Two cases of airway-centered fibroelastosis treated with an antifibrotic agent and corticosteroids.

Airway-centered fibroelastosis is characterized by peribronchovascular fibroelastosis, predominantly in the upper lobes, with little-to-no pleural involvement. In this study, we describe two cases of airway-centered fibroelastosis diagnosed based on radiological and pathological findings. The first case comprised a 44-year-old man whose forced vital capacity improved over three months following treatment with nintedanib. The second case involved a 50-year-old woman who was treated with oral corticosteroids but yielded an unfavorable outcome. An effective treatment for airway-centered fibroelastosis has not yet been identified; therefore, this study may help contribute to a more thorough discussion regarding treatment strategies for this disease.

Bladder Preservation After Bilateral Kidney Loss and Bladder Cancer After Renal Transplantation: A Case Report.

Kidney transplantation is now a well-established renal replacement therapy. However, renal transplant recipients are reported to have an increased incidence of cancer. Although the recommended waiting period after each cancerous event in a recipient is indicated in the literature, there is no absolute certainty that cancer will develop even after the recommended waiting period. In this study, we experienced a case of bladder cancer after the recommended waiting period in a patient who had bladder preservation after a right nephrectomy and left nephroureterectomy. A 61-year-old man lost his right kidney due to renal cancer in 2007 and his left kidney to urothelial carcinoma in November 2017. The patient wanted a kidney transplant and bladder preservation at the time of the left nephroureterectomy. The patient's wife offered to donate a kidney. After 2 years of hemodialysis, there was no recurrence or metastasis, and with the approval of the Ethics Committee, the patient received a kidney transplant in January 2020. Although the patient's renal function was good after the transplant, a bladder tumor was found 20 months later and was resected transurethrally. The pathology was nonmuscle invasive bladder cancer. This patient, who had lost both kidneys, was treated with bladder preservation therapy. After subsequent kidney transplantation, he developed bladder cancer. Explaining to the patient the possibility of recurrence after a certain period and the increased risk of cancer, in-depth consultation with the patient is necessary regarding bladder preservation. Regular checkups should be continued after transplantation.

PHOX2B is a Sensitive and Specific Marker for the Histopathological Diagnosis of Pheochromocytoma and Paraganglioma.

Pheochromocytomas (PCCs) and paragangliomas (PGLs) are non-epithelial neuroendocrine neoplasms originating from the adrenal medulla and paraganglion of the sympathetic and parasympathetic nervous system, respectively. PCCs and PGLs show histological similarities with other epithelial neuroendocrine neoplasms and olfactory neuroblastomas (ONBs), and the differential diagnosis of PGLs is particularly difficult. Therefore, we compared the sensitivity of PHOX2A, PHOX2B, and tyrosine hydroxylase (TH) in the histopathological diagnosis of PCCs and PGLs immunohistochemically using the tissue microarrays of 297 neoplasms including PCCs, PGLs, neuroblastomas, ganglioneuromas, epithelial neuroendocrine neoplasms, and ONBs. Using cutoff values of 25%, 5%, and 5% of tumor cells expressing PHOX2A, PHOX2B, and TH, respectively, as positive, 40 of 51 PCCs, 32 of 33 parasympathetic/head and neck PGLs (HNPGLs), 17 of 19 sympathetic/thoracoabdominal PGLs (TAPGLs), and 12 of 152 epithelial neuroendocrine neoplasms, including 123 well-differentiated and 29 poorly differentiated neuroendocrine neoplasms, were PHOX2A-positive. All 51 PCCs, 33 HNPGLs, and 19 TAPGLs were PHOX2B-positive, while all 152 epithelial neuroendocrine neoplasms were PHOX2B-negative. Moreover, 50 of 51 PCCs, 13 of 33 HNPGLs, all TAPGLs, and 12 of 152 epithelial neuroendocrine neoplasms were TH-positive. All ONBs were negative for PHOX2A, PHOX2B, and TH. PHOX2B was the most sensitive and specific diagnostic marker for PCCs and PGLs among PHOX2A, PHOX2B, and TH. PHOX2B can facilitate identification of PCCs and PGLs from epithelial neuroendocrine neoplasms and ONBs, especially in the case of HNPGLs, in which TH is often negative.

