Rare Vascular Tumor Research Articles

Multiple retroperitoneal cystic lymphangiomas in a pregnant woman: A case report

Cystic lymphangiomas are extremely rare benign vascular neoplasms generally found in childhood. Thought to arise from obstructed lymphatic channels during development, but may also arise secondary to trauma, inflammation, malignancy, and surgery or radiation exposure. They are usually seen in the head and neck or axillary region, and rarely in the abdominal cavity with only 1% occurring in the retroperitoneum. These are typically asymptomatic lesions incidentally identified by computer tomography, magnetic resonance imaging or ultrasound. Complete surgical resection with histologic confirmation of the diagnosis is the treatment of choice.We present a case of a 27-year-old woman with multiple cystic lymphangiomas in retroperitoneum, discovered incidentally by ultrasound during pregnancy.

Tracheal Hemangioma: A Rare Cause of Hemoptysis in Children.

Tracheal hemangioma is a very rare benign vascular tumor. Few cases of tracheal hemangioma in children are reported in the literature. Chronic cough is the main symptom of the disease; however, hemoptysis is rarely reported as a symptom of tracheal hemangioma in children. Here, we report a case of tracheal hemangioma in an eight-year-old girl admitted to our pediatric department for recurrent hemoptysis. Routine biological and radiological investigations were normal. Diagnosis was made using computed tomography and bronchoscopy. Beta-blocker therapy was not efficient. Bronchoscopic treatment by electrical excision enables complete recovery. Tracheal hemangioma should be considered a cause of recurrent hemoptysis when routine biological and radiological investigations are negative.

Sinonasal Glomangiopericytoma with Prolonged Postsurgical Follow-Up.

Sinonasal glomangiopericytoma is a rare vascular tumor of the respiratory epithelium. Treatment consists mainly of surgical resection, though there is no consensus regarding the use of adjuvant therapies or preoperative endovascular embolization. The postsurgical prognosis is favorable, though there is a high risk of delayed recurrence. Here, we present the case of a patient who underwent endoscopic resection of a sinonasal glomangiopericytoma and a review of the literature.

Hepatic epithelioid hemangioendothelioma: Clinical characteristics, diagnosis, treatment, and prognosis.

BACKGROUNDHepatic epithelioid hemangioendothelioma (HEHE) is a rare hepatic vascular tumor with unpredictable malignant potential. The etiology, characteristics, diagnosis, treatment, and prognosis of HEHE are not well-understood, and large-scale retrospective studies are required to understand better this disease.AIMTo determine the characteristics of HEHE and identify its optimal treatments and prognostic factors.METHODSThe clinical data of two patients diagnosed with HEHE at the Fourth Hospital of Hebei Medical University and 258 previously reported cases retrieved from the China National Knowledge Infrastructure and PubMed databases between 1996 and 2021 were combined and summarized. All cases were pathologically identified as HEHE. Information such as clinical features, laboratory examination findings, imaging findings, pathological characteristics, treatment, and survival periods was reviewed. Kaplan-Meir curves were used for survival analysis. Prognostic factors were identified by Cox regression analysis.RESULTSHEHE primarily affected middle-aged women. The typical manifestations included epigastric pain, hepatosplenomegaly, inappetence, distension, weight loss, and fatigue. Tumor markers were expressed normally. The incidence of extrahepatic metastasis was 34.5% at the time of diagnosis. The most common sites of extrahepatic involvement were the lungs (22.3%), lymph nodes (5.6%), peritoneum (3.6%), bones (6.6%), and spleen (5.1%). Furthermore, “capsular retraction”, “target sign”, and “lollipop sign” were the characteristic features of HEHE on imaging. The immunohistochemical profile for HEHE (expression of vascular markers, such as factor VIII-related antigen, CD31, and CD34; expression levels of D2-40) can facilitate and ensure an accurate diagnosis. The management options for patients with HEHE include liver resection (29.7%), liver transplantation (16.1%), palliative treatments (12.7%), transhepatic arterial chemotherapy and embolization (TACE, 10.2%), chemotherapy (11.0%), antiangiogenic therapy (15.3%), and other treatments (5.1%); the mean survival time was 158.6, 147.3, 4.2, 90.8, 71.4, 83.1, and 55.0 mo, respectively. The survival time of patients who underwent surgical treatment was longer than that of patients who did not. TACE and antiangiogenic therapy tended to prolong survival compared with other nonsurgical treatments. The 1-, 5-, and 10-year survival rates were 82%, 71%, and 64%, respectively. Multivariate analysis showed that liver function (P = 0.045), intrahepatic metastasis (P = 0.029), and treatment (P = 0.045) were independent prognostic factors. The presence of extrahepatic metastases was not an independent risk factor for poor prognosis (P = 0.558).CONCLUSIONThe clinical course of HEHE is rare and variable, and patients with intrahepatic metastases and liver dysfunction may have a poorer prognosis than those without. Surgical intervention, whether liver resection or transplantation, might be warranted regardless of extrahepatic metastasis. For patients without the option for surgery, clinicians should consider the use of TACE with antiangiogenic drugs in the treatment of HEHE.

