Solitary Fibrous Tumor of the Pleura (SFTP) is an uncommon neoplasm which grows slowly. For some cases, surgery is warranted. However, for unresectable ones, the standard strategy has not been established yet. We presented a rare case of recurrent malignant intrathoracic solitary fibrous tumor. It was impossible to resect the tumor. Radiotherapy alone achieved a significant improvement effect.
Radiotherapy; recurrent; solitary fibrous tumor of the pleura
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.
Patients with acute coronary syndrome (ACS) rarely manifest as recurrent syncope due to malignant ventricular arrhythmia. We report a case of a 56-year-old Chinese male with complaints of paroxysmal chest burning sensation and distress for 2 weeks as well as loss of consciousness for 3 days. The electrocardiogram (ECG) revealed paroxysmal multimorphologic ventricular tachycardia during attack and normal heart rhythm during intervals. Coronary angiograph showed 90% stenosis in left main coronary artery and 80% stenosis in anterior descending artery. Two stents sized 4.0∗18 mm and 2.75∗18 mm were placed at left main coronary artery and anterior descending artery, respectively, during percutaneous coronary intervention (PCI). The patient was discharged and never had ventricular arrhythmia again during a 3-month follow-up since the PCI. This indicated that ventricular tachycardia was correlated with persistent severe myocardial ischemia. Coronary vasospasm was highly suspected to be the reason of the sudden attack and acute exacerbation. PCI is recommended in patients with both severe coronary artery stenosis and ventricular arrhythmia. Removing myocardial ischemia may stop or relieve ventricular arrhythmia and prevent cardiac arrest.
Central nervous system (CNS) cryptococcosis is an opportunistic fungal infection that typically occurs in patients with reduced immunological function, such as patients with AIDS, patients receiving organ transplants, or patients receiving corticosteroid and immunosuppressive therapy. CNS cryptococcosis rarely occurs in immunocompetent patients. CNS cryptococcosis is characterized by meningitis and encephalitis and occasionally forms isolated granulomas. Isolated cerebellar cryptococcoma is a rare condition, especially in immunocompetent patients, and the misdiagnosis rate is high. A definite diagnosis must be based on pathology. To raise awareness of this disease, the clinical data of a patient with cryptococcomas in the right side of the cerebellum are reported.
cryptococcosis; cerebellar; central nervous system; magnetic resonance imaging; immunosuppressive
Primary rapidly progressive malignant tumor on breast is a very rare disease. We report a 62-year-old postmenopausal patient with history of one year breast lump and progressive increase in a period of two months. The giant breast tumor with a rare size about 7.5 × 7.0 cm, the skin of the breast to become resembling orange peel and metastasis to multiple organs. The histological sections of core needle biopsy confirmed the diagnosis of invasive adenocarcinoma. Hence, reduction surgery for local skin in the treatment of breast cancer increased the effectiveness of the docetaxel epirubicin (TE: docetaxel 75 mg/m2, day 1; epirubicin 75 mg/m2, day 1) chemotherapy by improving the patient’s general condition, and not continue to increase was observed during follow-up.
Breast cancer; giant tumor; pathology; tumor growth
Malignant germ cell tumors of the ovary are very rare and account for about 2-5% of all ovarian tumors of germ origin. Most patients are adolescent and young women, approximately two-thirds of them are under 20 years of age, occasionally in postmenopausal women. But clear cell carcinoma usually occurs in older patients (median age: 57-year old), and closely related with endometriosis. Here we report a case of a 55-year old woman with right ovarian mass that discovered by B ultrasonic. Her serum levels of human chorionic gonadotropin (hCG) and α-fetoprotein (AFP) were elevated. Pathological examination revealed the tumor to be a mixed germ cell tumor (yolk sac tumor, embryonal carcinoma and mature teratoma) with clear cell carcinoma in a background of endometriosis. Immunohistochemical staining showed SALL4 and PLAP were positive in germ cell tumor area, hCG, CD30 and OCT4 were positive in epithelial-like cells and giant synctiotrophoblastic cells, AFP, AAT, CD117 and Glyp3 were positive in yolk sac component, EMA and CK7 were positive in clear cell carcinoma, CD10 was positive in endometrial cells of endometriotic area. She was treated with surgery followed by seven courses of chemotherapy. She is well and serum levels of hCG and AFP have been decreased to normal levels.
