Language：English   Publisher：Medicine United States  

RATIONALE: Clear cell meningioma (CCM) is a rare and aggressive subtype of meningioma, classified as Grade II by the World Health Organization due to its higher recurrence risk. While CCMs predominantly arise in the cerebellopontine angle, intraspinal cases, particularly those without dural attachment, are exceedingly rare and present diagnostic and surgical challenges. PATIENT CONCERNS AND DIAGNOSIS: We report a case of a 66-year-old woman who presented with a one-year history of pain and numbness radiating from the right buttock to the posterior thigh, along with intermittent claudication. Physical examination showed no motor deficits or significant neurological abnormalities. Magnetic resonance imaging revealed a 32 × 16 mm intradural mass at the L5 level with iso-intensity on both T1- and T2-weighted images and uniform contrast enhancement. The lesion was diagnosed as CCM based on pathological examination following tumor resection. INTERVENTIONS AND OUTCOMES: The patient underwent lumbar laminectomy and complete tumor resection. Intraoperatively, the tumor was observed to compress the cauda equina nerve but was nonadherent to the dura mater. A nerve fiber connected the tumor to surrounding neural structures, requiring partial nerve root resection for complete tumor removal. Postoperatively, the patient reported complete resolution of pain, numbness, and gait disturbance. The visual analogue scale scores for leg pain and numbness improved from 7.4 and 7.3 to 0, respectively. The Japanese Orthopaedic Association score improved from 22 to 28 (out of 29). Follow-up magnetic resonance imaging at 1 year showed no evidence of recurrence. LESSONS: CCM without dural attachment is a rare variant of meningioma that presents challenges in diagnosis and surgical management. Complete resection during the first surgery led to favorable outcomes in this case. Although the recurrence rate for this subtype is lower, long-term follow-up with regular imaging is recommended.

DOI： 10.1097/MD.0000000000043193

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Language：English   Publisher：Human Pathology Reports  

Sacrococcygeal teratomas (SCT) with malignant transformation to squamous cell carcinoma are extremely rare, with only few cases reported in the literature. Herein, we report on a case of a primary sacrococcygeal mature cystic teratoma with malignant transformation into a squamous cell carcinoma. A 53-year-old woman presented with dysuria and lower abdominal pain caused by a huge SCT. Computed tomography (CT), magnetic resonance imaging, and positron emission tomography/CT findings were suggestive of SCT with malignant transformation. The treatment involved robotic-assisted laparoscopic abdominoperineal resection and bilateral lymph node dissection. Histopathology revealed squamous cell carcinoma within the teratoma. Postoperatively, the patient underwent six cycles of paclitaxel-carboplatin chemotherapy and showed no signs of recurrence during the 1-year follow-up.

DOI： 10.1016/j.hpr.2025.300773

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Language：Japanese   Publisher：(公社)日本臨床細胞学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Cancer Science  

Undifferentiated pleomorphic sarcoma (UPS) has a favorable objective response rate to anti-PD-1 drugs compared with other sarcomas. Tertiary lymphoid structure (TLS) is a favorable prognostic factor and a biomarker for immune checkpoint inhibitors (ICIs). Nevertheless, there are limited data on the tumor microenvironment (TME) to support a good response to ICIs in sarcoma. Therefore, this study was conducted to investigate the impact of TLS on prognosis and TME. A total of 52 of UPS with wide resection were divided into intratumoral TLS, extratumoral TLS, and without TLS groups. Survival analysis and immunohistochemistry were performed to evaluate immune cells and immune checkpoint molecules, and multiplexed immunofluorescence was conducted to evaluate T-cell exhaustion among the three groups. TLS was detected in 34 cases (65%), including 23 intratumoral TLS (44%) and 11 extratumoral TLS (21%) cases. Patients with TLS had significantly longer overall survival than those without TLS (log rank p = 0.020). The intratumoral TLS group had a significantly higher number of immune cells and higher expression of PD-L1 and IDO-1 than the without TLS group. Progenitor-exhausted T cells were also observed in patients with UPS. In conclusion, these findings could help predict prognosis in patients with UPS. TLS was demonstrated to be a favorable prognostic factor in patients with UPS. Intratumoral TLS may be a biomarker for the response to ICIs, especially anti-PD-1 drugs.

