Hemangioendothelioma Research Articles

Platelet functional abnormalities in pediatric patients with kaposiform hemangioendothelioma/Kasabach-Merritt phenomenon.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy that is commonly associated with a life-threatening thrombocytopenic condition, Kasabach-Merritt phenomenon (KMP). Platelet CLEC-2, tumor podoplanin interaction is considered the key mechanism of platelet clearance in these patients. Here, we aimed to assess platelet functionality in such patients. Three groups of 6 to 9 children were enrolled: group A with KHE/KMP without hematologic response (HR) to therapy; group B with KHE/KMP with HR; and group C with healthy children. Platelet functionality was assessed by continuous and end point flow cytometry, low-angle light scattering analysis (LaSca), fluorescent microscopy of blood smears, and exvivo thrombi formation. Platelet integrin activation in response to a combination of CRP (GPVI agonist) and TRAP-6 (PAR1 agonist), as well as calcium mobilization and integrin activation in response to CRP or rhodocytin (CLEC-2 agonist) alone, were significantly diminished in groups A and B. At the same time, platelet responses to ADP with or without TRAP-6 were unaltered. Thrombi formation from collagen in parallel plate flow chambers was also noticeably decreased in groups A and B. In silico analysis of these results predicted diminished amounts of CLEC-2 on the platelet surface of patients, which was further confirmed by immunofluorescence microscopy and flow cytometry. In addition, we also noted a decrease in GPVI levels on platelets from group A. In KHE/KMP, platelet responses induced by CLEC-2 or GPVI activation are impaired because of the diminished number of receptors on the platelet surface. This impairment correlates with the severity of the disease and resolves as the patient recovers.

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

BackgroundComposite 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 presentationThe 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.ConclusionsCHE 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.

Recurrence of composite hemangioendothelioma of the kidney after surgical resection

Composite hemangioendothelioma is an extremely rare form of kidney tumor. The tumor mainly occurs in the extremities, head and neck; internal organs involvement is rarely reported. Patient, 61-year-old male, was admitted to the urology department for a left kidney tumor, which was found accidentally during an ultrasound examination. Magnetic resonance imaging showed an irregularly shaped tumor measuring 5.0 × 6.0 × 4.0 cm and located in the lower pole of the left kidney. The tumor was surgically removed with resection of the capsule of the kidney lower pole. Immunohistochemical study revealed diffuse bright expression of CD31 (clone JC70A), CD34 (clone QBEnd 10), ERG (clone ER111), FLI-1 (clone MRQ-1) in tumor cells. The index of proliferative activity Ki-67 (clone SP6) was 40 %. The morphological picture and immunophenotype of the tumor correspond to composite hemangioendothelioma of the retroperitoneal space. Magnetic resonance imaging of the retroperitoneal space on follow-up visit in 9 months visualized a tumor of the left kidney measuring 8.3 × 8.4 × 7.8 cm. Radical nephrectomy was performed. Pathology examination showed that tumor tissue was mainly represented by solid fields of the spindle cell component. In samples of the border between the tumor and fatty pararenal tissue, tumor invasion was observed up to the adjacent striated muscles, tumor growth into the tissue of the kidney gate was also found. Taking into account the morphological picture and the earlier immunohistochemical study, the removed tumor corresponds to composite hemangioendothelioma.Composite hemangioendothelioma is a tumor of low malignant potential. It is extremely rare for this tumor to affect the kidney. At the same time, in the described case, the tumor was initially located in the retroperitoneal space, with involvement of the kidney capsule, and was assessed as a benign lesion. After 9 months, there was a recurrence of the tumor localized in the kidney with damage to the elements of the renal sinus, retroperitoneal tissue, and lumbar muscles. In this case, the tumor has significant malignant potential.

Incidence, demographics, and survival of malignant hemangioendothelioma in the United States.

