Primary signet-ring cell carcinoma of the bladder treated with laparoscopic radical cystectomy: a case report.

Primary bladder adenocarcinomas comprise 0.5-2% of all epithelial bladder neoplasms. Of these, primary signet-ring cell carcinoma of the bladder is particularly rare, accounting for 0.24% of all bladder malignancies. This tumor is frequently diagnosed at an advanced stage and has a poor prognosis. No standard treatment has yet been established. We here report a patient in whom laparoscopic cystectomy following neoadjuvant chemotherapy was effective. Our patient was a 69-year-old man who had had microscopic hematuria, undergone transurethral resection of a mass in the bladder, and been diagnosed pathologically with a primary signet-ring cell carcinoma of the bladder. No metastases were detected on computed tomography. The patient was treated with a combination of paclitaxel, cisplatin, and gemcitabine prior to undergoing laparoscopic cystectomy. The histopathological diagnosis on this operative specimen was dysplasia and no metastases were detected in the dissected lymph nodes. Complete remission has now been maintained for 9 years.

Tubulointerstitial nephritis with storiform fibrosis in a patient with angioimmunoblastic T-cell lymphoma.

We present a case of an angioimmunoblastic T-cell lymphoma (AITL) and tubulointerstitial nephritis with storiform fibrosis in a 76-year-old man. The patient exhibited lymphadenopathy, polyclonal hypergammaglobulinemia, and renal dysfunction and was diagnosed with AITL on the basis of lymph node biopsy findings. The serum IgG4 level was highly elevated. Renal biopsy revealed IgG4-positive plasma cells and storiform fibrosis without infiltration of AITL, and the findings indicated IgG4-related kidney disease (IgG4-RKD). Following THPCOP therapy for AITL, the renal function improved. While diagnosing IgG4-RKD in a patient with AITL poses challenges, follicular helper T cell involvement appeared crucial in AITL and renal tubulointerstitial lesions in this case.

ATYPICAL ENDOGENOUS FUNGAL ENDOPHTHALMITIS CAUSED BY CANDIDA RUGOSA.

PURPOSE: The purpose of this study was to report a case of atypical endogenous fungal endophthalmitis caused by Candida rugosa , a rare species of nonalbicans Candida . METHODS: This report describes a case of a 45-year-old woman who presented with a reduced visual acuity in the right eye in addition to vitreous opacity during breast cancer treatment, which was suspected as fungal endophthalmitis from medical examination and history. Various tests were performed for diagnosis. RESULTS: Blood test results were normal, including the blood beta-D-glucan level, and blood cultures were negative. Diagnosis could not be made using systemic computed tomography and magnetic resonance imaging results. Therefore, a lesion sample was collected by using vitrectomy. C. rugosa was identified through DNA (extracted from the lesion sample) analysis using Basic Local Alignment Search Tool. The visual acuity of the right eye improved after vitrectomy. CONCLUSION: We encountered a rare case of atypical endogenous fungal endophthalmitis caused by C. rugosa . Clinicians sometimes encounter invasive candidiasis caused by rare nonalbicans Candida species. DNA analysis using Basic Local Alignment Search Tool is effective for diagnosing such cases.

Spontaneous pleural aspergillosis in an immunocompetent young adult treated with minimally invasive surgery.

Spontaneous cases of pleural aspergillosis in healthy adults are rare, and the optimal therapeutic approach has not been established. Here we report a rare case of spontaneous pleural aspergillosis in an otherwise healthy young adult. Two-stage surgery with decortication and cavernostomy, followed by systemic antifungal therapy, finally resulted in a successful resolution of his empyema without any serious complications. In young patients with good pulmonary compliance, less invasive procedures, such as thoracoscopic decortication and/or carvernotomy, is a potential treatment option.

Immunoglobulin G4-related Disease with Marked Eosinophilia: A Case Report and Literature Review.

We encountered a 78-year-old Japanese man with IgG4-related sialoadenitis complicated with marked eosinophilia. We diagnosed him with IgG4-RD (related disease) with a submandibular gland tumor, serum IgG4 elevation, IgG4-positive plasma cell infiltration, and storiform fibrosis. During follow-up after total incision of the submandibular gland, the peripheral eosinophil count was markedly elevated to 29,480/µL. The differential diagnosis of severe eosinophilia without IgG4-RD was excluded. The patient exhibited a prompt response to corticosteroid therapy. His peripheral blood eosinophil count was the highest ever reported among similar cases. We also review previous cases of IgG4-RD with severe eosinophilia.

Peritoneal and nodal recurrence 7 years after the excision of a ruptured solid pseudopapillary neoplasm of the pancreas: report of a case.

