In Neurofibromatosis type 1, cerebral Unidentified Bright Objects are a well-known benign entity that has been extensively reported in the literature. In our case series, we wish to focus on a further possible location of such lesions, the spinal cord, which we have defined as medullary Unidentified Bright Objects. These have been, to our knowledge, scarcely described in previous works.We report the cases of 7 patients with medullary Unidentified Bright Objects in Neurofibromatosis type 1 that we have followed for up to 9 years in our Regional Referral Center for Neurofibromatosis. In all of our patients, these lesions were completely asymptomatic and reported on Magnetic Resonance exams the patients underwent for other clinical indications.The aim of our work is to increase awareness of the possibility of medullary Unidentified Bright Objects in Neurofibromatosis type 1 patients, which can simulate neoplastic lesions, suggesting a more conservative approach in these cases. Neurofibromatosis type 1 (NF1) is a rare  autosomal dominant genetic disorder . Spinal An 8-year old child with two older brothers presented with global developmental delays, genu and cubitus valgum, and bilateral clubfoot with a secondary gait disturbance. He was diagnosed with NF1, presenting with more than six café-au-lait spots (> 5 mm), inguinal and axillary freckling, one large hairy patch on the hand and a vascular malformation on the right leg. The neurological exam was negative. He underwent an X-ray exam of the spine, because of a mild scoliosis, showing scalloping of the posterior wall of T6-T9 vertebral bodies for which we performed an MR exam of the spine. The MR scan showed three intramedullary hyperintense lesions on T2WI localized in the cervical and thoracic tracts. The largest of these lesions was localized in the cervical tract (C1-C6) and presented a tumor-like appearance and was initially diagnosed as a low-grade glial neoplasm. Since the patient didn’t present any new symptoms at the time of the exam, a wait-and-see approach was chosen. Three months later, a follow-up MR exam of the spine was performed, showing partial spontaneous regression of the cervical spine lesion and a stability of the two other T2WI-hyperintense spinal lesions. On the subsequent MR follow-up exams done annually for three years and then biannually for 6 more years, the cervical lesion showed further spontaneous regression and then was unchanged through the end of the studies  was evaluated. He was 14 years old at the time of the first exam in our institution. He presented with cutaneous neurofibromas, macrocephaly, and short stature. In addition, a bilateral optic nerve pallor was noted for which he underwent a brain MR, showing multiple UBOs, non-enhancing bilateral optic gliomas and a pilocytic astrocytoma of the left thalamus. The exam also showed a small T2WI-hyperintense spinal lesion at the level of C1. The subsequent spinal MR examination confirmed the isolated cervical tumor-like lesion on the right side of the cord which showed no enhancement after gadolinium injection and with well-defined margins Fig. . This leAn 8-year-old male with no family history of NF1 was evaluated and found to have normal psychomotor development and growth. He presented typical features of NF1 and mild back pain for which a spinal MR exam was performed. It showed four small tumor-like lesions of the spinal cord which were moderately hyperintense on T2WI. These lesions were barely visible in sagittal scans, but axial images better defined the presence and characteristics of the aforementioned lesions. None of these lesions showed enhancement on post-contrast T1WI and they showed no evolution in the follow up exam one year later.This 11-year-old male patient had no family history for NF1 but had global development delays and mild congenital hypotonia. At the age of two months he was diagnosed with NF1, presenting with typical cafè-au-lait spots, macrocephaly and freckles. A brain MR examination, performed at the age of 10, showed a pre-rolandic pilocytic astrocytoma for which the patient underwent surgery.A spinal MR exam was performed because of the onset of a painful dorsal scoliosis. It showed four tumor-like lesions of the spinal cord and of the conus medullaris which were hyperintense on T2WI and showed no enhancement on post-contrast T1WI. A follow up exam after 6 months showed no variations. The neurological examination did not reveal any focal deficit.A 7 year-old male patient was diagnosed at the age of 18 months with NF1, presenting typical cafè-au-lait spots and freckles, without a family history for this pathology. Neuropsycological development and growth were normal. He underwent the first MR exam at our institution as he presented persistent headache and a reduction of the visual acuity, showing a low-grade astrocytoma localized in the tegmentum of the midbrain causing a triventricular hydrocephalus. An incidental lesion of the cervical spine was detected for which a spine MR was performed. Three tumor-like, T2WI-hyperintense lesions were detected, none of which showed enhancement on T1WI after gadolinium injection. All of these were stable at follow-up MR exams after 12 months.In previous studies, brain UBOs have been variously defined: hamartomas, altered myelination, or heterotopias –10. In tMore recent studies have confirmed this finding by using non-invasive MR techniques which showed a modification of the microstructural compartmentalization with an increase of extracellular-like intracellular water, an indication of intramyelinic edema, in UBOs confirming the previously mentioned histological findings .In NF1, intramedullary lesions are more frequently low-grade astrocytomas (15% of patients) . Rare caTo our knowledge only one previous report of a medullary lesion in a patient with NF1, analogous to those herein documented in our patients, has been published as of this date. The lesion, in line with the knowledge of the time, was defined as a hamartomatous spinal cord lesion .We report seven additional cases presenting such lesions localized in different segments of the spine, mainly in the cervical segment, describing their MR features and long-term clinical and radiological follow up. In our experience, patients more frequently had multiple asymptomatic medullary lesions, with a tumor-like appearance, always hyperintense in T2WI, hypo, iso or slightly hyperintense in T1WI, and were never enhancing in post-contrast T1WI. At follow up exams they were stable, except for one patient in which a spontaneous partial regression was documented . The aim of our work is to increase awareness of the possibility of mUBOs in NF1 patients which can simulate neoplastic lesions, suggesting a more conservative approach.


Current guidelines are vague for the management of soft tissue sarcomas, specifically malignant peripheral nerve sheath tumors (MPNST), regarding staging the disease with the use of routine abdominal imaging. The most recent guidelines from the National Comprehensive Cancer Network (NCCN) recommends to “consider” abdominal/pelvic CT imaging for certain sub groups of sarcomas , but provide no guidance on other sarcoma subtypes regardless of tumor size. We report a case of a very large large MPNST  in a 40 year-old-female with neurofibromatosis type 1 who was incidentally found to have adrenal metastasis. The majority of soft-tissue sarcomas occur in the limb or limb girdle or within the abdomen, with 40% being found in the lower extremities. These sarcomas are a histologically heterogeneous group of tumors (over 50 tumor subtypes have been identified) with a predilection for hematogenous spread. Distant metastatic disease is found in approximately 20–30% of patients3, with pulmonary lesions accounting for 75% of these cases5. Because of the relatively low incidence of extra-pulmonary metastasis, the current guidelines from the National Comprehensive Cancer Network (NCCN) recommends to only “consider” abdominal/pelvic CT imaging for certain subgroups of sarcomas 6. A similar recommendation is made by the European Society of Medical Oncology (ESMO)/European Sarcoma Network Working Group7. No specific recommendation is made for any additional sarcoma subtype regardless of size of the tumor, despite this being a known prognostic factor.Soft tissue sarcomas of the extremity are a rare disease process, comprising less than 1% of all malignancies10. We report a case of a malignant peripheral nerve sheath tumor of the thigh, without evidence of concomitant pulmonary metastases, which was found to have metastasized to the adrenal gland. Thus, we pose the question of should the staging work up for large sarcomas be expanded to include abdominal imaging, even if the CT chest is unrevealing?Malignant peripheral nerve sheath tumors (MPNST) are highly malignant sarcomas which originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells, or fibroblasts. MPNSTs compromise 5–10% of all soft tissue sarcomas with 22–50% of the tumors associated with a diagnosis of Neurofibromatosis-1 (NF1)A 40 year old woman withNF1 presented for a hemorrhagic right thigh mass which had been enlarging over the past 3 months. The mass had previously been stable in size for a few years and was thought to be consistent with a neurofibroma. On presentation her exam was notable for a very large soft tissue tumor of the right posterior upper leg (>15cm). The distal portion of the lesion revealed skin breakdown and active bleeding with exposed muscle and tumor. Laboratory analyses revealed a white blood cell count of 23.0k/µL and hemoglobin of 10.7 g/dL with an unremarkable basic metabolic panel. Computerized tomogram (CT) of the extremities revealed a large heterogeneously enhancing mass within the posterior compartment of the right thigh, measuring 13.4 × 13.4 × 24.8 cm. In addition, an incidental 2 × 2 cm left adrenal gland mass was noted. Of note, a CT scan of the thorax, completed for staging purposes, did not reveal any suspicious pulmonary nodules or masses. After an initial excisional biopsy of the thigh mass, she underwent a radical resection of the right thigh mass. Pathology confirmed a high grade spindle cell sarcoma, negative for S100 protein and SOX10 (variable expression in MPNST), Desmin (rhabdomyosarcoma differentiation), CD31 and AE1:AE3 , HMB45, MelanA (epitheloid MPNST). Given the clinical history of NF-1 and the lack of immunoreactivity for all performed markers for this tumor was most compatible with an undifferentiated MPNST  AMRI of 6, a screening CT of the abdomen/pelvis (A/P) would not be indicated and thus could have potentially missed the presence of metastatic disease.The above case describes a patient with a MPNST of the thigh with pathologically confirmed metastases to the adrenal gland, yet without evidence of pulmonary metastases. The adrenal mass was found incidentally upon imaging of the thigh mass, but based on current NCCN guidelineset al. evaluated 124 adult patients with sarcoma who underwent CT chest(C)/A/P imaging at their institution for staging and surveillance. Twenty (16%) of the patients had evidence of A/P metastasis, 7 on the initial scan and 13 on the surveillance11. Of note, six of the 20 patients (5% of the cohort) were found to have isolated A/P metastases without the development of pulmonary metastases during the study period. MPNST, specifically, made up 6% of the sarcomas evaluated and while no A/P metastases were found on screening, 2 patients had evidence on surveillance scans. Based on the finding that a wide variety of sarcoma subtypes were found to have extra-pulmonary disease, the authors conclude that A/P imaging should be included in the evaluation of all sarcoma subtypes. In contrast, Thompsonet al. reviewed 140 patients of all ages who had a diagnosis of a malignant neoplasm of the upper or lower extremity and underwent screening and/or surveillance with a CT C/A/P12. Of those patients, 14 (10%) had evidence of abdominal/pelvic metastasis, with only 4 (2.9%) with evidence of isolated A/P disease. Additionally, of the 10 patients who developed metastases to both the chest and abdomen/pelvis, none developed evidence of disease in the abdomen/pelvis prior to the chest. Of note, though, there were only 2 MPNSTs in the entire cohort and neither one had evidence of abdominopelvic disease on imaging. Based on their results, the authors offer up the contrary opinion from King and do not support routine abdomen/pelvis imaging.To the best of our knowledge there have only been 2 case series that comment on the indication for A/P imaging in sarcoma patient and they reached contradictory conclusions.. In the first case series, King,13. Currently, though, guidelines only recommend CT of the chest for all patients and to consider CT A/P in patients with myxoid/round cell liposarcoma, epithelial sarcoma, angiosarcoma and leiomyosarcoma. Based on our case and the literature, we also would suggest adding screening CT A/P for large (>5 cm), deep tumors of any histology.Our case adds a patient to the literature with a peripheral MPNST who was found to have an isolated adrenal metastasis without evidence of concomitant pulmonary disease. One striking feature of this case is the very large size of the primary tumor(>20 cm). Factors known to be associated with the development of metastases are tumor grade, tumor size, tumor depth, and certain histopathologies. Specifically, tumors > 5cm have been found to be associated with an increased risk of metastatic recurrenceSoft tissue sarcomas are a heterogeneous group of tumors with a variety of prognoses. Deciding on a unified set of guidelines will be challenging, but given the clinical significance of finding metastatic disease, adding additional parameters (size and depth) to a more complete screening process would seem prudent.Written informed consent was obtained by the patient for publication of their clinical details. There are no potentially identifying images included in this paper. Authors have addressed the majority of the concerns except few. Eg. No correction of epithelial sarcoma to epithelioid sarcoma, expanding on the physical and gross examination for thigh mass and adrenal metastasis. There is some problem with spacing between the words as well, eg- "AMRI", "withNF1". Please consider addressing these issues.