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Background: Pancreatic ductal occlusion can accompany pancreatic head cancer, leading to pancreatic exocrine insufficiency (PEI) and adverse effects on nutritional status and postoperative outcomes. We investigated its impact on nutritional status, body composition, and postoperative outcomes in patients with pancreatic head cancer undergoing neoadjuvant therapy (NAT). Methods: We analyzed 136 patients with pancreatic head cancer who underwent NAT prior to intended pancreaticoduodenectomy (PD) between 2015 and 2022. Nutritional and anthropometric indices (body mass index [BMI], albumin, prognostic nutritional index [PNI], Glasgow prognostic score, psoas muscle index, subcutaneous adipose tissue index [SATI], and visceral adipose tissue index) and postoperative outcomes were compared between the occlusion (n = 78) and non-occlusion (n = 58) groups, in which 61 and 44 patients, respectively, ultimately underwent PD. Results: The occlusion group showed significantly lower post-NAT BMI, PNI, and SATI (p = 0.011, 0.005, and 0.015, respectively) in the PD cohort. The occlusion group showed significantly larger main pancreatic duct, smaller pancreatic parenchyma, and greater duct–parenchymal ratio (p < 0.001), and these morphological parameters significantly correlating with post-NAT nutritional and anthropometric indices. Postoperative 3-year survival and recurrence-free survival (RFS) rates were significantly poorer (p = 0.004 and 0.013) with pancreatic ductal occlusion, also identified as an independent postoperative risk factor for overall survival (hazard ratio [HR]: 2.31, 95% confidence interval [CI] 1.08–4.94, p = 0.030) and RFS (HR: 2.03, 95% CI 1.10–3.72, p = 0.023), in multivariate analysis. Conclusions: Pancreatic ductal occlusion may be linked to poorer postoperative outcomes due to PEI-related malnutrition. [ABSTRACT FROM AUTHOR]

Background: Conversion surgery (CS) after chemotherapy is weakly recommended as a promising tool for improving prognoses in patients with unresectable gastric cancer. Moreover, several investigators have demonstrated the clinical efficacy of subtotal gastrectomy (sTG) with a small remnant stomach for the nutritional status and surgical outcome compared with total gastrectomy. Here, we report a patient with liver metastasis from human epidermal growth factor receptor 2 (HER2)-positive gastric cancer who underwent sTG and hepatectomy after trastuzumab-based chemotherapy. Case presentation: An 84-year-old male patient was diagnosed with HER2-positive gastric cancer with a single liver metastasis. He was treated with eight courses of trastuzumab in combination with S-1 and oxaliplatin as first-line chemotherapy. The primary tumor and liver metastasis shrank significantly. The metastatic liver lesion's reduction rate was 65%. According to the Response Evaluation Criteria in Solid Tumors, the patient had a partial response. Therefore, he underwent an sTG with D2 lymphadenectomy and partial hepatectomy of segment 2. Histopathological examination revealed a grade 3 histological response without lymph node metastases from the primary tumor. No viable cancer cells were observed in the resected liver specimens. The patient received adjuvant chemotherapy with S-1. The postoperative quality of life (QOL) evaluated using the Postgastrectomy Syndrome Assessment Scale-45 was maintained, and the patient was still alive 8 months after the CS without recurrence. Conclusions: An sTG with a small remnant stomach might be clinically useful for preventing a decline in QOL and improving prognoses in patients with stage IV gastric cancer after chemotherapy. [ABSTRACT FROM AUTHOR]

