DOCSLIB.ORG

Vascular Tumors of the Iris in 45 Patients the 2009 Helen Keller Lecture

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Vascular Tumors of the Iris in 45 Patients the 2009 Helen Keller Lecture CLINICAL SCIENCES Vascular Tumors of the Iris in 45 Patients The 2009 Helen Keller Lecture Jerry A. Shields, MD; Carlos Bianciotto, MD; Brad E. Kligman, BS; Carol L. Shields, MD Objective: To report on a series of vascular tumors of temic involvement occurred in a child. Of the 41 eyes the iris. with iris racemose hemangioma, none showed systemic involvement. Of all 54 eyes, transient hyphema was the Design: Noncomparative case series. A retrospective medi- main complication, found at some point in 30% or more cal record review of all patients with an iris vascular tu- of each affected eye except for iris capillary and race- mor was performed to identify the clinical features and de- mose hemangioma. Surgical resection was necessary in velop a simple classification of these lesions. Included were 1 cavernous hemangioma and 1 varix. The remainder were demographics, clinical features, systemic associations, com- managed with observation. plications, management, and histopathology. Conclusions: There are now well-documented ex- Results: There were 54 eyes in 45 patients with an iris amples of iris racemose hemangioma, cavernous heman- vascular tumor. These were categorized as racemose he- gioma, capillary hemangioma, varix, and microheman- mangioma (41 eyes: 29 simple and 12 complex), cav- giomatosis. Transient hyphema is the main complication. ernous hemangioma (3 eyes: 2 localized and 1 sys- Observation is usually advised. Most are solitary lesions temic), capillary hemangioma (1 eye, localized), varix (3 confined to the iris and some (cavernous hemangioma eyes, localized), and microhemangiomatosis (6 eyes, and microhemangiomatosis) can have important sys- localized). The hemangiomas occurred in adults at a temic associations. median age of 55 years, whereas capillary hemangioma occurred in infancy and cavernous hemangioma with sys- Arch Ophthalmol. 2010;128(9):1107-1113 HE EXISTENCE OF TRUE IRIS In our literature review and personal vascular tumors was once experience, there have been genuine questioned. In 1972, cases of iris vascular tumors and there Ferry1 reviewed reported are some that remain controversial.2-52 cases of histopathologi- In a recent case report of an iris varix, Tcally proven iris and ciliary body heman- Broaddus and associates3 provided a giomas on file at the Armed Forces Insti- review on iris vascular tumors. The pur- tute of Pathology and other sources. He pose of this contribution is to describe a came to the surprising conclusion that series of iris vascular tumors seen on an all cases had been misdiagnosed histo- ocular oncology service and to review pathologically and they actually repre- the English language literature of the sented vascular melanoma, juvenile subject. xanthogranuloma, florid iris neovascu- larization, or other lesions.1 He raised METHODS doubts about the existence of iris hemangioma and proposed that most reported cases in humans were not hem- This study was approved by the institutional angiomas. Since that time, however, review board of the Wills Eye Institute of there have been several reports of iris Thomas Jefferson University. A classification 2-34 was designed as listed below. Although not all hemangiomas. of these conditions represent true tumors in the Author Affiliations: Oncology Since the inception of our oncology classic sense of a distinct mass, they were in- Service Wills Eye Institute, service in 1974, we have coded and filed cluded in the classification of vascular tumors Thomas Jefferson University, all cases of iris vascular tumors and in this communication, in keeping with clas- Philadelphia, Pennsylvania. pseudotumors