Primary Vascular Tumors Research Articles

Primary splenic angiosarcoma: a rare case of embolization as a bridge to splenectomy

Primary splenic angiosarcoma is the primary malignant vascular neoplasm of the spleen. This tumor is highly aggressive and extremely rare with a nonspecific clinical presentation. Prognosis is poor and almost all patients die within one year of diagnosis. We present the case of a 69-year-old healthy woman with left-sided abdominal tenderness. An ultrasound examination of the abdomen was performed to evaluate the cause of abdominal pain and showed splenomegaly with inhomogeneous echotexture and the presence of free intraperitoneal fluid. An emergency abdominal contrast-enhanced computed tomography confirmed the splenomegaly and showed hemoperitoneum and a splenic mass with heterogeneous contrast enhancement. Due to the sudden worsening of the patient’s clinical conditions (hemoglobin level of 7 g/dl) and the unavailability of the operating room, embolization of the splenic artery was performed as a bridge to subsequent splenectomy under the suspicion of splenic rupture. Histological examination demonstrated a primary splenic angiosarcoma and the patient was referred to an oncological center. Due to the rarity of this malignancy, the optimal treatment of primary splenic angiosarcoma remains under investigation. Transcatheter splenic artery embolization is not common, but it could be useful for temporarily stabilizing the patient as a bridge to the subsequent splenectomy.

Case Report: Incidental Littoral Cell Angioma of The Spleen [Benign Splenic Tumor

littoral cell angioma is an unusual benign tumor of the spleen and is primary splenic vascular tumor arises from the normal littoral cells lining the sinus channels of the splenic red pulp we are report a case of 17 years male boy have history of abdomen pain for 2day with fever and he was managed with laparotomy and splenectomy and Postoperative pathology biopsy examination confirmed the final diagnosis of LCA of spleen.

The role of liver transplantation in the care of primary hepatic vascular tumours in children.

Liver transplantation (LT) is the standard of care for many liver conditions, such as end-stage liver diseases, inherited metabolic disorders, and primary liver malignancies. In the latter group, indications of LT for hepatoblastoma and hepatocellular carcinoma evolved and are currently available for many non-resectable cases. However, selection criteria apply, as the absence of active metastases. Evidence of good long-term outcomes has validated the LT approach for managing these malignancies in the context of specialist and multidisciplinary approach. Nevertheless, LT's role in treating primary vascular tumours of the liver in children, both benign and malignant, remains somewhat controversial. The rarity of the different diseases and the heterogeneity of pathological definitions contribute to the controversy and make evaluating the benefit/risk ratio and outcomes quite difficult. In this narrative review, we give an overview of primary vascular tumours of the liver in children, the possible indications and the outcomes of LT.