Background: Lymphatic metastasis is a major route of metastasis in distal cholangiocarcinoma (DCC). The present study aimed to elucidate the pattern of lymph node (LN) metastasis and the effectiveness of LN dissection and postoperative adjuvant chemotherapy in patients with DCC. Methods: Patients who underwent surgical resection with curative intent for DCC were enrolled. The nomenclature of the LN stations was defined according to the Japanese Society of Hepato-Biliary-Pancreatic Surgery guidelines. Effectiveness of LN dissection of each station was calculated using frequency of LN metastasis to the station and 5-year survival rate of patients with LN metastasis to that station. Results: Of the 105 patients included in the study, 46 (43.8%) had LN metastasis, and 43 (41.0%) underwent postoperative adjuvant therapy. LN metastasis, serum carbohydrate antigen (CA) 19–9 level > 37 U/mL, and positive bile duct margin were independent risk factors for shorter overall survival (OS). The most common metastatic LN station at surgery was No. 13 (32.7%), followed by No. 12 (19.2%), No. 17 (9.6%), and No. 8 (6.6%). There was no effectiveness of LN dissection of the station No. 8, 14, and 16. Adjuvant chemotherapy was significantly associated with longer OS in patients with LN metastasis but not in those with positive ductal margins or serum CA 19–9 level > 37 U/mL. Conclusions: Postoperative adjuvant chemotherapy was associated with a better prognosis in patients with DCC and LN metastasis. However, a more effective therapeutic strategy is required to improve the prognosis of patients with other negative prognostic factors. [ABSTRACT FROM AUTHOR]

Background: Locoregional recurrence and metastasis to the liver, peritoneum, and lung are the most common recurrent patterns of pancreatic ductal adenocarcinoma (PDAC) after radical resection. Recurrence in the abdominal wall is extremely rare. Herein, we report our experience with a patient who had recurrent PDAC in the abdominal wall with long-term survival by means of multidisciplinary therapy. Case presentation: A 76-year-old Japanese woman was diagnosed with resectable pancreatic tail cancer. She underwent distal pancreatectomy with regional lymphadenectomy after two cycles of gemcitabine plus S-1 as neoadjuvant therapy. She also received eight cycles of S-1 as adjuvant chemotherapy. Approximately 14 months after the initial surgery, imaging examinations identified a mass suggesting recurrence in the abdominal wall at the middle wound that involved the transverse colon. After two cycles of gemcitabine plus nab-paclitaxel, chemoradiotherapy (S-1 plus 45 Gy) and seven cycles of modified FOLFIRINOX (5-fluorouracil/leucovorin, irinotecan, and oxaliplatin) were administered. The patient did not develop any new recurrent lesions during chemotherapy and chemoradiotherapy. Therefore, the recurrent lesion in the abdominal wall and the involved transverse colon were resected. We confirmed the lack of peritoneal dissemination during surgery. Pathological examination revealed that the resected lesion was metastasis of primary PDAC, and the surgical margin was 1 mm. However, re-recurrence localized in the abdominal wall was detected 9 months later. The re-recurrent lesion was diagnosed as local recurrence of the first recurrent lesion. We performed a second resection of the abdominal wall using a femoral myocutaneous flap to achieve sufficient surgical margin. The pathological findings of the resected specimen were the same as those of the previous specimens, and the resection margin was negative. The patient's postoperative course was uneventful. Seven years after the initial surgery and 3 years and 7 months after the third surgery, the patient is alive with no signs of recurrence. Conclusions: Long-term survival could be achieved by radical resection with sufficient surgical margins for recurrence of PDAC in the abdominal wall if new other recurrent lesions, including peritoneal dissemination, are prevented through chemotherapy. [ABSTRACT FROM AUTHOR]

A 64-year-old man presented to our hospital with abdominal pain and 4–5 episodes of watery diarrhea per day for 2 months. Abdominal ultrasound examination revealed a mass in the peritoneal cavity, and computed tomography showed a 13.4 cm mass in the mesentery and a 3 cm mass in the mesocolon. The patient underwent laparoscopic partial resection for diagnosis. Microscopically, abundant fibrosis and numerous immunoglobulin (Ig) G4-positive plasma cells were observed. The serum level of IgG4 was 665 mg/dl postoperatively. These findings suggested that the lesion was consistent with IgG4-related sclerosing mesenteritis. Oral steroids resulted in rapid disappearance of symptoms and a decrease in masses. Recently, sclerosing mesenteritis are reported as IgG4-related disease or mimicking IgG4-related disease but multiple lesions rarely occur in the same organ. We report a case of IgG4-related sclerosing mesenteritis with multiple lesions without involvement of other organs, such as the pancreas and salivary glands. [ABSTRACT FROM AUTHOR]

