Intracranial Germinoma Masquerading as Secondary Granulomatous Hypophysitis: A Case Report and Review of Literature.

Germinomas are highly immunogenic tumors eliciting a strong peri-tumoral immune response that can spillover into the surrounding healthy tissues. This phenomenon can also occur in intracranial germinomas, manifesting as secondary hypophysitis. Herein, we report a case of 12-year-old-girl presenting with polyuria and polydispsia. She had central diabetes insipidus (CDI) and panhypopituitarism. Imaging revealed a sellar-suprasellar mass with infundibular stalk thickening. Transphenoidal biopsy revealed epithelioid granulomas with immunostaining negative for germinomatous cells. Other causes of hypophysitis were ruled out. Accordingly, she was diagnosed as primary granulomatous hypophysitis and treated with high-dose corticosteroids. Three years later she again presented with headache, vomiting and diminution of vision. Imaging showed a heterogeneous, solid-cystic peripheral rim-enhancing lesion at the same location with involvement of hypothalamus, ependyma and pineal gland. Cerebrospinal fluid beta-human chorionic gonadotropin was markedly elevated, confirming the diagnosis of an intracranial germ cell tumor. She was started on chemotherapy; however, she succumbed to febrile neutropenia. We performed a literature search and found 18 anecdotal cases of secondary hypophysitis associated with intracranial germinomas. There was a slight male preponderance (male:female 5:4). Two-thirds of the cases were below 18 years of age. Polyuria was the most common presenting manifestation (83%). CDI and panhypopituitarism were seen in 89 and 78% cases, respectively. Imaging evidence of pituitary stalk thickening was seen in 12 cases (67%), while pituitary enlargement and/or sellar mass were reported in 11 cases (61%). Pineal involvement was extremely rare, being reported in only 1 case, implying the predilection of suprasellar (rather than pineal) germinomas in causing secondary hypophysitis. Histologically, 82% had lymphocytic hypophysitis, while 18% had granulomatous hypophysitis. Initially, the diagnosis of germinoma was missed in 60% of the cases who were wrongly treated with corticosteroids. To conclude, physicians should make it a dictum that all children and adolescents presenting with CDI and pituitary stalk thickening should be rigorously screened for an underlying intracranial germinoma before labeling them as primary hypophysitis.

Impact of transsphenoidal surgery on asymptomatic cardiomyopathy in patients with acromegaly. A single-blinded study.

BACKGROUND: Patients with acromegaly have 2-3 times the expected mortality rates primarily due to cardiovascular risks. Echocardiographic studies showing improvement of cardiac function following transsphenoidal surgery (TSS) are limited. MATERIALS AND METHODS: All patients with acromegaly underwent preoperative echocardiography and tissue Doppler (Philips i33, 3D ECHO) for assessment of cardiac indices. In the prospective group of patients, echocardiography was repeated after 6 months of surgery. In the retrospective group of patients, echocardiography was performed for left ventricular diastolic dysfunction and ejection fraction. Biochemical cure was confirmed at least after 6 months by glucose-suppressed plasma growth hormone (GH) concentrations (GH-OGTT) of less than 0.4 ng/ml, random GH of less than 1 ng/ml, and normal age-corrected insulin-like growth factor (IGF-1) values. RESULTS: In the prospective group (38 patients), there was a significant decrease in the left ventricular mass (LVM) and LVM index (LVMI) in patients who were cured as well as in patients with postoperative growth hormone (GH) 1-5 ng/ml. In the prospective group, LVMI completely normalized in 2 and a new-onset deterioration was detected in 1 patient (who was not cured) and improved in 8 others. Left ventricular systolic function was abnormal at baseline in 18 (47.3%) patients, which normalized in 11 (61.1%) patients postoperatively, and in 7 patients, it improved significantly although it did not normalize completely. There was also a significant improvement in the left ventricular ejection fraction (P = 0.01). Post TSS, in patients with GH-OGTT >5 ng/ml, there was no significant decrease in the LVM, LVMI and ejection fraction (EF). In the retrospective group, 62 patients were analyzed for a change in the EF with a mean follow-up of 20.3 months. There was a significant improvement in the left ventricular EF in patients who were cured (P < 0.001). CONCLUSION: Reduction in growth hormone levels and insulin-like growth factor type 1 can decrease the LVM and LVMI, which directly or indirectly contributes to the improvement in diastolic as well as systolic function and probably mortality.

