Primary Endothelial Sarcoma of the Thoracic Aorta

Vascular ◽  
2004 ◽  
Vol 12 (2) ◽  
pp. 140-144
Author(s):  
Ralph I. Rückert ◽  
Birgit Rudolph ◽  
Patrik Rogalla ◽  
Michael Walter
Keyword(s):  
Thoracic Aorta ◽  
Poor Prognosis ◽  
Malignant Tumors ◽  
Polypoid Lesion ◽  
Aortic Tumor ◽  
Tumor Extirpation ◽  
Time Of Operation ◽  
Aortic Sarcoma ◽  
Primary Aortic Tumor

Primary malignant tumors of the aorta are extremely rare. The case of a 64-year-old woman who presented with peripheral embolism to both femoropopliteal arteries is reported. The search for a source revealed a polypoid lesion severely narrowing the lumen of the distal thoracic aorta. Differential diagnosis included thrombus and primary aortic tumor. Extirpation of the tumorous lesion was performed. Histologic examination revealed intimal aortic sarcoma of endothelial cell origin. Although the liver was the only site of suspected metastases at the time of operation, during the 18-month follow-up until the patient's death, generalized metastatic spread had developed. This case report thus demonstrates the generally poor prognosis of this rare variety of aortic sarcoma, in particular when symptoms have already occurred.

scholarly journals Manifestation of Pleomorphic Undifferentiated Aortic Sarcoma with Splenic Infarction: A Case Report

2020 ◽  
Vol 13 (2) ◽  
pp. 1002-1005
Author(s):  
Varun Kaushal ◽  
Behzad Amoozgar ◽  
Nabonita Barua ◽  
Shankar Chhetri ◽  
Bhaveshkumar Garsondiya
Keyword(s):  
Poor Prognosis ◽  
Spindle Cell ◽  
Tumor Resection ◽  
Splenic Infarction ◽  
Spindle Cell Sarcoma ◽  
Shortness Of Breath ◽  
Major Vein ◽  
Aortic Sarcoma ◽  
Ct And Mri

Aortic spindle cell sarcoma is a rare neoplasm with poor prognosis that is often found incidentally due to its adverse effects. CT and MRI with contrast are useful imaging modalities, but a tissue biopsy is the gold standard for diagnosis. Tumor resection is the ultimate treatment followed by chemotherapy. Our case was an adult female who presented mainly for shortness of breath, and further imaging workup demonstrated a soft tumor juxtaposed to a major vein with compressive effect. The patient’s tumor was resected, and the pathology result confirmed undifferentiated aortic sarcoma. The patient’s condition improved and she was discharged with outpatient oncology follow-up and possible treatment.

scholarly journals A Case of Malignant Melanoma Arising in Mediastinal Malignant Teratoma

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Ikuma Nozaki ◽  
Yumi Tone ◽  
Junko Yamanaka ◽  
Hideko Uryu ◽  
Yuko Shimizu-Motohashi ◽  
...  
Keyword(s):  
Malignant Melanoma ◽  
Malignant Transformation ◽  
Poor Prognosis ◽  
Mediastinal Mass ◽  
Bone Biopsy ◽  
Systematic Evaluation ◽  
Anterior Mediastinal Mass ◽  
Malignant Teratoma ◽  
Experienced Pain

We report about a 14-year-old boy who presented with an anterior mediastinal mass that was diagnosed as malignant teratoma. Surgical resection was performed along with pre- and postoperative chemotherapy. Although elevated alpha-fetoprotein became negative, he experienced pain in his right hip joint 3 months after resection. Systematic evaluation revealed multiple locations of metastasis, and the pathological diagnosis based on bone biopsy was malignant melanoma originating from malignant teratoma, which rapidly progressed. He died 15 months after diagnosis of the original malignant teratoma. Diagnosing and treating malignant transformation of teratoma, including malignant melanoma, is difficult because it is very rare. To our knowledge, this is the second reported case of malignant melanoma arising from a mediastinum malignant teratoma, with both cases having a poor prognosis. In addition to the follow-up of tumor markers, systematic evaluation, including imaging, should be considered even after remission to monitor malignant transformation of teratoma. We expect to establish a successful therapy and improve mortality rate after more such cases are accumulated.

