Concordance between HokUS-10 Scoring and Transjugular Liver Biopsy for the Diagnosis of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Allogeneic Stem Cell Transplantation.

The aim of this study was to evaluate the concordance between clinical diagnosis and pathologic findings of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) in post-hematopoietic stem cell transplantation recipients and to investigate the accuracy of the HOKUS-10 score in diagnosing VOD/SOS. We included 13 patients who underwent transjugular liver biopsy for clinical suspicion of VOD/SOS and collected their clinical, laboratory, imaging, and pathologic data. Eleven patients were confirmed to have VOD/SOS by pathologic examination. The median HokUS-10 score and hepatic venous pressure gradient were 6 points (range, 0 to 10 points) and 13 mmHg (range, 7 to 24 mmHg), respectively. There was no significant difference between these scores in VOD/SOS and non-VOD/SOS cases; however, patients with lower HokUS-10 scores tended to have milder histologic features of VOD/SOS compared with severe cases. This study highlights the potential discordance between clinical diagnosis and pathologic diagnosis of VOD/SOS and emphasizes the importance of liver biopsy to optimize treatment.

FDG PET/CT in Primary Retroperitoneal Serous Carcinoma.

ABSTRACT: Primary retroperitoneal tumors are rare. Of these, primary retroperitoneal serous carcinoma is extremely rare, with only 15 cases having been reported. A 62-year-old woman presented with abdominal bloating. PET/CT demonstrated 2 tumors showing increased FDG uptake lateral and medial to the right common iliac vessels, without other abnormal uptake. A laparotomy revealed no ascites or abnormalities except a myomatous uterus in the peritoneal cavity. After surgical removal of these retroperitoneal tumors, the patient received adjuvant paclitaxel/carboplatin chemotherapy and remained well for 80 months. This case points to the significance of 18 F-FDG PET/CT in determining the nature of retroperitoneal tumors.

Immunohistological analysis of pathogenic infiltrates in the epidermis and liver of a patient with toxic epidermal necrolysis accompanied by vanishing bile duct syndrome.

Toxic epidermal necrolysis (TEN) is a severe cutaneous adverse drug reaction characterized by extensive epidermal detachment, which is reportedly mediated by drug-specific cytotoxic CD8+ T cells, inflammatory monocytes, and neutrophils. Besides the skin, TEN often damages other organs, and it remains unknown whether they are mediated by similar pathogenic cells that cause epidermal damage. We experienced a case who developed TEN complicated with vanishing bile duct syndrome. Immunohistological analysis revealed the infiltration of CD8+ T cells, inflammatory monocytes, and neutrophil extracellular trap-forming neutrophils in the lesions of both the skin and liver with different degree of infiltration of these cells. These data suggest a difference of dominant pathogenic cells between skin and liver of patients with TEN.

[Ovarian Carcinosarcoma Associated with Cowden Syndrome-A Case Report].

Cowden syndrome is a rare autosomal dominant disorder characterized by multiple hamartomas in various tissues, including the skin, mucous membranes, and gastrointestinal tract. Germline mutations of the PTEN tumor suppressor gene are responsible for Cowden syndrome. Cowden syndrome is associated with an increased risk of breast, thyroid, renal and uterine cancers; however, ovarian cancer rarely develops in women with Cowden syndrome, although somatic PTEN mutation often occurs in some types of ovarian carcinomas. Herein we report the first case of ovarian carcinosarcoma that developed in a woman with Cowden syndrome. A 55-year-old woman with a history of breast cancer, thyroid goiter, and palatal papillomatosis presented with pelvic distention. CT scan revealed a pelvic tumor suggesting ovarian cancer. She underwent a total abdominal hysterectomy, a bilateral salpingo-oophorectomy, and an omentectomy, but the surgical cytoreduction was suboptima( l >2 cm residual disease). Pathological examination showed a mixed tumor composed of high-grade carcinoma and heterologous sarcoma. Immunohistochemically, tumor cells were positive for p53. She was diagnosed with stage ⅢC ovarian carcinosarcoma. Genetic testing detected a PTEN variant, confirming the diagnosis of Cowden syndrome. She received paclitaxel/ carboplatin chemotherapy. However, no response was observed and she died of disease 2 months postoperatively.