examined by our team. sic usage in the literature. (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 9), SEP 2010 WWW.ARCHOPHTHALMOL.COM 1107 Downloaded from www.archophthalmol.com at , on December 28, 2010 ©2010 American Medical Association. All rights reserved. Table 1. Patients With Vascular Tumors of the Iris Table 3. Referring Diagnosis in 54 Eyes of 45 Patients With Iris Hemangioma No. Referring Diagnosis No. (%) Tumor Type Patients Eyes Bilateral Iris Racemose 35 41 6 Hemangioma 8 (15) Cavernous 3 3 0 Melanoma 2 (4) Capillary 1 1 0 Nevus 5 (9) Varix 3 3 0 Cyst 1 (2) Microhemangiomatosis 3 6 3 Prominent vessels nonspecific 6 (11) Total 45 54 9 Ciliary body melanoma 2 (4) Choroidal melanoma 2 (4) No diagnosis submitted 27 (50) Table 2. Vascular Tumors of the Iris by Category RESULTS Tumor Type No. of Eyes Racemose 41 There were 54 eyes in 45 patients with an iris vascular Simple 29 tumor (Table 1). The tumors were classified as race- Complex 12 mose hemangioma in 41 eyes of 35 patients (29 simple Cavernous 3 and 12 complex), cavernous hemangioma in 3 eyes of 3 Localized 2 Systemic 1 patients (2 localized and 1 systemic), capillary heman- Capillary 1 gioma in 1 eye of 1 patient, localized varix in 3 eyes of 3 Localized 1 patients, and microhemangiomatosis in 6 eyes of 3 pa- Systemic 0 tients (Table 2). Histopathologic confirmation of the Varix 3 diagnosis was available in a single case of solitary cav- Localized 3 ernous hemangioma and a single case of varix, both fol- Systemic 0 Microhemangiomatosis 6 lowing removal of the lesion by iridectomy. The referral Localized 6 diagnoses are shown in Table 3. The diagnosis of iris Systemic 0 hemangioma was suspected in 8 of the 54 eyes and no Total 54 diagnosis was submitted in 27 (50%) cases. Uveal mela- noma (iris, ciliary body, and choroid) was the referral diagnosis in 6 cases. Based on our prior observations and literature review, we The demographic data are shown in Table 4. The me- classified iris vascular tumors and pseudotumors into the fol- dian patient age was 55 years at original examination in lowing categories: all cases with the exception of iris capillary hemangioma 1. Racemose hemangioma (0.1 year) and cavernous hemangioma with systemic in- Simple volvement (7 years). All but 1 tumor was found in white Complex patients. The iris color was blue or green in 48 cases (89%). 2. Cavernous hemangioma The intraocular pressure was under 22 mm Hg except for Localized 1 patient with open-angle glaucoma and a pressure of 37 Systemic mm Hg. Ectropion was absent in all cases. Corectopia was 3. Capillary hemangioma Localized found in 1 case of iris varix and 1 case of solitary cavern- Systemic ous hemangioma. Individual tumor features are shown in 4. Varix Table 4 and Table 5, and specific information per tumor Localized classification will be discussed. Systemic Racemose hemangioma (arteriovenous communica- 5. Microhemangiomatosis tion) was the most common lesion in our series, account- Localized ing for 35 of the 45 patients. In 29 patients, it was uni- Systemic lateral and in 6 patients bilateral. It appeared as a solitary Using this classification, a retrospective medical record review was large tortuous iris blood vessel, approximately twice the conducted on all patients coded with 1 of these diagnoses, and normal caliber and located within the iris stroma. The the patient, ocular, and tumor features were evaluated. The pa- racemose hemangioma intertwined in a sinuous fashion tient features that were recorded included age at diagnosis, race, within the iris stroma from iris root to the pupillary bor- sex, symptoms, and presence of related systemic hemangiomas der and then back to the root.8-14 In 29 patients, the ra- or syndromes. The ocular features recorded included iris color, cemose hemangioma