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. With interest, I read the revised version of manuscript. Authors have addressed almost all the comments. The manuscript needs minor English polishing prior to final publication  and many others throughout the manuscript.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The case report describes an interesting finding but it needs major revision. Some of the changes suggested as below-It describes an NF type-1 patient with MPNST with metastasis to the adrenal gland. The patients with NF type-1 have increased risk for pheochromocytoma. Likely the patient had undiagnosed pheochromocytoma (was the patient hypertensive?) and the patient developed metastasis to pheochromocytoma. So it describes more of cancer-to-cancer metastasis than collision tumor. Add more references with respect to cancer-to-cancer metastasis. Collision tumor is defined as two distinct tumors developing in juxtaposition to one another without areas of intermingling. In the current case, as in Figure 2, there is an intermingling of both components- so again it is not a collision tumor. The title needs to be changed and make it apt.Spelling errors like prognoses (mentioned in conclusion). There is nothing called epithelial sarcoma, the correct terminology is epithelioid sarcoma. MPNST stands for malignant peripheral nerve sheath tumor so no need for peripheral MPNST (mentioned in the first line of the last paragraph of discussion). Please check for grammatical errors as well.  Abstract- talks about guidelines for staging peripheral nerve sheath tumors, which sounds like it was a benign tumor. So use MPNST instead of just peripheral nerve sheath tumor. Abstract and discussion mention isolated adrenal metastasis, which is not true; she had both bone and adrenal metastasis. There is mention that- "She underwent primary and adrenal metastasis resection." consider revising it to primary tumor resection and adrenalectomy for metastasis. Expand on physical exam of thigh mass- size, tenderness, consistency etc. Elaborate on findings of MRI with details of imaging findings of adrenal metastasis.  Expand on gross findings of thigh mass and adrenal nodules- eg. size, color, infiltrative or well-circumscribed etc. Did both the adrenal nodules have similar histological findings? Were any immunostains done to confirm pheochromocytoma component?  Ref 9 talks about patients with malignant neoplasm of the upper or lower extremity. There is no mention of that those were sarcomas. Check for that.  Expand on abstract- add more about the significance of the findings of the case; expand on introduction and discussion- add more details for sarcoma, development of MPNST in association to NF-1, adrenal metastasis, management guidelines, more references for the recommendation for screening CT A/P for sarcomas. In the discussion, authors have suggested adding screening CT A/P for large (>5cm), deep tumors of any histology- expand more on this by adding more references.  Add limitations of the study, the major limitation being small sample size.  I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. We thank you for the review and the excellent suggestions. Our response is included belowt describes an NF type-1 patient with MPNST with metastasis to the adrenal gland. The patients with NF type-1 have increased risk for pheochromocytoma. Likely the patient had undiagnosed pheochromocytoma (was the patient hypertensive?) and the patient developed metastasis to pheochromocytoma. So it describes more of cancer-to-cancer metastasis than collision tumor. Add more references with respect to cancer-to-cancer metastasis. Collision tumor is defined as two distinct tumors developing in juxtaposition to one another without areas of intermingling. In the current case, as in Figure 2, there is an intermingling of both components- so again it is not a collision tumor. The title needs to be changed and make it apt.Response  - term collision tumor is removed.Spelling errors like prognoses (mentioned in conclusion). There is nothing called epithelial sarcoma, the correct terminology is epithelioid sarcoma. MPNST stands for malignant peripheral nerve sheath tumor so no need for peripheral MPNST (mentioned in the first line of the last paragraph of discussion). Please check for grammatical errors as well.   Response - correctedAbstract- talks about guidelines for staging peripheral nerve sheath tumors, which sounds like it was a benign tumor. So use MPNST instead of just peripheral nerve sheath tumor. Abstract and discussion mention isolated adrenal metastasis, which is not true; she had both bone and adrenal metastasis. There is mention that- "She underwent primary and adrenal metastasis resection." consider revising it to primary tumor resection and adrenalectomy for metastasis. Response - correctedExpand on physical exam of thigh mass- size, tenderness, consistency etc. Elaborate on findings of MRI with details of imaging findings of adrenal metastasis.   Response- correctedExpand on gross findings of thigh mass and adrenal nodules- eg. size, color, infiltrative or well-circumscribed etc. Did both the adrenal nodules have similar histological findings? Were any immunostains done to confirm pheochromocytoma component?  Response- immunostains are included in the findings describeddd limitations of the study, the major limitation being small sample size.Response- We intend to publish this as a case report With interest, I read the article Shaines and Arora. Overall, the article reads very well, and should deserve acceptance following some minor/major changes to improve its quality and scientific soundness. 1. Title: — You may want remove the phrase "case report". — Also, please change to "an adrenal metastasis". 2. Abstract. — Please expand the abstract. — You may want to include a couple of sentences about definition of soft tissue sarcomas (STSs), incidence, biological behavior  and percentage of MPNSTs. — You may also want to precisely mention the names of guidelines and dates. — All these recommended sentences above will highlight the importance of your case report. — The abstract says the STS of thigh was resected although this is not mentioned in the "case report" section.  — Lastly, check if journal mandates a STRUCTURED format for the abstract section.  3. Introduction: — It is recommended to add sentences that shed light on the most common sites of STSs and the percentage of MPNSTs among all STSs. — Mention the dates of guidelines. 4. Case Report: — Add details about physical examination. — Add details about initial laboratory findings. — Add CT scans of the thigh mass (to highlight its large size) and adrenal mass (to highlight the collision tumor). — You add the MRI picture of the adrenal mass, too (or the CT scan). — Please do mention of the patient received surgery for the STS of the thigh as it is not clearly mentioned in this section. 5.  Discussion: — Since the two series presented in manuscript had contradictory conclusions, it is recommended (if available) to add additional data from one to two more series.  — For reference (9), please add specific details about MPNSTs (if available). — Briefly mention how (why) MPNST are different from the myxoid liposarcoma, epithelial sarcoma, angiosarcoma etc in terms of the recommendation for CT A/P scanning. — please provide citations for the following sentence "Factors known to be associated with the development of metastases are tumor grade, tumor size, tumor depth, and certain histopathologies". 5. "Based on our case and the literature, we also would suggest adding screening CT A/P for large (>5cm), deep tumors of any histology". How depth was assessed in your case report. 6. Conclusion: — Modify the conclusion so it will reflect the specific histopathological variant of MPNST . 7. References: — Good 8. English: — Minor English polishing.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. We thank the reviewer for their excellent suggestions. Our response is included belowReviewers Response /1Abstract. — Please expand the abstract. — You may want to include a couple of sentences about definition of soft tissue sarcomas (STSs), incidence, biological behavior  and percentage of MPNSTs. — You may also want to precisely mention the names of guidelines and dates. — All these recommended sentences above will highlight the importance of your case report. — The abstract says the STS of thigh was resected although this is not mentioned in the "case report" section.  — Lastly, check if journal mandates a STRUCTURED format for the abstract section.Response- We  included much of the above in an updated version of the introductionIntroduction: — It is recommended to add sentences that shed light on the most common sites of STSs and the percentage of MPNSTs among all STSs. — Mention the dates of guidelines.Response -UpdatedCase Report: — Add details about physical examination. — Add details about initial laboratory findings. — Add CT scans of the thigh mass (to highlight its large size) and adrenal mass (to highlight the collision tumor). — You add the MRI picture of the adrenal mass, too (or the CT scan). — Please do mention of the patient received surgery for the STS of the thigh as it is not clearly mentioned in this section.Response- Updated much of this.here to see this.We have attached an image of the thigh mass with this response instead – please clickDiscussion: — Since the two series presented in manuscript had contradictory conclusions, it is recommended (if available) to add additional data from one to two more series.  — For reference (9), please add specific details about MPNSTs (if available). — Briefly mention how (why) MPNST are different from the myxoid liposarcoma, epithelial sarcoma, angiosarcoma etc in terms of the recommendation for CT A/P scanning. — please provide citations for the following sentence "Factors known to be associated with the development of metastases are tumor grade, tumor size, tumor depth, and certain histopathologies". 5. "Based on our case and the literature, we also would suggest adding screening CT A/P for large (>5cm), deep tumors of any histology". How depth was assessed in your case report.Response -Updated much of thisThese are the only 2 case series we could find that address this issue of A/P imagingwe removed “depth” from that last sentence as it really does appear that size if what mattersConclusion: — Modify the conclusion so it will reflect the specific histopathological variant of MPNST .Response- Wepurposely left this vague because this this was only one case of MPNST and there are so many variants of soft tissue sarcoma. I thought it would be more generalizable to state tumor size, in general, should be thought of as important when it comes to screening imaging since it is noted as a risk factor for all STS.


