A Unique Epithelioid Vascular Neoplasm of Bone Characterized by EWSR1 / FUS‐NFATC1 /2 Fusions

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.

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Epithelioid haemangioendothelioma in the parapharyngeal space

The Journal of Laryngology & Otology ◽

10.1017/s0022215106000314 ◽

2006 ◽

Vol 120 (6) ◽

pp. 505-507 ◽

Cited By ~ 7

Author(s):

J-C Lee ◽

B-J Lee ◽

S-G Wang ◽

H-W Kim

Keyword(s):

Endothelial Cells ◽

Facial Nerve ◽

Head And Neck ◽

Soft Tissues ◽

Parapharyngeal Space ◽

Malignant Potential ◽

Vascular Neoplasm ◽

Cytoplasmic Vacuolation ◽

Upper And Lower Extremities ◽

Epithelioid Haemangioendothelioma

Epithelioid haemangioendothelioma (EHE) is an uncommon vascular neoplasm which occurs rarely in the head and neck and has an unpredictable clinical behaviour. It is characterized by round or spindle-shaped endothelial cells with cytoplasmic vacuolation. Most often, EHE arises from the soft tissues of the upper and lower extremities, and it has borderline malignant potential. We describe the first reported case of EHE in the parapharyngeal space, which was treated successfully via a transcervical–transparotid approach (following angiography with embolization), without massive intra-operative bleeding or facial nerve damage.

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A cutaneous vascular neoplasm with hobnail microscopic morphology and unusual gross features

Journal of Cutaneous Pathology ◽

10.1111/j.1600-0560.2011.01823.x ◽

2011 ◽

Vol 39 (4) ◽

pp. 454-457 ◽

Cited By ~ 2

Author(s):

David Lukas Wachter ◽

Abbas Agaimy

Keyword(s):

Vascular Neoplasm ◽

Microscopic Morphology

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Hemangiopericytoma of the spinal canal

Journal of Neurosurgery ◽

10.3171/jns.1978.49.6.0914 ◽

1978 ◽

Vol 49 (6) ◽

pp. 914-920 ◽

Cited By ~ 42

Author(s):

Darrell J. Harris ◽

Victor L. Fornasier ◽

Kenneth E. Livingston

Keyword(s):

Endothelial Cells ◽

Differential Diagnosis ◽

High Risk ◽

Spinal Canal ◽

Vertebral Column ◽

Soft Tissues ◽

Surgical Removal ◽

Vascular Neoplasm ◽

Content Type ◽

Fine Print

✓ Hemangiopericytoma is a vascular neoplasm consisting of capillaries outlined by an intact basement membrane that separates the endothelial cells of the capillaries from the spindle-shaped tumor cells in the extravascular area. These neoplasms are found in soft tissues but have rarely been shown to involve the spinal canal. This is a report of three such cases. Surgical removal of the tumor from the spinal canal was technically difficult. A high risk of recurrence has been reported but in these three cases adjunctive radiotherapy appeared to be of benefit in controlling the progression of the disease. These cases, added to the six cases in the literature, confirm the existence of hemangiopericytoma involving the vertebral column with extension into the spinal canal. This entity should be included in the differential diagnosis of lesions of the spinal canal. The risk of intraoperative hemorrhage should be anticipated.

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Submucosal cavernous hemangioma of the esophagus. the rare clinical case of endoscopic diagnosis and treatment by the endoscopic submucosal dissection method (esd)

Клінічна та профілактична медицина ◽

10.31612/2616-4868.1(7).2019.05 ◽

2019 ◽

Vol 1 (7) ◽

pp. 33-40

Author(s):

V.A. Yakovenko ◽

A.M. Kiosov ◽

A.A. Denisenko ◽

V.P. Kovalenko ◽

S.V. Fen

Keyword(s):

Endoscopic Submucosal Dissection ◽

Cavernous Hemangioma ◽

Clinical Case ◽

Submucosal Tumor ◽

Integrated Approach ◽

University Hospital ◽

Vascular Neoplasm ◽

Submucosal Dissection ◽

Endoscopic Method ◽

Upper Third

The cavernous esophagus hemangiomais a rare, benign vascular neoplasm of the esophagus, which is asymptomatic and can be detected by chance during diagnostic video gastroscopy.The aim ofthe study is to describe in the rare clinical case of diagnosis and an integrated approach in choosing an endoscopic method of treating a submucosal benign vascular neoplasm of the esophagus (cavernous hemangioma).Material and method. A 46-year-old patient was sent to the University Hospital of ZSMU to conduct video esophagogastroduodenoscopy in order to examine and select the treatment method for the submucosaltumor of the upper third of the esophagus found in one of the city’s clinics.Results. A comprehensive examination of the patient with a submucosal neoplasm of the upper third of the esophagus, including video esophagogastroscopy with high-resolution endoscopes (EGD), endoscopic ultrasonography (EUS), and computer-aided tomography (CT) were performed. At the EGD a submucosal tumor was reviled in the upper third of the esophagus. It was bluish in color, had a wide base, 3cm in length and 2cm in height, movable, with soft-elastic consistency. EUS revealed that the tumor is not associated with the muscular layer, has many small vessels. The structure of the neoplasm was hypoechoic, inhomogeneous due to the inclusion of calcinates. On CT scan a rounded tumor was found in the lumen of the upper third of the esophagus, 20x15x30mm in size, with clear and even margins, homogeneous structure with a single calcinate in the structure was found. The patient was underwent of endoscopic surgery. The tumor was removed by the endoscopic submucosal dissection (ESD).Conclusion. Cavernous esophagus hemangioma is a rare vascular submucosal neoplasm of the esophagus, which is usually asymptomatic, but has a great potential for complications such as bleeding, which is sometimes fatal. Timely diagnosis and minimally invasive endoscopic treatment methods are the prevention of complications and the treatment of choice for the treatment of esophageal submucoustumors.