Mediastinal Haemangioma with Pericardial Effusion: A Rare Entity

Mediastinal haemangioma is a rare benign vascular tumour. A young male presented with complaints of cough and dyspnoea. Serial chest radiographs were suggestive of progressive mediastinal widening and cardiomegaly. Pericardiocentesis revealed haemorrhagic fluid which was negative for microbiology and malignant cells. Patient was unresponsive to antituberculosis treatment and steroids. Computed tomography (CT) of thorax revealed an anterior mediastinal mass lesion with pericardial effusion which on biopsy was found to be a mediastinal haemangioma.

Imaging Findings and Clinical Features of Primary Spinal Epithelioid Hemangioendothelioma.

Rationale and ObjectivesPrimary spinal epithelioid hemangioendothelioma (SEHE) is an extremely rare angiocentric vascular neoplasm with inherent metastatic potential, which pursues a clinical course intermediate between hemangioma and angiosarcoma. The present study sought to present computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical features of primary SEHE and review the literature.Materials and MethodsClinical data of four patients with primary SEHE confirmed by pathology from January 2011 to May 2020 were analyzed retrospectively. Two cases underwent CT scan, while 3 cases underwent MRI scan. Image observation included the tumor location, bone changes, morphology, density/signal characteristics, and enhanced features.ResultsThe four patients were all women who ranged in age from 25 to 74 years. Four cases of the lesions were in the vertebral body; among them, two cases involved the accessory of the vertebra. Soap bubble appearance was detected in 2 cases, honeycomb osteolytic appearance in 2 cases, a sclerotic margin in 3 cases, and accompanying vertebral compression fracture in 1 case. CT showed a slightly inhomogeneous low density with punctate high-density foci. MRI showed an inhomogeneous low signal on T1-weighted image (T1WI) and a high signal on T2WI. Contrast-enhanced CT/MRI demonstrated moderate to intensive inhomogeneous enhancement of the lesions. The discs were normal. In one case, lesions presented a dural tail sign.ConclusionsPrimary SEHE is so rare in the clinic as an aggressive vascular tumor. The relatively typical clinical features and radiographic findings can help in preoperative diagnosis.

Giant cervicofacial cystic lymphangioma

Cystic lymphangiomas are rare benign vascular tumors in children. Their most common location is cervico-facial. Lymphangiomas most often appear in the first two years of life. The diagnosis can be prenatal. Treatment varies depending on the type of lymphangioma, but surgery is classically and mostly recommended. We report a case of a giant cervical cystic lymphangioma in a 5-year-old child which has been confirmed by histology and treated surgically.