Histopathological diagnosis of pancreatic ductal adenocarcinoma (PDAC) on endoscopic ultrasonography-guided fine-needle biopsy (EUS-FNB) specimens has become the mainstay of preoperative pathological diagnosis. However, on EUS-FNB specimens, accurate histopathological evaluation is difficult due to low specimen volume with isolated cancer cells and high contamination of blood, inflammatory and digestive tract cells. In this study, we performed annotations for training sets by expert pancreatic pathologists and trained a deep learning model to assess PDAC on EUS-FNB of the pancreas in histopathological whole-slide images. We obtained a high receiver operator curve area under the curve of 0.984, accuracy of 0.9417, sensitivity of 0.9302 and specificity of 0.9706. Our model was able to accurately detect difficult cases of isolated and low volume cancer cells. If adopted as a supportive system in routine diagnosis of pancreatic EUS-FNB specimens, our model has the potential to aid pathologists diagnose difficult cases. [ABSTRACT FROM AUTHOR]

Background: Synovial sarcoma (SS) of the esophagus is extremely rare. Because of the microscopic features of SS, the monophasic type can easily be misdiagnosed as other spindle cell tumors. Here, we present the first case of a primary SS of the esophagus in the presence of SS18-SSX2 fusion transcripts. Case presentation: A 47-year-old Japanese woman was initially diagnosed with thyroid papillary carcinoma in the left lobe and leiomyoma of the cervical esophagus and subsequently underwent left thyroid lobectomy and enucleation of the esophageal tumor. Four years after the first surgery, the esophageal tumor recurred. Endoscopic biopsy of the tumor revealed atypical cell proliferation with spindle cell features and mitoses. Immunohistochemistry showed focal positivity for bcl-2 and HHF35. Furthermore, the presence of SS18-SSX2 fusion transcripts was confirmed by reverse transcription-polymerase chain reaction analysis, using a paraffin-embedded tumor specimen. Therefore, the tumor was diagnosed as monophasic SS of the cervical esophagus. We re-evaluated the surgical specimen enucleated 3 years previously, which was initially diagnosed as leiomyoma, and the diagnosis of SS was confirmed. The patient underwent cervical esophagectomy with isolated jejunal interposition reconstruction. Three years after the second surgery, SS recurred in the distal anastomotic site between the jejunum and the esophagus, and the patient underwent thoracoscopic esophagectomy with gastric conduit reconstruction. The pathological grade of the lesion worsened with every recurrence. Conclusions: Monophasic SS can be difficult to discriminate from other spindle cell tumors based on microscopy alone, and molecular analysis could be useful for confirming the precise diagnosis of monophasic SS. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this case series was to describe computed tomography (CT) and magnetic resonance imaging (MRI) features of undifferentiated carcinoma with osteoclast-like giant cells of the pancreas. Methods: Institutional ethics review board approval was obtained, and informed consent was waived for this case series. We reviewed CT and MRI of patients with pathologically confirmed undifferentiated carcinoma with osteoclast-like giant cells of the pancreas found in the medical records of our hospital between 2006 and 2016. Results: Seven patients (3 males and 4 females; age, 59–82 years (mean, 71)) with confirmation by surgical resection (n = 3) or biopsy (n = 4) were identified. They underwent CT (n = 7) and MRI (n = 6). The tumors 26–83 mm in diameter (mean, 44 mm) were located in the head (n = 4) or body (n = 3) of the pancreas. They were demonstrated as lower attenuation areas relative to the adjacent pancreas on CT images obtained in both pancreatic and portal vein phases (n = 7) with a well-defined smooth margin (n = 5). They were demonstrated as hypointense areas relative to the pancreas on T2-weighted images (n = 4) and T2*-weighted images (n = 4) and diffusion-weighted images (n = 3). They contained hemosiderin deposits on histology (n = 7). Conclusions: Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas might be present as low attenuation areas with a well-defined smooth margin on CT images obtained in pancreatic and portal vein phases, and hypointense areas on T2-, T2*-, and diffusion-weighted images caused by hemosiderin deposits. [ABSTRACT FROM AUTHOR]