was classified as simple, character- visual acuity, and intraocular pressure. The tumor features that ized by a single tortuous vessel (Figure 1A and B). Six were recorded included referring diagnosis, tumor quadrant lo- cation, size (base in millimeters and clock-hours), blood vessel of the simple racemose hemangiomas were bilateral. In feeding from the sclera or iris, and intrinsic blood vessels. Re- 12 cases, it was classified as complex, characterized by lated ocular findings such as glaucoma, cataract, hyphema, and multiple ramifying, intertwining blood vessels forming corneal changes were listed. Medical and surgical intervention a sessile vascular mass (Figure 1C and D).13 The com- were reviewed and the ultimate outcome was listed. plex cases were all unilateral. Racemose hemangioma was (REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 9), SEP 2010 WWW.ARCHOPHTHALMOL.COM 1108 Downloaded from www.archophthalmol.com at , on December 28, 2010 ©2010 American Medical Association. All rights reserved. Table 4. Iris Hemangioma in 54 Eyes of 45 Patients No. (%) by Hemangioma Type Racemose Cavernous Capillary, Microhemangiomatosis, Simple Complex Solitary Systemic Localized Varix Localized Characteristic (n=29) (n=12) (n=2) (n=1) (n=1) (n=3) (n=6) Age, median (mean, range), ya 56 (61, 30-82) 55 (52, 28-82) 56 (56, 55-56) 7 (7, 7-7) 0.1 (0.1, 0.1-0.1) 59 (57, 39-78) 70 (70, 66-73) Racea White 23 (51) 12 (27) 2 (4) 1 (2) 1 (2) 2 (4) 3 (7) African American 0 0 0 0 0 0 0 Hispanic 0 0 0 0 0 1 (2) 0 Asian 0 0 0 0 0 0 0 Sexa F 14 (31) 5 (11) 1 (2) 1 (2) 1 (2) 0 3 (7) M 9 (20) 7 (16) 1 (2) 0 0 3 (7) 0 Iris colorb Blue 24 (44) 10 (19) 2 (4) 1 (2) 1 (2) 1 (2) 6 (11) Green 2 (4) 1 (2) 0 0 0 0 0 Brown 2 (4) 0 0 0 0 2 (4) 0 Hazel 1 (2) 1 (2) 0 0 0 0 0 Visual acuityb 20/20-20/40 25 (46) 12 (22) 2 (4) 1 (2) 0 3 (6) 6 (11) 20/50-20/100 4 (7) 0 0 0 0 0 0 20/200-HM 0 0 0 0 1 (2) 0 0 Intraocular pressure, median 24 (16, 10-37) 15 (15, 10-19) 18 (18, 12-23) FTNc FTNd 17 (17, 15-19) 15 (14, 12-17) (mean, range), mm Hgb Abbreviations: FTN, finger tension normal; HM, hand motions.
Load more

Recommended publications
  • Tumors and Tumor-Like Lesions of Blood Vessels 16 F.Ramon
    16_DeSchepper_Tumors_and 15.09.2005 13:27 Uhr Seite 263 Chapter Tumors and Tumor-like Lesions of Blood Vessels 16 F.Ramon Contents 42]. There are two major classification schemes for vas- cular tumors. That of Enzinger et al. [12] relies on 16.1 Introduction . 263 pathological criteria and includes clinical and radiolog- 16.2 Definition and Classification . 264 ical features when appropriate. On the other hand, the 16.2.1 Benign Vascular Tumors . 264 classification of Mulliken and Glowacki [42] is based on 16.2.1.1 Classification of Mulliken . 264 endothelial growth characteristics and distinguishes 16.2.1.2 Classification of Enzinger . 264 16.2.1.3 WHO Classification . 265 hemangiomas from vascular malformations. The latter 16.2.2 Vascular Tumors of Borderline classification shows good correlation with the clinical or Intermediate Malignancy . 265 picture and imaging findings. 16.2.3 Malignant Vascular Tumors . 265 Hemangiomas are characterized by a phase of prolif- 16.2.4 Glomus Tumor . 266 eration and a stationary period, followed by involution. 16.2.5 Hemangiopericytoma . 266 Vascular malformations are no real tumors and can be 16.3 Incidence and Clinical Behavior . 266 divided into low- or high-flow lesions [65]. 16.3.1 Benign Vascular Tumors . 266 Cutaneous and subcutaneous lesions are usually 16.3.2 Angiomatous Syndromes . 267 easily diagnosed and present no significant diagnostic 16.3.3 Hemangioendothelioma . 267 problems. On the other hand, hemangiomas or vascular 16.3.4 Angiosarcomas . 268 16.3.5 Glomus Tumor . 268 malformations that arise in deep soft tissue must be dif- 16.3.6 Hemangiopericytoma .
