小林 一博

Abstract Purpose To evaluate the efficacy of MRI findings for differentiating between ovarian metastasis from stomach cancer (OMSC) and colorectal cancer (OMCC). Methods Twenty-six patients with histopathologically proven ovarian metastasis (n = 8 with 12 OMSCs and n = 18 with 25 OMCCs) were enrolled in the study. All patients had undergone pelvic MRI before surgery. We retrospectively reviewed MRI findings and compared them between the two pathologies. The black scrunchie sign was defined as a thick (> 5 mm) and lobulated hypointense rim (> 180°) with central hyperintense areas on T2-weighted images. Results Predominantly solid lesions (100% vs. 20%, p < 0.01), black scrunchie sign (33% vs. 0%, p < 0.01), and flow void (67% vs. 20%, p < 0.01) were frequently observed in OMSCs than in OMCCs. The signal intensity ratio of solid components on T2-weighted images (3.30 ± 0.70 vs. 2.52 ± 0.77, p < 0.01) and gadolinium-enhanced T1-weighted images (2.21 ± 0.57 vs. 1.43 ± 0.32, p < 0.01) were significantly higher in OMSCs than in OMCCs. Furthermore, hyperintense areas within cystic components on T1-weighted images (71% vs. 18%, p < 0.01) and stained-glass appearance (44% vs. 0%, p < 0.01) were frequently observed in OMCCs than in OMSCs. Conclusion The black scrunchie sign was only observed in OMSCs. OMSCs always exhibited predominantly solid lesions and had higher signal intensity of solid components on T2- and gadolinium-enhanced T1-weighted images. OMCCs usually presented as cystic lesions, usually accompanied by hyperintense areas within the cystic components on T1-weighted images.

OBJECTIVE: Transoral surgery for early-stage pharyngeal and laryngeal cancer provides good local control and is less invasive than external incisions. Postoperative pathological findings are considered the most important indicators for determining postoperative treatment, but detailed criteria have not been established. In this study, we evaluated the impact of postoperative pathological findings on prognosis of patients undergoing transoral surgery. METHODS: This study included patients with oropharyngeal, hypopharyngeal, and supraglottic cancer who underwent transoral surgery at Gifu University Hospital from April 2016 to December 2023. Resection margins were pathologically evaluated with horizontal and vertical margins, and vascular invasion was evaluated in three categories: lymphatic invasion, venous invasion, and perineural invasion. The correlation between each postoperative pathological finding and prognosis was evaluated. RESULTS: A total of 70 cases were assessed in this study. Cases of horizontal margin positive were 38.6 %, and cases of vertical margin positive were 27.1 %. Prognoses were comparable to previous reports. Despite the high margin positive rate, the 5-year overall survival rate was 77.1 %. The 5-year disease-specific survival rate was 89.7 %, and the 5-year local control rate was 85.3 %. Notably, when evaluated by margin direction, cases with positive horizontal margins had significantly worse prognoses. Although no significant correlation was found between vascular invasion and prognosis, cases of venous invasion tended to have a higher local recurrence rate. CONCLUSION: This study suggests that transoral surgery has good prognosis despite a high positive-margin rate. However, detailed criteria for additional treatment have not been developed, and further case accumulation is required. Intriguingly, positive horizontal margins are correlated with significantly worse prognosis. This result may be related to a high risk of multiple cancers, and careful follow-up after surgery is recommended.

PURPOSE: The present study aimed to investigate CT imaging features, pathological findings, and prognosis in patients with thyroid hemiatrophy (THA) associated with papillary thyroid carcinoma (PTC). METHODS: This retrospective study included 225 patients with histopathologically proven PTC treated by surgical resection who underwent preoperative CT scanning. On CT images, THA was defined as thyroid parenchymal hemiatrophy on the ipsilateral side of PTC. CT findings, overall survival, and disease-free survival were compared between patients with and without THA. Pathological findings were also assessed in PTCs with and without THA. RESULTS: THA was observed in 35 of 225 (16%) patients with PTC. Atrophic thyroid parenchyma was observed in the right lobe of 20 patients (57%) and in the left lobe of the remaining 15 patients (43%). With respect to the solid components within PTCs, contrast-enhanced CT attenuation (114.2 ± 18.2 vs. 126.7 ± 31.3 HU; p < 0.05) and CT attenuation change for contrast-enhanced CT minus unenhanced CT (60.2 ± 18.1 vs. 72.3 ± 31.0 HU; p < 0.05) were significantly lower in PTCs with THA than in those without THA. Histopathologically, almost all PTCs with THA (97%) had keloid-like collagen, which is broad bundles of hypocellular collagen with bright eosinophilic hyalinization, typically observed in keloid. However, no significant differences were observed in the prognosis between the two groups. CONCLUSION: THA was occasionally observed in patients with PTC. Weak contrast-enhancement was distinct characteristic of PTC patients with THA, which is probably caused by keloid-like collagen.

