scholarly journals Primary Hepatic Epithelioid Hemangioendothelioma: A Case Report and Literature Review

Author(s):  
Jing Rui Wang ◽  
Bei Lu ◽  
Yang Cai

Abstract Background: Epithelioid hemangioendothelioma(EHE) is a low-grade malignant tumor of vascular origin. The rarity of HEHE make the diagnosis and treatment of this entity very challenging. Case presentation: We report a case of a 69-year-old female patient suffering from HEHE who complained of "Abdominal distension pain with dizziness, appetite loss more than half a month". Enhanced computed tomography (CT) of the upper abdomen indicated multiple space-occupying lesions in the liver. The pathological results of color ultrasound puncture suggested HEHE. We performe transcatheter arterial chemoembolization (TACE) and relevant examinations according to the patient's condition and the patient choice. Follow-up examinations 18 months after discharge did not demonstrate tumor recurrence.Conclusions:HEHE is a relatively rare hepatic malignant tumor derived from vascular endothelial cells, with low incidence, atypical clinical manifestations, difficult diagnosis, and the diagnosis can only be confirmed with pathological results. Currently, appropriate treatment methods should be selected according to the specific conditions of patients.

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Saubhagya Kumar Jena ◽  
Pritinanda Mishra ◽  
Vandana Mohapatra ◽  
Sweta Singh

Serous psammocarcinoma is a rare variant of serous carcinoma arising from either ovary or peritoneum, characterized by massive psammoma body formation, low grade cytologic features, and invasiveness. Its clinical behavior is similar to serous borderline tumors with relatively favorable prognosis. We report herein a case of a 60-year-old postmenopausal woman who presented with abdominal distension. Contrast enhanced computed tomography (CECT) revealed calcified pelvic masses with ascites. Elevated serum CA-125 (970 U/mL) suggested malignant ovarian neoplasm. Patient underwent exploratory laparotomy with primary debulking surgery. Histopathology showed bilateral serous psammocarcinoma of ovary with invasive implants on omentum. Adjuvant chemotherapy was advised in view of advanced stage disease, although its benefits are poorly defined due to rarity of the tumor. However, patient opted out of it and is now on follow-up.


2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
M Kawka ◽  
S Mak ◽  
S Qiu ◽  
T M H Gall ◽  
L R Jiao

Abstract Hepatic epithelioid hemangio-endothelioma (EHE) is a rare, often misdiagnosed malignancy of vascular origin. We describe a case of a 36-year-old, who presented with a burning sensation in his chest on exercise and was believed to have bilobar intrahepatic cholangiocarcinoma. After receiving chemotherapy with partial response, the patient underwent staged resection modified ALPPS (associating liver partition with portal vein ligation for staged hepatectomy) procedure – laparoscopic radiofrequency assisted ALPPS (RALPPS). Histological examination of the stage 1 specimen revealed EHE deposits in the left lobe of the liver. The patient proceeded to have stage 2 open right hepatectomy with concurring histology. 90-days follow-up CT scan showed almost complete resolution of EHE lesions. Low incidence, non-specific clinical and radiological characteristics all contribute to high EHE misdiagnosis rate. Histological analysis can be used for confirmation of the diagnosis; however, specialist staining is required, which is not routinely performed. Multiple treatments are available against EHE, but only liver resection and liver transplantation are potentially curative. ALPPS and its modifications (such as RALPPS) offer an alternative for conventional two-stage hepatectomy for patients with extensive bilobar EHE, who are at risk of developing post-hepatectomy liver failure or patients with anomalous hepatic vasculature anatomy, which makes PVE challenging.


2016 ◽  
pp. 86-93
Author(s):  
M.Yu. Yegorov ◽  
◽  
A.A. Sukhanova ◽  

The objective: study the features of gynecological, physical history, diagnosis and treatment of patients with benign epithelial ovarian tumors (BeEOT) and borderline epithelial ovarian tumors (BEOT), determining the frequency of recurrence of ovarian tumors in the postoperative period. Patients and methods. According to a retrospective analysis of case histories of 112 women with epithelial ovarian tumors (EOT) underwent conservative or radical surgical treatment in a hospital, two groups were formed: I group – patients with benign epithelial ovarian tumors (BeEOT), which amounted to 85 (75.9%) women, and group II – patients with borderline epithelial ovarian tumors (BEOT), which amounted to 27 (24.1%) women. It was found that the main complaints of patients with EOT were pain (49.1%), abdominal distension (17%), and abnormal uterine bleeding (12.5%). The highest incidence of BeEOT (31.8%) observed in the age group of 41–50 years, while the peak incidence of BEOT (44.4%) corresponds to the age group of 51–60 years. Results. In BEOT endocrine pathology occurs significantly more frequently (p<0.05) than in BeEOT – 25.9% vs. 9.4%, respectively. Pathology of pancreatic-hepatobiliary system occurs significantly more frequently (p<0.05) in patients with BEOT compared with BeEOT – 81.5% versus 57.6%, respectively. Venous disorders (varicose veins of the pelvic organs, lower limbs, haemorrhoids) observed in BEOT significantly more frequently (p<0.05) than in BeEOT – 18.5% vs. 5.9%, respectively. EOT most often diagnosed in the period from 1 to 6 months after the first clinical manifestations with an average uptake of medical care 4.6±0.57 months. In assessing of peritoneal exudate cytogram the mesothelium cells are significantly more common for BeEOT (p<0.01) than BEOT – 79.4% versus 40.9%, respectively. Cervicitis is more likely significantly to occur in BeEOT (p<0.01) than in BEOT – 29.4% vs. 7.4%, respectively. The most common histological type among the benign tumors of the ovaries are endometriomas, which occurred in 48.2% of all BeEOT cases, and among the borderline tumors – serous tumors, which accounted for 59.3% of all BEOTs. Conclusion. The use of organ sparing surgery in EOT increases the risk of recurrence, especially in the case of endometrial histology or borderline variant of tumor. Key words: benign and borderline epithelial ovarian tumors, clinical-anamnestic analysis, diagnosis, treatment.


Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110108
Author(s):  
Ashley D Hickman ◽  
Evandro D Bezerra ◽  
Anja C Roden ◽  
Matthew T Houdek ◽  
Jonathan D Barlow ◽  
...  

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.


2021 ◽  
pp. 344-351
Author(s):  
Hiroki Kanno ◽  
Toshihiro Sato ◽  
Ryuta Midorikawa ◽  
Satoki Kojima ◽  
Shogo Fukutomi ◽  
...  

Hepatic epithelioid hemangioendothelioma (EHE) is a rare malignant tumor with unknown pathogenesis. Herein, we report a case of a hepatic EHE presenting synchronously with a hepatocellular carcinoma (HCC). To the best of our knowledge, this is the second case report of synchronous hepatic EHE and HCC. An 84-year-old man presented with back pain. During examination, a tumor in liver segment 3 was coincidentally detected. Tumor marker (carbohydrate antigen 19-9, alpha-fetoprotein, and protein induced by vitamin K absence or antagonist-II) levels were elevated. Contrast-enhanced computed tomography revealed perinodular enhancement in the arterial and portal phases. Another tumor was detected in liver segment 2, which was homogeneously enhanced in the arterial phase, followed by washout in the portal and late phases. Based on these imaging findings, we diagnosed the tumor in segment 3 as a solitary cholangiocellular carcinoma and the tumor in segment 2 as a solitary HCC. Lateral sectionectomy of the liver was performed. Microscopically, spindle-shaped and epithelioid cells were present in the tumor in segment 3. On immunohistochemistry, the tumor cells were positive for CD31 and CD34, focally positive for D2-40, and negative for AE1/AE3. Therefore, the tumor in segment 3 was ultimately diagnosed as an EHE and the tumor in segment 2 as a well-differentiated HCC. Preoperative diagnosis of EHE is difficult owing to the lack of specific findings. Intratumoral calcification, halo sign, and lollipop sign are occasionally found in EHE and are useful imaging findings for diagnosis. Clinical behavior is unpredictable, ranging from indolent growth to rapid progression. Clinical or pathological predictors of the course of EHE are urgently required.


2021 ◽  
Vol 70 (4) ◽  
pp. 207-213
Author(s):  
Andrea Kaliariková ◽  
Klára Perceová ◽  
Jan Machač ◽  
Michal Jurajda ◽  
Milan Urík

Objectives: Characterisation of clinical manifestations in children who had acute mastoiditis (AM) -related intracranial complications (ICCs) and to determine the incidence of ICCs in the study group. To define children with a higher risk of ICCs and gain new information on aetiological microbial agents of AM. Methods: A retrospective analysis of 137 paediatric patients with AM treated at a tertiary centre using standard dia­gnostic and therapeutic protocol between 2002 and 2019. Results: Altogether 137 patients with AM were hospitalised at our centre between 2002 and 2019. During this time, the occurrence of ICC in children with AM was low (n = 3, incidence 2.19%). Due to the low number of patients with ICC with AM in our patient group, we were unable to define a specific group of patients with a higher risk of ICC development in AM. Despite this fact, the average value and median of CRP were considerably higher in patients with AM-associated ICCs. The most frequent aetiological agent was Streptococcus pneumoniae. Conclusions: The low incidence of ICC in patients with AM in our patient group might be caused by routinely performed paracentesis in developed acute otitis, rational antibio­tic therapy and the good availability of an ENT specialist in the Czech Republic. The most common aetiological agent of AM was Streptococcus pneumoniae. Keywords: Streptococcus pneumoniae – acute mastoiditis – intracranial complications – aetiological agent – antibio­tic therapy – antromastoidectomy