    [Show full text]
  • Cavernous Hemangioma of the Gallbladder: a Case Report
    pISSN 2384-1095 iMRI 2019;23:264-269 https://doi.org/10.13104/imri.2019.23.3.264 eISSN 2384-1109 Cavernous Hemangioma of the Gallbladder: a Case Report Jae Hwi Park1, Jeong Sub Lee1, Guk Myung Choi1, Bong Soo Kim1, Seung Hyoung Kim1, JeongJae Kim1, Doo Ri Kim1, Chang Lim Hyun2, Kyu Hee Her3 1Department of Radiology, Jeju National University Hospital, Jeju National University School of Magnetic resonance imaging Medicine, Jeju, Korea 2Department of Pathology, Jeju National University Hospital, Jeju National University School of Medicine, Jeju, Korea 3Department of Surgery, Jeju National University Hospital, Jeju National University School of Medicine, Jeju, Korea Case Report Cavernous hemangioma of the gallbladder is an extremely rare benign tumor. The tumor has only a few cases being reported in literature. However, to the best of our knowledge, no reports focusing on the MRI findings of cavernous hemangioma of the Received: April 23, 2019 gallbladder have been published. This study reports a case of gallbladder hemangioma Revised: June 10, 2019 with pathologic and radiologic reviews, including MRI findings. Accepted: July 2, 2019 Correspondence to: Keywords: Cavernous hemangioma; Gallbladder; Magnetic resonance imaging Jeong Sub Lee, M.D. Department of Radiology, Jeju National University Hospital, Jeju National University School of Medicine, 15 Aran 13-gil, INTRODUCTION Jeju-si, Jeju-do 63241, Korea. Tel. +82-64-717-1371 Cavernous hemangioma of the gallbladder is an extremely rare benign tumor (1). Fax. +82-64-717-1370 Hemangioma occurs in several organs, including the liver, brain, lungs and skeletal E-mail: [email protected] muscle. It is the most common benign tumor in the liver, in which cavernous hemangioma represents the majority of tumors (2, 3).
    [Show full text]
  • Benign Hemangiomas
    TUMORS OF BLOOD VESSELS CHARLES F. GESCHICKTER, M.D. (From tke Surgical Palkological Laboratory, Department of Surgery, Johns Hopkins Hospital and University) AND LOUISA E. KEASBEY, M.D. (Lancaster Gcaeral Hospital, Lancuster, Pennsylvania) Tumors of the blood vessels are perhaps as common as any form of neoplasm occurring in the human body. The greatest number of these lesions are benign angiomas of the body surfaces, small elevated red areas which remain without symptoms throughout life and are not subjected to treatment. Larger tumors of this type which undergb active growth after birth or which are situated about the face or oral cavity, where they constitute cosmetic defects, are more often the object of surgical removal. The majority of the vascular tumors clinically or pathologically studied fall into this latter group. Benign angiomas of similar pathologic nature occur in all of the internal viscera but are most common in the liver, where they are disclosed usually at autopsy. Angiomas of the bone, muscle, and the central nervous system are of less common occurrence, but, because of the symptoms produced, a higher percentage are available for study. Malignant lesions of the blood vessels are far more rare than was formerly supposed. An occasional angioma may metastasize following trauma or after repeated recurrences, but less than 1per cent of benign angiomas subjected to treatment fall into this group. I Primarily ma- lignant tumors of the vascular system-angiosarcomas-are equally rare. The pathological criteria for these growths have never been ade- quately established, and there is no general agreement as to this par- ticular form of tumor.