DOI： 10.1111/cas.70018

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Language：English   Publisher：Pathology International  

NTRK-rearranged spindle cell neoplasm is a group of tumors characterized by NTRK1/2/3 gene fusion. Recently, tumors with other kinase fusion genes were reported to exhibit similar morphologies. Herein, we discuss an adult-onset soft tissue tumor with similar histologic patterns as kinase gene fusion-related tumors but with BRAF and NRAS mutations. A female in her 40s had a 40 mm tumor with an unclear border in the soft tissue of her foot joint. Short spindle-shaped tumor cell proliferation with abundant capillaries and collagen fiber bundles were observed. The tumor infiltrated the subcutaneous adipose tissue, exhibiting a lipofibromatosis-like pattern. Immunohistochemically, the tumor cells coexpressed CD34, S-100, and BRAF V600E. Whole-exome sequencing revealed BRAF p.V600E and NRAS p.Q61K mutations. Since BRAF activation occurs in BRAF fusion gene tumors and BRAF mutations, they could share a similar mechanism in tumorigenesis. This case suggests the further expansion of kinase-related spindle cell tumors.

DOI： 10.1111/pin.13499

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Cardio Oncology  

The advent of immune checkpoint inhibitors (ICIs) has significantly improved cancer treatment. With the increasing use of ICIs, ICI-related myocarditis has been recognized. However, an evidence-based therapeutic strategy has not been established because of the limited knowledge on ICI-related myocarditis. Here, we present four cases of ICI-related fulminant myocarditis (FM). Three of the four cases resulted in fatal outcomes despite aggressive treatment with mechanical circulatory support and immunosuppressive therapy with corticosteroids. Given the poor prognosis of ICI-FM, the establishment of rapid and adequate therapeutic interventions on the basis of clinical and pathological evaluation is imperative.

DOI： 10.1186/s40959-024-00288-0

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Cancer Cytopathology  

BACKGROUND: A standardized reporting system for bone and soft tissue tumor cytopathology has not yet been established. The objective of this study was to explore the potential utility of a classification modified from the Milan System for Salivary Gland Cytopathology and compared it with the upcoming World Health Organization (WHO) system for fine-needle aspiration of soft tissue lesions. METHODS: The authors reviewed 285 cytology cases of bone/joint (n = 173) and soft tissue (n = 112) lesions, scoring each within diagnostic categories. The results were compared with histologic diagnoses and the risk of malignancy (ROM) for each category, and diagnostic reliability was analyzed. RESULTS: All 285 cases were successfully classified into one of the following categories: nondiagnostic (6.3%), non-neoplastic (11.9%), atypia of uncertain significance (11.9%), benign neoplasm (5.6%), bone and soft tissue neoplasm of uncertain malignant potential (25.3%), suspicious for malignancy (1.4%), and malignant (37.5%). The ROM was 44.4% (eight of /18 cases) in nondiagnostic, 0% (zero of 34 cases) in non-neoplastic, 32.4% (11 of 34 cases) in atypia of uncertain significance, 0% (zero of 16 cases) in benign neoplasm, 16.7% (12 of 72 cases) in bone and soft tissue neoplasm of uncertain malignant potential, 75.0% (three of four cases) in suspicious for malignancy, and 100% (107 of 107 cases) in malignant categories. Using the WHO system, the proportion and ROM of the benign category (non-neoplastic and benign neoplasm) was 17.5% and 0%, respectively. Among benign and malignant lesions, the diagnostic accuracy, sensitivity, and specificity for detecting malignancy were 99.4%, 100%, and 98.0%, respectively. CONCLUSIONS: The modified Milan system as well as the WHO system may be a useful cytopathologic classification tool for both bone and soft tissue lesions.

