Verruciform Xanthoma of the Esophagus: Two Case Reports With Review of the Literature.

Verruciform xanthoma is a rare benign verrucopapillary lesion that develops in the oral mucosa and genital skin. Its development in the esophagus is extremely rare, with only 5 reported cases. We present 2 cases of verruciform xanthoma of the esophagus. Case 1 involved a 91-year-old woman, who had hypertension and chronic gastritis with Helicobacter pylori infection, with a 12-year history of a 10-mm white-yellow elevated lesion on the esophagus, 35 cm from the incisor teeth. Case 2 involved a 70-year-old man with fundic gland polyp, hyperlipidemia, and lung cancer, who had a 10-mm whitish granular/verrucoid lesion on the esophagus, 28 cm from the incisor teeth. Microscopically, these lesions show verrucous and papillomatous epithelial hyperplasia with neutrophilic intraepithelial exocytosis. The histological hallmark is the presence of numerous foamy histiocytes infiltrating the elongated squamous epithelial papillae. Although its etiology is unknown, irritation or trauma caused by radiotherapy has been suggested.

A comprehensive expression analysis of mucins in appendiceal carcinoma in a multicenter study: MUC3 is a novel prognostic factor.

BACKGROUND: Mucins are implicated in survival in various cancers, but there have been no report addressed on survival in appendiceal carcinoma, an uncommon disease with different clinical and pathological features from those of other colon cancers. We aimed to investigate the clinical implications of expression of mucins in appendiceal carcinoma. METHODS: Expression profiles of MUC1, MUC2, MUC3, MUC4, MUC5AC, MUC6, MUC16 and MUC17 in cancer tissue were examined by immunohistochemistry in 108 cases of surgically resected appendiceal carcinoma. RESULTS: The following relationships of mucins with clinicopathologic factors were identified: MUC1 with positive lymphatic invasion (p = 0.036); MUC2 with histological type (mucinous carcinoma, p<0.001), superficial invasion depth (p = 0.007), negative venous invasion (p = 0.003), and curative resection (p = 0.019); MUC3 with non-curative resection (p = 0.017); MUC5AC with histological type (mucinous carcinoma, p = 0.002), negative lymphatic invasion (p = 0.021), and negative venous invasion (p = 0.022); and MUC16 with positive lymph node metastasis (p = 0.035), positive venous invasion (p<0.05), and non-curative resection (p = 0.035). A poor prognosis was related to positive lymph node metastasis (p = 0.04), positive lymphatic invasion (p = 0.02), positive venous invasion (p<0.001), non-curative resection (p<0.001), and positive expression of MUC3 (p = 0.004). In multivariate analysis, positive venous invasion (HR: 6.93, 95% CI: 1.93-24.96, p = 0.003), non-curative resection (HR: 10.19, 95% CI: 3.05-34.07, p<0.001) and positive MUC3 expression (HR: 3.37, 95% CI: 1.13-10.03, p = 0.03) were identified as significant independent prognostic factors in patients with appendiceal carcinoma. CONCLUSIONS: Expression of MUC3 in appendiceal carcinoma is an independent factor for poor prognosis and a useful predictor of outcome in patients with appendiceal carcinoma after surgery.

¹8FDG PET for grading malignancy in thymic epithelial tumors: significant differences in ¹8FDG uptake and expression of glucose transporter-1 and hexokinase II between low and high-risk tumors: preliminary study.

PURPOSE: To evaluate (18)F-fluorodeoxyglucose (FDG) uptake to predict the malignant nature and analyze the correlation between FDG uptake and expression of glucose transporter 1 (Glut-1) and hexokinase II (HK-II) in thymic epithelial tumors. MATERIALS AND METHODS: Eleven patients with a thymic epithelial tumor who underwent FDG PET/CT before therapy were reviewed. The thymic tumors were classified by the WHO histological classification and Masaoka clinical staging. Comparison of maximum standardized uptake value (SUV(max)) of the lesion was made between the low-risk (Type A, AB and B1) and high-risk {Type B2, B3 and C (thymic cancer)} groups and among clinical stages. Expression of Glut-1 and HK-II was analyzed immunohistochemically. RESULTS: All 11 tumors showed FDG uptake visually. SUV(max) was significantly higher in the high-risk group (n=5, 5.24 ± 2.44) than the low-risk group (n=6, 3.05 ± 0.55) (P=0.008). Staining scores of both Glut-1 and HK-II were significantly higher in the high-risk group than in the low-risk group (Glut1: P=0.034 and HK-II: P=0.036). There were no significant differences in SUV(max) (P=0.11), Glut-1 (P=0.35) and HK-II scores (P=0.29) among clinical stages. SUV(max) was significantly correlated to each of the staining scores of Glut-1 (ρ=0.68, P=0.031) and HK-II (ρ=0.72, P=0.024). CONCLUSION: These preliminary results support the previously published view that SUV(max) may be useful to predict the malignant nature of thymic epitherial tumors and suggest that the degree of FDG uptake in the thymic epitherial tumors is closely related to the amount of Glut-1 and HK-II in the tumor.