Dose fractionated gamma knife radiosurgery for large arteriovenous malformations on daily or alternate day schedule outside the linear quadratic model: Proof of concept and early results. A substitute to volume fractionation.

BACKGROUND: To evaluate the feasibility, safety and efficacy of dose fractionated gamma knife radiosurgery (DFGKRS) on a daily schedule beyond the linear quadratic (LQ) model, for large volume arteriovenous malformations (AVMs). MATERIAL AND METHODS: Between 2012-16, 14 patients of large AVMs (median volume 26.5 cc) unsuitable for surgery or embolization were treated in 2-3 of DFGKRS sessions. The Leksell G frame was kept in situ during the whole procedure. 86% (n = 12) patients had radiologic evidence of bleed, and 43% (n = 6) had presented with a history of seizures. 57% (n = 8) patients received a daily treatment for 3 days and 43% (n = 6) were on an alternate day (2 fractions) regimen. The marginal dose was split into 2 or 3 fractions of the ideal prescription dose of a single fraction of 23-25 Gy. RESULTS: The median follow up period was 35.6 months (8-57 months). In the three-fraction scheme, the marginal dose ranged from 8.9-11.5 Gy, while in the two-fraction scheme, the marginal dose ranged from 11.3-15 Gy at 50% per fraction. Headache (43%, n = 6) was the most common early postoperative complication, which was controlled with short course steroids. Follow up evaluation of at least three years was achieved in seven patients, who have shown complete nidus obliteration in 43% patients while the obliteration has been in the range of 50-99% in rest of the patients. Overall, there was a 67.8% reduction in the AVM volume at 3 years. Nidus obliteration at 3 years showed a significant rank order correlation with the cumulative prescription dose (p 0.95, P value 0.01), with attainment of near-total (more than 95%) obliteration rates beyond 29 Gy of the cumulative prescription dose. No patient receiving a cumulative prescription dose of less than 31 Gy had any severe adverse reaction. In co-variate adjusted ordinal regression, only the cumulative prescription dose had a significant correlation with common terminology criteria for adverse events (CTCAE) severity (P value 0.04), independent of age, AVM volume, number of fractions and volume of brain receiving atleast 8 Gy of radiation. CONCLUSION: DFGKRS is feasible for large AVMs with a fair nidus obliteration rate and acceptable toxicity. Cumulative prescription dose seems to be the most significant independent predictor for outcome following DFGKRS with 29-30 Gy resulting in a fair nidus obliteration with least adverse events.

Pushing the limits of the Leksell stereotactic frame for spinal lesions up to C3: fixation at the maxilla.

BACKGROUND: Spinal radiosurgery is not considered in the domain of traditional Gamma Knife radiosurgery (GKRS) setup. The major obstacles in GKRS for upper cervical spine lesions remain in difficulty of frame fixation, avoiding collision and maintaining the integrity of the relative position of the lesion from image acquisition to treatment. METHODOLOGY: The supraorbital margin remains the standard lowest fixation point for Leksell stereotactic frame. We describe fixation at the maxilla to target and treat upper cervical spine lesions (up to C3 vertebra) with measures to ensure cervical immobilisation and precision of the GKRS treatment. RESULTS: We have treated two patients at the upper cervical spine up to C3 vertebra by fixing anterior pillars of the Leksell stereotactic frame at the maxilla. To ensure cervical immobilisation and precision of treatment, the neck was immobilised with a Philadelphia collar. The relative position between the head and sternum with the couch from image acquisition to the radiation delivery was kept constant. Docking angle was kept neutral (90 degrees) throughout the treatment (from image acquisition to actual treatment). CONCLUSIONS: The maxilla is a potential alternative for stereotactic frame fixation. Measures to ensure cervical immobilisation with lower-down frame position permits treatment of lesions as low as C3 vertebra.

Can immediate postoperative random growth hormone levels predict long-term cure in patients with acromegaly?