The First Case of Composite Hemangioendothelioma in The Heart

Composite hemangioendothelioma (CHE) is an extremely rare vascular neoplasm that is characterized by an admixture of benign, low-grade malignant, and malignant vascular components. It is usually located superficially in the dermis and subcutis of the extremities, and other sites involved include the head and neck region, oral mucosa, and viscera of the kidney and spleen. CHE has a low-grade malignant potential because it is locally aggressive. Here, we report a case of CHE in the heart in a 46-year-old man, who presented with a palpable mass arising from his right ventricle. Echocardiogram imaging revealed a 13.3 × 14.2 mm mass with high-signal intensity. Excision was performed, and microscopic examination revealed a heterogeneous mixture of vascular components, consisting of spindle-cell hemangioma, retiform hemangioendothelioma, and epithelioid-like hemangioendothelioma areas. To our knowledge, this is the first report on the behavior of this distinctive vascular neoplasm occurring in the right ventricle. Due to the unclear biological behavior of CHE in the heart and the paucity of cases, no further therapy was undertaken despite the risk of local recurrence and distant metastasis. The result of a six-month follow-up after surgery was disease-free.

Outcomes of Interventions for Angiosarcoma.

ObjectiveAngiosarcoma is a rare malignant vascular tumor, and the management and outcome of this disease are not well-described. The aim of this study was to report the incidence, patient demographics, and outcomes of angiosarcoma based on national data.MethodsData on patients with angiosarcoma were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Inverse probability treatment weights (IPTW) were used to assess the survival benefit of operation with additional chemo or radiation therapy compared to operation alone. These variables were further compared against patients who did not receive an operation despite being initially offered one. Cox regression was used to assess survival. Statistical analyses were performed on RStudio.ResultsFor this study, 5,135 patients (46% men; median age 69, range 0–102) with angiosarcoma were identified in the SEER database between 1975 and 2016. The age-adjusted incidence rate was 1–4%. Patients were mostly non-Hispanic Caucasian (75.4%). The average tumor size was 4.7 cm, range (.1–98.9). Tumor grades were high at presentation (Grade III 17.2, Grade IV 19, and unknown 50.6%), but half were considered localized tumors. Most patients underwent an operation (66.1%). In 5.6% of patients, the operation was recommended but not performed. The overall 5-year survival was 26.7% (95% CI 25.4–28.1%). IPTW with adjusted Cox proportional hazard model demonstrated worse survival, showing that operation compared to no operation and operation with chemo/radiation compared to operation alone had worse survival between months 0 and 25 but had improved survival after month 25.ConclusionsThe incidence of angiosarcoma is low and long-term survival is poor. Multimodal therapy in the form of neoadjuvant or adjuvant chemo/radiation therapy offers significant long-term survival benefits over operation alone.

Hémangioendothéliome épithélioïde pleural, une tumeur rare de diagnostic difficile : à propos d’un cas et revue de la littérature

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that develops in various organs. Primary pleural epithelioid hemangioendothelioma is an exceptional occurrence, with only forty cases reported in the literature. On account of its rarity, pleural EHE is difficult to diagnose. Differential diagnoses such as malignant pleural mesothelioma or lung carcinoma are often initially suspected. We herein describe the case of a 52-year-old man presenting with a primary pleural epithelioid hemangioendothelioma revealed by thoracic pain and having evolved for three months. Having reviewed the literature, we consider the clinical presentation and diagnosis modalities of this rare tumor. After an initial diagnosis of lung carcinoma, an ultrasound-guided biopsy was performed, confirming the diagnosis of pleural EHE in our patient. Ours is the first case of pleural EHE to be diagnosed with ultrasound-guided echography. Presentation of pleural EHE is often clinically and radiologically nonspecific. Most diagnoses are obtained by thoracoscopy, which allows for targeted biopsies and evacuation of pleural effusion. The diagnostic process for this rare tumor must be rigorous. Ultrasound-guided biopsy may be considered, provided that the lesions are accessible.

Lenvatinib as a Therapeutic Option in Unresectable Metastatic Pheochromocytoma and Paragangliomas.

