Two cases of asymptomatic double aortic arch with tetralogy of Fallot are reported. One presented with a non-dominant left arch and left-sided descending thoracic aorta and the other with a non-dominant left arch, a right-sided descending thoracic aorta and a patent ductus arteriosus. One-stage operation was performed and both patients were discharged free of symptoms and signs related to the double aortic arch and tetralogy of Fallot after the operation. A preoperative recognition of DAA is important, especially in echocardiographic investigation.
Double aortic arch; Tetralogy of Fallot; Congenital heart disease
In this paper, we described the symptoms and treatment of a patient with diabetic nephropathy accompanied by spontaneous retroperitoneal hemorrhage after hemodialysis. An elderly female patient with diabetic nephropathy presented with severe pain, numbness, and an increasing swelling in the left hip and left thigh after six sessions of hemodialysis involving the use of an antiplatelet drug and an anticoagulant agent. Her hemoglobin decreased to 46 g/L. An abdominal ultrasound showed a hematoma in the left retroperitoneal space, and computed tomography (CT) findings revealed a 6 cm×8 cm×10 cm hematoma in the left psoas muscle. After aggressive supportive therapy [the administration of packed red blood cell transfusion, carbazochrome sodium sulfonate injection, and continuous venovenous hemofiltration (CVVH)], the patient’s vital signs stabilized and her hemoglobin increased to 86 g/L. Repeat CT showed that the hematoma had been partially absorbed after two weeks. Eventually, the patient was discharged with stable vital signs. Physicians should be aware of the possibility of spontaneous retroperitoneal hemorrhage, particularly in patients with diabetic nephropathy undergoing hemodialysis involving the use of anticoagulant agents.
Spontaneous retroperitoneal hemorrhage; Diabetic nephropathy; Hemodialysis; Nadroparin calcium
Glioblastoma multiforme (GBM) remains refractory to conventional therapy. CD133+ GBM cells have been recently isolated and characterized as chemo-/radio-resistant tumor-initiating cells and are hypothesized to be responsible for post-treatment recurrence. In order to explore the molecular properties of tumorigenic CD133+ GBM cells that resist treatment, we isolated CD133+ GBM cells from tumors that are recurrent and have previously received chemo-/radio-therapy. We found that the purified CD133+ GBM cells sorted from the CD133+ GBM spheres express SOX2 and CD44 and are capable of clonal self-renewal and dividing to produce fast-growing CD133− progeny, which form the major cell population within GBM spheres. Intracranial injection of purified CD133+, not CD133− GBM daughter cells, can lead to the development of YKL-40+ infiltrating tumors that display hypervascularity and pseudopalisading necrosis-like features in mouse brain. The molecular profile of purified CD133+ GBM cells revealed characteristics of neuroectoderm-like cells, expressing both radial glial and neural crest cell developmental genes, and portraying a slow-growing, non-differentiated, polarized/migratory, astrogliogenic, and chondrogenic phenotype. These data suggest that at least a subset of treated and recurrent GBM tumors may be seeded by CD133+ GBM cells with neural and mesenchymal properties. The data also imply that CD133+ GBM cells may be clinically indolent/quiescent prior to undergoing proliferative cell division (PCD) to produce CD133− GBM effector progeny. Identifying intrinsic and extrinsic cues, which promote CD133+ GBM cell self-renewal and PCD to support ongoing tumor regeneration may highlight novel therapeutic strategies to greatly diminish the recurrence rate of GBM.
Electronic supplementary material
The online version of this article (doi:10.1007/s11060-009-9919-z) contains supplementary material, which is available to authorized users.
Glioblastoma; Cancer stem cells; Self-renewal; Radial glial cells; Neural crest cells; Expression microarray
Escherichia coli strains causing postweaning diarrhea (PWD) and edema disease (ED) in pigs are limited to a number of serogroups, with O8, O45, O138, O139, O141, O147, O149, and O157 being the most commonly reported worldwide. In this study, a DNA microarray based on the O-antigen-specific genes of all 8 E. coli serogroups, as well as 11 genes encoding adhesion factors and exotoxins associated with PWD and ED, was developed for the identification of related serogroups and virulence gene patterns. The microarray method was tested against 186 E. coli and Shigella O-serogroup reference strains, 13 E. coli reference strains for virulence markers, 43 E. coli clinical isolates, and 12 strains of other bacterial species and shown to be highly specific with reproducible results. The detection sensitivity was 0.1 ng of genomic DNA or 103 CFU per 0.3 g of porcine feces in mock samples. Seventeen porcine feces samples from local hoggeries were examined using the microarray, and the result for one sample was verified by the conventional serotyping methods. This microarray can be readily used to screen for the presence of PWD- and ED-associated E. coli in porcine feces samples.