Malignant hemangioendothelioma is an endothelial cancer with heterogeneous clinical behavior that can range from indolent to aggressive, of which the majority are epithelioid (EHE). Its incidence and demographics have not been previously well defined in a large cohort. This retrospective analysis used the US Cancer Statistics National Program of Cancer Registries - Surveillance Epidemiology End Results (SEER) combined database to identify patients in the US newly diagnosed with hemangioendothelioma between the years of 2001 and 2017 (n = 1986). Survival analyses were performed on a subset of patients within the SEER-18 database with survival information available (n = 417). Outcomes included incidence, demographics of patients newly diagnosed with hemangioendothelioma, extent of disease at presentation, and overall survival. The incidence of hemangioendothelioma in the US is 0.4 cases per million person-years. Although cases rose to 122 newly diagnosed in the year 2017 (90 EHE, 32 other hemangioendothelioma), incidence rates were stable. Skin and connective tissues were the most common presenting sites (33.4%), followed by liver (24.5%), lung (17.6%), and bone (12.5%). Median age at diagnosis was 55 years; 27.2% of patients were pediatric, adolescent, or young adult (<40 years). At presentation, 36.4% of patients had localized disease; 21.6% presented with regional and 41.7% with distant metastases. Observed survival at 3 years was 79.7%, 70.7%, and 46.0% for patients presenting with local, regional, and distant disease and most deaths occurred within the first 2 years. Malignant hemangioendothelioma is ultra-rare but meaningfully impacts affected patients. These data may provide benchmarks for comparison of new approaches to hemangioendothelioma therapy and highlight poor survival outcomes.

Impact of age and tumor size on the development of the Kasabach-Merritt phenomenon in patients with kaposiform hemangioendothelioma: a retrospective cohort study.

The Kasabach-Merritt phenomenon (KMP) is a severe complication of kaposiform hemangioendothelioma (KHE). The risk factors for KMP need further investigation. The medical records of patients with KHE were reviewed. Univariate and multivariate logistic regression models were used for the risk factors for KMP, and the area under the receiver operator characteristic (ROC) curve was used to assess the predictive power of risk factors. A total of 338 patients with KHE were enrolled. The incidence of KMP was 45.9%. Age of onset (P<0.001, odds ratio [OR] 0.939; 95% confidence interval [CI] 0.914-0.966), lesion size (P<0.001, OR 1.944; 95% CI 1.646-2.296), mixed type (P=0.030, OR 2.428; 95% CI 1.092-5.397), deep type (P=0.010, OR 4.006; 95% CI 1.389-11.556), and mediastinal or retroperitoneal lesion location (P=0.019, OR 11.864; 95% CI 1.497-94.003) were correlated with KMP occurrence through multivariate logistic regression. ROC curve analysis revealed that the optimal cutoffs were 4.75 months for the age of onset (P<0.001, OR 7.206, 95% CI 4.073-12.749) and a lesion diameter of 5.35 cm (P<0.001, OR 11.817, 95% CI 7.084-19.714). Bounded by a lesion size of 5.35 cm, we found significant differences in tumor morphology, age of onset, treatments, and hematological parameters. Using an onset age of 4.75 months as a cutoff, we found significant differences in tumor morphology, lesion size, hematological parameters, and prognosis. For KHE patients with an onset age<4.75 months and/or lesion diameter>5.35 cm, clinicians should be wary of the occurrence of KMP. Active management is recommended to improve the prognosis.

