scholarly journals Retiform hemangioendothelioma: a rare lesion of the vulva

2021 ◽  
Vol 49 (8) ◽  
pp. 030006052110277
Author(s):  
Mingyue Zhang ◽  
Xiaojiao Yin ◽  
Weikai Yao ◽  
Ning Liu ◽  
Ying Yue

Retiform hemangioendothelioma (RH) is a rare borderline-malignant vascular tumor with specific histological characteristics, usually occurring in the limbs and trunk. We report the case of a 63-year-old woman who presented with a painless, oval nodule that had been growing slowly on her left vulva for 3 years. Magnetic resonance imaging of the pelvic cavity revealed a 4.4- × 2.7- × 1.8-cm cystic lesion in the subcutaneous fat of the left vulva. Resection beyond the macroscopic border was performed. Pathology revealed vascular structures with elongated and narrow arborizing vascular channels that were arranged in a retiform pattern resembling rete testis tissue. Immunohistochemical endothelial staining was positive for CD31, CD34, and Friend leukemia integration-1 (FLI-1). The above features confirmed a diagnosis of RH. There was no local recurrence or metastasis during the 26-month follow-up. RH of the vulva is rare, and its diagnosis is supported by specific histological characteristics and immunohistochemical staining for CD31, CD34, and FLI-1. Wide surgical resection with tumor-free margins is important for a favorable prognosis.

2017 ◽  
Vol 20 (2) ◽  
pp. 163-167 ◽  
Author(s):  
Farah El-Sharkawy ◽  
Patricia Isabel Delgado ◽  
Antonello Podda ◽  
Holly Leigh Neville ◽  
Claudia Patricia Rojas

Angiosarcomas are highly aggressive malignancies of vascular origin and are very rarely found in children. We report a case of a 13-year-old girl with a history of abdominal pain and increased abdominal girth. Radiologic imaging showed significant ascites and large pelvic masses involving bilateral adnexa with abdominal spread. Microscopic examination of a biopsy revealed pleomorphic epithelioid and spindle cells with brisk mitotic activity, intracytoplasmic vacuoles, vascular channels, and large areas of hemorrhage and necrosis. Immunohistochemistry analysis showed strong and diffuse positivity for CD31, D2-40, ERG, FLI-1, and focally for CD34, vWF, and EMA. The diagnosis of metastatic angiosarcoma was rendered. The patient was treated aggressively with systemic chemotherapy, immunotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy, with a favorable response after 1-year follow-up. Angiosarcoma should be considered when encountering a vascular tumor with pleomorphism, brisk mitotic activity, and necrosis. Immunohistochemistry studies are necessary for proper diagnosis.


2005 ◽  
Vol 62 (10) ◽  
pp. 763-767
Author(s):  
Darko Mirkovic ◽  
Aleksandar Karamarkovic

Background. Solid-pseudopapillary tumors of the pancreas (SPT) is a very rare lesion. We presented the clinical and pathological characteristics of this tumor and reviewed the literature. Case report. We analyzed the treatment of the two female patients, aged 21 and 37 years, with this tumor. The first female patient had the tumor without involvement of the surrounding organs. The second female patient had the tumor with a high grade of malignancy which invaded blood vessels. We performed in both patients splenohemipancreatectomy, and then we reported the pathological characteristics of the tumors. We didn't find recidive in the observed postoperative follow-up of 4, and 6 years, respectively. Conclusion. SPT is a rare tumor of the pancreas that is diagnosed primarily in young women. The prognosis after surgical treatment was excellent. The histopathological analysis is the main procedure in establishing diagnosis, regardless the most recent diagnostic procedures.


2010 ◽  
Vol 12 (5) ◽  
pp. 490-496 ◽  
Author(s):  
Liu Xue-Song ◽  
You Chao ◽  
Yang Kai-Yong ◽  
Huang Si-Qing ◽  
Zhang Heng

Object An extensive sacrococcygeal chordoma is considered a challenge for neurosurgeons. Because of the complex anatomy of the sacral region, the risk of uncontrollable intraoperative hemorrhage, and the typically large tumor size at presentation, complete resections are technically difficult and the tumor recurrence rate is high. The aim of this study was to assess the value of using occlusion of the abdominal aorta by means of a balloon dilation catheter and electrophysiological monitoring when an extensive sacrococcygeal chordoma is removed. Methods Between 2004 and 2008, 9 patients underwent resection of extensive sacrococcygeal chordomas in the authors' department with the aid of occlusion of the abdominal aorta and electrophysiological monitoring. All of these operations were performed via the posterior approach. The records of the 9 patients were reviewed retrospectively. Results Wide resections were performed in 6 cases and marginal excisions in the other 3. Five patients underwent postoperative radiotherapy. Intraoperative hemorrhage was controlled at 100–400 ml. Postoperatively, none of the patients had any new neurological dysfunction, and 2 patients regained normal urinary and bowel function. The mean follow-up period was 31.4 months (range 10–57 months). No patient developed local recurrence or had metastatic spread of tumor during follow-up. Conclusions Occlusion of the abdominal aorta and electrophysiological monitoring are useful methods for assisting in resection of sacrococcygeal chordoma. They can reduce intraoperative hemorrhage and entail little chance of tumor cell contamination. They can also help surgeons to protect the organs in the pelvic cavity and neurological function. Use of these methods could give patients better quality of life.