Fatal thrombotic microangiopathy with rhabdomyolysis as an initial symptom after the first dose of mRNA-1273 vaccine: A case report.

We report a case of a Japanese man with severe rhabdomyolysis and multiple thrombosis of arterioles after the first dose of mRNA-1273 vaccine. He developed rapidly progressive rhabdomyolysis and infarctions of multiple organs. Antiplatelet factor 4 antibody test was negative. Despite the intensive supportive care, including aggressive fluid administration, hemodialysis, administration of anticoagulants, high-dose steroid, and eculizumab, the patient ultimately died of multiple organ failure. Autopsy revealed multiple thrombosis in the arterioles and organ necrosis. Low serum complements and C3 deposition in the renal glomeruli detected by immunofluorescence suggested a possible immune-mediated mechanism. To our knowledge, this is the first case report of rhabdomyolysis and multiple thrombosis of the arterioles as an adverse event following COVID-19 vaccination.

Long-term survival of a patient with uterine cancer-induced pulmonary tumor thrombotic microangiopathy following treatment with platinum-based chemotherapy and bevacizumab: A case report.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but fatal cancer-related disease. Owing to its non-specific findings, aggressive course, and lack of established treatment guidelines, only a few cases of antemortem diagnosis in long-term survivors have been reported. We aimed to report a case of uterine cervical cancer induced PTTM that was suspected based on pulmonary hypertension and successfully treated using combination chemotherapy despite of delayed diagnose. It is important to be aware that PTTM should be suspected when respiratory failure occurs in patients with unexplained pulmonary hypertension. Multidisciplinary treatments including molecular targeted therapies might be effective treatment options.

Bladder cancer invasion along a tension-free vaginal mesh.

INTRODUCTION: The effect of synthetic mesh after pelvic organ prolapse surgery on the progression of bladder cancer remains unclear. CASE PRESENTATION: A 79-year-old woman who underwent a tension-free vaginal mesh procedure 8 years prior was diagnosed with carcinoma in situ of the bladder. Although intravesical Bacillus Calmette-Guérin therapy was started, the tumor rapidly became muscle invasive. Laparoscopic radical cystectomy was performed following radiochemotherapy; however, the tumor extended to the left internal obturator muscle along the mesh arm. Pathological findings showed desmoplastic high-grade urothelial carcinoma infiltrating around the mesh. Finally, cancer recurred rapidly in the left internal obturator muscle. CONCLUSION: Synthetic mesh can become an abnormal anatomical pathway for tumor infiltration. Therefore, in high-risk bladder cancer patients who underwent a tension-free vaginal mesh procedure, radical cystectomy should be performed without delay before the tumor invades the perivesical tissue.

Positron Emission Tomography in Segmentectomy for cT1N0M0 Nonsmall Cell Lung Cancer.

BACKGROUND: This study was aimed to examine the significance of fluorodeoxyglucose positron emission tomography in predicting prognosis after segmentectomy in lung cancer. METHODS: This was a retrospective cohort study, including 227 patients with cT1N0M0 nonsmall cell lung cancer who underwent positron emission tomography followed by segmentectomy between 2012 and 2019. Significance of tumor histology, T-stage, tumor size, and standardized uptake value on positron emission tomography in relation to recurrence-free survival were examined using Cox's proportional hazard analysis. Median follow-up period was 56 months (range: 1-95 months). RESULTS: Tumor stages were Tis in 25 patients, T1mi/T1a in 51, T1b in 98, and T1c in 53. Twenty-six patients (11%) experienced recurrences, including local (n = 8) and distant (n = 18). Multivariate analysis showed that the significant variables for recurrence-free survival were T-stage and standardized uptake value (p = 0.002 and 0.015, respectively), whereas tumor histology and tumor size were not significant (p = 0.28 and 0.44, respectively). When tumor size was divided into ≤2 cm and >2 cm for analysis, it was not significant again (p = 0.49), whereas standardized uptake value remained significant (p = 0.008). While standardized uptake value of tumors with recurrences was significantly higher than those without (4.9-2.8 and 2.6-2.5, respectively, p < 0.001), there was no significant difference between local and distant recurrences (p = 0.32). Cut-off value of standardized uptake value for recurrences was 3.2. Five-year recurrence-free survival rates in tumors with standardized uptake value <3.2 and ≥3.2 were 86 and 65%, respectively (p < 0.001). CONCLUSION: Positron emission tomography could predict the prognosis after segmentectomy better than tumor size.

