The impact of multidisciplinary approaches on the outcomes of olfactory neuroblastoma treated with postoperative radiotherapy.

BACKGROUND: We investigated the outcomes of postoperative radiation therapy for olfactory neuroblastoma (ONB) and our cross-departmental collaboration to enhance the effectiveness of cancer treatment. METHODS: We retrospectively evaluated 22 patients with ONB who underwent postoperative radiotherapy after tumor resection. En bloc resection was performed; pathology specimens were prepared in coronal sections; and irradiation fields were determined after discussion with radiation oncologists, head and neck surgeons, and pathologists. RESULTS: The overall survival and local control rates were 95.5% and 100%, respectively, at a median 37-month follow-up. The 3- and 5-year disease-free survival (DFS) rates were 64.4% and 56.3%, respectively. Of the 22 patients, 9 (8 Kadish C and 1 Kadish B) had disease recurrence. Of the nine patients, five had positive margins and two had closed margins; cervical lymph node recurrence occurred in six, and distant metastasis with or without cervical lymph node recurrence occurred in three. DFS analysis of risk factors showed no statistically significant differences, but positive margins were a significant recurrence factor in multivariate analysis. CONCLUSIONS: The local control rate of ONB treated with postoperative radiation therapy was 100%. This may be attributed to cross-departmental cooperation between head and neck surgeons, pathologists, and radiation oncologists, which resulted in accurate matching of CT images for treatment planning with the location of the tumor and positive margins. Longer follow-up periods are required to evaluate the effectiveness of our strategy.

Distribution of proteinase K-resistant anti-α-synuclein immunoreactive axons in the cardiac plexus is unbiased to the left ventricular anterior wall.

Lewy body disease (LBD) is characterized by the appearance of Lewy neurites and Lewy bodies, which are predominantly composed of α-synuclein. Notably, the cardiac plexus (CP) is one of the main targets of LBD research. Although previous studies have reported obvious differences in the frequency of Lewy body pathology (LBP) in the CP, none of them have confirmed whether LBP preferably appears in any part of the CP. Thus, we aimed to clarify the emergence and/or propagation of LBP in the CP. In this study, 263 consecutive autopsy cases of patients aged ≥50 years were included, with one region per case selected from three myocardial perfusion areas (MPAs) and subjected to proteinase K and then immunohistochemically stained with anti-α-synuclein antibodies to assess LBP. We stained all three MPAs in 17 cases with low-density LBP and observed the actual distribution of LBP. LBP were identified in the CP in 20.2% (53/263) of patients. Moreover, we found that LBP may appear in only one region of MPAs, mainly in the young-old group (35.3% (6/17) of patients). These findings suggest that it is possible to underestimate LBP in the CP, especially in the young-old group, by restricting the search to only one of the three MPAs.

Immunoglobulin G4-related hepatic artery aneurysm.

A 49-year-old man who was a current smoker with a history of hypertension, dyslipidemia, and coronary artery disease after coronary stent placement presented because of abdominal and back pain. Contrast-enhanced computed tomography showed a 30-mm, large hepatic artery aneurysm. Resection of the aneurysm and autogenous vein bypass grafting was performed, which resulted in a successful outcome without any complications. Pathologic examination of the aneurysm confirmed that it was related to immunoglobulin G4 (IgG4). The patient's serum IgG4 level was within the normal range, and no other signs of IgG4-related organ lesions were observed.

Radiological features of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: case series and systematic review.

PURPOSE: To comprehensively summarize the clinical data and CT/MRI characteristics of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA). METHODS: Twenty-seven lesions from 25 study articles identified through a systematic review and three lesions from our institution associated with TL-LGNPPA were evaluated. RESULTS: The mean age of the patients at diagnosis was 35.7 years, and the male-to-female ratio was nearly half. The chief complaint was nasal obstruction, followed by epistaxis. All patients underwent excision. None of the patients had neck nodes or distant metastases. All patients survived with no locoregional/distant recurrence during 3-93 months of follow-up. All lesions were located at the posterior edge of the nasal septum, attached to the nasopharyngeal parietal wall, and showed no laterality. The mean lesion diameter was 1.7 cm. The margins of lesions were well-defined and lobulated, followed by well-defined smooth margins. None of lesions were associated with parapharyngeal space or skull base destruction. All lesions were iso- and low-density on non-contrast CT. Adjacent skull base sclerosis was detected in 63.6% of lesions. High signal intensity on T2-weighted imaging and mostly iso-signal intensity on T1-weighted imaging compared to muscle tissue. Most lesions were heterogeneous and exhibited moderate contrast enhancement. Relatively large lesions (≥1.4 cm) tended to be more lobulated than smooth margins compared to relatively small lesions (<1.4 cm) (p = 0.016). CONCLUSION: We summarized the clinical and radiological features of TL-LGNPPA to facilitate accurate diagnosis and appropriate management.