Pulmonary amyloidosis is rare and is often misdiagnosed due a lack of general awareness.
In this case report we describe a 69-year-old Chinese woman who presented with a right lower lobe pulmonary nodule. After video-assisted thoracoscopic lobectomy, a histopathologic diagnosis of pulmonary nodular amyloidosis was rendered. She has done well postoperatively, showing no local recurrence or distal disease in an 8-month follow-up period.
Distinguishing parenchymal nodular amyloidosis from a malignant lung tumor is often quite difficult. In the differential diagnosis of pulmonary nodules, nodular amyloidosis should be considered to avoid unnecessary lobectomy.
Amyloidosis; Differential diagnosis; Pulmonary nodules
Glomus tumors are uncommon benign tumors which usually arise from the distal portion of the digits. A tracheal glomus tumor with large size is extremely rare. We present a case of a large tracheal glomus tumor that was resected using posterolateral thoracotomy and successful primary reconstruction of the trachea was achieved. Severe haematemesis happened after the patient was discharged. An emergency exploratory thoracotomy was performed but no signs of anastomotic bleeding were observed, while intraoperative gastroscopy revealed plenty of blood and blood clots in the patient’s stomach. Medical treatments targeting hemorrhage of upper digestive tract were given and the patient finally recovered.
Glomus tumor; trachea; haematemesis
Hepatic pseudolesions detected by helical computed tomography (CT) are not rare, but it is difficult to make a final diagnosis when the hepatic lesion is complicated by the presence of greatly elevated alpha fetoprotein (AFP). Clinical treatment of non-typical hepatic pseudolesions complicated by greatly elevated AFP should confirm the diagnosis and minimize trauma.
Non-invasive procedures including ultrasonography, CT, and micro-invasive digital subtraction angiography could not safely differentiate this lesion from a malignant focus when it was complicated by greatly elevated AFP. Laparoscopic hepatectomy was performed, and pathological analysis showed chronic hepatitis, nodular regenerative hyperplasia, focal nodular hyperplasia of the liver, and mild vascular malformation. The tissue was HbsAg(−), HbcAg(−), and AFP(+).
Heightened awareness of hepatic pseudolesion complicated by primarily elevated AFP will help physicians avoid unnecessary invasive procedures. Hepatic biopsy is inevitable because of greatly elevated AFP. For suspected hepatic pseudolesion with elevated AFP, needle-core biopsy and follow-up surveillance instead of hepatectomy are recommended to find the source of AFP and make a final diagnosis of pseudolesion.
Hepatic pseudolesion; Alpha fetoprotein; Diagnosis
Total laparoscopic-assisted radical gastrectomy and the jejunal Roux-en-Y anastomosis were performed to treat cancer of the upper gastric body and fundic region. In the case of open anastomosis during total laparoscopic-assisted radical gastrectomy, an incision of 6-8 cm would be required due to the need for placing the stapler anvil. If using the Roux-en-Y procedure, however, the incision could be reduced to as small as 4-5 cm without increasing the length of operation and intraoperative bleeding that favors postoperative recovery.
Gastric cancer; laparoscopy; gastrectomy; gastrointestinal anastomosis
Intra-abdominal fibromatosis (IAF) commonly develops in patients who had abdominal surgery. In rare instances, it occurs subsequent to gastrointestinal stromal tumor (GIST). This special situation has clinical significance in imatinib era. About 1000 patients with GIST in our institution from 1993 to 2010 were re-evaluated based on their clinical and pathological data, the treatment strategies and the follow-up information. We identified 2 patients who developed IAF after GIST resection. Patient 1 was a 54 year-old male and had 5 cm × 4.5 cm × 3.5 cm jejunal GIST excised on February 22, 1994. Three years later, an abdominal mass with 7 cm × 6 cm × 3 cm was identified. He was diagnosed as recurrent GIST from clinical point of view. After excision, the second tumor was confirmed to be IAF. Patient 2 was a 45-year-old male and had 6 cm × 4 cm × 3 cm duodenal GIST excised on August 19, 2008. One year later, a 4 cm mass was found at the original surgical site. The patient refused to take imatinib until the tumor increased to 8 cm six months later. The tumor continued to increase after 6 months’ imatinib therapy, decision of surgical resection was made by multidisciplinary team. The second tumor was confirmed to be IAF with size of 17 cm × 13 cm × 11 cm. Although IAF subsequent to GIST is very rare, it is of clinical significance in imatinib era as an influencing factor for making clinical decision.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1076715989961803
GIST; Intra-abdominal fibromatosis (IAF); Imatinib