A solid pseudopapillary neoplasm (SPN) of the pancreas is generally regarded as a neoplasm of low malignant potential and there is rarely recurrence of the disease. A 12-year-old female underwent a pylorus preserving pancreaticoduodenectomy for a ruptured pancreatic SPN following a blunt abdominal trauma. The tumor showed no pathological features suggesting malignant potential. Follow-up imaging studies depicted small nodules adjacent to the superior mesenteric vein 7 years after surgery. A laparotomy was performed, and exploration revealed 3 nodules adjacent to the superior mesenteric vein and 4 small nodules in the mesointestine. All of these lesions were extirpated, and were histologically confirmed to be nodal and peritoneal recurrence of SPN. This case indicates that SPN of the pancreas has a latent ability to recur, regardless of its benign pathological features, and peritoneal spread may be promoted by trauma. A close postoperative follow-up is thus mandatory in all patients with SPN even after a radical resection.

Gastric rupture with necrosis following acute gastric dilatation: report of a case.

Gastric rupture with necrosis following acute gastric dilatation (AGD) is a rare and potentially fatal event; usually seen in patients with eating disorders such as anorexia nervosa or bulimia. A 12-year-old lean boy with no remarkable medical history was brought to our Emergency Department suffering acute abdominal symptoms. Emergency laparotomy revealed massive gastric dilatation and partial necrosis, with rupture of the anterior wall of the fundus of the stomach. We performed partial gastrectomy and the patient recovered uneventfully. We report this case to demonstrate that AGD and subsequent gastric rupture can occur in patients without any underlying disorders and that just a low body mass index is a risk factor for this potentially fatal condition.

Initial and advanced endoscopic findings of monomorphic epitheliotropic intestinal T-cell lymphoma in the duodenum: A case report.

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive malignant digestive system lymphoma. We report the case of a 68-year-old Asian woman who was diagnosed with MEITL of the duodenum and small intestine due to intestinal obstruction. MEITL is mainly located in the small intestine, and duodenal lesions are rare. Therefore, the endoscopic appearance of MEITL in the duodenum has been reported in only a few cases. In this case, we observed the initial and advanced endoscopic findings of MEITL in the duodenum. The initial findings were only slight mucosal changes; therefore, careful observation is required to detect early-stage MEITL.

Successful treatment of a patient with IgG4-related disease with a paravertebral mass lesion.

A 68-year-old woman was admitted with bleary eyes and lacrimal gland swelling. A biopsy specimen from a right paravertebral mass lesion detected by computed tomography showed remarkable IgG4-positive plasma cells. Because serum IgG4 was elevated to 3300 mg/dl, IgG4-related disease was diagnosed, with the assistance of fluorodeoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT). Administration of 30 mg of oral prednisolone effectively reduced the lacrimal gland swelling and paravertebral mass volume. Nine months after the initiation of prednisolone, serum IgG4 was reduced to 31.4 mg/dl, and there was norecurrence.

Predictive Factors Among Clinicopathological Characteristics for Sentinel Lymph Node Metastasis in T1-T2 Breast Cancer.

BACKGROUND: The axillary lymph node status is an important prognostic factor of breast cancer. This study explores the predictive factors for sentinel lymph node (SLN) metastasis among the preoperative clinicopathological features, including impaired glucose tolerance (IGT). METHODS: This study comprised patients diagnosed with breast cancer who underwent surgery at Nagasaki Harbor Medical Center between April 2014 and December 2019. The factors assessed using univariate and multivariate analyses were the clinicopathological data of these cancers, including the patient age, gender, menstrual status, breast or ovarian cancer family history, body mass index, glycosylated hemoglobin, clinical tumor size, nipple-tumor distance (NTD), tumor histology, histological grade, node status, estrogen receptor, progesterone receptor, human epidermal growth factor receptor type 2 status, and Ki67 labeling index. RESULTS: In the cohort of 313 cases, the ratio of SLN metastasis was 17.3%. A univariate analysis found that the tumor size, NTD, IGT, and clinical tumor stage were associated with SLN metastasis. In a multivariable analysis, the tumor size, NTD, and IGT were associated with SLN metastasis. The receiver operating characteristic curve showed a sensitivity and specificity of 61.1% and 65.6%, respectively, at a cut-off of 1.7 cm for the tumor size (area under the curve [AUC]: 0.664; 95% confidence interval: 0.592-0.736), and a sensitivity and specificity of 60.4% and 62.9%, respectively, at a cut-off of 2.0 cm for NTD (AUC: 0.651; 95% confidence interval: 0.571-0.731) to predict the risk of SLN metastasis. CONCLUSION: T1 and T2 breast cancer patients with a larger tumor size, tumor located closer to the nipple, and IGT have a higher risk of SLN metastases than others.

