Endometrioid adenocarcinoma arising from inguinal endometriosis during follow-up for gastric cancer surgery: a case report and literature review.

Malignant transformation of inguinal endometriosis is rare. A 56-year-old woman underwent surgery for advanced gastric cancer 5 years ago and received postoperative adjuvant chemotherapy. She had no recurrence since then. However, 5 years after surgery, contrast-enhanced computed tomography (CT) showed a mass in the right inguinal region suspected to be a hydrocele of the canal of Nuck, with a blood test showing a slightly elevated CA19-9 level (63.0 U/mL). Six months later, CT showed an enlarged mass in the right inguinal region and inflammation in the surrounding area. In addition, both inguinal lymph nodes and those in the right iliac artery area were enlarged, suggesting the possibility of malignancy. For diagnostic purposes, a right inguinal mass was excised. Histopathological examination revealed that it was endometrioid adenocarcinoma with ectopic endometriois as the origin. The differential diagnoses for inguinal masses in women include an inguinal hernia, hydrocele of the canal of Nuck, ectopic endometriosis, lymphoma, and metastatic malignancy. The presence of a primary malignancy in the inguinal region is sporadic but must be differentiated. This is the first case of malignant transformation of inguinal endometriosis developed during postoperative follow-up of another cancer.

Diabetes can increase the prevalence of EBV infection and worsen the prognosis of nasopharyngeal carcinoma.

Epstein‒Barr virus (EBV) infection is a primary oncogenic factor of nasopharyngeal carcinoma (NPC) that elicits epithelial-mesenchymal transition (EMT). Although diabetic patients are more susceptible to various infectious diseases, the pathological association with virus-related NPC has not yet been clarified. Herein, we evaluated the influence of diabetes on the clinicopathological changes of 70 patients with NPC. Disease-specific survival (DSS) modified by viral infection was also analysed. The proportion of NPC patients with diabetes was 32.9% (23/70 cases), and 91.3% (21/23 cases) were infected with EBV detected by EBER-I in situ hybridisation. NPC with diabetes showed an effect on EMT evaluated by immunostaining for E-cadherin and vimentin, which was correlated with HbA1c levels. Receiver operating characteristic (ROC) curve analysis determined a HbA1c level of 6.5% as the cut-off value for primary disease death at 2 years [area under the curve (AUC) 0.76; sensitivity 0.64; and specificity 0.81]. High HbA1c levels (≥6.5%) significantly increased the number of lymph node metastases in NPC compared to low HbA1c levels (<6.5%, p<0.01). Diabetic NPC patients had a significantly poorer prognosis than all non-diabetic patients (DSS, 72 months vs not reached, p<0.05). Diabetic EBV-positive NPC patients had a significantly poorer prognosis than non-diabetic EBV-positive patients (DSS, 35 months vs not reached, p<0.01). Multivariate analysis using the Cox proportional hazards model also suggested that HbA1c ≥6.5% was a significant factor in poor prognosis, with a hazard ratio of 6.84 (p<0.05). Collectively, our results revealed for the first time a high prevalence of EBV infection, poor prognosis and the importance of proper glycaemic control in diabetic NPC patients.

Massive platelet-rich thrombus formation in small pulmonary vessels in amniotic fluid embolism: An autopsy study.

OBJECTIVE: To identify pulmonary/uterine thrombus formation in amniotic fluid embolism (AFE). DESIGN: Retrospective, observational. SETTING: Nationwide. POPULATION: Eleven autopsy cases of AFE and control cases. METHODS: We assessed pulmonary and uterine thrombus formation and thrombus area in AFE and pulmonary thromboembolism (PTE) as a control. The area of platelet glycoprotein IIb/IIIa, fibrin, neutrophil elastase, citrullinated histone H3 (a neutrophil extracellular trap marker) and mast cell chymase immunopositivity was measured in 90 pulmonary emboli, 15 uterine thrombi and 14 PTE. MAIN OUTCOME MEASURES: Pathological evidence of thrombus formation and its components in AFE. RESULTS: Amniotic fluid embolism lung showed massive thrombus formation, with or without amniotic emboli in small pulmonary arteries and capillaries. The median pulmonary thrombus size in AFE (median, 0.012 mm2 ; P < 0.0001) was significantly smaller than that of uterine thrombus in AFE (0.61 mm2 ) or PTE (29 mm2 ). The median area of glycoprotein IIb/IIIa immunopositivity in pulmonary thrombi in AFE (39%; P < 0.01) was significantly larger than that of uterine thrombi in AFE (23%) and PTE (15%). The median area of fibrin (0%; P < 0.001) and citrullinated histone H3 (0%; P < 0.01) immunopositivity in pulmonary thrombi in AFE was significantly smaller than in uterine thrombi (fibrin: 26%; citrullinated histone H3: 1.1%) and PTE (fibrin: 42%; citrullinated histone H3: 0.4%). No mast cells were identified in pulmonary thrombi. CONCLUSIONS: Amniotic fluid may induce distinct thrombus formation in the uterus and lung. Pulmonary and uterine thrombi formation may contribute to cardiorespiratory collapse and/or consumptive coagulopathy in AFE.