A rare case of epithelial-myoepithelial carcinoma ex pleomorphic adenoma of the parotid gland: Radiologic-pathologic correlation.

Carcinoma ex pleomorphic adenoma is a carcinoma that arises from a primary or recurrent benign pleomorphic adenoma. The prevalence of epithelial-myoepithelial carcinoma is low, and this histological type accounting for only 1% of all salivary gland tumors. Here, we report a rare case of Epithelial-Myoepithelial Carcinoma ex pleomorphic adenoma of the parotid gland with a radiologic-pathologic correlation.

Prognostic survival biomarkers of tumor-fused dendritic cell vaccine therapy in patients with newly diagnosed glioblastoma.

Dendritic cell (DC)-based immunotherapy has been applied to glioblastoma (GBM); however, biomarkers informing response remain poorly understood. We conducted a phase I/IIa clinical trial investigating tumor-fused DC (TFDC) immunotherapy following temozolomide-based chemoradiotherapy in patients with newly diagnosed GBM and determined prognostic factors in patients receiving TFDC immunotherapy. Twenty-eight adult patients with GBM isocitrate dehydrogenase (IDH) wild-type (IDH-WT) were enrolled; 127 TFDC vaccine injections (4.5 ± 2.6 times/patient) were administered. Patients with GBM IDH-WT had a respectable 5-year survival rate (24%), verifying the clinical activity of TFDC immunotherapy, particularly against O6-methylguanine-DNA methyltransferase (MGMT) unmethylated GBM (5-year survival rate: 33%). To identify novel factors influencing overall survival (OS) in GBM IDH-WT treated with TFDC immunotherapy, clinical parameters were assessed and comprehensive molecular profiling involving transcriptome and exome analyses was performed. MGMT promoter methylation status, extent of tumor resection, and vaccine parameters (administration frequency, DC and tumor cell numbers, and fusion ratio) were not associated with survival following TFDC immunotherapy. Old age and pre- and post-operative Karnofsky performance status were significantly correlated with OS. Low HLA-A expression and lack of CCDC88A, KRT4, TACC2, and TONSL mutations in tumor cells were correlated with better prognosis. We validated the activity of TFDC immunotherapy against GBM IDH-WT, including chemoresistant, MGMT promoter unmethylated cases. The identification of molecular biomarkers predictive of TFDC immunotherapy efficacy in GBM IDH-WT will facilitate the design of and patient stratification in a phase-3 trial to maximize treatment benefits.

Clinical Features of Borderline Ovarian Seromucinous Tumor.

BACKGROUND/AIM: Ovarian seromucinous tumor is a histological type of ovarian neoplasm. Although seromucinous borderline tumors (BSMT) are associated with endometriosis, the frequency of their occurrence is low, and many aspects of their behavior remain unclear. In this study, we aimed to clarify the clinicopathological factors of BSMT. PATIENTS AND METHODS: We retrospectively reviewed 32 patients with pathologically diagnosed BSMT who underwent surgery at Jikei University Hospital. The survey items were patient characteristics, such as age, initial symptoms, preoperative tumor markers, surgical procedure and stage of surgery, presence of endometriosis, and recurrence. RESULTS: The median age was 45 years. Lower abdominal pain was the most common chief complaint, about one-third of patients were asymptomatic; one-sixth were discovered during follow-up for endometriosis. The majority had a high serum CA19-9 level. Twenty-five patients (78.1%) had unilateral masses, whereas seven patients (21.9%) had bilateral masses. More than 90% of the cases had coexisting endometriosis histologically. Thirty cases (93.8%) were stage I, only two were stage II, and none were stage III or IV. Recurrence was observed in two cases: one was borderline malignant and the other was a carcinoma. CONCLUSION: BSMT is a rare form of borderline malignancy. Its preoperative diagnosis is often difficult because of various clinical findings, but a history of endometriosis and an elevated serum CA19-9 level may aid in some cases.

