AIM: To evaluate the success rates, procedural time and adverse event rates of the modified methods in endoscopic ultrasonography-guided hepaticogastrostomy (EUS-HGS).
METHODS: Twenty-eight patients in a prospective case series who underwent EUS-HGS (phase I). Forty-six patients in a matched case-control study (phase II). The simplified technique for fistula dilation was the primary use of a 4 mm balloon catheter with a stainless steel stylet. The stent deployment was modified by deploying the metal stent inside a bile duct (half of the stent) under EUS and fluoroscopic guidance and gently pulling the echoendoscope after full deployment of the stent inside the echoendoscope channel (remaining portion of the stent) under fluoroscopic guidance. This cohort was compared with a matched historical cohort.
RESULTS: In phase I, the technical and clinical success with the modified method was 96% (27/28) and 89% (24/27 as per-protocol analysis). The overall adverse event rate was 7%. In phase II, there was no difference in technical and clinical success, stent patency and overall adverse events in each group. However, the procedural time (15.3 ± 5.2 min vs 22.3 ± 6.0 min, P < 0.001) and early adverse events (0% vs 26%, P = 0.02) were statistically improved in case cohort compared with control cohort.
CONCLUSION: Compared with the conventional EUS-HGS technique, the procedural time was shorter and early adverse events were less frequent with our simplified and modified technique.
Endoscopic ultrasonography; Biliary drainage; Hepaticogastrostomy; Treatment outcome; Adverse event
Hydatid cysts (echinococcosis) are caused by an infestation with larval tapeworms of the genus Echinococcus. The disease is extensively distributed worldwide, and it has been rarely reported in Korea. We describe the cytologic features of a case of hepatic hydatid cyst in a 28-year-old male. Computed tomography revealed a cystic mass in the right lobe of the liver. A right hemihepatectomy was performed. The aspirated fluid from the hepatic cystic mass was clear. The smears showed protoscolices, hooklets, and a laminated membrane.
Echinococcosis; Liver; Cytology
Soft tissue myoepithelioma is a rare neoplasm composed of myoepithelial cells. Here, we describe the cytologic features of soft tissue myoepithelioma arising on the right forearm in an 18-year-old man. The excised tumor (3.0×1.8×1.5 cm) was well-demarcated, yellow-gray, soft, and myxoid. The cytologic smears showed round to spindle, epithelioid, and plasmacytoid cells in the myxoid background. The nuclei were uniform, round to ovoid, with finely distributed chromatin and eosinophilic or pale cytoplasm. The tumor cells demonstrated immunoreactivity for cytokeratin (AE1/AE3), epithelial membrane antigen, S100 protein, and glial fibrillary acidic protein. Electron microscopy showed intermediate filaments, desmosomes, and basal lamina.
Soft tissue; Myoepithelioma; Cytology
Microcystic/reticular schwannoma is a recently described, rare, distinctive histological variant of schwannoma with a predilection for the gastrointestinal tract (GIT). The authors experienced the first case of a microcystic/reticular schwannoma occurring in the esophagus.
A 39-year-old male presented for an obstructive sensation during swallowing of several months duration. Endoscopy revealed a bulging mass with intact mucosa at 30 cm from incisors in the esophagus. The mass was excised and gross examination showed it was a well circumscribed, unencapsulated nodule, measuring 3.5×3.2×1.2 cm. On microscopic examination, the tumor showed a vague multinodular appearance with a pushing border and tumor cells arranged in a microcystic and reticular growth pattern with anastomosing and intersecting strands of spindle cells in a myxoid or collagenous/hyalinized stroma. Tumor cells showed diffuse nuclear and cytoplasmic positivity for S100.
The authors report the first case of microcystic/reticular schwannoma of the esophagus. Microcystic/reticular schwannoma is a distinctive histological variant of schwannoma with a benign clinical course. However, its histological findings are non-specific and may cause diagnostic difficulties. Awareness of this uncommon neoplasm with distinct histologic features is essential to prevent misdiagnosis.
