Mechanical Thrombectomy for Internal Carotid Artery Occlusion in a Patient with POEMS Syndrome.

Objective: To report a case of mechanical thrombectomy (MT) for internal carotid artery (ICA) occlusion in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, a rare systemic disease associated with plasma cell proliferation. Case Presentation: A 52-year-old woman was taking steroids due to autoimmune hepatitis. She was diagnosed with acute cerebral infarction due to left ICA occlusion. Although MT was performed, recanalization was not achieved. Therefore, recanalization was carried out using a vasodilator and percutaneous transluminal angioplasty (PTA) in combination. Conclusion: PTA may be effective for large-vessel occlusion (LVO) in patients with POEMS syndrome.

Percutaneous transluminal angioplasty for suspected vertebral artery stump syndrome.

Vertebral artery stump syndrome is a rare disease associated with a posterior circulation stroke after vertebral artery origin occlusion. However, few reports have addressed its management. We herein present a case involving a patient with vertebral artery stump syndrome who underwent successful intravascular intervention. We also present a literature review of previous cases of this rare disease. The present case involved a 91-year-old man with acute onset of vertigo and disturbance of consciousness. Diffusion-weighted imaging showed an acute ischaemic stroke in the bilateral cerebellar hemispheres. Magnetic resonance angiography revealed left vertebral artery origin occlusion. Angiography detected a nearly occluded left vertebral artery site, with distal antegrade collateral flow via the deep cervical artery at the C6 level. We observed intravascular stasis at the proximal end of the left vertebral artery via the collateral flow. We performed percutaneous transluminal angioplasty towards the occluded left vertebral artery site. The flow from the left vertebral artery was significantly improved. This is the first report of percutaneous transluminal angioplasty performed for vertebral artery stump syndrome. Although vertebral artery stump syndrome has a high risk of recurrence and a poor prognosis, endovascular intervention showed a better outcome than pharmacotherapy in our patient. However, such cases are rare and further investigations are needed.

Rare gastrointestinal stromal tumor in an ileal conduit detected by recurrent massive bleeding from the stoma.

INTRODUCTION: The development of secondary tumors is a well-known late adverse event after urinary diversion. However, the frequency of secondary tumors after an ileal conduit is the lowest compared to other methods used for urinary diversion. We observed a rare case of a gastrointestinal stromal tumor in an ileal conduit detected by recurrent massive bleeding from the stoma. CASE PRESENTATION: An 87-year-old female was hospitalized at our hospital due to recurrent bleeding from a stoma 22 years after radical cystectomy. Contrast-enhanced computed tomography revealed a 5-cm mass in her ileal conduit. She underwent a complete resection of the tumor, a histological examination of which revealed it to be a gastrointestinal stromal tumor. The condition of the patient has been good showing no recurrence or metastases 4 years after surgery. CONCLUSION: We report a rare secondary tumor, a gastrointestinal stromal tumor, arising from an ileal conduit.

Filiform polyposis with sigmoid colon adenocarcinoma: a case report.

BACKGROUND: Filiform polyposis is a rare form of inflammatory polyposis, which is occasionally formed in the colon of patients with history of inflammatory bowel disease (IBD). It is characterized by presence of several to hundreds of slender, worm-like polyps in the colon lined by histologically normal colonic mucosa and often coalesce, resulting in a tumor-like mass. Filiform polyposis is most frequently associated with a post-inflammatory reparative process in patients with IBD history, and only cases of filiform polyposis occurring in patients without IBD history have been reported. Filiform polyposis has been considered as a benign inflammatory polyposis without any risk of dysplasia, while the possibility of carcinogenesis of inflammatory polyps is not fully excluded. To date, only three cases of filiform polyposis coexisting with dysplasia have been reported. CASE PRESENTATION: A 59-year-old male patient with no past medical history of IBD underwent laparoscopic sigmoidectomy for obstructive filiform polyposis, which was associated with sigmoid colon adenocarcinoma. Based on the histological findings of the resected specimen, invasive sigmoid colon adenocarcinoma was surrounded by filiform polyposis, and adenocarcinoma also scattered uniformly on the surface of filiform polyposis. In immunohistochemistry, abnormal p53 expression was observed in adenocarcinoma, while it was not shown in mucosa on filiform polyposis. CONCLUSIONS: This is the fourth case of filiform polyposis that is closely associated with colon dysplasia or adenocarcinoma based on histological findings. However, immunohistochemical findings did not support the theory that inflammation initiates adenocarcinoma in filiform polyposis like IBD. Hence, further immunohistochemical and genetic analyses are needed to clarify the association between filiform polyposis and carcinogenesis.