18F-Fluoromisonidazole-Positron Emission Tomography and Immunohistochemistry Verified Tumor Oxygenation, Stemness, and Immunosupportive Microenvironment After Preoperative Neoadjuvant Bevacizumab for Newly Diagnosed Glioblastoma.

BACKGROUND: Cancer stemness and immunosuppressive tumor microenvironment (TME) in accordance with tumor oxygenation are variable during bevacizumab (Bev) therapy for glioblastoma (GBM). Positron emission tomography (PET) using 18F-fluoromisonidazole (FMISO) reflects hypoxic TME. The aim of this study was to compare FMISO-PET and immunohistochemical findings of tumor oxygenation in the TME of GBM during Bev treatment. METHODS: Seven patients with newly diagnosed IDH-wildtype GBM underwent FMISO-PET during follow-up. Three patients received preoperative neoadjuvant Bev (neo-Bev) and subsequently underwent surgical resection. Reoperation was performed at the recurrence. FMISO-PET was performed before and after neo-Bev. Four patients who underwent tumor resection without neo-Bev were included as the control group. Expressions of hypoxic markers (carbonic anhydrase; CA9), stem cell markers (nestin, FOXM1), and immunoregulatory molecules (CD163, FOXP3, PD-L1) in tumor tissues were analyzed by immunohistochemistry (IHC). RESULTS: All 3 patients treated with neo-Bev showed decrease in FMISO accumulation in accordance with expressions of CA9 and FOXM1 compared with the control group. Two of these 3 patients at the recurrence showed increase in FMISO accumulation. IHC showed increased CA9-and FOXM1-positive cells in recurrent tumors. Expression of PD-L1 tended to be lower after neo-Bev compared with the control group. CONCLUSIONS: FMISO-PET effectively visualized TME oxygenation after neo-Bev. Increased FMISO accumulation at the time of recurrence, even under Bev treatment, suggests that FMISO-PET might be useful for monitoring the duration of Bev efficacy by reflecting tumor oxygenation.

Fully-Endoscopic Resection of Deep-Seated Pilocytic Astrocytoma Under 5-Aminolevulinic Acid Fluorescence Guidance: A Technical Note.

AIM: To improve the extent and safety of resecting these deep-seated tumors, we report a novel procedure of minimally invasive endoscopic resection of deep-seated pilocytic astrocytomas under the guidance of 5-aminolevulinic acid (5-ALA) fluorescence undescribed until now. CASE DESCRIPTION: A 53-year-old male presented with a gradually progressing mild right hemiparesis. Imaging studies showed a solid tumor with degenerative cystic formation in the left basal ganglia. The tumor was removed endoscopically via right frontal small craniotomy. The tumor was positive for 5-ALA fluorescence and allowed better detection of the dissection margin of the solid tumor from the surrounding brain tissue. The histopathological diagnosis was pilocytic astrocytoma. No recurrence was observed on follow-up magnetic resonance imaging (MRI) 2 years after surgery, and the patient was fully independent after rehabilitation. CONCLUSION: This minimally invasive technique, enhanced by intraoperative fluorescence, might be a safe and feasible alternative to open surgery in the removal of deeply located gliomas.

Impact of Neoadjuvant Bevacizumab on Neuroradiographic Response and Histological Findings Related to Tumor Stemness and the Hypoxic Tumor Microenvironment in Glioblastoma: Paired Comparison Between Newly Diagnosed and Recurrent Glioblastomas.

