Composite hemangioendothelioma- report of two cases located in bone and review of the literature.

BACKGROUND: Composite hemangioendothelioma (CHE) is a rare intermediate-grade vascular tumor characterized by a complex histologic component. It occasionally metastasizes, but local recurrence is not uncommon. CHE is mainly located in the extremities' distal dermis and subcutaneous soft tissues. It is rarely located in the bone. We report here two cases originally occurred in bone. CASE PRESENTATION: The first case of CHE occurred solely on the left pubis. The second case is a patient post-resection of CHE in the manubrium sterni 10 years ago [1], who presented with multiple lesions in the left ilium and T6, T12 vertebra. All these lesions in the two cases showed osteolytic bone destruction on computed tomography (CT) scans and showed relatively high signal intensity on the fat-suppressed sequences of T2-weighted magnetic resonance (MR) images and isointense signal intensity on T1-weighted MR images. After injection of contrast agent (Gd-DTPA), the lesions showed inhomogeneous enhancement. 2-deoxy-2 [F-18] fluoro-D-glucose positron emission tomography-computed tomography (18FDG PET-CT) showed increased FDG uptake in these osteolytic bone destruction areas with SUVmax around 5.0. Both patients underwent surgery. Lesions in the left pubis and left ilium were confirmed by postoperative pathology while lesions on the vertebrae were only biopsied, not surgically resected. The first patient had no recurrence or metastasis in 5 years after surgery. The second patient had surgery recently and is still being followed up. CONCLUSIONS: CHE occurring in bone is rarely reported. Our report provides more detailed information on the diagnosis of CHE. Given that CHE is locally aggressive and occasionally metastatic, PET-CT may be helpful in staging and follow-up.

Intravascular papillary hemangioendothelioma disguised as a peripheral sheath tumor of median nerve at the wrist: a case report and literature review.

BACKGROUND: Intravascular papillary hemangioendothelioma (IVPH) is a benign lesion previously reported in the nasal cavity, neck, upper extremities, and breast. Diagnosis with cross-sectional imaging can prove difficult, with histopathological examination necessary for diagnosis. IVPH resulting in carpal tunnel symptoms is quite rare. CASE PRESENTATION: We report the case of a 37-year-old woman who presented with a radial, volar right wrist mass enlarging over the span of 5 years. She noted numbness and tingling in her wrist and thumb, exacerbated by minor accidental collisions and wrist hyperextension. There was no antecedent trauma. On examination, a mildly tender, mobile mass was evident at the volar aspect of the right wrist. Magnetic resonance imaging (MRI) with contrast demonstrated a lobulated, predominantly T2 hyperintense, heterogeneously enhancing mass thought to be a peripheral nerve sheath tumor. The patient elected for surgical excision of the mass, and the histopathological examination showed organizing thrombi with prominent papillary endothelial hyperplasia. At the 2-month follow-up, the patient had full range of motion of her fingers and wrist, with subjectively normal sensation in the distribution of the median nerve. CONCLUSION: Carpal tunnel syndrome, in exceedingly rare occasions, can result from an IVPH. MRI findings may be confused with more common entities. Histopathological confirmation remains necessary for conclusive diagnosis.

Kaposiform hemangioendothelioma of the heart: a case report and literature review.

Kaposiform hemangioendothelioma is a rare tumour of vascular origin that rarely occurs in the heart. We provided a rare case of a 26-day-old infant with tachypnoea. Echocardiography showed a solid tumour in the pericardial cavity and a large amount of pericardial effusion. The solid tumour was confirmed by surgery, and the pathology was kaposiform hemangioendothelioma. We analysed this case and reviewed the related literature to explore the clinical features and echocardiographic manifestations to improve the understanding, diagnosis, and treatment of this disease for clinicians and sonographers.

Spinal intravascular papillary endothelial hyperplasia. Case report and review of the literature.

Intravascular papillary endothelial hyperplasia (Masson's vegetated hemangioendothelioma) is a rare condition affecting the neuroaxis. In the literature, only eight cases of this lesion involving the vertebral canal with spinal cord compression has been reported. We present a 37-year-old man with thoracic location mimicking schwannoma. Differential diagnosis, management, and review of literature are discussed in this short report.

Multiple papillary intralymphatic angioendotheliomas in the spleen.