Dentinogenic ghost cell tumor treated with a combination of marsupialization and radical resection: a case report and review of the literature.

BACKGROUND: Dentinogenic ghost cell tumor is a rare benign tumor that accounts for less than 3% of all cases and consists of the stellate reticulum, which is made up of enamel epithelioid and basaloid cells. Although DGCT is a benign tumor, the local infiltration of the odontogenic epithelium or recurrences have been reported, and its detailed pathology and treatments remain unclear. CASE PRESENTATION: This report describes the case of a 60-year-old Japanese male diagnosed with a maxillary dentinogenic ghost cell tumor. Images showed well-circumscribed, multilocular cystic lesions with a calcified substance in the interior. Marsupialization was performed along with biopsy to prevent the expansion of the lesion, and a partial maxillectomy was performed 2 years after the initial examination. Histopathological findings showed ameloblastomatous proliferation containing clusters of ghost cells and dentinoid materials, resulting in the diagnosis of dentinogenic ghost cell tumor. This article also reviews recently reported cases of dentinogenic ghost cell tumor. CONCLUSION: It is important to perform marsupialization, proper resection, and postoperative follow-up because of possible recurrence.

[Subtotal Stomach-Preserving Pancreaticoduodenectomy for Large Laterally Spreading Early Duodenal Cancer-A Case Report].

A 76-year-old man came to our hospital for a close examination after an abnormal finding during a medical checkup. Upper gastrointestinal endoscopy revealed a circumferential flat lesion with irregularity in the second to third portions of the duodenum. Biopsy diagnosed papillary adenocarcinoma. Contrast-enhanced CT of the abdomen showed no evidence of lymph node enlargement and distant metastasis. Endoscopic depth of the lesion was estimated to be intramucosal carcinoma, but it was approximately 60 mm in size, circumferential, and located near the papilla Vater. Therefore, endoscopic resection was deemed difficult. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Postoperative pathological examination revealed type 0-Ⅱa, tub1>pap, pTis, Ly0, V0, 80×50 mm, BD1, Ex0, Pn0, pPM0, pDM0, pN0, pStage 0. There has been no recurrence since then. Lateral spreading duodenal carcinoma is a rare disease, and endoscopic resection, local resection, and pancreaticoduodenectomy have been reported as treatment options. We report a case of resection of a large lateral spreading duodenal carcinoma with a review of the literature.

Spontaneous rupture of an undifferentiated carcinoma with osteoclast-like giant cells of the pancreas presenting as intra-abdominal bleeding: a case report.