Intravascular large B-cell lymphoma involving large blood vessels, three autopsy cases.

Although intravascular large B-cell lymphoma (IVLBCL) is an extranodal lymphoma characterized by the selective growth of lymphoma cells within the lumina of small vessels, we here report three autopsy cases of IVLBCL characterized by the proliferation within large blood vessels. These three cases were diagnosed as IVLBCL of the bone marrow or skin biopsy. Two cases died suddenly before treatment, whereas the other died during treatment. Autopsies showed a large embolus of dense lymphoma cells extending from the truncus pulmonalis to the pulmonary arteries in Case 1, emboli of lymphoma cells in the aorta and carotis in Case 2, and a mass of lymphoma cells blocking the lumen of the aortic arch in Case 3. This is the first report of IVLBCL involving large blood vessels, and it is essential to note that this type of IVLBCL might cause sudden death because of tumor emboli within large blood vessels.

Pharmacokinetics of anticoagulants apixaban, dabigatran, edoxaban and rivaroxaban in elderly Japanese patients with atrial fibrillation treated in one general hospital.

Steady-state plasma concentrations of anticoagulants and the time since the previous administration in mainly outpatients with atrial fibrillation administered standard or reduced doses were analyzed for 110 elderly Japanese subjects (mean age, 76 years) treated with apixaban (2.5 or 5.0 mg twice daily), dabigatran etexilate (110 or 150 mg twice daily), edoxaban (30 or 60 mg once daily) or rivaroxaban (10 or 15 mg once daily) at one general hospital. The pharmacokinetics in patients treated with standard and reduced doses of the four anticoagulants using liquid chromatography-tandem mass spectrometry was compared with the concentration ranges estimated using physiologically based pharmacokinetic modeling. Reduced doses of anticoagulants resulted in relatively small pharmacokinetic variations compared with the standard dose. Statistical analyses revealed that renal impairment is likely not the sole determinant factor for high plasma concentrations of apixaban, dabigatran, edoxaban and rivaroxaban. Patients with atrial fibrillation should be treated with the correct doses of oral anticoagulants as specified in the package inserts (e.g. reduced doses for elderly patients, patients with low body weights and in combination with P-glycoprotein inhibitor drugs) to avoid excessive or insufficient doses of direct oral anticoagulants.

Serous borderline tumor with micropapillary pattern of the right ovary that developed 6 recurrences over 30 years after primary surgery.

Serous borderline tumors (SBTs) are nonaggressive and have excellent prognosis. Furthermore, SBTs with micropapillary pattern (SBT-MP) are known to be associated with a higher recurrence rate, microinvasions and invasive implants compared to typical SBTs, and these characteristics have adverse effects on prognosis. Here, we report a case of SBT with micropapillary pattern (SBT-MP) that developed 6 recurrences over 30 years after primary surgery. The patient was a 70 year-old woman. At 41 years of the age she underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, partial omentectomy and pelvic lymphadenectomy and was found to have an SBT-MP involving the right ovary (International Federation of Gynecology and Obstetrics 2014, stage IC2). She was administered chemotherapy (cyclophosphamide, adriamicin, and cisplatin). She repeatedly developed recurrences 6 times after primary surgery. A left inguinal recurrence at age 55, a right inguinal recurrence at age 56, a right inguinal recurrence at age 64, an umbilical recurrence at age 65, a right inguinal recurrence at age 68 and left axillary recurrence at age 70. Histopathological examinations revealed that all recurrences were SBT-MP with noninvasive implants. Our case strongly justifies the belief that recurrent SBTs carry an excellent prognosis unless they develop significant malignant transformation.

Richter's Type of Incarcerated Obturator Hernia that Presented with a Deep Femoral Abscess: An Autopsy Case Report.