The purpose of this study was to investigate the utility of reconstructed CT images perpendicular to the artery for assessing arterial involvement from pancreatic cancer and compare the interobserver variability between it and the current diagnostic imaging method. This retrospective study included patients with pancreatic cancer in the pancreatic body or tail who underwent preoperative pancreatic protocol CT and distal pancreatectomy. Five radiologists used axial and coronal CT images (current method) and perpendicular reconstructed CT images (proposed method) to determine if the degree of solid soft-tissue contact with the splenic artery was ≤180° or &gt;180°. The generalized estimating equations were used to compare the diagnostic performance of solid soft-tissue contact &gt;180° between the current and proposed methods. Fleiss’ ĸ statistics were used to assess interobserver variability. The sensitivity and negative predictive value for diagnosing solid soft-tissue contact &gt;180° were higher (p &lt; 0.001 for each) and the specificity (p = 0.003) and positive predictive value (p = 0.003) were lower in the proposed method than the current method. Interobserver variability was improved in the proposed method compared with the current method (ĸ = 0.87 vs. 0.67). Reconstructed CT images perpendicular to the artery showed higher sensitivity and negative predictive value for diagnosing solid soft-tissue contact &gt;180° than the current method and demonstrated improved interobserver variability.

BACKGROUND: Transient abnormal myelopoiesis (TAM) is characterized by leukocytosis with increased circulating megakaryoblasts that harbor N-terminal truncating mutations in the GATA1 gene. Approximately 10% of affected patients experience early death. OBSERVATIONS: A 2-month-old boy with Down syndrome was diagnosed with TAM and followed without treatment. Although the blasts in the peripheral blood disappeared, liver failure progressed. A pathological examination revealed liver fibrosis, and double-immunostaining for full-length GATA1 and CD42b identified megakaryocytes with a GATA1 mutation. CONCLUSIONS: This simple and cost-effective method can be applied in routine practice to detect TAM blasts during assessment in a TAM crisis.

PURPOSE: This study aimed to determine the MRI features of sporadic/simple lymphoepithelial cyst (SLEC) of the parotid gland. METHODS: Ten patients (seven men, three women; mean age, 60 years; age range, 38-77 years) with histopathologically and clinically proven SLEC of the parotid gland who underwent MRI before surgical resection were enrolled in this study. No enrolled patient had HIV infection or Sjögren syndrome. MRI findings of SLEC were retrospectively assessed. RESULTS: We confirmed 10 SLECs larger than 10 mm with a mean maximum diameter of 26.6 mm (range, 12-42 mm). Nine patients (90%) had a single cyst, and one (10%) had a large cyst accompanied by small cysts (<10 mm) within the ipsilateral parotid gland. Eight SLECs (80%) were unilocular, and two (20%) were bilocular, with complete septa. Among seven SLECs (70%) with internal septa, five unilocular SLECs (50%) had incomplete septa. Six SLECs (60%) had eccentric cyst wall thickening, and five (50%) were surrounded by small solid nodules that were isointense relative to lymph node. On T1-weighted images, all cyst contents were homogeneously hyperintense relative to cerebrospinal fluid. CONCLUSION: SLECs of the parotid gland are usually single unilocular lesions. Internal septa, eccentric cyst wall thickening, and small solid nodules surrounding the lesion were often observed. Cyst contents are always homogeneously hyperintense on T1-weighted images.

Objectives: This study aimed to evaluate the efficacy of using MRI findings to differentiate superficial spindle cell lipomas (SCLs) from atypical lipomatous tumor/well-differentiated liposarcomas (ALT/WDLs). Methods: This study included 12 patients with histopathologically proven superficial SCLs and 11 with ALT/WDLs. MRI findings for both pathologies were retrospectively reviewed and compared between the two pathologies. Results: The neck, upper back, and shoulder regions were more frequent locations of SCLs than of ALT/WDLs (100% vs 55%, p &lt; 0.05), whereas no significant differences were observed in age and sex. The median maximum diameter of the lesion was smaller in SCLs than in ALT/WDLs (44 mm [IQR: 35–63] vs 102 mm [IQR: 86–119], p &lt; 0.05). On T₁-weighted images, non-fatty area was more frequently observed in SCLs than in ALT/WDLs (73% vs 25%, p &lt; 0.05), and the median rate of non-fatty area was larger in SCLs than in ALT/WDLs (7.5% [IQR: 1.0–53] vs 0% [IQR: 0–0.2], p &lt; 0.05). On fat-suppressed T₂-weighted images, a solid hyperintense area was more frequently observed in SCLs than in ALT/WDLs (83% vs 27%, p &lt; 0.05). Conclusion: The maximum diameter, non-fatty area on T₁-weighted images, and solid hyperintense area on fat-suppressed T₂-weighted images were useful imaging features for differentiating superficial SCLs from ALT/WDLs. Advances in knowledge: In superficial lipomatous tumors, small tumor size and non-fatty solid area were valuable findings for diagnosing SCLs..