Background: Previously, we reported that bevacizumab (Bev) produces histological and neuroradiographic alterations including changes in tumor oxygenation, induction of an immunosupportive tumor microenvironment, and inhibition of stemness. To confirm how those effects vary during Bev therapy, paired samples from the same patients with newly diagnosed glioblastoma (GBM) who received preoperative neoadjuvant Bev (neoBev) were investigated with immunohistochemistry before and after recurrence. Methods: Eighteen samples from nine patients with newly diagnosed GBM who received preoperative neoBev followed by surgery and chemoradiotherapy and then autopsy or salvage surgery after recurrence were investigated. The expression of carbonic anhydrase 9 (CA9), hypoxia-inducible factor-1 alpha (HIF-1α), nestin, and Forkhead box M1 (FOXM1) was evaluated with immunohistochemistry.For comparison between neoBev and recurrent tumors, we divided the present cohort into two groups based on neuroradiographic response: good and poor responders (GR and PR, respectively) to Bev were defined by the tumor regression rate on T1-weighted images with gadolinium enhancement (T1Gd) and fluid-attenuated inversion recovery images. Patterns of recurrence after Bev therapy were classified as cT1 flare-up and T2-diffuse/T2-circumscribed. Furthermore, we explored the possibility of utilizing FOXM1 as a biomarker of survival in this cohort. Results: A characteristic "pseudo-papillary"-like structure containing round-shaped tumor cells clustered adjacent to blood vessels surrounded by spindle-shaped tumor cells was seen only in recurrent tumors. Tumor cells at the outer part of the "pseudo-papillary" structure were CA9-positive (CA9+)/HIF-1α+, whereas cells at the inner part of this structure were CA9-/HIF-1α+ and nestin+/FOXM1+. CA9 and HIF-1α expression was lower in T1Gd-GR and decreased in the "T2-circumscribed/T2-diffuse" pattern compared with the "T1 flare-up" pattern, suggesting that tumor oxygenation was frequently observed in T1Gd-GR in initial tumors and in the "T2-circumscribed/T2-diffuse" pattern in recurrent tumors. FOXM1 low-expression tumors tended to have a better prognosis than that of FOXM1 high-expression tumors. Conclusion: A "pseudo-papillary" structure was seen in recurrent GBM after anti-vascular endothelial growth factor therapy. Bev may contribute to tumor oxygenation, leading to inhibition of stemness and correlation with a neuroimaging response during Bev therapy. FOXM1 may play a role as a biomarker of survival during Bev therapy.

Radiological features of human papillomavirus-related multiphenotypic sinonasal carcinoma: systematic review and case series.

PURPOSE: To comprehensively summarize the radiological characteristics of human papillomavirus (HPV)-related multiphenotypic sinonasal carcinomas (HMSCs). METHODS: We reviewed the findings for patients with HMSCs who underwent computed tomography (CT) and/or magnetic resonance imaging (MRI) and included nine cases from nine publications that were identified through a systematic review and three cases from our institution. Two board-certified radiologists reviewed and evaluated the radiological images. RESULTS: The locations in almost all cases included the nasal cavity (11/12, 91.7%). The involved paranasal sinuses included the ethmoid sinus (6/12, 50.0%) and maxillary sinus (3/12, 25.0%). The mean long diameter of the tumors was 46.3 mm. The margins in 91.7% (11/12) of the cases were well-defined and smooth. Heterogeneous enhancement on contrast-enhanced CT, heterogeneous high signal intensities on T2-weighted images and heterogeneous enhancement on gadolinium-enhanced T1-weighted images were noted in 2/2, 5/5, and 8/8 cases, respectively. Mean apparent diffusion coefficient values in two cases of our institution were 1.17 and 1.09 × 10-3 mm2/s. Compressive changes in the surrounding structures were common (75%, 9/12). Few cases showed intraorbital or intracranial extension. None of the cases showed a perineural spread, neck lymph node metastasis, or remote lesions. CONCLUSIONS: We summarized the CT and MRI findings of HMSCs. Knowledge of such characteristics is expected to facilitate prompt diagnosis and appropriate management.

Two entities in pulmonary nodules of a diabetic patient receiving corticosteroid therapy for bullous pemphigoid: an autopsy case report.