OA28 A rare case of cardiac tumour in infancy mimicking sarcoidosis

Introduction/BackgroundSarcoidosis is a rare multisystemic inflammatory disorder with 2 distinct presentations in childhood. A monogenic form associated with mono-allelic mutation in NOD2 presents with the triad of uveitis, rash and arthritis in children less than 4-years (early-onset sarcoidosis). The other presentation affects older children with similar presentation to adults, with greater pulmonary and lymph node involvement. Histopathological evidence of non-caseating granulomas is essential for the diagnosis but does not exclude alternative diagnoses. We report a 17-month-old infant, who presented with atypical features suggestive of sarcoidosis, but was subsequently found to have a rare cardiac angiosarcoma driving an exaggerated granulomatous reaction.Description/MethodA previously healthy 17-month-old girl born to non-consanguineous Caucasian parents presented with recurrent fever for 3-months, associated with lethargy, weight loss and worsening anemia. Examination revealed an irritable febrile child, weight on 5th centile, with muffled heart sounds; there were no skin lesions, no lymphadenopathy, and no arthritis. Investigations showed severe anemia (Hb 46g/L), leukocytosis (WBC 24/L), thrombocytosis (662/L), high inflammatory markers (CRP >300mg/L, ESR 160mm/hr), high ferritin (600ug/L). CT thorax with cardiac angiogram revealed a large intra-atrial septal mass, extensive pulmonary nodules, bilateral hilar and mediastinal lymphadenopathy and pericardial effusion. She was managed with broad-spectrum antibiotics, blood transfusions and pericardiocentesis with endomyocardial biopsy. Extensive infectious workup, including TB from pericardial exudate, was negative. Serum angiotensin-converting enzyme was normal, with no blasts in blood film; eye examination was unremarkable. The endomyocardial biopsy was inconclusive, so underwent lung and lymph node biopsies, which were initially reported as revealing florid multiple non-necrotising granulomas, but no evidence of malignant cells. Whole-exome sequencing was performed, result pending at that stage. Differential diagnosis included malignancy, infection, immunodeficiency or autoimmune disorder including sarcoidosis, in light of histopathology. With malignancy and infection felt to be excluded, she was managed empirically for atypical sarcoidosis and treated with IV methylprednisolone 30mg/kg followed by tapering steroid and anti-TNF; IL-1 was added later. However, there was no clinical response with ongoing fever, irritability and static inflammatory markers (CRP∼300mg/L). Immunomodulating medications were subsequently stopped. Repeat chest imaging revealed progression of pulmonary findings and increasing cardiac mass. Open-heart bypass surgery with debulking of cardiac mass and further biopsies. Histopathology revealed an extremely rare primary cardiac malignant vascular tumour, angiosarcoma, with loss of SMARCB1 protein staining. Retrospective review and specific staining with SMARCB1 of initial lung biopsy subsequently confirmed that the granulomatous reaction was in response to micro-metastases of tumour throughout the lungs.Discussion/ResultsThe differential diagnosis of granulomatous disease in paediatric patients is very broad. Ultimately, the diagnosis of cancer driving and exaggerated immunological response was provided by a combination of very invasive surgical biopsy of the heart; combined with whole exome sequencing. Whole exome sequencing revealed 2 distinct genetic diagnoses which fully explained the phenotype: heterozygosity for a stop mutation in SMARCB1 (p.R158X) consistent with Rhabdoid Tumour Predisposition Syndrome Type 1; and homozygosity for the hypomorphic PRF1 p.A91V mutation explaining low (but not absent) intracellular perforin levels and exaggerated granulomatous immunological response to tumour. The rare angiosarcoma detected is scarcely reported in the literature, but entirely compatible with the type of cancer associated with RTPS1. Hypomorphic perforin mutation probably explains the exaggerated but frustrated granulomatous immunological response to tumour in this case.Key learning points/ConclusionThis case serves to remind us that granulomas can occur in response to malignancy in children. Lack of response to immunosuppression with ongoing severe systemic inflammation in this case provided an important clue to the need for more invasive diagnostic biopsies. Particular challenges relates to the time it takes to obtain genetic test results; but in this case whole-exome sequencing resolved the phenotype fully. Patients with rare and unusual presentations may have more than one genetic diagnosis, with a blending of phenotypes thereafter. Screening of other family members for the mutation associated with RTPS1 is ongoing.

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.

Multidisciplinary, articular surface-preserving treatment strategy for locally aggressive epithelioid hemangioma of the acetabulum employing serial bland transarterial embolization.

Epithelioid hemangioma is a rare, histologically benign but locally aggressive primary vascular neoplasm that can rarely arise in bone. Mainstay treatment is surgical resection or curettage with bone grafting. We report a novel multidisciplinary, joint-sparing treatment approach for an epithelioid hemangioma of bone arising in the acetabulum causing severe thinning of the subchondral bone plate. After 4 sessions of transarterial bland particle and ethanol embolization, the resultant increased ossification of the tumor allowed preservation of the articular surface during surgical resection. Imaging follow-up 14 months after surgical resection showed no evidence of recurrence and continued ossification of the portions of the lesion treated only with embolization.

Primary vascular tumours of the kidney

Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical, morphological, and immunohistochemical features. This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology, genetics, and prognosis. Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels. These tumours are rare, with about 75 cases reported in the literature. Most anastomosing haemangiomas are found incidentally on ultrasound, computed tomography, or magnetic resonance imaging. Common symptoms include abdominal pain, haematuria, and abdominal mass. Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes. The prognosis of anastomosing haemangioma is excellent. Primary renal angiosarcomas are malignant tumours showing endothelial differentiation. To date, 76 cases have been described in the literature. Primary renal angiosarcomas are frequently symptomatic. The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas, including abdominal pain, haematuria, and abdominal mass. Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR, TIE1, SNRK, TEK, and FLT1 are upregulated in angiosarcomas. Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment, chemotherapy, radiotherapy, or targeted therapy.

Primary malignant vascular tumors of the liver in children: Angiosarcoma and epithelioid hemangioendothelioma.