BACKGROUND: Undifferentiated carcinoma is a very rare histologic subtype, representing only 0.8% to 5.7% of all pancreatic exocrine neoplasms. Additionally, spontaneous abdominal hemorrhage is a particularly rare, life-threatening cause. CASE PRESENTATION: A 68-year-old man was taken by ambulance to our hospital because of sudden-onset abdominal pain. Contrast-enhanced abdominal computed tomography revealed a huge mass measuring 99 × 70 mm in the pancreatic tail with enhanced rim staining in the peripheral area. Imaging also showed extravasation and fluid collection beside the tumor. Hence, spontaneous rupture of the pancreatic tumor and intra-abdominal bleeding were diagnosed. Emergency laparotomy was performed because of acute abdominal pain with peritoneal signs. With an intraoperative diagnosis of rupture of the pancreatic tumor, distal pancreatectomy was successfully performed. Histologically, hematoxylin and eosin staining showed round to spindle-shaped, highly pleomorphic mononuclear cells and multinucleated giant cells as well as a component of ductal adenocarcinoma. Immunohistochemical staining showed that the tumor cells were negative for AE1/AE3, whereas the non-neoplastic osteoclast-like giant cells were positive for CD68. Taken together, these results led to a diagnosis of undifferentiated carcinoma with osteoclast-like giant cells. The patient's postoperative course was uneventful. CONCLUSION: We experienced an extremely rare case of spontaneous rupture of an undifferentiated carcinoma with osteoclast-like giant cells presenting as intra-abdominal bleeding. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. Undifferentiated carcinoma should be considered as a differential diagnosis in the case with spontaneous rupture of a pancreatic tumor.

Role of debulking surgery in combination with immune therapy: A successfully treated case of locally advanced mucosal melanoma.

Immune checkpoint inhibitors (ICIs) have markedly changed the treatment landscape for melanoma; however, their efficacy and applications are currently limited and medical requirements remain unmet. The present case study reports on a 85-year-old female patient who visited our outpatient clinic with a 1-month history of a buccal mucosa mass and was diagnosed with locally advanced mucosal melanoma of the head and neck. The patient's tumor progressed right after the administration of nivolumab, compromising oral intake. Palliative debulking surgery was performed. Subsequently, the other part of the melanoma on the hard palate slightly decreased in size without forming new lesions for more than one year after surgery. The present case exemplifies that tumor volume reduction surgery may increase the response to ICI and may prolong the duration of response. This combination therapy may be more effective in patients whose tumors increase in size after administration of ICIs or whose tumor is already large at the beginning of treatment. The combination of ICIs and debulking surgery may become an important treatment option in the future for locally advanced mucosal melanoma.

Successful resection of a huge hepatocellular adenoma.

Hepatocellular adenoma (HCA) is a rare benign liver tumor that has been reported to occur particularly more often in women who use contraceptives. A 72-year-old woman with no history of using contraceptives presented to our hospital for further examination of a liver tumor. Contrast-enhanced computed tomography and gadoxetic acid-enhanced magnetic resonance imaging revealed a huge solitary hepatic tumor measuring 83 × 76 mm in segments 4, 5, and 8. The differential diagnoses were cholangiocarcinoma and mixed-type hepatocellular carcinoma. Percutaneous needle biopsies were performed twice, and no malignant components were found. Central bi-segmentectomy of the liver was successfully performed. Immunohistochemical staining showed that ß-catenin was positive in the membrane of the tumor cells, while fatty acid-binding protein, glutamine synthetase, and amyloid A were negative. These results led to a diagnosis of HCA, hepatocyte nuclear factor-1α-inactivated subtype. The patient's postoperative course was uneventful, and she developed no recurrence for 10 months after surgery. We experienced a rare case of benign HCA. Obtaining a correct preoperative diagnosis is sometimes difficult at the first evaluation. HCA should be considered as a differential diagnosis of liver tumors.

Synchronous gastric cancer and primary lymphoma of right adrenal gland: a case report.

This report presents an extremely rare case of synchronous gastric cancer and primary adrenal diffuse large B-cell lymphoma (DLBCL). An 82-year-old man underwent computed tomography, which revealed a heterogeneous appearing and hypodense adrenal mass and a gastric mass with no enlarged lymph nodes in the neck, mediastinum, abdomen, and inguinal region. Upper gastrointestinal endoscopy revealed a protruding gastric tumor. The specimens obtained from endoscopic biopsy were histologically confirmed to be adenocarcinoma. The hormonal findings eliminated functional adrenal tumor. The patient underwent distal gastrectomy with regional lymph node resection for gastric cancer and incisional biopsy of the adrenal mass. Based on the pathological findings, diagnoses of mixed mucinous and tubular adenocarcinomas of the stomach and adrenal DLBCL were confirmed. Postoperation, the patient received rituximab combined with low-dose doxorubicin, cyclophosphamide, vincristine, and prednisone (R-miniCHOP). Six courses of R-miniCHOP were planned, but were completed in only one course at the patient's request. The patient died 2 months after surgery.