Schwannoma; Microcystic; Reticular; Esophagus
Individuals harboring germ-line DICER1 mutations are predisposed to a rare cancer syndrome, the DICER1 Syndrome or pleuropulmonary blastoma-familial tumor and dysplasia syndrome [online Mendelian inheritance in man (OMIM) #601200]. In addition, specific somatic mutations in the DICER1 RNase III catalytic domain have been identified in several DICER1-associated tumor types. Pituitary blastoma (PitB) was identified as a distinct entity in 2008, and is a very rare, potentially lethal early childhood tumor of the pituitary gland. Since the discovery by our team of an inherited mutation in DICER1 in a child with PitB in 2011, we have identified 12 additional PitB cases. We aimed to determine the contribution of germ-line and somatic DICER1 mutations to PitB. We hypothesized that PitB is a pathognomonic feature of a germ-line DICER1 mutation and that each PitB will harbor a second somatic mutation in DICER1. Lymphocyte or saliva DNA samples ascertained from ten infants with PitB were screened and nine were found to harbor a heterozygous germ-line DICER1 mutation. We identified additional DICER1 mutations in nine of ten tested PitB tumor samples, eight of which were confirmed to be somatic in origin. Seven of these mutations occurred within the RNase IIIb catalytic domain, a domain essential to the generation of 5p miRNAs from the 5′ arm of miRNA-precursors. Germ-line DICER1 mutations are a major contributor to PitB. Second somatic DICER1 “hits” occurring within the RNase IIIb domain also appear to be critical in PitB pathogenesis.
DICER1; Pituitary blastoma; miRNA; Pediatric tumors
Periosteal osteosarcoma is an extremely rare chondroblastic osteosarcoma in the flat bone. There were authors reporting of two cases of periosteal osteosarcoma in the highly unusual sites. One of them arose from the rib, in a 17-year-old male, which appeared as a hypodense juxtacortical mass with periosteal reaction on CT. The other one arose from the scapula, in a 17-year-old female, which showed the intermediate signal intensity (SI) on T1-weighted image (WI), heterogeneous high SI on T2WI, and rim-enhancement on contrast-enhanced T1WI with cortical destruction on MRI.
Bone surface tumor; Osteosarcoma; Rib; Scapula; Chest wall
Kikuchi-Fujimoto disease (KFD) is a benign disease, which is characterized by a cervical lymphadenopathy with fever, and it often mimics malignant lymphoma (ML). 2-[18F]fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) is a powerful imaging modality for the diagnosis, staging and monitoring of ML, with the limitations including the nonspecific FDG uptake in infectious or inflammatory processes. This study compared clinical manifestations and PET/CT findings between KFD and ML patients.
We retrospectively reviewed the medical records of 23 patients with KFD and 33 patients with ML, diagnosed histopathologically, between January 2000 and May 2013 at the Department of Pediatrics, Yeungnam University Medical Center. Among them, we analyzed the clinical manifestations, laboratory findings and characteristics, and the amount of 18F-FDG uptake between 8 KFD and 9 ML patients who had 18F-FDG PET/CT.
The 18F-FDG PET/CT maximum standardized uptake values (SUVmax) ranged from 8.3 to 22.5 (mean, 12.0) in KFDs, and from 5.8 to 34.3 (mean, 15.9) in MLs. There were no significant differences in SUVmax between KFDs and MLs. 18F-FDG PET/CT with ML patients showed hot uptakes in the extranodal organs, such as bone marrow, small bowel, thymus, kidney, orbit and pleura. However, none of the KFD cases showed extranodal uptake (P<0.001). 18F-FDG PET/CT findings of KFD with nodal involvement only were indistinguishable from those of ML.
Patients who had extranodal involvement on PET/CT were more likely to have malignancy than KFD.
