[A Case of Subphrenic Lymph Node Metastasis from Gastric Cancer in Which FDG-PET/CT Was Useful for Diagnosis].

A 70-year-old man was admitted for lymph node metastasis detected by FDG-PET/CT showing a mass 10mm in diameter. He had a history of a distal gastrectomy for advanced gastric cancer and was administered postoperative adjuvant chemotherapy consisting of 2 courses of TS-1 with CDDP and TS-1 only for 1 year. Lymph node recurrence was diagnosed and resected 4 years after the initial surgery. Histological examination revealed lymph node metastasis of the gastric cancer. He was administered adjuvant chemotherapy using TS-1 and has been followed-up without recurrences for 17 months after the second operation. We reported a case in which FDG-PET/CT was potentially beneficial for the diagnosis of the postoperative small lymph node metastasis.

[A Case Report of Neuroendocrine Carcinoma in the Gall Bladder Showing Recurrence Shortly after Surgery].

A55 -year-old woman with a 10×8mm protuberant gallbladder lesion visited our hospital. To determine possible malignancy, she underwent laparoscopic excision biopsy of the gallbladder, and pathological findings revealed an endocrine carcinoma in the gallbladder. Surgery involved extrahepatic bile duct resection, gallbladder bed resection, and hepatic portal lymph node resection. Recurrence in the peritoneal lymph node was observed in the early postoperative period. Chemotherapy was initiated with cisplatin and irinotecan, and continues 18 months post-operatively. Neuroendocrine carcinoma of the gallbladder is rare and is known to have a poor prognosis, with few confirmed treatments reported for this disease due to its rarity. Therefore, we report this case along with a review of the literature.

Three cases of collagenous fibroma with rim enhancement on postcontrast T1-weighted images with fat suppression.

Collagenous fibroma, also known as desmoplastic fibroblastoma, is a benign fibrous soft tissue tumor showing gradual growth, commonly without aggressive local infiltration. Today, preoperative radiological diagnosis is important to avoid over-treatment and unnecessary extensive procedures, but is difficult because diagnostic imaging findings for collagenous fibroma have not been established. We report MR imaging findings of three collagenous fibromas in correlation with their histopathology. The characteristic rim enhancement on post-contrast T1-weighted images with fat suppression was present in all three cases, and we consider this to represent the difference in vascularity between the outer capsule-like fibrous tissue and the inside of the tumor.

Tenosynovitis With Psammomatous Calcification Preoperatively Diagnosed as Intra-Articular Free Body of the Young Male Wrist: A Case Report.

Tenosynovitis with psammomatous calcifications is a rare condition primarily affecting female patients in the distal extremities. This case report presents a unique instance of tenosynovitis with psammomatous calcification in a 31-year-old man presenting with wrist pain. Initial misdiagnosis and unsuccessful steroid injections prompted further investigation, leading to the discovery of an extra-articular calcified mass. Arthroscopic resection was attempted but found to be unnecessary because the lesion was located outside the joint. Histopathological examination confirmed the diagnosis of tenosynovitis with psammomatous calcification. After mass removal, the patient experienced relief from wrist pain and resumed work within a month. Subsequent follow-ups at 9 months showed no recurrence of pain, with full range of wrist motion and no grip power weakness. This case highlights the importance of differentiating tenosynovitis with psammomatous calcification from intra-articular lesions, particularly in atypical presentations, and demonstrates the effectiveness of surgical intervention in resolving symptoms.

Adenosquamous carcinoma of the bile duct treated with pancreaticoduodenectomy in a patient presenting situs inversus totalis: A case report.

INTRODUCTION: Situs inversus totalis represents an unusual anomaly characterized by a mirror-image transposition of the abdominal and thoracic viscera. It often occurs concomitantly with other disorders, hindering the diagnosis and management of abdominal pathology. The relationship between situs inversus totalis and cancer remains unclear. PRESENTATION OF CASE: We describe a 79-year old Japanese female with situs inversus totalis who presented with obstructive jaundice. Imaging and endoscopic examinations showed a mass in the distal common bile duct, which was identified as an adenocarcinoma on biopsy. The patient was successfully treated by cephalic pancreaticoduodenectomy and the histological diagnosis was adenosquamous cell carcinoma. Ten months following surgery, the patient received chemotherapy and radiotherapy due to the presence of liver metastasis. DISCUSSION AND CONCLUSION: The occurrence of an adenosquamous carcinoma of the bile duct in a patient with situs inversus totalis is an extremely rare coincidence. In this setting, when the tumor is resectable, surgical management should be considered without contraindication and must be preceded by careful preoperative staging.