2020 ◽  
Vol 2020 ◽  
pp. 1-14
Author(s):  
Qingbo Su ◽  
Xiquan Zhang ◽  
Hui Zhang ◽  
Yan Liu ◽  
Zhaoru Dong ◽  
...  

Purpose. This study aimed to retrospectively review the diagnosis and surgical treatment of uterine intravenous leiomyomatosis (IVL). Methods. The clinical data of 14 patients with uterine IVL admitted to our hospital between 2013 and 2018 were retrospectively analyzed, including their demographics, imaging results, surgical procedures, perioperative complications, and follow-up results. Results. The tumors were confined to the pelvic cavity in 7 patients, 1 into the inferior vena cava, 4 into the right atrium, and 2 into the pulmonary artery (including 1 into the superior vena cava). Only one case was misdiagnosed as right atrial myxoma before the operation, which was found during the surgery and was treated by staging surgery; all the other patients underwent one-stage surgical resection. Three patients underwent complete resection of the right atrial tumor through the abdominal incision, and one patient died of heart failure in the process of resection of heart tumor without abdominal surgery. During the 6–60 months of follow-up, 4 patients developed deep venous thrombosis of the lower extremity, and 1 patient developed ovarian vein thrombosis and pulmonary embolism. After anticoagulation treatment, the symptoms disappeared. One patient refused hysterectomy and the uterine fibroids recurred 4 years after the operation. Conclusion. Specific surgical plans for uterine IVL can be formulated according to cardiac ultrasound and computed tomography (CT). For the first type of tumor involving the right atrium, the right atrium tumor can be completely removed through the abdominal incision alone to avoid thoracotomy. The disease is at high risk of thrombosis and perioperative routine anticoagulation is required.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Nasir Ud Din ◽  
Shabina Rahim ◽  
Tamana Asghari ◽  
Jamshid Abdul-Ghafar ◽  
Zubair Ahmad

Abstract Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.


2000 ◽  
Vol 58 (4) ◽  
pp. 1002-1008 ◽  
Author(s):  
CARMEN LISA JORGE ◽  
SUELY K. NAGAHASHI-MARIE ◽  
CHRISTIANE C. PEDREIRA ◽  
SÉRGIO ROSEMBERG ◽  
ROSA M.F. VALÉRIO ◽  
...  

This is a retrospective study of 21 surgically treated patients with temporal lobe tumors and epilepsy. Evaluation included clinical data, EEG findings, structural scans, pathological diagnosis and post-surgical follow-up. There were 9 cases of ganglioglioma, 5 pilocytic astrocytoma, 3 ganglioneuroma, 2 dysembryoplastic neuroepithelial tumor, 1 pleomorphic xantoastrocytoma, and 1 meningioangiomatosis. Mean follow-up time was 22 months and outcome was evaluated according to Engel's classification; 76.2% were classified in class I and 23.8% in II and III. All patients classes II and III had been submitted to mesial and neocortical resections. There were no differences related to clinical characteristics, pathological diagnosis or duration of follow-up in patients seizure-free or not. All patients had abnormal MRI and ten of these had normal CT; the MRI characteristics were compared to pathological diagnosis and specific histological characteristics of the tumors were not discernible by MRI. We concluded that MRI was essential for the diagnosis and precise location of TL tumors. Ganglioglioma was the most frequent tumor and lesionectomy associated to mesial resection doesn't guarantee a better prognosis.


2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
Author(s):  
Suruchi Mishra ◽  
Tamara B. Harris ◽  
Trisha Hue ◽  
Iva Miljkovic ◽  
Suzanne Satterfield ◽  
...  

Background. Abdominal adiposity and serum leptin increase with age as does risk of metabolic syndrome. This study investigates the prospective association between leptin and metabolic syndrome risk in relation to adiposity and cytokines.Methods. The Health, Aging, and Body Composition study is a prospective cohort of older adults aged 70 to 79 years. Baseline measurements included leptin, cytokines, BMI, total percent fat, and visceral and subcutaneous fat. Multivariate logistic regression was used to determine the association between leptin and metabolic syndrome (defined per NCEP ATP III) incidence after 6 years of follow-up among 1,120 men and women.Results. Leptin predicted metabolic syndrome in men (Pfor trend = 0.0002) and women (Pfor trend = 0.0001). In women, risk of metabolic syndrome increased with higher levels of leptin (compared with quintile 1, quintile 2 RR = 3.29, CI = 1.36, 7.95; quintile 3 RR = 3.25, CI = 1.33, 7.93; quintile 4 RR = 5.21, CI = 2.16, 12.56; and quintile 5 RR = 7.97, CI = 3.30, 19.24) after adjusting for potential confounders. Leptin remained independently associated with metabolic syndrome risk after additional adjustment for adiposity, cytokines, and CRP. Among men, this association was no longer significant after controlling for adiposity.Conclusion. Among older women, elevated concentrations of leptin may increase the risk of metabolic syndrome independent of adiposity and cytokines.


