Systemic Amyloid A Amyloidosis Secondary to Xanthogranulomatous Pyelonephritis.

The combination of systemic amyloid A (AA) amyloidosis and xanthogranulomatous pyelonephritis (XGP) resulting from a chronic urinary tract infection is extremely rare. We herein report a case of systemic AA amyloidosis secondary to XGP for which clinical remission developed after nephrectomy. To our knowledge, this is the first case report describing the clinical improvement of systemic AA amyloidosis secondary to XGP after nephrectomy in Japan. Clinicians should be aware of this uncommon combination and search for amyloid depositions in cases of XGP.

[A case of rectal cancer with immune thrombocytopenic purpura that achieved a complete response to continued chemotherapy with eltrombopag].

A 73-year-old male patient with postoperative recurrent rectal cancer developed thrombocytopenia after XELOX therapy. Thrombocytopenia persisted despite chemotherapy discontinuation;therefore, he was referred to our department for further evaluation. Bone marrow specimen examination revealed increased immature megakaryocytes and blood test results revealed elevated platelet-associated immunoglobulin G (PA-IgG) levels, leading to immune thrombocytopenic purpura diagnosis. His platelet count recovered after prednisolone therapy. Eltrombopag treatment was introduced considering thrombocytopenia secondary to chemotherapy resumption for rectal cancer. FOLFIRI therapy was continued without platelet count reduction, and PA-IgG levels decreased over time. The patient continued chemotherapy with eltrombopag and achieved a complete treatment response.

Clinical Significance of Extramural Tumor Deposits in the Lateral Pelvic Lymph Node Area in Low Rectal Cancer: A Retrospective Study at Two Institutions.

BACKGROUND: The presence of extramural tumor deposits without lymph node structure (EX) is an important prognostic factor for patients with colorectal cancer. However, the clinical significance of EX in the lateral pelvic lymph node area (LP-EX) remains unclear. This study aimed to determine the prognostic implications of LP-EX for patients with low rectal cancer. METHODS: This retrospective study involved 172 consecutive patients with stage 2 or 3 low rectal cancer who underwent curative surgery including lateral pelvic lymph node (LPLN) dissection. The patients were classified into the following three groups according to the metastatic status of the LPLN area: patients without metastasis (no-LP-M group), patients with lymph node metastasis (LP-LNM group), and patients with EX (LP-EX group). Potential prognostic factors of overall survival (OS) and relapse-free survival (RFS) were identified in uni- and multivariate analyses. RESULTS: Classification assigned 131 patients (76 %) to the no-LP-M group, 27 patients (16 %) to the LP-LNM group, and 14 patients (8 %) to the LP-EX group. The 5-year OS rate was 80.3 % in the no-LP-M group, 61.1 % in the LP-LNM group, and 34.9 % in the LP-EX group (P < 0.001). The corresponding 5-year RFS rates were 62.2, 33.8, and 14.3 %, respectively (P < 0.001). A multivariate Cox proportional hazards regression analysis showed that the presence of LP-EX was an independent prognostic factor for OS (P = 0.006) and RFS (P = 0.001). CONCLUSIONS: The LP-EX classification is a useful pathologic parameter that can be used to stratify patients with metastasis in the LPLN area.

Characteristics of neoplastic cardiac tamponade and prognosis after pericardiocentesis: a single-center study of 113 consecutive cancer patients.

BACKGROUND: Neoplastic cardiac tamponade (NCT) is a life-threatening complication of cancer. The interval between cancer diagnosis and NCT onset and the prognosis after pericardiocentesis may differ according to cancer type. METHODS AND RESULTS: We performed a retrospective study of 113 patients (54 % male) with NCT who underwent pericardiocentesis at Niigata Cancer Center Hospital between 1992 and 2013. Mean age at NCT was 61.2 years (range 15.9-94.8 years). The most common underlying cancers were lung cancer (59.2 %), breast cancer (21.2 %), lymphoma/leukemia (5.3 %), and gastric/esophageal cancer (5.3 %). The median time from cancer diagnosis to NCT onset was 9.0, 60.4, 5.6, and 8.0 months for lung cancer, breast cancer, lymphoma/leukemia, and gastric/esophageal cancer, respectively. Kaplan-Meier survival estimates were worse for breast cancer patients with NCT than for matched breast cancer patients without NCT (P < 0.0001). Median survival time after pericardiocentesis was 2.9, 4.2, 2.3, and 0.6 months for lung cancer, breast cancer, lymphoma/leukemia, and gastric/esophageal cancer, respectively; one-year survival after pericardiocentesis was 6.0, 16.7, 33.3, and 0 %, respectively. CONCLUSIONS: The interval between cancer diagnosis and NCT onset, the impact of NCT on prognosis, and the prognosis after pericardiocentesis differed according to cancer type. Healthcare practitioners caring for patients with NCT should recognize the differences between cancer types and customize their care accordingly.

Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum.

Malignant peripheral nerve sheath tumors (MPNSTs; malignant schwannomas) rarely occur in the anterior mediastinum, and their prognosis is poor. A 75-year-old man was referred to our hospital for examination of an anterior mediastinal tumor. A computed tomography-guided percutaneous needle biopsy revealed only fibrosis. The tumor was completely excised via a median sternotomy with partial resection of the pericardium and right upper lobe of the lung. Thereafter, the tumor was diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma. At 1 year after the surgery, a distant metastasis was found in the interlobular space between the right middle and lower lobes. The tumor was completely excised via a right posterolateral thoracotomy. Reexamination of the primary and secondary tumors revealed an MPNST. No recurrence was found up to 5 years after the second surgery without adjuvant chemotherapy or radiation therapy. However, he died from multiple lung metastases after 6 years.

What causes discrepancies in HER2 testing for breast cancer? A Japanese ring study in conjunction with the global standard.

We assessed interinstitutional and interobserver consistency of human epidermal growth factor receptor type-2 (HER2) testing using immunohistochemical analysis and fluorescence in situ hybridization (FISH) in a set of 20 breast cancer samples among 10 institutions in Japan and a Herceptin adjuvant study participating laboratory in Germany and identified factors that may lead to discordant results.We found a good agreement in immunohistochemical HER2 scoring between the coordinating institution and 10 participating laboratories (kappa = 0.718) and excellent agreement for FISH (kappa = 0.900). The results of a comparison between 10 Japanese laboratories and the German laboratory was good for immunohistochemical studies (kappa = 0.713) and excellent for FISH (kappa = 0.887). FISH retesting of equivocal samples (2+ immunohistochemically) improved agreement. Discrepancies between results were attributed to the evaluation process in 33.0% of the samples, staining procedures in 25.0%, and a combination of the two in 41.7%. Evaluation of samples according to the American Society of Clinical Oncology/College of American Pathologists guideline increased the number of 2+ immunohistochemical scores. By performing FISH retesting for these samples, consistency among multiple institutions could be archived. The quality of the staining procedures performed and the consistency of evaluations require regular assessment.

[Multicenter phase II trial of thrice-weekly docetaxel and weekly trastuzumab as preoperative chemotherapy in patients with HER 2-overexpressing breast cancer--Japan East Cancer Center Breast Cancer Consortium (JECBC) 02 Trial].

The efficacy and safety of combination therapy of 4 cycles with docetaxel 70 mg/m(2)every 3 weeks and trastuzumab as primary chemotherapy for operable breast cancer was determined in 21 patients (pts) by assessing the pathological complete response (pCR) rate, clinical response rate (RR), breast conservation surgery (BCS) rate and toxicities. To date, 19 pts have completed surgery. The pCR rate was 21% [95% CI 6%-46%] . The overall RR was 90% [95% CI 67%-99%] , with 5 CR, 12 PR, 2 SD and 0 PD. Grade 3 or 4 adverse events were leukopenia 48%, neutropenia 67%, hemoglobin 5%, and febrile neutropenia 10%. All non-hematological toxicities were mild and manageable. The pCR rate is not as low as that achieved in previous international studies. The combination of docetaxel and trastuzumab was a well-tolerated and very active regimen for the treatment of patients with HER 2-overexpressing operable breast cancer. This regimen promises to be one of the leading future treatments for progressive breast cancer.