We present a case of a 52-year-old male who developed Cushing's Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Work-up revealed ACTH-dependent hypercortisolemia, which did not suppress appropriately after high-dose dexamethasone. Subsequent imaging of the chest, abdomen, and pelvis did not reveal other possible ectopic sources of ACTH secretion besides the ENB. His hospital course was complicated by severe hypokalemia and hyperglycemia before successful surgical resection of the tumor, the biopsy of which showed ENB. Postoperatively, his ACTH level dropped below the limit of detection. In the ensuing 4 months, he underwent adjuvant chemoradiation with carboplatin and docetaxel with good response and resolution of hypokalemia and hyperglycemia, with no sign of recurrence as of 30 months postoperatively. His endogenous cortisol production is rising but has not completely recovered. Esthesioneuroblastoma (ENB), or olfactory neuroblastoma, is a tumor of the nasal and paranasal sinuses, derived from the olfactory neuroepithelium , 2 and r3− of 44 mmol/L (21–29). Surgery was postponed, and the endocrinology service was consulted. It was noted that the patient had been experiencing several weeks of severe weakness, polyuria, and more than 20 pounds of weight loss before the scheduled operation. He was initially treated with oral and intravenous potassium chloride (KCl), but his serum potassium continued to be refractory to acute repletion. In addition, he had increased insulin requirement to control his serum glucose levels.A 52-year-old Caucasian male with a past medical history of hypertension presented to our hospital for planned resection of a large skull base mass of the right cribriform plate by the neurosurgery service. He had initially presented at an outside hospital with anosmia and right nasal airway obstruction. He was diagnosed with new onset diabetes mellitus. Computed tomography (CT) scan of the head and magnetic resonance imaging (MRI) of the face revealed a 7.5 × 4.1 × 3.4 cm mass of the right cribriform plate, extending intracranially into the right anterior cranial fossa and displacing the frontal lobe with no sellar involvement. Imaging at our center confirmed the findings . HoweverAs part of his hypokalemia work-up, he was found to have a significantly elevated random plasma cortisol of 1851 nmol/L and plasma ACTH of 152 pmol/L (1.5–11.2). 24-hour urine free cortisol was also grossly elevated at 32,027 nmol/day (<165). His renin and aldosterone levels were normal. His TSH was also normal. Thus, CS was suspected, and imaging was ordered to locate possible sources. CT scan of his chest, abdomen, and pelvis noted bilateral adrenal enlargement, but no distinct nodules and no apparent sources of ACTH secretion were found. This raised the possibility of ENB being the ectopic source. High-dose (8 mg) dexamethasone suppression testing was performed, with the next-day morning cortisol of 1895 nmol/L, which was not suppressed at all, and ACTH was still elevated at 75.7 pmol/L. He continued to be aggressively treated with KCl. Eplerenone, magnesium oxide, and ketoconazole were also added. On day 5, his potassium was stabilized in the low-normal range. He was discharged on day 6, with a plan for a definitive resection of the tumor in 3 weeks, after the Christmas and New Year holidays.However, the patient was readmitted 3 days later, for confusion, hypotension, and continued hypokalemia refractory to his oral potassium medications. He was once again aggressively potassium repleted, and surgery was performed a week later. A bifrontal craniotomy was performed by the neurosurgery team and bilateral maxillary antrostomy, ethmoidectomy, and sphenoidotomy were performed by otolaryngology along with resection of the tumor under endoscopic guidance. The mass was revealed to be a mixed-consistency lesion eroding through the anterior right planum extending through the dura. Intraoperative biopsy showed that the tumor contained lobular growth of small round cells with uniform hyperchromatic chromatin, inconspicuous nucleoli, and scant fibrillary cytoplasm, with rare mitotic figures present . The celThe postoperative course was uncomplicated, with decreasing daily potassium and insulin requirements and resolution of metabolic alkalosis . He no lThis patient was diagnosed with ectopic ACTH syndrome (EAS) based on the preoperative findings of high plasma ACTH concentration, random serum cortisol, and 24-hour urinary cortisol. Other ectopic sources were ruled out by CT scan. At this point, it was strongly suggested that the ENB  was the source of the ectopic ACTH . This waOur report demonstrates an unusual presentation of CS due to ectopic ACTH secretion from ENB and adds key data to the literature concerning this rare condition. There are fewer than 25 cases in the literature. We describe an EAS-ENB with comprehensive follow-up up to 30 months after resection, which is one of the longest follow-up periods among studies to date , 7. Our Our patient required multiple medication dosage adjustments in order to manage his hypokalemia, including oral and intravenous KCl and magnesium, as well as antimineralocorticoid such as eplerenone and steroidogenesis inhibitors such as ketoconazole. These were titrated to give him low-normal potassium levels and to relieve symptoms of muscle weakness and numbness. Other case reports have described usage of metyrapone, etomidate, mitotane, and mifepristone as steroidogenesis inhibitors . As descThe patient was placed on different corticosteroid (dexamethasone and later hydrocortisone) for central adrenal insufficiency the months after tumor resection. Baseline ACTH and cortisol levels remained to be low-normal in our patient. He failed all his ACTH stimulation tests so far (cortisol level <500 nmol/L 60 min after 0.25 mg ACTH stimulation) but has had improvement of stimulated cortisol. The patient does not have clinical symptoms of adrenal insufficiency such as weight loss, cardiovascular collapse, and hypoglycemia. The chronic massive production of ectopic ACTH from EAS has suppressed his endogenous long term cortisol production (over 30 months). Central adrenal insufficiency usually only requires glucocorticoid replacement, not mineralocorticoid, with hydrocortisone preferred as the most physiological option .In conclusion, we report a case of olfactory neuroblastoma with ectopic ACTH secretion that was treated with resection and adjuvant chemoradiation. Given the paucity of this diagnosis, little is known about how best to treat these patients and how best to screen for complications such as adrenal insufficiency and follow-up. Our case adds more data for better understanding of this disease.