DOI： 10.1002/cncy.22888

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Language：Japanese   Publisher：西日本整形・災害外科学会  

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Clinical Pathology  

AIMS: Liposarcoma is a malignant soft tissue tumour with adipocytic differentiation. Dedifferentiated liposarcoma (DDLS) and myxoid liposarcoma (MLS) are classified as high-grade liposarcomas. Lipid droplet-associated protein (also known as perilipin 1 (PLIN1)) is the predominant perilipin and has utility as a specific marker of adipogenic differentiation. Adipose differentiation-related protein (also known as adipophilin (ADRP)) is ubiquitously expressed in a range of tissues. High ADRP expression is reportedly a poor prognostic factor in several cancer types. However, no previous studies have examined the association between PLIN1 or ADRP expression and prognosis in sarcoma. This study therefore aimed to evaluate the association between PLIN1 or ADRP expression and prognosis in liposarcoma. METHODS: In total, 97 primary resection specimens (53 MLS and 44 DDLS) were examined in this study. PLIN1 and ADRP expression was evaluated by immunohistochemistry. Survival analyses were performed for MLS and DDLS. RESULTS: Of the 53 MLS specimens, 15 (28.3%) exhibited high PLIN1 expression. PLIN1 expression was not observed in DDLS specimens. High PLIN1 expression was significantly associated with increased disease-free survival (DFS) among patients with MLS (p=0.045). Distinct ADRP expression was observed in 13 of 53 (24.5%) MLS specimens and 5 of 44 (11.4%) DDLS specimens. High ADRP expression was associated with shorter overall survival (OS) in MLS (p=0.042) and DFS and shorter OS in DDLS (p=0.024 and p<0.001, respectively). CONCLUSIONS: PLIN1 and ADRP expression is associated with poor prognosis in high-grade liposarcoma.

DOI： 10.1136/jcp-2023-208814

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BACKGROUND: Intramuscular hemangioma is an uncommon benign tumor found mainly in the limbs of adolescents and young adults. The local recurrence rate is high, ranging from 30 to 50%, necessitating wide local excision of intercostal intramuscular hemangiomas. However, preoperative diagnosis of intramuscular hemangiomas is challenging. Herein, we report a rare case of an intramuscular hemangioma arising from the chest wall. CASE PRESENTATION: A healthy 29-year-old asymptomatic man was referred to our hospital after an abnormal shadow was observed on his chest radiography. Computed tomography and magnetic resonance imaging revealed a 30-mm-sized mass in the right second intercostal space. Neoplastic lesions, such as schwannomas or solitary fibrous tumors, were included in the preoperative differential diagnosis. Tumor resection was performed using video-assisted thoracoscopic surgery. The tumor, which had a smooth surface covered with parietal pleura, was dissected from the external intercostal muscle and costal bone. Postoperative histopathological examination revealed proliferation of spindle-shaped endothelial cells arranged in a capillary vascular structure accompanied by entrapped smooth muscle fibers, adipose tissue, and muscle vessels. The final diagnosis was an intramuscular hemangioma with negative surgical margins. There was no evidence of recurrence during the 1-year postoperative follow-up period. CONCLUSION: Intramuscular hemangiomas should be considered in the differential diagnosis of chest wall tumors, particularly in young people, owing to their potential for recurrence. Moreover, postoperative follow-up may be necessary for resected intramuscular intercostal hemangiomas.

DOI： 10.1186/s40792-024-02023-4

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Language：English   Publisher：(一社)日本癌学会  

Language：Japanese   Publisher：(一社)日本消化器外科学会  

症例は75歳の男性で,2020年進行胃癌に対して腹腔鏡下胃全摘術を施行した.病理診断で口側断端にリンパ管侵襲を認め非治癒切除と判断した.免疫染色検査でHER2[3+]であり,術後化学療法としてトラスツズマブ+カペシタビン+シスプラチン療法8コースとカペシタビン単剤療法を16コース施行した.術後約2年で左下肢の脱力感および転倒のため近医搬送となった.CTで転移性脳腫瘍を指摘され当院へ転院となった.その他の転移はなく,脳神経外科で開頭腫瘍摘出術を施行した.術後病理より胃癌の転移性腫瘍と判断した.トラスツズマブ投与後の頭蓋内単独再発例であったため,頭蓋内も含めた全身の治療効果を期待してニボルマブ単独投与を行い,術後6ヵ月現在無再発経過中である.今回,我々はHER2陽性進行胃癌の切除後に抗HER2療法を行い,その後に単独脳転移を呈した1例を経験したため報告する.(著者抄録)