2021 ◽  
Author(s):  
David Andrew Cummins ◽  
Carl Kuschel

Abstract Background: Bilious vomiting in the neonate is an important presenting sign of intestinal obstruction. We conducted a review of the presentation and management of term neonates admitted with bilious vomiting (BV) to determine the incidence of a surgical pathology in our population.  Design: Retrospective cohort study using a prospectively maintained database.  Participants: All term infants admitted to NICU with BV at the Royal Women’s Hospital Melbourne during a 5-calendar year period.  Results: All 153 babies had at least one imaging study. 128 (83.7%) had plain abdominal radiographs. 127 (83%) underwent upper gastrointestinal contrast scan (UGI) and 103 (67.3%) had both. 6 (3.9%) UGI studies were abnormal, with 3 babies (1.9%) subsequently having surgical pathology (2 volvulus, 1 Hirschsprung disease). Only 6 (3.9%) babies in our cohort had a surgical pathology identified (4 Hirschsprung disease, 2 malrotation). Babies with surgical pathology were more likely to present later (median 40 hours versus 23 hours). Abdominal distension was highly sensitive for surgical pathology.  Conclusion: The incidence of surgical pathology in this cohort was low compared to other studies. It is more likely in infants presenting with BV after 24 hours. 


2014 ◽  
Vol 15 (2) ◽  
pp. 131-134
Author(s):  
Oliur Rahman ◽  
Anwarul Kabir ◽  
Prodip Kumar Biswas ◽  
AMM Shoriful Islam ◽  
AB Siddik ◽  
...  

Background: Tuberculosis is common in our country. It commonly affects the lungs but in up to one third of the cases involve other organs. Abdominal tuberculosis is one of the important extrapulmonary tuberculosis.It has varied presentation, frequently mimicking other common and rare diseases. Materials and Methods:It is a prospective observational study done to see the clinical presentation and treatment outcome of abdominal tuberculosis in medicine, surgery and gastroenterology department Sylhet M A G Osmani Medical College Hospital during Jan 2008-Dec2008. Sample was collected purposively. All patients were undergone detailed history, clinical examination, relevant investigation and follow up till the end of six months anti tubercular chemotherapy.Data recorded in predesigned record form. Statistical analysis was done with SPSS software. All data presented as percentage or mean. Result: From the 50 cases of abdominal TB, 26 (52%) were male and 24 (48%) were female. Male and female ratio was 1.04:1. Here most of the patients were in the age of 21-30 years and mean age was 29.3 years,majority of them were from poor socioeconomic status(18%) had associated pulmonary tuberculosis.The common presentation exhibited by the patients were weight loss (100%), low grade fever (88%), abdominal pain (86%), bowel disturbance (84%), anorexia (80%), nausea and vomiting (70%), abdominal distension (46%), , ascites (42%).Most of the patients were anaemic with raised ESR.Ultrasonography revealed ascites,abdominal lymphadenopathy and thick walled bowel. Colonoscopy found nodularity and ulceration with narrowing. Ascitic fluid was exudative with predominant lymphocyte count. Laparoscopy and laparotomy revealed tubercle on the intestine and the mesentery with thickening and bowel stricture. All the biopsy reports were consistent with tuberculosis. Site of involvement in decreasing frequency were ileocaecal (44%), peritoneal (16%), abdominal lymph node (8%), ascending colon (8%), small bowel (6%),transverse colon (4%), sigmoid colon (2%), rectum (2%), gastric (2%) and duodenal (2%) but multiple site in 6%.The treatment was given as per the WHO guidelines with category I drugs and found- 86% cured with anti TB alone, 4% needed surgery for intestinal obstruction along with anti TB, and 10% drop out from follow up.Conclusion:The clinical presentations of abdominal TB appear not specific for the condition. Thus, careful approach and supportive results are required in order to issue the final diagnosis. If diagnosed early, it can be treated successfully with the conventional anti-TB drugs. DOI: http://dx.doi.org/10.3329/jom.v15i2.20686 J MEDICINE 2014; 15 : 131-134


2012 ◽  
Vol 43 (2) ◽  
pp. 205-207 ◽  
Author(s):  
Y. Hata ◽  
R. Yuasa ◽  
F. Sato ◽  
H. Otsuka ◽  
H. Goto ◽  
...  

2021 ◽  
Vol 11 ◽  
Author(s):  
Zhongben Tang ◽  
Feng Lin ◽  
Jiarong Xiao ◽  
Xiaojun Du ◽  
Jian Zhang ◽  
...  

Primary pulmonary adenoid cystic carcinomas are salivary tumors that are low-grade malignant and prone to recurrence and metastasis. Surgery is currently the main treatment, but there is no standard with regard to postoperative adjuvant therapy. Adenoid cystic carcinoma is more sensitive to radiotherapy and patients benefit less from chemotherapy, but few studies have focused on targeted therapy, and their conclusions are inconsistent. With respect to primary pulmonary adenoid cystic carcinoma, large-scale studies cannot be conducted due to its low incidence, and studies on the targeted therapy of it are very scarce. A few case reports indicate that targeted therapy can be effective however, suggesting that it may be a good option. The current report is the first on the occurrence of human epidermal growth factor receptor 2 amplification in pulmonary adenoid cystic carcinoma. The patient was treated with pyrotinib for 6 months and achieved stable disease.


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