Endoscopic snare papillectomy with biliary and pancreatic stent placement for tumors of the major duodenal papilla.

BACKGROUND: This study aimed to evaluate the feasibility, safety, and follow-up results of endoscopic papilletomy (ESP) with pancreatic and biliary duct stent placement for ampullary tumors. The therapeutic approach to benign ampullary tumors remains unsettled. The ESP procedure is a curative treatment option for benign papillary tumors, but ESP raises concerns about a relatively high risk for procedure-related complications such as pancreatitis. A pancreatic stent may protect against complications. METHODS: Between September 2000 and June 2008, 36 patients with ampullary tumors confined to the mucosa and no intraductal tumor growth underwent ESP. The preprocedural diagnostic tools included endoscopic ultrasound, transpapillary intraductal ultrasound, and endoscopic retrograde cholangiopancreatography. Pancreatic and biliary stent placement was attempted if feasible. Endoscopic follow-up evaluation was conducted periodically as surveillance for recurrence. RESULTS: En bloc ESP was achieved for 94% of lesions with a median size of 14 mm. There were 26 adenomas including 4 high-grade intraepithelial neoplasias (HGINs), 5 carcinomas in adenoma, and 3 intramucosal cancers. Complete resections with tumor-free lateral and basal margins was achieved for 81% of the cases. During the median follow-up period of 14 months, there was one recurrent adenoma, which was successfully eradicated by a repeat ESP. A pancreatic stent was placed in 35 cases and a biliary stent in 29 cases. Mild acute pancreatitis and bleeding, managed endoscopically, occurred in 3 cases each (8%). CONCLUSION: The ESP procedure can be feasible for benign ampullary adenoma, HGIN, and noninvasive cancer without intraductal tumor growth. Prophylactic stent placement in the pancreatic and bile ducts may reduce procedure-related complications.

Rare Case of Floating Intimal Flap Associated with Atheromatous Carotid Plaque.

BACKGROUND: A mobile carotid plaque can be detected by duplex ultrasonography and is a high-risk factor for embolic stroke. CASE DESCRIPTION: We herein present a case involving an 80-year-old man with an asymptomatic carotid floating flap diagnosed by duplex ultrasonography and treated with carotid endarterectomy. Intraoperatively, an ulceration was found immediately proximal to the neck of the floating flap, and the shape and size of the ulceration were quite similar to those of the floating flap. In a histopathologic examination of the specimen resected by carotid endarterectomy, the plaque lacked the internal elastic lamina (IEL) at the ulceration, calcification was observed in the plaque and medial layer at the ulceration, and the floating flap consisted of the IEL accompanied by calcification, fibrin, and foamy cells. CONCLUSIONS: Progression of the atheroma and Mönckeberg sclerosis might have affected disruption of the IEL, causing the IEL to finally peel off. A floating intimal flap accompanied by an atheroma without intraplaque hemorrhage is a rare cause of mobile plaque formation. This type of mobile plaque might not be dissolved by medical treatment alone. In such cases, surgical treatment is a suitable therapeutic choice to prevent stroke.

[A case of secondary central nervous system lymphoma presenting marked hypoglycorrhachia].

A 67-year-old male was transferred to our hospital with diplopia, decreased deep tendon reflex and ataxia. He had been suspected Fisher syndrome because of previous upper respiratory tract infection. A cerebrospinal fluid examination showed marked hypoglycorrhachia, pleocytosis and elevated protein, and cytological examination suggested malignant lymphoma. Abdominal computed tomography revealed a left adrenal mass. A biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. He was treated with a combination of R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, oncovin and prednisolone) and intrathecal administration of methotrexate, cytarabine and prednisolone. Neurological symptoms were gradually improved. Malignancy should be considered in addition to bacterial, fungal or tuberculous meningitis in a case with marked hypoglycorrhachia.

Acute appendicitis associated with the presence of schistosome eggs in a sailor: a case report.