ContextMetastatic pheochromocytomas and paragangliomas (mPPGL) are rare vascular neuroendocrine tumors that highly express vascular growth factors. Systemic treatment options in cases of unresectable multisite disease are limited. Multikinase inhibitors that inhibit angiogenesis, such as lenvatinib, have proven effective in several other malignancies, and may be a viable option for mPPGL.ObjectiveWe aimed to evaluate the efficacy of lenvatinib as salvage therapy in mPPGLs.MethodsThis was a retrospective analysis of mPPGL patients ≥ 18 years of age who received lenvatinib from 2015 to 2020 at a tertiary referral center. Patients were started on lenvatinib 20 mg daily and dose was adjusted according to tolerance or disease progression.ResultsEleven patients were included. Median treatment duration was 14.7 months (95% CI, 2.3-NE). Treatment was discontinued due to disease progression, adverse events, or death. Overall survival at 12 months was 80.8% (95% CI, 42.3-94.9%) but its median was not reached. Median progression-free survival was 14.7 months (95% CI, 1.7-NE). Among the 8 patients with measurable disease, overall response rate was 63%, as 5/8 experienced a partial response and 3/8 had stable disease. Worsening hypertension and anemia were the most common adverse events.ConclusionLenvatinib may be a viable treatment option for mPPGL, although at the potential risk of worsening hypertension. Larger, multicenter studies are needed to better characterize treatment efficacy.

PLACENTAL CHORANGIOMA AND ITS CLICOPATHOLOGICAL SPECTRUM: A CASE SERIES

Introduction:Chorangioma is a rare primary vascular tumour of placenta with an incidence of about 1% in carefully examined term placenta. Chorangiomas in the past have been described as hemangiomas. Chorangioma is a benign, vascular, non-trophoblastic tumor of plancenta that is potentially threatening and can lead to hazardous feto-maternal catastrophy. Objectives: To analyse clinicopathological characteristics, diagnosis, differential diagnoses, treatment, and prognoses of placental chorioangioma (PCA). Results: Mean age of the patients was 38 years. 2 patients were below 30 years and 2 were above 30 years. Only 1 patient was primigravida, rest 3 were multigravida. Mean gestational age was 31.8 weeks. 3 out of 4 cases were term gestations. Out of 4 patients, 3 had an emergency LSCS and 1 had normal labor. 1 out of the 4 patients delivered a dead fetus. Grossly, all the specimens werecomplete placenta. All lesions were extraplacental, solitary and all were >5cm. The mean tumor size was 7.3 cm (range: 6-8.5cm). All the tumowere nodular, well circumscribed, solid masses. On cut-section, lesions were reddish-brown to tan-off-white, solid, firm, hemorrhagic masses as depicted in the pictures. Microscopically, the lesions were composed of network of proliferating capillaries lined by benign endothelium in enlarged villi covered by trophoblastic epithelium. Focal hyalinized areas were seen in one of the cases as depicted in the pictures. Conclusion: Giant chorangiomas are relatively rare, however, their diagnosis and management pose a big challenge owing to serious antenatal feto-maternal complications they lead to. Careful radiological and histopathological examination are thus of utmost importance.

Giant carotid chemodectoma treated with a combination of surgery and CyberKnife radiotherapy: a case report and review of the literature

BackgroundParagangliomas are rare vascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissue. They occur most commonly at the carotid bifurcation, where they are known as carotid body tumors. Most paragangliomas are benign, locally aggressive, infiltrative tumors. Approximately 10% of patients with paragangliomas develop distant metastases, 10% present with multiple or bilateral tumors (mostly carotid body tumors), and 10% have a family history of paragangliomas. The malignant transformation of carotid body tumors has been reported in 6% of cases.Case presentationWe present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes.ConclusionsThe management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.

Health-Related Quality of Life in Children With Kaposiform Hemangioendothelioma.

