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Home , Angiosarcoma, Cavernous hemangioma, Hemangioma, Hemangiosarcoma

- - - - - 2,3 Surgery is the treatment of choice for non- 2,3,7 1,3 Physical examination revealed an ECOG-Performance A A healthy 46-year-old female presented with a severe weight loss, jaundice or abdominal distension. There was no medication history of oral contraceptive She pills, consumption. tobacco or anabolic alcohol no as well as , was not exposed to any known . exhibit rapid progression and poor prognosis (median overall (median prognosis poor and progression rapid exhibit with months 12 and treatment without months six of survival treatment). and reserve hepatic adequate is there if disease metastatic limited to one lobe. It cannot be performed in disease. metastatic CASE PRESENTATION fatigue, without months, two over for pain quadrant upper right , Francisco Castro e SOUSA , Francisco Castro e 1 750 Copyright © Ordem dos Médicos 2017 https://doi.org/10.20344/amp.8593

▪ , Helena GERVÁSIO 1 Hepatic angiosarcomas Hepatic 3,6 R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m , João RIBEIRO 1 are more frequent in male patients  Clinical manifestations are nonspecific, Cavernoso/diagnóstico; Hemangiossarcoma/diagnóstico; Neoplasias Hepáticas/diagnóstico Hemangioma Cavernoso/diagnóstico; Hemangiossarcoma/diagnóstico; 1-4 5 Hemangioma, Cavernous/diagnosis; /diagnosis; /diagnosis Hemangioma, Cavernous/diagnosis; Hemangiosarcoma/diagnosis; Nevertheless, it is the most common malignant 1,2 Hepatic angiosarcomas represent 2% of primary falecido 10 meses após a cirurgia. Este caso exemplifica a dificuldade do diagnóstico do baseado apenas nos exames exames baseado apenas nos angiosarcoma do diagnóstico dificuldade do exemplifica a caso cirurgia. Este a após meses falecido 10 angiossarcomas Os diagnóstico. o sugeriu nenhuma e abdominais computadorizadas tomografias duas realizadas Foram imagem. de rea é raramente entanto, No curativo. tratamento único o cirurgia a sendo reservado, prognóstico com agressivos muito tumores são à distância. lizada devido a doença irressecável ou disseminação Palavras-chave: como o angiossarcoma. É descrito o caso de doente do sexo feminino de 46 anos, previamente saudável sem exposição a carcinogé a exposição sem saudável previamente anos, 46 de feminino sexo do doente de caso o descrito É angiossarcoma. o como neos, refere queixas de dor abdominal durante dois meses. O estudo analítico revelou elevação da gama glutamil transferase e lac descrita fígado, do lobo direito grandes dimensões no de lesão nodular abdominal evidenciou uma ecografia a e desidrogenase tato como hemangioma. Um mês mais tarde, realizou uma tomografia computadorizada abdominal que revelou aumento dasdimensões e direita hepatectomia a submetida foi doente A hemangioma. de imagiológicas características mantendo cm), 20 para (13,5 lesão da a histologia revelou a Após presença cirurgia, de uma angiossarcoma. tomografia por emissão de positrões-tomografia computadori paclitaxel, tendo quimioterapia paliativa com radioterapia e submetida a foi doente A óssea. hepática e evidenciou metastização zada radiotherapy, but radiotherapy, died 10 