A 18-year-old man presented with multiple asymptomatic masses in the spleen that had been detected on ultrasonography performed during a physical screening. He had no history of tuberculosis, and was otherwise well. Abdominal MR demonstrated multiple masses with internal stellate scars, which appeared as marked hypointensity on T2WI and contrast-enhanced MR. Most lesions showed inhomogeneous enhancement. The capsular enhancement was also revealed at delay phase. The patient underwent laparoscopic splenectomy. Pathological examination indicated papillary intralymphatic angioendothelioma (PILA), with the following immunohistochemistry results: CK (-), CR (-), ERG (+), CD34 (+), CD31 (+), D2-40 (+), Ki67 (3%+). The patient was feeling well at 6 months of follow-up.

A case of complex hemangioendothelioma of the liver in an infant.

A physical examination of a 9-month-old female infant presenting with vomiting and diarrhea revealed tenderness in the right upper abdomen and heightened abdominal muscle tone. Abdominal ultrasonography identified an irregular hypoechoic area within the right lobe of the liver. While a subsequent enhanced CT examination disclosed a well-defined lesion exhibiting internal focal calcification and delayed heterogeneous enhancement. Subsequently, she underwent surgical resection, and postoperative pathology revealed areas of epithelioid hemangioendothelioma and cavernous hemangioma. Immunohistochemistry demonstrated positive expression of CD34, CD31, FLI-1, and F-VIII. The pathologic diagnosis was confirmed as composite hemangioendothelioma (CHE).

Intravascular Endothelial Hyperplasia of the Foot.

Intravascular endothelial hyperplasia is a benign soft tissue mass rarely reported in the foot. Advanced imaging and confirming a benign diagnosis are critical for any soft tissue mass. This paper identifies 2 patients that developed intravascular endothelial hyperplasia tumors which required surgical excision. A 17-year-old male patient presented to clinic complaining of a painful bump to the arch of his right foot which he related to an injury 9 months prior. Magnetic resonance imaging of the right foot revealed a mass within the plantar subcutaneous fat that was serpiginous in nature similar to adjacent branching vessels favoring a low-flow vascular malformation. A 38-year-old female with Multiple Sclerosis presented with complaints of persistent symptoms of pain to the 1st interspace, difficult ambulation and neuritis. Ultrasound and MRI observed solid, multilobulated mass, with internal vascular malformation, MRI describing intrinsic involvement along the abductor musculature and flexor tendons. Both lesions were surgically excised and sent for pathology. Pathology report indicated a diagnosis of intravascular papillary endothelial hyperplasia or Masson's tumor in both cases. Pathology diagnosis of intravascular papillary endothelial hyperplasia is generally good with wide resection leading to low recurrence rates. Both patients in the current study have progressed postoperatively with resolution of symptoms and without recurrence.

Primary cardiac composite hemangioendothelioma with calcification mimicking a right atrial myxoma: A rare entity.

Composite hemangioendothelioma (CHE) is a rare vascular tumor which shows varying combination of benign, low-grade, and malignant vascular components on pathology. CHE is usually located on the surface of the dermis and subcutaneous tissue of the extremities. We report an unusual case of CHE in the heart.

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.

Intravascular Papillary Endothelial Hyperplasia of the Mandible: A Rare Entity.

ABSTRACT: Intravascular papillary endothelial hyperplasia (IPEH) is a lesion characterized by the proliferation of endothelial cells within the lumen of the vessel, it was first described by Masson in 1923.The pathogenesis of Masson's tumor is still unknown, currently the most accredited hypothesis is the reactive 1. Clinically and symptomatologically it can have different presentations, it is, therefore, of fundamental importance to make a correct differential diagnosis. Diagnosis is mainly based on histological criteria. Intravascular papillary endothelial hyperplasia usually occurs in the skin and subcutaneous tissues, less commonly in the skeleton. The present is the fifth case of intravascular papillary endothelial hyperplasia of the mandible.

Treatment modalities and long-term outcomes of hepatic hemangioendothelioma in the United States.