Novel germ line mutation p53-P177R in adult adrenocortical carcinoma producing neuron-specific enolase as a possible marker.

Adrenocortical cancer (ACC) is a rare and aggressive endocrine tumor. The patient presented with a large retroperitoneum tumor and lung metastases. Removal of the adrenocortical tumor with part of the transverse colon and tail of the pancreas, spleen and kidney was successfully performed following chemotherapy. Levels of serum neuron-specific enolase (NSE) were found to be markedly high before surgery and may be clinically useful markers for monitoring tumor status during management. Immunohistochemical studies showed that the cancer cells were positive for NSE and overexpression of p53. We identified a novel germ line variant of the 177 mutant (Pro to Arg; P177R) of p53 by genomic sequencing. The genetic and biochemical data presented in this case confirm the importance of screening for p53 status in ACC with inherited cancer syndrome.

Adenocarcinoma of small intestinal type in retroperitoneal mature teratoma.

We report a case of small intestinal type adenocarcinoma arising in retroperitoneal mature cystic teratoma in a young male. The patient initially experienced intermittent abdominal pain, and radiographic workup revealed an encapsulated retroperitoneal mass. A laparotomy was performed and the resected specimen represented a cystic lesion, the inner surface of which was mostly covered by sebaceous debris with hairs. Microscopically, mature derivatives of all three germ layers as well as completely developed gastrointestinal tract were identified. Adenocarcinoma without stromal invasion was observed adjacent to the small intestinal mucosa. Immunohistochemistry of the adenocarcinoma tissue revealed p53 overexpression and high Ki-67 labeling index as well as positive staining for CD10, cytokeratin 7, and cytokeratin 20. Therefore, the diagnosis of small intestinal adenocarcinoma was made. To our knowledge, this is the first case of small intestinal adenocarcinoma arising in retroperitoneal mature cystic teratoma. A unique feature of this case is that malignant transformation in retroperitoneal mature teratoma arose even in the fully developed intestine. Favorable prognosis due to detection in the 'early stage' is also discussed.

[A randomized controlled trial of postoperative adjuvant chemotherapy for colorectal cancer-optimal duration of the treatment].

METHODS: Subjects were 239 patients with colorectal cancer who underwent curative resection surgery from December 1994 to March 1997(Stage I-III b). The patients were given 5'-DFUR for postoperative 10 months as scheduled. They had been allocated into either a 1-year group or a 3-year group by dynamic randomization. 5'-DFUR was administered by an intermittent regimen such as 1,200 mg/body/day for five days followed by two days rest. All patients were followed for five years at least. RESULTS: 239 patients were enrolled in the study. Favorable prognoses in both groups were observed. Although no statistically significant differences in overall survival curves of full analysis set based on the drug administration durations, were detected(log-rank test, p=0.734), a better prognosis was found in the 3-year group(5-year OS: 92.0%; 1- year group, 91.4%; 3-year group). Adverse drug reactions resulted in low rates such as 14.8% in the 1-year group and 19.5% in the 3-year group. Grade 3 was found in either group. CONCLUSIONS: Due to a result in the present study that 5-year survival rates in both groups were far higher than anticipated, we could not finally clarify the optimal administration duration of 5'-DFUR. However, the results of the present study indicate that 5'-DFUR results in a good prognosis for colorectal cancer patients and is safe over a long / administration period.

Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases.