Penile Lymphoepithelioma-Like Carcinoma: A Rare Case With PD-L1 Expression

2021 ◽  
pp. 106689692098834
Author(s):  
Raquel Machado-Neves ◽  
Bernardo Teixeira ◽  
Elsa Fonseca ◽  
Pedro Valente ◽  
Joaquim Lindoro ◽  
...  
Keyword(s):  
Human Papillomavirus ◽  
Tumor Cells ◽  
Rare Case ◽  
Malignant Tumors ◽  
Squamous Cell Carcinomas ◽  
The Third ◽  
The Past ◽  
First Case ◽  
Lymphoplasmacytic Infiltrate

Most malignant tumors of the penis are squamous cell carcinomas (SCC), being divided in 2 groups, one human papillomavirus (HPV)-related and another non-HPV-related, with lymphoepithelioma-like carcinoma (LELC) being one of the rarest HPV-related SCC. In this article, we report a case of a 50-year-old man who presented testicular swelling and pain for the past 3 months. A penile mass was identified, and the patient was submitted to a total penectomy. The penectomy specimen showed an ulcerated lesion at the glans reaching the cavernous bodies. Microscopic examination showed undifferentiated epithelial cells with syncytial growth pattern mix with a dense lymphoplasmacytic infiltrate, consistent with LELC. The tumor cells expressed p16 and all 3 different clones of PDL1 (22C3, SP263, and SP142). The patient is alive and well with a follow-up of 3 months. To our knowledge, this is the third LELC of the penis reported in literature and the first case reported with PDL1 expression.

scholarly journals Heterogeneity of the Clinical Presentation of the MEN1 LRG_509 c.781C>T (p.Leu261Phe) Variant Within a Three-Generation Family

Genes ◽  
2021 ◽  
Vol 12 (4) ◽  
pp. 512
Author(s):  
Aleksandra Gilis-Januszewska ◽  
Anna Bogusławska ◽  
Kornelia Hasse-Lazar ◽  
Beata Jurecka-Lubieniecka ◽  
Barbara Jarząb ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Clinical Presentation ◽  
Age Of Onset ◽  
Malignant Tumors ◽  
Genetic Disorder ◽  
Family Members ◽  
Pancreatic Neuroendocrine Tumor ◽  
Index Patient ◽  
Generation Family

Multiple neuroendocrine neoplasia type 1 (MEN1) is a rare genetic disorder with an autosomal dominant inheritance, predisposing carriers to benign and malignant tumors. The phenotype of MEN1 syndrome varies between patients in terms of tumor localization, age of onset, and clinical aggressiveness, even between affected members within the same family. We describe a heterogenic phenotype of the MEN1 variant c.781C>T (LRG_509t1), which was previously reported only once in a family with isolated hyperparathyroidism. A heterozygous missense variant in exon 4 of the gene was identified in the sequence of the MEN1 gene, i.e., c.781C>T, leading to the amino acid change p.Leu261Phe in a three-generation family. In the screened family, 5/6 affected members had already developed hyperparathyroidism. In the index patient and two other family members, an aggressive course of pancreatic neuroendocrine tumor (insulinoma and non-functioning neuroendocrine tumors) with dissemination was diagnosed. In the index patient, late diagnosis and slow progression of the disseminated neuroendocrine tumor have been observed (24 years of follow-up). The very rare variant of MEN1, LRG_509t1 c.781C>T /p.Leu261Phe (LRG_509p1), diagnosed within a three-generation family has a heterogenic clinical presentation. Further follow-up of the family members should be carried out to confirm the spectrum and exact time of clinical presentation.

scholarly journals The importance of early and late ventricular arrhythmias detection and prediction in acute myocardial infarction

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Krljanac ◽  
D Trifunovic ◽  
M Asanin ◽  
L Savic ◽  
J Vratonjic ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Acute Myocardial Infarction ◽  
Poor Prognosis ◽  
Longitudinal Strain ◽  
Coronary Intervention ◽  
Funding Source ◽  
Clinical Center ◽  
Percutaneous Coronary ◽  
One Year