Postoperative solitary liver metastasis from esophageal squamous cell carcinoma achieving a clinical complete response to chemotherapy with cisplatin and 5-fluorouracil followed by stereotactic body radiotherapy: A case report.

Treatment strategies for distant organ metastasis have changed markedly since the concept of oligometastasis was introduced. The perception that distant organ metastasis is a systemic disease and not eligible for local therapy is now a thing of the past. Therefore, the present report details a case of postoperative solitary liver metastasis from esophageal squamous cell carcinoma (ESCC), which achieved a clinical complete response to chemotherapy with cisplatin and 5-fluorouracil (5-FU) followed by stereotactic body radiotherapy (SBRT). A 76-year-old male patient underwent esophagectomy for lower thoracic ESCC. At 7 months after surgery, abdominal CT revealed a solitary hypovascular mass, 28 mm in size, in segment 7 of the liver. After three courses of chemotherapy with cisplatin and 5-FU, abdominal CT revealed that the liver mass had shrunk to 7 mm in size. SBRT was then administered with a 6 MV X-ray beam generated by a linear accelerator. A total dose of 50 Gy was given in 5 fractions of 10 Gy to the liver mass. At 1 month after SBRT, abdominal CT revealed that the liver mass had disappeared. The patient received no further adjuvant chemotherapy and had no recurrence at 18 months after diagnosis of liver metastasis and 13 months after SBRT.

Bcl-2-negative IGH-BCL2 translocation-negative follicular lymphoma of the thyroid differs genetically and epigenetically from Bcl-2-positive IGH-BCL2 translocation-positive follicular lymphoma.

AIMS: Follicular lymphoma (FL), comprising a minor subset of primary thyroid lymphomas, is divided into two groups based on Bcl-2 expression and IGH-BCL2 translocation. The clinicopathological features exhibited by Bcl-2-negative IGH-BCL2 translocation-negative FL of the thyroid (Bcl-2- /IGH-BCL2- tFL) are different from those of conventional FL; however, its lymphomagenesis remains unclear. Here, we collected samples from seven patients with Bcl-2- /IGH-BCL2- tFL to investigate their epigenetic and genetic aberrations. METHODS AND RESULTS: The immunohistochemical profiles of epigenetic modifiers and the methylation status of histones were examined, including EZH2, MLL2/KMT2D, CBP/CREBBP, EP300, H3K27me3 and H3K4me3, in Bcl-2- /IGH-BCL2- tFL and Bcl-2-positive IGH-BCL2 translocation-positive FL of the thyroid (Bcl-2+ /IGH-BCL2+ tFL). Most Bcl-2- /IGH-BCL2- tFLs retained the positivity of epigenetic modifiers and lower expression of H3K27me3, although Bcl-2+ /IGH-BCL2+ tFLs exhibited aberrant immunohistochemical patterns of EZH2 and CBP/CREBBP and overexpression of H3K27me3. Samples from seven cases were further analysed using targeted sequencing, focusing on the exons of 409 key tumour suppressor genes and oncogenes. Bcl-2- /IGH-BCL2- tFLs do not have pathogenic mutations of epigenetic modifiers, such as EZH2, MLL2/KMT2D, MLL3/KMT2C, EP300 and ARID1A, which have been reported in FLs in the literature, whereas Bcl-2+ /IGH-BCL2+ tFLs are probably pathogenic/pathogenic missense mutations or frameshift mutations of these genes. Additionally, novel mutations in TET2 and EP400 were detected in Bcl-2- /IGH-BCL2- tFLs. CONCLUSIONS: Different genetic and epigenetic abnormalities might be involved in the oncogenesis of Bcl-2- /IGH-BCL2- tFLs from Bcl-2+ /IGH-BCL2+ tFLs and other FLs.

SS-A52 antigen expression in thymic carcinoma accompanied with Sjögren syndrome: A case report.