Multiparametric magnetic resonance imaging of plasmacytoid urothelial carcinoma with histopathological correlation: A case report.

Plasmacytoid urothelial carcinomas of the bladder are rare, aggressive variants with a poor prognosis. Few reports have described the correlation of histopathological features with multiparametric magnetic resonance imaging findings in the local staging of plasmacytoid urothelial carcinoma. An 82-year-old woman with hematuria was referred to our hospital. Magnetic resonance imaging showed diffuse bladder wall thickening, with different signal intensities in the 2 layers-inner and outer. This case suggests that the presence of diffuse bladder wall thickening and varying signal intensities in the 2 layers could aid in the local staging of plasmacytoid urothelial carcinoma. A thickened bladder wall with restricted diffusion suggests tumor invasion, indicating that the tumor can invade the organ in contact with the thickened bladder wall.

A case of cystic mucinous adenocarcinoma of the prostate treated by robot-assisted radical prostatectomy.

A 66-year-old male visited with constipation. Intra-pelvic cystic mass (332 cm3) was found, and PSA was 32.4 ng/ml. Biopsy of the prostate was performed. It wasn't adequate specimen for diagnosis, but the PSA of the fluid was 4791.0 ng/ml. RARP was performed. To make surgical field more visible, fine needle was stuck from the patient's perineum into the cyst intraoperatively. The patient was discharged with nothing complications. It was mucinous adenocarcinoma of the prostate, pT3b, RM1. The patient is now receiving ADT but no recurrence and metastasis are seen to date.

Pregnancy-onset thrombotic thrombocytopenic purpura with nephrotic syndrome: a case report.

Pregnancy-onset thrombotic thrombocytopenic purpura (TTP) was reported by many obstetricians and hematologists, but less by nephrologists, and the detailed clinical course of its renal complication is not known. Here, we report a case of a 33-year-old pregnant woman who suffered from pregnancy-onset TTP with nephrotic syndrome which was controlled by the termination of pregnancy. On admission, she had periorbital and lower leg edema at 32 weeks of gestation. Her serum albumin level was 2.8 g/dL and the urine protein/creatinine ratio was 4.1 g/g Cr. Besides those, she had thrombocytopenia, hemolytic anemia, and severe deficiency of A Disintegrin-like and Metalloproteinase with Thrombospondin type 1 motifs 13 (ADAMTS-13) activity. Thus, she was diagnosed with nephrotic syndrome due to pregnancy-onset TTP. A cesarean section was performed without complications for the patient and her baby. Then, all her symptoms improved shortly. She was suspected of congenital TTP because of no ADAMTS-13 inhibitor results and the persistent deficiency of ADAMTS-13 activity even after her condition improved. Pregnancy-onset TTP can cause nephrotic syndrome. Termination of pregnancy should be considered in cases with pregnancy-onset TTP to protect kidney function.

Aseptic Cavernosal Abscess: An Unrecognized Feature of Neutrophilic Dermatosis.

A 50-year-old man developed a sterile cavernosal abscess followed by prominent features of necrotizing neutrophilic dermatosis. We conducted a literature review, which revealed that aseptic abscesses in the corpus cavernosum occur in association with neutrophilic dermatosis. Patients with this condition frequently receive unnecessary antibiotic treatment and surgical interventions. Although this condition responds to systemic corticosteroids, the functional prognosis of the penis is poor. Abscess formation may be the initial presentation of neutrophilic dermatoses, and underlying conditions may even be absent. Clinicians need to be aware of this condition to distinguish it from bacterial infection and initiate early disease-specific treatments.

Two Cases of Rare Intratumoral Hemorrhage Following COVID-19 Vaccination.