Abstract Background Malignant arrhythmias, ventricular tachycardia or ventricular fibrillation (VT/VF) in acute myocardial infarction (AIM) carry ominous prognosis including sudden cardiac death (SCD). It is not clear whether the timing of VT/VF occurrence always affects the poor prognosis of patients with AMI. Aim To investigate the prognosis of patients who undergoing primary percutaneous coronary intervention (PCI) in accordance with timing of VT/VF and to find the power predictors of their occurrence. Methods 307 consecutive patients in PREDICT-VT study (NCT03263949), 57.9±10.6 year old, 72.3% males were analysed. Of these patients, 27.7% had VT/VF from the symptoms onset, within 48 hours of AIM (early VT/VF group). 8.1% of patients had VT/VF after 48h, during one year follow up (late VT/VF group). Results The frequency of VT/VF occurrence was high between symptoms onset and the end of 2nd month and during 5th and 6th month of AIM. The parameters of conventional echocardiography were significantly impaired in late VT/VF group, as well as parameters of longitudinal strain (LS) (table). Moreover, the MACE (cardiovascular mortality, SCD, new infarction, emergency revascularisation, and hospitalized heart failure) was the highest in late VT/VF group (p=0.000). The most significant predictor of late VT/VF was systolic LS (cut off −12.72%, ROC 0.680, Sen 71%, Sp 64%, p=0.006). Conclusions Although late VT/VF occurrence after primary PCI were less frequent than early VT/VF occurrence, patients with late VT/VF had a very poor prognosis. The most power predictor of late VT/VF were systolic longitudinal strain. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Clinical Center of Serbia

scholarly journals Blunt Traumatic Aortic Injury: 10-Year Single-Center Experience

Aorta ◽  
2021 ◽  
Author(s):  
Ahmet Can Topcu ◽  
Kamile Ozeren-Topcu ◽  
Ahmet Bolukcu ◽  
Sinan Sahin ◽  
Avni U. Seyhan ◽  
...  
Keyword(s):  
Thoracic Aorta ◽  
Subclavian Artery ◽  
Open Repair ◽  
Left Subclavian Artery ◽  
Aortic Injury ◽  
Blunt Thoracic Aortic Injury ◽  
Phone Calls ◽  
Primary Treatment Modality ◽  
Thoracic Aortic Injury

Abstract Objective In blunt trauma patients, injury of the thoracic aorta is the second most common cause of death after head injury. In recent years, thoracic endovascular aortic repair (TEVAR) has largely replaced open repair as the primary treatment modality, and delayed repair of stable aortic injuries has been shown to improve mortality. In light of these major advancements, we present a 10-year institutional experience from a tertiary cardiovascular surgery center. Methods Records of patients who underwent endovascular or open repair of the ascending, arch or descending thoracic aorta between January 2009 and December 2018 were retrospectively analyzed. Patients without blunt traumatic etiology were excluded. Perioperative data were retrospectively collected from patient charts. Long-term follow-up was performed via data from follow-up visits and phone calls. Results A total of 1,667 patients underwent 1,740 thoracic aortic procedures (172 TEVAR and 1,568 open repair). There were 13 patients (12 males) with a diagnosis of blunt thoracic aortic injury. Mean patient age was 43.6 years (range, 16–80 years). Ten (77%) patients underwent TEVAR, two (15.4%) underwent open repair, and one (7.7%) was treated nonoperatively. Procedure-related stroke was observed in one (7.7%) case. Procedure-related paraplegia did not occur in any patients. Left subclavian artery origin was covered in seven patients. None developed arm ischemia. Hospital survivors were followed-up for an average of 60.2 months (range, 4–115 months) without any late mortality, endoleak, stent migration, arm ischemia, or reintervention. Conclusion Blunt thoracic aortic injury is a rare but highly fatal condition. TEVAR offers good early and midterm results. Left subclavian artery coverage can be performed without major complications.

scholarly journals Correctable biliary atresia and cholangiocarcinoma: a case report of a 63-year-old patient

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Masaki Nio ◽  
Motoshi Wada ◽  
Hideyuki Sasaki ◽  
Hiromu Tanaka ◽  
Masatoshi Hashimoto ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Liver Tumors ◽  
Malignant Tumors ◽  
Porta Hepatis ◽  
Case Presentation ◽  
Cancer Occurrence ◽  
Long Term Survivors ◽  
Near Future

Abstract Background Although cancer occurrence following surgery for biliary atresia has gradually increased, the development of cholangiocarcinoma in a native liver survivor of biliary atresia is extremely rare. Case presentation A 3-month-old female patient with the correctable type of biliary atresia underwent a cystoduodenostomy. At 16 years of age, she underwent multiple surgeries including lysis of intestinal adhesions, ileostomy, and gastrojejunostomy at another hospital. At 54 years of age, she underwent lithotomy at the porta hepatis, resection of the residual cystic bile duct with gallbladder, and hepaticojejunostomy in Roux-en-Y fashion. As she approached the age of 63, her computed tomography scan showed no liver tumors. In the following year, she developed cholangiocarcinoma at the porta hepatis and underwent chemotherapy. However, the cancer progressed, and she died before she reached the age of 64 years. Conclusions Cholangiocarcinoma is extremely rare in patients with biliary atresia. However, physicians should follow up patients with biliary atresia as closely as possible, as malignant tumors secondary to biliary atresia may increase in number in the near future because of the growing number of long-term survivors with biliary atresia.