BACKGROUND: Invasive pulmonary aspergillosis (IPA) is a serious complication occurring in immunocompromised patients, who often show multiple nodular lesions with or without cavitation. Due to high mortality and poor prognosis, the earlier detection and initiation of treatment are needed, while the definitive diagnosis is often difficult to make in clinical settings. Septic pulmonary embolism (SPE) is a complication that occurs in patients with bloodstream infections (e.g., infectious endocarditis). Patients with SPE also present with multiple nodules, nodules with or without cavitation, which are quite similar to the findings of IPA. We herein report an autopsy case that showed multiple nodules due to IPA and infectious endocarditis-related SPE. CASE: A 69-year-old man receiving maintenance hemodialysis due to diabetic nephropathy was admitted with worsening skin rash due to bullous pemphigoid and toxic epidermal necrolysis. He was treated with intravenous methylprednisolone followed by an increased dose of oral prednisolone. On the 6th week of admission, he was diagnosed with infectious endocarditis after the isolation of Corynebacterium in blood samples, with a nodule lesion with cavitation in the right lung. Intravenous vancomycin was initiated. After antibacterial treatment, the nodules in the right lung gradually diminished, whereas a nodule with cavitation in the left lung emerged. The nodule in the left lung showed rapid growth along with elevation of serum ß-D-glucan and galactomannan antigen. Despite starting treatment with antifungal agents, he died from respiratory failure. An autopsy revealed Groccott staining-positive aspergillus in the left lung, but not in the right lung. We found fibrosis with mitral valve vegetation, indicating a recovery from infectious endocarditis. CONCLUSION: Although similar features of nodules with cavitation on CT imaging were shared with SPE and IPA, this case demonstrated that these heterogeneous diseases can occur within the lungs and the distinctly different transitions of CT imaging are helpful for suspecting the presence of multiple pathogeneses.

GFAP variant p. Tyr366Cys demonstrated widespread brain cavitation in neonatal Alexander disease.

Alexander disease is a rare form of leukodystrophy caused by heterozygous mutations in the gene encoding glial fibrillary acidic protein (GFAP). Brain cavitation in the white matter, predominantly distributed in the frontal periventricular area, has been described in some cases. Here, we present a case of a 1-year-old boy with neonatal Alexander disease caused by the p. Tyr366Cys GFAP variant, with rapid and widespread white matter cavitation. This case broadens the radiological spectrum of Alexander disease and suggests a possible genotype-phenotype correlation between the p. Tyr366Cys variant and cavitation.

Pathological Changes in Axillary Hyperhidrosis and Axillary Osmidrosis Induced by Microwave Treatment: Comparison of Single- and Double-Pass Irradiation.

BACKGROUND AND OBJECTIVES: To analyze histopathological changes and degree of damage to the axillary tissue due to single- and double-pass irradiation therapy using a microwave energy-based device. STUDY DESIGN/MATERIALS AND METHODS: We included 15 axillary hyperhidrosis and axillary osmidrosis patients who received microwave irradiation therapy between March 2017 and March 2019. Ten patients underwent single-pass irradiation and five underwent double-pass irradiation, after which skin samples were collected from the right and left axillae for pathological analysis. Samples were taken in a consistent manner from Patient 6 onwards and a comparative study of five single-pass and five double-pass patients was conducted (n = 10). RESULTS: Histopathological analysis showed destruction and fibrosis in addition to necrosis and damage to the adipose tissue in apocrine and eccrine sweat glands. In the superficial microvasculature, blood vessel wall damage and thrombus formation were observed as well as damage in the hair follicles and hair bulbs. No obvious damage was observed in the epidermis and nerves. The amount of damage to sweat glands was higher in patients undergoing double-pass instead of single-pass irradiation. CONCLUSION: From a histopathological point of view, microwave energy-based irradiation therapy can be considered efficient, as there was no damage to epidermis and nerves and favorable destruction of apocrine and eccrine glands. As the amount of damaged sweat glands was higher after double-pass irradiation, it can be considered more effective than single-pass irradiation. Lasers Surg. Med. © 2021 Wiley Periodicals LLC.

Spontaneously changing MRI findings of primary central nervous system vasculitis: A case report.

Spontaneously disappearing lesions on magnetic resonance imaging (MRI) has been described in primary central nervous system lymphoma. In this case, we report our experience of spontaneously changing MRI findings of primary central nervous system vasculitis (PCNSV) confirmed histopathologically. A 69-year-old man presented with sudden unsteady gait. Fluid-attenuated inversion recovery (FLAIR) images showed high-intensity areas in the right deep white matter. Contrast-enhanced T1-weighted MRI demonstrated a nodular lesion in the white matter of the left occipitoparietal lobe. On repeat MRI 7 days later, FLAIR hyperintense lesions had spontaneously disappeared and contrast-enhanced lesions had progressed, with new contrast lesions in the right corpus callosum. Repeat MRI 14 days after admission demonstrated contrast-enhancing lesions either increased or decreased in intensity in both occipitoparietal lobes. Contrast-enhancing lesions were therefore biopsied. Histopathological examination revealed vasculitis with fibrinoid necrosis. PCNSV was diagnosed without any signs of inflammation in blood vessels other than cerebral blood vessels. Spontaneously changing MRI findings may play an important role in diagnosing PCNSV.