The increase in the rate of mRNA vaccination against coronavirus disease 2019 (COVID-19) worldwide has been accompanied by reports of an increase in the side effects of the vaccine. In the field of neurosurgery, several cases of venous thrombosis have been reported as possible complications after COVID-19 vaccination. However, no such side effects have been reported in patients with brain tumors, and COVID-19 vaccination is considered safe for this patient population. In this report, we present the cases of two patients with brain tumors who experienced intratumoral hemorrhage as a possible side effect of the COVID-19 vaccine. In the first case, a 54-year-old man who had received CyberKnife treatment for a vestibular schwannoma eight years prior presented with tongue discomfort, right-side facial numbness, and dizziness since the day after his COVID-19 vaccination. MRI revealed intratumoral hemorrhage of the vestibular schwannoma. The second patient was a 60-year-old woman who presented with a sudden-onset headache and vomiting that had started three days after her COVID-19 vaccination. CT revealed a meningioma with intratumoral hemorrhage. Both patients had undergone surgery prior to this presentation, and their symptoms had improved. They had no risk factors for intratumoral hemorrhage, suggesting that it may be a side effect of the mRNA vaccine against COVID-19. Although the causal relationship is unclear, acute inflammation with predominantly lymphocytic infiltration and thrombogenicity after COVID-19 vaccination may damage the fragile microcirculation of the tumor.

Drug-induced hypersensitivity syndrome caused by minodronic acid hydrate.

BACKGROUND: Drug-induced hypersensitivity syndrome (DIHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an important adverse reaction caused by a few drugs. Reactivation of human herpesvirus 6 (HHV-6) is known to be associated with its pathogenesis. DIHS occasionally manifests as pulmonary lesions with a variety of imaging findings. CASE PRESENTATION: An 83-year-old woman started taking minodronic acid hydrate 5 years before admission. She noticed a generalized skin rash 44 days before admission and started oral betamethasone-d-chlorpheniramine maleate combination tablets for allergic dermatitis. She developed a fever and cough in addition to the rash, and was referred to our hospital. Laboratory data showed a high level of eosinophils and liver and biliary enzymes. Computed tomography (CT) studies revealed bilateral diffuse ground-glass opacities with ill-defined centrilobular nodules from the central to peripheral regions of the lungs. Transbronchial lung cryobiopsy specimens showed that lymphocyte infiltration was observed in the alveolar walls and fibrinous exudates and floating macrophages in the alveolar lumina. Immunohistochemistry of biopsy specimens showed more CD4+ lymphocytes than CD8+ lymphocytes, while few Foxp3+ lymphocytes were recognized. The serum anti-HHV-6 immunoglobulin G titer increased at 3 weeks after the first test. Based on these findings, we diagnosed her with DIHS. We continued care without using corticosteroids since there was no worsening of breathing or skin condition. Eventually, her clinical symptoms chest CT had improved. Minodronic acid hydrate was identified as the culprit drug based on the positive results of the patch test and drug-induced lymphocyte stimulation test. CONCLUSIONS: We described the first case of DIHS caused by minodronic acid hydrate. Lung lesions in DIHS can present with bilateral diffuse ground-glass opacities and ill-defined centrilobular nodules on a CT scan during the recovery phase. Clinicians should be aware of DIHS, even if patients are not involved with typical DIHS/DRESS-causing drugs.

A case of pulmonary sclerosing pneumocytoma diagnosed preoperatively using transbronchial cryobiopsy.

BACKGROUND: The preoperative diagnosis of pulmonary sclerosing pneumocytoma (PSP) is complicated since PSP has several histological structural patterns in the same neoplasm; hence, it is sometimes pathologically misdiagnosed as adenocarcinoma or carcinoid. In recent years, with the prevalence of transbronchial cryobiopsy (TBLC), we are able to obtain larger specimens than previously. However, to date, there have been no reports describing PSP diagnosed using TBLC. CASE REPORTS: A 43-year-old man was referred to our hospital for an abnormal lesion in the left lung discovered on routine health examination. A computed tomography scan of the chest revealed a 14-mm heterogeneous round nodule with surrounding ground-glass opacity in the left lower lobe. The tumor size increased to 18 mm in three weeks, and he developed bloody sputum. TBLC was performed using radial endobronchial ultrasonography and fluoroscopy. An occlusion balloon and prophylactic epinephrine were used to prevent severe bleeding. Histologically, epithelioid cells with solid proliferation, various papillary lesions, and hemosiderin-laden histiocytes were observed. Immunohistochemical staining revealed the histiocytes positive for thyroid transcription factor-1 and vimentin, and the type II pneumocyte-like-cells positive for cytokeratin 7. The tumor was preoperatively diagnosed as a PSP; the patient underwent left basal segmentectomy and consequently, a final diagnosed of PSP was formulated. CONCLUSION: We report the first case of PSP preoperatively diagnosed using TBLC. Therefore, cryobiopsy could be beneficial in the preoperative diagnosis of PSP.

