Low-grade Malignant Vascular Tumor Research Articles

Sinonasal hemangiopericytoma: Report of two cases and review of literature

IntroductionHemangiopericytoma is a low grade malignant vascular tumor. It is rare and is characterised by a poorly clinical presentation and often confused with other fibrous tumor, which could poses difficulties on diagnosis. The nasal cavity is an uncommon site and have a distinct behaviour compared to others location.Case presentationWe report the case of two women who consulted for epistaxis and nasal obstruction. Vascular masses were visualized with nasal endoscopy – one in the left nasal cavity and the second one had a destructive mass of the right hemiface. CT scan and MRI helped identifying the mass, its margins and its extirpability. The Diagnosis was confirmed by histology examination. In one patient, surgery was performed. The second patient had an aggressive and invading tumor for which chemotherapy was performed. The first patient remained free of disease after surgery for a follow-up of 3 years, the second one had a fatal evolution.DiscussionSurgical treatment is the important mainstay, and it still controversial regarding wetherwhether the endonasal surgery could control tumor resection despite the wide performance, nowadays, of endoscopic techniques is still controversial. The use of embolization before surgical removal in place of chemotherapy is still unclear. We report our experience through two cases.ConclusionThe high rate of recurrence of this tumor requires a long follow-up after the most radical surgery possible. The external way must be adopted easily in front of any doubt of complete removal by endoscopy. Endoscopic techniques were regarded as appropriate for small low-vascularized tumors only.

Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease

BackgroundEpithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth.Case presentationWe present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion’s clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications.ConclusionsWith this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment.

Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature.

This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25–54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle-shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low- to moderate-level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

MRI findings of a hepatic epithelioid hemangioendothelioma.

Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Hepatic EHE can manifest as diffuse nodular lesions with predilection of peripheral subcapsular growth, nodular confluence, together with "halo" sign and "capsular retraction" sign. These imaging features help to achieve the diagnosis.

Hepatic Epithelioid Hemangioendothelioma: Is Hepatitis C Viral Infection an Etiologic Factor?

Introduction: Hepatic Epithelioid Hemangioendothelioma (HEHE) is an infrequent vascular tumor of endothelial cell origin. There is no clearly established risk factor for its development. It behaves as a lowgrade malignant vascular tumor with slow progressive phenotype. Liver transplantation should be considered the first-line of therapy given the large tumor burden and favorable long-term outcome. Case: We report a 48-year-old Caucasian female who complained of abdominal pain and weight loss. She has a history of hepatitis C viral infection that was treated with interferon and ribavirin and subsequently became RNA-negative on repeated follow-up testing pre-transplant. Her work-up revealed multiple confluent infiltrating bilobar liver masses diagnosed as HEHE (biopsy-proven) and cirrhosis. She underwent a successful liver transplant and is tumor-free without evidence of recurrent HCV infection 22 months post-transplant. Conclusion: Liver transplant provides remarkable long-term survival for HEHE/HCV cirrhotic patient considering the large tumor burden and lack of evidence of recurrent tumor and/or HCV infection.Figure: [CT of the Abdomen]Figure: [Photomicrograph of HEHE in Hep C cirrhosis]

Epitheloid Hemangioendothelioma and Liver Transplantation

Purpose: A 45 year old man presented with low grade fever, progressive weight loss and right upper abdominal discomfort of 3 wks duration was found to have a diffuse infiltrating mass involving the right and majority of the left lobe. The patient underwent a needle biopsy of the mass in an outside institution and was diagnosed as hepatocellular carcinoma. He underwent two sessions of Transarterial chemoembolization (TACE) with doxorubicin beads. Post TACE his symptoms worsened, he presented to our hospital with worsening fatigue, dyspnea, jaundice and rapid increase in abdominal girth. Computerized tomography (CT) of the abdomen revealed large ascites and hepatic hydrothorax. He underwent paracentesis and thoracocentesis draining 5L and 1L respectively. The fluid cytology was negative for malignancy. The laboratory findings included the following. Bilirubin: 27 mg/dL, alkaline phosphates: 449 U/L, alanine aminotransferase: 77 U/L, aspartate aminotransferase: 123 U/L, albumin: 1.8 g/dL, all hepatitis, viral and autoimmune panels were negative. INR: 1.54, AFP: 2.3 ng/ml, CA 19-9: 4.2, CEA: <0.5 mcg/L. CT of the abdomen showed a nodular, shrunken liver with a large diffuse hypodense lesion involving the entire right lobe of the liver, and the medial lateral segment of the left lobe of the liver. The lesion in the right lobe of liver was causing mass effect on the intrahepatic portion of the inferior vena cava. No intrahepatic biliary dilatation. Review of the needle biopsy revealed epitheloid cells with immune positivity to CD31 and CD34. A diagnosis of epitheloid hemangioendothelioma was entertained and he underwent liver transplantation. Post transplant period was uneventful and, he is doing quite well and fully functional five months after his transplant. Discussion: Hepatic epithelioid hemangioendothelioma(EHE) is a rare, low-grade malignant vascular tumor. It is usually misdiagnosed as HCC. The EHE has a better prognosis compared to same grade of HCC. EHE presents histologically as small groups or isolated neoplastic cells surrounded by a generous amount of fibro-sclerotic stroma, which eventually obliterates the hepatic plates. All tumors should be positive for at least one endothelial marker, such as CD34, CD31, or factor VIII related antigen with immunohistochemical staining. Most common treatment is OLT followed by liver resection (LRx). LRx for HEHE is restricted to patients with limited involvement of the liver. Most patients with HEHE have large, multifocal tumors at the time of diagnosis rending OLT as the most feasible option. Patient survival rates after OLT at one and five years were 80% and 64%, respectively. Allograft survival rates were 70% and 55%, respectively.