Inadvertent spillage of gall stones is a rare yet important cause of delayed postcholecystectomy complications. Varied presentations and difficulty in diagnosis are the hallmarks, making it crucial to have a high index of suspicion to detect and intervene appropriately. Peritoneo‐cutaneous fistulae from the retained stone can be completely excised in toto. Question: A 54‐yr‐old male presented with persistent sero‐purulent discharge from sinus at right lateral port site nearly 8 months after laparoscopic cholecystectomy. He was of generally good health, with no fever or weight loss, good appetite, and bowel function. By contrast, cross‐sectional imaging with sinogram showed an 8‐cm long tract, with minimal collection but no obvious mass. The procedure performed involved coring out of entire tract with contained material. In view of the images provided, what may be the most likely diagnosis?Intra‐abdominal abscessSpilled gall stonesBiliary fistulaEntero‐ or Colo‐cutaneous fistulaAnswer: B) Spilled gall stones.Spillage of gall stones following conventional and single incision laparoscopic procedures is on the rise due to the increasing numbers of difficult cases performed, namely elderly age group, obese patient, acute inflammation, and adhesions. Literature reports an overall incidence of spilled stones as 5–40%, but causing morbidity in only about 0.08–0.3% of cases The patient underwent conventional laparoscopic cholecystectomy at a district level hospital 8 months prior to presentation. He complained of initial serous, followed by intermittent purulent discharge from the right midclavicular working port site. He was otherwise healthy, with no previous comorbid illness, or associated symptoms. Superficial probe ultrasound showed no parietal wall collections, with no obvious detectable intra‐abdominal pathology. Contrast enhanced computed tomography (CT) with sinogram subsequently showed evidence of an 8‐cm‐long tract extending from skin to the subhepatic region without evidence of any stone or mass at the end Figures  and 3. USpillage of gall stones can occur either during dissection or at specimen retrieval phase in cholecystectomy Intra‐abdominal abscess is the commonest reported complication, with a mean incidence of 0.1–2.9% in patients with spilled stones. This may present anytime between 10 days and 72 months post index surgery Management of fistulae such as the peritoneo‐cutaneous fistula in our case includes diagnosis via computed tomography or magnetic resonance cross‐sectional imaging and sinogram, to identify the length and course of tract, its extent and termination; and identify any associated anatomical disturbances. When associated with intra‐abdominal abscess, local exploration and drainage may be worthwhile, but definitive treatment is by complete tract excision along with the culprit stone, thorough lavage, and primary or delayed closure, depending on extent of purulent collection. Needless to say, prophylaxis still remains the forefront, with meticulous dissection, specimen retrieval, and final thorough inspection prior to closure.Option (A), intra‐abdominal abscess would cause systemic symptoms with abdominal pain. Its management would initially involve percutaneous drainage. Option (C), biliary fistula would usually present with icterus, general ill‐health, raised liver markers, and show dilated intrahepatic biliary radicles on imaging. Management would involve invasive or noninvasive cholangio‐pancreatography, which would further decide course of treatment. Option (D), entero‐ or colo‐cutaneous fistula is quite a morbid condition, with patient showing systemic symptoms, deranged nutritional status and sarcopenia. Hence, in this case, best answer is Option (B), spilled gall stones Figs , 2, 3.Peritoneo‐cutaneous fistula is a rare but important presentation of spilled gall stones following laparoscopic cholecystectomy. There is thus need to suspect this and manage accordingly, especially in cases presenting more than 6 months following index procedure. Wide excision of fistula tract along with culprit stone and along with thorough lavage is the treatment of choice.SK: Member of the primary treating surgical team. Preparation of manuscript, study design, and proof reading. Photographs taken and edited as per requirements. Literature review and discussions section.The author hereby declares no conflict of interests


The pediatric dental approach to the oral cavity of newborns requires special attention, as many aspects are unique and peculiar to this period of life. It is important that pediatricians and pediatric dentists be aware of the characteristics within normal newborn patterns and prepared to make a correct diagnosis of abnormalities at early stages. Congenital eruption cysts (ECs) are rarely observed in newborns, as at this stage of a child’s life, tooth eruption is unusual. This study reports a case of EC treated successfully by monitoring of the lesion, without any surgical procedure. In the 4th month, the lesion had completely regressed, and the deciduous central incisors had erupted without problems. The clinical and radiographic monitoring of ECs in newborns seems to be a satisfactory management procedure, similar to what is recommended for older children.How to cite this article: de Oliveira AJ, Silveira MLG, Duarte DA, Diniz MB. Eruption Cyst in the Neonate. Int J Clin Pediatr Dent 2018;11(1):58-60. A radiograph of the mandibular anterior occlusal revealed the superficial placement of the deciduous central incisors . The proFollow-up visits were scheduled after 15 days and monthly thereafter. After approximately 1 month, the lesion size had decreased, and the color changed from yellowish to normal gingiva color . By the 568 The origin of the EC is still debated. Certain authors attribute its origin to degenerative changes of the reduced enamel epithelium after the end of amelo-genesis or suggest that the cyst develops from the remnants of the dental lamina that coats the erupting teeth.5 Regardless of the precise etiology, the term EC is correctly used when the affected tooth is located in the soft tissues lining the alveolar bone during tooth eruption. When the tooth is surrounded by bone, the same lesion should be referred to as a dentigerous cyst, according to the classification adopted by the World Health Organization.349 In this case study, the radiographs showed normal deciduous central incisors with no evidence of bone pathology.Eruption cyst is a pathological condition found in the oral cavity during childhood, represented by swelling of the soft tissue located on the alveolar ridge, adjacent to the erupting tooth.7Clinically, the EC appears as a circumscribed, floating, often translucent volumetric increase in the alveolar impeller at the site of tooth eruption. When the cir-cumcoronal cystic cavity contains blood, the swelling appears purplish or dark blue and is called an eruption hematoma.4The clinical findings observed in this case are characterized by a fluctuating and compressible swelling suggestive of fluid retention, resembling a mucocele. However, secondary salivary glands are not found in the alveolar impeller, excluding this hypothetical diagnosis.10Although most reports of ECs are in the first decade of life, only a few studies have shown this type of lesion in newborns.3 reported a case of neonate presenting a lesion 2 × 2 cm in diameter, exophytic, soft, yellowish, and compressible in the anterior mandibular region. The diagnosis of EC was made by clinical, radio-graphic, and histopathological examination. At 4 months, the lesion had completely disappeared, and the deciduous central incisors had erupted without problems.Aleman Navas et al4 described a case of EC in a newborn, in which clinical follow-up was adopted. Forty-five days after the first consultation, the lesion had nearly disappeared. Complete regression was observed after 60 days, and 4 months after the initial examination, the central incisors had already erupted, showing small hypoplastic areas on the incisal edges.Ricci et al11 reported a case of a newborn presenting two symmetrical lesions in the anterior dome of the mandible alveolar ridge. Treatment was established, and periodic follow-up visits were scheduled. At 2 months, both incisors were completely erupted.Peters and Schock356 Radiographic examination in this case revealed normal deciduous central incisors, with no evidence of bone pathology.Although the EC cannot be detected on radiographic examination due to the lack of bone involvement, radiography is recommended to evaluate the morphology of the affected tooth and its surrounding bone.3 When poorly implemented, the natal or neonatal tooth is dangerous to the newborns, as it can loosen during breastfeeding and be aspirated; thus, extraction is indicated.31213 In the clinical case in question, the central incisors were palpable in the cyst; however, the decision to preserve them was made because they did not present hypermobility, did not interfere with feeding, and did not cause discomfort in the mother or the child.3Eruption cysts may be associated with natal or neonatal teeth and have been classified as mature natal or neonatal teeth when the tooth is almost or fully developed and has relatively good prognosis to be maintained or as immature natal or neonatal teeth when the tooth has incomplete or nonstandard structure, resulting in poor prognosis.34911 marsupialization,10 or surgical extraction of the involved tooth.3510 When these cysts prevent the eruption of a deciduous tooth, marsupi-alization may be considered. Extraction is indicated when the teeth present mobility or interfere with lactation.11 In this case of congenital EC, the treatment of choice was monitoring of the lesion, without any surgical intervention or tooth extraction.The treatment options for EC have been thus far no procedure and monitoring,In newborns, clinical and radiographic monitoring of the EC seems to be adequate, similar to the procedure recommended for older children.


Neurenteric cyst in the thoracic cavity may produce a myriad of clinical features. We report a 7-month-old girl who presented with significant bleeding per rectum. On imaging, a mediastinal cystic structure with air-fluid levels was evident with cervico-thoracic vertebral anomalies. The cyst was excised and histopathology showed intestinal mucosal lining with heterotopic pancreatic tissue confirming the diagnosis of neurenteric cyst. A 7-month-old female baby brought to the pediatric surgery outpatient department with intermittent bleeding per rectum since last 10 days. The baby was diagnosed to have a mediastinal cystic lesion on antenatal scan. Baby was delivered at term without any perinatal problems. During past 7 months, 3 episodes of lower respiratory tract infections occurred which were treated medically.On examination, child had mild tachypnea along with pallor. Laboratory tests showed anemia (7g/dl) with a normal total leukocyte and platelet count. Chest radiograph revealed multiple cervico-thoracic vertebral segmentation anomalies. Left side upper rib anomalies were also seen along with associated scoliosis with concavity to left side. A well defined radio-opacity was seen projecting over cardiac shadow extending into left hemithorax. Few small bowel loops were seen in right upper quadrant of abdomen . A contrast enhanced computed tomography (CECT) scan revealed a well-defined cystic lesion with mucosal enhancement in posterior mediastinum with air fluid level extending to left side . Esophagus could be traced upto mid thorax only. Stomach could not be clearly delineated among all bowel loops in upper abdomen as small bowel loops were also seen in Morrison’s pouch and retroperitoneum. SMA-SMV relationship could not be relied upon as pancreas was displaced anteriorly by the bowel loops. Azygos continuation of IVC was seen draining into SVC. Hepatic veins were seen to drain into right atrium via a short common channel. Heart and descending thoracic aorta were displaced anteriorly by the cystic lesion along with compression of left lung. Multiple vertebral fusion and segmentation anomalies were seen extending from C5 to T4 vertebrae, along with left upper rib abnormalities and associated scoliosis. No obvious intra-spinal extension of the lesion was seen .An upper gastrointestinal (GI) contrast study was done which showed normal location of stomach and no opacification of mediastinal lesion by contrast . Meanwhile, child had 3 episodes of malena during the preoperative hospital stay and intermittent hemodynamic instability with drop in hemoglobin which was managed with blood transfusions. After hemodynamic stabilization, left lateral thoracotomy was done. Intraoperatively, a large mediastinal cyst was seen communicating with a proximal jejunal loop via a thin tubular tract. Communication was present on the mesenteric side of jejunal loop. Cyst was resected completely along with a small part of jejunum followed by end to end jejunal anastomosis . Bowel malrotation was present, which was corrected. Histopathological examination of the cyst showed jejunal type of intestinal mucosal lining. Islands of heterotopic pancreatic tissue were seen in the wall of the communicating tract between jejunum and mediastinal cyst .The baby recovered well and was discharged on 10th postoperative day. On a follow up of 6 months after the surgery, the child is thriving well. Postoperatively, on retrospective examination of the upper GI contrast study we observed that an oblique view from this series showed a contrast outlined tract arising from small bowel loops and was extending slightly above the diaphragm .Mediastinal cysts contribute 15%–20% of all mediastinal masses and include bronchogenic cysts, esophageal duplication cysts, pericardial cysts, neurenteric cysts, meningocele, thymic cysts, cystic teratoma, and lymphangioma. Exact characterization of these cystic lesions on imaging may be sometimes difficult and a final diagnosis is made after histopathological examination.Symptoms may be present at birth if the mediastinal cyst is large in size. Infants may present later with a wide variety of symptoms based on size, location and type. Respiratory distress  can occur due to mass effect. These cysts may cause recurrent chest infections in infants.[7] Our patient did not have any respiratory distress at birth, however had three episodes of lower respiratory tract infections which required treatment with intravenous antibiotics. Hemorrhagic complications like hemoptysis or hematemesis or malena can occur due to heterotopic gastric or pancreatic tissue within the cyst wall causing erosions and peptic ulceration.[1] In our case also, pancreatic tissue was seen in the wall of communicating tract between jejunum and mediastinal cyst resulting in malena episodes. Additionally, spontaneous hemorrhage may occur within the cyst itself.Azygos continuation of the IVC means absence of the hepatic segment of the IVC with azygos continuation. The prevalence is 0.6% .The renal portion of the IVC receives venous return from bilateral kidneys and passes posterior to the diaphragm to enter the thorax as the azygos vein. In our case, all three hepatic veins were draining via a common channel into right atrium. The prominent azygos vein joins the superior vena cava at the normal location.[4]Primary management of neurenteric cysts is surgical resection with the goal of total resection. Partial resection due to vertebral anomalies or extensive adhesions is the main reason for the recurrence.[5]In conclusion, neurenteric cysts have a wide spectrum of associated anomalies with different clinical presentation. This case illustrates the importance of multimodality imaging before surgical strategy planning. Clinico-radio-pathological correlation is a must to reach a final diagnosis in such complex cases for better patient management.Source of Support: NilConflict of Interest: None declared


Xanthogranulomatous cystitis is a rare, benign, chronic inflammatory disease of the bladder, mimicking malignancy with unknown etiology. Herein, we report a 57-year-old man who presented with pollakiuria, nocturia, dysuria, left flank pain, and a palpable mass on the right lower abdomen. Computerized tomography demonstrated an obstructing 10-mm stone in the lower third of the left ureter and a 6-cm solid mass on the right at the anterolateral wall of the bladder. The mass presented local perivesical invasion at the anterolateral side. Cystouretroscopy revealed a mass protruding into the bladder cavity with edematous smooth surface. Frozen section analysis of the partial cystectomy specimen could not rule out malignancy. Therefore, radical cystoprostatectomy and ureterolithotomy were performed. Histologically, fibrosis, numerous plasma cells, eosinophils, and, immunohistochemically, CD68-positive epithelioid and foamy macrophages were detected. Localized prostatic adenocarcinoma was also found. The present case of xanthogranulomatous cystitis is the 23rd to be reported in the world literature.