    [Show full text]
  • Cavernous Hemangiomas of the Orbit: Value of CT, Angiography, and Phlebography
    741 Cavernous Hemangiomas of the Orbit: Value of CT, Angiography, and Phlebography Mario Savoiardo 1, Liliana Strada, and Angelo Passerini Neuroradiologic studies performed in 18 cases of surgically magnified x 2 in nine cases. In four cases selective extern al carotid verified intraorbital cavernous hemangioma are reported. Skull injection was performed. CT was available in the last 14 cases. It films usually showed enlargement of the orbit and evidence of was usually performed with an EMI 1010 scanner with 5 mm cuts, soft-tissue mass. Phlebography rarely demonstrated filling of the a 320 x 320 matrix, and , when feasible, with coronal cu ts. The cavernous hemangioma or enlarged draining veins. On angiog­ radiologic findings were compared with the description of the sur­ raphy, in addition to displacement of vessels, pooling of contrast gical intervention. medium in the cavities of the hemangioma was observed in more than half the cases. Computed tomography (CT) demonstrated a rounded, hyperdense, enhancing lesion usually in the superior Results segment of the intraconal space. Although CT may be sufficient Skull films were completely normal in three cases, and in two for planning the surgical approach, in most cases a combination of CT and angiography will give better, more specific preopera­ others only minimal enlargement of the involved orbit was observed. Definite en largement was found in 11 , often with evid ence of tive diagnosis. increased soft-tissue density. In two cases, enlargement and poor definition of the margin of the optic canal were observed. In one of Cavernous hemangiomas are by far the most common vascular these the cavernous hemangioma, 4 mm in diameter, had grown malformation of the orbit and are among the most common of all the within the optic nerve at the apex of the orbit.
    [Show full text]
  • View Open Access Histological Variants of Cutaneous Kaposi Sarcoma Wayne Grayson1 and Liron Pantanowitz*2
    Diagnostic Pathology BioMed Central Review Open Access Histological variants of cutaneous Kaposi sarcoma Wayne Grayson1 and Liron Pantanowitz*2 Address: 1Histopathology Department, Ampath National Laboratory Support Services, Johannesburg, South Africa and 2Department of Pathology, Baystate Medical Center, Tufts University School of Medicine, Springfield, Massachusetts, USA Email: Wayne Grayson - [email protected]; Liron Pantanowitz* - [email protected] * Corresponding author Published: 25 July 2008 Received: 23 July 2008 Accepted: 25 July 2008 Diagnostic Pathology 2008, 3:31 doi:10.1186/1746-1596-3-31 This article is available from: http://www.diagnosticpathology.org/content/3/1/31 © 2008 Grayson and Pantanowitz; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS) lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage); morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented. Introduction progression, (2) KS variants alluded to in the older litera- Kaposi sarcoma (KS) is a vascular lesion of low-grade ture, (3) more recently described KS variants, and (4) KS malignant potential that presents most frequently with lesions as a consequence of therapy.
    [Show full text]
  • Massive Cavernous Lymphangioma of the Breast and Thoracic Wall: Case Report and Literature Review
    147 Lymphology 39 (2006) 147-151 MASSIVE CAVERNOUS LYMPHANGIOMA OF THE BREAST AND THORACIC WALL: CASE REPORT AND LITERATURE REVIEW U. Krainick-Strobel, B. Krämer, R. Walz-Mattmüller, E. Kaiserling, C. Röhm, A. Bergmann, M. Hahn, D. Wallwiener, S. Brucker University of Tübingen Medical School, Department of Obstetrics and Gynecology (UK S,BK,CR, AB,MH,DW,SB), Breast Center and Pathology Institute (RW-M,EK), Tübingen, Germany ABSTRACT logical examination reveals multiple protrusions lined with thin endothelium. Lymphangiomas are benign lesions but They are generally located in the head, neck are associated with high morbidity when they area (dorsally or laterally; 75%), the axilla become very large, occur in critical locations, (25%), mediastinum or, more rarely, in the or when surgically removed, develop secondary retroperitoneum, abdominal organs, skeleton wound infections. Almost all lesions require or scrotum. Cystic lymphangiomas are most surgical treatment. Complete excision is commonly diagnosed in young children. curative; however, relapses must be anticipated 50–65% of lymphangiomas are present clini- with incomplete excision. We report the case cally in the newborn, and 90% are apparent of a patient with a long history of massive by the age of 2 years (2). Lymphangiomas are cavernous lymphangioma of the breast and generally cavernous lesions and are very rare thoracic wall extending into the axilla in in the breast, especially in adults. Secondary whom complete excision was not possible. lymphangiomas after mastectomy and subsequent irradiation of the thoracic wall Keywords: breast, cavernous lymphangioma, have been described (3) and only isolated hemangioma, cystic lesions, vascular cases have been reported in the literature (4-7).