RATIONALE: The relationship between thymic tumors and Sjögren syndrome (SjS) is unknown, and surgical resection has not been optimized. Especially, thymic carcinoma with autoimmune disease is rare. Analysis of SS-A52, germinal centers, plasma cells, and Foxp3+ Treg in thymic carcinoma has never been reported, and their pathological roles in causing SjS have not been studied. PATIENT CONCERNS: A 78-year-old man presented with sputum production and xerostomia while asleep. Chest computed tomography showed a homogeneous and hypodense mass in the anterosuperior mediastinum. Serum levels of the antinuclear antibody, antibody to SS-A, and antibody to SS-B were positive. DIAGNOSES: Thymic carcinoma (squamous cell carcinoma) and SjS. INTERVENTIONS: Video-assisted thoracoscopic resection of the mediastinal tumor and postoperative radiation therapy was performed. OUTCOMES: The histological diagnosis was thymic squamous cell carcinoma. Histologically, the squamous carcinomatous cells were arranged in nests and cords in the fibrohyaline stroma with capsular invasion. In the stroma, dense lymphoid tissues containing large reactive germinal centers and many plasma cells were also noted. In the involuted thymus, CD20-positive mature lymphocytes infiltrated, and germinal centers were noted. Double immunohistochemical staining revealed that SS-A52 antigen was positive in both the carcinoma component and CD20-positive mature B cells. Postoperatively, the xerostomia persisted, and serum SS-A and SS-B remained positive. No evidence of carcinoma recurrence with chest computed tomography scan was observed at 18-months follow-up. LESSONS: In the surgical treatment of thymic tumors with SjS, extended thymectomy might be worth considering to stop the progressive destruction of the targets of SjS-specific autoantibodies. However, the postoperative symptoms may not dramatically improve because the target organs might have changed irreversibly, and memory B cells might persist. This is the first report that demonstrated the SS-A52 antigen presentation in a thymic tumor to the best of our knowledge.

Maternal death analysis based on data from the nationwide registration system in Japan (2010-2018).

The maternal mortality rate in Japan was 3.5 per 100 000 live births in 2017, similar to that reported in other developed countries. To reduce the number of maternal deaths, a Japanese nationwide registration and analysis system was implemented in 2010. Between January 2010 and April 2018, 367 maternal deaths were reported. Among them, by reviewing 80 autopsy records, the direct obstetric causes of death were identified in 52 women. The major causes of deaths were amniotic fluid embolism and acute pulmonary thromboembolism. The other 26 maternal deaths were associated with indirect obstetric causes including invasive Group A Streptococcus infection, aortic dissection, cerebral stroke and cardiomyopathies. This review highlights the importance of autopsy in maternal deaths. On analyzing 42 autopsy specimens obtained from registered cases of maternal death during 2012-2015, the 36% of causes of death by autopsy were discordant with the clinical diagnosis. Moreover, of the 38% of non-autopsied maternal death, the cause of death could not be clarified from the clinical chart. We emphasized that detailed autopsies are necessary to clarify the precise pathologic evidence related to pregnancy and delivery, especially causes of unexpected death such as amniotic fluid embolism.

[Small Bowel Metastasis of Breast Cancer-A Case Report].

A 69-year-old female had hormone therapy for liver metastasis after surgery for right breast cancer. She came to the hospital with a complaint of abdominal pain and was admitted with a diagnosis of small bowel obstruction. She had previously undergone surgery for an ovarian tumor and was suspected of having an intestinal obstruction caused by adhesions in her pelvis. She promptly improved with conservative treatment of fasting only. In the following months, she developed 2 intestinal obstructions, and CT scan revealed a neoplastic lesion in the small intestine. With the diagnosis of small intestinal tumor, laparoscopic surgery was performed. A neoplastic lesion was found in the ileum. A small bowel resection was performed. She was discharged with a good postoperative course. The pathological results showed breast cancer metastasis in the small intestine. Based on the diagnosis, postoperative chemotherapy has been started. Gastrointestinal metastasis of breast cancer is relatively rare and rarely causes clinical problems. We report a case of small intestinal metastasis of breast cancer.

Analysis of preventability of malignancy-related maternal death from the nationwide registration system of maternal deaths in Japan.