Background and ObjectiveKaposiform hemangioendothelioma (KHE) is a rare, aggressive and borderline vascular tumor mainly occurring in infants and children. The aim of this study was to determine the health-related quality of life (HRQOL) in children with KHE.MeasuresA total of 91 children with KHE participated in this cross-sectional study. The HRQOL was assessed by the age-specific Pediatric Quality of Life Inventory Version 4.0 (PedsQL™ 4.0) Infant Scales, Family Information Form (FIF), Family Impact Module (FIM) and Generic Core Scales (GCS). For comparison, demographically matched healthy children were recruited as a control group. The main outcome measure of HRQOL was analyzed in the two groups. We determined related factors that influenced the HRQOL in children with KHE and their parents by using a stepwise multiple regression analysis.ResultsThe study found that the scores of each item in the family impact module (FIM) were lower than 75, which suggesting that KHE can make the parents of patients in a state of poor quality of life. The scores of physiological and psychosocial domains in all age groups of patients with KHE were lower than those of normal children (P < 0.01). Activity dysfunction is the factor influencing the physiological function score of all-age patients. KMP is the factor influencing the psychosocial function score of all-age patients.ConclusionsThe findings presented here suggest that patients with KHE have a poor HRQOL. KMP and activity dysfunction are risk factors for poor HRQOL in patients with KHE. However, lesion size, lesion location and education level of the mother and father were not related to the HRQOL.

Three cases of hepatic epithelioid hemangioendothelioma evaluated using conventional and contrast‐enhanced ultrasound: Case reports

Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare vascular endothelial cell tumor, which lacks typical clinical manifestations and specificity of imaging features. Whether the background of fatty liver and the difference in Contrast enhanced ultrasound (CEUS) characteristics between large and small lesions has not been well defined. In this case reports, we described the ultrasound image features of three patients with HEHE. These three patients with HEHE have certain similar characteristics of conventional ultrasound and CEUS. CEUS imaging features include large nodules show earlier perfusion than liver parenchyma, with rim‐enhancement, nonenhancing regions in the center, while small nodules show earlier perfusion than liver parenchyma, with hyperenhancement. All nodules show faster washout than hepatic parenchyma, showing heterogeneous hypoenhancement, and more washout lesions can be found in the PVP and LP. Conventional ultrasound and CEUS not only help to improve the diagnostic confidence of HEHE of rare liver tumors, but also can guide the biopsy area, making it easier to make accurate pathological diagnosis.

Epithelioid Hemangioma of Bone: A Rare Vascular Neoplasm. A Case Report and Literature Review

Epithelioid hemangioma (EH) of bone is a rare benign, albeit locally aggressive vascular neoplasm. It is usually solitary and involves the metaphysis or diaphysis of long tubular bones, especially in the lower extremities. Rarely it may present as multifocal lesions. The differential diagnosis includes malignant vascular bone tumors such as epithelioid hemangioendothelioma and epithelioid angiosarcoma. Clinical presentation and radiographic and histological findings are not specific and diagnosis is based mostly on immunohistochemical and molecular studies. There is no consensus regarding the optimal treatment. Curettage and bone grafting or en bloc resection are the current treatment options, however local recurrence have been reported. We present a case of multifocal EH of the distal tibia, distal fibula and hindfoot in a 38 year-old male managed with curettage, radiofrequency ablation and cement osteoplasty. The imaging features, histological findings and treatment options of this rare vascular tumor are discussed.

Meningeal Hemangiopericytoma: A Rare Intracranial Tumor—About a Case

Introduction: Hemangiopericytoma is a very rare vascular tumor, difficult to diagnose, often confused with a meningioma. Imaging is not very specific and the diagnostic certainty remains histological. Observation: We report a case of meningeal hemangiopericytoma in a 48-year-old patient with a history of active smoking and whose sister died of glioblastoma. He was brought to the emergency room for a fall, confusion, and tonic-clonic seizures. The diagnosis was suspected on imaging (CT and MRI, in front of a syndrome of right temporal intraventricular mass measured at 37 mm in diameter, centered on the choroid plexus, with perilesional vasogenic edema and posterior white commissure. There was homogeneous enhancement of the lesion after gadolinium injection. The diagnosis was confirmed by immunohistochemical study, which showed diffuse expression of STAT6 and partial labeling for CD34, without significant expression of SSTR2. Expression of neurofilament protein was also found in the brain parenchyma. Conclusion: Hemangiopericytoma is a rare tumor, characterized by its malignant potential, its high rate of recurrence and distant metastasis. The management is based on a total microsurgical resection followed by radiotherapy.