Focal lymphoid hyperplasia in a lipoma, mimicking liposarcoma.

Lipomas may contain nonadipose tissue, which can confound the correct imaging diagnosis because features of such neoplasms can mimic findings associated with well-differentiated liposarcomas. Intratumoral nonadipose tissue, such as muscle, cartilage, and fibrous tissue, may be present in lipomas; however, lymphoid hyperplasia in a lipoma is extremely rare. This report describes a case involving a heterogeneous lipoma in a 55-year-old woman in whom the heterogeneous portion of the tumor was in fact a reactive lymphoid hyperplasia encased by the lipoma. The imaging features of this lesion as well as pathological findings of this unusual tumor are discussed.

Resected well-differentiated fetal pulmonary adenocarcinoma and summary of 25 cases reported in Japan.

We report a case of well-differentiated fetal adenocarcinoma (WDFA), which is a variant of pulmonary blastoma. A 36-year-old man was found to have a tumor shadow in the right middle field of a chest radiograph as part of a mass screening examination, and chest computed tomography (CT) showed a 4.5-cm pulmonary mass in the right lower lobe. A diagnosis of adenocarcinoma of the lung was made based on a CT-guided needle biopsy, and right middle and lower lobectomy and lymph node dissection were performed. The postoperative pathological diagnosis was well-differentiated fetal adenocarcinoma. WDFA has a better prognosis than conventional pulmonary blastoma (biphasic pulmonary blastoma). We summarize the cases of WDFA reported in Japan and review the literature.

KIT expression in normal and neoplastic renal tissues: immunohistochemical and molecular genetic analysis.

Renal chromophobe cell carcinomas (ChCC) and oncocytomas express KIT. This character seems to reflect their common histogenesis from distal nephrons. In the normal kidney, however, the expression and localization of KIT are unclear. KIT expression in angiomyolipoma and congenital mesoblastic nephroma (CMN), is still controversial. c-kit mutations are reportedly rare in ChCC, but there is little information in other renal neoplasms, and no reported data on mutations of platelet-derived growth factor receptor (PDGFR). In order to address these issues the authors examined five ChCC, five oncocytomas, seven papillary cell carcinomas, two collecting duct carcinomas, 12 angiomyolipomas, and three CMN, as well as 10 normal renal tissues. In the normal kidney KIT was specifically expressed in the distal nephrons. Nine of 12 (75%) angiomyolipomas contained scattered KIT-positive cells, whereas all three CMN were completely negative for KIT. The presence of KIT-positive cells in angiomyolipomas was likely to correspond to that of melanocytic marker-positive cells, which mainly showed epithelioid morphology. Polymerase chain reaction-single-strand conformation polymorphism showed no evidence of mutations of c-kit or PDGFR in any of the tumors examined.

Expression of heart-type fatty acid-binding protein in human gastric carcinoma and its association with tumor aggressiveness, metastasis and poor prognosis.

OBJECTIVE: Fatty acid-binding proteins (FABPs) are involved in lipid metabolism by intracellular transport of long-chain fatty acids. Heart-type (H-) FABP has been reported to inhibit cell growth and induce cell differentiation, but to our knowledge the significance of H-FABP expression in human gastric carcinoma has not been elucidated. The aim of the current study was to examine the expression of H-FABP and its relation to clinicopathologic parameters and fatty acid synthase (FAS) status of gastric carcinoma, since gastric cancer shows increased expression of FAS. METHODS: Immunohistochemistry with anti-H-FABP antibody was performed in 669 gastric carcinomas and 60 tubular adenomas of the stomach. H-FABP-positive and H-FABP-negative carcinomas were analyzed for their clinicopathologic characteristics and FAS status. RESULTS: None of the adenomas expressed H-FABP, whereas 127 of 669 carcinomas (19.0%) were positive for the protein. H-FABP positivity was associated with the depth of invasion (p <0.0001), vascular invasion (p <0.0001), lymph node metastasis (p <0.0001), hepatic metastasis (p=0.0011), stage of the carcinoma (p <0.0001) and FAS status of the carcinoma (p=0.0476). A higher survival rate was noted in H-FABP-negative cases compared with H-FABP-positive cases (p=0.0004). CONCLUSIONS: A subset of human gastric carcinoma expresses H-FABP and its expression is associated with FAS status, disease progression, tumor aggressiveness and poor patient survival.

