scholarly journals A Multidisciplinary Approach to the Management of Atypical Osseous Epithelioid Hemangioendothelioma

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
J. K. Ma ◽  
J. Barr ◽  
S. Vijayakumar
Keyword(s):  
Multidisciplinary Approach ◽  
Pathological Fracture ◽  
Soft Tissues ◽  
Intensity Modulated Radiation Therapy ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Histopathological Study ◽  
Incisional Biopsy ◽  
Multidisciplinary Intervention ◽  
Imaging Characteristics

Hemangioendothelioma is a rare vascular tumor of intermediate malignant potential. Though epithelioid hemangioendothelioma (EH) is commonly found in soft tissues, it has been known to be present in skeletal tissues. The authors present a case of a 50-year-old woman diagnosed with EH of the iliac bone and acetabulum, who experienced pathological fracture at presentation. This report describes a multidisciplinary approach to the management that includes initial incisional biopsy, curettage, and bone grafting, followed by Intensity Modulated Radiation Therapy. The patient finally underwent hemipelvic resection with allograft reconstruction after recurrence. Histopathological study revealed osseous EH of low mitotic activity that stained positively for CD31, CD34, vimentin, and Factor VIII. Herein, the authors discuss the imaging characteristics, histopathological aspects, cytogenetic findings, and the radiobiological behavior of osseous EH. After an aggressive multidisciplinary intervention, the patient is able to achieve local control with no evidence of distal metastatic disease.

scholarly journals Metastatic cutaneous epithelioid hemangioendothelioma involving right atrium - a case report

2016 ◽  
Vol 3 (3) ◽  
pp. 47
Author(s):  
Sandy Mpho Mosenye ◽  
Rahul Daimari ◽  
Neha Sharma ◽  
Princess Lelani Chikanda ◽  
Feng Zhou ◽  
...  
Keyword(s):  
Right Atrium ◽  
Cardiac Involvement ◽  
Soft Tissues ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Malignant Potential ◽  
Vascular Endothelial ◽  
Rare Occurrence ◽  
Scalp Lesion ◽  
The Common

Epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial tumour with unpredictable malignant potential. The common sites involved are the lung, liver and soft tissues; with cardiac involvement being a very rare occurrence. The diagnosis of this rare vascular tumor is made by typical histopathological findings and confirmed by specific immunohistochemistry. We present a case of a patient with a long standing painless scalp lesion who presented with pulmonary symptoms and ultimately diagnosed to have metastatic cutaneous EHE involving the lungs, pericardium and right atrium.

scholarly journals Multicentric epithelioid hemangioendothelioma of humerus and scapula

2021 ◽  
Vol 0 ◽  
pp. 1-5
Author(s):  
S B Dhanya ◽  
Ramakrishna Narayanan ◽  
Anu Kapoor ◽  
Shantveer G. Uppin
Keyword(s):  
Adult Population ◽  
Bone Lesion ◽  
Vascular Tumor ◽  
Epithelioid Hemangioendothelioma ◽  
Adolescent Male ◽  
Lytic Lesion ◽  
Imaging Features ◽  
Post Contrast ◽  
Imaging Characteristics ◽  
The Right

Epithelioid hemangioendothelioma (EH) is a rare malignant vascular tumor occurring mainly in the liver and lungs, with bones being a rare site and primarily seen in the adult population. We present a rare case of multicentric EH in the right humerus in an adolescent male, who presented with complaints of pain and swelling in the right shoulder. Radiographs and Computed tomography showed a large expansile lytic lesion in the proximal end of the right humerus with areas of cortical destruction and matrix calcification. On Magnetic resonance imaging, the lesion was predominantly isointense on T1-weighted image, hyperintense on T2-weighted, and Short Tau Inversion Recovery images with prominent flow voids within. On post-contrast sequences, the lesion showed intense heterogenous enhancement with a non-enhancing central necrotic area. Multiple smaller lytic lesions with similar imaging characteristics were also found in the mid and distal shaft of the right humerus and the right coracoid process. Differentials of Telangiectatic osteosarcoma, Giant cell tumor, brown tumors of hyperparathyroidism, and metastasis were considered. Core biopsy revealed that the lesion was an EH. Though the imaging features of EH are non-specific, it may be considered in the differential diagnosis of an expansile lytic bone lesion with no periosteal reaction, showing cortical break and soft tissue component, especially if it is multifocal and multicentric.