BACKGROUND: Growth hormone (GH) levels following oral glucose tolerance test (OGTT) at 12 weeks or later after surgery have been accepted as the most reliable parameter for defining remission and/or cure in patients with acromegaly. However, the role of random GH in predicting remission in the immediate postoperative period using modern criteria is not known. This study was undertaken to evaluate the role of random GH levels in first 5 postoperative days as an early predictive tool for long-term remission of patients with acromegaly following transsphenoidal pituitary surgery (TSS). PATIENTS AND METHODS: Seventy-five consecutive acromegaly patients with at least three postoperative OGTT values at 3, 6, and 12 months of follow-up were included in the study. GH levels were measured just before surgery, in the immediate postoperative period, at 6 h and on day 1 to day 5 after surgery. Remission was defined as normal age-specific insulin-like growth factor-1 and either basal fasting GH <1 ng/ml or a nadir GH following OGTT <0 .4 ng/ml at 3 months of surgery. RESULTS: Of the 75 patients with acromegaly who underwent TSS, long-term remission was achieved in 42 (56%) patients. GH values ≤1.55 ng/ml at 6 h of surgery showed the highest predictive power for long-term remission, with a sensitivity of 81.2% and a specificity of 83.3%. The duration of disease and tumor volume had no effect on the 6 h GH value-related prediction of cure. CONCLUSION: Early postoperative GH values may be used to predict long-term cure. A value of ≤1.5 ng/ml at 6 h following surgery may predict long-term cure in two-thirds of the patients with acromegaly who undergo TSS.

Visual outcome in 2000 eyes following microscopic transsphenoidal surgery for pituitary adenomas: Protracted blindness should not be a deterrent.

OBJECTIVE: To study the visual outcome after surgery for pituitary adenomas with visual deficits. MATERIALS AND METHODS: All patients with pituitary adenoma, who presented from 2003-2014 in a tertiary care institute, were included in the study. Surgical outcome was measured in terms of difference in visual acuity, visual fields and optic fundus parameters documented before surgery, immediate post-operatively and at the third, and twelfth months following surgery. RESULTS: At the initial presentation, visual involvement was seen in 87.2% patients. One year after surgery, 93.2% patients having abnormal vision had improvement in visual acuity and visual fields; whereas visual parameters were static in 5.2%. Visual deterioration occurred only in 1.3% patients. Moreover, five-percent of those who did not even have perception of light at presentation experienced significant improvement in vision after surgery. The shorter the duration of visual symptoms, the more was the percentage of patients having faster recovery in the early postoperative period. CONCLUSION: Post-operative visual outcome was directly proportional to the pre-operative visual acuity. Though the visual outcome was good in the long run irrespective of the duration of symptoms, the speed of recovery was proportional to the duration of visual deficits. However, presence of long-standing visual symptoms should not deter us to subject the patient to surgery. Even patients who are completely visually impaired for years should be subjected to surgery as early as feasible.

Clinical profile and outcome of patients with acromegaly according to the 2014 consensus guidelines: Impact of a multi-disciplinary team.

AIM: The diagnosis and treatment of acromegaly, a rare and possibly curable disease, has undergone a paradigm shift in the past few decades. Our aim was to study the changing trends in clinical presentation, management and outcome of the disease in the last fifteen years. METHODOLOGY: 271 consecutive patients with acromegaly treated at the Departments of Endocrinology and Neurosurgery, PGIMER, Chandigarh, between 2000 and 2014, were included in the study. Clinical and hormonal profiles, comorbidities, treatment modalities, outcome and mortality data were evaluated. The cure rate was assessed according to the present consensus criteria. RESULTS: The gender distribution was equal with the mean age (±SD) of 37.1 ± 12.3 years at diagnosis. The average lag period to diagnosis was 4.7 ± 4.2 years. The most common presenting manifestations were acral enlargement and headache followed by visual deficits. The overall mortality rate was 5%, with the perioperative mortality being 1.5%. The most prevalent comorbidities in our series were hypertension (17.7%), diabetes mellitus (16.2%), arthropathy (11.8%) and obstructive sleep apnea (10.3%). Overall, 2 patients in our series suffered from extra-pituitary neoplasms and 12 patients had apoplexy as the presenting manifestation. As per the present consensus criteria, cure rate in our series was 28.5%. The cure rate was only 7.9% when many surgeons were operating. It increased to 25.5% when surgeries were being performed by one surgeon exclusively; and, when a sub-specialty clinic exclusively for pituitary diseases was set up, the cure rates improved upto 56%. CONCLUSION: Acromegaly has wide-ranging manifestations from acral enlargement to altered sensorium; incidental diagnosis was not prevalent in our series. Majority of the cases were due to the presence of a pituitary macroadenoma. Better cure rate can be achieved only when a dedicated group of multi-disciplinary team is involved.