Differences between lung adenocarcinoma and squamous cell carcinoma in histological distribution of residual tumor after induction chemoradiotherapy.

AIMS: To facilitate dose planning for convergent beam radiotherapy in non-small cell lung cancer (NSCLC), tumor response and histological distribution of residual tumors after induction chemoradiotherapy (ICRT) were compared between adenocarcinoma (AD) and squamous cell carcinoma (SQ). METHODS: Ninety-five patients with N1-2 or T3-4 NSCLC were treated with ICRT followed by surgery; 55 had AD and 40 had SQ. For the evaluation of distribution of residual tumors, the location of the external margin of residual tumors was assessed on surgical materials as follows: radius of whole tumor ("a"); distance between the center of tumor and the external margin of residual tumor ("b"); and its location ("b/a"). RESULTS: Of the 55 AD cases, 8 (15%) showed pathological complete remission, which was significantly less frequent than 22 of 40 SQ cases (55%) (p < 0.001). AD showed the residual tumors at the most periphery of tumor (b/a = 1.0) more frequently than SQ, i.e., 39/55 (71%) versus 6/40 (15%), respectively (p < 0.001). Even in 65 cases other than the pathological complete remission, external margins in 47 AD cases located more periphery than those in 18 SQ cases, of which mean b/a values were 0.97 ± 0.17 and 0.70 ± 0.29, respectively (p < 0.001). CONCLUSION: AD showed worse tumor response to ICRT than SQ. After ICRT, AD remained at the periphery of primary tumor more frequently than SQ. It seems that, also in the convergent beam radiotherapy, the periphery part of AD would be more resistant than that of SQ.

Tumoral Calcinosis of Thoracic Spine Associated with Vertebral Fracture and Inflammatory Reactions.

Tumoral calcinosis involving the spine is rare. The involvement of the thoracic spine is rarer than that of the cervical or lumbar spine. We report a case of thoracic tumoral calcinosis accompanied by vertebral fracture with increased concentrations of inflammatory markers and no abnormalities in serum calcinosis and phosphorus concentration. A 60-year-old woman presented with complete paraplegia. Her white blood cell count and C-reactive protein (CRP) concentration were elevated. The thoracic magnetic resonance imaging revealed vertebral fracture and an epidural mass that demonstrated low intensity on both T2- and T1-weighted images at the T9/10 dorsal side of the central canal. This lesion is larger in size than that observed in the previous 2 months. Her laboratory data showed signs of infection, and only decompression surgery without fixation for treatment and diagnosis was performed. Histopathological examination was consistent with tumoral calcinosis. Postoperatively, the patient's white cell count and CRP concentration were normalized. We found that tumoral calcinosis can occur at the thoracic level on the basis of the spinal instability due to the vertebral compression fracture and the accompanying increase in inflammation indicated by increased white blood cell count and CRP concentration.

Ribcage procedure after neoadjuvant chemoradiotherapy for non-small cell lung cancer involving the chest wall.