scholarly journals Angiographic Embolization followed by Excision of Large Cellular Angiofibroma of the Vulva

2019 ◽  
pp. 221-225
Author(s):  
Torres SB ◽  
Diez Brito M ◽  
Peremateu MS ◽  
Wernicke A ◽  
Saez Perrotta C ◽  
...  
Keyword(s):  
Surgical Planning ◽  
Multidisciplinary Approach ◽  
Soft Tissues ◽  
Buenos Aires ◽  
Histopathological Diagnosis ◽  
Angiographic Embolization ◽  
Internal Pudendal Artery ◽  
Cellular Angiofibroma ◽  
Pathology Section ◽  
Vulvar Mass

Introduction: Cellular angiofibroma is a rare benign mesenchymal tumor, occurring almost exclusively in the superficial soft tissues of the genital region. Some of these lesions presents as large, hypervascularized masses. There are no reports in the literature to date on the challenges of resection of hypervascularized vulvar masses, or of hypervascularized cellular angiofibroma.Materials and methods: We describe a case of a cellular angiofibroma of the vulva and the surgical approach with tumoral vessel embolization and posterior resection.Case: A 47-year-old women was referred to vulvar pathology section of Italian Hospital of Buenos Aires for evaluation of left vulvar mass. A pelvic angiography was performed 5 days prior to surgery. It showed a hypervascularized formation at the level of the left vulva at the expense mainly of branches of the left internal pudendal artery. A complete resection was performed afterwards at our institution. The histopathological diagnosis was compatible with cellular angiofibroma.Conclusion: We consider that surgical planning is essential and has to be performed with a multidisciplinary approach. Angiographic embolization should be taken into account as a valid resource when planning a surgery with a high risk of blood loss in order to decrease morbidity.

scholarly journals Living-related liver transplant to treat epithelioid hemangioendothelioma – a case report

2005 ◽  
Vol 8 (4) ◽  
pp. 449-451
Author(s):  
Marcelo A. F. Ribeiro Jr ◽  
Christian Evangelista Garcia ◽  
Telma Eugênio dos Santos ◽  
Adavio de Oliveira e Silva ◽  
Regina Leitão ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Case Report ◽  
Liver Transplant ◽  
Soft Tissues ◽  
Liver Tumors ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Living Related Liver Transplant ◽  
Transplantation Technique ◽  
Living Related

Introduction: Epithelioid hemangioendothelioma of liver is a rare, low-grade neoplasm of vascular origin that has an unpredictable malignant potential. It preferentially arises in soft tissues and bones, and seldom in the liver. Biologically, it trends to be multi-focal and often unresectable. Objective: The outcome of a patient submitted to a living-related liver transplant to treat epithelioid hemangioendothelioma of the liver. Case report: The reported case involves a 39-year old female patient with an immense epithelioid hemangioendothelioma of the liver, involving segments III, IV, V, VII, and VIIl. The prognostic to any kind of treatment was considered unfavorable, and the decision of submitting her to a liver transplant was made. It was suggested a living-related liver transplant. The donor was the patient’s 36-year old brother, who donated his right liver lobe (segments V, VI, VII, VIII). The recipient´s original liver was large, presenting very solid consistence and no node evidence. The transplant itself was performed using the conventional living-related liver transplantation technique. The recovery was good, and she was discharged from hospital on the 15th postoperative day using micophenolate mofetil, tacrolimus, and corticoid. Conclusion: Liver transplantation for epithelioid hemangioendothelioma of the liver can be performed with acceptable survival rate. The living related liver transplant opens a great perspective to that group of patients presenting liver tumors and who are unfeasible to wait for an organ on a waiting list.