A 45-year-old male patient, hospitalized and under investigation of mediastinal mass, presenting with sudoresis, malaise, and dysphagia. Computed tomography showed eccentric parietal thickening of the middle third of esophagus, with mass effect and upstream ectasia, with no expressive increase of metabolic activity. Additionally, there are cervical, axillary, pulmonary hilar, portocaval space, and unspecific iliac lymph nodes  and 2.On endoscopy, it was noted that the tumor was related to the aortic arch, left atrium, esophageal wall, and main bronchi. Transesophageal ultrasound-guided punctures were performed. Pathological and immunohistochemical studies showed fusiform cells with no mitotic activity and necrosis, with expression of smooth muscle desmin and actin, concluding the diagnosis of leiomyoma  and 4. In this case, analyzing the age range, symptoms, and tomographic findings, the initial suspicion was of lymphoma, which was excluded only after pathological examination.Lymphadenopathy is the most common lesion of the middle mediastinum, including lymphoma, sarcoidosis, and metastatic lung tumor. Leiomyoma is the most common, accounting for about 60% of benign esophageal tumors. In general, they are small, asymptomatic lesions, which are incidentally detected in exams. In 2% of cases, they present as extraesophageal lesions, and cause compression of adjacent mediastinal structures. Leiomyomata are mesenchymal tumors, typically derived from the muscularis propria, but they can also originate from the muscularis mucosae. Microscopically, there are spindle-cell fascicles with no nuclear atypia. Immunohistochemistry can assist in the differential diagnosis of lymphoma, leiomyosarcoma, gastrointestinal stromal tumor, among others.,5Benign tumors of the esophagus are rare, with a prevalence of up to 0.5%. Surgical treatment is reserved for symptomatic tumors, larger than 2cm or that present with image alterations or growth of more than 1cm during clinical follow-up.Since they are usually small, asymptomatic lesions, with a benign behavior, resection is not mandatory. Clinical follow-up can be carried out by means of endoscopy. Endoscopic resection can be performed in lesions up to 2cm in size.This patient was symptomatic and was referred to surgery. The surgical specimen confirmed the diagnosis.Therefore, leiomyoma should be considered in the differential diagnosis of mediastinal tumors. Endoscopic ultrasound is an important tool for mediastinal assessment. Additionally, it is less invasive than thoracoscopy and mediastinoscopy to collect specimens.


Magnetic resonance-guided radiation therapy (MRgRT) offers advantages for image guidance for radiotherapy treatments as compared to conventional computed tomography (CT)-based modalities. The superior soft tissue contrast of magnetic resonance (MR) enables an improved visualization of the gross tumor and adjacent normal tissues in the treatment of abdominal and thoracic malignancies. Online adaptive capabilities, coupled with advanced motion management of real-time tracking of the tumor, directly allow for high-precision inter-/intrafraction localization. The primary aim of this case series is to describe MR-based interventions for localizing targets not well-visualized with conventional image-guided technologies. The abdominal and thoracic sites of the lung, kidney, liver, and gastric targets are described to illustrate the technological advancement of MR-guidance in radiotherapy. During the last two decades, remarkable progress has been made in the implementation of numerous imaging modalities for the simulation, planning, and delivery of radiation therapy. The early, prominent role of stand-alone computed tomography (CT) imaging systems has been enhanced by the onboard image-guidance technologies of portal imagers, orthogonal radiographic capabilities, megavoltage CT, optical tracking, and cone-beam CT (CBCT). Nonetheless, current CT-based image-guidance techniques still may result in suboptimal image-guidance capabilities due to insufficient soft-tissue contrast, motion degradation, surrogate-based tracking assumptions, and/or a lack of real-time tumor information. Magnetic resonance imaging (MRI) may allow for improvement upon prior CT-based image-guided radiotherapy (IGRT) capabilities. A new era in image-guided technology is rapidly evolving with the integration of an onboard magnetic resonance imaging (MRI) with a radiotherapy treatment system, with the emergence of magnetic resonance-guided radiation therapy (MRgRT).In this case series, we illustrate unique capabilities of MRI guidance with a clinical MRgRT system  for localizing targets not well-visualized by conventional IGRT techniques . The abdMRgRT workflowThe ViewRay MRIdian system is an integrated MRgRT system with onboard treatment planning and delivery capabilities. The MRIdian system combines a split-magnet 0.345 Tesla MRI design with a radiotherapy source of either cobalt-60 or six MV linac accelerator (LINAC) . An overMR and CT simulation sessions are performed for anatomical guidance and electron density information, respectively. Simulation and delivery for a majority of MRgRT cases are performed under a comfortably moderate maximum inspiration breath-hold (MIBH). A balanced steady-state free precession sequence (TrueFISP) acquired in 17-25 second is utilized with a 1.5-mm in-plane resolution and 3.0-mm slice thickness [For treatment delivery, initial patient localization is achieved through MR guidance, with the same 17-25 second MRIdian imaging protocol. When online adaptive radiotherapy is warranted, deformation of electron density and auto-segmentation are performed and evaluated, followed by plan re-optimization on the anatomy of the day. During treatment, the target is continuously tracked on an MR sagittal plane at four frames per second. The radiation beam is then gated based on the percentage volume of the tracking region of interest that strays beyond a pre-specified range.Lung SBRTA 67-year-old male, former smoker, was diagnosed with bilateral synchronous Stage I (T1a N0 M0) squamous cell carcinoma (SCC) of the left upper lobe (LUL) and Stage IA (T1a N0 M0) SCC of the right upper lobe (RUL). An 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT scan showed the LUL nodule was mild to moderately hypermetabolic with maximum standardized uptake values (SUV) of 4.0. The RUL was mildly hypermetabolic with a maximum SUV of 2.1. A biopsy was obtained from the LUL; it was positive for poorly differentiated SCC. A subsequent biopsy of the RUL cavitary lesion confirmed moderately differentiated SCC. MRI brain staging did not show metastases. These lesions were felt to be most consistent with two synchronous early stage lung cancers.3 was registered to the treatment planning CT simulation scan on the first fraction using CBCT localization  measuring 0.89 cmn Figure . The autn Figure , minimizFor MR-guided SBRT, the RUL GTV was defined as the tracking structure with a 5.0-mm margin expansion delineating the gating boundary. Tracking was performed at four frames per second with the treatment beam turned off when ≥10% of the GTV exceeded the gating boundary. Gating latency for the ViewRay MRIdian cobalt-60 system includes image frequency (250 msec), source shuttling, and processing time and has been reported at 436 msec [The deform-generated GTV  was visually inspected during the pretreatment preview cine, in addition to the real-time cine acquired during treatment delivery. Even with a limited in-plane resolution of 3.5 mm for sagittal plane tracking, the deformable image registration (DIR) algorithm appropriately identified and segmented the GTV, as observed with visual inspection  SCC of the right tonsil. He underwent transoral robotic surgery of the right tonsil and level II-IV selective lymph node dissection with one of 25 lymph nodes involved with extra-capsular extension and adjuvant 66Gy in 22 fractions radiation with concurrent weekly cisplatin (40 mg/m2) to the primary and bilateral neck. One year later, he presented with two oligometastatic lung lesions. He initiated cisplatin and 5-fluorouracil. Cisplatin was transferred to carboplatin due to peripheral neuropathy. After disease progression with carboplatin and docetaxel, SBRT was treated to the two lesions. Seven months later a PET/CT showed a new intense focal 18F-FDG uptake near the right hilum associated with a new 2.3x1.8 cm metastatic malignancy. The PET scan revealed hyperintense metabolic activity in the superior pole of the right kidney, measuring 4.2x4.4 cm. A biopsy of the renal mass confirmed metastasis SCC. The renal function was 43.1% (right kidney) and 56.9% (left kidney) with a creatinine of 1.17 (estimated glomerular filtration rate = 70).Respiratory motion for potential internal target volume (ITV)-based treatment with conventional CT-based IGRT was assessed using four-dimensional 4DCT) with abdominal compression; kidney motion up to 0.75 cm along the cranial-caudal direction was observed under shallow breathing. With excellent control of the previously treated lung sites and limited treatment options, we planned treatment to both sites with MRI-guided SBRT utilizing MR-guided gating under MIBH. Treatment was planned for 40Gy in five fractions to the hilar mass. A treatment course to the superior right kidney while sparing the uninvolved lower right kidney was planned for 50Gy in five fractions  and the patient was initially planned to undergo transarterial chemoembolization. Pulmonary function testing revealed anesthesia as a significant risk. He was, therefore, planned to undergo SBRT to the liver GTV. The patient was scheduled for 55Gy in five fractions of MR-guidance; however, due to non-patient-related circumstances, he was transferred to CBCT-based LINAC for the remaining four fractions. This case was unique, as it allowed a direct comparison of onboard image guidance with MRI vs CBCT.Since treatment was performed with CBCT-based