Ulcerated Epithelioid Hemangioendothelioma of the Right Armpit in Childhood

Epithelioid hemangioendotheliomas are unique vascular tumors characterized by epithelioid or histiocytoid endothelial cells that mainly affect adults. This low-grade malignant vascular tumor was described as a distinctive condition in 1982 by Weiss and Enzinger. Although the tumor is classified in between an angiolymphoid hyperplasia with eosinophilia and an epithelioid angiosarcoma, it sometimes takes a clinical course resembling that of angiosarcoma. We describe that case of a 12-year-old boy who presented with an approximately 6-month history of a spontaneous chronic lesion in his right armpit and became a painful ulceration in the prior 2-month period. The histopathologic examination revealed small nests and cords of spindling epithelioid endothelial cells, intracytoplasmic lumina containing erythrocytes, pinocytotic vesicles and a necrotic area. Immunohistochemical staining was positive for the endothelial markers CD31, CD34, CK(+), and vim(+). On the basis of these findings the diagnosis of EHE was made. After surgery, pathologic examination revealed metastasis in the lymph nodes. So polychemotherapy was started. As our case report shows, it is possibility that cutaneous ulceration of a malignant tumor such as EHE should be considered, even in children.

Transient hepatic attenuation difference on computed tomography associated with hepatic epithelioid hemangioendothelioma

Hepatic epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular tumor with a clinical course between cavernous hemangioma and angiosarcoma. Several imaging findings have been described in EHEs. We report a case of an EHE which demonstrated an associated transient hepatic attenuation difference (THAD) on arterial-phase computed tomography examination. THAD associated with an EHE has not previously been reported. The presence of a THAD may be related to the propensity of this tumor for vascular invasion.

Composite Hemangioendothelioma

Composite hemangioendothelioma (HE) is a low-grade malignant vascular tumor showing varying combinations of benign, low-grade malignant, and malignant vascular components. The predominant histologic components are histologically identical to epithelioid HE and retiform HE. To our knowledge, there have been only 12 cases of composite HE reported in the English literature and its nature and biologic behavior remains unknown. In this study, the clinicopathologic and immunohistochemical features of 5 cases of composite HE including a case with associated Maffucci syndrome are described. The patients were 4 females and 1 male with a median age of 43.4 years (range, 22 to 75 y). All tumors occurred in the dermis and/or subcutis. The tumors arose in the foot or lower leg in 3 patients, in the jaw in 1 patient, and as multiple tumors in the left upper extremity in 1 patient. Two patients had congenital tumors, in the lower thigh and foot, and upper extremity, respectively. The lesions were usually of several years duration. The size of individual tumors ranged from 1.5 to 30 cm. The tumors were composed of a complex admixture of histologic components resembling various vascular lesions. The predominant components, present in all cases, resembled retiform HE and epithelioid HE. Angiosarcomalike areas were observed in 3 cases. Lymphangiomalike areas were found in 2 cases. Areas of spindle cell hemangioma, cavernous hemangioma, or arteriovenous malformation were identified in 1 case each. The 2 congenital cases, which exhibited multiple lesions, had angiosarcomalike components and an angiomatosislike growth pattern. One patient each was associated with Kasabach-Merritt or Maffucci syndrome. Immunohistochemically, all tumors showed expression of at least 2 endothelial markers (CD31, CD34, and/or factor VIII-related antigen). Of 4 cases with follow up (median duration, 8.6 y), 1 tumor recurred locally. To date, none of the patients have developed metastases. There was no difference of biologic behavior among cases with various combinations of histology in this study and previously reported cases. We conclude that composite HE should continue to be regarded as a low-grade malignant vascular tumor (HE), with significant potential for local recurrence, but little if any potential for distant metastasis.