Laparoscopic resection of a neuroendocrine tumor that almost fully replaced tailgut cysts: a case report.

BACKGROUND: Neuroendocrine tumors (NETs) originate from neuroendocrine cells, which are found throughout the body. NETs occur principally in the gastrointestinal tract (approximately 65%) and bronchopulmonary tract (approximately 25%) but rarely occur in the presacral space. Aside from primary and metastatic lesions, there have been reports of NETs occurring in the presacral space arising from tailgut cysts, teratomas, and imperforate anus. We herein report a rare case of laparoscopic resection of a NET in the presacral space, which almost fully replaced tailgut cysts. CASE PRESENTATION: A 68-year-old woman was referred to our hospital for surgery of a right inguinal hernia, but preoperative computed tomography revealed an asymptomatic 43-mm mass in the presacral space. Magnetic resonance imaging showed a multilocular solid mass with clear boundaries and a slightly high signal intensity on T1- and T2-weighted images. Positron emission tomography showed 18F-fluorodeoxyglucose uptake. Thus, we suspected a malignant tumor and performed laparoscopic resection to obtain a definitive diagnosis. Macroscopically, the tumor was 43 mm in size with clear boundaries, and the cut surface was a gray-white solid component. Histopathological findings revealed that the tumor was composed of relatively uniform cells with fine chromatin, with round to oval nuclei arranged in solid, trabecular, or rosette-like growth patterns. Small cysts lined with stratified squamous epithelium and columnar epithelium were observed along with solid components of the tumor, which is a feature of tailgut cysts. Therefore, the final diagnosis was NET Grade 1 arising from tailgut cysts. No recurrence was observed within 1 year after surgery. CONCLUSIONS: We performed en bloc laparoscopic resection of a NET arising from tailgut cysts in the presacral space without injury. In cases of a solid lesion in the presacral space, not only the primary disease but also the pathological condition with tissue transformation and replacement should be considered, as in this case.

Differentiating between glioblastomas with and without isocitrate dehydrogenase gene mutation by findings on conventional magnetic resonance images.

Various studies using advanced techniques have estimated the isocitrate dehydrogenase (IDH) gene mutation status in glioblastoma (GBM) from preoperative images. However, it is important to be able to predict mutation status using conventional MRI, which is more widely used in clinical practice. In this study, we examined the features of GBM with and without IDH gene mutation on conventional MRI. Twenty-three patients with GBM in whom IDH gene mutation status had been pathologically and molecularly confirmed in tumor specimens were included. The cases were divided into an IDH-wildtype group (n = 17) and an IDH-mutant group (n = 6). We retrospectively compared the following imaging parameters between the two groups: tumor location (superficial or deep), borders on T2-weighted images (regular or irregular), borders of enhancing lesions (regular or irregular), number of lesions showing contrast enhancement (solitary or multiple), presence or absence of intralesional bleeding, and presence or absence of a low-grade glioma in the background around the enhancing lesion. IDH-wildtype tumors were significantly more likely to be superficial than were IDH-mutant tumors (p < 0.05). Enhancing lesions in the IDH-wildtype group were less likely to have an irregular border (p = 0.059). Low-grade glioma was a background lesion in 5 patients (83.3%) in the IDH-mutant group and 9 (52.9%) in the IDH-wildtype group. The IDH mutation status is likely to be wildtype in patients with superficial GBM in which the enhancing lesion has a regular border and when low-grade glioma is not found as a background lesion on MRI.

Novel Characteristic Skull Magnetic Resonance Imaging Features Associated With Meningioma.