Epilepsy surgery has improved over the last decade, but non-seizure-free outcome remains at 10%–40% in temporal lobe epilepsy (TLE) and 40%–60% in extratemporal lobe epilepsy (ETLE). This paper reports a complex multifocal case. With a normal magnetic resonance imaging (MRI) result and nonlocalizing electroencephalography (EEG) findings (bilateral TLE and ETLE, with more interictal epileptiform discharges [IEDs] in the right frontal and temporal regions), a presurgical EEG-functional MRI (fMRI) was performed before the intraoperative intracranial EEG (icEEG) monitoring (icEEG with right hemispheric coverage). Our previous EEG-fMRI analysis results (IEDs in the left hemisphere alone) were contradictory to the EEG and icEEG findings (IEDs in the right frontal and temporal regions). Thus, the EEG-fMRI data were reanalyzed with newly identified IED onsets and different fMRI model options. The reanalyzed EEG-fMRI findings were largely concordant with those of EEG and icEEG, and the failure of our previous EEG-fMRI analysis may lie in the inaccurate identification of IEDs and wrong usage of model options. The right frontal and temporal regions were resected in surgery, and dual pathology (hippocampus sclerosis and focal cortical dysplasia in the extrahippocampal region) was found. The patient became seizure-free for 3 months, but his seizures restarted after antiepileptic drugs (AEDs) were stopped. The seizures were not well controlled after resuming AEDs. Postsurgical EEGs indicated that ictal spikes in the right frontal and temporal regions reduced, while those in the left hemisphere became prominent. This case suggested that (1) EEG-fMRI is valuable in presurgical evaluation, but requires caution; and (2) the intact seizure focus in the remaining brain may cause the non-seizure-free outcome.
EEG-fMRI; focus localization; presurgical evaluation; epilepsy surgery
Spontaneous atraumatic true axillary artery aneurysm is a relatively unusual disorder. Although most cases are asymptomatic, complications of axillary artery aneurysms may result in acute vascular insufficiency and neurological deficits. Prompt treatment, therefore, should be employed in the management of this condition. To date, the standard treatment for peripheral aneurysms is still surgical resection with end-to-end anastomosis. However, aneurysmectomy and interposition grafting with autologous or artificial vessels are more invasive and time-consuming. The ideal treatment for axillary artery aneurysm should be relatively noninvasive, safe and free of significant complications, cost-effective, cosmetically acceptable, and incur less absence from usual daily activities. Endovascular stent grafts have also been successfully used to treat these aneurysms. Management of select aneurysms using stent grafts has become more prevalent with the developing endoluminal technology.
We report a case of a spontaneous atraumatic axillary artery aneurysm where the patient was a 48-year-old ethnic Han Chinese woman with a gradually enlarging left axillary pulsatile mass. She was treated with endovascular stent grafts. The postoperative course of the patient was uneventful during the six-month follow-up.
We show that there are significant early advantages with the endovascular management technique versus the conventional operation in the management of axillary artery aneurysm.
Axillary artery aneurysm; Endovascular management
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/7644172219178472
Hydroa vacciniforme-like lymphoma (HVL) is a rare type of Epstein-Barr virus (EBV)-positive lymphoma of cytotoxic T-cell or natural killer cell origin that mainly affect children, characterized by a vesicopapular skin eruption that clinically resemble hydroa vacciniforme (HV). In current study, we report an adult patient with the tumor. The patient presented similar morphologic, immunophenotypic and genotypic changes of the disease with that occurred in children, whereas clinically, he showed a prolonged clinical course without hepatosplenomegaly or generalized lymphadenopathy. Whether there are some differences in biologic behavior between children and adults still remains unknown and it is necessary to collect more data to observe and to investigate in the future.
Hydroa vacciniforme-like lymphoma; Epstein-Barr virus; Adult
Aggressive natural killer cell leukemia/lymphoma (ANKL) is a rare aggressive form of NK-cell neoplasm. We report an uncommon case of 36-year-old male who showed jaundice and spontaneous splenic rupture. The diagnosis was established by the biopsy of liver and spleen. The monomorphous medium-size neoplastic cells infiltrated into portal areas and sinus of liver as well as the cords and sinus of the spleen. Necrosis, mitotic figures and significant apoptosis could be seen easily. These neoplastic cells demonstrated a typical immunophenotype of CD3ε+, CD56+, CD16+, Granzyme B+, TIA-1+. T-cell receptor γ (TCR-γ) gene rearrangement analysis showed germline configuration and the result of in situ hybridization for Epstein-Barr virus-encoded RNA (EBER-ISH) was positive. The patient has undergone an aggressive clinical course and died of multi-organ function failure 14 days later after admission. To the best of our knowledge, this is the first case of ANKL with spontaneous splenic rupture, and we should pay more attention to recognize it.
