Endoscopic-assisted resection of pleomorphic adenoma in the accessory parotid gland.

Background : An accessory parotid gland (APG) is a common anatomical structure that occurs in 10%-56% of individuals. Pleomorphic adenomas are the most common benign tumors of the APG, and their ideal treatment is surgical excision, although there is a risk for aesthetic disorders and facial nerve damage due to the site of origin. Moreover, despite being benign, these tumors are known to recur. Therefore, it is necessary to achieve both reliable excision and avoidance of facial nerve damage. Case presentation : We report a case of a 49-year-old Japanese man with a mass in his left cheek. The lesion was diagnosed as a benign salivary gland tumor derived from the APG by computed tomography imaging, magnetic resonance imaging and fine needle aspiration cytology. We resected the tumor using modified high submandibular incision under the endoscopic-assisted field of view. Discussion and Conclusions : The tumor was less invasive and reliably resected using an endoscope. In surgical treatment, the endoscopic-assisted technique is very useful to achieve complete tumor resection and prevent relapse while avoiding serious complications due to surgical procedures. J. Med. Invest. 68 : 376-380, August, 2021.

Anti-proteinase 3 antineutrophil cytoplasmic antibody reflects disease activity and predicts the response to steroid therapy in ulcerative colitis.

BACKGROUND: Serum anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) is a disease-specific antibody against granulomatosis with polyangiitis. PR3-ANCA is a useful serological marker for disease severity in ulcerative colitis (UC). The purpose of this study was to investigate whether PR3-ANCA levels could also predict the success of induction therapy and to compare its performance against other markers, including serum CRP and fecal hemoglobin. METHODS: This was a multicenter retrospective study. In total, 159 patients with active-phase UC underwent colonoscopy. Disease activity was measured using the Mayo endoscopic subscore (MES). PR3-ANCA positivity and the response to induction therapy, either 5-aminosalicylic acid or steroid, were assessed. PR3-ANCA, CRP, and fecal hemoglobin were measured during the active phase, and during clinical remission. RESULTS: Eighty-five (53.5%) of 159 patients with active UC were positive for PR3-ANCA. PR3-ANCA titers were significantly higher in the group of patients with MES 3 compared to patients with MES 1 (P = 0.002) or MES 2 (P = 0.035). Steroid therapy was administered to 56 patients with a median partial Mayo score of 7 (5-9), which is equivalent to moderate-to-severe disease activity. PR3-ANCA positivity of non-responders to steroid therapy was significantly higher than that of responders (71.9% vs, 41.7%, P = 0.030), whereas CRP and fecal hemoglobin were not predictive of steroid response. Multivariate analysis demonstrated that PR3-ANCA positivity was associated with non-response to steroid therapy (odds ratio 5.19; 95% confidence interval, 1.54-17.5; P = 0.008). Of the 37 patients treated to clinical remission who were also positive for PR3-ANCA during the active phase, 27 had an MES of ≥ 1, and 10 patients had an MES of 0. In clinical remission, the proportion of patients with MES 0 in 17 patients whose PR3-ANCA became negative was significantly higher than that in 20 patients whose PR3-ANCA remained positive (47.1% vs. 10.0%, P = 0.023). CONCLUSIONS: PR3-ANCA not only serves as a marker of disease activity, but also predicts the failure of steroid therapy in moderate-to-severe UC. TRIAL REGISTRATION: This study was retrospectively registered in the UMIN Clinical Trials Registry System (000039174) on January 16, 2020.

6p21 translocation renal cell carcinoma: A case report.

6p21 translocation renal cell carcinoma (RCC) was newly classified in the WHO 2016 classification as a subtype of microphthalmia-associated transcription factor (MIT) family translocation RCC.A 42-year-old man was referred to our hospital with an asymptomatic solid mass in the right kidney identified during routine medical checkup. Computed tomography (CT) revealed a 14-mm buried-type solid mass accompanied by punctate calcification. CT-guided biopsy suggested clear-cell carcinoma. He underwent robotic-assisted partial nephrectomy. Pathological findings revealed 6p21 translocation RCC based on diffuse nuclear immunoreactivity for TFEB and TFEB gene rearrangement in tumor cells by FISH analysis.

Histopathological analysis of autoimmune hepatitis with "acute" presentation: Differentiation from drug-induced liver injury.