BACKGROUND: Schistosomiasis is prevalent in tropical and subtropical areas and rarely reported in developed countries. Schistosomiasis often occurs as a chronic illness, which can cause liver and intestinal damage. Appendicitis is an unusual complication of schistosomiasis. We herein present a case of acute appendicitis associated with the presence of schistosome eggs in a sailor from the Philippines. CASE PRESENTATION: A 34-year-old Filipino man who worked as a sailor presented to our hospital with a 2-day history of acute right lower quadrant abdominal pain and fever. A physical examination revealed right lower quadrant abdominal pain with signs of peritoneal irritation, including rebound tenderness. Computed tomography revealed thickening of the appendix associated with mural calcification and fluid collection around the cecum. Based on these findings, the preoperative diagnosis was acute appendicitis. Laparoscopic appendectomy was performed. Swelling of the appendix and contaminated ascites were observed intraoperatively, but there was no evidence of appendiceal perforation. A histopathological examination showed inflammation of the appendix wall and numerous ovoid bodies present within the submucosa, many of which were calcified. Severe infiltration of lymphocytes and fibrosis were recognized around the oval bodies. The numerous oval bodies were morphologically consistent with schistosomiasis. The final diagnosis was acute phlegmonous appendicitis associated with the presence of schistosome eggs. We examined the patient for signs of adult worm activity, but the results of stool ova and parasite examinations performed twice were negative. He was discharged and returned to his country on postoperative day 9. CONCLUSIONS: The incidence of schistosomal appendicitis, which is seldom reported in developed countries, is expected to increase in Japan in the near future. Clinicians should suspect schistosome eggs as a cause of acute appendicitis in patients who have emigrated from or are traveling from endemic areas, and when mural calcification of the appendix is observed on imaging.

Atypical Familial Mediterranean Fever Complicated with Gastrointestinal Amyloidosis Diagnosed due to Paroxysmal Arthralgia and Intractable Diarrhea, Successfully Treated with Tocilizumab.

A 53-year-old man with recurrent episodes of large joint pain and a low-grade fever at irregular intervals for 16 years developed right knee and ankle arthralgia, watery diarrhea, and abdominal pain. Following an ileum and colon biopsy, he was diagnosed with gastrointestinal amyloidosis. We suspected familial Mediterranean fever (FMF) based on his history and administered colchicine; his symptoms subsequently improved. Thus, he was diagnosed with atypical FMF. After tocilizumab administration, the amyloid deposits disappeared. This case suggests that physicians should consider FMF even in cases with atypical symptoms in order to prevent the progression of amyloidosis and that amyloid deposits can be eliminated by interleukin (IL)-6 inhibition.

Iatrogenic Removal of the Intima in the Middle Cerebral Artery by a Stent Retriever: A Report of Two Cases.

BACKGROUND: Mechanical thrombectomy improves functional outcomes in patients with acute ischemic stroke. However, stent retrievers have the risk of vascular damage. CASE DESCRIPTION: We present 2 cases of patients with acute internal carotid artery occlusion who experienced removal of the intima by a stent retriever. In both patients, a 6 × 30-mm Solitaire stent was fully deployed from the M2 portion and slowly withdrawn. White membranes were retrieved outside the strut in both patients. Histopathologic examination showed that one membrane consisted of thickened intima and internal elastic lamina and the other consisted of calcified intima and internal elastic lamina. One patient who suffered embolic stroke experienced recurrent infarction within 24 hours after operation, and the damaged vessel was occluded on magnetic resonance angiography 21 days after stroke. In another patient with carotid artery dissection, the damaged vessel showed asymptomatic stenosis on magnetic resonance angiography 90 days after stroke. Arteries with both atherosclerosis and vessel dissection may be vulnerable to high radial expansion force. CONCLUSIONS: Full deployment of a relatively large-sized stent into a vulnerable vessel may cause vessel dissection after removal of the intima. Appropriate material selection and treatment strategy while considering stroke etiology and the occlusion site are important to prevent vessel damage.

Artefact as the pathologist's friend: peritumoral retraction in in situ and infiltrating duct carcinoma of the breast.

Peritumoral retraction artefact appears in tissue sections as an empty space partially or completely encircling a nest of tumor cells, usually in conformity with the rounded or angular outline of that particular nest. The present study was designed to test this finding in a large series of cases and to quantify the appearance of peritumoral retraction artefact in, in situ and infiltrating duct carcinoma of the breast. We examined 199 cases of infiltrating duct carcinoma (IDC) and 188 cases of ductal carcinoma in situ (DCIS). Of the total of 387 cases, 111 were core needle biopsies, whereas the others were larger resections. In each specimen, retraction was evaluated on hematoxylin and eosin-stained slides as negative, 1+ (1% to 25% of tumor showing retraction), 2+ (26% to 50%), 3+ (51% to 75%), or 4+ (76% to 100%). Overall, peritumoral retraction was noted in 168 of 199 cases (84.4%) of IDC, versus 30 of 188 cases (16%) of DCIS (P < 0.0001). Peritumoral retraction scored as 2+ or greater (26% to 50%) was seen in only 1 of 188 DCIS specimens, compared with 77 of 199 IDC. Thus, peritumoral retraction artefact appears to be a significant finding seen during the evaluation of hematoxylin and eosin specimens for the diagnosis of carcinoma. We discuss the possibility that this phenomenon might represent true prelymphatic space involvement rather than a fixation artefact.