Objectives: To assess whether extracellular volume (ECV) fraction with equilibrium contrast-enhanced multidetector computed tomography (MDCT) predicts outcomes for unresectable pancreatic adenocarcinoma patients treated with chemotherapy METHODS: Sixty-seven patients (42 men, 25 women; mean age, 67.5 years; range, 45-83 years) with histologically confirmed surgically unresectable pancreatic adenocarcinoma underwent contrast-enhanced MDCT before systemic chemotherapy. Tumour contrast enhancement (CE) and ECV fraction were calculated using region-of-interest measurement within the pancreatic adenocarcinoma and aorta on unenhanced and equilibrium phase-enhanced CT. The effect on survival variables including age, sex, tumour location, tumour size, TNM stage, carbohydrate antigen (CA) 19-9, carcinoembryonic antigen (CEA), tumour CE and tumour ECV fraction was determined on univariate and multivariate analyses using Cox proportional hazards regression model.Results: Median overall survival was 10.5 months. On univariate analysis, elevated serum CA19-9 (hazard ratio (HR), 1.00; p = 0.006) and CEA (HR, 1.02; p = 0.011) levels were found to be associated with a negative effect on overall survival. Increasing tumour CE (HR, 0.98; p < 0.001) and ECV fraction (HR, 0.97; p = 0.001) were associated with a positive effect. Multivariate analysis revealed that only tumour ECV fraction was an independent predictor of overall survival (HR, 0.97; p = 0.012).Conclusions: ECV fraction with equilibrium contrast-enhanced MDCT could be a useful imaging biomarker for predicting patient survival after chemotherapy for unresectable pancreatic adenocarcinoma.Key Points: • Tumour aggressiveness and response to therapy are influenced by the extravascular extracellular space. • Extracellular volume (ECV) fraction can be quantified with equilibrium contrast-enhanced CT. • Patients with higher tumour ECV fraction had better prognosis after chemotherapy. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to investigate the maintenance of volume as a spacer by comparing vascular supply and apoptosis in an implanted autologous-free dermal fat graft (FDFG) and free fat graft (FFG). An autologous FDFG is a material used in plastic surgery and oncoplastic breast surgery that is ideal for immediate volume replacement after partial mastectomy because of its easy availability and minimal invasion of the donor site; however, immunohistochemical findings and survival procedures have not yet been reported. Methods: An experimental protocol using a unique animal model was designed for the present study. The expression of vascular endothelial growth factor (VEGF) was measured in FDFGs and FFGs implanted onto the pectoral major muscle of Wistar rats. Thirty Wistar rats were divided into two groups and postoperatively 1, 2, 4, 8, and 16 weeks (POW1, 2, 4, 8, 16). Six samples from three rats in each group were used as control samples (POW0). Results: The thickness of the implanted FDFG was not significantly different from the control sample at POW1, 2, 4, 8, and 16 between FDFG and FFG group; however, the thickness at POW8 and 16 was significantly lesser in the FFG group than in the control samples. The average proportion of fatty tissue to whole tissue ranged from 34.2 to 48.6 % in the FDFG group and from 57.2 to 76.7 % in the FFG group during the observation period; however, there was no significant difference in the proportion of fatty tissue between these two groups. There were no significant differences between the average number of VEGF-positive cells in the FDFG group and the FFG group at POW1, 2, 4, 8, and 16. The average number of TUNEL-positive cells in the early period at POW1 was significantly lower in the FDFG group than in the FFG group. Conclusions: This rat model was useful for investigating the mechanisms of angiogenesis, apoptosis, structure maintenance, and fibromatous changes. From the present experimental study, we believe that FDFG is one of the most convenient materials currently available to repair small defects at the time of BCS even in the clinical field. [ABSTRACT FROM AUTHOR]