Prenatal detection of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon: a case report

BackgroundKaposiform Hemangioendothelioma is a rare vascular neoplasm that typically presents in neonates and infancy. Most of the cases of Kaposiform Hemangioendothelioma are reported in neonates (60%) and infancy (93%), and very few cases have been reported prenatally. We describe here a case which was diagnosed prenatally in the 3rd trimester with rapid growth of upper limb and appearance of non-immune hydrops.Case presentationA 25-year-old primigravida, an antenatally booked case, presented for routine third trimester level III scan at 34-week gestation. Level I scan for nasal bone and nuchal translucency at 12 weeks and level II anomaly scan at 20 weeks were normal. Level III ultrasound scan revealed increased echogenicity and hypertrophy of soft tissues of right upper limb and trunk involving both the subcutaneous and muscular compartments. Fetal middle cerebral artery Doppler, echocardiography and magnetic resonance imaging revealed fetal anemia, cardiac failure, and non-immune hydrops. These findings with hemodynamic changes and rapid evolution were suggestive of Kaposiform Hemangioendothelioma with Kasabach–Merritt phenomenon.ConclusionThe diagnosis of Kaposiform Hemangioendothelioma should be suspected in prenatal period if there is unilateral hypertrophy of limb which has evolved rapidly over a short interval of time and shows findings suggestive of Kasabach–Merritt Phenomenon. Prenatal presentation is life threatening if it is associated with poor fetal hemodynamics. Prenatal counseling in such cases should include advice on termination of pregnancy with emphasis on neonatal management.

Infantile Hämangiome

With an incidence of approximately 4% infantile hemangiomas are the most common vascular tumors in children and show characteristic growth dynamics. In order to avoid erroneous treatment, they need to be differentiated from other vascular tumors (granuloma pyogenicum and kaposiform hemangioendothelioma) and vascular malformations. Of all infantile hemangiomas 85% are uncomplicated and undergo spontaneous resolution starting towards the end of the first year of life. First-line treatment for complicated infantile hemangiomas (15%), i.e. those with imminent obstruction (eyes and nose), ulceration or permanent disfigurement, is oral propranolol (2 mg/kg BW and day for at least 6months).

Cutaneous vascular tumors: an updated review

Cutaneous vascular tumors constitute a heterogeneous group of entities that share overlapping morphologic and immunohistochemical features, which can be diagnostically challenging for pathologists and dermatopathologists. Our understanding and knowledge of vascular neoplasms have improved over time, resulting in both a refinement of their classification by the International Society for the Study of Vascular Anomalies (ISSVA) and an improvement in the accurate diagnosis and clinical management of vascular neoplasms. This review article aims to summarize the updated clinical, histopathological, and immunohistochemical characteristics of cutaneous vascular tumors, as well as to highlight their associated genetic mutations. Such entities include infantile hemangioma, congenital hemangioma, tufted angioma, spindle cell hemangioma, epithelioid hemangioma, pyogenic granuloma, Kaposiform hemangioendothelioma, retiform hemangioendothelioma, pseudomyogenic hemangioendothelioma, Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma.

Clinical and imaging features of Kaposiform hemangioendothelioma in infants

ObjectiveKaposiform hemangioendothelioma (KHE) is a locally aggressive tumor of vascular origin. This study investigated the clinical and imaging features of KHE to provide a reference for its early diagnosis. MethodsThe clinical and imaging findings of 27 clinically confirmed KHE cases (including 21 with focal lesions and 6 with diffuse lesions) between January 2016 and December 2021 were retrospectively analyzed. ResultsThe mean age of the 27 patients was 105 ± 80.27 days. Twenty-two (81.5%) of these patients had Kasabach–Merritt phenomenon. Most KHEs were located in the trunk and/or extremities (22/27). Ultrasonography showed heterogeneous echogenicity and/or striated hypoechoic bands with abundant or patchy blood flow within the tumor. On plain computed tomography (CT), they appeared as heterogeneous lesions isodense with the muscles, with a CT value of 29.58 ± 11.53 HU. In the arterial phase, the KHEs showed striated or lamellar heterogeneous enhancement, with a CT value of 153.91 ± 52.11 HU after enhancement. All KHEs showed uneven and high signal intensity on T2-weighted imaging, mixed high and low signal intensity on fat-saturated images, and no significant diffusion restriction on diffusion-weighted imaging. ConclusionKHEs can occur in various locations and present as highly infiltrative and heterogeneous masses that can invade the skin, adjacent muscles, and bones. A vascularized mass with purpuric skin changes, with uneven and high T2WI signal is highly suggestive of the diagnosis of KHE.