localization for four of five fractions, case details are limited to fraction 1 delivery with MRgRT. Gadoxetate has been demonstrated to be well-tolerated in MRgRT for repeated use over an SBRT liver course for tumor localization and real-time tracking  but is nThe GTV was assigned as the tracking structure for MIBH MR-gated delivery Figure . When ≥1Gastric lymphomaA 70-year-old man with stage IIAE-X gastric diffuse large B-cell lymphoma, germinal center type International Prognostic Index 3.0 underwent esophagogastroduodenoscopy and biopsy, demonstrating a gastric mass in the body of the stomach, with the biopsy showing diffuse large B-cell lymphoma, germinal center type. The PET scan demonstrated a marked hypermetabolic soft tissue conglomerate, diffuse thickening of the stomach wall extending to the first portion of the duodenum, and multiple hypermetabolic soft tissue deposits adjacent to the stomach, consistent with disease extension. The patient received six cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy, with an interim PET scan after cycle 5 read as Deauville 4.0, nonspecific for the residual lymphoma versus component of post-chemotherapy inflammation with biopsies demonstrating inflammation.Consolidative involved site radiotherapy to the stomach and perigastric lymph nodes was recommended, and 30Gy in 20 fractions was delivered using MRI-guided IMRT. The patient was instructed to not eat or drink starting four hours prior to treatment. Significant inter-fractional stomach variations on the order of 5.0 cm were observed Figure .Over the course of MRgRT, large deformation and inter-fraction variations were systematically observed between two geometries, as shown in Figure MR-guidance represents a fundamental shift in IGRT utility and methodology. Prior IGRT technologies are often based on the philosophy of daily matching the patient to a prior snapshot of anatomy , requiring substantial and variably effective immobilization. MRgRT allows for the ability to dynamically adjust to the patient’s anatomy of the day by tailoring changes in dose distributions and tumor tracking.The quality and accuracy of online adaptation and real-time tracking rely on the performance of the DIR algorithm. Deformation and dosimetric accuracy for an MR-guided online adaptive radiotherapy program have been evaluated with a deformable, anthropomorphic phantom . The meaThe dosimetric effects of a gating latency of 436 msec [While such daily precision and personalized medicine of MRgRT may not be warranted for all anatomical sites of radiotherapy, the role of MR-guidance in the SBRT of the thoracic and abdominal sites offers clear advantages, as demonstrated by the cases presented here. Prior reporting of the clinical utilization of MRgRT has been limited in the literature to abdominal areas ,6-7 or rMRgRT has great promise to allow for the safe delivery of a higher biologically effective dose to the treatment area that may not have been feasible with prior technology. Adaptive radiotherapy allows for dose escalation to a target when the neighboring organ at risk (OAR) has moved away from the treatment area or for dose de-escalation to safely achieve normal tissue toxicity constraints when OAR has moved into the treatment area. The new era of MR guidance may enable changes to clinical practice, including a higher dose per fraction, reduced treatment margins with potentially reduced toxicity, and a lower likelihood of geometric miss. Clinical trials will be necessary to fully understand the potential benefits of this technology.MR guidance for thoracic and abdominal tumors of the lung, liver, stomach, and kidney is feasible and allows for improved localization over conventional CT-based IGRT capabilities. Superior soft-tissue visualization combined with the MRgRT ability to dynamically adjust the treatment plan and/or gate the treatment delivery to account for inter-/intrafractional anatomical changes offers great promise to further enhance treatment precision for abdominal and thoracic anatomical sites. This approach brings valuable opportunities to decrease overall toxicity profiles.


Hepatocellular carcinoma (HCC) is the leading cause of death among patients with cirrhosis. Therefore, a focal hepatic lesion in a patient with cirrhosis must always be investigated for its high risk of cancer. However, when hepatic lesions in an imaging exam do not present the typical characteristics of a malignant or a benignant tumor, diagnosis may be a challenge. The biopsy can be used in these circumstances, but, as shown by this case, even that can be misleading. A 54-year-old male patient with cirrhosis presented with abdominal pain, jaundice, nausea and vomits. He performed a biopsy at another service, with the result being focal nodular hyperplasia (FNH). He presented adequate hepatic function, and alpha-fetoprotein level was 6.4. Upon first consultation, we required the slides to be brought to our service and reviewed. Our revision also showed no signs of malignancy. Magnetic resonance imaging showed a large hepatic tumor in segments V and VI, predominantly exofitic, with a central scar. The tumor was surgically removed, and its dimensions were 14 × 10 × 9 cm. Microscopic examination revealed an HCC. Even though histological diagnosis was not necessary to indicate surgery, due to its exofitic nature and adequate hepatic function, we discuss the diagnostic characteristics of both HCC and FNH that could help other medical groups in cases where the position of a liver tumor could make the decision to operate more difficult. Hepatocellular carcinoma (HCC) is a common malignant tumor of the liver, but the differential diagnosis with benign tumors of the liver may be a challenge. When typical findings of these tumors are not present in imaging exams, it may be required to extirpate the tumor without previously knowing the diagnosis, or resort to a biopsy. However, as shown by this case report, even the biopsy may be misleading.In this case report, we present the case of a patient with alcoholic cirrhosis that developed abdominal pain. Imaging exams showed a large mass originating from segments V and VI of the liver. A biopsy was performed and showed signs suggestive of focal nodular hyperplasia (FNH). However, after the tumor was surgically removed, it was identified as HCCs.A 54-year-old male patient, with previous diagnosis of alcoholic cirrhosis, presented with abdominal pain for 5 years, in the right hypocondrium, associated to jaundice, nausea, vomits and the loss of 12 kg of weight in the last year. The pain had no relation to eating. He reported a previous hospitalization in another hospital to perform a liver biopsy, which showed signs of FNH. He was referred to our service due to the lack of a proper structure to operate this patient. Upon the first consultation, we requested the slides of the biopsy to be brought to our service for a review, which revealed an altered lobular structure due to portal fibrosis, forming large fibrous septa, and no signs of a malignant tumor.We performed a magnetic resonance imaging (MRI) exam , which rBiochemical analyses performed at this time showed that his alpha-fetoprotein value was 6.4. With this finding, the biopsy and the imaging exam, his diagnosis was set as FNH.Other biochemical analyses performed were aspartate transaminase: 34, alanine transaminase: 27, albumin: 2.8, direct bilirubin: 0.5, indirect bilirubin: 0.3, international normalized ratio: 1.51. Given he had no signs of encephalopathy or ascites, he was considered Child-Pugh A. Hepatitis B and C serologies were negative.Surgical intervention was performed because of the symptoms. The tumor was successfully extirpated . After hMacroscopically, the dimensions of the tumor were 14 × 10 × 9 cm, and weighed 701 g. Upon being sectioned in half , a margiThe relationship between cirrhosis and HCC is well established. HCC is the sixth most common cancer worldwide, and is the leading cause of death among patients with cirrhosis . TherefoThe imaging exam of choice for this patient was the MRI. Several studies have demonstrated the superiority of MRI in both lesion detection and characterization for focal hepatic lesions when compared to CT . FNH tipThe size of the tumor, 14 × 10 × 9 cm, also contributes to the diagnostic doubt. Even though FNH can present itself as a volumous abdominal mass, it is usually of smaller size. One study found that only two out of 86 patients (2%) had a tumor with a diameter greater than 10 cm [This patient presented himself to us already with a biopsy, which showed no signs of malignancy. The biopsy was brought to our service and reviewed, and the result was the same. The material was obtained through open liver biopsy, and it is possible it only sampled the area around the lesion. Classic FNH is characterized by the presence of abnormal nodular architecture, malformed vessels and cholangiolar proliferation, and nonclassic is characterized by the presence of two of the previous criteria, but always need to present cholangiolar proliferation . ConsideA further refinement in the exams performed in this case that could have contributed to the diagnosis would be the hepatic tissue-specific MR contrast media, such as superparamagnetic iron oxide (SPIO) or gadoxetic acid. Iso- or hyperintensity, or sometimes ring enhancement, of the lesion in the hepatocyte image of Gd-EOB-DTPA-enhanced MR images is a very specific finding of FNH .In the present case, due to the satisfactory hepatic function, and the predominantly exofitic location, surgery was indicated independently of hystological diagnosis, and therefore it did not present doubts on the conduction of the case. But a different case, in which determining malignancy of a tumor in a central location of the liver is a challenge, may benefit from this discussion about the diagnostic characteristics of liver tumors, which could contribute to the decisions made by a different medical group.