Background: Both MUC1 and MUC4 are high molecular weight glycoproteins and are independent indicators of worse prognosis in many human epithelial cancers including oral squamous cell carcinoma (OSCC). However, there has been no investigation of the clinical importance of the co-expression of MUC1 and MUC4 in OSCC. The aim of this study was to evaluate the co-expression profile of MUC1/MUC4 and analyze the prognostic significance in OSCC. Methods: We examined the expression profile of MUC1 and MUC4 in OSCC tissues from 206 patients using immunohistochemistry. The co-expression profile of MUC1/MUC4 and its prognostic significance in OSCC was statistically analyzed. Results: MUC1 and MUC4 overexpression were strongly correlated with each other ( p < 0.0001) and a combination of both MUC1 and MUC4 expression was a powerful indicator for tumor aggressiveness such as tumor size ( p = 0.014), lymph node metastasis (0.0001), tumor stage ( p = 0.006), diffuse invasion ( p = 0.028), and vascular invasion ( p = 0.014). The MUC1/MUC4 double-positive patients showed the poorest overall and disease-free survival. Multivariate analysis revealed that MUC1/MUC4 double-positivity was the strong independent prognostic factor for overall and disease-free survival ( p = 0.007 and ( p = 0.0019), in addition to regional recurrence ( p = 0.0025). Conclusions: Taken together, these observations indicate that the use of a combination of MUC1/MUC4 can predict outcomes for patients with OSCC. This combination is also a useful marker for predicting regional recurrence. MUC1 and MUC4 may be attractive targets for the selection of treatment methods in OSCC. [ABSTRACT FROM AUTHOR]

Objective: To examine correlations of (18)F-fluorothymidine (FLT) uptake with pathological tumour size and immunohistochemical Ki-67, and thymidine kinase 1 (TK-1) expressions in primary and metastatic node colorectal cancer foci.Methods: Thirty primary cancers (PCs) and 37 metastatic nodes (MNs) were included. FLT uptake was assessed by visual scores (non-visible: 0-1 and visible: 2-4), standardized uptake value (SUV), and correlated with size, Ki-67, and TK-1. SUV was measured in visible lesions. FLT heterogeneity was assessed by visual scores (no heterogeneous uptake: 0 and heterogeneous uptake: 1-4).Results: Forty-two lesions were visible. The visible group showed significantly higher values than the non-visible group in size, Ki-67, and TK-1 (each p < 0.05). Size correlated significantly with visual score (PC; ρ = 0.74 and MN; ρ = 0.63), SUVmax (PC; ρ = 0.49, and MN; ρ = 0.76), and SUVmean (PC; ρ = 0.40 and MN; ρ = 0.76) (each p < 0.05). Visual score correlated significantly with size (ρ = 0.86), Ki-67max (ρ = 0.35), Ki-67mean (ρ = 0.38), TK-1max (ρ = 0.35) and TK-1mean (ρ = 0.25) (each p < 0.05). No significant correlations were found between FLT uptake and Ki-67 or TK-1 in 42 visible lesions (each p > 0.05). Heterogeneous FLT uptake was noted in 73 % (22/30) of PCs.Conclusion: FLT uptake correlated with size. Heterogeneous FLT distribution in colorectal cancers may be one of the causes of weak or lack of FLT uptake/Ki-67 or TK-1 correlation.Key Points: FLT uptake correlated well with tumour size in colorectal cancer. Weak or lack of FLT uptake/Ki-67 and TK-1 correlations were observed. Immunohistochemical Ki-67 and TK-1 expressions are not always correlated with FLT uptake. [ABSTRACT FROM AUTHOR]