Retiform hemangioendothelioma of the breast in a man with 18F-flurodeoxyglucose accumulation on positron emission tomography: a case report

BackgroundRetiform hemangioendothelioma (RH) is a rare, intermediate-grade vascular tumor that often arises in the trunk and extremities. The clinical and radiological features of RH remain largely unknown.Case presentationA male patient in his 70s presented with shortness of breath on exertion, and computed tomography incidentally revealed a tumor in his right breast. Positron emission tomography (PET) revealed moderate 18F-fluorodeoxyglucose (FDG) uptake in the tumor. RH was observed in the resected specimens. Three months after surgery, the patient was free of local recurrence and distant metastasis.ConclusionsRH was found in the male breast and was accompanied by FDG uptake on PET. PET may be useful in diagnosing RH. Although metastasis is rare in RH, local recurrence may occur, and careful follow-up is required.

CT appearances and classification of hepatic epithelioid hemangioendothelioma

BackgroundHepatic epithelioid hemangioendothelioma (HEH) is extremely rare, and CT features have never been analyzed in a large group of patients.MethodsA retrospective study was designed to review the contrast-enhanced CT images of HEH patients. Intrahepatic lesions were categorized into three types: nodular, locally coalescent (coalescent lesion contained in one segment) or diffusely coalescent (coalescent lesion occupied more than one segment). CT features were compared among lesions of different sizes and patients with different lesion types.ResultsA total of 93 HEH patients were included in this study, and 740 lesions were analyzed. The results of per-lesion analysis showed that medium lesions (2–5 cm) had the highest rate of lollipop sign (16.8%) and target-like enhancement (43.1%), while lesions in large group (> 5 cm) had the highest rate of capsular retraction (38.8%) and vascular invasion (38.8%). The differences on enhancement pattern and the rates of lollipop sign and capsular retraction were significant among lesions of different sizes (p < 0.001, respectively). The results of per-patient analysis showed that patients in locally coalescent group had the highest rates of lollipop sign (74.3%) and target sign (94.3%). All patients in diffusely coalescent group had capsular retraction and vascular invasion. CT appearances of capsular retraction, lollipop sign, target sign and vascular invasion differed significantly among patients with different lesion types (p < 0.001, p = 0.005, p = 0.006 and p < 0.001, respectively).ConclusionCT features variated among HEH patients with different lesion types, and radiological appearances of HEH should be classified into nodular type, locally coalescent type and diffusely coalescent type.

Long-term outcomes of sirolimus treatment for kaposiform hemangioendothelioma: Continuing successes and ongoing challenges.

Treatment with sirolimus, an inhibitor of the mammalian target of rapamycin pathway, has improved the prognosis of patients with kaposiform hemangioendothelioma (KHE). However, the efficacy, durability and tolerability of long-term sirolimus treatment in patients with KHE have not been well elucidated. We performed efficacy and safety assessments based on more than 4.5 years of follow-up in patients receiving sirolimus therapy for KHE. One hundred sixty-seven patients were analyzed, including 102 (61.1%) patients with the Kasabach-Merritt phenomenon (KMP). Follow-up was conducted after a median of 56.0months. A total of 154 (92.2%) patients had a durable response to sirolimus treatment. No difference in durable response was found between patients without KMP and patients with KMP (95.4% vs 90.2%; difference, 5.2%; 95% confidence interval [CI], -4.0% to 13.1%). Rebound growth occurred in 17.3% of patients upon sirolimus discontinuation. Early treatment discontinuation (odds ratio [OR]: 3.103; 95% CI: 1.529-6.299; P=.002) and mixed lesion type (OR: 2.271; 95% CI: 0.901-5.727; P=.047) were associated with tumor rebound growth. No KHE-related deaths occurred in this cohort. At the last follow-up, approximately 17.4% of patients had active disease and/or changes in body structures to a variable extent. Serious adverse events occurred most commonly during the first year of sirolimus therapy. Follow-up of almost 4.5 years demonstrated that the efficacy of sirolimus persisted over time and that long-term treatment with sirolimus was not associated with unacceptable cumulative toxicities. However, nonresponse, tumor relapse and long-term sequelae remained challenges despite intensified and prolonged sirolimus therapy.