Background. Abdominal pain during pregnancy has a broad differential diagnosis which includes spontaneous adrenal hemorrhage (SAH). There is scant literature available on optimal mode of delivery in stable patients. Cases. Patient 1 was a 35-year-old nullipara who presented at 36 weeks of gestation with left flank pain. Patient 2 was a 27-year-old multipara at 38 weeks who presented with left upper quadrant pain. Diagnosis of SAH was made by CT scan and both were managed with pain control, serial hemoglobin assessments, and abdominal exams resulting in uncomplicated vaginal deliveries. Conclusion. SAH, although rare, is an important consideration when evaluating abdominal and flank pain in pregnancy. Management options vary from conservative management to surgical intervention depending on the stability of the patient. Abdominal pain, a common complaint during pregnancy, has a broad differential diagnosis which includes spontaneous adrenal hemorrhage (SAH). While autopsy reports reveal that between 0.03 and 1.8% of unselected cases demonstrate adrenal hemorrhage, the incidence among pregnant women is unknown , 3. RareA 35-year-old nulliparous patient at 36 weeks of gestation presented to labor and delivery complaining of sudden onset, left sided back pain which radiated anteriorly. She denied any other gastrointestinal or urinary symptoms. The patient had a past medical history significant only for chronic hypertension and a past surgical history of laparoscopic Roux en Y gastric bypass two years priorly. She denied any prior complications related to this surgical history. The pregnancy was further complicated by gestational diabetes class A1. On presentation the patient had a blood pressure of 162/80 and heart rate of 70 beats per minute and was saturating 99% on room air. Initial exam revealed the abdomen to be tender to palpation in left upper quadrant with no guarding and no peritoneal signs. There were no symptoms of labor and fetal status was reassuring.Complete blood count, coagulation studies, liver function tests, amylase, and lipase were all within normal limits with a hemoglobin of 12.4 g/dL. A urinalysis demonstrated rare bacteria and calcium oxalate crystals, but no blood. A renal ultrasound showed no evidence of hydronephrosis, mass, or stone. An obstetrical ultrasound revealed a live singleton fetus with a normal appearing anterior placenta and appropriate fetal growth. Intravenous narcotics were required for adequate pain control. General surgery was consulted given her history of gastric bypass and concern for a possible related complication. A CT scan of the abdomen demonstrated a mildly enlarged left adrenal gland with areas of hyperdensity consistent with acute left adrenal hemorrhage . This waA 27-year-old multiparous patient presented to labor and delivery at 38 weeks of gestation for evaluation of sudden onset, left upper quadrant pain that radiated to the midline. The patient also reported regular, painful contractions. She had a past medical history significant for nephrolithiasis during her previous pregnancy, bipolar disease, and migraines. Prior surgeries included a diagnostic laparoscopy for chronic pelvic pain, Loop Electrosurgical Excision Procedure, and 2 elective terminations of pregnancy. The patient was a 5-6-cigarette/day smoker. On admission vital signs were within normal limits. Physical examination revealed her abdomen to be soft and minimally tender to palpation. A complete blood count was within normal limits with a hemoglobin of 12.4 g/dL. A urine drug screen was negative and a urinalysis revealed no blood or evidence of infection. Fetal status was reassuring and an obstetric ultrasound revealed no apparent pathology. The patient's pain improved with intravenous narcotics, antacids, and a muscle relaxer and was attributed to a likely musculoskeletal etiology. However, the patient was found to be in spontaneous labor and progressed to deliver a male infant without complication. Intrapartum pain relief was provided via epidural. On the first postpartum day the patient again complained of left sided flank and upper abdominal pain and was presumptively treated for nephrolithiasis, given the clinical presentation and history, with intravenous narcotics and hydration. Despite treatment, the pain worsened and was associated with nausea and two episodes of emesis. A CT scan of the abdomen revealed enlargement and mild heterogeneity of the left adrenal gland with surrounding fat stranding consistent with acute left adrenal hemorrhage. An MRI performed at the request of the attending physician confirmed these findings as seen in If unrecognized, adrenal hemorrhage can lead to adrenal crisis, shock, and theoretically death for both mother and fetus and should be considered in the differential diagnosis of abdominal pain in pregnancy , 4. PresWhile the initial abdominal imaging study in pregnancy is typically ultrasound, sonographic findings of adrenal hemorrhage are nonspecific. MRI or CT scan is needed to confirm the diagnosis and to evaluate for potential underlying etiology such as pheochromocytoma or malignant tumor . On MRI,Appropriate management of SAH in pregnancy depends on the stability of the patient. Conservative management includes supportive therapy with intravenous fluids, pain control, and serial hemoglobin assessments with blood transfusion and correction of coagulopathy as indicated . Close mSAH, although rare, is an important consideration when evaluating abdominal and flank pain in pregnancy. Diagnosis requires a high index of suspicion, particularly when more common etiologies of pain are excluded as was demonstrated in the two cases presented in this report. Diagnosis can be made by MRI or CT scan. In a clinically stable pregnant patient with SAH conservative management and vaginal delivery are safe and appropriate.


Identification of the underlying cause of pulsatile tinnitus is important for treatment decision making and for prognosis estimation. For this, an adequate diagnostic imaging strategy is crucial.Both CT and MRI can be useful, and in general, these modalities provide complementary diagnostic information. The scanning protocol can be optimized based on the estimated a priori chance for finding specific pathology, or the need to rule out more rare but clinical significant disease. In recent years, dynamic CTA, also referred to as 4D-CTA, has become available as a new technique that enables non-invasive evaluation of hemodynamics for the detection, classification, and follow-up of vascular malformations.The value of different diagnostic imaging modalities in the work-up of pulsatile tinnitus is discussed in relation to the differential diagnosis. Furthermore, imaging findings of different diseases are presented, both for CT and MRI.The online version of this article (doi:10.1007/s40134-017-0199-7) contains supplementary material, which is available to authorized users. Tinnitus is defined as an auditory perception of internal origin, and can have a significant influence on the well-being and performance in daily activities of affected subjects . The audIn about 70% of the cases with pulsatile tinnitus, an underlying cause can be identified by adequate diagnostic work-up . VasculaWhen the auditory perception is only perceived by the patient and cannot be heard by the clinician by auscultation, it is called subjective tinnitus. In case of objective tinnitus, which can be heard by the clinician, more frequently the etiology can be found by ancillary investigations and there is usually a genuine physical source of sound in contrary to subjective tinnitus . SubjectIdentification of the underlying cause of pulsatile tinnitus is important for adequate treatment and for prognosis estimation. Different guidelines are available for the diagnostic work-up of pulsatile tinnitus , 11, 16.In this review, we will discuss imaging strategies for the diagnostic work-up of pulsatile tinnitus. Furthermore, the differential diagnosis of pulsatile tinnitus is discussed and imaging findings of different diseases are presented, both for CT and MRI. We differentiate vascular, neoplastic, and osseous etiologies.Considering the broad differential diagnosis of pulsatile tinnitus, the optimal diagnostic imaging strategy depends on the initial clinical evaluation. Both CT and MRI can be useful, and in general, these modalities are complementary. The scanning protocol can be optimized based on the estimated a priori chance for finding specific pathology, or the need to rule out more rare but clinical significant disease. The value of imaging modalities for the detection and characterization of different pathologies, which can cause pulsatile tinnitus, is presented in Table For screening for underlying pathology and for the evaluation of a possible soft tissue mass or intracranial pathology, initial evaluation with MRI and MR angiography (MRA) is recommended with reported high diagnostic accuracy •. An appFor the evaluation of osseous pathology of the temporal bone, a limited scanning range of thin-sliced (submillimetric) CT is sufficient. Multiplanar reconstruction (MPR) of the acquisition is crucial for adequate evaluation, such as the identification of bony dehiscence of vascular canals or the skull base.Multi-detector CTA or CT venography (CTV) of the head and neck region can be performed for the evaluation of vascular pathology. Bone window images of the skull and temporal bone can be reconstructed from a multi-detector CTA or CTV acquisition, obviating the need for a separate acquisition, which reduces radiation exposure. While the anatomic evaluation of multi-detector CTA/CTV is excellent, the evaluation of flow dynamics is limited because only a single time point is obtained during the passage of a contrast bolus. Dynamic CTA, also referred to as 4D-CTA, is a technique that combines the non-invasive nature of CTA with the dynamic acquisition of digital subtraction angiography (DSA) •. 4D-CTAThe role of DSA in the diagnostic work-up of pulsatile tinnitus has been minimized, and should be reserved for the indication to rule out vascular pathology in case MRI/MRA and CT/(4D-)CTA have not revealed the cause of pulsatile tinnitus. DSA can also be performed for the purpose of treatment planning, e.g., for determining the exact angioarchitecture of a vascular malformation or in preoperative tumor embolization.The role of duplex ultrasound in the diagnostic work-up of pulsatile tinnitus is limited, although duplex ultrasound is an effective screening tool for the evaluation of superficial tissue structures or superficial vascular malformations and for the evaluation of vessel wall pathology of the carotid arteries.An AVM consists of a network of tortuous dilated arteries and veins, referred to as a nidus, through which shunting occurs between arteries and veins without the presence of a normal intervening arteriole-capillary bed. Typically, an AVM develops in adolescence or young adulthood but can remain occult for a long period . An AVM An AVF is an, usually acquired, abnormal connection between an artery and a vein without an intervening nidus. Located along the dura or within a dural sinus, these are called dural AVF (dAVF). A direct arteriovenous shunt between a cerebral artery and a cortical draining vein is called a pial AVF, and occur within the brain or along the brain surface. An AVF can have a simple (single AVF) or more complex (multihole AVF) angioarchitecture.The pathophysiology of dAVFs is controversial, but mostly considered a result of local hypoperfusion within a thrombosed dural sinus . ProgresAVF is more frequent in pulsatile tinnitus than AVM, with reported prevalence numbers varying from 2 to 27% , 22, 23.The nidus and tortuous vessels of an AVM can be detected either by MRI/MRA or CTA Fig. , althougThe main arterial feeders and the patterns of venous drainage of a dAVF or AVM as detected by 4D-CTA or MRA seem to be sufficient in