Kikuchi-Fujimoto disease; Lymphoma; Positron emission tomography
Percutaneous transhepatic cholangioscopy (PTCS) is the most widely used modality for diagnosis and treatment of biliary disease. Although many other novel technologies have been developed based on recent advances in endoscopy, PTCS has its own role. In diagnostics, PTCS is used for evaluation of indeterminate biliary strictures, bile duct tumors, and postoperative biliary strictures that cannot be reached by a peroral approach. In therapeutics, the removal of bile duct stones, dilatation of bile duct strictures including postoperative anastomosis site strictures, and local tumor therapy are indications of PTCS. Especially in a therapeutic role, PTCS has the advantage of maneuverability due to a shorter endoscopic length compared to other cholangioscopic modalities. Hence, PTCS has its own indispensable diagnostic and therapeutic roles.
Percutaneous transhepatic cholangioscopy; Diagnostic role; Therapeutic role
A 58-year-old woman presented with a solitary myofibroma that arose in the sigmoid colon. Computed tomography revealed a highly enhanced intramural mass (1.3-cm maximum diameter) in the proximal sigmoid colon. Histologically, the tumor exhibited a biphasic growth pattern, which comprised haphazardly arranged, interwoven fascicles of plump, myoid-appearing spindle cells with elongated nuclei and abundant eosinophilic cytoplasm, and more cellular areas of primitive-appearing polygonal cells that were arranged in a hemangiopericytomatous pattern. The tumor cells were positive for smooth muscle actin (SMA), and negative for desmin, h-caldesmon, CD34, cytokeratin, S100 protein, and CD117. The Ki-67 labeling index was not high (up to 7%). Based on these histologic and immunohistochemical features, our patient was diagnosed with a myofibroma of the sigmoid colon. The presence of solitary myofibroma in the intestine of an adult requires attention to avoid misdiagnosis as a more aggressive mesenchymal tumor.
The virtual silde(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2096403796957687
Myofibroma; Solitary; Sigmoid colon; Adult
Elevated levels of serum amyloid A (SAA) is a risk factor for cardiovascular diseases, however, the role of SAA in the pathophysiology of atherosclerosis remains unclear. Here we show that SAA induced macrophage foam cell formation. SAA-stimulated foam cell formation was mediated by c-jun N-terminal kinase (JNK) signaling. Moreover, both SAA and SAA-conjugated high density lipoprotein stimulated the expression of the important scavenger receptor lectin-like oxidized low-density lipoprotein receptor 1 (LOX1) via nuclear factor-κB (NF-κB). A LOX1 antagonist carrageenan significantly blocked SAA-induced foam cell formation, indicating that SAA promotes foam cell formation via LOX1 expression. Our findings therefore suggest that SAA stimulates foam cell formation via LOX1 induction, and thus likely contributes to atherogenesis.
Serum amyloid A; Foam cell; Atherosclerosis; Lectin-like oxidized low-density lipoprotein; receptor 1
There is confusion in the diagnosis and biological behaviors of gastroenteropancreatic neuroendocrine tumors (GEP-NETs), because of independently proposed nomenclatures and classifications. A standardized form of pathology report is required for the proper management of patients.
We discussed the proper pathological evaluation of GEP-NET at the consensus conference of the subcommittee meeting for the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists. We then verified the prognostic significance of pathological parameters from our previous nationwide collection of pathological data from 28 hospitals in Korea to determine the essential data set for a pathology report.
Histological classification, grading (mitosis and/or Ki-67 labeling index), T staging (extent, size), lymph node metastasis, and lymphovascular and perineural invasion were significant prognostic factors and essential for the pathology report of GEP-NET, while immunostaining such as synaptophysin and chromogranin may be optional. Furthermore, the staging system, either that of the 2010 American Joint Cancer Committee (AJCC) or the European Neuroendocrine Tumor Society (ENETS), should be specified, especially for pancreatic neuroendocrine neoplasms.