Mixed germ cell tumor; ovary; clear cell carcinoma
Diffuse alveolar hemorrhage (DAH) is a life-threatening clinical pathologic syndrome caused by a variety of diseases. We report a case of DAH related to combination therapy of chemoradiotherapy and erlotinib. As to know, DAH following chemoradiotherapy was only reported among hematopoietic stem cell transplant recipients with hematologic malignancies till now. DAH associated with chemoradiotherapy for oesophageal carcinoma has not been reported. This is the first DAH report on erlotinib-combined chemoradiotherapy for esophageal cancer. The authors believe epidermal growth factor receptor tyrosine kinase inhibitor erlotinib increased the lung injury. Molecular targeted drugs are gradually applied to be combined with chemoradiation, whether this combination will cause the increase of serious adverse reactions need further study. This case can provide certain reference for erlotinib in the treatment. Meanwhile, after long term hormone therapy for DAH, the patient was diagnosed with pneumocystis carinii pneumonia. It reminds us to attach importance to the immunosuppressive diseases after long-term hormone treatment.
Diffuse alveolar hemorrhage; chemoradiotherapy; erlotinib; esophageal carcinoma
Systemic Epstein-Barr virus-positive T/natural killer-cell lymphoproliferative disorder (EBV + LPD) has predominantly been observed among pediatric patients as a life-threatening condition. The present study presents a rare case of EBV + LPD in an adult with good outcome. This patient’s history is more than 2 years and her condition was stable. She received 6 cycles of chemotherapy cyclophosphamide/doxorubicin/vincristine/prednisolone (CHOP). The evaluation was complete remission. The low levels of EBV-DNA in the peripheral blood may have potential benefit factor for the sensitivity to the chemotherapy and good outcome.
Epstein-Barr virus; T/natural killer-cell; lymphoproliferative disorder
Carcinoid tumors of the middle ear are very rare. Here we describe a 37-year-old man with multiple recurrent carcinoid tumor of the right middle ear. The CT demonstrated the recurrent mass that filled the tympanum and mastoid with osteolytic invasion, and the tumor was removed by surgery. The pathological findings showed the tumor cells, without necrosis and mitotic activity, had round, oval, or slightly irregular nuclei and finely-dispersed chromatin, arranged in cords, nests, and glandular structures. They were strongly positive for synaptophysin and CD56, but were negative for S-100 and chromogranin A. Ki-67 proliferation activity was low (<2%). With a review of the literature, the clinical, pathological characteristics and treatment modalities of this rare tumor are discussed.
Middle ear; carcinoid; neuroendocrine carcinoma; immunohistochemistry
T-lymphoblastic lymphoma (T-LBP) is a high-grade malignant lymphoma, which possesses the characteristic of high metastasis and high mortality without treatment. We are presenting a special T-lymphoblastic proliferation involving in the oropharynx, nasopharynx, sinus and trachea in a patient with local involved about 15-years without systemic dissemination. The immunophenotype of this case was similar to T-LBP. The proliferous cells were positive for terminal deoxynucleotidyl transferase (TdT), CD3, and appeared co-expression CD4 and CD8. No clonal rearrangements of TCRγ and/or TCRβ gene were detected. Indolent T-lymphoblastic proliferations rarely occurred or unusually could not be diagnosed, combing with the relevant literature and clinically indolent manifestation, we interpreted this case as indolent T-lymphoblastic proliferation (iT-LBPs). So far, the mechanism of the T-lymphoblastic proliferations is still uncertain and requires further study.
T-lymphoblastic lymphoma; indolent T-lymphoblastic proliferation; upper aerodigestive tract
This is a case report of a 67-year-old patient with distant metastasis of prostate cancer to the right ureter which caused hydronephrosis. At the beginning, both of the cytology of the morning urine and imaging findings were consistent with urothelial carcinoma. Nephroureterectomy was subsequently performed. Interestingly, the pathological examination of the excised ureter revealed that the malignancy was derived from the prostate. No skeletal metastasis was detected. However, after four months of follow-up, several abnormal signal shadows were reported in skeletal scintigraphy and the prostate specific antigen (PSA) was gradually increasing. We present such a case for its unique presentation. A review of the literature is also provided.