Language：Japanese   Publisher：(公社)日本整形外科学会  

Language：Japanese   Publisher：日本消化器病学会-九州支部  

Language：Japanese   Publisher：(公社)日本整形外科学会  

Language：English   Publisher：Thoracic Cancer  

Tracheomediastinal fistula is a rare but life-threatening complication of cancer. We report a case of tracheomediastinal fistula induced by concurrent chemoradiotherapy in limited stage small cell lung cancer. Despite the treatment response, the metastatic paratracheal lymph node increased gradually during concurrent chemoradiotherapy, resulting in the occurrence of tracheomediastinal fistula and mediastinitis. Without any surgical intervention, the patient achieved successful recovery from mediastinitis through antibiotic treatment, although the tracheomediastinal fistula remained open. In this report, we also review previous studies of tracheomediastinal and bronchomediastinal fistulas and summarize the clinical features.

DOI： 10.1111/1759-7714.15270

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Virchows Archiv  

Currently, it is difficult to predict the prognosis of myxoid liposarcoma (MLS) in biopsy specimens. In this study, we determined whether nuclear morphology may be used to predict the prognosis of MLS in primary biopsy specimens. Two pathologists evaluated nuclear morphology using the modified WHO/ISUP and Fuhrman grades. Survival analyses were performed by grouping nuclear high- and low-grades. We examined 53 MLS cases, which included 29 (54.7%) male and 24 (45.3%) female patients with a median age of 46 years (interquartile range, 37 - 60). In total, 7 (13.2%) and 16 (30.2%) cases were assigned to the high nuclear grade group based on the modified WHO/ISUP and Fuhrman gradings, respectively. Survival analyses revealed a significantly worse disease-free survival in the high-grade group (hazard ratio (HR), 7.51; 95% confidence interval (CI), 2.67-21.1, p < 0.001 by the modified WHO/ISUP grading; HR, 4.45; 95% CI, 1.63-12.1, p = 0.001 by the modified Fuhrman grading). Moreover, the modified WHO/ISUP grade showed a significantly worse overall survival in the high-grade group (HR, 4.39; 95% CI, 1.04-18.6, p = 0.028), and the modified Fuhrman grade exhibited a similar, but not significant, trend. Our results indicate that nuclear morphology grading is a good predictor of patient prognosis at the time of biopsy in MLS. Even when cell density is sparse, treatment strategies should be carefully considered when individual tumor cells exhibit atypical nuclei.

DOI： 10.1007/s00428-024-03796-7

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Language：Japanese   Publisher：日本消化器病学会-九州支部  

Language：Japanese   Publisher：西日本整形・災害外科学会  

Language：Japanese   Publisher：(公社)日本皮膚科学会  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Acta Dermato Venereologica  

DOI： 10.2340/actadv.v103.4407

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Virchows Archiv  

Intimal sarcoma is one of the most common and well-known primary malignant neoplasms of the aorta and heart. The authors reviewed cases of intimal sarcoma from histological, immunohistochemical and genetic perspectives. Twenty cases of intimal sarcoma were retrieved. Immunohistochemistry and FISH of MDM2 and PDGFRA genes were performed. All 20 tumours were composed of spindle-shaped, stellate, oval or polygonal tumour cells with irregular hyperchromatic nuclei arranged in a haphazard pattern, accompanied by nuclear pleomorphism and frequent mitotic figures. Other histological findings were as follows: abnormal mitosis in 10 cases (50%), necrosis in 15 cases (75%), myxoid stroma in 12 cases (60%), cartilaginous formation in 1 case (5%), haemorrhage in 12 cases (60%) and fibrinous deposition in 14 cases (70%). The tumours were positive for MDM2 in 16 cases (80%), ERG in 4 cases (20%), alpha-smooth muscle actin in 6 cases (30%), desmin in 5 cases (25%) and AE1/AE3 in 4 cases (20%). Immunohistochemical positivity was focal in each case. Loss of H3K27me3 expression was noted in 2 cases (10%). MDM2 and PDGFRA gene amplifications were detected in 11 cases (55%) and 1 case (5%), respectively. Fisher's exact test revealed a significant correlation between MDM2 gene amplification and myxoid stroma (p = 0.0194). No parameters showed any association with the anatomical location of the tumours. It was suggested that myxoid histology of intimal sarcoma may be associated with MDM2 gene amplification and that intimal sarcoma may be divided into myxoid and non-myxoid types.