AIM: Presently, no standardized definition or acceptable diagnostic criteria have been established for acute presentation of autoimmune hepatitis (AP-AIH), making it difficult to differentiate that condition from drug-induced liver injury (DILI). This study aimed to characterize clinical and histological features for distinguishing between AP-AIH and DILI. METHODS: Clinical, biochemical, and histological characteristics of AP-AIH and DILI in clinically well-characterized cases were compared in a standardized manner to clarify differences. RESULTS: In clinical evaluations, immunoglobulin G level and rate of anti-nuclear antibody positivity were greater in AP-AIH than DILI cases. As for diagnosis of each condition, significant (P < 0.01) differences were found for 10 features: lobular necrosis/inflammation, cobblestone appearance of hepatocytes, plasma cell infiltration in liver parenchyma, centrilobular fibrosis, hepatic rosette formation in areas with cobblestone appearance, portal inflammation, interface hepatitis, prominent plasma cells in portal areas, bile duct injury, and hepatic rosette formation in periportal areas. The area under the curve and cut-off values for the combination of these 10 features were 0.95 and 9 (sensitivity 86%, specificity 90%), respectively. CONCLUSION: Combinations of histological features were found to be helpful for differentiating AP-AIH from DILI, but we were not able to statistically identify an individual feature as definitive.

Clinicopathological examination of ESD as salvage therapy for esophageal cancer after definitive chemo-radiation therapy.

BACKGROUND AND STUDY AIMS: Salvage therapy for esophageal cancer following chemo-radiation therapy (CRT) has not been established. We aimed to evaluate endoscopic submucosal dissection (ESD) as a salvage therapy based on histopathological features of lesions. PATIENTS AND METHODS: We compared 10 lesions in eight patients with local residual, recurrent, or metachronous esophageal squamous cell carcinoma treated by ESD after CRT (CRT group) and 59 lesions treated by ESD without CRT (non-CRT group) during the same period. RESULTS: The en bloc resection rate was 100 % while the complete resection rate was 80.0 % in the lesions after CRT, indicating no difference between the CRT and non-CRT groups. Pathological examination showed that fibrosis was more intense in the lamina propria mucosa, muscularis mucosa, and submucosa. The muscularis mucosa was thicker in both non-tumor and tumor sites in the CRT group compared to the non-CRT group. However, severe submucosal fibrosis was observed only in one lesion in the CRT group. The maximum diameter of the submucosal artery was significantly larger in the CRT group ( P  < 0.001). CONCLUSIONS: Compared to the non-CRT group, the lesions in the CRT group were accompanied by fibrosis while the muscularis mucosa were thicker; however, severe fibrosis of the submucosa was rare. It is important to dissect the muscularis mucosa appropriately during ESD, which makes successful dissection of the submucosa possible. Attention should be paid to bleeding from large arteries.

Clinicopathological Study of Autoimmune Hepatitis Cases That Were Difficult to Differentiate from Drug-Induced Liver Injury.

AIM: Acute-onset autoimmune hepatitis (AIH) histopathologically presents with features of acute hepatitis and lacks a specific diagnostic method. Also, AIH is often difficult to differentiate from drug-induced liver injury (DILI). We aimed to investigate the final clinical diagnosis of these cases, and compare the clinical, biochemical, and histological characteristics of AIH vs. DILI. METHODS: We examined the Digestive Disease Week Japan 2004 (DDW-J) scale scores, AIH scores, clinical data, and pathological findings in 20 patients in whom it was difficult to differentiate autoimmune liver disease from DILI. RESULTS: In cases with a DDW-J scale score of ≥5, there was a good correlation between the final diagnosis and DDW-J scale assessments, but in cases with a DDW-J scale score of ≦4 they did not correlate well. The scores for pathological findings, such as cobblestone hepatocellular change (p = 0.015), interface hepatitis (p = 0.012), and prominent plasma cells in portal areas (p = 0.011), were higher in the AIH group than in the DILI group. CONCLUSION: This study showed that DDW-J scale was useful for differentiating AIH from DILI in cases with a DDW-J scale score of ≧5. The histologic features of AIH were characterized by cobblestone hepatocellular change, interface hepatitis, and plasma cell infiltration of the portal region.