Utility of radial endobronchial ultrasonography combined with transbronchial lung cryobiopsy in patients with diffuse parenchymal lung diseases: a multicentre prospective study.

BACKGROUND: Radial endobronchial ultrasonography (R-EBUS) has been used in conjunction with transbronchial lung cryobiopsy (TBLC) to diagnose diffuse parenchymal lung disease (DPLD) and to decrease the risk of bleeding complications. The diagnostic utility of different R-EBUS signs, however, remains unknown. OBJECTIVES: This study aimed to determine whether different R-EBUS signs could be used to more accurately diagnose DPLD and whether bronchial bleeding could be prevented with use of R-EBUS during TBLC. METHOD: Eighty-seven patients with DPLD were included in this multicentre prospective study, with 49 patients undergoing R-EBUS. R-EBUS signals were characterised as displaying either dense or blizzard signs. Pathological confidence of specimens obtained from TBLC was compared between patients with dense versus blizzard signs, and severity of bronchial bleeding was determined based on whether R-EBUS was performed or not. RESULTS: All patients with dense signs on R-EBUS showed consolidation on high-resolution CT (HRCT) imaging. Pathological confidence of lung specimens was significantly higher in patients with dense signs versus those with blizzard signs (p<0.01) and versus those who did not undergo R-EBUS (p<0.05). Patients who underwent TBLC with R-EBUS were more likely to experience no or mild bronchial bleeding than patients who did not undergo R-EBUS (p<0.01), with shorter procedure times (p<0.01). CONCLUSIONS: The dense R-EBUS sign corresponded with consolidation on HRCT. High-quality lung specimens may be obtainable when the dense sign is observed on R-EBUS, and R-EBUS combined with TBLC may reduce risk of bronchial bleeding and shorten procedure times.

A rare case of gallbladder cancer with multiple metastases to the colon.

Gallbladder cancer is often diagnosed with metastasis and is known to have poor prognosis. Although, gallbladder cancer often metastasizes to liver, lung, or lymph nodes, metastasis to gastrointestinal tract is not common. Several autopsy studies reported colorectal metastasis from gallbladder cancer, but most of these cases were supposed to be gastrointestinal invasion from peritoneal dissemination. We experienced a rare case of hematogenous colon metastasis from gallbladder cancer. Colonoscopy for a 76-year-old man who was diagnosed as gallbladder cancer revealed two 5-mm flatly elevated lesions with central erosion in the transverse and sigmoid colon. Endoscopic mucosal resection (EMR) revealed poorly differentiated adenocarcinoma in hematoxylin and eosin staining. Additional immunohistochemistry examination showed strongly positive CK7 expression with negative CDX2, and suggested colon metastases from gallbladder cancer. Despite the positive vertical margin in EMR specimens, autopsy finding denied colorectal invasion from peritoneal dissemination and proved vascular spread colon metastases. Gastrointestinal metastasis is often misdiagnosed as a primary tumor, and thus, it is important to recognize gallbladder cancer as a potential origin of gastrointestinal metastasis.

Clinical impact of the radiological indeterminate for usual interstitial pneumonia pattern on the diagnosis of idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease associated with significant morbidity and mortality. The international clinical practice guidelines for the diagnosis of IPF have recently been revised. METHODS: In this single-center retrospective study conducted between June 2006 and March 2018, 27 patients with a newly classified indeterminate for usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) who had undergone surgical lung biopsy were enrolled at the Japanese Red Cross Medical Center. Clinical and pathological characteristics and prognosis were retrospectively analyzed from patient records. RESULTS: On the basis of multidisciplinary discussion (MDD), IPF was diagnosed in six patients (22%), unclassifiable interstitial pneumonia in 5 (19%), chronic hypersensitivity pneumonitis in 10 (37%), collagen vascular disease-associated interstitial lung disease in 5 (19%), and lymphoproliferative disorder in 1 (4%) patient. Ground-glass opacity, peribronchovascular distribution, upper or middle lobe distribution, mosaic attenuation, consolidation patterns, and honeycombing were found on HRCT. Histological UIP or probable UIP was observed in seven patients. The median survival time from the initial visit was 2770 days (92.3 months). There was a significant difference in survival time in the GAP stage and honeycombing on HRCT according to the log-rank test. CONCLUSIONS: Patients with an indeterminate for UIP pattern on HRCT were more likely to have non-IPF than IPF through pathological diagnosis and MDD. GAP stage and honeycombing on HRCT may be significant risk factors for all-cause mortality.