The desmoplastic small round cell tumor (DSRCT) is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. A 14-year-old girl presented with intermittent epigastric pain; abdominal computed tomography and upper gastrointestinal barium X-ray revealed an 8 cm × 10 cm space-occupying mass in the duodenal region. The patient underwent pancreaticoduodenectomy and the final pathologic diagnosis was DSRCT. Although multi-agent systemic chemotherapy was given, the patient died of metastasis 8 months later. Early diagnosis and surgical treatment with adjuvant chemotherapy seems to be the best treatment choice for this disease.
chemotherapy; desmoplastic small round cell tumor; duodenum; surgery
The association of low-grade endometrioid carcinoma with undifferentiated carcinoma (UC) was first reported in endometrium carcinoma, termed with dedifferentiated carcinoma (DC). However, the coexistence of low-grade endometrioid carcinoma (LGEC) or serous carcinoma (LGSC) with UC has received minimal attention in ovary, and the behavior of this kind of neoplasm remains at further discussion. In this study, we reported a case of low-grade ovarian endometrioid carcinoma associated with UC and reviewed another four cases previously reported. We found a histological continuity between the LGEC and UC components in H&E section, which suggested a dedifferentiation from LGEC to UC components. In summary, this kind of pathological type has aggressive behavior and these patients have very poor prognosis regardless of the amount of undifferentiated carcinoma.
Ovarian carcinoma; undifferentiated carcinoma; dedifferentiated carcinoma; low-grade endometrioid carcinoma
Pancreatic tuberculosis (TB) is a rare condition, even in immunocompetent hosts. A case is presented of pancreatic TB that mimicked pancreatic head carcinoma in a 40-year-old immunocompetent male patient. The patient was admitted to our hospital after suffering for nine days from epigastralgia and obstructive jaundice. Computed tomography revealed a pancreatic mass that mimicked a pancreatic head carcinoma. The patient had undergone an operation four months prior for thoracic TB and was undergoing anti-TB therapy. A previous abdominal ultrasound was unremarkable with the exception of gallbladder steroid deposits. The patient underwent surgery due to the progressive discomfort of the upper abdomen and a mass that resembled a pancreatic malignancy. A biopsy of the pancreas and lymph nodes was performed, revealing TB infection. The patient received a cholecystostomy tube and recovered after being administered standard anti-TB therapy for 15 mo. This case is reported to emphasize the rare contribution of pancreatic TB to pancreatic masses and obstructive jaundice.
Anti-tuberculosis therapy; Pancreatic head carcinoma; Pancreatic tuberculosis; Pancreatic mass; Tube cholecystostomy
Lipoblastoma is a rare benign mesenchymal tumor that composes of embryonal white fat tissue and typically occurs in infants or young children under 3 years of age. It usually affects the extremities, trunk, head, and neck. The perineum is a rare location with only 7 cases reported in the literature. We describe a case of 3-year-old girl with a lipoblastoma arising from perineum. An approximately 4.5 cm × 3.5 cm × 2.5 cm nodule was resected in left perineum with satisfied results. Pathological examination showed that it was composed of small lobules of mature and immature fat cells, separated by fibrous septa containing small dilated blood vessels. The left perineal lipoblastoma, although rare, should be differentiated from some other mesenchymal tumors with similar histologic and cytological features.
Perineum; lipoblastoma; myxoid liposarcoma; childhood; pathology
Primary bone lymphoma (PBL) is a rare but distinct clinicopathological disease. Because it is not common, the optimal treatment strategy has not been established. Here, we present a patient with PBL of the left radius and review the related literature. We focus on the standard treatment for PBL. Many aspects such as rehabilitation, local control and overall survival need to be considered. Studies on this disease should be carried out to clarify the optimal treatment in the future.
Bone lymphoma; Chemotherapy; Radiotherapy
Chorea is a rare complication of polycythemia. We report the case of a 70 year-old woman whose polycythemia vera (PV), with Janus Kinase-2 (JAK2) mutation, presented as chorea. Chorea resolved quickly after hydroxyurea therapy.
chorea; polycythemia vera; elderly; JAK2
Gastric duplication cysts are rare congenital abnormalities, and malignant transformation of these duplications is also thought to be rare.
During a routine health checkup, a 28-year-old man underwent abdominal sonography followed by computed tomography (CT) with contrast agent, which revealed a cystic lesion with no enhancement. Laparoscopic surgery showed a 10 × 10 cm cyst adhering to the gastric corpus. However, attempts to remove the lesion en bloc were unsuccessful, and the ruptured cyst had contaminated the peritoneal cavity. Gastric duplication was diagnosed from microscopic examination of the cyst. Seven months later, the patient suffered a progressive increase in ascites, and repeated cytological analysis showed small nests of adenocarcinoma cells, with primary lesion unknown. Diagnostic laparoscopy showed multiple white nodules scattered over the surface of the liver, greater omentum, and peritoneum. Biopsy of the omental nodules confirmed adenocarcinoma, while carcinomatosis was diagnosed in the peritoneum.