A Case of Spontaneous Tumor Lysis Syndrome in a Patient with Ovarian Cancer.

Tumor lysis syndrome (TLS) is a potentially life-threating complication of tumors or chemotherapy treatment. TLS commonly occurs in hematological malignancies, but it is very rare in patients with a solid tumor. In cases of solid tumors, TLS usually occurs spontaneously and after the initiation of anticancer therapy, and it has a high mortality rate. We present the novel case of a 62-year-old woman with an ovarian tumor who spontaneously developed TLS. Surgical reduction of the tumor mass vastly improved her condition. She showed no sign of tumor recurrence 8 months after treatment. As TLS is life-threatening, successful treatments must be seriously considered.

Prediction of invasion depth for submucosal differentiated gastric cancer by magnifying endoscopy with narrow-band imaging.

The usefulness of determining gastric cancer invasion depth by magnifying endoscopy with narrow-band imaging (NBI-ME) has not been established. The objective of our study was to retrospectively compare NBI-ME images of differentiated submucosal (SM) 1 cancer with those of SM2 to identify the indicators of invasion depth for SM2 gastric cancer. Fifteen patients with SM1 differentiated gastric cancer and 20 with SM2 removed by endoscopic submucosal resection (ESD) were included. NBI-ME images matching the invasion depth of pathological specimens were examined to define the following three findings as diagnostic indicators of SM2: non-structure, scattery vessels and multi-caliber vessels. The relationship between indicators and invasion depth and between indicator score and invasion depth was examined in 27 patients (SM1/SM2: 11/16) with depressed-type gastric cancer (D-GC) and in 8 (SM1/SM2: 4/4) with protruding-type gastric cancer (P-GC). Diagnostic accuracy for invasion depth determined by four endoscopists using regular endoscopic images was compared with that determined by the same endoscopists using NBI-ME. In D-GC, all three indicators were significantly more frequent in SM2 than in SM1 (p<0.05). All D-GC with ≥2 points were SM2, demonstrating a significant difference in score distribution between SM1 and SM2 (p<0.05). In D-GC, diagnostic accuracy by NBI-ME was higher than that by regular endoscopy by all 4 endoscopists (p<0.05). NBI-ME findings of non-structure, scattery vessels and multi-caliber vessels can possibly serve as indicators of SM2 invasion in differentiated D-GC. Scoring of the three indicators was significant.

Atypical lipomatous tumor of the tongue: report of a case.

The term atypical lipomatous tumor (ALT) is synonymous with well-differentiated liposarcoma (WDL). This tumor occurs very rarely in the tongue. Thus, it is difficult to predict its prognosis. Although recurrence of ALT/WDL is thought to be unlikely after complete excision, long-term follow-up is necessary when considering the pathologic conditions of this tumor at other sites. Here, we report a case of an ALT of the tongue, with a review of the literature. A 68-year-old man was referred to our hospital because of a tumor on the left side of his tongue. Upon palpation, the tumor was 12mm in diameter, circumscribed, elastic and hard, well demarcated, movable, and painless. We diagnosed the lesion as a lipoma and extirpated the tumor under local anesthesia. Because the specimen was histopathologically diagnosed as an ALT, as a precaution, we excised an additional 5mm from the area surrounding the original tumor under general anesthesia. Three years after the operation, the tongue demonstrated good healing without paresthesia or dysfunction, and to date there has been no evidence of recurrence.

[A case of neurofibromatosis-1 in which severe bleeding was observed from a neurofibroma under the scalp].

Neurofibroma is a representative external abnormality observed along with café-au-lait spots in association with neurofibromatosis type 1 (NF-1). We encountered a case of NF-1 in which severe bleeding was observed from a neurofibroma under the scalp due to minor trauma. Only four similar cases have been reported in the past literature and we believed that it was a significantly rare case, and we herein report the case with bibliographical considerations. The subject was a 23-year-old male. He was gently hit on the right side of the head during work and the bruised site gradually became bloated. Even on the following day, the bloating continued and he also started feeling severe pain, and as a result, he visited our emergency department. A head CT scan revealed a subcutaneous high-density area from the right-frontal area of the head to the side of the head that appeared to be a hematoma. The pain was severe and we therefore performed emergency surgery to remove the subcutaneous hematoma, but due to severe bleeding during the operation, we ultimately removed only part of the hematoma. However, because the pain was relieved, he was discharged from the hospital and he subsequently stopped visiting the hospital regularly. Three years later, he visited our department again with similar head bloating due to a mild head bruise. When surgery was performed again, an obvious neoplastic lesion was observed along with the subcutaneous hematoma. The pathological findings suggested it was a neurofibroma but no malignant findings were observed.