months after This surgery. case shows how difficult it is to diagnose hepatic angiosarcoma relying only on Two abdominal computedimaging tomography findings. -scans were performed and none Angiosarcoma suggested is this diagnosis. due feasible rarely is it However, available. treatment curative only the is Surgery prognosis. adverse an with tumour aggressive very a to unresectable disease or distant . Keywords: Apesar do diagnóstico de hemangioma ser habitualmente simples e baseado na ecografia, a presença de sintomas, o crescimento rápido ou a presença de atipias imagiológicas devem-nos fazer considerar a hipótese de outras entidades, algumas delas malignas findings should make us consider other diagnoses, including malignant tumorsmonths. two such for pain abdominal with as presented who carcinogens angiosarcomas.to exposure of We history a without describefemale 46-year-old healthy thepreviously case of a described ultrasound Abdominal levels. dehydrogenase lactate and transferase gamma-glutamyl elevated revealed work-up Diagnostic a large nodular in hemangioma. the a right of lobe characteristics of imaging the typical liver maintaining described cm, as 20 a to hemangioma. 13.5 One from month grown a later, had computed which tomography-scan lesion, was same the revealed and made A right hepatectomy was performed and pathology revealed an angiosarcoma. After surgery, a positron emission tomography-com puted tomography scan showed hepatic and metastasis. The patient started taxane-based and lumbar palliative are usually diagnosed based on ultrasound Thefindings. presence of symptoms, rapid growth or atipical imagiological Autor correspondente: Ana Rita Garcia. [email protected] Ana Rita Garcia. [email protected] Autor correspondente: Angiosarcoma Hepático e Hemangioma: Um Diagnóstico Um Diagnóstico Hepático e Hemangioma: Angiosarcoma Desafiante Diferencial Challenging Differential Diagnosis Differential Challenging Hepatic Angiosarcoma Masquerading as Hemangioma: A A as Hemangioma: Masquerading Angiosarcoma Hepatic ABSTRACT Recebido: 23 de dezembro de 2016 - Aceite: 03 de maio de 2017 | Recebido: 23 de dezembro de 2016 - 1. Serviço de Oncologia Médica. Instituto Português de Oncologia de Coimbra. Coimbra. Portugal. A. Centro Hospitalar e Universitário de Coimbra. Coimbra. Portugal. 2. Serviço de Cirurgia 3. Departamento de Cirurgia. Faculdade de Medicina. Universidade de Coimbra. Coimbra. Portugal.  atypical hepatocellular , hypervascular metastases a to leading , epithelioid hepatic and diagnosis. differential challenging more frequently. Due to low incidence and insidious course, insidious and incidence low to Due frequently. more diagnosis is often include hepatic delayed. similar to cavernous hemangiomas, Radiological findings may than fibrosarcoma,leyomiosarcoma. malignant fibrohistiocytomawith abdominal pain, and fatigue and weight loss occurring between the ages of 50 and 70 and risk factors may include may factors risk and 70 and 50 of ages the between chemical carcinogens (thorium dioxide, vinyl chloride and ). mesenchymal tumour of the liver, and is more frequent INTRODUCTION hepatic tumours, RESUMO Ana Rita GARCIA Acta Med Port 2017 Oct;30(10):750-753