We encountered two cases of epithelial-myoepithelial carcinoma (EMC), a rare low-grade malignant tumor of the parotid gland. These two EMCs showed similar imaging findings: gradual contrast enhancement on dynamic magnetic resonance imaging (MRI), intense (18)F-fluorodeoxyglucose (FDG) uptake in the solid tumor portion, and little enhancement and little FDG uptake in the hemorrhagic necrotic tumor portion. When a parotid gland tumor shows gradual MRI contrast enhancement and focally intense FDG uptake, EMC should be included in the differential diagnosis. [ABSTRACT FROM AUTHOR]

A 62-year-old man was admitted to our hospital for dysphagia, leukocytosis, and pyrexia. The serum level of granulocyte colony-stimulating factor (G-CSF) was high and immunostaining of a biopsy specimen of esophageal tumor using anti-granulocyte-macrophage colony-stimulating factor (anti-GM-CSF) antibody demonstrated GM-CSF expression in the cytoplasm of the tumor cells. After esophagectomy, the leukocyte count and serum G-CSF level normalized. Histologically, this tumor was diagnosed as a carcinosarcoma with two components: squamous cell carcinoma and sarcoma. Tumor cells were also positive for G-CSF receptor, suggesting autocrine growth regulation by G-CSF. Moreover, tumor cells were positive for Ki-67, cyclin D1, p53, and epidermal growth factor receptor (EGFR), which were related to the acquisition of more aggressive tumor behavior. Although G-CSF-producing esophageal carcinosarcoma is very rare, we should consider such disease when a patient has symptoms of leukemia such as leukocytosis and high fever. [ABSTRACT FROM AUTHOR]

A rare case of a carcinosarcoma of the extrahepatic bile duct demonstrating interesting features is described. A 75-year-old woman with a history of choledocholithotomy presented with acute obstructive suppurative cholangitis. Ultrasonography and computed tomography showed a thickened choledochal wall, with calcification. Percutaneous transhepatic and endoscopic retrograde cholangiography revealed a round filling defect accompanied by an irregular obstruction in the common bile duct. Carcinosarcoma was diagnosed from a protruding lesion in the common bile duct obtained by intraoperative frozen sectioning, and pylorus-preserving pancreatoduodenectomy was performed. Histological examination by light microscopy showed a transition between the carcinomatous and sarcomatous components and positive immunoreactivity for epithelial markers in the sarcomatous component. The patient died of a local recurrence 2 years after the surgery. Polypoid growth and ossification in the tumor could be representative features of carcinosarcoma of the extrahepatic bile duct. [ABSTRACT FROM AUTHOR]