A neonate with Kaposiform haemangioendothelioma, complicated with Kasabach-Merritt phenomenon

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.

Successful Treatment of a Malignant Hemangioendothelioma of the Thyroid with Metastasis to the Lung. A Case Report

Successful Treatment of a Malignant Hemangioendothelioma of the Thyroid with Metastasis to the Lung. A Case Report

Prevalence and clinical features of orbital vascular anomalies in children

ABSTRACT Purpose To report the prevalence, clinical characteristics, and natural history of orbital vascular anomalies diagnosed among children over a 50-year period. Methods The medical records of all patients <19 years diagnosed with any form of an orbital vascular anomaly (OVA) at Mayo Clinic, Rochester, Minnesota from January 1 1966, through December 31 2015, were retrospectively reviewed. Results A total of 109 children were diagnosed with OVA during the 50-year period, of which 24 were from Olmsted County, MN, yielding a birth prevalence of 1 in 4,305 live births. The median age at diagnosis for the 109 patients was 1.2 years (range, 0–17.9 years) and 67 (61.5%) were female. Common presenting symptoms included proptosis in 80 (73.4%) patients, abnormalities in skin color in 45 (41.3%) patients, and pain in 18 (16.5%) patients. There were 55 (50.5%) vascular malformations [50 (91%) low-flow lymphatic malformations, 3 (5.5%) high-flow arteriovenous malformations, and 2 (3.5%) low-flow venous malformations] and 54 (49.5%) vascular tumors [53 (98%) capillary hemangiomas and 1 (2%) kaposiform hemangioendothelioma]. During a mean follow-up of 5.95 years (range 0–27.7 years), amblyopia and/or strabismus were diagnosed in 46 (43.4%) patients. Conclusions Capillary hemangiomas and low-flow lymphatic malformations comprise most of the orbital vascular anomalies in this cohort of children. Amblyopia and strabismus are common sequelae, highlighting the importance of early diagnosis and appropriate management.

Multiply Recurrent Composite Hemangioendothelioma of Penis with Histologic Progression to High-Grade Features

Composite hemangioendothelioma (CHE) is a very rare low-grade malignant vascular neoplasm. Here, we present the first case of it occurring on a penis with two local recurrences over a 9 year span and its progression to a high-grade morphology.

Malignant undifferentiated epithelioid neoplasms with MAML2 rearrangements: A clinicopathologic study of seven cases demonstrating a heterogenous entity.