A standardized pathology report is crucial for the proper management and prediction of prognosis of patients with GEP-NET.
Neuroendocrine tumors; Digestive system; Pathology; Staging; Grading; Prognosis
A 36-year-old man visited Yeungnam University Hospital with a sudden onset of palpitation, headache, and was found to be hypertensive. Chest radiography showed a 6 cm sized mass lesion on the posterior mediastinum. A biochemical study showed elevated levels of catecholamines. An I-123 metaiodobenzylguanidine scan revealed a hot uptake lesion on the posterior mediastinum. The patient was prepared for surgery with α and β blocking agents. Two months later, we removed the tumor successfully. A histological study proved that the resected tumor was mediastinal pheochromocytoma. Functional mediastinal pheochromocytomas are rare. Therefore, we reported the case with a literature review.
Mediastinum; Surgery; Hypertension; Pheochromocytoma
Vascular abnormality of the intestine is rare, except angiodysplasia. We report on an unusual case of atypical florid vascular proliferations of the appendix. A 41-year old male presented with melena. Adhesioned blood clots in the appendiceal orifice were observed by colonoscopy. He underwent laparoscopic appendectomy. Microscopically, a tiny exophytic polypoid mass was observed. The mass showed pyogenic granuloma-like features in the superficial portion and infiltrative florid vascular proliferations in the deeper portion. Endothelial cells showed minimal nuclear atypia, and mitotic figures were observed infrequently and showed positivity for CD31 and CD34 and negativity for HHV-8. Differential diagnoses include from benign vascular tumor to angiosarcoma or Kaposi’s sarcoma, but this lesion does not fit the description of any defined vascular entity. We diagnosed atypical florid vascular proliferations and the patient has been well during the five-month postoperative follow-up.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1386921325843104
Blood vessel; Proliferation; Atypical; Appendix
Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.
Intradural extramedullary spinal cord neoplasm; Neonate; Congenital
As a result of various independently proposed nomenclatures and classifications, there is confusion in the diagnosis and prediction of biological behavior of gastroenteropancreatic neuroendocrine tumors (GEP-NETs). A comprehensive nationwide study is needed in order to understand the biological characteristics of GEP-NETs in Korea.
Materials and Methods
We collected 4,951 pathology reports from 29 hospitals in Korea between 2000 and 2009. Kaplan-Meier survival analysis was used to determine the prognostic significance of clinicopathological parameters.
Although the GEP-NET is a relatively rare tumor in Korea, its incidence has increased during the last decade, with the most significant increase found in the rectum. The 10-year survival rate for well-differentiated endocrine tumor was 92.89%, in contrast to 85.74% in well differentiated neuroendocrine carcinoma and 34.59% in poorly differentiated neuroendocrine carcinoma. Disease related death was most common in the biliary tract (62.2%) and very rare in the rectum (5.2%). In Kaplan-Meier survival analysis, tumor location, histological classification, extent, size, mitosis, Ki-67 labeling index, synaptophysin expression, lymphovascular invasion, perineural invasion, and lymph node metastasis showed prognostic significance (p<0.05), however, chromogranin expression did not (p=0.148). The 2000 and 2010 World Health Organization (WHO) classification proposals were useful for prediction of the prognosis of GEP-NET.
The incidence of GEP-NET in Korea has shown a remarkable increase during the last decade, however, the distribution of tumors in the digestive system differs from that of western reports. Assessment of pathological parameters, including immunostaining, is crucial in understanding biological behavior of the tumor as well as predicting prognosis of patients with GEP-NET.