Extra-Pituitary Birth Defects May Predict Diagnosis of Congenital Hypopituitarism in a Short Child.

BACKGROUND: Extra-pituitary birth defect (EPBD) in children with congenital hypopituitarism is largely unknown. OBJECTIVE: The study aims to evaluate the incidence and pattern of EPBD in children with congenital hypopituitarism and to evaluate whether it can serve as a clue to diagnose this condition. PATIENTS AND METHODS: Retrospective analysis of hospital record of patients of short stature due to various etiology from which patients with congenital hypopituitarism with age ≥18 years were recruited for the analysis. Clinical, hormonal, radiological and ocular electrophysiological studies were done in all patients and all EPBD were noted. RESULTS: Twenty seven patients (79%) had multiple pituitary hormone deficiency (MPHD) of which growth hormone was universal followed by gonadotropin (62%), TSH (59%), ACTH (44%) and prolactin (12%). Nineteen patients (56%) had multiple EPBD in various combinations. Twenty three ocular abnormalities were present in 12 patients (35%). Nine patients (26%) had other associated EPBD along with ocular abnormalities while 3 had ocular abnormalities without any other associated birth defect. Skeletal defects were present in 10 patients (29.5%). On the contrary, 5 patients in the EPBD group had total 15 visual defects. The most common abnormality of the visual system were abnormal visual evoke response (VER, 18%), followed by strabismus (15%), visual acuity (VA, 12%), electroretinogram (ERG) and electrooculogram (EOG) 8% each and visual field defect 6%. There was a trend towards early age at presentation with EPBD. CONCLUSIONS: Presence of EPBD in a short child is a sensitive marker to diagnose congenital hypopituitarism. Subtle abnormalities of visual pathway without absent septum pellucidum or midline brain defects were common.

Microsurgical anatomy of the anterior cerebral artery in Indian cadavers.

BACKGROUND: The microanatomy features of cerebral arteries may be variable and may be different in different ethnic groups. AIM: To study the anterior cerebral artery (ACA) anatomy in North-West Indian cadavers. MATERIALS AND METHODS: Microanatomy features of the ACA were studied in 15 formalin fixed human cadaveric brains under microscope. The outer diameter, length, and number of perforating branches with respective anomalies were measured for each of the following vessels: ACA (proximal A1 segment to distal A2 segment), anterior communicating artery (ACoA), Recurrent artery of Heubner (RAH), and callosomarginal artery and photographed for documentation. RESULTS: The mean length and external diameter of right and left A1 segment was 12.09 mm and 12.0 mm and 2.32 mm and 2.36 mm respectively. Narrowing, duplication, and median ACA were seen in 6.6%, 3.3% and 6.6% of the vessels respectively. Complex ACoA type was seen in 40% cadavers. RAH originated at an average point of 0.2 mm distal to ACoA, but in one cadaver it arose 5 mm proximal to ACoA. Double RAH was found in 26.6%. The course of RAH in relation to A1 was superiorly in 60%, in anteriorly 30% and posteriorly in 10% of cadavers. The orbitofrontal artery (OFA) and frontopolar artery (FPA) arose from A2 in 83.3% to 40% respectively. The mean distance of OFA and FPA from ACoA was 4.17 mm and 8.5 mm respectively. After giving rise to central, callosal and cortical branches, pericallosal artery terminated near the splenium of the corpus callosum or on the precuneus as the inferomedial parietal artery. CONCLUSION: Knowledge of the microvascular anatomy is indispensable and it is mandatory to be aware of the possible variations in the anomalies to minimize morbidity.

Dysregulated Genes and Signaling Pathways in the Formation and Rupture of Intracranial Aneurysm.

Intracranial aneurysm (IA) has the potential to rupture. Despite scientific advances, we are still not in a position to screen patients for IA and identify those at risk of rupture. It is critical to comprehend the molecular basis of disease to facilitate the development of novel diagnostic strategies. We used transcriptomics to identify the dysregulated genes and understand their role in the disease biology. In particular, RNA-Seq was performed in tissue samples of controls, unruptured IA, and ruptured IA. Dysregulated genes (DGs) were identified and analyzed to understand the functional aspects of molecules. Subsequently, candidate genes were validated at both transcript and protein level. There were 314 DGs in patients with unruptured IA when compared to control samples. Out of these, SPARC and OSM were validated as candidate molecules in unruptured IA. PI3K-AKT signaling pathway was found to be an important pathway for the formation of IA. Similarly, 301 DGs were identified in the samples of ruptured IA when compared with unruptured IAs. CTSL was found to be a key candidate molecule which along with Hippo signaling pathway may be involved in the rupture of IA. We conclude that activation of PI3K-AKT signaling pathway by OSM along with up-regulation of SPARC is important for the formation of IA. Further, regulation of Hippo pathway through PI3K-AKT signaling results in the down-regulation of YAP1 gene. This along with up-regulation of CTSL leads to further weakening of aneurysm wall and its subsequent rupture.