    [Show full text]
  • Giant Cavernous Hepatic Hemangioma Diagnosed Incidentally in a Perimenopausal Obese Female with Endometrial Adenocarcinoma: a Case Report
    ANTICANCER RESEARCH 36: 769-772 (2016) Giant Cavernous Hepatic Hemangioma Diagnosed Incidentally in a Perimenopausal Obese Female with Endometrial Adenocarcinoma: A Case Report TIVADAR BARA JR.1, SIMONA GURZU2, IOAN JUNG2, MIRCEA MURESAN2, JANOS SZEDERJESI3 and TIVADAR BARA1 Departments of 1Surgery, 2Pathology, and 3Intensive Care, University of Medicine and Pharmacy of Tirgu-Mures, Tirgu-Mures, Romania Abstract. Hemangiomas are the most common benign Macroscopically, LHs are hypervascular poorly tumors of the liver, considered giant when they exceed 50- circumscribed lesions. Microscopically, they consist of large 100 mm in diameter. In the present report, we present a case cavities filled with venous blood coming from the hepatic of a 5.2-kg hemangioma of the right hepatic lobe, with artery, lined by endothelial cells and separated by fibrous septa hemangiomatous foci in the left lobe, which was incidentally (1). Due to unreported malignant transformation of LHs, their diagnosed in a 53-year-old obese female hospitalized for slow growth and low risk for bleeding, simple observation of uterine bleeding. The computed tomographic scan and asymptomatic lesions is usually recommended (1). physical examination revealed a giant abdominal tumor and LHs can be single or multiple and their size can vary from hepatic hemangioma of the right hepatic lobe was suspected. a few millimeters to over 20 cm (5). The term 'giant Right hepatectomy and total hysterectomy with bilateral hemangioma' is commonly used for lesions larger than 4 cm ovariectomy was performed. The histological examination of in diameter (1-5). LHs over 10 cm are considered extremely the surgical specimens confirmed the extremely giant large or massive, and only occasional cases over 30 cm or cavernous hepatic hemangioma, and a synchronous pT1a weighing more than 2 to 3 kg have been reported (3, 4).
    [Show full text]
  • Hepatic Angiosarcoma Masquerading As Hemangioma
    Hepatic Angiosarcoma Masquerading as Hemangioma: A CASO CLÍNICO Challenging Differential Diagnosis Angiosarcoma Hepático e Hemangioma: Um Diagnóstico Diferencial Desafiante Ana Rita GARCIA1, João RIBEIRO1, Helena GERVÁSIO1, Francisco Castro e SOUSA2,3 Acta Med Port 2017 Oct;30(10):750-753 ▪ https://doi.org/10.20344/amp.8593 ABSTRACT Hemangiomas are usually diagnosed based on ultrasound findings. The presence of symptoms, rapid growth or atipical imagiological findings should make us consider other diagnoses, including malignant tumors such as angiosarcomas. We describe the case of a previously healthy 46-year-old female without a history of exposure to carcinogens who presented with abdominal pain for two months. Diagnostic work-up revealed elevated gamma-glutamyl transferase and lactate dehydrogenase levels. Abdominal ultrasound described a large nodular lesion in the right lobe of the liver described as a hemangioma. One month later, a computed tomography-scan was made and revealed the same lesion, which had grown from 13.5 to 20 cm, maintaining typical imaging characteristics of a hemangioma. A right hepatectomy was performed and pathology revealed an angiosarcoma. After surgery, a positron emission tomography-com- puted tomography scan showed hepatic and bone metastasis. The patient started taxane-based chemotherapy and lumbar palliative radiotherapy, but died 10 months after surgery. This case shows how difficult it is to diagnose hepatic angiosarcoma relying only on imaging findings. Two abdominal computed tomography -scans were performed and none suggested this diagnosis. Angiosarcoma is a very aggressive tumour with an adverse prognosis. Surgery is the only curative treatment available. However, it is rarely feasible due to unresectable disease or distant metastasis.