CT Halo Sign in Pulmonary Epithelioid Hemangioendothelioma: A Case Report

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare low-grade malignant vascular tumor of minimal clinical expression in young women. Bilateral multiple pulmonary nodules, up to 2 cm in size, are typical CT findings. We describe an atypical case of PEH with a fatal outcome in a 65-year-old male, showing multiple pulmonary nodules with a typical CT halo sign by pulmonary hemorrhage.

Hemangioendothelioma of the spine.

A review of the patient files from our institution's oncology department showed that between 1950 and 2000, 11 patients were diagnosed with hemangioendothelioma of the spine, a rare, low-grade malignant vascular tumor. After reevaluation of the diagnoses by orthopaedic oncologists and pathologists, three patients were excluded; therefore eight patients formed the study group. The eight lesions were located in the thoracic (three) or lumbar spine (five, one in each vertebral level). Computed tomography scans revealed expansile lytic process. All lesions involved the vertebral body, but only one was diffuse with spinous process involvement. Treatment included: external beam irradiation alone (one patient), curettage and external beam irradiation (one patient), laminectomy and external beam irradiation (two patients), anterior resection only (two patients), and anterior resection with postoperative external beam irradiation (two patients). Patients without spinal stabilization had chronic low back pain; no patient with spinal stabilization had such pain. One patient who had surgical resection and radiation therapy had a radiation-induced sarcoma develop after 4 years. One patient who was treated with radiation therapy alone had a metastatic lung lesion develop. No other patient had tumor recurrence or progression. Therefore, patients with hemangioendothelioma of the spine may be treated with radiation therapy alone, surgery alone, or a combination thereof. However, because the number of patients in the current series was small, no definitive recommendations regarding treatment may be made. Resection of large lesions and stabilization of vertebral collapse may decrease back pain.

A case of childhood epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a unique tumor, mainly of adults, which is characterized by epithelioid or histiocytoid endothelial cells. It is a low-grade malignant vascular tumor first described as a distinctive entity in 1982 by Weiss and Enzinger. An 8-year-old girl presented with an approximately 3-year history of an erythematous plaque on the left frontal area of the scalp. The diagnosis of EHE confined to the skin was made by clinicopathologic and radiologic evaluation. To our knowledge, there are only 5 reported cases of EHE with skin involvement, 2 of which showed concomitant systemic involvement, and all occurred in adults. Our case is the fourth reported case of EHE confined to the skin and the first to describe childhood onset of isolated cutaneous EHE. (J Am Acad Dermatol 2000;42:897-9.)

Composite hemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma.

Eight cases of a previously uncharacterized vascular neoplasm, showing varying combinations of benign, low-grade malignant, and malignant vascular components are described. Seven tumors occurred in the dermis and/or subcutis and one occurred in the oral submucosa. The patients were all adults with a median age of 39.5 years (range, 21-71 years). Five patients were men. The tumors arose predominantly in the hands and feet, and the lesions were usually of several years duration. The tumors were composed of a complex admixture of histologic components that varied from tumor to tumor, such that no two tumors looked precisely the same. This was due to variation in the proportions of each component as well as the manner in which each component was distributed throughout each lesion. The predominant histologic components were epithelioid hemangioendothelioma (HE) and retiform HE, which were each present in seven of the tumors. Areas of spindle cell HE were identified in four lesions. Angiosarcoma-like elements were identified in seven tumors. One of the tumors was associated with an arteriovenous malformation and one was associated with an area of lymphangioma circumscriptum. Of six cases with follow up (median duration, 6.5 years), three have recurred locally and, to date, only one has metastasized. We think composite HE is best regarded as a low-grade malignant vascular neoplasm, and the available data suggest that it behaves more favorably than conventional angiosarcoma. The existence of these composite lesions has led to careful reexamination of the concept of HE. The term HE, in that it is currently synonymous with a low-grade malignant vascular tumor, should be reserved for lesions that have true metastatic potential, albeit with low frequency.

右前腕の筋肉内に発生したＳｐｉｎｄｌｅ ｃｅｌｌ ｈｅｍａｎｇｉｏｅｎｄｏｔｈｅｌｉｏｍａの１例

Spindle cell hemangioendothelioma is a new entity defined by Weiss and Enzinger in 1986, as a low-grade malignant vascular tumor. Although the neoplasm has involved in the dermis and subcutaneous fatty tissues, no case has occurred in the muscle. The first case of spindle cell hemangioendothelioma originated from the muscle is reported. A 48-year-old female had a tumor which occurred in the extensor digiti minimi of right forearm.CT scan demonstaratetd multinodules in an isodensity region within the skeltal muscle.MRI showed multinodules in a high signal intensity region on both T1-and T2-weighted images.The patient was treated with wide excision and had no evidence of local recurrence or metastasis 5 months later.