Malignant Pleural Mesothelioma with Marked Lymphatic Involvement: A Report of Two Autopsy Cases.

We herein report two cases of malignant pleural mesothelioma with marked lymphangiosis. The patients included a 68-year-old man and a 67-year-old man who both had a history of exposure to asbestos. Computed tomography (CT) on admission showed pleural effusion with pleural thickening. In both cases, a histopathological examination of the pleura confirmed the diagnosis of epithelioid malignant mesothelioma. They received chemotherapy, but the treatment was only palliative. The chest CT assessments during admission revealed marked pleural effusion and mediastinal lymphadenopathy. CT also showed a consolidative mass with bronchovascular bundle and septal thickening in the lungs suggesting pulmonary parenchymal involvement and the lymphangitic spread of the tumor. These CT findings mimicked lung cancer with pleuritis and lymphangitic carcinomatosis. Autopsy was performed in both cases. Macroscopically, the tumor cells infiltrated the lung with the marked lymphatic spread of the tumor. Microscopy also revealed that the tumor had invaded the pulmonary parenchyma with the marked lymphatic spread of the tumor. Although this growth pattern is unusual, malignant pleural mesothelioma should be considered as the differential diagnosis, especially in patients with pleural lesions.

Analysis of unilateral adrenal hyperplasia with primary aldosteronism from the aspect of messenger ribonucleic acid expression for steroidogenic enzymes: a comparative study with adrenal cortices adhering to aldosterone-producing adenoma.

Unilateral adrenal hyperplasia with primary aldosteronism is very rare and shows similar endocrine features to aldosterone-producing adenoma and bilateral adrenal hyperplasia. In this study, the mRNA expression of steroidogenic enzymes in unilateral adrenal hyperplasia was examined by in situ hybridization. We found subcapsular micronodules composed of spironolactone body-containing cells, which showed intense expression for 3beta-hydroxysteroid dehydrogenase, 11beta-hydroxylase, 18-hydroxylase, and 21-hydroxylase but not 17alpha-hydroxylase, indicating aldosterone production. This expression pattern was the same as that in unilateral multiple adrenocortical micronodules, reported recently. Additionally, it was noted that a nodule with active aldosterone production was closely adjacent to one showing intense 17alpha-hydroxylase expression. In the adrenal cortices adhering to aldosterone-producing adenoma, the majority of hyperplastic zona glomerulosa and hyperplastic nodules demonstrated a decreased steroidogenic activity. However, minute nodules indicative of active aldosterone production were found at high frequency. These results suggest that the subcapsular micronodules observed might be the root of aldosterone-producing adenoma. Furthermore, we emphasize the need for long-term follow-up after unilateral adrenalectomy or enucleation of the adenoma because of the possibility that buds with autonomous aldosterone production may still be present in the contralateral or remaining adrenal tissue.

Gallbladder small cell carcinoma: a case report and literature review.

Gallbladder small cell carcinoma (SCC) comprises only 0.5 % of all gallbladder cancer and consists of aggressive tumors with poor survival outcomes against current treatments. These tumors are most common in elderly females, particularly those with cholecystolithiasis. We report the case of a 79-year-old woman with gallbladder small cell carcinoma. The patient had intermittent right upper quadrant abdominal pain and was admitted to our hospital due to suspected acute cholecystitis. She regularly received medical treatment for diabetes, hypertension, and dyslipidemia. On initial laboratory evaluation, the levels of aspartate aminotransferase (AST), total bilirubin, and C-reactive protein (CRP) were markedly elevated. She underwent computed tomography (CT) for screening. CT images showed a thick-walled gallbladder containing multiple stones and multiple 3-cm-sized round nodular lesions, which were suggestive of metastatic lymph nodes. After percutaneous transhepatic gallbladder drainage was performed, endoscopic ultrasound-guided fine needle aspiration of enlarged lymph nodes resulted in a diagnosis of small cell carcinoma or adenocarcinoma. However, we could not identify the primary lesion before the surgery because of no decisive factors. We performed cholecystectomy because there was a possibility of cholecystitis recurrence risk and also partial liver resection because we suspected tumor invasion. The final pathological diagnosis was neuroendocrine carcinoma of the gallbladder, small cell type. The tumor stage was IVb, T3aN1M1. The patient died 13 weeks after the surgery. In the present paper, we review the current available English-language literature of gallbladder SCC.