The virtual slide(s) for this article can be found here:
Aggressive natural killer-cell leukemia; Jaundice; Spontaneous splenic rupture
Situs inversus is an uncommon anomaly with rare incidence. Some cases of situs inversus totalis have been described with different types of associations. Here we report a case of situs inversus with carcinoma of the gastric cardia.
Situs inversus; Carcinoma of the gastric cardia; Case report
The incidence of Mycobacterium marinum infection has been increasing. First-line antituberculous drugs and other common antibiotics are effective for most cutaneous M. marinum infections; however, treatment failure still occurs in some rare cases. We report a case of a 70-year-old man with refractory cutaneous infection caused by M. marinum. Reasons for delayed diagnosis and related factors of the refractory infection are also discussed.
Samples of lesional skin were inoculated on Löwenstein–Jensen medium for acid-fast bacilli. Species of mycobacterium were identified by polymerase chain reaction–restriction fragment length polymorphism (PCR-RFLP) analysis. We then carried out genotyping by using mycobacterial interspersed repetitive units and sequencing of heat shock protein 65 (hsp65) and 16S rDNA genes.
Tissue cultures for acid-fast bacilli were positive. PCR-RFLP analysis and sequencing of hsp65 and 16S rDNA genes confirmed the isolated organisms to be M. marinum. Systemic therapy with rifampicin, clarithromycin, and amikacin empirically over 6 months led to complete resolution of skin lesions leaving only some residual scars.
Key diagnostic elements for M. marinum infections include a high index of suspicion raised by chronic lesions, poor response to conventional treatments, and a history of fish-related exposure. Strong clinical suggestion of M. marinum infection warrants initial empirical treatment. The duration of therapy is usually several months or even longer, especially for elderly patients. Amikacin can be considered in multidrug therapy for treatment of some refractory M. marinum infections.
amikacin; clarithromycin; skin infection; Mycobacterium marinum; nontuberculous mycobacteria
Intradural lumbar disc herniation is a rare complication of disc disease. The reason for the tearing of the dura matter by a herniated disc is not clearly known. Intradural disc herniations usually occur at the disc levels and are often seen at L4–L5 level but have also been reported at other intervertebral disc levels. However, intradural disc herniation at mid-vertebral levels is rare in the literature and mimics an intradural extramedullary spinal tumor lesion in radiological evaluation. Although magnetic resonance imaging (MRI) with gadolinium is useful in the diagnosis of this condition, preoperative correct diagnosis is usually difficult and the definitive diagnosis must be made during surgery. We describe here a 50-year-old female patient who presented with pain in the lower back for 6 months and a sudden exacerbation of the pain that spread to the left leg as well as numbness in both legs for 2 weeks. MRI demonstrated an intradural mass at the level of L5. Laminectomy was performed, and subsequently durotomy was also performed. An intradural disc fragment was found and completely removed. The patient recovered fully in 3 months. Intradural lumbar disc herniation must be considered in the differential diagnosis of mass lesions in the spinal canal.
Intervertebral disc herniation; Intradural disc herniation; Intraspinal tumor; L5
In 2006, dedifferentiated endometrioid adenocarcinoma (undifferentiated carcinoma associated with low-grade endometrioid carcinoma) of the uterus was first proposed. Dedifferentiated endometrioid carcinoma is part of the spectrum of undifferentiated carcinoma of the endometrium which is a highly aggressive tumor even when the undifferentiated component represents only 20% of the entire neoplasm. Therefore, accurate diagnosis and appropriate classification of this neoplasm are important in patient management. Lack of the recognition may lead to misclassification of dedifferentiated endometrioid adenocarcinoma as a pure endometrioid adenocarcinoma which is less aggressive. Only 4 papers have appeared in the literature so far on the topic of dedifferentiated endometrioid carcinoma. We report herein a first case of endometrial dedifferentiated endometrioid carcinoma in a 51-year old woman in Chinese population. We performed immunoperoxidase studies for 12 markers. Among them, cytokeratins, keratin 7, keratin 18, EMA, estrogen receptor (ER), progesterone receptor (PR), and vimentin show significantly differential expression between differentiated and undifferentiated area.