Primary malignant vascular neoplasms of the liver, angiosarcoma and epithelioid hemangioendothelioma, are extremely rare entities in the pediatric population. International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group. In this article, we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population. Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis. The distinct histologic features include whorls of atypical spindled cells and eosinophilic globules, in addition to the general findings of angiosarcoma. Histologic diagnosis of pediatric hepatic angiosarcoma is not always straightforward, and the diagnostic challenges are discussed in the article. Hepatic epithelioid hemangioendothelioma also demonstrates a female predominance, but is more commonly identified in adolescents (median age at diagnosis: 12 years). Histologically, the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid stroma. While WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma, there are currently no known signature genetic alterations seen in pediatric hepatic angiosarcoma. Advancement in molecular pathology, particularly for pediatric hepatic angiosarcoma, is necessary for a better understanding of the disease biology, diagnosis, and development of targeted therapies.

Multi-Focal Splenic Tumour in a Belgian Patient and a Brief Review of the Literature on Littoral Cell Angioma.

Littoral cell tumour, although a very rare neoplasm, must be included in the differential diagnosis of splenic lesions observed by imaging.As imaging cannot differentiate between benign and malignant lesions, a definitive diagnosis is made only by histopathology and immunohistochemistry.Individuals diagnosed with littoral cell angioma must be carefully evaluated to exclude associated primary, secondary and synchronous malignancies as well as accompanying inflammatory/autoimmune disease.

Aberrant keratin expression is common in primary hepatic malignant vascular tumors: A potential diagnostic pitfall

Malignant vascular neoplasms such as epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS) can arise within the liver. The aim of this study was to study the expression of keratins CK7, AE1/AE3 and OSCAR in primary hepatic EHE and AS. 9 cases of hepatic EHE and 13 cases of hepatic AS were stained with ERG, CK7, keratin AE1/AE3 and keratin OSCAR. Their expression was graded as 1+ (1–25% of tumor cells positive), 2+ (26–50%), 3+ (51–75%) or 4+ (>75%). ERG was positive in all 9 (100%) EHEs and all 13 (100%) ASs. CK7 was positive in 5/9 (56%) EHEs (2, 1+; 1, 2+; 1, 3+; 1, 4+) and 1/13 (8%) AS (2+). Keratin OSCAR was positive in 6/9 (67%) EHEs (5, 1+; 1, 2+) and 4/13 (31%) ASs (2, 1+; 1, 2+; 1, 4+). Keratin AE1/AE3 was positive in 6/9 (67%) EHEs (3, 1+; 3; 2+) and 4/13 (31%) ASs (2, 1+; 1, 2+; 1, 4+). Overall, 6/ 9 (67%) EHEs were positive for at least one keratin marker, of which 5 were positive for all 3 keratins (AE1/AE3, OSCAR and CK7) while 1 was positive only for 2 keratins (OSCAR and AE1/AE3). 4/13 (31%) of ASs were positive for both keratins OSCAR and AE1/AE3, of which 1 case was also positive for CK7. Aberrant keratin expression is common in primary hepatic EHEs (67%) and ASs (31%). Awareness of this diagnostic pitfall is important for avoiding misdiagnosis of these primary hepatic malignant vascular tumors as carcinomas.

Primary hepatic malignant vascular tumors: a follow-up study of imaging characteristics and clinicopathological features