CASO CLÍNICO alpha- and B andCviruswerenegative. 19-9 (CA) fetoprotein). antigen carbohydrate (CEA), antigen (carcinoembryonic normal were markers 4xULN, Tumour and (ULN) normal of limit upper x (5 elevated were levels (LDH) dehydrogenase lactate and (GGT) transferase Gamma-glutamyl normal. were levels phosphatase alkaline and transaminases Bilirubin, tests. masses or signs ofportalhypertension. nopalpable were There drugs. inflammatory anti- nonsteroidal with relief partial and position, antalgic right or irradiation upper without tenderness, and epigastric and quadrant exam pain analog cardiopulmonary verbal 10, normal - (0 scale), 5 at rated pain 0, of Status of higherdensityinterspersedwithhypodenseareas heterogeneous hepatic mass in the right lobe of the liver, with areas 1 Figure Figure 2 A n lrsud eeld htrgnos bly and bulky heterogeneous, a revealed ultrasound An clotting and count blood normal a revealed tests Blood –CT scanwithprogressive centripetalenhancementduring portalphase( – boia C sa (eoe otat diitain - administration) contrast (before scan CT Abdominal

Human immunodeficiency Garcia AR, etal.Hepaticangiosarcomamasqueradingashemangioma, ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R

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he wes svn yls, ih tbe ies o CT- on disease stable with cycles), (seven weeks three started mg/m patient 175 paclitaxel the with radiotherapy, chemotherapy palliative After T3). to C6 vertebrae. from thoracic week, 1 first / fr 5 / the Gy (20 started was in radiotherapy Palliative lesion lytic and lesions two months after surgery described three hepatic metastatic atypical 45 andpancytokeratins AE1/AE3. 20 than more mitosis, positivity to CD31 and CD34 and negativity to with HMB cells, of consisting pleomorphic hepatic margins, hepatic a surgical free showed with specimen angiosarcoma surgical was hepatectomy The right performed. a pain, abdominal patient’s the 3). (Fig. hemangioma cm, 20 identified, nodular was same lesion the and performed was CT-scan another late phases (Fig.s1,2). and portal arterial, with lesion, this assess further to tomography (CT) scan with contrast injection was performed computed triphasic abdominal An disease. liver chronic or the liver, of described as lobe a hemangioma, with right no signs of the steatosis in wide, cm 13.5 mass, hyperechoic to be suspicious of hepatic angiosarcoma if a patient has patient a if angiosarcoma hepatic of suspicious be to important is it but symptoms, or signs pathognomonic no especially in a young female patient such as ours. There are DISCUSSION patient diedafter10monthsinitialdiagnosis. from week, 1 / T11fr Unfortunately,started. were L4) to the 5 / Gy (20 radiotherapy antalgic and medication analgesic lumbar first opioid D, vitamin and the calcium Bisphosphonates, vertebrae. in fracture pathological a revealed scan later,CT-lumbar a pain, back lower of complaints following neuropathy.Twomonths 1 grade and neutropenia 1 grade anaemia, and alopecia 2 grade included Toxicities scan. B psto eiso tmgah (E)C performed (PET)-CT tomography emission positron A pain, abdominal worsening to due later, month One ie agoacm i a ae om f eai cancer, hepatic of form rare a is angiosarcoma Liver Acta MedPort2017Oct;30(10):750-753 A ) andlatephase ( Due to this significant growth and growth significant this to Due B ) n cniee a hepatic a considered and 2 every

CASO CLÍNICO Liver Open :24-32. 1,4,13 2,10,11 We chose 1,2,7 Therefore, the role of 9 Malignant vascular tumors of the liver: J Radiol Case Rep. 2014;8 Our patient was treated with seven seven with treated was patient Our Liver angiosarcoma, a rare liver malignancy, 12 2,4,13

Acta Med Port 2017 Oct;30(10):750-753 Port 2017 Acta Med 6,9 However, in case series, percutaneous was 2 Buetow PC, Buck JL, Ros PR. Shalini T, Archana R, Arvind A, Sarin S. Angiosarcoma of the liver: The authors declare that the procedures were followed use in protocols the followed having declare authors The All authors report no conflict of interest. This research received no specific grant from any The diagnosis can be difficult even with biopsy. Because surgery is rarely possible, systemic treatment 904. Chin-Ying C, Cheng-cheng H, Chun-nan Y, Huang-yang C, W, Jui-Teng Chan-Siu C, et al. presented with intra-abdominal due to rupture - a case report. J Surg Oncol. 2012;10:23 World radiologic- pathologic correlation. Radiographics. 1994;14:153-66. imaging of a rare salient entity. 4. 5. 6. on CT-scan, despite worsening of clinical condition. on CT-scan, Angiosarcoma is radioresistent and radiotherapy recommended, is except not for recurrence high to due recommended not is transplantation symptom control. rate and poor results. PROTECTION OF HUMANS AND ANIMALS according to the regulations established Research by the and Clinical Ethics Association. Medical Declaration of the World Committee and to the Helsinki CONFIDENTIALITY DATA at their working center regarding data patients’ publication. Informed consent was duly obtained from the patient. CONFLICTS OF INTEREST FUNDING SOURCES funding agency in the public, commercial, or not-for-profit sectors. Typically, Typically, they demonstrate early followed by arterial progressive wash in of enhancement contrast, without wash hepatocellular to compared when feature distinctive a – out carcinoma. Misdiagnosis by fine-needle aspiration biopsy may occur due to the heterogeneity of malignant and vascular the presence tumours of necrotic zones, leading to high of rate inconclusive histological/cytological results. According to some authors, this should not be used due to of the risk bleeding and biopsy-induced liver metastases. or laparoscopy may be more accurate and safe. described as a safe procedure. controversial. still is angiosarcoma for sampling histological can be an alternative. However, recommended there are chemotherapy currently efficacious highly and used no widely are they regimens. because taxanes in vascular-derived tumours. Different schemes used may and a progression-free be survival (PFS) of 7.6 months recently. obtained was disease stable and toxicity tolerable with paclitaxel of cycles