most cases to correctly identify and classify an AVM or AVF, which could save the patient a pre-treatment invasive DSA , 28, 32.In the elderly population, atherosclerotic disease of the carotid or vertebral arteries is thought to be the most common cause of pulsatile tinnitus . In a siFibromuscular dysplasia (FMD) is a segmental non-atheromatous, non-inflammatory vascular disease of unknown etiology. Often it is a disease of the young leading to vascular stenosis and cerebral ischemia. In medium-sized arteries, like the vertebral and carotid arteries, fibroblast-like changes of the smooth muscle cells cause narrowing of the arteries and seem to cause pulsitale tinnitus more frequently than in atherosclerotic disease. This is probably due to the location of arterial stenosis in FMD. In FMD, stenosis of the carotid artery is frequently located at the upper cervical level, resulting in easily transmitted vascular turbulence to the temporal bone. The classical imaging appearance of FMD is the so-called “string of beads” pattern shown on angiographic studies.Vascular loops and elongated arteries are occasionally described as a possible cause of pulsatile tinnitus [tinnitus , 36. ConAn aberrant course of the internal carotid artery and persistence of the stapedial artery are congenital variants that need to be recognized on imaging studies. An aberrant course of the internal carotid artery in the middle ear may mimic a soft tissue mass or paraganglioma at otoscopy. The aberrant carotid artery enters the tympanic cavity via an enlarged tympanic canaliculus and then runs though the middle ear where it, due to a dehiscence in the carotid plate, enters the horizontal carotid canal Fig. . The ascA persistent stapedial artery fails to regress in early fetal development. As a result, the proximal course of the middle meningeal artery will not develop and the foramen spinosum will be absent Fig. . In addiVenous tinnitus is heard as a continuous murmer that exaggerates in systole. By light pressure on the ipsilateral jugular vein, the murmur decreases. Light pressure on the contralateral jugular vein will increase the murmur. Rotating the patient’s head away from the involved side may relieve the murmur. Symptoms may increase by rotating the head toward the involved side. There seems to be an association with congenital variants such as a high-riding, enlarged, or diverticulum of the jugular bulb, which can be best depicted on thin-sliced high-resolution CT . PrevaleParaganglioma, also known as glomus tumor, is the most frequent neoplastic cause of pulsatile tinnitus , 41. MosBeing a highly vascularized lesion, it is one of the most common causes of pulsatile tinnitus. Involving only the jugular bulb (glomus jugulare), the middle ear or mastoid (glomus tympanicum) or both (glomus jugulotympanicum), most of the paragangliomas located in the temporal bone will present with pulsatile tinnitus . BilaterA tympanic paraganglioma can be detected by otoscopic inspection as a red, pulsatile tympanic mass and can be very small at presentation measuring only a few millimeters. Paragangliomas grow along planes of least resistance following existing pathways, through bony canals or along the vessels and nerves. A tympanic paraganglioma can arise from glomus bodies anywhere along the Jacobsen nerve, a tympanic branch of the glossopharyngeal nerve.Both CT and MRI can be used for the detection and evaluation of a paraganglioma. The majority of tympanic paragangliomas are located on the promontory as a small well-defined tympanic mass with soft tissue mass. Usually, there is no or little surrounding bone erosion. These small tumors are best evaluated using thin-sliced CT with a bone algorithm Fig. .Fig. 4GlThere is an anatomic close relation between the tympanic cavity and the jugular foramen. As a result, a large percentage of the paragangliomas will present as the jugulotympanic variant due to a tumor located in the jugular foramen with extension through the jugular plate into the hypotympanum. MRI can be used to evaluate bony invasion, although this is usually better appreciated on CT. A CT scan with bone algorithm may show irregular erosions of the adjacent bone Fig. . These bThe classical MRI characteristics of a paraganglioma include a mixture of hypo- and hyper-intensity on T1-W and T2-W sequences, also referred to as a ‘salt-and-pepper’ pattern, based on multiple vascular flow voids within the lesion. Due to the hypervascular nature, a paraganglioma enhances avidly Fig. . DynamicPaget disease is a primary bone disorder, which can be located in different parts of the skeleton, including the skull and temporal bone. The disease is characterized by osteoclastic resorption, osteoblastic regeneration, and mosaic bone replacement, seen as areas of abnormal lytic or sclerotic changes of bony structures on CT or MRI. Increase in the number and size of local vessels in Paget disease could be the cause of pulsatile tinnitus .Otosclerosis, also known as otospongiosis, is an idiopathic infiltrative process of the petrous bone. It causes both sensorineural and conductive hearing loss, and can be the cause of pulsatile tinnitus , 47. TwoHighly vascularized bone lesions, like osseous hemangioma, basal meningeoma, Langerhans cell histiocytosis, or bone metastases, have been described as possible causes of pulsatile tinnitus –50.osseous hemangioma is a sharply demarcated expansile intraosseous lesion. Typically, aggravated trabecular thickening is seen on CT with preservation of the inner and outer cortex. MRI will show heterogeneous hyper-intense signal on T1-W and T2-W sequences, and less frequently hypo- or iso-intense signal intensity. A hemangioma enhances diffusely after contrast administration.An meningioma, a tumor originating from the meninges, can show permeative changes and sclerosis of the adjacent bone but can also have a significant intraosseous localization. A meningeoma is characterized by homogeneous contrast enhancement and typically shows a dural tail configuration , which predominantly affects young obese women, may cause pulsatile tinnitus, although IIH is primarily characterized by symptoms of headache and blurred vision due to increased cerebrospinal fluid pressure ••. BrainPulsatile tinnitus can lead to significant morbidity, and identification of the underlying cause is important for adequate treatment and prognosis estimation. In general, head and neck MRI and CT provide complementary diagnostic imaging information. Which imaging modality to choose as well as the optimal scanning protocol depends on the estimated a priori chance for finding specific pathology, or the need to rule out more rare but clinical significant disease.For screening for underlying pathology, and for the evaluation of a possible soft tissue mass or intracranial pathology, evaluation with MRI and MR angiography (MRA) is recommended. In case MRI/MRA does not reveal an underlying cause of pulsatile tinnitus, 4D-CTA can be considered as a non-invasive alternative to DSA for the detection of a dAVF. When pathology of the tympanic cavity or osseous pathology of the temporal bone is suspected based on the clinical evaluation, initial evaluation with CT(A) is advised. The role of DSA is minimized, and can be considered to rule out vascular pathology, in case of high suspicion of vascular pathology while MRI/MRA and CT/(4D-)CTA have not revealed the cause of pulsatile tinnitus. DSA can also be performed for the purpose of treatment planning.By using an adequate diagnostic imaging strategy, the underlying pathology of pulsatile tinnitus can be identified in the majority of patients.Video 14D-CTA of dAVF located in the right sigmoid sinus, as shown in Fig. Video 2DSA of dAVF located in the right sigmoid sinus, as shown in Fig. Below is the link to the electronic supplementary material.


Ewing sarcoma (ES) is a primary malignant bone tumor which most commonly arises in children and young adults. The common clinical presentation with ES includes nighttime pain or pain related to activity, though patients may also present with a combination of localized swelling, a palpable mass, pathologic fracture, and constitutional symptoms. Clinical diagnosis may be delayed when a patient presents with clinical or imaging findings that overlap with non-malignant etiologies, such as fibrous dysplasia (FD) or osteomyelitis. Furthermore, multimodality imaging, including computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine may prove inconclusive in particular cases. Suspicion for malignancy should not be overlooked. A biopsy must be considered, unless the diagnosis is evident, such as a clinical response to antibiotics in the setting of osteomyelitis.  Ewing sarcoma (ES) is a primary malignant bone tumor that has been classified within a larger group of neoplasms termed Ewing sarcoma family of tumors (ESFT) . ES predHistologically, ES may vary in its degree of neural differentiation, though it most commonly consists of sheets of small, uniform cells with round nuclei, often with an infiltration of the surrounding tissue, hemorrhage and necrosis . The speA typical clinical presentation with ES includes nighttime pain or pain related to an activity . PatientIn certain cases, however, ES may not demonstrate radiographically aggressive features. Important considerations during the workup of indeterminate lucent bone lesions in the pediatric and young adult population include fibrous dysplasia (FD) and osteomyelitis, both of which demonstrate clinical and imaging overlap with ES -8. We prA 13-year-old male presented for an evaluation of a progressive left hip pain, worsening over the past five months, with no history of trauma. The patient reported that his hip pain had increased markedly over the past couple of months, resulting in a limp. Furthermore, the patient expressed increasing fatigue, though he denied any night sweats or unintended weight loss. After an evaluation by his primary care physician (PCP), the patient was referred to a pediatric orthopedist, and an imaging of the hip was ordered.An initial imaging of the left hip consisted of a plain radiograph Figure . The radThe pathology demonstrated small, primitive cells infiltrating fibrous tissue and bone Figure . An immuA surgical intervention was offered to the patient and the family. However, after a full consideration of surgical vs. non-surgical treatment options, the decision was made to pursue chemotherapy and radiation. The patient has now completed one full year of curative chemotherapy with vincristine, doxorubicin, and Cytoxan and will begin a short interval of surveillance.Ewing sarcoma is an infrequent primary bone tumor with a preference for the diaphysis and meta-diaphysis of long bones, accounting for approximately six to eight percent of malignant primary bone tumors in younger patients , 8. The A benign presentation on imaging may mimic the appearance of FD, a benign entity characterized by a nonaggressive, geographic lesion with a variable matrix, often associated with bone expansion and remodeling. A review of published literature revealed multiple cases of patients presenting with benign-appearing long bone lesions and characteristic findings of FD with a subsequent pathology diagnostic for ES , 8. EvalIn cases of localized bone pain and swelling with associated fevers and elevated inflammatory markers, osteomyelitis may be initially favored. However, ES may have an identical presentation with fevers and elevated inflammatory markers secondary to tumor necrosis . For thiEwing sarcoma is a primary malignant bone tumor that affects children and young adults. The clinical presentation and imaging findings may have significant overlap with non-malignant etiologies, including fibrous dysplasia and osteomyelitis. In cases of an indeterminate bone lesion with appropriate clinical context, Ewing sarcoma should remain in the differential with the possibility of bone biopsy.