Clear cell sarcoma (CCS) of soft tissue is a rare sarcoma with morphologic similarities to malignant melanoma but a distinct genetic background including a chromosomal translocation, t(12;22)(q13;q12), or a resultant EWSR1-ATF1 fusion gene. In addition, the tumors occurring in the gastrointestinal tract may have a variant fusion gene EWSR1-CREB1. This study analyzed the clinicopathologic and molecular genetic features of 33 CCSs of soft tissue. The patients' ages ranged from 13 to 73 years (median, 30 y), and there was a male predominance (20 males, 13 females). The tumors were located in the deep soft tissues of the extremities (N=25) or in the trunk or limb girdles (N=8). The median tumor size was 4 cm (range, 1 to 15 cm). The tumor cells were either spindle or epithelioid, and they were arranged predominantly in a short fascicular (N=19) or a solid sheetlike growth pattern (N=14). Minor histologic variations included the existence of rhabdoid cells (N=8), bizarre pleomorphic cells (N=6), alveolar structures due to loss of cellular cohesion (N=3), and a seminomalike pattern (N=2). Tumor necrosis was evident in 14 tumors, and the mitotic activity ranged from 0 to 43 mitotic figures (MF)/10 high-power fields (HPF) (mean: 4 MF/10 HPF). Immunohistochemically, the tumors were consistently positive for S-100 protein (33/33) and variably or focally for HMB45 (32/33), microphthalmia transcription factor (26/32), Melan A (23/32), CD57 (25/33), bcl-2 (30/32), synaptophysin (14/32), CD56 (7/32), epithelial membrane antigen (12/33), cytokeratin (AE1/AE3) (1/32), CD34 (3/32), c-erbB-2 (10/32), c-kit (5/32), and c-met (5/32). alpha-Smooth muscle actin, desmin, and cytokeratin (CAM5.2) were negative. Reverse transcription-polymerase chain reaction using RNA extracted from formalin-fixed, paraffin-embedded tissues demonstrated transcripts of the EWSR1-ATF1 (31/33) or EWSR1-CREB1 fusion gene (2/33). In 26 cases with available clinical information, local recurrences and metastases developed in 2 and 15 patients, respectively. Ten patients were dead of the disease, and the overall survival rate was 63% at 5 years. However, no clinicopathologic or molecular variables associated with the patients' prognosis were identified. This study confirms that CCS is an aggressive soft tissue tumor with a melanocytic phenotype and wider morphologic variations than had been generally considered. In cases with unusual histologic findings, molecular detection of the EWSR1-ATF1/CREB1 fusion genes provides critical information regarding the diagnosis of the tumor.

[Immunohistochemical and clinical features of patients with benign middle ear tumors].

The middle ear is a rare site for benign tumors. We report six patients with benign middle ear tumors surgicaly removed: three with carcinoid, one with adenoma, and two with paraganglioma. These tumors showed similar histological features in trabecular and glandular architecture, so further immunohistochemical study was needed to make a definitive histopathological diagnosis. When we analyzed clinical features of patients diagnosed immunohistochemically, the differentiation of two patients with paraganglioma from the remaining four with adenomatous tumors such as adenoma and carcinoid was relatively easy from otomicroscopic findings and enhancement in temporal bone MRI. It was however difficult to differentiate patients with adenomatous tumors from each other because they induced similar clinical symptoms and showed similar findings in images. For a definitive diagnosis, it is necessary to conduct exploratory tympanotomy or removal of the tumors to obtain enough specimen for histological and immunohistochemical studies. Tumors induced no bone destruction or complications. We recommend staged operations for total removal of tumors and for good postoperative hearing.

Generation of the novel monoclonal antibody against TLS/EWS-CHOP chimeric oncoproteins that is applicable to one of the most sensitive assays for myxoid and round cell liposarcomas.

The fusion oncoproteins, TLS-CHOP and EWS-CHOP, are characteristic markers for myxoid and round cell liposarcomas (MLS/RCLS). Especially, the peptide sequence of 26 amino acids corresponding to the normally untranslated CHOP exon 2 and parts of exon 3 (5'-UTR) is a unique structure for these chimeric proteins. In this report, we have generated monoclonal antibodies against the unique peptide sequence of TLS/EWS-CHOP oncoproteins. These antibodies reacted with TLS-CHOP fusion protein, but not reacted with normal TLS and CHOP proteins by Western blot analysis. In addition, one of the antibodies also recognized the chimeric oncoprotein in archival paraffin-embedded tissue samples of MLS/RCLS. The oncoprotein was detectable by the antibody even in the paraffin-embedded tissue samples whose mRNAs were too degraded to be detected by a nested reverse transcription-polymerase chain reaction-based assay. Thus, the molecular assay using the novel antibody is expected to be one of the most sensitive diagnostic assays for MLS/RCLS.

Molecular detection of FUS-CREB3L2 fusion transcripts in low-grade fibromyxoid sarcoma using formalin-fixed, paraffin-embedded tissue specimens.