BACKGROUND: We analyzed the outcomes of patients with hepatic epithelioid hemangioendothelioma (HEHE) in the United States after stratification by their most definitive treatment. METHODS: The National Cancer Data Base was used to identify patients with HEHE between 2004 and 2018. Patients were divided in four treatment groups: no surgical therapy, ablation, liver resection or liver transplantation. Demographics and clinical characteristics were compared, and Kaplan Meier functions and Cox-regression were used for unadjusted and adjusted survival analyses. RESULTS: Among a total of 334 patients, 218 (65.2%) were managed non-surgically, 74 (22.1%) underwent hepatic resections, 35 (10.4%) underwent liver transplantation and 7 (2.1%) underwent ablations. The overall median survival was 111 months (95%CI 73-149) after liver transplantation, 69 months (95%CI 45-92) after hepatic resection, 38 months (95%CI 0-78) after ablation and 80 months (95%CI 70-90) for patients managed by watchful waiting (P < 0.001). After adjustment, patients who underwent liver transplantation were found to have a better survival when compared to other therapies (Hazard Ratio: 0.61, 95% Confidence Interval: 0.38-0.97, p = 0.035). CONCLUSIONS: This study reports the outcomes of the largest cohort of patients with HEHE. The longest survival was observed after liver transplantation, followed by non-surgical management and hepatic resection. Because of selection bias, future studies to better characterize what criteria should be used for the selection of treatment modalities for HEHE are urgently needed.

YAP1-TFE3-fused hemangioendothelioma: a multi-institutional clinicopathologic study of 24 genetically-confirmed cases.

YAP1-TFE3-fused hemangioendothelioma is an extremely rare malignant vascular tumor. We present the largest multi-institutional clinicopathologic study of YAP1-TFE3-fused hemangioendothelioma to date. The 24 cases of YAP1-TFE3-fused hemangioendothelioma showed a female predominance (17 female, 7 male) across a wide age range (20-78 years old, median 44). Tumors were most commonly located in soft tissue (50%), followed by bone (29%), lung (13%), and liver (8%), ranging from 3 to 115 mm in size (median 40 mm). About two-thirds presented with multifocal disease, including 7 cases with distant organ metastasis. Histopathologically, we describe three dominant architectural patterns: solid sheets of coalescing nests, pseudoalveolar and (pseudo)vasoformative pattern, and discohesive strands and clusters of cells set in a myxoid to myxohyaline stroma. These patterns were present in variable proportions across different tumors and often coexisted within the same tumor. The dominant cytomorphology (88%) was large epithelioid cells with abundant, glassy eosinophilic to vacuolated cytoplasm, prominent nucleoli and well-demarcated cell borders. Multinucleated or binucleated cells, prominent admixed erythrocytic and lymphocytic infiltrates, and intratumoral fat were frequently present. Immunohistochemically, ERG, CD31, and TFE3 were consistently expressed, while expression of CD34 (83%) and cytokeratin AE1/AE3 (20%) was variable. CAMTA1 was negative in all but one case. All cases were confirmed by molecular testing to harbor YAP1-TFE3 gene fusions: majority with YAP1 exon 1 fused to TFE3 exon 4 (88%), or less commonly, TFE3 exon 6 (12%). Most patients (88%) were treated with primary surgical resection. Over a follow-up period of 4-360 months (median 36 months) in 17 cases, 35% of patients remained alive without disease, and 47% survived many years with stable, albeit multifocal and/or metastatic disease. Five-year progression-free survival probability was 88%. We propose categorizing YAP1-TFE3-fused hemangioendothelioma as a distinct disease entity given its unique clinical and histopathologic characteristics in comparison to conventional epithelioid hemangioendothelioma.

Intracranial kaposiform hemangioendothelioma presenting as epistaxis: a rare case report with review of literature.

INTRODUCTION: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignancy with tendency for local invasion and recurrence. The tumor almost exclusively occurs in children, especially in infants. Intracranial KHE are extremely rare with only two cases reported in the literature. REPORT: We report the clinical and pathological features of this rare tumor arising from basitemporal region in a 21-month child. Our case did not present with Kasabach-Merritt phenomenon. Histopathological examination confirmed the diagnosis of KHE. CONCLUSION: KHE should be considered in the differential diagnosis of intracranial extra-axial neoplasm in children, and histopathological examination plays an important role in distinguishing KHE from its morphologic mimics. It is essential to diagnose KHE due to its locally aggressive nature.