Clinical significance of primary lesion FDG uptake for choice between oesophagectomy and endoscopic submucosal dissection for resectable oesophageal squamous cell carcinomas.

OBJECTIVES: To correlate primary oesophageal squamous cell carcinoma (SCC) (18)F-fluoro-deoxyglucose (FDG) uptake with pathological factors and examine its significance regarding choice of therapy. METHODS: We retrospectively examined the factors affecting visible and non-visible FDG uptake in 37 primary lesions in 32 oesophageal SCC patients who underwent PET/CT before oesophagectomy or endoscopic submucosal dissection (ESD). We divided the lesions into pathological depth invasion ≥sm2 oesophagectomy (n = 18) and ≤sm1 ESD (n = 19) indicated groups and compared the diagnostic accuracy of FDG-PET with that of endoscopic ultrasound (EUS) performed for 23 superficial lesions to discriminate between these groups. RESULTS: There were 17 visible and 20 non-visible lesions. The lesion visibility was significantly higher in the larger (≥40 mm), non-flat type, more deeply invaded, positive vascular invasion (P < 0.001 each), positive nodal metastasis (P = 0.04) and higher Glut-1 score (P = 0.005) tumour groups. When the visible and non-visible lesions indicated a need for oesophagectomy and ESD respectively, the sensitivity, specificity and accuracy of oesophagectomy were 94% (17/18), 100% (19/19) and 97% (36/37) and those of EUS were 75% (3/4), 79% (15/19) and 78% (18/23) respectively. CONCLUSIONS: Primary lesion FDG visibility can be one of the indicators for choosing between oesophagectomy and ESD for resectable oesophageal SCCs.

An atypical extrahepatic metastasis of the distal phalanx from hepatocellular carcinoma.

A 49-year-old woman presented with severe swelling and pain of the left little finger, which had exacerbated rapidly, in October 2006. The patient had a history of hepatocellular carcinoma and metastatic lung cancer. She had undergone partial hepatectomy for the hepatocellular carcinoma in September 2001 and pulmonary resection for metastatic lung cancer in November 2005. Roentgenogram of the hand on admission showed complete destruction of the distal phalanx of the left little finger. The final pathological result of the tissue obtained by disarticulation was an extrahepatic metastasis of hepatocellular carcinoma. Extrahepatic metastasis from primary hepatocellular carcinoma to the hand is very rare and only four cases have been reported in the literature. All the patients with extrahepatic metastases from the primary hepatocellular carcinoma to the hand had poor prognoses, but the patient presented in this article exhibited an unusual clinical course. The patient is still alive 1 year after the diagnosis of extrahepatic bone metastasis of the distal phalanx.

Effect of clinicopathologic factors on visibility of colorectal polyps with FDG PET.

OBJECTIVE: The objective of our study was to clarify which clinicopathologic factors affect the FDG PET visibility of colorectal polyps. MATERIALS AND METHODS: We used statistical methods in a retrospective examination of factors affecting the visibility of 87 colorectal polyps in 50 patients who underwent PET for cancer screening. RESULTS: PET depicted 37% (32/87) of polyps. Univariate analysis revealed significant associations between polyp visibility and polyp size, histologic grade (p < 0.001 each), type (p = 0.004), and patient age (p = 0.049) but not sex or polyp location. The visualization rate increased with increases in polyp size (< or = 5 mm, 12%; 6-10 mm, 47%; > or = 11 mm, 59%) and severity of dysplasia (indefinite or low-grade dysplasia, 13%; high-grade dysplasia, 67%; polyp with early carcinoma, 75%) and was higher for pedunculated polyps (59%) than for nonpedunculated polyps (27%). Multivariate analysis showed that histologic grade was the strongest factor (p < 0.001) among three independent factors (histologic grade, type, and age). The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy for visualization of high-grade or early carcinoma polyps to be removed were 71%, 87%, 78%, 82%, and 80%. Maximum standardized uptake values did not differ significantly between visualized polyps with indefinite or low-grade dysplasia and visualized polyps with high-grade dysplasia or early carcinoma. CONCLUSION: Histologic grade is the strongest independent factor in FDG PET visibility of colorectal polyps. FDG PET visibility may be helpful for predicting whether a polyp should be removed or observed. FDG PET findings also may suggest the need to alter the treatment of patients with colorectal polyps.

CD8 positive T-cell infiltration in the dentate nucleus of paraneoplastic cerebellar degeneration.

Recent reports have discussed the presence of cytotoxic T cells in paraneoplastic cerebellar degeneration (PCD). We report an autopsy case of PCD associated with anti-Hu antibody, in which we revealed infiltration of CD8+ T cells in and around the dentate nucleus but not in the cerebellar cortex, in addition to severe Purkinje cell loss. Some infiltrated mononuclear cells expressed cytotoxic cell marker, Granzyme B. Decrease of neurons and reduced presynapses were demonstrated in the dentate nucleus. This is the first report that suggests the possibility of the dentate nucleus being primarily attacked followed by Purkinje cell loss in PCD.

Alpha-fetoprotein (AFP)-producing adrenocortical carcinoma--long survival with various therapeutic strategies including a lung resection: report of a case.