[A CASE OF IgG4-RELATED DISEASE OF THE URETER DIAGNOSED BY TRANSVAGINAL ULTRASOUND-GUIDED BIOPSY].

IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder that systemically causes tissue fibrosis due to infiltration of IgG4-positive plasma cells. Here, we reported a rare case of ureteral IgG4-RD that formed a nodular lesion and diagnosed by trans-vaginal ultrasound-guided needle biopsy.A 72-year-old woman presented with loss of appetite. The patient underwent Computed Tomography (CT), and she was pointed out the thickening of the left side bladder wall. So we performed a transurethral bladder biopsy under lumber anesthesia, but histopathological findings were almost normal. After that, she developed pyelonephritis repeatedly. We performed CT again. A CT revealed a nodular lesion at the end of her left ureter and hydronephrosis. The tumor was gradually getting larger. So we performed placement the ureteral stent for urinary tract obstruction. Left ureteral urine cytology was classIIIa. We performed transvaginal ultrasound needle biopsy for the nodular lesion of the left ureter. Histopathological findings showed infiltration of lymphocytes and fibrosis and infiltration of IgG4 positive plasma cells: the ratio of IgG4/IgG positive cells>0.6, 30>IgG4 positive plasma cells/high power field. The serum IgG and IgG4 levels were also elevated 1,943 and 210 mg/dl. We finally diagnosed IgG4-RD of the ureter and started using steroid for her treatment. One month later, the tumor had reduced after steroid treatment. The ureteral stent was removed. Since then, recurrent ureteral obstruction of the left ureter has not occurred.IgG4-RD of the ureter with nodular type is rare, and the imaging findings are similar to malignant tumors. Accurate diagnosis is very important to rule out malignancy. In our case, transvaginal needle biopsy was helpful to reach final diagnosis.

Engrailed Homeobox 1 and Cytokeratin 19 Are Independent Diagnostic Markers of Eccrine Porocarcinoma and Distinguish It From Squamous Cell Carcinoma.

OBJECTIVES: The diagnostic utility of En1 in the histopathologic differentiation of eccrine porocarcinoma (EPC) from invasive squamous cell carcinoma (SCC) was investigated. METHODS: Expression of En1 and CK19 in 16 cases of EPC was immunohistochemically examined and compared with that in 32 cases of SCC. RESULTS: In all 16 EPCs, En1 was expressed in 3% to 100% of tumor cells. In 20 of the 32 SCCs, En1 was expressed in 3% to 90% of tumor cells. A total of 13 of the 16 EPCs and five of the 32 SCCs were judged as En1 positive, with a cutoff value of 25%. In addition, 11 of the 16 EPCs and four of the 32 SCCs were CK19 positive. The frequencies of En1- and CK19-positive cases were significantly higher in EPCs than in SCCs. In a logistic regression analysis for predicting EPC, En1 and CK19 were independent markers. When expression patterns of En1 and CK19 were combined, none of the 32 SCCs was both positive. In contrast, 15 of the 16 EPCs were positive for either En1 or CK19. CONCLUSIONS: A combination of En1 and CK19 expression can improve the accuracy of histologic diagnosis of EPC.

Efficacy and safety of semi-flexible thoracoscopic cryobiopsy in the diagnosis of elderly tuberculous pleurisy.