Colonic neoplasia in acromegaly: increased proliferation or deceased apoptosis?

Patients with acromegaly have higher prevalence of colorectal neoplasms. The pathogenetic mechanism is still unclear and may be related to sustained increase in serum GH-IGF1. We aimed to evaluate the proliferative and apoptotic markers in samples of colonic mucosa obtained during screening colonoscopic biopsy from patients with acromegaly and study their relationship to serum IGF-1 and GH levels. The study subjects included 32 patients with acromegaly (4 female), 10 healthy controls (irritable bowel syndrome) and 10 positive controls (non-acromegalic colonic adenocarcinoma). Patients with acromegaly were divided into two groups, active disease (AD) and disease in remission (AR). Two biopsies each were obtained during colonoscopy from the right colon, transverse colon and rectosigmoid region. All the polyps were biopsied and subjected to histopathological examination. Immunohistochemistry for proliferation marker (Ki-67) and apoptotic markers (caspase-3 and TdT-Mediated dUTP Nick-End Labeling (TUNEL) was carried out in the histopathological samples. Indices of proliferation were significantly different in patients with acromegaly as compared to healthy controls. The mean Ki-67 positivity was 45.1 ± 17.7% in AD and 45.6 ± 23.1% in AR, as compared to 10 ± 5% in healthy controls. While none of the healthy controls had Ki-67 positivity beyond the lower third of crypts, among patients with acromegaly 12/32 (37.5%) had mid-third positivity (P = 0.000) and 15/32 (46.8%) had full length of crypt positively (P = 0.00). Immunostaining for caspase-3 was negative in patients with acromegaly and healthy controls. TUNEL was strongly positive in patients with colonic adenocarcinoma but not in healthy controls and patients with acromegaly. IGF-1 levels were higher in those with Ki-67 positivity in the superficial mucosa. Patients with acromegaly have increased proliferation of colonic epithelial cells. Elevated levels of serum IGF1 are associated with increase proliferation in the superficial crypt cells.

Acrogigantism and facial asymmetry: McCune-Albright syndrome.

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monoostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies. Association of MAS with GH excess is rare, and in most of the instances somatotropinoma has not been documented. Treatment of patients of MAS with acromegaly is difficult because of thickened calvarium and dysplastic skull bone. We report a 17-year-old girl, who presented with cranio-facial fibrous dysplasia, café-au-lait macules and also had acromegaly due to pituitary macroadenoma, and treated with gamma knife radiosurgery.

Intracerebral infarcts following clipping of intracranial aneurysms: incidence, clinical correlation and outcome.

Subarachnoid hemorrhage (SAH) is a significant health care problem. One of the major determinants of outcome following surgery of intracranial aneurysms is development of intracranial infarcts. All patients underwent clipping for aneurysms in one year in the department of neurosurgery, PGIMER, Chandigarh were studied. Data regarding age, sex, date of ictus, date of admission, any co-morbidity, clinical grades at presentation, CT findings, infarcts, intraoperative rupture, and clinical status in the postoperative period were recorded. Outcome at discharge was assessed by Glasgow outcome scale (GOS). First, 174 patients were included in the study. Radiological cerebral infarctions occurred in 69 patients (39%). The most frequent location of infarct was deep perforator infarct followed by ACA territory infarct. 69.58% of patients developed infarct on the same side of aneurysm and 20.28% of patients developed infarct on opposite side, whereas 11% developed bilateral infarcts. Infarcts that occur early after surgery may be related to surgical factors whereas the late infarcts were probably as results of delayed ischemic deficits. Anatomical distribution of infarcts also showed two different patterns, infarcts limited to one vascular territory (more commonly seen in early onset infarcts) or multiple, cortical, bilateral infarcts (more commonly seen in late onset infarct). Patients with poor H&H grade, higher Fisher's grade, intraoperative rupture and prolonged temporary clipping had more chances of developing an intracranial infarct.