752 Primary Our patient Our 8

7 R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m Garcia AR, et al. Hepatic angiosarcoma masquerading as hemangioma, as hemangioma, masquerading Hepatic angiosarcoma AR, et al. Garcia The natural history of a hepatic angiosarcoma that was difficult to – CT scan with early arterial enhancement – CT Okano A, Sonoyama H, Masano T, Taniguchi Ohana Y, M, Kusumi F, Hur CJ, Min BR, Lee YJ, Jang BK, Hwang JS, Kim ES, et al. Clinical Zhang JL, Zhang Hui XW, ZZ, YW, Zheng Du WJ, Li RM, et al. Our case is an example of the pitfalls of the differential It can be speculated whether a magnetic resonance The ultrasound revealed a hyperechoic mass in the liver the in mass hyperechoic a revealed ultrasound The eight cases. Korean J Gastroenterol. 2015;65:229-35. hepatic angiosarcoma and potential treatment options. J Gastroenterol Hepatol. 2014;29:906–11. et al. differentiate from . Intern Med. 2012;51:2899- courses of primary hepatic angiosarcoma: retrospective analysis of 3. 1. 2. REFERENCES can present as large solitary masses with heterogeneous appearance, intratumural haemorrhage necrosis, which can be and confused with malignant tumours. areas of diagnosis of focal hepatic lesions. Despite advances in CT technology, distinction between cavernous hemangioma and hepatic angiosarcoma is based sometimes only difficult in when imaging findings. Hepatic hemangiomas that they were there two and months she later, would have not been subjected to surgery. imaging (MRI) could have been useful. was If suspected angiosarcoma in an MRI, the diagnosed patient with metastatic could disease have (or not), been knowing now our patient. Worsening symptoms and rapid growth of the lesion led to a right hepatectomy. described this lesion again as a hemangioma. With multi- hemangioma and of misdiagnosis imaging, CT-scan phasic angiosarcoma is not as common as before the introduction of this technique. this Unfortunately, was not the case with recent EASL guidelines on benign liver tumors. liver benign on guidelines EASL recent had no history of chronic , exam but This performed. the being CT-scan significant a led lesion the of size and a presumptive diagnosis of hemangioma was made. are they if ultrasound by diagnosed are hemangiomas Most small (< 3 cm) and occur in a healthy liver, according to rapidly worsening symptoms, even with normal liver function liver normal with even symptoms, worsening rapidly test and normal tumor markers. Figure 3