Gastro-enteropancreatic neuroendocrine tumor; Incidence; Prognosis; Pathology
Follicular dendritic cell sarcoma (FDCS) is a rare malignancy arising from the antigen-presenting cells in the lymph node and extranodal tissue. We describe a 31-year-old male patient who presented with a swelling of the left parapharynx. The radiologic findings showed a 4.7×4.5×1.9 cm-sized, ill-defined mass in the left parapharyngeal space. A fine-needle aspiration cytology was performed and it showed scattered, irregular, cohesive clusters of tumor cells with a spindle-to-ovoid shape with irregular contours in a background of lymphocytes. Based on these findings, a diagnosis of spindle cell neoplasm was made. The surgically resected tumor was composed of elongated, ovoid or polygonal cells showing positive immunohistochemistry for CD21, CD23, and CD35. Postoperatively, the residual tumor was observed to undergo a rapidly growth. There is an overlap in the cytologic and histologic findings between FDCS of the parapharynx and other tumors. Pathologists should therefore be aware of its characteristics not only to provide an accurate diagnosis but also to recommend the appropriate clinical management.
Dendritic cell sarcoma, follicular; Extranodal; Parapharynx
Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described mesenchymal tumor of the stomach. We report the first case of PAMT in Korea. A 52-yr-old man underwent esophagogastroduodenoscopy due to dyspepsia for 2 yr. There was a submucosal mass with small mucosal ulceration in the gastric antrum. The tumor measured 3.5 × 2.3 cm in size and showed multinodular plexiform growth pattern of bland-looking spindle cells separated by an abundant myxoid or fibromyxoid matrix rich in small thin-walled blood vessels. The tumor cells were negative for CD117 (c-KIT), CD34 and S-100 protein, but diffusely positive for smooth muscle actin consistent with predominant myofibroblastic differentiation. The patient is doing well without recurrence or metastasis for 5 months after surgery. Although there have been limited follow-up data, PAMT is regarded as a benign gastric neoplasm with histological and immunohistochemical charateristics distinguished from gastrointestinal stromal tumor and other mesenchymal tumors of the stomach.
Plexiform Angiomyxoid Myofibroblastic Tumor; Stomach; Myofibroblast; Fibromyxoma; Plexiform
Survivin, a member of the inhibitors of apoptosis protein family, is expressed during development and in various human cancers. However, the clinical relevance of survivin in cancer is still a matter of debate. Genes induced by hepatocyte growth factor (HGF) were screened using cDNA microarray technology in the stomach cancer cell lines, NUGC3 and MKN28. The levels of JunB, survivin, and uro-plasminogen activator (uPA) were up-regulated in cells treated with HGF in a dose-dependent manner. HGF-induced up regulation of JunB, survivin, and uPA was inhibited by pre-treatment with a MEK inhibitor (PD 98059). HGF-induced up-regulation of uPA was repressed by survivin knockdown. HGF enhanced the binding activity of JunB to the survivin promoter in control cells, but not in the JunB-shRNA cells. Transfection with survivin-shRNA resulted in a decrement of cell proliferation, as determined with MTT assays. In an in vitro invasion assay, significantly fewer cells transfected with survivin shRNA than control cells were able to invade across a Matrigel membrane barrier. In conclusion, survivin appeared to play an important role in the up-regulation of uPA induced by HGF via JunB and might contribute to HGF-mediated tumor invasion and metastasis, which may serve as a promising target for gastric cancer therapy.
BIRC5 protein, human; hepatocyte growth factor; neoplasm metastasis; transcription factor AP-1; urokinase-type plasminogen activator
Hepatic involvement of amyloidosis is common. Diffuse infiltration with hepatomegaly is a usual radiologic finding of hepatic amyloidosis. To our knowledge, this is the first case of amyloidosis involving the liver that presented as a mass.
Amyloidosis; Computed tomography (CT)
Ganglion cysts usually arise from the tendon sheaths and tissues around the joints. It is usually associated with degenerative arthritic changes in older people. Ganglion cyst in the spine is rare and there is no previous report on case that located in the intervertebral foramen and compressed dorsal root ganglion associated severe radiculopathy. A 29-year-old woman presented with severe left thigh pain and dysesthesia for a month. Magnetic resonance imaging revealed a dumbbell like mass in the intervertebral foramen between second and third lumbar vertebrae on the left side. The lesion was removed after exposure of the L2-L3 intervertebral foramen. The histological examination showed fragmented cystic wall-like structure composed of fibromyxoid tissue but there was no lining epithelium. A ganglion cyst may compromise lumbar dorsal root ganglion when it located in the intervertebral foramen. Although it is very rare location, ganglion cyst should be included in the differential diagnosis for intervertebral foraminal mass lesions.