2001 ◽  
Vol 95 (1) ◽  
pp. 93-95 ◽  
Author(s):  
Federico Roncaroli ◽  
Bernd W. Scheithauer ◽  
Savvas Papazoglou

✓ A case of polymorphous hemangioendothelioma of the spinal cord is described. This 55-year-old woman presented with an 18-month history of lower-extremity sensorimotor deficit. A magnetic resonance image revealed an enhancing, intradural, extramedullary nodule at the T1–2 level. On gross inspection, the lesion measured 3.5 cm and was firmly attached to spinal cord parenchyma and adjacent nerve roots. It was completely removed. Fourteen months after surgery the patient's neurological deficit had resolved. Polymorphous hemangioendothelioma is a rare vascular tumor of borderline malignancy. Most occur in lymph nodes. None has been reported to occur in the central and peripheral nervous system. Based on current experience, resection and close follow up seems the best therapeutic approach.


2013 ◽  
Vol 35 (6) ◽  
pp. E7 ◽  
Author(s):  
Anil Nanda ◽  
Sudheer Ambekar ◽  
Vijayakumar Javalkar ◽  
Mayur Sharma

Object Tuberculum sellae meningiomas (TSMs) and diaphragma sellae meningiomas (DSMs) are challenging lesions to treat due to their proximity to neurovascular structures. Methods The authors reviewed the medical records of patients who underwent surgical excision of TSMs and DSMs from 1990 to 2013. They also describe the technical strategies used to minimize injury to the optic apparatus, vascular structures, and pituitary stalk. Results Twenty-four patients with TSM and 6 patients with DSM were included in the study. Seventy percent of the tumors were large (≥ 5 cm). The pterional approach was employed in most cases. Optic canal involvement was observed in 4 patients. Twenty-one patients (70%) had visual dysfunction before surgery. At follow-up (median 18 months), visual improvement was noted in 10 (47.6%) of 21 patients. Gross-total excision was achieved in 22 patients (91.6%) with TSM and 5 (83.3%) with DSM. At last follow-up, 28 patients (93.3%) had a Glasgow Outcome Scale score of 5. There were no deaths in this series. Conclusions Tuberculum and diaphragma sellae meningiomas present a unique subset of tumors due to their location. They can be safely excised with minimal morbidity and mortality using microsurgical techniques. Attention to technical details during surgery leads to greater respectability and superior visual outcome.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 15071-15071
Author(s):  
G. El Maalouf ◽  
C. Le Tourneau ◽  
V. Paradis ◽  
F. Degos ◽  
O. Farges ◽  
...  

15071 Background: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare subtype of primary HCC which often arises in young patients without underlying hepatic disease or viral infection. Clinical and pathological factors of prognosis are warranted. Methods: Descriptive, univariate and multivariate analysis of survival parameters were analyzed in a monocenter cohort of FL-HCC patients completely resected. Main variables were WHO-PS, age, AJCC stage, AFP level and detailed pathological features including EGFR, p- AKT, and PTEN expression. Results: 23 consecutive patients (median age: 30, M/F:6/17) with pathologically reviewed FL-HCC were analyzed. Median tumor size was 11 cm (range 4–23) and 6 patients had AFP>200ng/ml. two patients had respectable metastasis (lung or peritoneum), 5 patients had vascular invasion and 3 patients had lymph node metastasis achieving the following AJCC staging: I (70%), III (22%) and IV (8%). With median follow-up of 36 months, 3-year survival was 76% . Median time of recurrence was 23 months. Sites of first recurrence were hepatic in 5 patients, extra-hepatic in 4 patients, and both in 3 of 12 patients who recurred (52%). Metastasis occurred in lung, peritoneum, bone, skin and lymph nodes. Expression of EGFR, p-AKT, and PTEN was found in 94%, 69% and 0% of patients, respectively. None of the clinical parameters including AJCC staging predicted survival. In multivariate analysis, bad prognostic factors of recurrence were satellite micro-nodules (p=0.04) and micro-vascular tumor invasion (p=0.03) on pathological specimens. There was a trend toward a better survival in patients with <30% EGFR expression (p=0.06). Conclusions: FL-HCC patients with primary complete resection may develop extra-hepatic metastasis (58%) or isolated intra-hepatic recurrence (42%) within a median follow-up of 2 years. Prognostic factors of survival are pathological satellite nodules and micro-vascular tumor invasion. No significant financial relationships to disclose.


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