CASO CLÍNICO ecie icuig gsritsia smtm and symptoms well gastrointestinal are intoxication including: paracetamol described of common most consequences The Portugal. in marketed still analgesic aniline oritsderivatives. and nitrites sulfonamides, anesthetics, local dapsone, are associated frequently most the these, other Among substances. or drugs of doses excessive from resulting usually Acta MedPort2017Oct;30(10):753-756 Recebido: 22dejaneiro de2017- Aceite: 27demarço2017 |  3. ServiçodeNefrologia.CentroHospitalar Vila NovadeGaia-Espinho.Vila NovadeGaia.Portugal. 2. ServiçodeMedicinaInterna.CentroHospitalar deVila NovadeGaia-Espinho.Vila NovadeGaia.Portugal. 1. ServiçodeDermatologia.HospitalSanta Maria.CentroHospitalarLisboaNorte.Lisboa.Portugal. * value beingcalledmethemoglobinemia. level hemoglobin total the of 1% - 0% is methemoglobin of range normal The oxygen. bind reversibly cannot it means which state, oxidized the INTRODUCTION Catarina QUEIRÓS 10. 9. 8. 7. ABSTRACT RESUMO Caso Clínico Metemoglobinemia Após IngestãodeParacetamol: Um Report Methemoglobinemia After ParacetamolIngestion: A Case Co-primeirosautores. Autor correspondente:CatarinaQueiró[email protected] Keywords: with normalizationofmethemoglobinlevels. color, her in improvement clear a had patient the symptoms of onset the after days nine reassessment on and discontinued was Paracetamol aminotransferase. alanine and bilirubin total in increase an showing biochemistry with levels, methemoglobin in increase symptoms. diffuseother a without of tone, appearance skin the brownish noticing after hospital the an revealed analysis blood Arterial ease undergoing hemodialysis who was self-medicated with acetaminophen for seven days (at a daily dose of 3 g); the patient went to dis polycystic dominant autosomal with patient a reported. describes been report have case paracetamol This with associated cases three only Todate, drugs. this certain by intoxication after acquired mainly condition, fatal potentially a is Methemoglobinemia Palavras-chave: sintomas adoenteapresentavaumaclaramelhoriadacorpele,comnormalizaçãodosvaloresdemetemoglobina. dos início o após dias nove reavaliação na e suspensa, foi alanina. paracetamol da Ade aminotransferase toma da e total bilirrubina da aumento um apresentado bioquímica a tendo metemoglobina, da aumento revelou sem arterial sangue acastanhada, de sintomas. Aanálise difusamente outros cutânea tonalidade uma de aparecimento o notado ter após hospital ao recorreu doente a g); 3 de renal poliquística autossómica dominante, sob hemodiálise, que se automedicou com paracetamol durante sete dias (numa dose diária até à data estão relatados apenas três casos associados à toma de paracetamol. Este caso clínico descreve uma doente com doença fármacos; determinados por intoxicação após adquirida maioritariamente fatal, potencialmente condição uma Aé metemoglobinemia ehmgoi rfr t hmgoi wt is rn in iron its with hemoglobin to refers Methemoglobin preoperatively was malignancy which in hemangioma cavernous of MRimaging. and Radiology. CT 2002;222:667-73. at findings angiosarcoma: hepatic Primary LJ. J tumours. liver benign of Hepatol. 2016;65:386-98. management the on Guidelines Practice hepatic angiosarcoma. Ann Oncol. primary stage advanced of outcomes treatment and features Shigeaki H, Joji K, TakehiroJoji H, al. Shigeaki et Oe J, H, AKotani Kurooka A, H, case Burgart K, Notohara MS, Kuo CD, Johnson JG, Fletcher T, Koyama Clinical EASL (EASL). Liver the of Study the for Association European SH, Cheon SY, Rha HR, Kim aaeao i crety h ol aniline-derivate only the currently is Paracetamol The vast majority of methemoglobinemias are acquired Acetaminophen/adverse effects; Methemoglobinemia

Acetaminofeno/efeitos adversos;Metemoglobinemia Queirós C,etal.Methemoglobinemiaafterparacetamolingestion, *  1 , PedroSALVADOR 2009; ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R 1 20:780-7. o JK Roh with any increase above this above increase any with , ak YN Park ▪

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3 13. 12. 11. eiae wt aeyslclc cd clim acetate/ calcium acid, magnesium carbonate,quetiapine andhydroxyzine. acetylsalicylic with medicated cognitive mild on chronically a was She dementia. vascular to as due impairment disease well as kidney months 30 for polycystic hemodialysis dominant autosomal of CLINICAL CASE drug. this of complication serious potentially but rare a ingestion, paracetamol from resulting myocardial methemoglobinemia of case a acute hypoglycemia. injury; and pericarditis necrosis; kidney acute hepatotoxicity; , DanielaLOPES 2010;16:61-6. adults. in angiosarcoma hepatic primary for resection the improve significantly metastasectomy outcome. Ann Oncol. and paclitaxel weekly YM hepatic mimic not do hemangioma. CT helical contrast-enhanced multiphasic on excluded byFDG-PET. Ann NuclMed.2008;22:327-30. Zhou YM, Li B, Yin ZF, Xu F, Wang B, Xu W, et al. et W, Xu B, F,WangZF, Xu Yin B, Li YM, Zhou I, Ray-Coquard Italiano A, N, Penel findings angiosarcoma: Hepatic SC. Rankin RL, Baron MS, Peterson Our patient came to the hospital due to the appearance the to due hospital the to came patient Our a was patient Our e al. et , Acta MedPort2017Oct;30(10):753-756 eattc nisroa: oouii-ae regimens, -based angiosarcomas: Metastatic AJR Am JRoentgenol.2000 3 2012; 78-year-old 23:517-23. Chaigneau L Chaigneau woman ; 175:165-70. 3 , ee e report we Here Delcambre C Delcambre ih history a with Results of hepatic of Results e Si Monit. Sci Med , Robin -

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