DOI： 10.1007/s00428-022-03293-9

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Language：Japanese   Publisher：(一社)日本病理学会  

Histiocytic sarcoma(HS)は稀な疾患で、とりわけ結腸発生例は数例しか報告がない。今回我々は下行結腸に発生したHSの1例を報告する。症例は47歳女性。内視鏡検査で下行結腸に腫瘤を認め、CTでリンパ節と肝臓への転移が疑われた。腸重積を来しており、左側結腸切除術が施行された。腫瘍は軟らかく淡褐色充実性で、組織学的に上皮様または組織球様の異型細胞がびまん性に増殖し、血球貪食像もみられた。免疫染色で異型細胞はCD68とCD163に陽性を示した。以上の所見よりHSと診断した。(著者抄録)

Language：English   Publishing type：Research paper (scientific journal)  

Malignant peripheral nerve sheath tumor (MPNST) is a very aggressive peripheral nerve sheath-derived sarcoma, which is one of the most difficult tumors to diagnose due to its wide spectrum of histological findings and lack of specific immunohistochemical markers. Recently, it has been reported that losses of expression of H3K27me3 and H3K27me2 caused by PRC2 dysfunction may be useful diagnostic markers for MPNST, but there is no consensus on their clinicopathological significance. Here, we investigated the relationship between loss of H3K27 methylation and various parameters and clarified the clinicopathological significance of such loss. We analyzed the clinicopathological and immunohistochemical features in 84 MPNST cases. Complete losses of H3K27me3 and H3K27me2 were observed in 37 (44%) and 29 (35%) cases, respectively. Losses of H3K27me3 and H3K27me2 were significantly correlated with myogenic immunopositivity (H3K27me3 vs. desmin, P = 0.0051; H3K27me3 vs. myogenin, P = 0.0009; H3K27me2 vs. myogenin, P = 0.042). Meanwhile, there were significant correlations between preservation of immunohistochemical neurogenic markers and intact H3K27me3 and H3K27me2 (H3K27me3 vs. S-100 protein, P = 0.0019; H3K27me3 vs. SOX10, P = 0.014; H3K27me2 vs. S-100 protein, P = 0.0011; H3K27me2 vs. SOX10, P = 0.0087). In multivariate analysis, local recurrence, distant metastasis, high FNCLCC grade, and loss of SOX10 expression were independent prognostic factors for overall survival. H3K27me3 and H3K27me2 expression was retained in all 26 cases of rhabdomyosarcoma non-alveolar subtype. In conclusion, we suggest that H3K27me3 and H3K27me2 immunonegativity is useful but not definitive for diagnosing MPNST. Complete loss of H3K27 methylation may be involved in aggressive transdifferentiation from neural differentiation to skeletal muscle differentiation in MPNST.

DOI： 10.1007/s00428-021-03189-0.

Language：English   Publishing type：Research paper (scientific journal)  

Synovial sarcoma (SS) is a malignant soft tissue neoplasm harboring SS18-SSX fusion gene and is histologically characterized by spindle cells and epithelial components. Some investigations have demonstrated that desmoplastic reaction (DR) is an independent prognostic factor of cancers. However, it remains unknown whether DR is of predictive value for the prognosis of synovial sarcoma patients. Here, we reviewed the clinical and histological findings of 88 patients with SS. We defined DR as hyalinized collagenous structures and classified the degree of DR as follows: none, mild, moderate, and severe. Overall, 23 SS cases (24%) showed moderate or severe DR histologically. Statistically, the cases with moderate or severe degree of DR showed poorer prognosis than those with no or mild DR (local recurrence: P = 0.0059, distant metastasis: P = 0.0002, tumor death: P = 0.0382). The findings of the study suggest that the DR of synovial sarcoma could be an important prognostic factor.