    [Show full text]
  • Tumor-Associated Protein Profiles in Kaposi Sarcoma and Mimicking
    International Journal of Molecular Sciences Article Tumor-Associated Protein Profiles in Kaposi Sarcoma and Mimicking Vascular Tumors, and Their Pathological Implications Kyoung Bun Lee 1, Kyu Sang Lee 2 and Hye Seung Lee 2,* 1 Department of Pathology, Seoul National University Hospital, Seoul 110-799, Korea 2 Department of Pathology, Seoul National University Bundang Hospital, Seongnam 463-707, Korea * Correspondence: [email protected]; Tel.: +82-31-787-7714; Fax: +82-31-787-4012 Received: 14 May 2019; Accepted: 26 June 2019; Published: 27 June 2019 Abstract: We investigated protein profiles specific to vascular lesions mimicking Kaposi sarcoma (KS), based on stepwise morphogenesis progression of KS. We surveyed 26 tumor-associated proteins in 130 cases, comprising 39 benign vascular lesions (BG), 14 hemangioendotheliomas (HE), 37 KS, and 40 angiosarcomas (AS), by immunohistochemistry. The dominant proteins in KS were HHV8, lymphatic markers, Rb, phosphorylated Rb, VEGF, and galectin-3. Aberrant expression of p53, inactivation of cell cycle inhibitors, loss of beta-catenin, and increased VEGFR1 were more frequent in AS. HE had the lowest Ki-67 index, and the inactivation rates of cell cycle inhibitors in HE were between those of AS and BG/KS. Protein expression patterns in BG and KS were similar. Clustering analysis showed that the 130 cases were divided into three clusters: AS-rich, BG-rich, and KS-rich clusters. The AS-rich cluster was characterized by high caveolin-1 positivity, abnormal p53, high Ki-67 index, and inactivated p27. The KS-rich cluster shared the features of KS, and the BG-rich group had high positive expression rates of galectin-3 and low bcl2 expression.
    [Show full text]
  • A 44-Year-Old Woman with Abdominal Pain Giant Pedunculated Cavernous Hepatic Hemangioma
    JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY CLINICAL CASE OF THE MONTH A 44-Year-Old Woman with Abdominal Pain Giant Pedunculated Cavernous Hepatic Hemangioma Caroline Smith, PA, Richard W. Hartsough, MD, Bradley Spieler, MD, Taylor Dickerson, MD, Catherine Hudson, MD, Fred Lopez, MD, Richard Marshall, MD, Adam Riker, MD Hemangiomas are the most common benign hepatic tumors in adults, but they are rarely pedunculated. To the best of our knowledge, less than 30 cases of giant pedunculated hepatic hemangiomas have been reported since 1985. This article focuses on a case of a woman who presented with mild epigastric pain and a large abdominal mass on imaging. INTRODUCTION Hepatic hemangiomas are the most common tumors of the liver CASE REPORT with an estimated prevalence of up to 20%.¹ Liver hemangiomas tend to be small and mostly asymptomatic, rarely requiring A 44 year-old obese woman with a past medical history of further treatment due to their benign nature and tendency iron deficiency anemia and abnormal uterine bleeding who towards slow growth.² They consist of large blood-filled cavities presented to her primary care provider for intermittent, lined by abnormal endothelial cells which are sustained by the cramping epigastric abdominal pain usually within 30 minutes hepatic arterial circulation.²,³ Hemangiomas mostly occur in of her last meal. She noted a weight gain of approximately 35 women between the ages of 40-60 years and are often found pounds over the prior year as well as occasional nausea and incidentally on abdominal ultrasound, computed tomography acid reflux that was somewhat improved with a proton pump (CT), or magnetic resonance imaging (MRI).¹,⁴ Although the inhibitor.