BACKGROUND Richter's obturator hernia is a rare abdominal hernia that is difficult to diagnose. The purpose of this case report is to show an unusual presentation of a fatal Richter's obturator hernia that was accompanied by a femoral abscess. CASE REPORT An 89-year-old woman complained of sudden left coxalgia and a gait disorder but no abdominal symptoms. She had no history of trauma or surgery in the inguinal area. Twenty-three days after her first complaint of coxalgia, the patient was admitted in a coma with necrotizing fasciitis in the left inguinal area. The patient died of asystole due to hyperkalemia. During surveillance for the cause of death, a Richter's type of incarcerated obturator hernia was identified at autopsy. The incarcerated small intestine had penetrated into the left obturator foramen to form an abscess that extended into the deep femoral region. CONCLUSIONS Obturator hernia accompanied by femoral abscess is extremely rare, but it should be suspected when a patient with no history of trauma or surgery has a femoral abscess, even in the absence of abdominal symptoms.

Thyroid gland metastasis of rectal cancer.

A 72-year-old woman with a history of rectal cancer was admitted to our hospital to undergo thyroidectomy and left adrenalectomy. She had undergone low anterior resection and regional lymph node dissection for rectal cancer 52 months pre-admission (T3 N1 M0, stage IIIb according to International Union Against Cancer tumor-node-metastasis), and she had also undergone metastasectomy for lung metastases and right adrenal gland metastasis after the rectal surgery. Follow-up computed tomography scans detected nodules in the bilateral lobes of the thyroid gland and in the left adrenal gland. Subtotal thyroidectomy and left adrenalectomy were performed, and pathological examination revealed metastases of rectal cancer to the thyroid gland and left adrenal gland.

[A case of metastatic spermatic cord tumor from acute myelogenous leukemia].

A 60-year-old man with a history of acute myelogenous leukemia (AML) presented with a complaint of right groin mass. Three years ago the patient underwent an allogeneic bone marrow transplantation. Owing to successful engraftment, remission was achieved for three years. Radiological examinations showed tumor in the groin area along the right spermatic cord (diameter, 30 mm). Right high inguinal orchiectomy was performed. On preoperative tests, there was no other organ metastasis and no recurrence of AML. Pathological examination demonstrated an invasion of AML in the spermatic cord. It was considered that this case was an isolated extramedullary relapse of AML in the spermatic cord after allogeneic bone marrow transplantation. 21 months later, he is alive with no recurrence of AML. Isolated extramedullary relapses of AML after bone marrow transplantation has not been reported, it is possible that this is the first case of metastatic spermatic cord tumor from AML in Japan.

Pulmonary tumor with notochordal differentiation: report of 2 cases suggestive of benign notochordal cell tumor of extraosseous origin.

Intraosseous benign notochordal cell tumor (BNCT) is a lesion postulated to be of notochordal cell origin. BNCT has recently been recognized as a potential precursor of classic chordoma, a rare malignant neoplasm usually presenting in the sacrococcygeal region, skull base, or mobile spine. Extra-axial chordoma is extremely rare, and only 2 cases of pulmonary chordoma have been reported previously. We describe herein 2 cases of hitherto-unreported lung tumors that were diagnosed as BNCT. The patients were a middle-aged asymptomatic man and woman who were each incidentally found to have a 15-mm pulmonary nodule on computed tomography. They underwent surgical resection of the tumors under a diagnosis of probable benign tumor of uncertain nature. Histopathologically, both tumors showed solid sheets of peculiar adipocyte-like univacuolated cells, multivacuolated cells, and less vacuolated cells with small, round nuclei and mildly eosinophilic cytoplasm. Mitosis was absent. These features were typical of BNCT. Immunohistochemically, the tumor cells in both cases were positive for brachyury, a transcription factor essential for notochordal cell differentiation and for other markers of notochordal cells including cytokeratins, vimentin, and S-100 protein. Postoperatively, extensive radiographic examination of the whole body revealed no evidence of a primary tumor elsewhere, and both patients are alive and well, with no evidence of disease 1 year after surgery. These 2 cases raise the possibility of a new explanation for the histogenesis of extra-axial chordomas: BNCT may be a precursor lesion of not only conventional axial chordoma but also of extra-axial chordoma.