DOI： 10.1016/j.prp.2021.153668.

Language：English   Publishing type：Research paper (scientific journal)  

Pathological review of cardiac amyloidosis using autopsy cases in a single Japanese institution.

DOI： 10.1016/j.prp.2021.153635.

Language：English   Publishing type：Research paper (scientific journal)  

Background: Undifferentiated pleomorphic sarcoma (UPS) is a sarcoma with a poor prognosis. A clinical trial, SARC028, revealed that treatment with anti-PD-1 drugs was effective against UPS. Studies have reported that UPS expresses PD-L1, sometimes strongly (≥ 50%). However, the mechanism of PD-L1 expression in UPS has remained unclear. CKLF-like MARVEL transmembrane domain containing 6 (CMTM6) was identified as a novel regulator of PD-L1 expression. The positive relationship between PD-L1 and CMTM6 has been reported in several studies. The aim of this study was thus to examine CMTM6 expression in UPS and evaluate the relationship between PD-L1 and CMTM6 in this disease.
Materials and methods: Fifty-one primary UPS samples were subjected to CMTM6 and PD-L1 immunostaining. CMTM6 expression was assessed using proportion and intensity scores. CMTM6 gene copy number was also evaluated using a real-time PCR-based copy number assay. We also analyzed the mRNA expression and copy number variation of PD-L1 and CMTM6 in The Cancer Genome Atlas (TCGA) data.
Results: TCGA data indicated that the mRNAs encoded by genes located around 3p22 were coexpressed with CMTM6 mRNA in UPS. Both proportion and intensity scores of CMTM6 positively correlated with strong PD-L1 expression (≥ 50%) (both p = 0.023). CMTM6 copy number gain increased CMTM6 expression. Patients with UPS with a high CMTM6 intensity score had a worse prognosis for overall survival.
Conclusions: UPS showed variation in CMTM6 copy number and CMTM6 expression. CMTM6 expression was significantly correlated with PD-L1 expression, especially with strong PD-L1 expression.

DOI： 10.1007/s00432-021-03616-4.

Language：English   Publishing type：Research paper (scientific journal)  

Whole slide imaging (WSI) is being increasingly used worldwide. Although previous studies have asserted the validity of WSI diagnosis, they have primarily targeted only small specimens and excluded cases requiring immunohistochemistry or special staining, such as lymphoma. The purpose of this study was to evaluate the accuracy of WSI diagnosis of lymphoma, for which 240 biopsies and resections of lymphoma cases were selected from the study set of lymphomas. All slides including H&E, immunohistochemical and special staining were digitized using a WSI image scanner. An experienced pathologist performed the WSI diagnoses, which were compared with original diagnoses based on light microscopic examinations. Discrepancy between the two interpretations were classified into three categories: concordance, minor discrepancy (no clinical significance), and major discrepancy (with clinical significance). Overall concordance between the light microscopic and WSI diagnosis was found in 223 cases (92.92%; 95%CI = 88.90-95.82), minor discrepancy in fifteen (6.25%; 95%CI = 3.54-10.10), and major discrepancy in two (0.83%; 95%CI = 0.10-2.98). Diagnosis of lymphoma using WSI appeared to be mostly accurate, suggesting that WSI may be a reliable technology for the diagnosis of lymphoma.

DOI： 10.1111/pin.12808.

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Language：Japanese   Presentation type：Oral presentation (general)  

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Language：Japanese   Presentation type：Oral presentation (invited, special)  

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Language：Japanese   Presentation type：Symposium, workshop panel (public)  

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Language：Japanese   Presentation type：Symposium, workshop panel (nominated)  

Language：Japanese   Presentation type：Oral presentation (general)  

Language：Japanese   Presentation type：Oral presentation (general)  

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