BackgroundOwing to its low incidence, there is insufficient clinical awareness and diagnostic experience with primary hepatic malignant vascular tumors (PHMVTs). The aim of our study was to investigate the imaging and clinicopathological features of patients with PHMVTs and analyze the clinicopathological correlations.MethodsWe retrospectively analyzed 42 patients who had pathologically confirmed PHMVT during the period from June 2012 to December 2019 and enrolled them in our study. The computed tomography (CT) and magnetic resonance (MR) images and pathological findings of each patient were recorded.ResultsThere were more female (29/42) than male patients. The imaging features of primary hepatic angiosarcoma (PHA) (n = 11) included ill-defined margins (11/11, 100%), necrosis (5/11, 45%), calcification (3/11, 27%) and “slow in-slow out” centripetal enhancement (7/11, 64%). Patients with epithelioid hemangioendothelioma (EHE) (n = 15) presented with ill-defined margins (15/15, 100%), necrosis (6/15, 40%), calcification (2/15, 13%), “fast in-slow out” centripetal enhancement (10/15, 67%), halo sign (15/15, 100%), pseudocapsule sign (4/15, 27%), lollipop sign (2/15, 13%) and capsule retraction sign (2/15, 13%). Patients with malignant hemangiopericytoma (MHP) (n = 3) showed ill-defined margins (3/3, 100%), necrosis (3/3, 100%) and “fast in-slow out” progressive enhancement (3/3, 100%). Infantile hemangioendotheliomas (IHEs) (n = 13) were defined by ill-defined margins (7/13, 54%), necrosis (8/13, 62%), calcification (5/13, 38%) and “fast in-slow out” centripetal enhancement (13/13, 100%). Immunohistochemistry showed strong positive expression of CD31, CD34, ERG, FaVIII and FLI-1. Patients with IHE (96 months) and EHE (88 months) had the longest survival times, followed by those with MHP (23 months), while patients with PHA (15 months) had the shortest survival time.ConclusionOn CT and MR images, most PHMVTs were ill-defined, heterogeneous, hypervascular masses with centripetal progressive enhancement and possibly calcification, especially in female patients. The prognosis of patients with PHMVT was associated with the pathological type of the tumor.

Vascular Neoplasms and Related Lesions of Kidney

Vascular tumors arising in the kidney are distinctly uncommon occurrences and have not been well-characterized in the literature; most cases have been documented in single reports or small case series. These tumors often exhibit nonspecific clinical and radiologic features and may mimic malignant epithelial tumors such as renal cell carcinoma (RCC) on imaging studies, necessitating nephrectomy with pathologic examination for definitive diagnosis. In addition, since primary renal vascular tumors are rare and infrequently encountered in surgical pathology specimens, they are often not considered in the differential diagnosis and therefore may be systematically under-diagnosed. In this review, we discuss the clinical and morphologic features of benign, borderline, and malignant vascular neoplasms and related lesions arising in the kidney, with a focus on recent, clinically relevant advances in imaging, histopathologic diagnosis, and treatment.

Posttraumatic intraosseous epidermoid inclusion cyst. A case report and short literature review

Abstract Epidermoid bone inclusion cysts are pathological structures bordered by a layer of stratified squamous epithelium containing keratin debris. They are also called epidermal bone cysts. Intraosseous epidermoid cysts are rare entities; these lesions may occur as a result of trauma (puncture wound or pressure erosion). They appear to be well defined, translucent lytic lesions with sclerotic margins on imaging studies. The most frequent sites affected are: the distal phalanx and the calvaria - intradiploic, but a few cases of larger bone involvement have been reported. The imagistic differential diagnosis of this lesion includes intraosseous extension of a glomus tumor, enchondroma, osteoid osteoma, acral metastases, some primary vascular bone tumors, giant cell reparative granuloma, subungual nodular melanoma or other lytic non-neoplastic lesions such as osteomyelitis. We present the case of a 70-year-old man experiencing anxiety and pain due to distal phalanx expansion of the IV digit, following hand trauma. An X-ray was performed, showing a radiotransparent lesion located in the distal phalanx. An intramedullary inclusion cyst must be taken into account as a clinical differential diagnosis. Due to the extensive destruction of the distal phalanx, amputation was necessary. Gross examination of the surgical specimen revealed a cystic lesion that contained whitish, creamy material with extensive necrotic areas. Microscopic examination revealed a ruptured intraosseous epidermoid inclusion cyst, which was successfully excised.

Impact of tumor histology and socioeconomic factors on survival of patients suffering from malignant vascular tumors of liver and hepatocellular carcinomas: A SEER database analysis.