PURPOSE: Non-small cell lung cancer (NSCLC) involving the chest wall is usually treated with en bloc rib resection or parietal pleurectomy; however, the former causes chest wall deformity and the latter is associated with local recurrence. To prevent both these sequalae, we performed the "ribcage" procedure for tumors involving the chest wall after induction chemoradiotherapy. METHODS: This was a single center retrospective study conducted from 2012 to 2018. The "ribcage" procedure is designed to preserve the ribs of patients with lung tumors involving chest wall and involves peeling the intercostal muscles and periosteum from the ribs, resulting in a birdcage-like appearance. Seventeen patients with NSCLC clearly involving the chest wall, but not destroying the ribs, were treated with induction chemoradiotherapy, followed by the ribcage procedure. A negative margin at the ribs was confirmed by intraoperative frozen sections in 16 of these patients, who then underwent the ribcage procedure. RESULTS: Complete resection was achieved in all 16 patients, none of whom experienced major postoperative complications. After a median follow-up period of 37 months, there was no evidence of local recurrence in any of the patients. CONCLUSION: Our findings suggest that the ribcage procedure is the preferable surgical option as it can prevent chest wall deformities as well as local recurrence.

Positron Emission Tomography in T3/T4 Non-Small Cell Lung Cancer After Induction Chemoradiotherapy.

BACKGROUND: To predict pathological response and survival in T3/T4 non-small cell lung cancer after induction chemoradiotherapy by assessing fluorodeoxyglucose uptake with positron emission tomography. METHODS: In this retrospective observational study, standard uptake values of whole tumors and extrapulmonary involvement sites were measured after induction chemoradiotherapy. The values were then compared with pathological responses and recurrence-free survival. RESULTS: Fifty-seven patients with clinical T3/T4 tumors were treated by chemoradiotherapy followed by surgery. Pathological complete response was observed in 33% of patients (19 of 57). With the cutoff value of 3.3 for whole tumor fluorodeoxyglucose uptake for estimating complete response, 38% patients (9 of 24) with values lower than the cutoff value were false-negative. Tumor remission at extrapulmonary involvement sites was observed in 82% patients (47 of 57), and the cutoff value of the extrapulmonary uptake was 3.0 without any false negatives. Recurrence-free survival was significantly better in patients with values lower than both the whole- and extrapulmonary-uptake cutoff values than in patients with higher values (P = .016 and P = .001, log-rank test, respectively). Among 7 patients who avoided en bloc resections of involved structures because of lowered extrapulmonary uptakes and negative findings in intraoperative frozen sections, none experienced margin recurrence. CONCLUSIONS: Even when whole tumor uptakes in patients with T3/T4 tumors markedly decrease after induction chemoradiotherapy, surgical treatment is still indicated because of possible residual tumors. Tumor remission at extrapulmonary involvement sites could be predicted by extrapulmonary uptake values. Both whole- and extrapulmonary-uptake values after induction chemoradiotherapy could be used to predict prognosis.

Recurrent intestinal inflammation with a perianal abscess is not always Crohn's disease: a patient with a complex Meckel's diverticulum diagnosed by double-balloon endoscopy.

Recurrent intestinal inflammation and refractory perianal abscesses are typical manifestations of Crohn's disease. However, these conditions are not always due to Crohn's disease. A 25-year-old male with recurrent perianal abscesses for 1 year, suspected to be due to Crohn's disease, was referred for further evaluation. Computed tomography scan showed a perianal abscess abutting the small intestine. A complicated Meckel's diverticulum was suspected based on these findings. Meckel's diverticulum scintigraphy was negative. Bidirectional double-balloon endoscopy (DBE) identified a long diverticulum in the ileum. In this long diverticulum, a tight stricture was seen 5 cm distal to the diverticular opening. A selective contrast study showed a 10 cm diverticulum distal to the stricture, with three strictures in the long diverticulum. Inflammation of the Meckel's diverticulum due to bacterial overgrowth was suspected as a cause of the refractory perianal abscesses. Laparoscopic diverticulectomy was performed, and the specimen showed a 10 cm diverticulum containing post-inflammatory changes with scar formation. The perianal abscess was confirmed to be caused by an inflamed Meckel's diverticulum. The patient has been asymptomatic for 6 years after resection. DBE before exploratory laparotomy should be considered to investigate the cause of an abscess that could be secondary to small intestinal pathology.