Well-differentiated liposarcoma associated with benign lipoma.

The objective of this study was to review the clinicopathological features of seven patients presenting with well-differentiated liposarcoma (WDL), which was associated with subcutaneous lipoma. From 1980 through 2002, 34 individuals displaying WDL were treated in our institutions. Lipoma was observed in seven of these 34 patients (five men and two women, mean age of 66.7 years). The rate of co-existence of lipoma in WDL [20.6% (7/34)] cases was significantly higher than the corresponding rate in the other liposarcoma subtypes [2.5% (1/40)]. Immunohistochemically, cdk4 was positive in all WDLs (100%). ki-67 was positive in 57.1% (4/7) and mdm2 and p53 were positive in 14.5% (1/7) of the WDL cases. Weak cdk4 immunoreactivity was detected in two lipomas. All lipomas were negative for mdm2, p53 and ki-67. Comparison of the expression profile in these malignant and benign tumors, which had arisen in identical genetic backgrounds, confirmed the involvement of these proteins, especially cdk4, in the tumorigenesis process of WDL.

Liver-type fatty acid-binding protein is highly expressed in intestinal metaplasia and in a subset of carcinomas of the stomach without association with the fatty acid synthase status in the carcinoma.

OBJECTIVE: To investigate the relation of liver-type fatty-acid-binding protein (L-FABP) expression to the clinicopathological characteristics or the fatty acid synthase status of gastric cancers. METHODS: L-FABP expression was examined immunohistochemically in 667 gastric cancers, 60 gastric adenomas, and non-neoplastic epithelium contiguous with cancer tissue including normal foveolae, intestinal metaplasia, regenerative epithelium, and gastric glands. RESULTS: L-FABP was positive in 38% (high in 9% and low in 29%) of gastric cancers. It occurred preferentially in papillary carcinomas, female cases, and in patients under 50 years. In gastric cancers, L-FABP expression had no intimate correlation with the FAS status, and it showed no relationship with prognosis and cancer progression as indicated by venous and lymphatic permeation, and nodal or hepatic metastasis. Gastric tubular adenomas mainly revealed low (22%) expression of L-FABP while intestinal metaplasia showed the most frequent (>95%) and intense L-FABP expression. Normal foveolae and gastric glands showed no or less L-FABP expression. CONCLUSIONS: L-FABP is highly and intensely expressed in metaplasia and in a subset of gastric adenocarcinomas, without association with progression, prognosis and fatty acid synthase status of the carcinoma.

Criteria for intentional limited pulmonary resection in cT1N0M0 peripheral lung cancer.

OBJECTIVE: The purpose of this study was to establish selection criteria for intentional limited resection in patients with peripheral lung cancer. METHODS: Six hundred eighty-nine cases of cT1N0M0 peripheral non-small cell lung cancer were divided into groups according to maximum tumor diameter. The cases in each group were examined for histopathological invasive factors, and the results of a pilot study of intentional limited resection were assessed. RESULTS: The positive rate of invasive factors was 30.8% among the patients with tumors 2 cm or less in diameter, and significantly lower than the 44.0% noted in those whose tumor diameter was in the 2.1-3.0 cm range. The positive rate was significantly lower in 90 patients with type A or B adenocarcinoma, and none of these patients developed postoperative recurrence. In 24 of these 90 patients, the tumor diameter was in the 2.1-3.0 cm range. The 5-year survival rate in the 74 patients with pT1N0M0 and tumors 2 cm or less in diameter who underwent limited resection was 89.1%. CONCLUSIONS: We attempted to establish selection criteria for limited resection, with the aim of obtaining survival rates that was comparable to those obtained after lobectomy. The selection criteria established in this study are: 1, cT1N0M0 peripheral non-small cell lung cancer; 2, maximum tumor diameter 2 cm or less on diagnostic images, but a tumor diameter in the range of 2-3 cm in adenocarcinoma of Noguchi type A or B cases; 3, limited resection feasible based on tumor location.