Purpose: CD227 (MUC1), a membrane-associated glycoprotein expressed by many types of ductal epithelia, including pancreas, breast, lung, and gastrointestinal tract, is overexpressed and aberrantly glycosylated by malignant cells. We sought to define epitopes on MUC1 recognized by the different cell-mediated immune responses by an in vivo assay. Epitopes identified by this assay were evaluated for efficacy to protect mice transgenic for human MUC1 (MUC1.Tg) against MUC1-expressing tumor growth. Methods: We investigated contributions of the tandem repeat (TR) and the cytoplasmic tail (CT) of MUC1 to the MUC1-specific immunological rejection of tumor cells. MUC1 cDNA constructs, in which the TR region was deleted or the CT was truncated, were transfected into two different murine tumor cell lines (B16 and Panc02), which were used to challenge mice and evaluate immunological rejection of the tumors. We used tumor rejection in vivo to define epitopes on the TR and CT of MUC1 recognized by T cell-mediated immune responses in a preclinical murine model. Results: Our findings demonstrated that the TR and a portion of the MUC1 CT contributed to CD4+ T cell rejection of MUC1-expressing B16 tumor cells, but not rejection of MUC1-expressing Panc02 tumor cells. A separate epitope in the CT of MUC1 was necessary for CD8+ T cell rejection of Panc02 tumor cells. Based on these studies, we sought to evaluate the efficacy of immunizing mice transgenic for (and immunologically tolerant to) human MUC1 with peptides derived from the amino acid sequence of the CT of MUC1. Results showed that survival can be significantly prolonged in vaccinated MUC1.Tg mice challenged with MUC1-expressing tumor cells, without induction of autoimmune responses. Conclusions: These studies demonstrated that MUC1 peptides may be utilized as an effective anticancer immunotherapeutic, and confirmed the importance of immunogenic epitopes outside of the TR. [ABSTRACT FROM AUTHOR]

We experienced a rare case of spindle cell carcinoma of the pancreas. The patient was a 74-year-old man who complained of abdominal pain and loss of weight. Ultrasonographic scans revealed a hypoechoic solid mass in the head of the pancreas, 4 cm in diameter, with a high echoic spot suggestive of central necrosis or hemorrhage. The mass was hypodense on enhanced computed tomographic scans and hypovascular on angiograms. At laparotomy, the tumor had invaded to the mesocolon, but dissemination and distant metastasis were not found. We therefore performed pylorus-preserving pancreatoduodenectomy and ascending colectomy. The patient was discharged on postoperative day 26 after an uneventful recovery. Two months later, he was readmitted because of ascites, with positivity of spindle cells shown on cytology; he died on day 92 after surgery. In the resected specimen, the tumor had a mostly sarcomatous component, consisting of spindle-shaped cells, and a small glandular component. Immunohistochemically, both components were positive for cytokeratin and epithelial membrane antigen, but negative for vimentin and desmin. The tumor was diagnosed as a spindle cell carcinoma of the pancreas. Perinural invasion, lymphatic permeation, and blood vessel invasion were found, but lymph node metastasis was not found. Although a curative operation was performed, the outcome in this patient was very poor. [ABSTRACT FROM AUTHOR]

Background: Primary leiomyosarcoma (LMS) of vascular origin is a rare lesion, and patients with LMS of vascular origin have poorer prognoses than patients with LMS of other origins. The inferior vena cava is the most commonly affected vessel and accounts for 60% of all vascular cases. However, LMS originating from the ovarian vein is extremely rare, and we are only aware of 15 reported cases. Therefore, we report our experience with a case of LMS originating from the right ovarian vein and review the related literature. Case presentation: A 71-year-old Japanese woman with no symptoms was admitted to our hospital because of abnormal findings in a routine abdominal ultrasonography check-up. Contrast-enhanced computed tomography of the abdomen revealed a well-defined, lobulated solid mass with a diameter of 5.5 cm in the right retroperitoneal space. The mass exhibited relatively low uptake during 18F-fluorodeoxyglucose positron emission tomography. Based on these findings, the differential diagnosis included a retroperitoneal tumor, such as a desmoid tumor, leiomyoma, LMS, and malignant mesothelioma. Operative findings confirmed that the mass had originated from the right ovarian vessels, and en bloc excision was performed for the mass and the right ovarian vessels. The final pathological diagnosis was LMS originating from the right ovarian vein, and the surgical resection margins were free from tumor cells. After histological findings confirmed the LMS diagnosis, the patient underwent adjuvant radiation therapy and has not exhibited signs of local recurrence or metastasis in the 6 months after surgery. Conclusions: We encountered a 71-year-old woman with LMS originating from her right ovarian vein. The prognosis of vascular LMS is generally poor. Therefore, careful follow-up will be required for our patient. [ABSTRACT FROM AUTHOR]