Among mesenchymal tumors, MAML2 gene rearrangements have been described in a subset of composite hemangioendothelioma and myxoinflammatory fibroblastic sarcoma (MIFS). However, we have recently encountered MAML2-related fusions in a group of seven undifferentiated malignant epithelioid neoplasms that do not fit well to any established pathologic entities. The patients included five males and two female, aged 41-71 years old (median 65 years). The tumors involved the deep soft tissue of extremities (hip, knee, arm, hand), abdominal wall, and the retroperitoneum. Microscopically, the tumors consisted of solid sheets of atypical epithelioid to histiocytoid cells with abundant cytoplasm. Prominent mitotic activity and necrosis were present in 4 cases. In 3 cases, the cells displayed hyperchromatic nuclei or conspicuous macronucleoli, and were admixed with background histiocytoid cells and a lymphoplasmacytic infiltrate. By immunohistochemistry (IHC), the neoplastic cells had a nonspecific phenotype. On targeted RNA sequencing, MAML2 was the 3' partner and fused to YAP1 (4 cases), ARHGAP42 (2 cases), and ENDOD1 (1 case). Two cases with YAP1::MAML2 harbored concurrent RAF kinase fusions (RBMS3::RAF1 and AGK::BRAF, respectively). In 2 cases with targeted DNA sequencing, mutations in TP53, RB1 and PTEN were detected in 1 case, and PDGFRB mutations, CCNE1 amplifications and CDKN2A/2B deletion were detected in another case, which showed strong and diffuse PDGFRB expression by IHC. Of the 4 cases with detailed clinical history (median follow-up period 8months), three developed distant metastatic disease (one of which died of disease); one case remained free of disease 3 years following surgical excision. In conclusion, we describe a heterogeneous series of MAML2-rearranged undifferentiated malignant epithelioid neoplasms, a subset of which may overlap with a recently described MIFS variant with YAP1::MAML2 fusions, further expanding the clinicopathologic spectrum of mesenchymal neoplasms with recurrent MAML2 gene rearrangements.

Hemangioendotelioma Kapasiforme mediastinal complicado com síndrome de Kasabach-Merrit: relato de caso

OBJECTIVES: This paper reports the case of a child diagnosed with mediastinal kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt Syndrome at a referral hospital in Salvador, Bahia, Brazil. CASE REPORT: A two-month-old with a history of cyanosis and nasal congestion had an echocardiogram with findings consistent with pericardial effusion. The patient underwent multiple pericardial drainage procedures and was eventually sent to a pediatric referral center in the city of Salvador. Contrast-enhanced computed tomography images showed a mediastinal mass, which was resected. Postoperative pathology revealed the patient had a KHE. He was also suspected with Kasabach-Merritt syndrome due to consumption coagulopathy. Corticoid therapy and chemotherapy with vincristine were performed. The patient progressed well and was discharged and placed on outpatient follow-up, without recurrence of symptoms. COMMENTS: KHE is rare and aggressive, although with a benign histopathology. It can complicate with consumption coagulopathy, characteristically seen in Kasabach-Merritt syndrome.

Birthmarks and Vascular Anomalies in Children: Classification, Diagnosis and Management

Birthmarks are a common presentation among newborns and the majority do not require any medical intervention. However, vascular anomalies, which are an umbrella of diverse pathological conditions require special attention for a multitude of factors. Vascular anomalies lesions can appear at any stage of development, including prenatal, perinatal, and childhood. They are broadly classified as vascular malformations and vascular tumours. While many lesions have a straightforward natural course, more complex, extensive, or progressive lesions can pose a threat to life due to mass effects and severe complications including haemorrhage, or high-volume arteriovenous shunting. Infantile haemangioma, congenital haemangioma, and kaposiform hemangioendothelioma are the sub-classifications of vascular tumours, with infantile haemangioma being the most prevalent. Depending on the nature, location, extent, and accompanying manifestations of the tumour, there are several management options for vascular tumours, including conservative methods, pharmacological therapy, and surgical intervention. Low-flow and high-flow lesions are two subcategories of vascular malformations. Capillary, venous, and lymphatic malformations are examples of low-flow lesions, while arteriovenous malformations, a highly varied set of lesions that can present in a number of ways, are classified under high-flow lesions. Endovascular or surgical obliteration is the basis of treatment for these dynamic lesions. The purpose of this research is to review the available information about birthmarks and vascular anomalies in children; classification, diagnosis, and management.

A Case Study of De-Staging and Reverse Transformation of a IDH Mutated Malignant Metastatic Sarcoma to Benign Tumor and Complete Clinical Remission by Application of Multi Targeted Epigenetic Therapies

The evolution of spindle cell hemangiomas (SCH) to hemangiosarcomas is a phenomenon where although rare can occur. The fate of disease will certainly depend on its molecular behavior and as such clinical manifestation of the disease will depend on this transformation.