Rare epithelioid hemangioendothelioma in the brachiocephalic vein for long-term survival after surgery: A case report

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor. In this report, we describe the case of a 62-year-old man who presented with pain in the left clavicle and swelling of the left upper limb. Contrast-enhanced computed tomography revealed an intravascular tumor, which was completely resected surgically. Histopathological examination and immunohistochemical staining revealed that it was epithelioid hemangioendothelioma with occurrence in the left brachiocephalic vein. It has been 6 years since the surgery was performed, and no recurrence has been observed. Epithelioid hemangioendothelioma may recur or metastasize and therefore requires careful follow-up.

A Case Report of Intraocular Radiotherapy with Strontium-90 for Juxtapapillary Retinal Capillary Haemangioma

Juxtapapillary retinal capillary haemangiomas are rare benign vascular tumors located adjacent to the optic disc. They may occur sporadically or in association with von Hippel-Lindau disease. Treatment modalities for juxtapapillary retinal capillary haemangiomas vary from observation to aggressive vitreoretinal surgery. A combination of different treatment modalities is often tried with modest success. We report a patient diagnosed with right-sided juxtapapillary retinal capillary haemangioma at the age of 36 in an amblyopic eye previously treated with patching and surgery. In 2013, at the age of 45 he presented with increasing shadow in his right visual field. The ophthalmological examination revealed a right-sided subtotal serous retinal detachment with 6.2×5.6×3.5 mm juxtapapilllary retinal capillary haemangioma and visual acuity of hand movements. He underwent vitreoretinal surgery, radiation of juxtapapillary retinal capillary haemangioma with intraocular strontium applicator and insertion of silicone oil, all in one session. We describe a novel treatment, the intraocular radiation with strontium. This can be applied in complicated cases where vitrectomy is necessary.

Liver Transplantation for Diffuse Hepatic Epithelioid Hemangioendothelioma

Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare vascular tumor. His treatment is not clearly defined. In diffuse HEHE, liver transplantation (LT) is the only possible surgery. Male, 60 years old, medical history: allergic to acetylsalicylic acid and hiatal hernia, presents anorexia, asthenia, weight loss and abdominal distension. Ultrasound, abdominal CT, MRI and PET-CT showed multiple bilobar nodules that diffusely affect liver parenchyma, ascites without portal thrombosis and no extrahepatic disease. FNAC: HEHE. The patient presents clinical and liver function worsening due to thrombosis of the central and right portal branch. He was evaluated in the Transplant Committee and listed with MELD 27. In July 2021, he underwent LT with vena cava preservation. Cold ischemia time (275 minutes), warm ischemia (32 minutes) and total surgery (288 minutes). A portal thrombectomy and hilar lymphadenectomy was also performed. Immunosuppression is started with corticosteroids, mycophenolate, and basiliximab (single dose). On the third day started with Advagraf®. He was discharged on the 11th day post-LT without Clavien-Dindo≥ IIIa complications. Pathology: diffuse HEHE with intrahepatic venous invasion and gallbladder wall; portal tumor thrombus and metastases in 5 /23 lymph nodes excised. Positivity for CD31, CD34 and negative for CKAE1/AE3. Low proliferative activity with Ki67:3%. Diffuse HEHE is an extraordinary rare tumor. The survival of HEHE after LT, according to the ELTR, is 88.6% and 79.5% at 1-year and 5-years respectively. 25% of LT patients have recurrence (median:18 months). The results obtained with LT are excellent, although the risk of recurrence is not negligible.