Glucose-dependent Insulinotropic Polypeptide (GIP) Resistance and ß-cell Dysfunction Contribute to Hyperglycaemia in Acromegaly.

Impaired insulin sensitivity (IS) and ß-cell dysfunction result in hyperglycaemia in patients of acromegaly. However, alterations in incretins and their impact on glucose-insulin homeostasis in these patients still remain elusive. Twenty patients of active acromegaly (10 each, with and without diabetes) underwent hyperinsulinemic euglycaemic clamp and mixed meal test, before and after surgery, to measure indices of IS, ß-cell function, GIP, GLP-1 and glucagon response. Immunohistochemistry (IHC) for GIP and GLP-1 was also done on intestinal biopsies of all acromegalics and healthy controls. Patients of acromegaly, irrespective of presence or absence of hyperglycaemia, had similar degree of insulin resistance, however patients with diabetes exhibited hyperglucagonemia, and compromised ß-cell function despite significantly higher GIP levels. After surgery, indices of IS improved, GIP and glucagon levels decreased significantly in both the groups, while there was no significant change in indices of ß-cell function in those with hyperglycaemia. IHC positivity for GIP, but not GLP-1, staining cells in duodenum and colon was significantly lower in acromegalics with diabetes as compared to healthy controls possibly because of high K-cell turnover. Chronic GH excess induces an equipoise insulin resistance in patients of acromegaly irrespective of their glycaemic status. Dysglycaemia in these patients is an outcome of ß-cell dysfunction consequent to GIP resistance and hyperglucagonemia.

Continuation of poor surgical outcome after elderly brain injury.

BACKGROUND: In spite of the decline in mortality among trauma patients, with advanced trauma care, the outcome for elderly patients remains poor. Both operative and nonoperative outcome for elderly patients after head trauma has resisted improvement. METHODS: Forty-five consecutive patients 70 years or older were included in the study. All these patients were admitted from January 2000 to June 2005. Road-traffic accidents caused most of the head injuries. RESULTS: Most of the patients (n = 33) belonged to severe head injury category. Contusions were the commonest CT scan finding (n = 27), for which surgery was indicated. Unexplained clinical deterioration, in spite of timely surgery and satisfactory postoperative CT scans, in a significant number of patients (n = 29) was noteworthy. Over the same period, the comparative group of younger patients (20-40 years, n = 1026) was also analyzed. CONCLUSION: Elderly patients experienced higher mortality and poorer functional outcome. The natural history of traumatized brain among elderly patients remains unchanged till the present times. The nihilistic scenario asks for reevaluation of interventions, relook into the neurobiology of aging brain, and aggressive research for remedial measures for such patients, especially among severe head injury group.

Invasive intracranial aspergillosis: the management dilemmas.

BACKGROUND: Invasive intracranial aspergillosis remains a disease with high morbidity and mortality. The rapid increase in the incidence of this disease led us to review the literature and formulate a treatment protocol for such patients. METHODS: An analysis of 46 patients with invasive intracranial aspergillosis is presented and the subgroups of extradural and intradural variety are evaluated with different treatment strategies. RESULTS: Patients with extradural form of disease had 100% survival, whereas antifungal chemotherapy preloading tends to provide a better outcome. CONCLUSIONS: Extradural aspergillosis does not need chemotherapy preloading, whereas intradural variant may have improved survival chances after preloading. Liposomal formulation has an advantage of shortened time duration for preloading, because the daily administration dose is 6 times higher than conventional preparation.

Contrasting behavior of endolymphatic sac tumors: a report of 2 cases and literature review.

BACKGROUND: Endolymphatic sac is an established source of low-grade neoplasms, posing a difficult problem in local tumor control. CASE DESCRIPTION: Two cases of endolymphatic sac tumors are described in detail with regard to their clinical presentation and radiological findings. Both cases had diametrically opposite clinical outcomes in spite of repeated surgical interventions. A case with severe paraneoplastic syndrome and death after "cancer cachexia" is described. CONCLUSIONS: Although endolymphatic tumors are known for their low-grade aggressiveness, in occasional cases, they may behave in a highly malignant behavior. The present communication highlights the contrast in clinical outcome and makes the clinician cautious of this special subgroup of tumors with a highly aggressive nature.