Abstract Sometimes, radiologists encounter malignant skin tumors (MSTs) during image interpretation. As MSTs require different clinical management modalities for each histological subtype, accurate preoperative diagnosis is essential. The histological subtypes of MST can be easily assessed by visual inspection or biopsy. Therefore, the significant role of radiological imaging in MSTs is to evaluate the extent of local invasion, nodal involvement, and distant metastasis, and the histological estimation of MSTs by radiological imaging has not been reported until a few years ago. However, recent studies have revealed characteristic radiological features for differential diagnosis of MSTs, such as configuration, intratumoral homogeneity, signal intensity, cyst formation, and hemorrhage. Other important clinical data for determining the histological subtype of MST include age, gender, and site of occurrence. MSTs can be categorized as epidermal, melanocytic, adnexal, and mesenchymal tumors based on the origin and have distinctive characteristics. Hence, this review article was designed to describe the clinical and radiological features of MSTs.

PURPOSE: This study aimed to describe the MRI findings of epithelial-myoepithelial carcinoma (EMC) of the parotid gland. MATERIALS AND METHODS: Seven patients (four males and three females) aged 40-86 years (mean age, 64 years) with histologically proven EMC of the parotid gland who underwent surgical resection after preoperative MRI were enrolled. MRI images were retrospectively reviewed and contrasted with pathological findings. RESULTS: Five patients (71%) had predominantly solid lesions, and two (29%) had predominantly cystic lesions. All seven lesions had well-demarcated margins and capsules without the invasion of adjacent structures. The capsules were incomplete in five lesions (71%) and complete in two (29%). Four lesions (57%) exhibited a multinodular structure with internal septa. Cystic components were observed in three lesions (43%). On T1-weighted images, the solid components were frequently homogeneous (5/7, 71%), and demonstrated isointensity in five lesions (71%) and hypointensity in two (29%) relative to the spinal cord. On T2-weighted images, the solid components were usually heterogeneous (6/7, 86%), and demonstrated hyperintensity in five lesions (71%) and isointensity in two (29%) relative to the spinal cord. The mean apparent diffusion coefficient value of the solid components was 0.967 × 10-3 mm2/s. CONCLUSION: Parotid gland EMCs usually appeared as predominantly solid lesions with well-demarcated margins and capsules. A multinodular structure with internal septa was characteristics of EMCs.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome has recently been described as an autoinflammatory disease associated with severe adult-onset inflammatory manifestations. The various clinical manifestations include recurrent high-grade fever, neutrophilic dermatoses, cutaneous vasculitis, chondritis of the ear and nose, pulmonary infiltrates, cytopenia, uveitis, gastrointestinal pain or inflammation, aortitis, hepatosplenomegaly, and hematological disorders. VEXAS syndrome is caused by somatic mutations of the ubiquitin-like modifier activating enzyme 1 (UBA1) gene in myeloid-lineage cells. It is characterized by vacuolated myeloid and erythroid progenitor cells seen by bone marrow biopsy. We report the case of a 64-year-old Japanese man with VEXAS syndrome. At age 63, he was referred to us with a recurrent erythema on the hands associated with a general fever of 38-40°C that had persisted for 4 or 5 days and had recurred about once a month for a year. The skin rash appeared 2 or 3 days after the onset of each fever episode. Computed tomography (CT) of the chest revealed bilateral hilar lymphadenopathy (BHL), and the mediastinal lymph nodes were swollen. Sarcoidosis was suspected but was ruled out by several tests. Laboratory examinations showed elevated inflammatory markers. Bone marrow examination showed the vacuolization of myeloid precursor cells. A skin biopsy revealed dense dermal, predominantly perivascular, infiltrates. These consisted of mature neutrophils admixed with myeloperoxidase-positive CD163-positive myeloid cells, lymphoid cells and eosinophils. Sequencing analysis identified the somatic UBA1 variant c.122T > C, which results in p.Met41Thr. Treatment with oral prednisone (15 mg/day) and monthly intravenous tocilizumab injections (400 mg) completely resolved the symptoms. Neutrophils are a major source of reactive oxygen species, and the present case demonstrated numerous neutrophilic infiltrates. We hypothesize that the patient might have had elevated derivatives of reactive oxygen metabolites (d-ROMs). d-ROM quantification is a simple method for detecting hydroperoxide levels, and clinical trials have proven it useful for evaluating oxidative stress. In this study, we measured serum d-ROM before and after oral prednisone and tocilizumab treatment. The levels decreased significantly during treatment.