scholarly journals Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Nasir Ud Din ◽  
Shabina Rahim ◽  
Tamana Asghari ◽  
Jamshid Abdul-Ghafar ◽  
Zubair Ahmad
Keyword(s):  
Surgical Resection ◽  
Case Series ◽  
Liver Function Tests ◽  
Vascular Tumor ◽  
Metastatic Carcinoma ◽  
Epithelioid Hemangioendothelioma ◽  
Clinicopathological Features ◽  
University Hospital ◽  
Detailed Review

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.

scholarly journals Major Trauma and Severe Burn: A Synergistic Mortality. Case Report and Literature Review

2020 ◽  
Vol 61 (01) ◽  
pp. 023-027
Author(s):  
Oriol Pujol ◽  
Jordi Selga ◽  
Jordi Serracanta ◽  
Juan Antonio Porcel ◽  
José Vicente Andrés-Peiró
Keyword(s):  
Synergistic Effect ◽  
Multidisciplinary Approach ◽  
Open Fracture ◽  
Major Trauma ◽  
Soft Tissues ◽  
Total Body Surface Area ◽  
High Energy ◽  
Complex Case ◽  
Severe Burns ◽  
The Right

Abstract Case Presentation A 42-year-old man was transferred to our institution after a high-energy accident (an explosion). He presented second-degree burns on 20% of the total body surface area (TBSA), affecting the lower hemiabdomen and the lower right limb. He also presented a supraintercondylar open fracture of the right femur (Gustilo I). The burns were treated with debridement and coverage with mesh graft, while the fracture required an early transitory transarticular external fixation with delayed definitive osteosynthesis. Our patient presented good local and systemic evolution. We can offer a eleven-month follow-up. Discussion The literature supports that the combination of trauma and burn injuries is a relatively rare pattern, which may explain the lack of knowledge and studies on this subject. This double injury has demonstrated a synergistic effect on mortality. The management of soft tissues in the coexistence of an open fracture and a burn in the same limb is a challenge. The method and timing of the treatment of the fracture directly impacts the treatment of the burn (and vice versa), and most authors tend to treat the fracture first. All of the studies reviewed emphasized the importance of the multidisciplinary approach. Conclusions We have presented a complex case combining major trauma and severe burns. Although there is a lack of studies in the literature on this subject, the papers state that this is an unusual pattern with a synergistic effect on mortality. In our experience, the management of soft tissues and the multidisciplinary approach play a central role, as it is also stated in the literature. The management of these patients is still controversial, and more studies are needed.

scholarly journals Pediatric patient with diagnosis of extra osseous Ewing’s sarcoma of the tongue: case report

2018 ◽  
Vol 66 (4) ◽  
pp. 399-403
Author(s):  
Neira CHAPARRO ◽  
Amaurys DIAZ ◽  
Luis HERRERA ◽  
Rafael PINEDA ◽  
Betty PEROZO ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Soft Tissues ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Malignant Neoplasm ◽  
Histopathological Study ◽  
Histopathological Diagnosis ◽  
Unusual Behavior ◽  
Volume Increase ◽  
Tumoral Lesion