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Extragingival benign growth-Case report at a rare site

International Journal of Oral Health Dentistry ◽

10.18231/j.ijohd.2021.045 ◽

2021 ◽

Vol 7 (3) ◽

pp. 223-225

Author(s):

Merlin Thomas ◽

Leena Shettar

Keyword(s):

Case Report ◽

Pyogenic Granuloma ◽

Final Diagnosis ◽

Unusual Site ◽

Vascular Neoplasm ◽

Lower Lip ◽

Betel Quid Chewing ◽

Rare Site ◽

The Common ◽

Atypical Presentations

To present a case report of extragingival pyogenic granuloma on lower lip which is an uncommon site for pathological entity and also to emphasize the reason for its occurrence. Pyogenic granuloma (PG) is a benign vascular neoplasm more often appear as lobulated or smooth red nodular lesion commonly in gingiva. Extragingival occurrence of PG is very rare. The present article reports a rare case of 55-year-old female with PG presenting rapid growing mass on the lower lip. This article highlights the possibility on the reason for its occurrence which is important for the diagnosis in this atypical location. The primary etiology of lower lip lesion is trauma and contributory factors being poor oral hygiene and betel quid chewing. Pyogenic granuloma is one of the common lesions in oral cavity. This article highlights the pyogenic granuloma on lip which is an unusual site. Although it can be diagnosed clinically, atypical presentations lead to inappropriate diagnosis. Therefore, further investigation by biopsy confirms final diagnosis upon which treatment is instituted.

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Extensive nasopharyngeal angiofibroma case report

Bangladesh Journal of Otorhinolaryngology ◽

10.3329/bjo.v15i2.5061 ◽

1970 ◽

Vol 15 (2) ◽

pp. 75-77

Author(s):

M Alamgir Chowdhury ◽

Mousumi Malakar ◽

SM Golam Rabbani ◽

Naseem Yasmeen ◽

Shahidul Islam

Keyword(s):

Case Report ◽

Physical Examination ◽

Nasal Cavity ◽

Neck Region ◽

Lateral Rhinotomy ◽

Male Population ◽

Vascular Neoplasm ◽

Nasopharyngeal Angiofibroma ◽

Recurrent Epistaxis ◽

History Of

Juvenile nasopharyngeal angiofibroma is a benign vascular neoplasm, but it is locally aggressive. This accounts for less than 0.5% of all the neoplasm in the head & neck region in the male population only. Here we report a case of 10-year-old boy with a blackish red smooth polypoidal mass in the nasal cavity, with history of recurrent epistaxis. On physical examination it was suspected as nasopharyngeal angiofibroma. We removed it totally by lateral rhinotomy approach. And the diagnosis was nasopharyngeal angiofibroma on histopathology. Key words: Angiofibroma; Nasopharyngeal. DOI: 10.3329/bjo.v15i2.5061 Bangladesh J Otorhinolaryngol 2009; 15(2): 75-77

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Epithelioid Hemangioendothelioma Arising Within Mediastinal Myelolipoma: A WWTR1-Driven Composite Neoplasm

International Journal of Surgical Pathology ◽

10.1177/1066896919837611 ◽

2019 ◽

Vol 27 (6) ◽

pp. 664-668

Author(s):

Julio A. Diaz-Perez ◽

Jaylou Velez-Torres ◽

Oleksii Iakymenko ◽

Nestor Villamizar ◽

Andrew E. Rosenberg

Keyword(s):

Endothelial Cells ◽

Blood Vessels ◽

Chimeric Protein ◽

Epithelioid Hemangioendothelioma ◽

Adrenal Myelolipoma ◽

Vascular Neoplasm ◽

First Report

In this article, we describe a case of conventional epithelioid hemangioendothelioma (EHE) arising within an extra-adrenal myelolipoma. This composite neoplasm arose in the mediastinum of a 51-year-old female. The tumor was composed of a large myelolipoma that contained nodules of EHE consisting of CD31-positive epithelioid endothelial cells that grew in solid cords and were enmeshed in a basophilic hyalinized stroma. Both EHE and myelolipoma are characterized genetically by alterations of WWTR1. We demonstrated the expression of CAMTA-1 chimeric protein by immunohistochemistry both in the neoplastic endothelial cells of EHE and some of the endothelial cells lining the blood vessels in the myelolipoma. To the best of our knowledge, this is the first report of a malignant vascular neoplasm arising in association with myelolipoma.

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