scholarly journals Appendiceal cancer leading to intussusception detected incidentally during follow-up for Peutz–Jeghers syndrome

2020 ◽  
Vol 13 (6) ◽  
pp. 1136-1143
Author(s):  
Kazuhiro Kurihara ◽  
Takanori Suganuma
Keyword(s):  
Malignant Tumors ◽  
Endoscopic Examination ◽  
Autosomal Dominant Disorder ◽  
Tomography Image ◽  
Elevated Lesion ◽  
Poorly Differentiated ◽  
Lower Endoscopy ◽  
Peutz Jeghers Syndrome ◽  
Computed Tomography Image

AbstractPeutz–Jeghers syndrome is an autosomal dominant disorder characterized by hamartomatous polyposis, pigmentation, and malignant tumors. We report a case of ileocecal carcinoma that was incidentally detected during follow-up for Peutz–Jeghers syndrome. A 39-year-old man with solitary Peutz–Jeghers syndrome had undergone three abdominal surgeries. He had been followed up via upper and lower gastrointestinal endoscopy and small intestinal endoscopy. In the endoscopic examination of the lower gastrointestinal tract, a 35 mm large, bumpy, elevated lesion was observed in the cecum. This lesion was not observed 9 months earlier during lower endoscopy. Biopsy of the specimen confirmed tubulovillous adenoma and carcinoma. This lesion was judged to be an indication for operation, and we performed ileocecectomy + D3 lymph node dissection. From the excised specimen, poorly differentiated carcinoma and adenoma components in contact with Peutz–Jeghers-type polyps in the appendix were recognized. A review of the computed tomography image obtained 2 years ago confirmed appendiceal swelling. We suspect that the ileocecal carcinoma in the appendix may have rapidly developed within the 9 months, and was incidentally detected on lower endoscopic examination during follow-up. For the prevention of appendicular tumorigenesis, prophylactic appendectomy may be considered in certain cases during follow-up for Peutz–Jeghers syndrome.

Posterior Lumbar Interbody Fusion Using an En-Bloc Resected Lamina as an Interbody Spacer in Combination with Spinal Instruments

1998 ◽  
Vol 02 (04) ◽  
pp. 325-332
Author(s):  
Shigeru Hirabayashi ◽  
Kiyoshi Kumano ◽  
Takeshi Uchida
Keyword(s):  
Interbody Fusion ◽  
Spinous Process ◽  
Degenerative Spondylolisthesis ◽  
Spinal Instrumentation ◽  
Posterior Lumbar Interbody Fusion ◽  
Bony Union ◽  
Lumbar Interbody Fusion ◽  
En Bloc ◽  
Time Of Operation

We developed a new method of posterior lumbar interbody fusion (PLIF) using an en-bloc resected lamina with or without a hydroxyapatite block as an interbody spacer instead of auto-iliac bone, in combination with rigid-type spinal instrumentation. The purpose of this study was to evaluate the effectiveness of our method. There were 23 patients (13 males, 10 females, age at the time of operation: 21–71 years, mean 50.5 years; follow-up: 1–4 years, mean 2 years and 3 months). In 10 patients with spondylolitic spondylolisthesis and 3 patients with spondylolysis, the floating lamina was resected enbloc by mid-line splitting. In 7 patients with degenerative spondylolisthesis and 3 patients with unstable spine, a cleavage was made at the isthmus and then the complex of lamina and inferior spinous process was resected en-bloc. Seventeen patients with olisthesis underwent reduction. PLIF was performed at the L4/L5 level in 10 patients and the L5/S1 level in 13 patients. Sixteen patients with preoperative low back pain recovered, except for one patient with instability at the adjacent vertebra. All of the seven patients with preoperative gait disturbance recovered. The ratio of olisthesis changed from preoperative 30% to postoperative 18% on average. Good bony union was obtained in both the patients with and those without a hydroxyapatite spacer. Posterior lumbar interbody fusion using an en-bloc resected lamina as an interbody spacer in combination with rigid-type spinal instrumentation was useful.

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