Sebaceous epithelial-myoepithelial carcinoma of the salivary gland: clinicopathologic and immunohistochemical analysis of 6 cases of a new histologic variant.

Epithelial-myoepithelial carcinoma (EMC) of the salivary glands is an uncommon, low-grade malignant tumor. A recent report demonstrates sebaceous differentiation in this tumor even though its significance has never been documented as a precise histologic variant. Six cases of EMC exhibiting sebaceous differentiation (sebaceous EMC) of the parotid gland were analyzed for their clinicopathologic features and immunohistochemical characteristics. In addition, primary salivary sebaceous carcinomas were also examined for comparison. In our series, the incidence of sebaceous EMC was 0.2% among 3012 cases of parotid gland tumors and 14.3% of all EMC cases. The 6 patients comprised 2 men and 4 women, age ranging from 77 to 93 years (mean, 83.7 y). Neither cervical lymph node nor distant organ metastases were found in any cases of sebaceous EMC and no patients died of disease, though local recurrences developed in 1 patient. Conversely, cervical lymph node metastasis was detected in 2 of 3 patients with sebaceous carcinoma, 1 of whom died of disease at 12 months. Histologically, all 6 tumors had an area of sebaceous differentiation admixed with features of bilayered ductal structures typical of EMC. A component of sebaceous differentiation was distributed diffusely in 4 tumors and focally in 2. Cytologic atypia of sebaceous EMCs was lesser than that of sebaceous carcinomas. Immunohistochemically, putative myoepithelial markers such as alpha-smooth muscle actin, calponin, p63, cytokeratin 14, S-100 protein, and vimentin were highly expressed in sebaceous EMC. However, the expression of the latter 4 markers was also observed in primary sebaceous carcinomas, whereas these tumors were all negative for alpha-smooth muscle actin and calponin. Positive immunoreactivity for epithelial membrane antigen, adipophilin, and perilipin confirmed sebaceous differentiation in EMC. These results indicate that sebaceous EMC is a low-grade malignancy, similar to conventional EMC. Our data also suggest that immunohistochemical examination of specific myoepithelial markers is helpful in distinguishing sebaceous EMC from sebaceous carcinoma, which may occasionally be associated with an aggressive clinical course.

True intraspinal neurenteric cyst in the lumbosacral region--case report.

A 56-year-old man presented with a very rare true neurenteric cyst in the conus medullaris without evidence of vertebral or visceral anomaly manifesting as a 6-month history of mild low back and bilateral inguinal pain. No motor weakness was found in the bilateral lower extremities. He had also suffered dysesthesia in the bilateral feet for several weeks before admission. Lumbar spine magnetic resonance (MR) imaging demonstrated a cystic intradural extramedullary mass at the L1-2 levels without enhancement after gadolinium injection. MR imaging, computed tomography, and radiography detected no vertebral anomaly. Lumbar laminectomy at the L1-2 levels was performed and the lesion was incompletely removed. Histological examination showed the cystic wall lined with ciliated columnar epithelium. Neurenteric cyst should be considered in the diagnosis of isolated cystic mass lesion at the lumbosacral region even in the absence of vertebral or visceral abnormality.

Ureteral metastasis from appendiceal cancer: report of a case.

We report a case of primary appendiceal cancer that metastasized to the ureter. The patient was a 51-year-old woman who complained of persistent left back pain and right lower abdominal pain. After a detailed examination she was diagnosed to have cancer of the appendix, multiple liver metastases, and left hydronephrosis. Since an obstruction of the ileocecum was obvious, she underwent an ileocecal resection and a resection of the spindle-shaped tumor invading the left ureter. Lymph node metastases were found at the root of the superior mesenteric artery. The spindle-shaped tumor was considered to be due to invasion of the appendiceal cancer to the left ureter. The patient died 5 months postoperatively.

Complexity in the treatment of pulmonary large cell neuroendocrine carcinoma.