BACKGROUND: Thoracoscopic pleural biopsy is an efficient procedure in patients with undiagnosed exudative pleurisy. Rigid or flexible forceps have been widely used for this procedure. Recently, the use of cryo-techniques was reported in pleural biopsy during semi-rigid thoracoscopy; however, the feasibility and safety of pleural cryobiopsy in elderly patients have not yet been fully elucidated. CASE REPORTS: We describe two elderly patients who safely underwent semi-rigid thoracoscopic cryobiopsy and were diagnosed with tuberculous pleurisy. Both were >85 years of age, and chest auscultation revealed reduced breath sounds in the right lower zones. Laboratory investigations revealed an elevated level of C-reactive protein without leukocytosis in both patients. Computed tomography scan of the chest revealed right pleural effusion in both patients. Pleural fluid biochemical analysis results were indicative of an exudate. Sputum cultures demonstrated no bacterial growth and smears were negative for the presence of acid-fast bacilli. For definitive diagnosis, pleural biopsy was performed via thoracoscopic cryobiopsy. Specimens obtained from the cryoprobe demonstrated 200-300-µm caseating and non-caseating epitheloid cell granulomas with Langerhans type giant cells. Based on the above results, both patients were diagnosed with TB pleurisy. Anti-tuberculosis treatment resulted in good clinical outcome in both patients. CONCLUSION: Cryobiopsy is easier and more efficient than biopsy with conventional forceps. Our findings in these patients suggest that semi-rigid thoracoscopic cryobiopsy might be a useful alternative diagnostic method for undiagnosed pleural effusion in elderly patients.

Management and utility of transbronchial lung cryobiopsy in Japan.

BACKGROUND: Transbronchial lung cryobiopsy (TBLC), which is transbronchial lung biopsy performed using a cryoprobe, has emerged as a new method for obtaining lung tissue specimens for diagnosing pulmonary diseases. TBLC has been widely performed, and many reports have documented its safety and feasibility in diagnosing pulmonary diseases such as lung cancer and diffuse parenchymal lung disease. However, to date, no reports have yet been published from Japan. Therefore, the purpose of this study was to describe our experience with TBLC in daily practice in Japan and compare it with those reported in previous studies from other countries. We also evaluated the efficacy and feasibility of TBLC. METHODS: We retrospectively evaluated the clinical records of 50 consecutive patients who had undergone TBLC between November 2017 and May 2018 at the Japanese Red Cross Medical Center. Demographic data, procedure details, and the diagnostic yield were analyzed. RESULTS: We analyzed the records of 50 patients who underwent diagnostic TBLC. Their median age was 71 years, and they included 34 men (68%). The median maximal diameter of the cryobiopsy specimens was 5.3 mm (range, 2.0-23.0 mm), and the median area of the cryobiopsy specimens was 15.5 mm2 (range, 3.0-136.5 mm2). The rate of obtaining adequate specimens was 91%, and the pathological diagnostic yield was 76%. No complications were observed, except for one case of pneumonia. CONCLUSIONS: This is the first report from Japan analyzing the efficacy and feasibility of TBLC in daily clinical practice. TBLC was considered efficacious, safe, and feasible for Japanese patients with various lung diseases.

Nivolumab and stereotactic radiation therapy for the treatment of patients with Stage IV non-small-cell lung cancer.

BACKGROUND: Radiation therapy might modify the cancer immune environment to enhance the antitumor effect of immune checkpoint inhibitors. We performed a feasibility study of nivolumab following stereotactic radiation therapy for chemotherapy pretreated advanced non-small-cell lung cancer. PATIENTS AND METHODS: Pretreated advanced/recurrent non-small-cell lung cancer patients received stereotactic radiation therapy to one of the disease sites. Nivolumab at a dose of 3 mg/kg was given within 2 weeks after the completion of stereotactic radiation therapy and continued every 2 weeks thereafter until disease progression or unacceptable toxicities. The primary endpoint was the occurrence rate of Grade 3 pneumonitis (within 12 weeks) or other non-hematological toxicity (within 8 weeks). RESULTS: From September 2016 to September 2017, six patients were enrolled. Five received stereotactic radiation therapy to their primary lesions. All patients received nivolumab on the following day after stereotactic radiation therapy completion. Grade 3 pneumonitis occurred in one patient, but no other serious adverse events were reported for the other patients. One complete response and two partial responses were achieved. Four patients had measurable lesions outside the irradiated area, of whom three patients responded to the treatment. The initial progression sites were mainly outside the irradiated field, including one brain metastasis. CONCLUSIONS: Nivolumab therapy immediately following stereotactic radiation therapy was well tolerated. This sequential combination warrants further study.