Objective: We reviewed malignancy related maternal deaths in Japan to ascertain if there were avoidable factors.Methods: Malignancy-related maternal death in Japan reported to the Maternal Death Exploratory Committee, from 2010 to 2016 inclusive.Results: There were 12 cases of maternal death caused by malignancy. There were four gastric cancers (two poorly differentiated adenocarcinoma, one signet ring cell carcinoma with adenocarcinoma, one histology not available), 3 leukemia (two acute myeloid leukemia, one aggressive NK cell leukemia), two ureteral cancers (histology not available), one malignant lymphoma (diffuse large B-cell lymphoma with translocation), one brain tumor (gliomatosis cerebri), and one cervical cancer (glassy cell carcinoma). Two gastric cancer patients had chronic gastric pain before conception. In two cases the physicians commented that they had avoided computed tomography and the brain biopsy needed for diagnosis because the patient was pregnant. At diagnosis, the clinical stages were II-IV in 9, and the performance status was 3-5 in 8. Indication for delivery was exacerbated maternal condition in 5, for treatment in 3, spontaneous labor in 3, and one patient declined elective delivery. Median [interquartile rage] (range) gestational weeks of delivery was 29 [24-30] (19-40). One cervical cancer patient had a radical hysterectomy and chemotherapy for 10 months. However, three leukemia and one gastric cancer patients had chemotherapy within 10 d because they deteriorated rapidly. Another seven cases did not have any treatment because of poor general condition or because they remained undiagnosed. In all cases, the Committee considered that there was no evidence of substandard care.Conclusion: In these cases, both the clinical stages and biological degree of malignancy were high. In two-thirds of cases, early termination of the pregnancy was indicated because of deteriorating maternal condition. Chemotherapy was not effective because of short available time for therapy and the advanced stage of the cancers when diagnosed. Encouraging women to have a thorough medical assessment before conception, and early diagnosis and treatment before pregnancy, appears to be the only practical way to reduce deaths from malignancy while a woman is pregnant.

Successful resection of a neuroendocrine tumor in the gallbladder: a case report.

BACKGROUND: Gallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder. In 2019, the World Health Organization updated the classification of NETs based on their molecular differences. The mutation status of DAXX and ATRX has been added to the criteria for well-differentiated NETs. CASE PRESENTATION: A 50-year-old man presented to our hospital for further examination of a gallbladder polyp. He had no right quadrant pain, fever, jaundice, weight loss, or carcinoid syndrome-related symptoms. The patient hoped to avoid cholecystectomy. During the 3-year observation period, the polyp gradually increased in size from 8.3 to 9.9 mm. He decided to undergo surgery, and whole cholecystectomy was successfully performed. Immunohistochemical staining revealed positivity for chromogranin A, synaptophysin, and CD56. The Ki-67 index was < 3%. Taken together, these results led to a diagnosis of a grade 1 GB-NET. We also performed immunohistochemical staining of DAXX and ATRX, which revealed that DAXX protein expression was negative. The patient's postoperative course was uneventful, and he developed no recurrence for 8 years after surgery. CONCLUSION: We experienced a very rare case of GB-NET. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. A GB-NET should be considered as a differential diagnosis of gallbladder tumors.

Occult follicular lymphoma in a swollen regional lymph node of gastric schwannoma.

BACKGROUND: Regional lymphadenopathy is more commonly noted in gastric schwannomas than in other gastric submucosal tumors. Most of the swollen lymph nodes associated with gastric schwannomas are non-metastatic lymphadenopathy. CASE PRESENTATION: A 69-year-old Japanese woman was referred to our hospital with a chief complaint of abdominal discomfort. Contrast-enhanced computed tomography (CT) of the abdomen revealed an extraluminal tumor with heterogeneous enhancement at the middle stomach on the lesser curve, accompanied with one swollen lymph node approximately 10 mm in size and several small lymph nodes in the perigastric region. These lymph nodes were flat; therefore, we considered them to be non-metastatic. The main tumor was removed via wedge resection. Soft and slightly swollen lymph nodes, which were compatible with the lymph nodes noted in the preoperative CT, were found near the main tumor in the fatty tissue at the lesser curvature of the stomach. An excisional biopsy of the largest lymph node was performed for the diagnosis. Based on pathological findings, a diagnosis of gastric schwannoma and follicular lymphoma (FL) was confirmed. The patient is doing well without recurrence of either the gastric schwannoma or FL 28 months postsurgery. CONCLUSIONS: The present report detailed an extremely rare case of FL coincidentally discovered in the swollen regional lymph node of gastric schwannoma.