e16612 Background: Primary malignant vascular tumors of the liver are rare, aggressive and poorly understood subtypes of liver cancers. In this analysis, we aim to determine the impact of tumor histology and other socio economic factors on survival of these tumors and hepatocellular carcinomas. Methods: Patients with malignant histopathological diagnoses of hepatocellular carcinoma not otherwise specified (HCC NOS), hepatocellular carcinoma with spindle cell (HCC SP), fibrolamellar (HCC F), clear cell (HCC CL), scirrhous (HCC SC) and pleomorphic variants ( HCC PL), combined hepatocellular carcinoma and cholangiocarcinoma (Mixed), hepatic angiosarcoma (AS), hemangioendothelioma (HE), its epithelioid variant (EHE) and hemangiopericytoma (HP) were identified using the SEER (version 2018) database. Overall survival was studied with Kaplan–Meier with the log rank method. Multivariable analysis was performed to assess the impact of race, ethnicity, marital and insurance status on survival of these patients. Results: We analyzed de-identified data of 104502 patients from the year 1973 to 2016 with hepatocellular carcinomas and malignant vascular tumors of liver (including 101851 patients with HCCNOS, 70 with HCC SP, 378 with HCC F, 104 with HCC SC, 593 with HCC CL, 23 with HCC PL, 950 with Mixed, 367 with AS, 36 with HE, 120 with EHE and 10 with HP ) respectively. Median overall survival (OS) of HCC NOS was calculated to be 7 months. Among hepatocellular carcinoma patients HCC F had the longest median OS of 29 months and HCC SP had the shortest median OS of 3months (P < 0.001). Additionally, among patients with malignant vascular tumors, AS had the shortest median OS (1 month) while patients with EHE had the longest median OS of 81 months (P < 0.001). Overall, married and insured patients had a better overall survival than unmarried and uninsured patients (P < 0.04). Conclusions: Malignant vascular tumors of liver are rare in comparison to hepatocellular carcinomas. Spindle cell variant of HCC and liver angiosarcomas carry the worst prognosis and fibrolamellar variant of HCC, hemangioendothelioma (HE) and its epithelioid variant (EHE) have the best prognosis. Insurance and marital status has a positive impact on overall survival of liver cancer patients.

Prognostic Determinants Analysis and Nomogram for Bone Malignant Vascular Tumors: A Surveillance, Epidemiology and End Results (SEER) Analysis.

BackgroundThe aim and objective of our investigations were to explore the prognostic value of various clinical and pathological factors of bone malignant vascular tumors and establish a nomogram for their outcome predictions.Material/MethodsAll data of primary bone malignant vascular tumors (MVTs) patients were randomly selected from the Surveillance, Epidemiology and End Results (SEER) database. However, selected patients were clinically diagnosed with various cancers during 1988–2015. The potential prognostic factors were analyzed using SPSS (Windows, version 22.0). All prognostic factors were combined to formulate a nomogram to predict the overall survival (OS).ResultsA total of 266 selected patients were included in our study. In the univariate model, age (P<0.001), sex (p=0.0255), primary site (P<0.001), surgery (P<0.001), histologic type (P<0.001), metastasis (p=0.000), and pathological grade (P<0.001) were statistically significant for patient survival. The results of Cox analysis revealed that age (≥64) HR: 3.636, 95% CI [1.955–6.762], p=0.000, the primary site in skull HR: 2.6, 95% CI [1.584–4.268], p<0.001], without surgery HR: 1.473 95% CI [1.239–1.751], p<0.001, metastasis HR: 3.076 95% CI [1.983–4.771] p=0.000, man HR 1.802, 95% CI [1.032–3.004], p=0.045, and high malignant grade HR: 3.029, 95% CI [2.101–4.366], p=0.003 were independent unfavorable prognostic factors. Angiosarcoma had highest mortality rate among all vascular malignancies. The nomogram predicting overall survival achieved a C-index of 0.694 (95% CI 0.631, 0.745) in the SEER cohort.ConclusionsSurgery can significantly increase OS survival time for bone MVTs, and low-grade malignancy is a significant factor for OS. However, advanced age, tumor metastasis, primary site in skull, AS, and male sex are predictors of poor prognosis.

Primary Vascular Tumors of Bone: A Monoinstitutional Morphologic and Molecular Analysis of 427 Cases With Emphasis on Epithelioid Variants.