ABSTRACT Ewing’s sarcoma is a malignant tumor that arises mainly from bone tissue, so that its extra-osseous presentation is not very common and even more unusual, in the soft tissues of the oral cavity. The objective of this case was to describe the clinical characteristics of an extra-osseous Ewing’s Sarcoma of the tongue in a pediatric patient. An 11-year-old male school patient, who attended an oral medicine consultation referring a volume increase in the tongue. Intraorally, a tumoral lesion was observed on the left lateral edge of the tongue, indurated, with the same color of the mucosa with ulcerated areas, well defined edges and symptomatic on palpation, with an approximate size of 2.5 cm. A cervical and maxillofacial MRI was indicated, observing a partially delimited hyperintense and non-infiltrating lesion in the described area. An incisional biopsy was performed with histopathological diagnosis of malignant neoplasm of blue round cells, theCD99 marker was found to be positive in the immunohistochemical study and was key to the definitive diagnosis of this tumor. A thoraco-abdomino-pelvic tomography was requested by the Pediatric Oncology service, as well as biopsy and aspiration of bone marrow, in which no neoplastic infiltrations were evidenced. After four cycles of chemotherapy, total removal of the lesion was performed with a consecutive histopathological study of the surgical piece, indicating free edges of the lesion. Ewing’s sarcoma is a tumor with aggressive behavior, so this case represents a finding of clinical and epidemiological relevance, both due to its extra-osseous appearance and its unusual behavior.

scholarly journals Langerhans cell histiocytosis of the rib presenting with pathological fracture: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Tao Zuo ◽  
Ping Jiang ◽  
Junjie Yu ◽  
Ke Zhao ◽  
Yong Liu ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pathological Fracture ◽  
Soft Tissues ◽  
Langerhans Cell ◽  
Rib Fracture ◽  
Unknown Etiology ◽  
Case Presentation ◽  
Left Chest ◽  
Rib Tumor

Abstract Introduction Langerhans cell histiocytosis (LCH) is a rare neoplastic hyperplasia with an unknown etiology. It is clinically rare for patients with solitary rib lesion and pathological fracture; moreover, its diagnosis and treatment are quite difficult. The purpose of this study is to present a case for the pathogenesis, clinical features, imaging, and treatment of this disease. Case presentation A 52-year-old female patient complained of left chest pain for one week. CT showed a fracture in the left 5th rib. The rib tumor was then resected and the surrounding muscles and soft tissues were accordingly resected. The patient was diagnosed with pathological rib fracture, and the patient was pathologically diagnosed with LCH. After surgery, no local recurrence or distant metastasis was reported during the two-year follow-up. Conclusions LCH should be treated by observation, chemotherapy, radiotherapy, or surgery, or using a combination of several methods. Moreover, primary tumor should be considered when rib fracture without trauma and tumor metastasis.

scholarly journals Klippel-Trenaunay syndrome (KTS): A report of two patients and review of literature

Imaging ◽  
2021 ◽  
Author(s):  
Luca Procaccini ◽  
Bruno Consorte ◽  
Daniela Gabrielli ◽  
Antonietta Cifaratti ◽  
Massimo Caulo
Keyword(s):  
Multidisciplinary Approach ◽  
Soft Tissues ◽  
Vascular Malformations ◽  
Drainage System ◽  
White Men ◽  
Venous Drainage ◽  
Complex Disorder ◽  
Congenital Condition ◽  
Severity Of The Disease ◽  
Klippel Trenaunay Syndrome

AbstractKlippel-Trenaunay syndrome (KTS) is an uncommon congenital condition, resulting in vascular malformations affecting capillary, venous, and lymphatic systems and bone and/or soft tissue hypertrophy. Magnetic Resonance Angiography (MRA) may be useful in assessing the severity of the disease and for treatment planning. We present two cases of two white men with the typical clinical presentation of Klippel-Trenaunay syndrome i.e. vascular malformations (capillary, venous and lymphatic) and localized bone and/or soft tissues hypertrophy. Splenic hemangiomas were evidenced in both patients and MRA was helpful in assessing and delineating the abnormal venous drainage system. KTS is a complex disorder whose true prevalence and etiology are still unknown. In most cases the emblematic clinical manifestation consisting in vascular malformations and extremity overgrowth is represented. KTS may be associated with several different conditions including scoliosis and splenic hemangiomas. The presence of the lateral marginal vein (LMV) is pathognomonic. Imaging is fundamental in confirming the diagnosis and for therapeutic strategies. An effective treatment does not exist to date and a multidisciplinary approach is usually required to prevent complications.

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