We experienced a very rare case of late pulmonary metastasis from ACC. The patient was a 40-year-old woman who had undergone a left adrenectomy 12 years earlier. Instead of a large metastatic lung tumor with hemothorax and the existence of metastases in other organs, combined therapy of repeated resections for metastases and adjuvant therapy allowed for almost a 36-month survival following the first recurrence and a good quality of life. In addition, a blood and pathological study revealed that the tumor in this case was an alpha-fetoprotein-producing ACC, which is, as far as we could ascertain, the first case of its kind.

A case of mixed connective tissue disease complicated with thymic carcinoma and Hashimoto's thyroiditis.

We report the case of a 63-year-old woman who suffered from mixed connective tissue disease (MCTD) complicated with thymic carcinoma and Hashimoto's thyroiditis. Although many systemic syndromes associated with thymoma and thymic carcinoma, i.e., myasthenia gravis, pure red cell aplasia, hypogammaglobulinemia, and Hashimoto's thyroiditis, are known, this is the first report of MCTD complicated with thymic carcinoma. It was suggested that MCTD may be a paraneoplastic syndrome associated with thymic carcinoma.

Epstein-Barr virus-associated enteritis with multiple ulcers after stem cell transplantation: first histologically confirmed case.

The present case involves unique enteritis forming multiple ulcers associated with Epstein-Barr virus (EBV). A 57-year-old man had undergone a reduced intensity allogeneic stem cell transplantation for a relapse of multiple myeloma following sequential autologous peripheral blood stem cell transplantation. The ileum, resected for massive melena, showed multiple irregular ulcers with occasional cobblestone-like appearance. There was inflammation including numerous plasma cells in the ulcer bases and surrounding areas, where many EBV-infected plasma cells were detected by double staining with EBV-encoded small RNA-1 (EBER-1) in situ hybridization and CD79a, while EBV-infected epithelial cells were not noted. The number of EBER-1-positive cells in the ileum (mucosa, 1451 cells/mm(2); submucosa, 465 cells/mm(2)) was much larger than in control samples (malignant lymphoma or leukemia after allogeneic stem cell transplantation, n = 4, range 0-113 cells/mm(2); malignant lymphoma after chemotherapy, n = 14, range 0-0.89 cells/mm(2); colon cancer, n = 12, range 0-3.5 cells/mm(2)). In the mucosa near the ulcers, EBER-1-positive cells often surrounded and involved the glandular epithelium, forming lymphoepithelial-like lesions. The histological findings differ from post-transplant lymphoproliferative disorders or intestinal thrombotic microangiopathy, and this is the first case of EBV-associated enteritis with ulcers characterized by numerous plasma cells and lymphoepithelial-like lesions after stem cell transplantation.

A familial case of multicystic dysplastic kidney.

A familial case of multicystic dysplastic kidney (MCDK) is described. The proband is a one-year-old boy with left MCDK, and his father was also revealed to have unilateral MCDK. The mother had two abortions; the second pregnancy was terminated because of bilateral MCDK of the fetus (Potter anomaly). The two patients and the aborted male fetus did not have any malformations except for MCDK. Thus in this family MCDK occurs as an isolated phenomenon in three individuals within two generations, presumably as a result of autosomal dominant inheritance.

Primary synovial sarcoma of the sternum: computed tomography and magnetic resonance imaging findings.

We report the computed tomography (CT) and magnetic resonance imaging (MRI) findings of a rare case of synovial sarcoma of the sternum in an 86-year-old man. CT demonstrated an inhomogenously enhanced soft-tissue-density mass of the sternum that destroyed bone cortex and protruded anteriorly. On MRI, the tumor showed a multinodular mass with internal septation and heterogeneous enhancement. These CT and MRI findings were nonspecific, but were similar to those of soft tissue synovial sarcomas. The tumor was more clearly demarcated by MRI than CT. This is the first report concerning the CT and MRI findings of synovial sarcoma of the sternum. Synovial sarcoma should be added to the gamut of primary malignant neoplasms of the sternum.

Intraosseous epidermal cyst of the distal phalanx of the thumb: radiographic and magnetic resonance imaging findings.

We report conventional radiographic and magnetic resonance imaging (MRI) findings of an intraosseous epidermal cyst of the distal phalanx of the right thumb in a 39-year-old man. Conventional radiographs showed a sharply well-circumscribed osteolytic lesion of the distal phalanx with interruption of its tip. The osteolytic lesion itself showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images without contrast enhancement. However, the soft tissues surrounding the osteolytic lesion demonstrated contrast enhancement on MRI. The combined conventional radiographic and MRI findings suggested the diagnosis of intraosseous epidermal cyst rather than enchondroma, giant cell tumor, intraosseous glomus tumor, aneurysmal bone cyst, or simple bone cyst.

Synchronous multicentric thymona: report of a case.

We report a case of multiple thymoma with different histological subtypes, not associated with myasthenia gravis. We describe the histological findings, especially the results of immunohistochemical staining, which support the possibility of multicentric thymoma. The validity of extended thymectomy is also discussed.