OBJECTIVE: Meningioma-related skull magnetic resonance imaging findings other than hyperostosis are not widely recognized. We evaluated the novel findings of the skull adjacent to meningiomas. METHODS: Records from patients with meningiomas located adjacent to the skull on magnetic resonance imaging (n = 32) were included. Three skull findings (intramedullary prominent vessel, intramedullary enhancement, intramedullary T2-hyperintensity) and the widely known hyperostosis were retrospectively visually assessed. The frequency of these 3 findings and the relevance to each other, and their relationships with hyperostosis, size, length adjacent to the skull, and relative signal intensity of the meningioma were examined. RESULTS: The incidence of the three findings was 46.88%, 53.13%, and 62.5%, respectively, and that of hyperostosis was 46.88%. Each association involving the findings was strong, and they were significantly related to the size and length. CONCLUSIONS: Intramedullary prominent vessel, intramedullary enhancement, and intramedullary T2-hyperintensity may be novel characteristic skull findings associated with meningioma.

A rare solitary fibrous tumor in the ischiorectal fossa: a case report.

BACKGROUND: A solitary fibrous tumor (SFT) is a rare mesenchymal tumor that occurs mostly in pleural sites, and an SFT occurring in the ischiorectal fossa is extremely rare. Because of the rarity, there are few reports detailing an SFT in the ischiorectal fossa. CASE PRESENTATION: A pararectal tumor was incidentally found in a 42-year-old man during a routine medical examination. The patient had no symptoms and no previous medical history. In the physical examination, a smooth-margined and hard elastic mass was felt, and in a digital rectal examination, the rectal mucosa appeared normal. A computed tomography (CT) scan showed a 5-cm, well-defined, solid mass in the left ischiorectal fossa. Contrast-enhanced CT in the early phase showed intense heterogeneous enhancement that persisted during the delayed phase. T2-weighted images of magnetic resonance imaging yielded heterogeneous intermediate and low signal intensity. Intense arterial enhancement suggested a hypervascular nature, and persistent delayed enhancement and low signal bands on T2-weighted images suggested a fibrous component of the mass. An SFT was suspected. Most SFTs are benign but have malignant potential. Our patient did not hope for surgery if the tumor was benign; therefore, an ultrasound-guided transperineal core needle biopsy was performed to decide on a treatment strategy. Microscopic examination showed tumor cells appearing as spindle and fibroblast-like cells within a collagenous stroma. Immunohistochemistry identified CD34 and vimentin, supporting the diagnosis of an SFT. The patient consented to excision of the mass. He was placed in a prone jackknife position, and the tumor was removed transperineally using a posterior approach (modified Kraske procedure). The levator ani muscle, external sphincter muscles, and rectum were not involved and separated from the tumor. The tumor was successfully resected en bloc with no complications. Five uneventful days post surgery, the patient was discharged. There was no local recurrence during the year following surgery. CONCLUSION: Imaging findings reflect the tissue characterization such as hypervascularity and fibrous nature of SFTs. We have presented a rare case of an SFT in the ischiorectal fossa with useful imaging findings for diagnosis, treatment strategy, and successful surgical removal using a posterior approach.

P-Tau and Subunit c Mitochondrial ATP Synthase Accumulation in the Central Nervous System of a Woman with Hurler-Scheie Syndrome Treated with Enzyme Replacement Therapy for 12 Years.

We report an autopsy case of a woman with mucopolysaccharidosis type I (MPS I) Hurler-Scheie syndrome who was treated with enzyme replacement therapy (ERT) for 12 years. This was the first case of MPS I treated with ERT in Japan. Pathological analysis showed no glycosaminoglycan accumulation in the liver and spleen as a result of long-term ERT, although severe aortic stenosis, diffuse intimal hyperplasia of the coronary artery, and fibrous hypertrophy of the endocardium were observed. Additionally, we detected subunit c mitochondrial ATP synthase (SCMAS) accumulation and mild tauopathy (hyperphosphorylated tau or p-tau, both 3-repeat and 4-repeat tau accumulation) in the same area of the cerebral limbic system and central gray matter of the mid brain and pons. Tauopathy is an important pathological finding in Alzheimer's disease and other neurodegenerative disorders; however, in MPS I, it is unclear whether tauopathy is a primary or secondary phenomenon. Thus, in this report, we describe pathological accumulation of p-tau and SCMAS in the context of MPS I and discuss the mechanisms and importance of these findings in the pathogenesis of MPS I.