Clinical presentation and chronological developments indicated that the malignancy probably originated from the gastric duplication cyst. This case highlights the importance of accurate preoperative diagnosis and optimal surgical management for gastric duplication as well as considering the potential existence of malignant transformation during surgical evaluation of adult patients with gastric duplication cysts.
Endovascular aneurysm repair (EVAR) has been a revolutionary development in the treatment of abdominal aortic aneurysms (AAAs). Meanwhile, unfavorable anatomy of the aneurysm has always been a challenge to vascular surgeons, and the application of EVAR in emergent and elderly patients are still in dispute.
A 79-year-old woman presented as an emergency of abdominal pain with acute hypotension, heart rate elevation and a rapid decrease of hemoglobin. Emergent computed tomographic angiography (CTA) showed a ruptured AAA (rAAA) extending from below the opening of bilateral renal arteries down to the celiac artery and elongated to both common iliac arteries. The hostile neck and severely tortuous iliac artery made the following procedure a great challenge. An emergent endovascular approach was performed in which an excluder aortic main body was deployed below the origin of the bilateral renal arteries covering the ruptured aortic segment. Two iliac legs were placed superior to the opening of the right hypogastric respectively. In order to avoid the type Ib endoleak, we tried to deploy another cuff above the bifurcation of the iliac artery. However, the severely tortuous right iliac artery made this procedure extremely difficult, and a balloon-assisted technique was used in order to keep the stiff wire stable. Another iliac leg was placed above the bifurcation of the left iliac artery. The following angiography showed a severe Ia endoleak in the proximal neck and therefore, a cuff was deployed distal to opening of the left renal artery with off-the-shelf solution. The patient had an uneventful recovery with a resolution of the rAAA. She is well and symptom-free 6 months later.
Endovascular aneurysm repair (EVAR) in emergent elderly rAAA with hostile neck and severe tortuous iliac artery is extremely challenging, and endovascular management with integrated technique is feasible and may achieve a satisfactory early result.
Endovascular aneurysm repair (EVAR); Ruptured abdominal aortic aneurysm (rAAA); Hostile anatomy; Elderly patient
The first case of human infection with avian influenza A (H7N9) virus was identified in March, 2013 and the new H7N9 virus infected 134 patients and killed 45 people in China as of September 30, 2013. Family clusters with confirmed or suspected the new H7N9 virus infection were previously reported, but the family cluster of H7N9 virus infection in Shandong Province was first reported.
A 36-year-old man was admitted to Zaozhuang City Hospital with progressive respiratory distress and suspicion of impending acute respiratory distress syndrome on April 21. The chest radiography revealed bilateral ground-glass opacities and pulmonary lesions. The second case, the first case’s 4 year old son, was admitted to the same hospital on April 28 with fever and multiple patchy shadows in the bilateral lungs. Both of the two cases were confirmed to infect with H7N9 virus by the results of real-time reverse transcriptase-polymerase-chain reaction (rRT-PCR), virus isolation and serum antibody titer. At the same time, one environment samples was detected positive for H7N9 virus in the living poultry market in Zaozhuang. The homologous analysis of the full genome sequence indicated that both viruses from the patients were almost genetically identical. The field epidemiology investigation showed that the two cases had no recognized exposure to poultry, but had the exposure to the environment. The second case had substantial unprotected close exposure to his ill father and developed symptoms seven days after his last contact with his father. After surgery, the index case and his son were discharged on May 16 and May 6, respectively. 11 close contacts of both patients were identified and tested negative both the throat swabs and the serum antibody.
The infection of the index case probably resulted from contact with environmentally contaminated material. For the son, the probable infection source was from the index case during unprotected exposure, but the possibility from the environment or other sources could not be completely ruled out.
Avian Influenza A (H7N9) virus; Epidemiology; Infectious source
A malignant fibrous histiocytoma (MFH) rarely originates from the chest wall.
In this case, we describe a 59-year-old Chinese woman who presented with an enormous mass originating from the left chest wall and involving the left upper pulmonary lobe.
After a radical en-block resection of the entire chest mass with left upper pulmonary lobectomy, and the chest wall reconstruction, a histopathologic diagnosis of the giant cell MFH was rendered. She has done well postoperatively, showing no local recurrence or distal disease in an 8-month follow-up period.
Although a MFH originating from the chest wall is rare, it should be considered in the differential diagnosis of a chest wall tumor.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8895569301129379
Malignant fibrous histiocytoma; Differential diagnosis; Chest wall tumor
Behcet’s disease (BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn’s disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.
Intestinal Behcet’s disease; Hemorrhage; Skip ulcers