Recent molecular discoveries have refined vascular bone tumor classification. To investigate the clinical relevance of these refinements, we reviewed all cases of primary vascular bone tumors treated at our Institute. On the basis of morphology, cases were assessed immunohistochemically and molecularly. A total of 427 cases of primary vascular tumor of bone with available follow-up and histologic material were retrieved and reclassified according to the most recent diagnostic criteria as follows: 289 hemangiomas, 38 epithelioid hemangiomas, 21 epithelioid hemangioendotheliomas, 2 retiform hemangioendotheliomas, 1 intraosseous papillary intralymphatic angioendothelioma, 24 pseudomyogenic hemangioendotheliomas, and 52 angiosarcomas (of these, 45 were epithelioid angiosarcomas and 7 spindle cell secondary angiosarcoma). Both epithelioid and classic hemangiomas behave as benign tumors with excellent prognosis. The distinction between cellular and conventional type of epithelioid hemangioma was not associated with a different clinical course. Conversely, epithelioid hemangioendothelioma exhibited a more aggressive clinical behavior than hemangioma, with higher rates of multifocality and distant spread. Immunohistochemical positivity for CAMTA1 or TFE3 did not have a prognostic implication. In epithelioid hemangioendothelioma, the presence of morphologic malignant features was associated with reduced disease-free (P=0.064) and overall survival (P=0.055). Pseudomyogenic hemangioendothelioma featured local aggressiveness in 5/24 patients exhibiting a clinical behavior closer to epithelioid hemangioma than epithelioid hemangioendothelioma. Last, 32/45 patients with epithelioid angiosarcoma died of disease with a median survival time of 10 months from diagnosis. In conclusion, the integration of morphologic, immunohistochemical, and molecular features allows a better stratification of primary vascular tumors of bone with significant prognostic and therapeutic implications.

Malignant hepatic vascular tumors in adults: Characteristics, diagnostic difficulties and current management.

Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation (LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma (HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation Association-European Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma (HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient. Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches, alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.

Primary vascular bone tumors in the spine: a challenge for pathologists and spine oncology surgeons

PurposePrimary vascular bone tumors of the spine represent a challenge for oncology surgeons, due to the need of planning a surgical strategy appropriate to the tumor behavior. But these tumors represent a challenge also for pathologists, as immunohistochemical and molecular analyses have recently refined the terminology.MethodsA cohort of 81 cases was retrospectively reviewed, targeting the evolution of diagnoses and the treatment-related outcome. Sixty-six cases (including one case originally diagnosed as chordoma) were diagnosed before 2011, and 15 were diagnosed and treated in the period 2011–2017. Fully documented outcome studies are available for 46 patients whose immunohistochemical and molecular analyses were available. The follow-up ranges from 6 months of the early patient death to 300 months of the longest disease-free survival.ResultsThe outcome was related not only to the treatment performed, but also to the evolution of diagnoses. The term Hemangioendothelioma that defined a benign aggressive (Enneking stage 3) tumor is now obsolete and replaced by the diagnosis of Epithelioid Hemangioma for benign aggressive tumor and Epithelioid Hemangioendothelioma for low-grade malignant tumor. En bloc resection was appropriate for local control, but 2 out of 7 cases of epithelioid Hemangioendotheliomas died due to the spread of the disease None of the Hemangiomas recurred after intralesional excision with or without radiotherapy, or after vertebroplasty. Both cases of Angiosarcoma had a fast and lethal evolution.ConclusionsSurgeons must be aware of the evolution of terminology to decide the most appropriate treatment options.Graphical abstractThese slides can be retrieved from Electronic Supplementary Material.

Bone: Vascular Tumors

Note Benign vascular lesions of bone are relatively common and occur most frequent as an asymptomatic incidental finding in the skull or spine. Primary malignant vascular tumors of bone are rare. They represent less than 1% of primary malignant bone tumors reported by the Netherlands Committee on Bone Tumors and 0,5% of those registered at the Mayo Clinic. Clinically they are extremely aggressive and have a very poor prognosis. Survival rates are unknown, but in those cases reported patients die very soon after diagnosis due to wide spread metastasis and lack of tailored therapy.

Unicentric epithelioid hemangioendothelioma of the calcaneus: a case report and review of literature

BackgroundThis review of the literature combined with a clinical case will allow the illustration of a favorable outcome of this variable low grade malignancy, display a role for limb salvage surgery with intralesional treatment, and offer a clinical example of epithelioid hemangioendothelioma, a rare malignancy.Case presentationThe case report presents a case of solitary epithelioid hemangioendothelioma (EHE) of the calcaneus in a 60-year-old male. Primary vascular tumors of the bone are rare; however, EHE is one of the most common primary malignant vascular tumors to occur in bone. A review of the literature found few cases that involved the calcaneus; those cases found that involved the calcaneus were either part of a multifocal or metastatic disease process. Our case presents a 45-month clinical follow-up of solitary EHE in the calcaneus treated with surgical excision by curettage and cementing.ConclusionThis case has clinical follow-up greater than 2 years post-operatively and could be a guide for treatment of a rare disorder with a substantial paucity of literature.