A diagnosis of low-grade fibromyxoid sarcoma (LGFMS) remains problematic because of its bland-looking histologic features that can be potentially confused with other benign or low-grade fibromyxoid lesions. Recent cytogenetic and molecular analyses have shown that most LGFMSs have a characteristic chromosomal abnormality, t(7;16)(q33;p11), resulting in the FUS-CREB3L2 fusion gene. However, such assays have only rarely been used to analyze formalin-fixed, paraffin-embedded tumor samples. In the present study, we conducted a reverse transcription-polymerase chain reaction assay to detect the FUS-CREB3L2 fusion transcripts using formalin-fixed, paraffin-embedded tumor tissue specimens from 16 LGFMSs including 3 cases with giant collagen rosettes. The primers were newly designed to specifically amplify most of the junctional regions of the FUS-CREB3L2 fusion gene transcripts previously reported. The FUS-CREB3L2 fusion gene transcripts were detected in 14/16 (88%) cases of LGFMS. A nucleotide sequence analysis of the PCR products revealed that different portions of the FUS exon 6 or 7 were fused with various sites of the CREB3L2 exon 5, resulting in 12 different nucleotide sequences. We also tested a primer set to detect the FUS-CREB3L1 fusion transcript, which is a rare variant of the gene fusion in LGFMS, although no PCR products were identified in any case. The FUS-CREB3L2 fusion transcripts were not detected in any of the 123 other soft-tissue tumors, including desmoid-type fibromatoses, myxofibrosarcomas, soft-tissue perineuriomas, and congenital or adult fibrosarcomas. These data suggest that our reverse transcription-polymerase chain reaction assay is a reliable method to detect FUS-CREB3L2, which can thus help in accurately diagnosing LGFMS.

Brown tumor with fluid-fluid levels in a patient with primary hyperparathyroidism: radiological findings.

We report a case of a brown tumor with fluid-fluid levels in a patient with primary hyperparathyroidism. A 19-year-old woman presented with a 3-month history of pain in the left pubic region. The laboratory data showed elevated serum calcium and intact parathyroid hormone, confirming the diagnosis of primary hyperparathyroidism. Plain radiography and computed tomography (CT) showed an expansile lytic lesion of the superior ramus of the left pubis. The cortex was thinned. On magnetic resonance (MR) images, the lesion was solid and cystic. The solid area of the lesion showed heterogeneous low to intermediate signal intensity on T1-weighted images and heterogeneous low to high signal intensity on T2-weighted images. The cystic area showed several fluid-fluid levels on T2-weighted images. Dynamic contrast-enhanced MR images after administration of Gd-DTPA showed marked, early enhancement of the solid area of the lesion. A delayed image showed prolonged enhancement of the solid area and enhancement of the septa and walls of the cystic area. Histopathology of a biopsy specimen showed fibroblastic proliferation, abundant giant cells, and focal hemosiderin deposition, which supported the diagnosis of a brown tumor. After removing the parathyroid adenoma, the brown tumor regressed and became sclerotic on radiographs.

Prevalence of ischemic enterocolitis in patients with acute pancreatitis.

BACKGROUND: A considerable number of acute pancreatitis cases have been reported to be complicated by nonocclusive mesenteric ischemia. However, no reports have ever referred to the incidence of ischemic enterocolitis in patients with acute pancreatitis, using a series of autopsy cases. Here, we report our review of autopsy cases of patients with acute pancreatitis to examine the incidence of associated ischemic enterocolitis. METHODS: The intestinal and pancreatic slides of 48 autopsy cases of patients with acute pancreatitis were reviewed and the incidence of ischemic enterocolitis was determined. Clinical case records were also reviewed. RESULTS: Thirteen (27%) of 48 autopsy cases of patients with acute pancreatitis were complicated by ischemic enterocolitis. The frequency of shock was significantly higher in patients with ischemic enterocolitis than in those without ischemic enterocolitis. The intestinal lesion was diffuse in many cases and gangrene was not an unusual finding. CONCLUSIONS: The incidence of ischemic enterocolitis in patients with acute pancreatitis was much higher than that in the previous reports. Clinicians who treat patients with acute pancreatitis should consider ischemic enterocolitis as one of the frequent and severe complications of this condition.

Ovarian fibroma (fibrothecoma) with extensive cystic degeneration: unusual MR imaging findings in two cases.