Pulmonary sclerosing hemangioma with pleural dissemination: report of a case.

Pulmonary sclerosing hemangioma is relatively rare and is usually considered a benign tumor. There have been no reports of pulmonary sclerosing hemangioma with pleural dissemination. This report presents an extremely rare case of pulmonary sclerosing hemangioma with pulmonary dissemination. A 57-year-old woman was found to have an abnormal shadow in the right lower lung field on chest X-ray. Chest computed tomography (CT) indicated a 2.5-cm mass in the right lower lobe. A bronchoscopic biopsy failed to identify malignant cells, which led to the patient undergoing an excisional lung biopsy. Intraoperative findings showed a tumor in the right lower lobe with multiple small nodules in the pleura. The pathological findings revealed that the tumor was sclerosing hemangioma with pleural dissemination. Annual follow-up CT showed irregular pleural thickness, which suggested progressive dissemination 3 years after the operation. Although pulmonary sclerosing hemangioma is regarded as a benign tumor, the potential for malignancy may be a consideration in this setting.

Mediastinal teratoma complicated with acute mediastinitis.

We report a case of a mediastinal teratoma associated with acute mediastinitis that required an emergency operation. These tumors cause a variety of complications, but reports of acute mediastinitis are rare. A 24-year-old woman was admitted to our hospital for complaints of chest pain and fever and was subsequently diagnosed as having an anterior mediastinal tumor. Follow-up computed tomography showed rapidly progressing acute mediastinitis, which was diagnosed as a perforation of the teratoma. We performed emergency surgical extirpation of the tumor and mediastinal drainage. The histopathologic diagnosis was a mature teratoma that included pancreatic tissue. Although the apparent site of the rupture was not obvious, there was a wide area of acute inflammation in the mediastinal adipose tissue. The patient did well and was discharged from the hospital without major complications.

Malignant pleural mesothelioma: clinicopathology of 16 extrapleural pneumonectomy patients with special reference to early stage features.

The earliest pathological events in the development of malignant pleural mesothelioma (MPM) are not understood. The aim of the present study was to elucidate the early histopathological features of MPM. A total of 16 extrapleural MPM pneumonectomy patients were investigated. Early stage mesothelioma was arbitrarily defined as a tumor < or =5 mm in thickness regardless of the nodal status or other organ involvement. Eight of these patients (six with epithelioid, two with biphasic) had early stage mesothelioma by this definition. Macroscopically there was no visible tumor, but the parietal and visceral pleura were thickened and there was focal adhesion between them. Microscopically, the mesothelioma lesions were multifocal and discontinuous on the pleura. In extremely early cases of epithelioid mesothelioma, tumor cells were generally arrayed in a single layer, but papillary proliferation was observed elsewhere. In sarcomatoid mesothelioma, mesothelioma cells proliferated, forming multiple small polypoid nodules on the pleura. Epithelial membrane antigen was helpful to distinguish reactive from neoplastic mesothelium, but glucose transporter-1 was not. Mesothelioma cells disseminate diffusely throughout the parietal and visceral pleura and mediastinal fat tissue before becoming visible. Stage Ia mesothelioma (neoplasm limited to the parietal pleura) would not be observed in daily practice.

Hyperactivated STAT3 in ALK-positive diffuse large B-cell lymphoma with clathrin-ALK fusion.

Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma is a rare subtype of diffuse large B-cell lymphoma (DLBCL). Although a few cases of ALK-positive large B-cell lymphoma harbor nucleophosmin-ALK chromosomal translocation similar to ALK-positive anaplastic large cell lymphoma, most reported cases are characterized by t(2;17)(p23;q23) involving the clathrin gene. Here, we report 2 cases of ALK-positive DLBCL. The 2 cases presented similar morphologic features and immunohistochemical characteristics, that is, positivity for ALK, IgA, CD138, and MUM1; weak positivity for CD30 and CD79a; and negativity for CD20. The clathrin-ALK transcript was identified by reverse transcription-polymerase chain reaction, and the sequence was determined by direct sequencing. Recently, the essential role of STAT3 activation as well as STAT 5 activation in nucleophosmin-ALK fusion protein-mediated lymphomagenesis was reported. However, differential effects of ALK-fusion variant proteins on proliferation, transformation, and invasion properties were reported. Thus, we evaluated the phosphorylation status of STAT 3 and STAT 5, and found highly hyperphosphorylated STAT 3 on tyrosine 705 but not STAT 5 in our 2 cases of ALK-positive DLBCL with clathrin-ALK fusion. Furthermore, STAT 5A expression was not detected in either of the ALK-positive DLBCL cases, although 11 of the 36 ALK-negative DLBCL cases revealed STAT 5A expression. Expression of the antiapoptotic proteins survivin and BCL-X(L), which were believed to be the targets of STAT 3, was investigated. However, there were no significant associations between expression of survivin or BCL-X(L) and ALK positivity among the diffuse large B-cell lymphomas. In summary, similar signaling transduction mechanism involving STAT proteins seems to underlie DLBCL harboring the clathrin-ALK or nucleophosmin-ALK fusion gene.

Aberrant expression of human ortholog of mammalian enabled (hMena) in human colorectal carcinomas: implications for its role in tumor progression.

The human ortholog of mammalian enabled (hMena), a family of enabled/vasodilator-stimulated phosphoprotein (Ena/VASP), is an actin regulatory protein involved in the regulation of cell motility. Increasing evidence suggests that hMena over-expression is involved in human breast cancers, whereas the significance of hMena expression in colorectal carcinomas remains to be elucidated. In this study, we assessed the relative mRNA level of hMena using real-time PCR, showing that there is a statistically significant increase of hMena transcripts in matched human colorectal carcinomas and adjacent non-neoplastic colorectal epithelium (n=6, P=0.046). We also performed immunohistochemical analysis of the expression of hMena protein in 50 cases of paraffin-embedded archival colorectal tissues, and found that an elevated hMena expression is correlated to the cases with advanced TNM stages of colorectal carcinomas (P<0.001). On further inspection of immunohistochemical features of each specimen, we observed intensified hMena staining in the invasive front of colorectal carcinomas, especially in tumor budding, a transition from glandular structure to single or small clusters of cells at the invasive front. We demonstrated that there was a significantly increased hMena staining in the tumor budding as compared with more morphologically-differentiated areas of colorectal carcinomas, indicating that hMena over-expression may have a role in the initial steps of tumor invasion from primary sites. We performed in vitro motility assays to show that transient hMena transfection markedly enhanced the chemotactic/chemokinetic activity of HeLaS3 cells (P<0.001). Taken together, these results suggest that hMena over-expression is implicated in the progression of colorectal carcinomas by positively affecting the migratory phenotype of cancer cells.

Carbon ion radiation therapy for primary renal cell carcinoma: initial clinical experience.

PURPOSE: Renal cell carcinoma (RCC) is known as a radioresistant tumor, and there are few reports on radiotherapy for primary RCC. We evaluated the efficacy of carbon ion radiotherapy (CIRT) for patients with RCC. METHODS AND MATERIALS: Data for patients with RCC who received CIRT were analyzed. A median total dose of 72 GyE (gray equivalents) in 16 fractions was administered without any additional treatment. Clinical stage was determined based on TNM classification by the International Union Against Cancer (UICC). Local recurrence was defined as definite tumor regrowth after treatment. RESULTS: Data for 10 patients were included in the analyses, including 7 patients with Stage I and 3 patients with Stage IV (T4NxM0 or TxN2M0) disease. The median maximum diameter of the tumor was 43 mm (24-120 mm). The median follow-up for surviving patients was 57.5 months (9-111 months). The 5-year local control rate, progression-free survival rate, cause-specific survival rate, and overall survival rates were 100%, 100%, 100%, and 74%, respectively. Interestingly, treated tumors showed very slow shrinkage, and the tumor in 1 case has been shrinking for 9 years. One patient with muscular invasion (T4 tumor) developed Grade 4 skin toxicity, but no other toxicity greater than Grade 2 was observed. CONCLUSIONS: This is one of the few reports on curative radiotherapy for primary RCC. The response of the tumor to treatment was uncommon. However despite inclusion of T4 and massive tumors, favorable local controllability has been shown. The results indicate the possibility of radical CIRT, as well as surgery, for RCC.