer

A 43 year old woman was admitted with a two week history of intermittent headache and a three month history of progressive malaise and headache. She had a history of node positive lobular breast cancer, treated with right mastectomy, chemotherapy, and tamoxifen eight years before. Fig 1 Postcontrast sagittal magnetic resonance imaging, showing suprasellar mass Magnetic resonance imaging of the brain showed a large suprasellar mass (figure). Visual acuity was normal and visual fields were full. Baseline and dynamic endocrine investigation confirmed anterior pituitary dysfunction affecting the glucocorticoid (morning cortisol low at 21 nmol/l, with adequate adrenal response to synthacten); growth hormone (no response to growth hormone after giving glucagon); and thyroid axis (inappropriately normal thyroid stimulating hormone at 2.76 mU/l with reduced thyroxine at 8.00 pmol/l). Prolactin concentrations were within the normal range at 132 mU/l. She was treated with replacement hydrocortisone and thyroxine. A few weeks later she was admitted with polyuria and polydipsia. A paired serum concentration of 308 mmol/kg with …

Hypertriglyceridemia-induced pancreatitis: A case-based review

Hypertriglyceridemia is an established cause of pancreatitis. In a case-based approach, we present a review of hypertriglyceridemia and how it can cause pancreatitis. We outline how to investigate and manage such patients. A 35 year old man presented to the emergency department with abdominal pain and biochemical evidence of acute pancreatitis. There was no history of alcohol consumption and biliary imaging was normal. The only relevant past medical history was that of mild hyperlipidemia, treated with diet alone. Physical exam revealed epigastric tenderness, right lateral rectus palsy, lipemia retinalis, bitemporal hemianopsia and a delay in the relaxation phase of his ankle reflexes. Subsequent laboratory investigation revealed marked hypertriglyceridemia and panhypopituarism. An enhanced CT scan of the head revealed a large suprasellar mass impinging on the optic chiasm and hypothalamus. The patient was treated supportively; thyroid replacement and lipid lowering agents were started. He underwent a successful resection of a craniopharyngioma. Post-operatively, the patient did well on hormone replacement therapy. He has had no further attacks of pancreatitis. This case highlights many of the factors involved in the regulation of triglyceride metabolism. We review the common causes of hypertriglyceridemia and the proposed mechanisms resulting in pancreatitis. The incidence and management of hypertriglyceridemia-induced pancreatitis are also discussed.