Management of Refractory Mandibular Kaposiform Hemangioendothelioma with Sirolimus: A Case Report and Review of the Literature.

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm of intermediate malignancy that generally occurs in infancy and early childhood. Typically, the lesion arises from superficial or deep soft tissues of the extremities, trunk and retroperitoneum. The paucity of reported cases of head and neck KHEs is evidence of the rarity of the disease in this region. We report on the presentation and treatment of KHE in an 11-month-old boy who presented with a mandibular lesion. We include a brief discussion about the differential diagnosis of KHE. Management involved preoperative interventional radiology, surgical excision and chemotherapeutic treatment with Sirolimus. The lesion resolved without evidence of relapse 12 months later.

Pseudomyogenic Hemangioendothelioma of the Talocalcaneal Coalition: A Case Report.

Pseudomyogenic hemangioendothelioma is a rare soft tissue tumor most often found in the lower extremities and predominantly occurring in males. The talocalcaneal coalition is an anatomic anomaly that develops between the talus and calcaneus bones, can cause hindfoot pain and subtalar joint stiffness, and has a prevalence of less than 1%. We present what is to our knowledge the first case report of a 17-year-old male with pseudomyogenic hemangioendothelioma occurring within a talocalcaneal coalition. The patient was treated with local excision of the tumor and the coalition. His American Orthopaedic Foot & Ankle Society ankle/hindfoot score went from 70 preoperatively to 92 at 1 year postoperatively, and he has had no evidence of recurrence at 1 and 3 years postoperatively. These tumors have suggestive but not diagnostic computed tomography, magnetic resonance imaging, and histopathological findings, and they are associated with a uniquely characteristic immunophenotype, including immunochemical reactivity to CD31, FLi-1, INI-1, ERG, and FOSB. Primary treatment of pseudomyogenic hemangioendothelioma most often involves local excision (but can require amputation) and may include adjuvant radiotherapy and/or chemotherapy. It has a relatively favorable prognosis, with a moderate risk of local recurrence and a low risk of metastases. Because metastases have been reported many years after treatment, long-term follow-up is necessary.

Pseudomyogenic Hemangioendothelioma: A Rare Vascular Tumor of the Oral Cavity.

Pseudomyogenic hemangioendothelioma is a vascular neoplasm that presents a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Up to date, only 1 case of this entity has been reported in the oral cavity.

Papillary endothelial hyperplasia (Masson's tumor) developed in the capsule of the implant in a breast cancer patient treated with mastectomy and radiation therapy.

Papillary endothelial hyperplasia (PEH) is an uncommon benign vascular proliferation appearing in soft tissues in different body areas including the breast, which may be related to radiation therapy. A 48-year-old woman previously treated for breast cancer with mastectomy followed by radiation therapy and reconstruction with an implant presented with a newly developing mass in the implant capsule. Pathological diagnosis was PEH. Imaging features of this rare entity are described, and lesions included in differential diagnosis are discussed.

[Recurrent hemangioendothelioma of the lower and middle segments of inferior vena cava with spread to previously installed prosthesis]. / Retsidivnaya gemangioendotelioma nizhnego i srednego segmentov nizhnei poloi veny s rasprostraneniem na ranee ustanovlennyi protez.

We report surgical treatment of a 65-year-old patient with recurrent hemangioendothelioma of inferior and middle segment of inferior vena cava with spread to previously established prosthesis. Advanced resection of inferior vena cava and right-sided nephrectomy were not followed by complications and resulted R0 resection. Surgery time was 180 min. Inferior vena cava cross-clamping time was 30 min. Total blood loss was 300 ml. Hemangioendothelioma is a rare tumor with unpredictable potential for malignant transformation and obligatory indications for surgical treatment. Resection and reconstruction of inferior vena cava and renal veins with a synthetic conduit is effective and safe procedure.

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.

CASE REPORT: A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child's condition has markedly improved. DISCUSSION AND CONCLUSIONS: A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child's pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.

Intravascular Papillary Endothelial Hyperplasia of the Foot.

A case of intravascular papillary endothelial hyperplasia is presented. This proliferation usually forms inside thrombotic processes. It is critical to be able distinguish these lesions from low-grade angiosarcomas because they can have similar pathologic findings.