Endometrium; dedifferentiated endometrioid adenocarcinoma; undifferentiated carcinoma; endometrioid adenocarcinoma
A 60-year-old male patient was admitted to our hospital for recurrent upper quadrant pain for 1 month. He had a past history of coronary artery disease. After admission, he repeatedly suffered from high-grade fever, chills and upper quadrant pain. Computed tomography (CT) showed a round hypodense mass in the left lobe of the liver, approximately 2.7 × 2.2 cm in size, and a fish bone was confirmed by surgery in the left lobe of liver. The patient was cured completely after surgical removal of the fish bone and liver abscess. CT scan 1 month after discharge showed that the liver abscess had disappeared completely.
Liver abscess; Fish bone; Coronary artery disease; Gastric ulcer
An HIV infected hemophilia patient with huge inflammatory pseudotumor was in severe ill condition. The operation of right hip joint amputation was performed on the patient with perioperative infusion of coagulation factor VIII and highly active antiretroviral therapy (HAART). The pathological found that Inflammatory cell infiltration, formation of folliculus lymphaticus, muscular fiber breakage, and fibrous tissue hyperplasy, necrosis in both soft tissue and bone were seen in inflammatory pseudotumour. The vital signs remained stable during the operation and patient's overall health condition improved significantly ten months after operation. With the infusion of coagulation factor VIII and HAART, HIV infected hemophilia patient can be safety operation and may get effective result.
Angioleiomyomas (ALMs) involving the central nervous system are exceedingly rare, and no ALM involving the parafalx region has ever been clinically reported. We report the first case of ALM involving the parafalx region on its surgical procedure and radiological feature. A 50-year-old man had a 6 month history of frontal headache and occasional seizure attacks. The radiological examination showed a parafalx occupation similar to meningioma. The tumour was very hypointense on T1 weighted magnetic resonance imaging (MRI) and hyperintense on T2 weighted MRI, which enhanced homogeneously with intravenous administration of gadolinium. The tumour was removed totally by sinusoidal transverse scalp incision and bilateral parafalx approach. To our surprise, the tumour was prominently debulked using bipolar shrinkage to coagulate the tumour feeding vessels, resulting in less bleeding during total removal of the tumour.. The lesion was confirmed as ALM by histological examination. The prognosis was good for the patient after surgery during the 18 month follow-up.
Primary malignant fibrous histiocytoma (MFH) of the spleen is extremely rare. Since the first description of primary splenic MFH reported by Govoni et al in 1982, to the best of our knowledge, only 13 cases of MFH of the spleen have been reported in the English language literature in Medline. We herein report a rare case of primary splenic MFH accompanying with hepatic cyst in a 48-year-old Chinese female who treated successfully by laparoscopic splenectomy and fenestration, which has not yet been reported in the literature. Compared with the 13 previously cases of MFH of the spleen, our case is the first case accompanied with other disease, and also the first case treated successfully by laparoscopic splenectomy. A literature review of MFH of the spleen previously reported in the English language literature in Medline is also provided.
Malignant Fibrous Histiocytoma; Hepatic Cyst; Laparoscopic Splenectomy
Psammocarcinoma is a rare form of serous carcinoma of the ovary or peritoneum, and it is characterised by extensive psammoma body formation and invasion of surrounding structures. This report describes the case of a 42-year-old woman who presented with large ascites and raised CA125 level. Following a full staging laparotomy, she was made stable in stage IIIC. Despite the limited number of cases reported, the clinical prognosis of carcinomas resembling the serous borderline lesions seems much more favourable than the common serous carcinomas. A summary of all the reported cases is provided to highlight the clinical and prognostic features of this scarce tumour.
Bone size is an important determinant of bone strength and is under strong genetic control.
To identify quantitative trait loci (QTL) for areal bone size variation, a large‐scale genomewide linkage scan was carried out in 451 Caucasian families.
Participants and methods
Of 4124 people with phenotypes, 3899 were genotyped with 410 microsatellite markers. Multipoint linkage analyses were carried out in the entire sample, as well as in men and women separately. Potential epistatic interactions between identified genomic regions were also assessed.
Several potentially important genomic regions were identified, such as 8q24 for hip bone size (logarithm of the ratio of the odds that two loci are linked (LOD) 3.27) and 2p24 (LOD 2.04) for spine bone size. 8q24 may also interact with 19p13 to affect hip bone size. Several sex‐specific QTL were also detected, such as 14q21 (LOD 2.94) for wrist bone size in women and 16q12 (LOD 2.19) for hip bone size in men.
Together with previous findings, this study has further delineated the genetic basis of bone size and laid a foundation for future studies to eventually elucidate the mechanisms of bone size regulation and associated fracture risks.