PURPOSE: According to the World Health Organization (WHO) classification of pulmonary large cell neuroendocrine carcinoma (LCNEC), one of the neuroendocrine tumors of the lung, is considered as a variant of non-small cell lung carcinoma. The objective of this study was to investigate the treatment strategy for LCNEC. METHODS: We retrospectively reviewed the clinical information of 12 patients with LCNEC. RESULTS: Three patients with stage I disease underwent curative resection but all relapsed within 20 months. One with stage IIA disease underwent non-curative resection received adjuvant chemoradiotherapy (cisplatin plus etoposide) and is well with no evidence of recurrence. Two with stage IIIB disease received concurrent chemoradiotherapy. Both achieved partial response (PR) but relapsed within 2 months. One elderly patient with stage IIIA disease received vinorelbine alone and did not respond. Of five patients with stage IV disease, three received platinum-based chemotherapy but no patient achieved PR. Of five patients with gefitinib as salvage therapy, one achieved PR. CONCLUSIONS: The prognosis of LCNEC is poor. To improve the outcome, we must evaluate the effectiveness of adjuvant or neoadjuvant therapy in patients with resectable disease. In addition, the evaluation of systemic and multimodality treatment strategies similar as in small cell lung cancer is worthy of consideration.

Ciliated foregut cyst of the gallbladder: report of a case.

We report the rare case of a gallbladder cyst arising from the foregut remnants. A 36-year-old woman was referred to our hospital after screening ultrasonography (US) detected a tumor in the gallbladder. On admission, she was well and her blood analyses were all normal. US showed a cystic mass with internal high-echoic lesions, and computed tomography (CT) demonstrated a protruding tumor with slight enhancement in the gallbladder. Angiography provided no additional information; however, sequential CT-arteriography (CTA) clearly demonstrated that this tumor was a cystic lesion. Surgical exploration was performed, first because of the difficulty in establishing a definite diagnosis, and also because the patient wanted the tumor removed. The resected specimen contained a unilocular cystic tumor that looked like a submucosal tumor. Histologically, the wall of the cyst was lined by ciliated stratified columnar epithelium with interspersed goblet cells and underlying smooth muscle fibers. The mass was finally diagnosed as a congenital ciliated foregut cyst of the gallbladder. Cysts of the gallbladder are uncommon and the majority are acquired. To our knowledge, this represents only the fourth report of a ciliated foregut cyst of the gallbladder in the literature. Although rare, an awareness of this entity could allow a preoperative diagnosis to be made, whereby surgical exploration may be avoided. CT-A is a very useful diagnostic tool, especially when the nature of the tumor presents a difficult differential diagnosis.

Wegener granulomatosis manifesting as meningitis. Case report.

The authors describe a unique presentation of Wegener granulomatosis (WG) manifesting predominantly as meningitis. Magnetic resonance imaging demonstrated diffuse meningeal enhancement, including the pia mater, in a 28-year-old man with meningitis. A diagnosis of atypical WG was based on the findings of a dural biopsy sample and an elevated cytoplasmic antineutrophil cytoplasmic antibody (cANCA) titer, although the patient did not have any of the lesions common to WG. Immunosuppressive therapy was quite effective. With treatment, the meningeal enhancement resolved and the cANCA titer normalized. Meningeal granulomatosis as the sole lesion in WG has never been reported in the literature. This atypical course of WG should be noted.

A gastric duplication cyst with an aberrant pancreatic ductal system: report of a case.

We report an extremely rare case of a gastric duplication cyst together with an aberrant pancreatic ductal system, which communicated with the stomach rather than the pancreatic ductal system with no evidence of pancreatitis. A 46-year-old woman developed severe abdominal pain after a 10-year history of occasional mild abdominal pain. Upper gastrointestinal barium radiography showed a rigidity of the stomach wall, and gastroscopy revealed a fistula orifice at a greater curvature of the gastric body. Subsequent endoscopic suction of mucous secretion from within the fistula provided immediate pain relief. Abdominal computed tomography and ultrasonography showed a cystic mass contiguous with the stomach wall. Surgical exploration revealed an uncommon anomaly of a gastric duplication cyst with the aberrant pancreatic lobe. The patient made an uneventful recovery and remains well 4 years after surgery. We also herein review ten other similar cases of this uncommon congenital anomaly reported in the literature.