Combined pulmonary fibrosis and emphysema with myeloperoxidase-antineutrophil cytoplasmic antibody positivity that resolved upon smoking cessation.

Myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA) is well-known as a serological marker for small-vessel vasculitis. However, when a smoker with interstitial lung disease (ILD) exhibits serum ANCA positivity without systemic vasculitis, diagnosis is a matter of debate; the relationship between smoking and ANCA is unknown. We report a case of combined pulmonary fibrosis and emphysema (CPFE) with elevated MPO-ANCA. Surgical lung biopsy showed emphysema and fibrotic interstitial pneumonia without vasculitis. The MPO-ANCA level decreased after smoking cessation, and no vasculitis or progression was observed during 3 years of follow-up. This suggested that smoking cessation was related to normalization of MPO-ANCA and corresponding disease activity.

Xanthogranulomatosis of the spleen: a case report.

BACKGROUND: Xanthogranulomatous inflammation is recognized as a subtype of cholecystitis; however, it can also occur in other organs. Xanthogranulomatosis of the kidney, bone, ovary, endometrium, vagina, prostate, lymph nodes and pancreas was reported. Herein, we report a case of laparoscopic splenectomy in a patient with xanthogranulomatosis of the spleen that was difficult to diagnose preoperatively. CASE PRESENTATION: A 63-year-old man with a past medical history of hyperlipidemia had gradually growing multiple splenic masses, which were revealed on abdominal ultrasonography. Preoperative imaging suggested hamartoma, extramedullary hematopoiesis, or an inflammatory pseudotumor. Although metastatic splenic tumors and malignant lymphoma are atypical, they were considered in the differential diagnosis. Thus, laparoscopic splenectomy was performed. Pathological results confirmed a diagnosis of splenic xanthogranulomatosis. An increase in the postoperative triglyceride levels indicated that hyperlipidemia was the cause of xanthogranulomatosis of the spleen. CONCLUSIONS: Xanthogranulomatosis should be considered in the differential diagnosis of multiple splenic mass lesions in patients with splenomegaly. Additionally, fine-needle aspiration biopsy should be considered for the preoperative diagnosis.

Postoperative Cerebral Ischemia Due to Hypotension in Moyamoya Patient with Autonomic Dysfunction.

BACKGROUND: Hypotension is a significant risk factor for the development of ischemic complication following revascularization surgery for moyamoya disease (MMD). However, it is currently unknown whether autonomic dysfunction also plays a role. CASE DESCRIPTION: Here we report a case of MMD in which hypotension due to autonomic dysfunction caused postoperative cerebral ischemia. A 30-year-old female patient with MMD had a history of transient right hemiparesis following laughter. Single-photon emission computed tomography showed impaired cerebral blood flow (CBF) in both cerebral hemispheres, so she underwent revascularization surgery in her left cerebral hemisphere. She awoke from anesthesia uneventfully; however, 1 hour after the surgery her blood pressure suddenly dropped to 90/40 mm Hg and she became comatose. A perfusion computed tomography scan demonstrated a widespread reduction in CBF in the left hemisphere. Bezold-Jarisch reflex was thought to be the cause of the hypotension. Following treatment with a vasopressor agent, her BP increased and her consciousness rapidly recovered. The reduced CBF had almost completely recovered the next day. Head-up tilt test conducted 2 weeks after surgery demonstrated latent vasopressor-type autonomic dysfunction, which was possibly another cause of the hypotension. She was discharged from the hospital, and the laughter-induced hemiparesis gradually resolved. CONCLUSION: Situational neurologic deterioration in patients with MMD suggests latent autonomic dysfunction, which may be a risk factor for postoperative ischemic complications.