Ganglion cyst; Radiculopathy; Lumbar vertebra; Posterior longitudinal ligament
Cures for advanced hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) are rare and difficult. We report a case of pathologically confirmed complete remission of HCC induced by hepatic arterial infusion chemotherapy (HAIC). A 45-year-old male patient had a massive HCC in the right lobe of the liver and tumor thrombus in the right and main portal veins. He achieved a partial response after two cycles of HAIC with 5-fluorouracil (750 mg/m2) and cisplatin (25 mg/m2). After the completion of six cycles he received a curative partial hepatectomy, and histopathology revealed complete necrosis without any viable tumor cell. He was in good health at a 4-month follow-up. These results suggest that this regimen is a promising therapeutic modality for the treatment of advanced HCC with PVTT.
Hepatocellular carcinoma; Portal vein tumor thrombosis; Chemotherapy; 5-fluorouracil; Complete remission
Flash pulmonary edema typically exhibits sudden onset and resolves rapidly. It generally is associated with bilateral renal artery stenosis or unilateral stenosis in conjunction with a single functional kidney. We describe a patient who presented with flash pulmonary edema treated by percutaneous therapy with stent implantation. Our case is unique in that the flash pulmonary edema occurred in the setting of unilateral renal artery stenosis with bilateral functioning kidneys.
Pulmonary edema; Renal artery obstruction; Stents
Intramural hematoma formation is not a well-studied complication of percutaneous coronary intervention. We describe a patient with stable angina who developed an intramural hematoma during elective percutaneous coronary intervention (PCI) in the right coronary artery (RCA). Total occlusion with dense dye staining developed a long way from the distal RCA, near the posterior descending artery bifurcation site. The true lumen was compressed by the enlarged, tense, false lumen. The patient was successfully treating with intravascular ultrasound-guided fenestration using a cutting balloon, and a stent was implanted in the distal RCA.
Hematoma; Angioplasty; Intravascular ultrasonography
To measure the hypermethylation of four genes in primary tumors and paired plasma samples to determine the feasibility of gene promoter hypermethylation markers for detecting breast cancer in the plasma.
Materials and Methods
DNA was extracted from the tumor tissues and peripheral blood plasma of 34 patients with invasive breast cancer, and the samples examined for aberrant hypermethylation in cyclin D2, retinoic acid receptor β (RARβ), twist and high in normal-1 (HIN-1) genes using methylation-specific PCR (MSP), and the results correlated with the clinicopathological parameters.
Promoter hypermethylation was detected at high frequency in the primary tumors for cyclin D2 (53%), RARβ (56%), twist (41%) and HIN-1 (77%). Thirty-three of the 34 (97%) primary tumors displayed promoter hypermethylation in at least one of the genes examined. The corresponding plasma samples showed hyperme thylation of the same genes, although at lower frequencies (6% for cyclin D2, 16% for RARβ, 36% for twist, and 54% for HIN-1). Overall, 22 of the 33 (67%) primary tumors with hypermethylation of at least one of the four genes also had abnormally hypermethylated DNA in their matched plasma samples. No significant relationship was recognized between any of the clinical or pathological parameters (tumor size, axillary lymph node metastasis, stage, or Ki-67 labeling index) with the frequency of hypermethylated DNA in the primary tumor or plasma.
The detection of aberrant promoter hypermethylation of cancer-related genes in the plasma may be a useful tool for the detection of breast cancer.
Methylation; Plasma; Breast neoplasms