Background: In an earlier monocentric study, we have developed a novel non-invasive test system for the prediction of renal allograft rejection, based on the detection of a specific urine metabolite constellation. To further validate our results in a large real-world patient cohort, we designed a multicentric observational prospective study (PARASOL) including six independent European transplant centers. This article describes the study protocol and characteristics of recruited better patients as subjects. Methods: Within the PARASOL study, urine samples were taken from renal transplant recipients when kidney biopsies were performed. According to the Banff classification, urine samples were assigned to a case group (renal allograft rejection), a control group (normal renal histology), or an additional group (kidney damage other than rejection). Results: Between June 2017 and March 2020, 972 transplant recipients were included in the trial (1,230 urine samples and matched biopsies, respectively). Overall, 237 samples (19.3%) were assigned to the case group, 541 (44.0%) to the control group, and 452 (36.7%) samples to the additional group. About 65.9% were obtained from male patients, the mean age of transplant recipients participating in the study was 53.7 ± 13.8 years. The most frequently used immunosuppressive drugs were tacrolimus (92.8%), mycophenolate mofetil (88.0%), and steroids (79.3%). Antihypertensives and antidiabetics were used in 88.0 and 27.4% of the patients, respectively. Approximately 20.9% of patients showed the presence of circulating donor-specific anti-HLA IgG antibodies at time of biopsy. Most of the samples (51.1%) were collected within the first 6 months after transplantation, 48.0% were protocol biopsies, followed by event-driven (43.6%), and follow-up biopsies (8.5%). Over time the proportion of biopsies classified into the categories Banff 4 (T-cell-mediated rejection [TCMR]) and Banff 1 (normal tissue) decreased whereas Banff 2 (antibody-mediated rejection [ABMR]) and Banff 5I (mild interstitial fibrosis and tubular atrophy) increased to 84.2 and 74.5%, respectively, after 4 years post transplantation. Patients with rejection showed worse kidney function than patients without rejection. Conclusion: The clinical characteristics of subjects recruited indicate a patient cohort typical for routine renal transplantation all over Europe. A typical shift from T-cellular early rejections episodes to later antibody mediated allograft damage over time after renal transplantation further strengthens the usefulness of our cohort for the evaluation of novel biomarkers for allograft damage.

Giant cell glioblastoma (GCG) is a rare subtype of glioblastoma multiforme (GBM), and it often occurs in younger patients; however, its onset in children is extremely noticeable. A 7-year-old girl presented with a headache and restlessness. A giant tumor that was 7 cm in diameter was found by magnetic resonance imaging (MRI) in the left frontal lobe with intracranial dissemination. Because the tumor had extended to the lateral ventricles and occluded the foramen of Monro causing hydrocephalus, she underwent ventricular drainage and neuro-endoscopic biopsy from the left posterior horn of the lateral ventricle. The initial pathological diagnosis was an atypical teratoid/rhabdoid tumor (AT/RT). When the dissemination subsided after the first chemotherapy with vincristine, doxorubicin, and cyclophosphamide, she underwent the first tumor resection via a left frontal transcortical approach. After surgery, the second chemotherapy with ifosfamide, cisplatin, and etoposide was not effective for the residual tumor and intracranial dissemination. The second surgery via a transcallosal approach achieved nearly total resection leading to an improvement of the hydrocephalus. The definitive pathological diagnosis was GCG. Despite chemo-radiation therapy, the dissemination in the basal cistern reappeared and the hydrocephalus worsened. She was obliged to receive a ventriculo-peritoneal (VP) shunt and palliative care at home; however, her poor condition prevented her discharge. Ten months after admission, she died of tumor progression. The peritoneal dissemination was demonstrated by cytology of ascites. In conclusion, although unusual, pediatric GCG may be disseminated at diagnosis, in which case both tumor and hydrocephalus control need to be considered.

The legends of Figs. 1 and 3 in the published original version of the above article are incorrect.