    [Show full text]
  • Vascular Tumors of the Iris in 45 Patients the 2009 Helen Keller Lecture
    CLINICAL SCIENCES Vascular Tumors of the Iris in 45 Patients The 2009 Helen Keller Lecture Jerry A. Shields, MD; Carlos Bianciotto, MD; Brad E. Kligman, BS; Carol L. Shields, MD Objective: To report on a series of vascular tumors of temic involvement occurred in a child. Of the 41 eyes the iris. with iris racemose hemangioma, none showed systemic involvement. Of all 54 eyes, transient hyphema was the Design: Noncomparative case series. A retrospective medi- main complication, found at some point in 30% or more cal record review of all patients with an iris vascular tu- of each affected eye except for iris capillary and race- mor was performed to identify the clinical features and de- mose hemangioma. Surgical resection was necessary in velop a simple classification of these lesions. Included were 1 cavernous hemangioma and 1 varix. The remainder were demographics, clinical features, systemic associations, com- managed with observation. plications, management, and histopathology. Conclusions: There are now well-documented ex- Results: There were 54 eyes in 45 patients with an iris amples of iris racemose hemangioma, cavernous heman- vascular tumor. These were categorized as racemose he- gioma, capillary hemangioma, varix, and microheman- mangioma (41 eyes: 29 simple and 12 complex), cav- giomatosis. Transient hyphema is the main complication. ernous hemangioma (3 eyes: 2 localized and 1 sys- Observation is usually advised. Most are solitary lesions temic), capillary hemangioma (1 eye, localized), varix (3 confined to the iris and some (cavernous hemangioma eyes, localized), and microhemangiomatosis (6 eyes, and microhemangiomatosis) can have important sys- localized). The hemangiomas occurred in adults at a temic associations.
    [Show full text]
  • Cavernous Haemangioma of the Orbit
    Eye (1989) 3, 90-99 Cavernous Haemangioma of the Orbit B. LEATHERBARROW, J. L. NOBLE, I. C. LLOYD Manchester Summary Cavernous haemangioma is considered to be the most common primary orbital tumour. We present 3 cases of cavernous haemangioma of the orbit managed by medial orbitotomy com· bined with medial orbital decompression. This approach was performed for lesions occurring medial to the optic nerve in an attempt to reduce the surgical morbidity reported following lat· eral orbitotomy or transcranial orbitotomy. Cavernous haemangioma, often cited as the based on the observation that progressive most common primary orbital tumour, expansion of the lesion eventually leads to occurs infrequently.l,2 As a cause of unilat­ optic nerve compression, extreme proptosis eral proptosis it is outnumbered by primary with the risk of corneal exposure, and greater congenital varices in a ratio of 3:1.2 Cavern­ difficulty in surgical removal. ous haemangioma is an acquired benign vas­ Harris and Jakobiec4 showed, however, cular tumour which occurs in adults and that surgery performed on patients who had a exhibits a slow growth pattern. Histologically tumour located deep in the orbit causing it is composed of large, dilated vascular chan­ choroidal folds, disc swelling, envelopment nels lined by flattened endothelial cells, and of the optic nerve, periosteum, or muscles, possesses a well-defined fibrous pseudo-cap­ had a greater risk of resulting in a post-opera­ sule. The capsule enables excision of the tive worsening of visual acuity. Tumours in entire lesion without fragmentation, follow­ this location are usually approached by a ing which recurrence does not usually occur.
    [Show full text]