Two cases of ovarian fibroma (fibrothecoma) with extensive cystic degeneration are described. One case revealed a large multiloculated cystic mass with a small solid part. Each locule showed variable signal intensity on both T1- and T2-weighted magnetic resonance (MR) images. The other case revealed a parviloculated cystic mass with a small solid part. In both cases, the solid parts were located at the periphery of the mass and showed distinct low signal intensity on both T1- and T2-weighted MR images and slight enhancement. It should be noted that ovarian fibromas (fibrothecomas) with extensive cystic degeneration are a rare exception to the rule that solid components in a cystic adnexal mass imply malignancy.

Expression of cyclooxygenase-2 in chondroblastoma: immunohistochemical analysis with special emphasis on local inflammatory reaction.

To investigate the frequency and mechanism of the peritumoral inflammatory reaction in chondroblastoma, we evaluated the relationship between clinicoradiological findings and immunohistochemical expression of cyclooxygenase-2 (COX-2) in excised tumors. Twenty-one cases of chondroblastoma were studied. Imaging analysis was performed with radiographs and T1- and T2-weighted magnetic resonance images in all cases and with computed tomography scan and bone scintigraphy in some cases. Immunohistochemical study for COX-2 was carried out using formalin-fixed paraffin-embedded tissues. Periosteal reaction was observed in 6 cases (29%) and bone marrow edema in 15 cases (71%). Soft-tissue edema, joint effusion, and synovitis were found in 10 cases (48%), in 7 cases (33%), and in 9 cases (43%), respectively. Immunohistochemical expression of COX-2 in chondroblastoma cells was found in 15 of 21 cases (71%). The intensity of COX-2 immunoreactivity was correlated statistically with the presence of periosteal reaction, bone-marrow edema, soft-tissue edema, and synovitis. Our results indicate that activation of eicosanoid synthesis by COX-2 expression in the tumor itself is probably an important factor, inducing peritumoral inflammatory changes in chondroblastomas.

Ulcerative colitis followed by the development of Behçet's disease.

A 34-year-old man who had a history of ulcerative colitis (UC) was admitted to our hospital with complaints of arthralgia, erythema nodosum, recurrent oral aphthous ulcers and bloody stools. A colonoscopy revealed multiple aphthous ulcers on his cecum and colon and also revealed a transmural ulcer on his rectum consistent with a diagnosis of UC. The patient was HLA-B51 positive. Based on clinical evidence [recurrent oral ulcers, skin lesions (erythema nodosum), positivity for pathergy test] this patient was diagnosed as having Behçet's disease with gastrointestinal involvement. We describe this rare case of Behçet's disease with colitis and discuss the difficulties in making a differential diagnosis between Behçet's disease and the inflammatory bowel diseases.

CT and MR findings of basal cell adenoma of the parotid gland.

We report a case of basal cell adenoma in the left parotid gland. A 34-year-old woman presented with a non-tender mass in the left parotid gland. She had first noted it 7- years previously, and it had been gradually increasing in size. The tumor was well-circumscribed with a smooth contour. On noncontrast-enhanced CT, the tumor showed homogeneous soft tissue attenuation. No cystic portion or calcification was seen. The tumor showed homogeneous moderate enhancement on contrast-enhanced CT. Capsule-like ring enhancement was demonstrated. On T1-weighted MR imaging, the tumor was homogeneously hypointense to the surrounding parotid tissue and isointense to muscle. On T2-weighted imaging the tumor was homogeneously hyperintense to muscle but slightly hypointense to the surrounding parotid tissue. On Gd-enhanced T1-weighted imaging, the tumor demonstrated homogeneous moderate enhancement. Capsule-like ring enhancement was also demonstrated. Salivary (technetium-99m-pertechnetate) scintigraphy did not show any uptake in the tumor.

Brown tumor of the sphenoid sinus in a patient with secondary hyperparathyroidism: CT and MR imaging findings.

We present a case of brown tumor of the sphenoid sinus in a patient with secondary hyperparathyroidism. CT showed an expansile soft-tissue attenuation mass centered in the sphenoid sinus. CT at bone window setting demonstrated expansile, lytic change and remodeling of the surrounding bone. On MR imaging, the lesion showed iso-intensity to gray matter on T1-weighted images and heterogeneous hyperintensity on T2-weighted images, and showed intense enhancement. The extent of the lesion and its relationship to the surrounding structures were best evaluated by CT and MR imaging.